name: Pathologic Stage IV Gastroesophageal Junction Adenocarcinoma AJCC v8

description: Stage IV includes: IVA: (T4a, N2, M0, Any G); (T4b, N0-2, M0, Any G); (Any T, N3, M0, Any G); IVB: (Any T, Any N, M1, Any G). T4a: Tumor invades the pleura, pericardium, azygos vein, diaphragm, or peritoneum. T4b: Tumor invades other adjacent structures, such as the aorta, vertebral body, or airway. N0: No regional lymph node metastasis. N1: Tumor with metastasis in one or two regional lymph nodes. N2: Tumor with metastasis in three to six regional lymph nodes. N3: Tumor with metastasis in seven or more regional lymph nodes. M0: No distant metastasis. M1: Distant metastasis. (AJCC 8th ed.)


name: Pathologic Stage IB Gastroesophageal Junction Adenocarcinoma AJCC v8

category: biolink:Disease biolink:DiseaseOrPhenotypicFeature biolink:BiologicalEntity biolink:ThingWithTaxon biolink:NamedThing

description: Stage IB includes: (T1a, N0, M0, G2); (T1b, N0, M0, G1-2); (T1b, N0, M0, GX). T1a: Tumor invades the lamina propria or muscularis mucosae. T1b: Tumor invades the submucosa. N0: No regional lymph node metastasis. M0: No distant metastasis. G1: Well-differentiated. GX: Grade cannot be assessed. G2: Moderately differentiated. (AJCC 8th ed.)


name: Refractory High Grade Burkitt-Like Lymphoma

description: High grade B-cell lymphoma Burkitt-like lymphoma resistant to treatment.


name: Recurrent High Grade Burkitt-Like Lymphoma

description: The reemergence of high grade B-cell lymphoma Burkitt-like lymphoma after a period of remission.


name: Stage II High Grade Adult Non-Hodgkin's Lymphoma


name: Stage II High Grade Burkitt-Like Lymphoma

description: Stage II NHL means involvement of two or more lymph node regions on the same side of the diaphragm (II) or localized involvement of a single associated extralymphatic organ or site and its regional lymph nodes with or without other lymph node regions on the same side of the diaphragm (IIE). Note: The number of lymph node regions involved may be indicated by a subscript (e.g., II3). (from PDQ)


name: High Grade B-Cell Non-Hodgkin's Lymphoma


name: Stage I High Grade Burkitt-Like Lymphoma

description: Stage I NHL means involvement of a single lymph node region (I) or localized involvement of a single extralymphatic organ or site (IE). (from PDQ)


name: Recurrent High Grade Adult Non-Hodgkin's Lymphoma


name: Stage IV High Grade Adult Non-Hodgkin's Lymphoma


name: Stage IV High Grade Burkitt-Like Lymphoma

description: Stage IV NHL means disseminated (multifocal) involvement of one or more extralymphatic sites with or without associated lymph node involvement or isolated extralymphatic organ involvement with distant (nonregional) nodal involvement. (from PDQ)


name: Stage III High Grade Adult Non-Hodgkin's Lymphoma


name: Stage I High Grade Adult Non-Hodgkin's Lymphoma


name: Stage III High Grade Burkitt-Like Lymphoma

description: Stage III NHL means involvement of lymph node regions on both sides of the diaphragm (III) that may also be accompanied by localized involvement of an extralymphatic organ or site (IIIE), by involvement of the spleen (IIIS), or both (IIIS+E). (from PDQ)


High Grade Burkitt-Like Lymphoma

description: A high grade B-cell lymphoma showing greater pleomorphism in nuclear size and shape than the classical Burkitt lymphoma.


name: Patchy sclerosis of the proximal phalanx of the 3rd toe

category: biolink:PhenotypicFeature biolink:DiseaseOrPhenotypicFeature biolink:BiologicalEntity biolink:ThingWithTaxon biolink:NamedThing


name: Stage IVB Esophageal Squamous Cell Carcinoma


chronic sphenoidal sinusitis

description: Inflammation of the sphenoid sinus that typically lasts beyond eight weeks. It is caused by infections, allergies, and the presence of sinus polyps or a deviated septum. Signs and symptoms include headache, nasal discharge, swelling in the face, dizziness, and breathing difficulties.


Benign Nasolacrimal Duct Neoplasm

description: A neoplasm that arises from the lacrimal duct and is characterized by the absence of atypical or malignant cytological and architectural features, and absence of invasive features or metastatic potential.


name: Hodgkin lymphoma, nodular sclerosis, cellular phase

description: A nodular sclerosis Hodgkin lymphoma characterized by the presence of lacunar cells, nodular growth, and the absence of fibrosis.


name: Unresectable Cervical Adenosquamous Carcinoma

description: Cervical adenosquamous carcinoma that is not amenable to surgical resection.


Vesicovaginal fistula

description: The presence of a fistula connecting the urinary bladder to the vagina.


Urogenital fistula

description: The presence of a fistula affecting the genitourinary system.


Rectoperineal fistula

description: The presence of a fistula between the perineum and the rectum.


Urethrovaginal fistula

description: The presence of a fistula between the vagina and the urethra.


H-type rectovestibular fistula

description: Rectovestibular fistula with a normal anus is known as H-type fistula or double termination of the alimentary tract.


Perineal fistula

description: The presence of a fistula between the bowel and the perineum.


Anoperineal fistula

description: The presence of a fistula (abnormal tunnel) between the anal canal and the perineum.


Vaginal fistula

description: The presence of a fistula of the vagina.


Urachus fistula

description: Persistence of the urachal canal with drainage of urine from the bladder through the persistent allantois canal to the umbilicus.


Rectourethral fistula

description: An abnormal connection (fistula) between the rectum and the urethra.


Rectovaginal fistula

description: The presence of a fistula between the vagina and the rectum.


Rectoureteral fistula

description: An abnormal connection (fistula) between the rectum and the ureter.


Rectovestibular fistula

description: A congenital malformation characterized by an abnormal connection (fistula) between the rectum and the vulval vestibule, at the lower aspect of the vaginal opening.


Rectal fistula

description: The presence of a fistula affecting the rectum.


Bladder fistula

description: The presence of a fistula connecting the urinary bladder to another organ or the skin. The fistula can involve the bowel, the vagina, or rarely, the skin.


Colovesical fistula

description: An aberrant, pathological communication between the colon and the bladder.


Urethral fistula

description: The presence of an abnormal connection between the urethra and another organ or the skin.


Rectovesical fistula

description: An aberrant, pathological communication between the rectum and the bladder.


Recurrent Unspecified Lung Carcinoma

description: Lung carcinoma that has re-emerged after a period of remission.


Large cell lung cancer recurrent

description: The reemergence of large cell lung carcinoma after a period of remission.


name: Recurrent Lung Non-Small Cell Squamous Carcinoma

description: The reemergence of lung squamous non-small cell carcinoma after a period of remission.


Recurrent Bronchogenic Carcinoma

description: The reemergence of bronchogenic carcinoma after a period of remission.


name: Locally Recurrent Lung Non-Small Cell Carcinoma

description: The reemergence of lung non-small cell carcinoma after a period of remission, at or adjacent to the site of the original tumor.


Non-small cell lung cancer recurrent

description: The re-emergence of non-small cell lung carcinoma after a period of remission.


name: Recurrent Lung Non-Squamous Non-Small Cell Carcinoma

description: The reemergence of non-squamous non-small cell lung carcinoma after a period of remission.


name: Adenosquamous cell lung cancer recurrent

description: The reemergence of adenosquamous lung carcinoma after a period of remission.


Lung adenocarcinoma recurrent

description: The re-emergence of lung adenocarcinoma after a period of remission.


Small cell lung cancer recurrent

description: The reemergence of small cell carcinoma of the lung after a period of remission.


Lung squamous cell carcinoma recurrent

description: The re-emergence of squamous cell lung carcinoma after a period of remission.


name: Recurrent Extensive Stage Lung Small Cell Carcinoma

description: Extensive stage small cell lung carcinoma that has reemerged after a period of remission.


pituitary gland basophilic carcinoma

description: A malignant epithelial neoplasm of the anterior pituitary gland in which the neoplastic cells stain positive with basic dyes.


Stage IA Vaginal Cancer AJCC v8

description: Stage IA includes: T1a, N0, M0. T1a: Tumor confined to the vagina, measuring 2.0 cm or less. N0: No regional lymph node metastasis. M0: No distant metastasis. (AJCC 8th Ed.)


Stage I Vaginal Cancer AJCC v8

description: Stage I includes: IA: T1a, N0, M0; IB: T1b, N0, M0. T1a: Tumor confined to the vagina, measuring 2.0 cm or less. T1b: Tumor confined to the vagina, measuring more than 2.0 cm. N0: No regional lymph node metastasis. M0: No distant metastasis. (AJCC 8th Ed.)


Stage IB Vaginal Cancer AJCC v8

description: Stage IB includes: T1b, N0, M0. T1b: Tumor confined to the vagina, measuring more than 2.0 cm. N0: No regional lymph node metastasis. M0: No distant metastasis. (AJCC 8th Ed.)


name: Non-Neoplastic Chronic Lower Respiratory System Disorder

description: A non-neoplastic longstanding disorder of the lower respiratory system.


name: vulvar proximal-type epithelioid sarcoma

description: An epithelioid sarcoma of the proximal type involving the vulva.


microlissencephaly-micromelia syndrome

description: Microlissencephaly-micromelia syndrome is a syndrome of abnormal cortical development, characterized by severe prenatal polyhydramnios, postnatal microcephaly, lissencephaly, upper limb micromelia, dysmorphic facies (coarse face, hypertrichosis, and short nose with long philtrum), intractable seizures, and early death. Hypoparathyroidism was noted in one case.


name: neuronopathy, distal hereditary motor, autosomal recessive 10


name: Decreased renal tubular phosphate excretion


name: Recurrent Castration-Sensitive Prostate Carcinoma

description: The reemergence of hormone-sensitive prostate carcinoma in an anatomic site other than the prostate gland, after a period of remission.


Refractory Testicular Lymphoma

description: Testicular lymphoma that is resistant to treatment.


testicular lymphoma

description: A lymphoma that arises from the testis and is not associated with lymphoma in another anatomic site.


Recurrent Testicular Lymphoma

description: The reemergence of testicular lymphoma after a period of remission.


Testicular Follicular Lymphoma

description: A distinctive variant of follicular lymphoma arising from the testis. It has been reported with higher frequency in children, but is also seen rarely in adults. It differs biologically from nodal follicular lymphoma because it lacks evidence of the BCL2 translocation. It is usually of high cytological grade, usually grade 3A, but has a good prognosis, even without additional therapy beyond surgical excision. (WHO 2017)


name: Primary Diffuse Large B-Cell Lymphoma of the Testis

description: A diffuse large B-cell lymphoma that arises from the testis. It predominantly affects older men. Patients usually present with a unilateral hard painless mass. Bilateral masses have been described in a minority of patients.


name: Testicular Nasal Type Extranodal NK/T-Cell Lymphoma

description: A nasal type extranodal NK/T-cell lymphoma affecting the testis. Patients usually present with unilateral testicular enlargement. Bilateral involvement is rare. The prognosis is poor.


Malignant Pancreatic Somatostatinoma

description: A neuroendocrine tumor that arises from the delta cells of the pancreas. It is characterized by inappropriate secretion of somatostatin and associated with diabetes mellitus, hypochlorhydria, gallbladder disease, diarrhea, steatorrhea, anemia, and weight loss. It displays vascular invasion and metastasizes to other anatomic sites.


name: Pancreatic Somatostatin-Producing Neuroendocrine Tumor

description: A usually malignant, somatostatin producing neuroendocrine tumor, arising from the delta cells of the pancreas.  It may or may not be associated with inappropriate secretion of somatostatin and an associated clinical syndrome.


pancreatic somatostatinoma

description: A neuroendocrine tumor arising from the delta cells of the pancreas. It is characterized by inappropriate secretion of somatostatin and associated with diabetes mellitus, hypochlorhydria, gallbladder disease, diarrhea, steatorrhea, anemia, and weight loss.


major salivary gland carcinoma

description: A carcinoma that arises from the parotid gland, submandibular gland, or sublingual gland.


name: major salivary gland carcinoma ex pleomorphic adenoma

description: A carcinoma that arises from a pleomorphic adenoma in the major salivary glands. It usually originates in the parotid gland. Patients usually present with a history of a long-standing tumor mass which grew rapidly in the past few months. Patients with non-invasive or minimally invasive carcinoma have an excellent prognosis. In cases where there is invasion of the surrounding tissues, the clinical course is aggressive.


Recurrent Submandibular Gland Carcinoma

description: The reemergence of submandibular gland carcinoma after a period of remission.


name: Recurrent Parotid Gland Adenoid Cystic Carcinoma

description: The reemergence of parotid gland adenoid cystic carcinoma after a period of remission.


Parotid gland adenocarcinoma

description: An adenocarcinoma that arises from the parotid gland.


name: Stage IV Major Salivary Gland Carcinoma without Metastasis

description: Stage IV carcinoma of the major salivary glands without regional lymph nodes or distant metastases.


submandibular gland cancer

description: A malignant neoplasm involving the submandibular gland.


name: squamous cell carcinoma of submandibular gland

description: An invasive squamous cell carcinoma that arises from the submandibular gland.  It usually affects elderly patients and presents as a rapidly enlarging tumor mass, often associated with pain.


name: Stage IVC Major Salivary Gland Cancer AJCC v8

description: Stage IVC includes: Any T, Any N, M1. M1: Distant metastasis. (AJCC 8th ed.)


name: Recurrent Submandibular Gland Undifferentiated Carcinoma

description: The reemergence of submandibular gland undifferentiated carcinoma after a period of remission.


name: mucoepidermoid carcinoma of submandibular gland

description: A mucoepidermoid carcinoma that involves the submandibular gland.


parotid gland squamous cell carcinoma

description: An invasive squamous cell carcinoma that arises from the parotid gland. It usually affects elderly patients and presents as a rapidly enlarging tumor mass, often associated with pain.


name: Recurrent Submandibular Gland Acinic Cell Carcinoma

description: The reemergence of submandibular gland acinic cell carcinoma after a period of remission.


name: Stage III Major Salivary Gland Cancer AJCC v7

description: Stage III includes: (T3, N0, M0); (T1, N1, M0); (T2, N1, M0); (T3, N1, M0).  T3: Tumor measuring more than 4 cm in greatest dimension, and/or tumor having extraparenchymal extension.  Extraparenchymal extension is clinical or macroscopic evidence of invasion of soft tissues. Microscopic evidence alone does not constitute extraparenchymal extension for classification purposes.  T1: Tumor measuring 2 cm or less in greatest dimension without extraparenchymal extension. T2: Tumor measuring more than 2 cm, but not more than 4 cm in greatest dimension without extraparenchymal extension.  N0: No regional lymph node metastasis.  N1: Metastasis in a single ipsilateral lymph node, 3 cm or less in greatest dimension. M0: No distant metastasis. (AJCC 7th ed.)


name: Stage IV Major Salivary Gland Cancer AJCC v8

description: Stage IV includes: IVA (T4a, N0, M0); (T4a, N1, M0); (T0, N2, M0); (T1, N2, M0); (T2, N2, M0); (T3, N2, M0); (T4a, N2, M0); IVB (T4b, Any N, M0); (Any T, N3, M0); IVC (Any T, Any N, M1). T4a: Moderately advanced disease.  Tumor invades skin, mandible, ear canal, and/or facial nerve. T0: No evidence of primary tumor. T1: Tumor measuring 2 cm or less in greatest dimension without extraparenchymal extension. T2: Tumor measuring more than 2 cm, but not more than 4 cm in greatest dimension without extraparenchymal extension. T3: Tumor measuring more than 4 cm in greatest dimension, and/or tumor having extraparenchymal extension. T4b: Very advanced disease.  Tumor invades skull base and/or pterygoid plates and/or encases carotid artery. N0: No regional lymph node metastasis. N1: Metastasis in a single ipsilateral lymph node, 3 cm or less in greatest dimension. N2: Metastasis in a single ipsilateral lymph node, 3 cm or less in greatest dimension and ENE(+); or more than 3 cm but not more than 6 cm in greatest dimension and ENE(-); or metastases in multiple ipsilateral lymph nodes, none more than 6 cm in greatest dimension and ENE(-), or in bilateral or contralateral lymph nodes, none more than 6 cm in greatest dimension and ENE(-). N3: Metastasis in a lymph node larger than 6 cm in greatest dimension and ENE(-); or in a single ipsilateral node larger than 3 cm in greatest dimension and ENE(+); or multiple ipsilateral, contralateral, or bilateral nodes any with ENE(+). M0: No distant metastasis. M1: Distant metastasis. (AJCC 8th ed.)


Major Salivary Gland Adenocarcinoma

description: An adenocarcinoma that arises from the parotid gland, submandibular gland, or sublingual gland.


name: Submandibular Gland Acinic Cell Carcinoma

description: An adenocarcinoma with serous acinar cell differentiation that arises from the submandibular gland.


name: Submandibular Gland Undifferentiated Carcinoma

description: A carcinoma that arises from the submandibular gland and is characterized by the presence of undifferentiated, anaplastic malignant epithelial cells.


adenocarcinoma of sublingual gland

description: An adenocarcinoma that arises from the sublingual gland.


name: Major Salivary Gland Cancer by AJCC v8 Stage

description: A term that refers to the staging of major salivary gland cancer according to the American Joint Committee on Cancer, 8th edition.


name: Stage I Major Salivary Gland Cancer AJCC v7

description: Stage I includes: T1, N0, M0.  T1: Tumor measuring 2 cm or less in greatest dimension without extraparenchymal extension. Extraparenchymal extension is clinical or macroscopic evidence of invasion of soft tissues. Microscopic evidence alone does not constitute extraparenchymal extension for classification purposes.  N0: No regional lymph node metastasis.  M0: No distant metastasis. (AJCC 7th ed.)


name: Stage IVA Major Salivary Gland Cancer AJCC v7

description: Stage IVA includes: (T4a, N0, M0); (T4a, N1, M0); (T1, N2, M0); (T2, N2, M0); (T3, N2, M0); (T4a, N2, M0). T4a: Moderately advanced disease.  Tumor invades skin, mandible, ear canal, and/or facial nerve. T1: Tumor measuring 2 cm or less in greatest dimension without extraparenchymal extension.  Extraparenchymal extension is clinical or macroscopic evidence of invasion of soft tissues. Microscopic evidence alone does not constitute extraparenchymal extension for classification purposes.  T2: Tumor measuring more than 2 cm, but not more than 4 cm in greatest dimension without extraparenchymal extension.  T3: Tumor measuring more than 4 cm in greatest dimension, and/or tumor having extraparenchymal extension.  N0: No regional lymph node metastasis.  N1: Metastasis in a single ipsilateral lymph node, 3 cm or less in greatest dimension.  N2: Metastasis in a single ipsilateral lymph node, more than 3 cm but not more than 6 cm in greatest dimension, or in multiple ipsilateral lymph nodes, none more than 6 cm in greatest dimension, or in bilateral or contralateral lymph nodes, none more than 6 cm in greatest dimension.  M0: No distant metastasis. (AJCC 7th ed.)


name: Recurrent Submandibular Gland Mucoepidermoid Carcinoma

description: The reemergence of submandibular gland mucoepidermoid carcinoma after a period of remission.


name: Recurrent Parotid Gland Carcinoma ex Pleomorphic Adenoma

description: The reemergence of parotid gland carcinoma ex pleomorphic adenoma after a period of remission.


name: Stage II Major Salivary Gland Cancer AJCC v8

description: Stage II includes: T2, N0, M0. T2: Tumor measuring more than 2 cm, but not more than 4 cm in greatest dimension without extraparenchymal extension. N0: No regional lymph node metastasis. M0: No distant metastasis. (AJCC 8th ed.)


Recurrent Parotid Gland Carcinoma

description: The reemergence of parotid gland carcinoma after a period of remission.


name: Stage IVB Major Salivary Gland Cancer AJCC v8

description: Stage IVB includes: (T4b, Any N, M0); (Any T, N3, M0). T4b: Very advanced disease.  Tumor invades skull base and/or pterygoid plates and/or encases carotid artery. N0: No regional lymph node metastasis. N3: Metastasis in a lymph node larger than 6 cm in greatest dimension and ENE(-); or in a single ipsilateral node larger than 3 cm in greatest dimension and ENE(+); or multiple ipsilateral, contralateral, or bilateral nodes any with ENE(+). M0: No distant metastasis. (AJCC 8th ed.)


name: Recurrent Parotid Gland Squamous Cell Carcinoma

description: The reemergence of parotid gland squamous cell carcinoma after a period of remission.


carcinoma of parotid gland

description: A carcinoma that involves the parotid gland.


name: Recurrent Submandibular Gland Squamous Cell Carcinoma

description: The reemergence of submandibular gland squamous cell carcinoma after a period of remission.


name: Stage IVA Major Salivary Gland Cancer AJCC v8

description: Stage IVA includes: (T4a, N0, M0); (T4a, N1, M0); (T0, N2, M0); (T1, N2, M0); (T2, N2, M0); (T3, N2, M0); (T4a, N2, M0). T4a: Moderately advanced disease.  Tumor invades skin, mandible, ear canal, and/or facial nerve. T0: No evidence of primary tumor. T1: Tumor measuring 2 cm or less in greatest dimension without extraparenchymal extension. T2: Tumor measuring more than 2 cm, but not more than 4 cm in greatest dimension without extraparenchymal extension. T3: Tumor measuring more than 4 cm in greatest dimension, and/or tumor having extraparenchymal extension. N0: No regional lymph node metastasis. N1: Metastasis in a single ipsilateral lymph node, 3 cm or less in greatest dimension. N2: Metastasis in a single ipsilateral lymph node, 3 cm or less in greatest dimension and ENE(+); or more than 3 cm but not more than 6 cm in greatest dimension and ENE(-); or metastases in multiple ipsilateral lymph nodes, none more than 6 cm in greatest dimension and ENE(-), or in bilateral or contralateral lymph nodes, none more than 6 cm in greatest dimension and ENE(-). M0: No distant metastasis. (AJCC 8th ed.)


sublingual gland carcinoma

description: A carcinoma that arises from the sublingual gland. Representative examples include cystadenocarcinoma and mucoepidermoid carcinoma.


name: Stage 0 Major Salivary Gland Cancer AJCC v8

description: Stage 0 includes: Tis, N0, M0. Tis: Carcinoma in situ. N0: No regional lymph node metastasis. M0: No distant metastasis. (AJCC 8th ed.)


name: Major Salivary Gland Squamous Cell Carcinoma

description: A squamous cell carcinoma that arises from the parotid or submandibular gland.


name: Recurrent Parotid Gland Mucoepidermoid Carcinoma

description: The reemergence of parotid gland mucoepidermoid carcinoma after a period of remission.


name: major salivary gland mucoepidermoid carcinoma

description: A carcinoma that arises from the major salivary glands. It usually arises from the parotid gland. It is the most common primary carcinoma of the salivary glands and usually presents as a firm and painless mass. It is characterized by the presence of epidermoid cells, mucus producing cells, and cells of intermediate type. The majority of cases have a favorable outcome.


name: Recurrent Parotid Gland Acinic Cell Carcinoma

description: The reemergence of parotid gland acinic cell carcinoma after a period of remission.


name: major salivary gland adenoid cystic carcinoma

description: An aggressive carcinoma that arises from the major salivary glands. It is characterized by the presence of malignant epithelial and myoepithelial cells forming cribriform, tubular, and solid patterns. It usually presents as a slow growing mass. Patients develop pain because of the tendency of these carcinomas to invade perineural tissues.


name: Major Salivary Gland Cancer by AJCC v7 Stage

description: A term that refers to the staging of major salivary gland cancer according to the American Joint Committee on Cancer, 7th edition.


name: Submandibular Gland Carcinoma ex Pleomorphic Adenoma

description: A carcinoma that arises from a pleomorphic adenoma in the submandibular gland.  Patients usually present with a history of a long-standing tumor mass which grew rapidly in the past few months.  Patients with non-invasive or minimally invasive carcinoma have an excellent prognosis.  In cases where there is invasion of the surrounding tissues, the clinical course is aggressive.


name: Major Salivary Gland Acinic Cell Carcinoma

description: An adenocarcinoma with serous acinar cell differentiation that arises from the major salivary glands.  The vast majority of cases occur in the parotid gland.  Patients usually present with a slow growing mass in the parotid area.


parotid gland acinic cell carcinoma

description: An adenocarcinoma with serous acinar cell differentiation that arises from the parotid gland. Patients usually present with a slow growing mass in the parotid area.


submandibular gland adenocarcinoma

description: An adenocarcinoma that arises from the submandibular gland. Representative examples include polymorphic low-grade adenocarcinoma and acinic cell carcinoma.


name: mucoepidermoid carcinoma of parotid gland

description: A mucoepidermoid carcinoma that involves the parotid gland.


name: parotid gland carcinoma ex pleomorphic adenoma

description: A carcinoma that arises from a pleomorphic adenoma in the parotid gland. Patients usually present with a history of a long-standing tumor mass which grew rapidly in the past few months. Patients with non-invasive or minimally invasive carcinoma have an excellent prognosis. In cases where there is invasion of the surrounding tissues, the clinical course is aggressive.


name: Stage IV Major Salivary Gland Cancer AJCC v7

description: Stage IV includes: IVA (T4a, N0, M0); (T4a, N1, M0); (T1, N2, M0); (T2, N2, M0); (T3, N2, M0); (T4a, N2, M0); IVB (T4b, Any N, M0); (Any T, N3, M0); IVC (Any T, Any N, M1). T4a: Moderately advanced disease.  Tumor invades skin, mandible, ear canal, and/or facial nerve. T1: Tumor measuring 2 cm or less in greatest dimension without extraparenchymal extension.  Extraparenchymal extension is clinical or macroscopic evidence of invasion of soft tissues. Microscopic evidence alone does not constitute extraparenchymal extension for classification purposes.  T2: Tumor measuring more than 2 cm, but not more than 4 cm in greatest dimension without extraparenchymal extension.  T3: Tumor measuring more than 4 cm in greatest dimension, and/or tumor having extraparenchymal extension.  T4b: Very advanced disease.  Tumor invades skull base and/or pterygoid plates and/or encases carotid artery. N0: No regional lymph node metastasis.  N1: Metastasis in a single ipsilateral lymph node, 3 cm or less in greatest dimension.  N2: Metastasis in a single ipsilateral lymph node, more than 3 cm but not more than 6 cm in greatest dimension, or in multiple ipsilateral lymph nodes, none more than 6 cm in greatest dimension, or in bilateral or contralateral lymph nodes, none more than 6 cm in greatest dimension.  N3: Metastasis in a lymph node, more than 6 cm in greatest dimension.  M0: No distant metastasis. M1: Distant metastasis. (AJCC 7th ed.)


name: Recurrent Submandibular Gland Carcinoma ex Pleomorphic Adenoma

description: The reemergence of submandibular gland carcinoma ex pleomorphic adenoma after a period of remission.


name: Stage I Major Salivary Gland Cancer AJCC v8

description: Stage I includes: T1, N0, M0. T1: Tumor measuring 2 cm or less in greatest dimension without extraparenchymal extension. Extraparenchymal extension is clinical or macroscopic evidence of invasion of soft tissues. Microscopic evidence alone does not constitute extraparenchymal extension for classification purposes. N0: No regional lymph node metastasis. M0: No distant metastasis. (AJCC 8th ed.)


name: Parotid Gland Undifferentiated Carcinoma

description: A carcinoma that arises from the parotid gland and is characterized by the presence of undifferentiated, anaplastic malignant epithelial cells.


parotid gland adenoid cystic carcinoma

description: An aggressive carcinoma that arises from the parotid gland. It is characterized by the presence of malignant epithelial and myoepithelial cells forming cribriform, tubular, and solid patterns. It usually presents as a slow growing mass. Patients develop pain because of the tendency of these carcinomas to invade perineural tissues.


name: Recurrent Submandibular Gland Adenoid Cystic Carcinoma

description: The reemergence of submandibular gland adenoid cystic carcinoma after a period of remission.


name: Stage III Major Salivary Gland Cancer AJCC v8

description: Stage III includes: (T3, N0, M0); (T0, N1, M0); (T1, N1, M0); (T2, N1, M0); (T3, N1, M0).  T3: Tumor measuring more than 4 cm in greatest dimension, and/or tumor having extraparenchymal extension.  T0: No evidence of primary tumor. T1: Tumor measuring 2 cm or less in greatest dimension without extraparenchymal extension. T2: Tumor measuring more than 2 cm, but not more than 4 cm in greatest dimension without extraparenchymal extension.  N0: No regional lymph node metastasis.  N1: Metastasis in a single ipsilateral lymph node, 3 cm or less in greatest dimension and ENE(-). M0: No distant metastasis. (AJCC 8th ed.)


name: Stage IVC Major Salivary Gland Cancer AJCC v7

description: Stage IVC includes: Any T, Any N, M1. M1: Distant metastasis. (AJCC 7th ed.)


name: Stage II Major Salivary Gland Cancer AJCC v7

description: Stage II includes: T2, N0, M0. T2: Tumor measuring more than 2 cm, but not more than 4 cm in greatest dimension without extraparenchymal extension. Extraparenchymal extension is clinical or macroscopic evidence of invasion of soft tissues. Microscopic evidence alone does not constitute extraparenchymal extension for classification purposes.  N0: No regional lymph node metastasis.  M0: No distant metastasis. (AJCC 7th ed.)


name: Recurrent Parotid Gland Undifferentiated Carcinoma

description: The reemergence of parotid gland undifferentiated carcinoma after a period of remission.


name: submandibular gland adenoid cystic carcinoma

description: An aggressive carcinoma that arises from the submandibular gland. It is characterized by the presence of malignant epithelial and myoepithelial cells forming cribriform, tubular, and solid patterns. It usually presents as a slow growing mass. Patients develop pain because of the tendency of these carcinomas to invade perineural tissues.


name: Stage IVB Major Salivary Gland Cancer AJCC v7

description: Stage IVB includes: (T4b, Any N, M0); (Any T, N3, M0).  T4b: Very advanced disease.  Tumor invades skull base and/or pterygoid plates and/or encases carotid artery. N3: Metastasis in a lymph node, more than 6 cm in greatest dimension.  M0: No distant metastasis. (AJCC 7th ed.)


autosomal recessive osteopetrosis 4

description: Any autosomal recessive malignant osteopetrosis in which the cause of the disease is a mutation in the CLCN7 gene.


name: AIDS-Related Plasmablastic Lymphoma of the Oral Mucosa

description: A plasmablastic oral mucosa lymphoma in a patient with acquired immunodeficiency syndrome.


Plasmablastic Lymphoma of Mucosa Site

description: A plasmablastic lymphoma that arises from a mucosal site.


name: AIDS-Related Plasmablastic Lymphoma of Mucosa Site

description: A plasmablastic mucosal site lymphoma in a patient with acquired immunodeficiency syndrome.


name: Plasmablastic Lymphoma of the Oral Mucosa

description: A plasmablastic lymphoma that arises from the oral mucosa. It is usually associated with HIV infection.


Early Stage Esophageal Carcinoma

description: A stage term referring to esophageal carcinoma that is confined to the esophageal mucosa or submucosa.


name: localized junctional epidermolysis bullosa, non-Herlitz type

description: Junctional epidermolysis bullosa, localized non-Herlitz-type is a form of non-Herlitz junctional epidermolysis bullosa (JEB-nH) characterized by localized blistering, and dystrophic or absent nails.


name: junctional epidermolysis bullosa, non-Herlitz type

description: Junctional epidermolysis bullosa, non-Herlitz (JEB-nH) is a subtype of junctional epidermolysis bullosa (JEB) characterized by the presence of skin and mucosal blistering, nail dystrophy or nail absence and enamel hypoplasia.


name: generalized junctional epidermolysis bullosa non-Herlitz type

description: Generalized non-Herlitz-type junctional epidermolysis bullosa is a form of non-Herlitz-type junctional epidermolysis bullosa (JEB-nH) characterized by generalized skin blistering, atrophic scarring, nail dystrophy or nail absence, and enamel hypoplasia, with extracutaneous involvement.


nonsyndromic congenital nail disorder 9

description: Deformity or discoloration of a fingernail or toenail.


Leukonychia

description: White discoloration of the nails.


ischemic bowel disorder

description: Disease of the large or small intestine that is caused by inadequate blood supply.


acute intestinal ischemia

description: Ischemia of the intestine that is rapid in onset.


chronic gonococcal salpingitis

description: Chronic form of gonococcal salpingitis.


response to fungicide

category: biolink:BiologicalProcess biolink:Occurrent biolink:OntologyClass biolink:BiologicalProcessOrActivity biolink:BiologicalEntity biolink:ThingWithTaxon biolink:NamedThing biolink:PhysicalEssenceOrOccurrent

description: Any process that results in a change in state or activity of a cell or an organism (in terms of movement, secretion, enzyme production, gene expression, etc.) as a result of a fungicide stimulus. Fungicides are chemicals used to kill fungi.


response to tetralin

description: Any process that results in a change in state or activity of a cell or an organism (in terms of movement, secretion, enzyme production, gene expression, etc.) as a result of a tetralin stimulus.


response to symbiotic fungus

description: Any process that results in a change in state or activity of a cell or an organism (in terms of movement, secretion, enzyme production, gene expression, etc.) as a result of a stimulus from a symbiotic fungus, a fungus living in close physical association with another organism.


detection of symbiotic fungus

description: The series of events in which a stimulus from a symbiotic fungus, a fungus living in close physical association with another organism, is received and converted into a molecular signal.


Breast Cellular Fibroadenoma

description: A fibroadenoma characterized by the presence of a pericanalicular growth pattern, increased stromal cellularity, and less than 2 mitoses per 10 high-power fields.


description: A disorder seen following cancer chemotherapy. It typically manifests a few years after initiation of epipodophyllotoxin chemotherapy. Mutagenic potential of these non-intercalating DNA topoisomerase II inhibitors is believed to be increased with concurrent use of asparaginase or granulocyte colony-stimulating factor. Balanced translocations involving chromosomal bands 11q23 and 21q22 are commonly associated with this disorder. Clinical signs may include fatigue, dyspnea, bruising and frequent infections. Clinical course usually progresses to acute myeloid leukemia though most epipodophyllotoxin-related leukemias do not have an antecedent myelodysplastic phase. Prognosis is dismal with survivability usually less than one year.


name: liver inflammatory myofibroblastic tumor

description: A multinodular intermediate fibroblastic neoplasm arising from the liver. It is characterized by the presence of spindle-shaped fibroblasts and myofibroblasts, and a chronic inflammatory infiltrate composed of eosinophils, lymphocytes and plasma cells.


paratyphoid fever

description: A condition resembling typhoid fever that is caused by infection by Salmonella enterica serovar Parathyphi.


name: phosphoenolpyruvate carboxykinase deficiency, cytosolic

description: PEPCK1 deficiency is a rare inborn error of metabolism disorder, characterized by the deficiency of the enzyme PEPCK1, one of the enzymes needed for gluconeogenesis, the process by which organisms produce sugars (namely glucose) from non-carbohydrate precursors (such as amino acids). The symptoms described in the few cases reported in the medical literature suggest that there may be variation in the severity of the symptoms ranging from severe early-onset cases, to milder late-onset presentations. In severe cases symptoms may include persistent and very low levels of blood's sugar in newborns (neonatal hypoglycemia), failure to thrive, build-up of lactic acid in the blood (lactic acidosis), liver enlargement (hepatomegaly) and liver failure leading to neurological degeneration. Milder cases present during childhood with fewer and less serious liver problems. Infections and fasting may trigger the symptoms. PEPCK1 deficiency inheritance is autosomal recessive. It is caused by mutations in the PEPCK1 gene. Some researchers believe that the severity of the disease depend upon the mutations resulting in less or more PEPCK1 activity (the more active the enzyme is, the less severe the disease is, and vice versa). Treatment depend on the symptoms and may include giving extra carbohydrates during heavy exercise and illness or other times of fasting (formal sick day regimen) by the dietitian.PEPCK1 is the cytosolic form of the phosphoenolpyruvate carboxykinase (PEPCK) enzyme, the other being the mitochondrial (PEPCK2).


Eventration

description: A localized protrusion of a structure into an adjacent structure or cavity.


diaphragmatic eventration

description: A congenital or acquired abnormality characterized by elevation of the hemidiaphragm.


name: Epidermal Growth Factor Receptor Inhibitor-Induced Acneiform Lesion

description: Acneiform dermatitis caused by epidermal growth factor receptor inhibitory therapy in a dose-dependent manner.


acneiform dermatitis

description: Cutaneous eruptions resembling acne, characterized by the presence of papulonodules, pustules, comedones, or cysts in the face, trunk, and extremities. Causes include infections and the use of certain medications (e.g., antibiotics and steroids).


EBV-Related Carcinoma

description: Carcinoma that develops in individuals infected with Epstein-Barr virus (EBV), and in which there is a well-documented association between the neoplastic process and EBV.


name: Epstein-Barr virus-associated gastric carcinoma

description: Epstein-Barr virus (EBV)-associated gastric carcinoma (EBVaGC) is a rare form of gastric carcinoma (seen in approximately 10% of cases) with a male predominance, characterized by a latent EBV infection in gastric carcinoma cells, diffuse-type histology, a proximal location (in the body and cardia of the stomach) and a relatively favorable prognosis.


leukocyte adhesion deficiency 3

description: Leukocyte adhesion deficiency type III (LAD-III) is a form of LAD characterized by both severe bacterial infections and a severe bleeding disorder.


name: Low Grade Pancreatic Intraepithelial Neoplasia

description: Pancreatic intraepithelial neoplasia characterized by the presence of low grade epithelial dysplasia.


Pancreatic Intraepithelial Neoplasia-1A

description: A pancreatic intraepithelial neoplasia characterized by the presence of tall columnar cells that form flat architectural patterns.  Mild cytological atypia is present.


Moderate pancreatic duct dysplasia

description: A pancreatic mucinous intraepithelial neoplasia characterized by the presence of papillary and less often flat architectural patterns.  Moderate cytological atypia is present with some loss of nuclear polarity, nuclear crowding or hyperchromatism.


Pancreatic Intraepithelial Neoplasia-1B

description: A pancreatic intraepithelial neoplasia characterized by the presence of tall columnar cells that form papillary, micropapillary, or basally pseudostratified architectural patterns.  Mild cytological atypia is present.


Pancreatic Intraepithelial Neoplasia-1

description: A pancreatic intraepithelial neoplasia characterized by the presence of tall columnar cells that form flat or papillary, micropapillary, and basally pseudostratified architectural patterns.  Mild cytological atypia is present.


Early balding

description: Loss of scalp hair at an earlier than normal age.


Erectile dysfunction

description: A multidimensional but common male sexual dysfunction that involves an alteration in any of the components of the erectile response, including organic, relational and psychological.


Autonomic erectile dysfunction

description: Impotence (inability to develop or maintain an erection) resulting from abnormal functioning of the autonomic nervous system.


name: Patient-Specific Neoantigen-Positive Malignant Solid Neoplasm

description: A malignant solid neoplasm that expresses unique tumor-specific neoantigens in a single patient.


name: B Acute Lymphoblastic Leukemia with TCF3-HLF Rearrangement

description: An exceptionally rare childhood B-acute lymphoblastic leukemia associated with TCF3-HLF gene rearrangement resulting in the formation of TCF3-HLF chimeric transcription factor. The prognosis is poor.


name: B Acute Lymphoblastic Leukemia with HLF Rearrangement

description: An exceptionally rare childhood B-acute lymphoblastic leukemia associated with HLF gene rearrangement. The prognosis is poor.


name: B Acute Lymphoblastic Leukemia with TCF4-HLF Rearrangement

description: An exceptionally rare childhood B-acute lymphoblastic leukemia associated with TCF4-HLF gene rearrangement resulting in the formation of TCF4-HLF chimeric transcription factor. The prognosis is poor.


Retinal fold

description: A wrinkle of retinal tissue projecting outward from the surface of the retina and visible as a line on fundoscopy.


name: Retinal detachments and defects, unspecified

description: An imperfection if the retina of the eye in association with detachment of the retina from its blood supply.


Retinal defect, unspecified

description: An imperfection of the retina of the eye.


Tractional retinal detachment

description: A type of retinal detachment arising due to a combination of contracting retinal membranes, abnormal vitreoretinal adhesions, and vitreous changes. It is usually seen in the context of diseases that induce a fibrovascular response, e.g. diabetes.


Exudative retinal detachment

description: A type of retinal detachment arising from damage to the outer blood-retinal barrier that allows fluid to access the subretinal space and separate the neurosensory retina from the retinal pigment epithelium.


name: chromosome 4Q32.1-q32.2 triplication syndrome


name: partial duplication of the long arm of chromosome 4

description: Chromosome 4q duplication is a chromosome abnormality characterized by an extra copy (duplication) of genetic material on the long arm (q) of chromosome 4. The severity and specific symptoms depend on the size and location of the duplication, and which genes are involved. Features that have been described in some people with chromosome 4q duplication include developmental delay, intellectual disability, behavioral problems, birth defects, and distinctive facial features. Most cases are inherited from an unaffected parent with a chromosomal rearrangement called a balanced translocation. Some cases are not inherited and occur sporadically. Treatment is based on the signs and symptoms present in each person.


distal trisomy 4q

description: Distal trisomy 4q is a rare chromosomal anomaly syndrome, resulting from the partial duplication of the long arm of chromosome 4, with highly variable phenotype typically characterized by psychomotor delay, intellectual disability, craniofacial dysmorphism (microcephaly, low-set, prominent ears, downslanting palpebral fissures, hypertelorism, epicanthic folds, broad, prominent nasal bridge, high arched and cleft palate, micro-/retrognathia), seizures, as well as tooth and digital anomalies (clinodactyly, polydactyly). Cardiac malformations, renal anomalies, cryptorchidism, hypotonia and hearing impairment have also been reported.


Chronic gouty nephropathy

description: Chronic kidney disease resulting from deposition of urate crystals or microtophi in the medullary interstitium.


Non-Neoplastic Kidney Disorder

description: A non-neoplastic disorder that affects the renal parenchyma and/or renal pelvis. Representative examples include glomerulonephritis and pyelonephritis.


Minimal mesangial lupus nephritis

description: A subclass of lupus nephritis with normal glomeruli by light microscopy, but mesangial immune deposits by immunofluorescence.


Nephrotic Syndrome - ADCK4 Associated

description: Nephrotic syndrome attributed to mutation(s) in the ADCK4 gene.


multicystic dysplastic kidney

description: Multicystic dysplastic kidney (MCDK) is a congenital anomaly of the kidney and urinary tract (CAKUT) in which one or both kidneys (unilateral or bilateral MCDK respectively) are large, distended by multiple cysts, and non-functional.


rapidly progressive glomerulonephritis

description: Inflammation of the glomeruli that is characterized by a rapid loss in renal function with glomerular crescent formation observed on biopsy; it is often seen in patients with concomitant autoimmune disease, like Goodpasture's syndrome or systemic lupus erythematosus.


Nephrotic Syndrome - MYO1E Associated

description: Nephrotic syndrome attributed to mutation(s) in the MYO1E gene.


karyomegalic interstitial nephritis

description: Any interstitial nephritis in which the cause of the disease is a mutation in the FAN1 gene.


Obesity Related Glomerulopathy

description: Diffuse glomerular (greater than 50% of glomeruli) hypertrophy (greater than 250 micron diameter) that occurs in the context of obesity.


name: tubulointerstitial kidney disease, autosomal dominant, 2

description: An inherited disorder that causes a gradual loss of kidney function, caused by a mutation in the MUC1 gene that leads to production of an abnormal mucin 1 protein, which deposits in the kidney and leads to slow loss of kidney function.


Proximal tubulopathy

description: Dysfunction of the proximal tubule, which is the portion of the duct system of the nephron of the kidney which leads from Bowman's capsule to the loop of Henle.


Thrombosis of renal artery

description: The formation of a thrombus in the renal artery.


name: Crouzon syndrome-acanthosis nigricans syndrome

description: Crouzon syndrome with acanthosis nigricans (CAN) is a very rare, clinically heterogeneous form of faciocraniostenosis with Crouzon-like features and premature synostosis of cranial sutures (Crouzon disease), associated with acanthosis nigricans (AN).


Membranous Nephropathy - THSD7A Induced

description: Membranous nephropathy due to thrombospondin type-1 domain-containing protein 7A (THSD7A) antibodies.


name: Pauci-Immune Glomerulonephritis - Renal Limited

description: Glomerulonephritis in which anti-neutrophil cytoplasm antibody (ANCA) is almost always present and vasculitis is limited to the kidney.


Acute Cortical Necrosis

description: Acute kidney injury caused by ischemic necrosis of the renal cortex.


steroid-resistant nephrotic syndrome

description: Nephrotic syndrome, occurring in the pediatric population, in which proteinuria does not normalize with administration of steroids; this condition is unresponsive to a minimum of four weeks administration of oral corticosteroids.


end stage renal failure

description: Long-standing and persistent renal disease with glomerular filtration rate (GFR) less than 15 ml/min.


Nephropathy due to Ciclosporin

description: Kidney damage resulting from ciclosporin.


name: Chronic kidney disease due to hypertension

description: Longstanding kidney disease as a complication of hypertension.


Stage 1 chronic kidney disease

description: A type of chronic kidney disease with normal or increased glomerular filtration rate (GFR at least 90 mL/min/1.73 m2).


name: Nephrotic Syndrome - Simian Virus 40 Associated

description: Nephrotic syndrome associated with a simian virus 40 infection.


name: Cryoglobulinemic Glomerulonephritis Secondary to Systemic Disease

description: Cryoglobulinemia glomerulonephritis associated with chronic disease, often inflammatory conditions.


Pierson syndrome

description: Pierson syndrome is characterized by the association of congenital nephrotic syndrome and ocular anomalies with microcoria.


name: Nephrotic Syndrome - Human Immunodeficiency Virus Associated

description: Nephrotic syndrome associated with human immunodeficiency virus infection.


pyonephrosis

description: Pus within the collecting system of the kidney.


Uric acid nephropathy acute

description: Kidney damage resulting from uric acid precipitation within the renal tubules.


contrast-induced nephropathy

description: Kidney damage resulting from exposure to contrast agents.


Diffuse lupus nephritis

description: A subclass of lupus nephritis with active or inactive diffuse, segmental or global endocapillary or extracapillary glomerulonephritis involving at least 50% of all glomeruli, typically with diffuse subendothelial immune deposits, with or without mesangial alterations.


description: Tubulointerstitial nephritis resulting from a drug exposure.


Renal Pelvis Fibroepithelial Polyp

description: A benign, mesodermal tumor located in the renal pelvis.


name: Nephrotic syndrome with lesion of proliferative glomerulonephritis

description: A renal functional disorder characterized by proteinuria, edema, hyperlipidemia and hypoalbuminemia. It results from damage to the renal vascular filtration apparatus. It is further characterized by an inflammatory reaction of the glomerular capillaries due to the proliferation of mesangial cells and expansion of the mesangial matrix. Sequelae may include hypertension, atherosclerosis, infection, hypercoagulablity and renal failure.


Reflux nephropathy (disorder)

description: Chronic kidney damage due to vesicoureteral reflux.


Nephropathy due to Tacrolimus

description: Kidney damage resulting from tacrolimus.


name: Congenital Nephrotic Syndrome - Rubivirus Associated

description: Nephrotic syndrome associated with rubella, most commonly presenting in the first three months of life.


nephrocalcinosis

description: Nephrocalcinosis is a disorder that occurs when too much calcium is deposited in the kidneys. It commonly occurs in premature infants. Individuals may not have symptoms or may have symptoms related to thecondition causing nephrocalcinosis. If kidney stones are present, symptoms may include blood in the urine, fever and chills, nausea and vomiting, and severe pain in the belly area, sides of the back (flank), groin, or testicles. Later symptoms may be associated with chronic kidney failure. It may be caused by use of certain medications or supplements, infection, or any condition that leads to high levels of calcium in the blood or urine including hyperparathyroidism, renal tubular acidosis, Alport syndrome, Bartter syndrome,and a variety of other conditions. Some of the underlying disorders that can cause nephrocalcinosis are genetic, with the inheritance pattern depending on the specific disorder. Treatment differs depending on the cause of nephrocalcinosis and often aims to prevent more calcium from being deposited in the kidneys.


name: Nephrotic Syndrome - Infection Associated

description: Nephrotic syndrome associated with an infectious process.


Glomerular vascular disorder

description: A disorder that is caused by pathologic changes in the glomerular vasculature.


name: Nephrotic Syndrome - SMARCAL1 Associated

description: Nephrotic syndrome attributed to mutation(s) in the SMARCAL1 gene.


name: glomerulopathy with fibronectin deposits 2

description: Any fibronectin glomerulopathy in which the cause of the disease is a mutation in the FN1 gene.


Nephrotic Syndrome - CFH Associated

description: Nephrotic syndrome attributed to mutation(s) in the CFH gene.


acute pyelonephritis

description: Sudden onset pyelonephritis.


Chronic glomerulonephritis

description: A chronic, persistent inflammation of the glomeruli, which is slowly progressive, leading to impaired kidney function.


name: Acute renal papillary necrosis with renal failure

description: Sudden and sustained deterioration of the kidney function with decreased glomerular filtration rate, and concommittant necrosis of the renal papillae; representative causes include sickle cell disease, heave analgesic use, diabetes mellitus, and pyelonephritis.


nephritis

description: Inflammation of renal tissue.


Nephrotic Syndrome - ITGA3 Associated

description: Nephrotic syndrome attributed to mutation(s) in the ITGA3 gene.


name: Pauci-Immune Glomerulonephritis associated with Microscopic Polyangiitis

description: Glomerulonephritis in the context of systemic, small vessel vasculitis in which anti neutrophil cytoplasm antibody (ANCA) is almost always present.


Nephropathy due to Aminoglycosides

description: Kidney damage resulting from aminoglycosides.


description: Mesangial proliferative glomerulonephritis (MPGN) is a condition that affects the kidneys. Many experts consider it a variant of minimal change disease, but some experts believe it is a separate condition. It may present with nephrotic syndrome, which is a group of symptoms that include protein in the urine (proteinuria), low blood protein levels, high cholesterol levels, high triglyceride levels, and swelling. It can also present with blood in the urine (hematuria). MPGN is characterized by an increased number of mesangial cells in the glomeruli in the kidneys and damage to the glomeruli. Glomeruli are the structures that help filter wastes and fluids. MPGN may occur in several renal diseases such as IgA nephropathy (commonly), IgM nephropathy, lupus nephritis, and C1q nephropathy.However, in some cases, the underlying cause of MPGN remains unclear. Treatment may depend on the cause (if known) and may include steroids, mycophenolate mofetil, and/or cyclophosphamide, and other therapies to treat specific symptoms. Most people with MPGN have a good prognosis, but some may develop chronic kidney disease, which can progress to end stage renal failure.


name: Chronic glomerulonephritis with lesion of membranous glomerulonephritis

description: A slowly progressive inflammation of the glomeruli characterized by immune complex deposits on the epithelial side of the glomerular basement membrane.


description: Idiopathic membranous glomerulonephritis (IMG) is a primary glomerular disease characterized by proteinuria, usually in the nephrotic range, with preserved renal function.


name: Membranous Nephropathy - Autoimmune Disorder Associated

description: Membranous nephropathy associated with an autoimmune disorder.


Nephrotic Syndrome - CRB2 Associated

description: Nephrotic syndrome attributed to mutation(s) in the CRB2 gene.


obstructive nephropathy

description: Renal damage and impaired renal function secondary to urinary tract obstruction.


Familial immunoglobulin A nephropathy

description: IgA nephropathy secondary to a genetic mutation that is transmitted from parents to offspring.


Acquired calyceal diverticulum

description: An out-pouching of the calyx into the renal parenchyma.


cystic kidney disease

description: A congenital or acquired kidney disorder characterized by the presence of renal cysts.


Nephrotic Syndrome - PDSS2 Associated

description: Nephrotic syndrome attributed to mutation(s) in the PDSS2 gene.


name: Nephrotic Syndrome with Lesion of Focal Glomerulosclerosis

description: A renal functional disorder characterized by proteinuria, edema, hyperlipidemia and hypoalbuminemia. It results from damage to the renal vascular filtration apparatus. It is further characterized by scarring of some of the glomerular capillaries. Sequelae may include hypertension, atherosclerosis, infection, hypercoagulablity and renal failure.


diffuse glomerulonephritis

description: Inflammation of the glomeruli, in which all glomeruli are affected, resulting in renal failure.


Nephrotic Syndrome - COQ2 Associated

description: Nephrotic syndrome attributed to mutation(s) in the COQ2 gene.


name: Systemic Lupus Erythematosus Nephritis Class V

description: Systemic lupus erythematosus nephritis, with global or segmental subepithelial immune deposits or their morphologic sequelae by light microscopy and by immunofluorescence or electron microscopy, with or without mesangial alterations. (Weening, Jan J. et al. (2004). The Classification of Glomerulonephritis in Systemic Lupus Erythematosus Revisited. Journal of the American Society of Nephrology 15(2), 241-50.)


Hantavirus nephropathy

description: Tubulointerstitial hemorrhage resulting from hantavirus infection.


Henoch-Schonlein purpura nephritis

description: Glomerulonephritis in the context of Henoch-Schönlein purpura.


name: Chronic Kidney Disease due to Diabetes Mellitus

description: Longstanding kidney disease as a complication of diabetes.


Renal infarction

description: Ischemic necrosis of the kidney caused by interruption of the blood supply to the area.


Steroid-dependent nephrotic syndrome

description: A type of nephrotic syndrome in which two consecutive relapses (albumin urine dipstick at least 3+ or proteinuria greater than 40 mg/m2/d or urinary protein to creatinine ratio at least 2000 mg/g for 3 consecutive days) occur during corticosteroid therapy, or within 14 days of ceasing therapy.


name: Acute Kidney Injury with Renal Replacement Therapy Related to COVID-19

description: Abrupt reduction in kidney function as measured by urine output and renal biomarkers requiring any renal replacement therapy in a patient with probable or confirmed acute COVID-19.


Leptospirosis Nephropathy

description: Interstitial nephritis due to Leptospira, which may be associated with non-oliguric acute kidney injury.


idiopathic nephrotic syndrome

description: Nephrotic syndrome for which no cause has been identified.


Cryoglobulinemic glomerulonephritis

description: Glomerulonephritis in the context of cryoglobulinemia.


name: Chemotherapeutic Drug - Induced Nephropathy

description: Kidney damage resulting from exposure to chemotherapeutic drugs.


IgM - Associated Nephropathy

description: A condition characterized by deposition of IgM antibody in the glomerulus.


NSAID-Induced Nephropathy

description: Kidney damage resulting from exposure to non-steroidal anti-inflammatory drugs (NSAIDs).


Idiopathic Membranous Glomerulopathy

description: Chronic degenerative changes in the kidney characterized by thickened and inflamed glomeruli and proteinurea, the cause of which is unknown.


Microcystic renal disease

description: A congenital renal disorder characterized by the presence of small cysts in the renal cortex and/or renal medulla.


Focal lupus nephritis

description: A subclass of lupus nephritis with active or inactive focal, segmental or global endocapillary or extracapillary glomerulonephritis involving less than 50% of all glomeruli, typically with focal subendothelial immune deposits, with or without mesangial alterations


Idiopathic Renal Papillary Necrosis

description: Necrosis of the renal papillae, for which no underlying cause has been identified.


Diffuse glomerulosclerosis

description: A renal disorder characterized by glomerular damage due to ischemia. It leads to progressive deterioration of renal function.


name: Nephrotic Syndrome with Lesion of Persistent Glomerulonephritis

description: A renal functional disorder characterized by proteinuria, edema, hyperlipidemia and hypoalbuminemia. It results from damage to the renal vascular filtration apparatus. It is further characterized by a chronic inflammatory reaction in the glomerular capillaries. Sequelae may include hypertension, atherosclerosis, infection, hypercoagulablity and renal failure.


name: Systemic Lupus Erythematosus Nephritis Class IV G

description: Systemic lupus erythematosus nephritis, characterized by active or inactive diffuse, global endo- or extracapillary glomerulonephritis that involves 50% or more of all glomeruli. (Weening, Jan J. et al. (2004). The Classification of Glomerulonephritis in Systemic Lupus Erythematosus Revisited. Journal of the American Society of Nephrology 15(2), 241-50.)


name: Nephrotic Syndrome - ARHGAP24 Associated

description: Nephrotic syndrome attributed to mutation(s) in the ARHGAP24 gene.


chronic renal failure syndrome

description: Impairment of the renal function due to chronic kidney damage.


familial nephrotic syndrome

description: An instance of nephrotic syndrome that is caused by an inherited modification of the individual's genome.


Stage 4 chronic kidney disease

description: A type of chronic kidney disease with severely reduced glomerular filtration rate (GFR 15-29 mL/min/1.73 m2).


proliferative glomerulonephritis

description: A constellation of renal disorders characterized by an increase number of cells in the glomerulus; these disorders generally present with nephrotic syndrome, and generally progress to end stage renal failure over a matter of weeks to years, depending on the etiology. Examples include IgA nephropathy, membranoproliferative glomerulonephritis, and rapidly progressive glomerulonephritis.


Chronic kidney disease stage 3B

description: Long-standing and persistent renal disease with glomerular filtration rate (GFR) between 30-44 ml/min.


Advanced sclerotic lupus nephritis

description: A subclass of lupus nephritis with at least 90% of glomeruli globally sclerosed without residual activity.


name: Calcineurin Inhibitor - Induced Nephropathy

description: Kidney damage resulting from exposure to calcineurin inhibitors.


name: Congenital Nephrotic Syndrome - Cytomegalovirus Associated

description: Nephrotic syndrome associated with a cytomegalovirus infection, most commonly presenting in the first three months of life.


name: Light Chain Proximal Tubulopathy without Crystals

description: A morphologic variant of light chain proximal tubulopathy with no evidence of crystal formation. It is characterized by acute tubular injury with no evidence lysosomal immunoglobulin crystals.


name: familial juvenile hyperuricemic nephropathy type 1

description: A rare kidney disorder characterized by hyperuricemia, progressive nephropathy, and gout occurring at an early age.


Nephrotic Syndrome - CD2AP Associated

description: Nephrotic syndrome attributed to mutation(s) in the CD2AP gene.


polycystic kidney disease 2

description: Autosomal dominant polycystic kidney disease caused by a mutation in PKD2.


name: Nephrotic Syndrome - Toxoplasmosis Associated

description: Nephrotic syndrome associated with a toxoplasmosis infection.


Nephrotic Syndrome - ARHGDIA Associated

description: Nephrotic syndrome attributed to mutation(s) in the ARHGDIA gene.


Acute glomerulonephritis

description: An acute inflammation of the glomeruli, generally secondary to infection or injury.


name: Nephrotic Syndrome - Syphilis Associated

description: Nephrotic syndrome associated with a syphilis infection.


Nephrotic Syndrome - NPHS2 Associated

description: Nephrotic syndrome attributed to mutation(s) in the NPHS2 gene.


name: C3 Glomerulopathy with Monoclonal Gammopathy

description: A subset of C3 glomerulopathy in which there is an associated finding of monoclonal gammopathy.


Nephrotic Syndrome - CUBN Associated

description: Nephrotic syndrome attributed to mutation(s) in the CUBN gene.


crescentic glomerulonephritis

description: A histopathologic term for a pattern of diseases characterized by extensive crescent formation in the glomeruli; patients present clinically with rapid deterioration of renal function, and possible progression to end-stage renal failure within weeks or months.


Malignant Hypertensive Nephropathy

description: Hypertensive nephropathy secondary to malignant hypertension.


name: Systemic Lupus Erythematosus Nephritis Class IV S

description: Systemic lupus erythematosus nephritis characterized by active or inactive diffuse, segmental endo- or extracapillary glomerulonephritis that involves 50% or more of all glomeruli. (Weening, Jan J. et al. (2004). The Classification of Glomerulonephritis in Systemic Lupus Erythematosus Revisited. Journal of the American Society of Nephrology 15(2), 241-50.)


Lithium - Induced Nephropathy

description: Kidney damage resulting from lithium which may include distal or collecting tubular cysts, proteinuria, interstitial nephritis and nephrogenic diabetes insipidus.


Secondary Collapsing Glomerulopathy

description: Collapsing glomerulopathy for which an underlying cause has been identified.


Focal Segmental Glomerulosclerosis 7

description: An autosomal dominant type of focal segmental glomerulosclerosis caused by mutation(s) in the PAX2  gene, encoding which paired box protein Pax-2.


polycystic kidney disease

description: A usually autosomal dominant and less frequently autosomal recessive genetic disorder characterized by the presence of numerous cysts in the kidneys leading to end-stage renal failure. The autosomal dominant trait is associated with abnormalities on the short arm of chromosome 16. Symptoms in patients with the autosomal dominant trait usually appear at middle age and include abdominal pain, hematuria, and high blood pressure. Patients may develop brain aneurysms and liver cysts. Patients with the autosomal recessive trait present with progressive renal failure early in life and symptoms resulting from hepatic fibrosis. The autosomal recessive trait is associated with abnormalities of chromosome 6. Polycystic kidney disease may also result as a side effect in patients on renal dialysis.


Postpartum acute renal failure

description: Renal failure that occurs postpartum due to any partum problem (hemorrhage, sepsis, preeclampsia).


Nephrotic Syndrome - INF2 Associated

description: Nephrotic syndrome attributed to mutation(s) in the INF2 gene.


name: Congenital Nephrotic Syndrome - Toxoplasma Associated

description: Nephrotic syndrome associated with toxoplasmosis, most commonly presenting in the first three months of life.


xanthogranulomatous pyelonephritis

description: Chronic, destructive infection of the kidney characterized by lipid-laden macrophages in the setting of obstruction secondary to infected renal stones, most commonly caused by Proteus or Escherichia coli.


Nephrotic Syndrome - ANLN Associated

description: Nephrotic syndrome attributed to mutation(s) in the ANLN gene.


Acute cellular allograft nephropathy

description: A sudden onset of T-cell mediated immune response occurring after transplantation, directed against donor kidney alloantigens.


Scleroderma renal crisis

description: The onset of acute renal failure, normally coupled with marked hypertension in a patient with scleroderma.


HIV-associated nephropathy

description: Renal disease in human immunodeficiency virus (HIV)-infected patients. It is characterized by nephrotic syndrome, azotemia, normal to large kidneys on ultrasound images, and focal segmental glomerulosclerosis on renal biopsy findings.


chronic pyelonephritis

description: Persistent pyelonephritis.


Nephrotic Syndrome - EMP2 Associated

description: Nephrotic syndrome attributed to mutation(s) in the EMP2 gene.


name: Membranous Nephropathy - Malignancy Associated

description: Membranous nephropathy in the context of malignancy.


kidney hypertrophy

description: Global enlargement of the renal parenchyma in one or both kidneys.


name: Acute Kidney Injury without Renal Replacement Therapy Related to COVID-19

description: Abrupt reduction in kidney function as measured by urine output and renal biomarkers but not requiring any renal replacement therapy in a patient with probable or confirmed acute COVID-19.


name: Nephrotic Syndrome with Lesion of Membranous Glomerulonephritis

description: A renal functional disorder characterized by proteinuria, edema, hyperlipidemia and hypoalbuminemia. It results from damage to the renal vascular filtration apparatus. It is further characterized by an inflammatory reaction in the glomerular capillaries due to the deposition of immune complexes and subsequent thickening of the glomerular basement membrane. Sequelae may include hypertension, atherosclerosis, infection, hypercoagulablity and renal failure.


acute urate nephropathy

description: Urolithiasis in which the composition of the stones is predominantly urate.


lipoid nephrosis

description: A glomerular disorder characterized by the electron microscopic finding of loss of podocyte foot processes. Light microscopic examination does not show glomerular changes. Patients present with proteinuria and nephrotic syndrome.


name: Congenital Nephrotic Syndrome - Focal Segmental Glomerulosclerosis

description: Nephrotic syndrome within the first three months of life, characterized by scarring of the glomerulus, in which only part of the glomerulus is involved, and less than 50% of the glomeruli are affected.


name: autosomal dominant polycystic kidney disease

description: Autosomal dominant form of polycystic kidney disease.


Ischemic nephropathy

description: Nephropathy due to hypoperfusion of the kidney.


name: Nephrotic Syndrome - Frequently Relapsing

description: Nephrotic syndrome in which there is relapse occurring two or more times in the first six months, or four or more times in a year.


name: Nephrotic Syndrome - Infrequently Relapsing

description: Nephrotic syndrome in which there is a relapse occurring less than twice in the first six months, or less than four times in a year.


Balkan nephropathy

description: A chronic tubulointerstitial nephropathy that affects people in certain rural areas along the Danube river in the Balkans. It leads to end-stage renal disease.


name: Nephrotic Syndrome - Hepatitis B Virus Associated

description: Nephrotic syndrome associated with hepatitis B.


name: nephrotic syndrome of childhood - steroid sensitive

description: Nephrotic syndrome, occurring in the pediatric population, characterized by the normalization of proteinuria with the administration of corticosteroids.


Arterionephrosclerosis

description: Scarring and atrophy of the renal cortex that occurs in hypertensive patients and in old age.


renal artery disease

description: A disease involving the renal artery.


Dent disease

description: Dent disease is a rare genetic renal tubular disease characterized by manifestations of proximal tubule dysfunction.


Nephropathy due to Cisplatin

description: Kidney damage resulting from cisplatin.


description: Membranous nephropathy associated with infectious disease.


cystinosis

description: Cystinosis is a metabolic disease characterized by an accumulation of cystine inside the lysosomes, causing damage in different organs and tissues, particularly in the kidneys and eyes. Three clinical forms have been described: nephropathic infantile, nephropathic juvenile and ocular.


Renal transplant rejection

description: A sudden immune response occurring after transplantation, directed against donor kidney alloantigens.


Acute humoral allograft nephropathy

description: A sudden onset of B-cell mediated immune response occurring after transplantation, directed against donor kidney alloantigens.


name: Nephropathy Associated with Rhabdomyolysis

description: Nephropathy associated with rhabdomyolysis.


IgA Nephropathy, Infection-associated

description: IgA nephropathy co-occurring with infectious disease.


acute kidney tubular necrosis

description: Acute renal failure caused by the cell death of the renal tubules. Causes include nephrotoxins, cytotoxic drugs, and antibiotics.


Nephrotic Syndrome - LAMB2 Associated

description: Nephrotic syndrome attributed to mutation(s) in the LAMB2 gene.


name: Primary Focal Segmental Glomerulosclerosis

description: Focal scarring of the glomerulus, which may result in isolated proteinuria or nephrotic syndrome, which affects only part of the glomerulus and only some of the glomeruli. The condition is considered primary in cases for which no underlying cause has been identified.


name: autosomal recessive polycystic kidney disease

description: An inherited disorder characterized by the development of cysts affecting the collecting ducts. It is frequently associated with hepatic involvement.


Nephropathy due to Analgesic Drugs

description: Kidney damage resulting from analgesic drugs.


Complex renal cyst

description: A renal cyst characterized by epithelium lined space (squamous/columnar) with septations.


glomerular disorder

description: A disease involving the renal glomerulus.


C3 glomerulonephritis

description: Glomerulonephritis characterized by C3 accumulation with little or absent deposition of immunoglobulin, in the absence of ultrastructural electron-dense transformation seen in dense deposit disease.


polycystic kidney disease 1

description: Any autosomal dominant polycystic kidney disease in which the cause of the disease is a mutation in the PKD1 gene.


name: Nephrotic Syndrome - Cytomegalovirus Associated

description: Nephrotic syndrome associated with a cytomegalovirus infection.


Tip Lesion Glomerulopathy

description: Segmental glomerulopathy of localized intracapillary foam cells with adjacent, often vacuolated glomerular epithelial cells confluent to the origin of the proximal tubule.


Bone Marrow Transplantation Nephropathy

description: Kidney dysfunction resulting from complications of bone marrow transplantation.


Schistosomiasis Nephropathy

description: Kidney disease associated with Schistosoma infection. Injury to the kidney may be a result of immunologically mediated glomerular or interstitial injury and/or a result of reflux nephropathy.


Chronic kidney disease stage 3A

description: Long-standing and persistent renal disease with glomerular filtration rate (GFR) between 45-59 ml/min.


acute diffuse glomerulonephritis

description: An acute inflammation of the glomeruli, in which all glomeruli are affected, resulting in acute renal failure.


name: Secondary focal segmental glomerulosclerosis

description: Focal segmental glomerulosclerosis due to another medical condition.


Bisphosphonate - Induced Nephropathy

description: Kidney damage resulting from exposure to bisphosphonates.


Stage 2 chronic kidney disease

description: A type of chronic kidney disease with mildly reduced glomerular filtration rate (GFR 60-89 mL/min/1.73 m2).


nephronophthisis

description: Progressive tubulointerstitial injury, inherited in an autosomal recessive pattern, caused by mutations in genes involved in ciliary function, which may result in an end stage renal failure.


Nephrotic Syndrome - DGKE Associated

description: Nephrotic syndrome attributed to mutation(s) in the DGKE gene.


Nephrotic Syndrome - TRPC6 Associated

description: Nephrotic syndrome attributed to mutation(s) in the TRPC6 gene.


nephronophthisis 1

description: Progressive tubulointerstitial nephritis inherited in an autosomal recessive manner. It is caused by mutations in the NPHP1 gene. Patients present with anemia, polyuria, and polydipsia during childhood. The progressive bilateral kidney damage results in renal failure.


Calcineurin Nephrotoxicity

description: Kidney injury caused by calcineurin inhibitor immunosuppressive therapy which may lead to diminished kidney function.


interstitial nephritis

description: Inflammation of the renal tubules and supporting tissues of the kidney.


Primary Collapsing Glomerulopathy

description: Collapsing glomerulopathy for which no underlying cause has been identified.


immunotactoid glomerulopathy

description: Immunotactoid glomerulopathy (ITG) is a very rare condition characterized by glomerular accumulation of microtubules in the mesangium and the glomerular basement membrane, that mainly presents with proteinuria, micro-hematuria, nephrotic syndrome, renal insufficiency and hematologic malignancy. ITG and non-amyloid fibrillary glomerulopathy (non-amyloid FGP) are often grouped together as pathogenetically related diseases.


Nephrotic Syndrome - LMX1B Associated

description: Nephrotic syndrome attributed to mutation(s) in the LMX1B gene.


Drug-induced Nephropathy

description: Kidney damage resulting from exposure to drugs.


name: Congenital Nephrotic Syndrome - Treponema Pallidum Associated

description: Nephrotic syndrome associated with syphilis, most commonly presenting in the first three months of life.


Radiation Nephropathy

description: Tissue damage to the glomerulus and renal tubules resulting from irradiation, which is characterized by vascular endothelial damage, mesangial damage, platelet aggregation in the capillary loops, thickening of the glomerular arteriolar intimal layer, and atrophic tubules.


name: Focal Segmental Glomerulosclerosis Collapsing Variant

description: A variant of FSGS characterized by glomerular tuft collapse, which may result in scarring. (D'Agati VD, et al. "Pathologic Classification of Focal Segmental Glomerulosclerosis: A Working Proposal." Am J Kidney Dis 43.2 (2004): 368-82.)


description: Inflammation of the glomeruli secondary to presence of autoantibodies directed at specific antigenic targets within the glomerular basement membrane, causing hematuria, proteinuria, and impaired renal function.


name: Focal Segmental Glomerulosclerosis Cellular Variant

description: A variant of FSGS characterized by hypercellularity of the glomerulus; this excludes the tip and collapsing FSGS. (D'Agati VD, et al. "Pathologic Classification of Focal Segmental Glomerulosclerosis: A Working Proposal." Am J Kidney Dis 43.2 (2004): 368-82.)


name: Membranous Nephropathy - Drug Associated

description: Membranous nephropathy associated with exposure to a drug.


name: Focal AND segmental proliferative glomerulonephritis

description: Glomerulonephritis characterized by proliferation of endothelial or mesangial cells, affecting the glomeruli in a focal and segmental pattern.


Multiple glomerular cysts

description: The presence of many cysts in the glomerulus of the kidney related to dilatation of the Bowman's capsule.


name: Chronic glomerulonephritis with lesion of proliferative glomerulonephritis

description: A slowly progressive inflammation of the glomeruli occurring status post infection with streptococcus.


exudative glomerulonephritis

description: Inflammation of the glomeruli with infiltration by polymorphonuclear leukocytes.


Nephrotic Syndrome - PLCE1 Associated

description: Nephrotic syndrome attributed to mutation(s) in the PLCE1 gene.


kidney failure

description: An acute or chronic condition that is characterized by the inability of the kidneys to adequately filter the blood.


pyelitis

description: Inflammation of the renal pelvis.


Membranous Nephropathy - Secondary

description: Membranous nephropathy due to another medical condition.


Nephrotic Syndrome - Relapse

description: Nephrotic syndrome characterized by recurrent proteinuria by dipstick of first morning urine: 2+ or greater for three days or longer, or the presence of edema with proteinuria of any duration following a remission.


Nephrotic Syndrome - ACTN4 Associated

description: Nephrotic syndrome attributed to mutation(s) in the ACTN4 gene.


name: Proliferative Glomerulonephritis with Monoclonal IgG Deposits

description: A sub-type of monoclonal gammopathy of renal significance, characterized by restriction to a single immunoglobulin G heavy chain subclass and a single light chain isotype. Light microscopy often shows an endocapillary proliferative or membranoproliferative glomerulopathy. Electron microscopy reveals electron-dense subendothelial and mesangial deposits.


nephropathic cystinosis

description: An autosomal recessive condition caused by mutation(s) in the CTNS gene, encoding cystinosin. It is a sub-type of cystinosis, in which accumulation of cystine in the kidney results in renal dysfunction.


name: Nephrotic Syndrome with Lesion of Hypocomplementemic Glomerulonephritis

description: Nephrotic syndrome caused by glomerulonephritis associated with complement deficiency. It can result from an acute post-infectious glomerulonephritis, membranoproliferative glomerulonephritis or lupus glomerulonephritis.


Prevalent Nephrotic Syndrome

description: Pre-existing nephrotic syndrome.


Antimicrobial - Induced Nephropathy

description: Kidney damage resulting from exposure to antimicrobials.


Chronic Kidney Disease, Stage 0

description: Long-standing and persistent renal disease without kidney damage.


Glomerulomegaly

description: Abnormally large size of glomeruli.


name: IgA nephropathy associated with liver disease

description: IgA nephropathy secondary to hepatobiliary disease.


name: Nephrotic Syndrome - Parvovirus B19 Associated

description: Nephrotic syndrome associated with a parvovirus B19 infection.


Nephrotic Syndrome - MEFV Associated

description: Nephrotic syndrome attributed to mutation(s) in the MEFV gene.


name: Nephrotic Syndrome - Epstein-Barr Virus Associated

description: Nephrotic syndrome associated with Epstein-Barr infection.


Nephropathy due to Amphotericin

description: Kidney damage resulting from amphotericin.


Nephrotic Syndrome - NEIL1 Associated

description: Nephrotic syndrome attributed to mutation(s) in the NEIL1 gene.


name: congenital nephrotic syndrome, Finnish type

description: Congenital nephrotic syndrome, Finnish type is characterized by protein loss beginning during fetal life.


name: Pauci-Immune Glomerulonephritis - ANCA Negative

description: Glomerulonephritis with paucity of glomerular staining for immunoglobulins that may be accompanied by systemic, small vessel vasculitis containing no anti neutrophil cytoplasm antibody (ANCA).


Nephrotic Syndrome - COQ6 Associated

description: Nephrotic syndrome attributed to mutation(s) in the COQ6 gene.


oligomeganephronia

description: Oligomeganephronia is a developmental anomaly of the kidneys, and the most severe form of renal hypoplasia, characterized by a reduction of 80% in nephron number and a marked hypertrophy of the glomeruli and tubules.


Simple renal cyst

description: An isolated cyst of the kidney.


renal artery obstruction

description: Narrowing or occlusion of the renal artery or arteries. It is due usually to atherosclerosis; fibromuscular dysplasia; thrombosis; embolism, or external pressure. The reduced renal perfusion can lead to renovascular hypertension (hypertension, renovascular).


medullary sponge kidney

description: Medullary sponge kidney (MSK) is a birth defect of the tubules - tiny tubes inside the kidneys. In a normal kidney, urine flows through these tubules as it is being formed. In MSK, tiny sacs called cysts form in the medulla (the inner part of the kidney), creating a sponge-like appearance. The cysts keep urine from flowing freely through the tubules. MSK is present at birth but symptoms typically do not occur until adolescence or adulthood. Problems caused by MSK include blood in the urine, kidney stones, and urinary tract infections. MSK rarely leads to more serious problems, such as total kidney failure. There is no cure for this condition, so treatment is aimed at removing kidney stones and treating urinary tract infections with antibiotics.


name: Nephrotic Syndrome-Remission, Partial Remission

description: Nephrotic syndrome in which proteinuria has decreased by at least 50% and below the nephrotic range cutoff from disease onset, but does not normalize following a defined course of treatment.


description: Glomerulonephritis characterized by endocapillary proliferation and the presence of inflammatory cells affecting 50% or more of all glomeruli.


Stage 3 chronic kidney disease

description: A type of chronic kidney disease with moderately reduced glomerular filtration rate (GFR 30-59 mL/min/1.73 m2).


name: Nephrotic Syndrome - Hepatitis C Virus Associated

description: Nephrotic syndrome associated with a hepatitis C infection.


nephrotic syndrome, type 4

description: Nephrotic syndrome within the first three motnhs of life, characterized initially by increased mesangial matrix, with or without hypertrophy and hyperplasia of podocytes, and eventual glomerular sclerosis.


name: Focal Segmental Glomerulosclerosis, Not Otherwise Specified

description: Segmental scarring of the glomerulus, which may result in isolated proteinuria or nephrotic syndrome, which affects only part of the glomerulus and only some of the glomeruli. Additionally, the Not Otherwise Specified classification excludes FSGS tip, perihilar, collapsing, and cellular variants. (D'Agati VD, et al. "Pathologic Classification of Focal Segmental Glomerulosclerosis: A Working Proposal." Am J Kidney Dis 43.2 (2004): 368-82.)


Nephrotic Syndrome - WT1 Associated

description: Nephrotic syndrome attributed to mutation(s) in the WT1 gene.


Nephrotic Syndrome - SCARB2 Associated

description: Nephrotic syndrome attributed to mutation(s) in the SCARB2 gene.


Membranous lupus nephritis

description: A lupus nephritis subclass with global or segmental subepithelial immune deposits or their morphological sequelae by light microscopy and by immunofluorescence or electron microscopy, with or without mesangial alterations. May occur in combination with class III or IV, in which case both classes are diagnosed. May show advanced sclerosis


name: Nephrotic Syndrome with Lesion of Endothelial Glomerulonephritis

description: A renal functional disorder characterized by proteinuria, edema, hyperlipidemia and hypoalbuminemia. It results from damage to the renal vascular filtration apparatus. It is further characterized by an inflammatory reaction in the endovascular lining of the glomerular capillaries. Sequelae may include hypertension, atherosclerosis, infection, hypercoagulablity and renal failure.


Mesangial proliferative lupus nephritis

description: A subclass of lupus nephritis with purely mesangial hypercellularity of any degree or mesangial matrix expansion by lightmicroscopy, with mesangial immune deposits. A few isolated subepithelial or subendothelial deposits may be visible by immunoflourescence or electron microscopy, but not by light microscopy.


Infundibular Stenosis

description: Narrowing of the infundibulum to the calyx, which produces an impediment to urine flow.


Collapsing glomerulopathy

description: Segmental or global glomerulopathy with tuft wrinkling, collapse and contraction without increased matrix or cells, but with adjacent podocyte hypertrophy and hyperplasia.


Membranous Nephropathy - NEP Induced

description: Membranous nephropathy due to neural endopeptidase (NEP) antibodies.


name: Congenital Nephrotic Syndrome - Infection Associated

description: Nephrotic syndrome presenting within the first three months of life, and which is associated with an infectious process.


acute proliferative glomerulonephritis

description: Inflammation of the glomeruli status post infection with nephritogenic streptococci, most often group A beta hemolytic streptococcus.


subacute glomerulonephritis

description: A term that refers to glomerular damage resulting in hematuria, proteinuria, and azotemia. The histopathologic changes include rapidly progressive glomerulonephritis and membranoproliferative glomerulonephritis.


name: Type I Cryoglobulinemic Glomerulonephritis

description: Glomerulonephritis caused by cryoglobulins which are composed of monoclonal immunoglobulins IgG, IgA, or IgM. It occurs in patients with lymphoproliferative disorders.


immune-complex glomerulonephritis

description: Inflammation of the glomeruli characterized by the accumulation of antibody-antigen immune complexes, resulting in glomerular damage and impaired kidney function.


Membranous Nephropathy - PLA2R Induced

description: Membranous nephropathy due to phospholipase 2 receptor (PLA2R) antibodies.


name: Focal Segmental Glomerulosclerosis Tip Lesion Variant

description: A variant of FSGS characterized by scarring of the glomerulus adjacent to the origin of the proximal convoluted tubule; this occurs in the absence of collapsing and perihilar FSGS. (D'Agati VD, et al. "Pathologic Classification of Focal Segmental Glomerulosclerosis: A Working Proposal." Am J Kidney Dis 43.2 (2004): 368-82.)


Nephropathy caused by heavy metals

description: Damage to the kidney and renal tubules resulting from heavy metal exposure.


Nephrotic Syndrome - ITGB4 Associated

description: Nephrotic syndrome attributed to mutation(s) in the ITGB4 gene.


name: Focal Segmental Glomerulosclerosis Perihilar Variant

description: A variant of FSGS characterized by scarring of the glomerulus where at least 50% of the scars are adjacent to the hilum and must have hyalinosis; this excludes cellular, tip and collapsing FSGS. (D'Agati VD, et al. "Pathologic Classification of Focal Segmental Glomerulosclerosis: A Working Proposal." Am J Kidney Dis 43.2 (2004): 368-82.)


COVID-19-Associated Acute Kidney Injury

description: Acute kidney injury caused by severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2).


mTOR Inhibitor - Induced Nephropathy

description: Kidney damage resulting from exposure to mechanistic target of rapamycin (mTOR) kinase inhibitors.


name: Nephrotic syndrome with lesion of membranoproliferative glomerulonephritis

description: A renal functional disorder characterized by proteinuria, edema, hyperlipidemia and hypoalbuminemia. It results from damage to the renal vascular filtration apparatus. It is further characterized by an inflammatory reaction of the glomerular capillaries due to the proliferation of mesangial cells, expansion of the mesangial matrix and deposition of immune complexes causing subsequent thickening of the glomerular basement membrane. Sequelae may include hypertension, atherosclerosis, infection, hypercoagulablity and renal failure.


Nephrotic Syndrome - PTPRO Associated

description: Nephrotic syndrome attributed to mutation(s) in the PTPRO gene.


focal embolic glomerulonephritis

description: Inflammation of a specific segment of glomeruli, which is associated with subacute bacterial endocarditis, and frequently produces microscopic hematuria without azotemia.


name: Pauci-Immune Glomerulonephritis associated with Eosinophilic Granulomatosis with Polyangiitis

description: Glomerulonephritis in the context of eosinophilic-rich granulomatosis with polyangiitis, eosinophilia, asthma and commonly anti-neutrophil cytoplasmic antibody.


Sickle cell nephropathy

description: Nephropathy secondary to sickle cell disease, characterized by the presence of sickled erythrocytes in the renal medullary vessels, renal ischemia and microinfarctions, renal papillary necrosis, and renal tubular abnormalities.


glomerulosclerosis

description: A hardening of the kidney glomerulus caused by scarring of the blood vessels.


Nephrotic Syndrome - Malaria Associated

description: Nephrotic syndrome associated with malaria.


name: Nephrotic Syndrome with Lesion of Segmental Hyalinosis

description: A renal functional disorder characterized by proteinuria, edema, hyperlipidemia and hypoalbuminemia. It results from damage to the renal vascular filtration apparatus. It is further characterized by an inflammatory reaction in the filtering capillaries and the deposition of plasma components between the endothelium and glomerular basement membrane. Sequelae may include hypertension, atherosclerosis, infection, hypercoagulablity and renal failure.


Incident Nephrotic Syndrome

description: New onset nephrotic syndrome.


Nephritic syndrome

description: A syndrome characterized by hematuria with dysmorphic red blood cells, red blood cell casts, and proteinuria; systemic manifestations may be present, including hypertension, edema, oliguria.


name: sessile serrated polyposis cancer syndrome


name: squamous cell carcinoma of buccal mucosa

description: A squamous cell carcinoma that involves the buccal mucosa.


mucinous adenocarcinoma of the appendix

description: Mucinous adenocarcinoma of the appendix is a very rare, slow growing, well-differentiated epithelial neoplasm of the appendix characterized by abundant mucin production. Clinically, it presents as acute appendicitis (with abdominal pain, fever, leukocytosis) or as pseudomyxoma peritonei (wide-spread presence of mucin within the peritoneal cavity), however some patients may be completely asymptomatic at the time of diagnosis. In many cases, a second gastrointestinal malignancy is present.


name: pancreatic delta cell neuroendocrine tumor

description: A usually malignant neuroendocrine tumor arising from the delta cells of the pancreas. It may be associated with inappropriate secretion of somatostatin and an associated clinical syndrome, or it may be hormonally inactive (non-functioning).


colon Kaposi sarcoma

description: A Kaposi sarcoma arising from the colon.


pancreatic cystadenocarcinoma

description: A cystic adenocarcinoma that arises from the pancreas. It includes the acinar cell and serous cystadenocarcinoma subtypes.


inflammatory bowel disease 6

description: An inflammatory bowel disease that has material basis in variation in the chromosome region 19p13.


cholangitis

description: An acute or chronic inflammatory process affecting the biliary tract.


name: extrahepatic bile duct adenosquamous carcinoma

description: A carcinoma that arises from the extrahepatic bile ducts. It is characterized by the presence of glandular and squamous malignant epithelial components.


polycystic liver disease 1

description: A polycystic liver disease in which the cause of the disease is a mutation in the PRKCSH gene, and is characterized by the appearance of numerous cysts spread throughout the liver.


name: nodular regenerative hyperplasia of the liver

description: Nodular regenerative hyperplasia of the liver is a rare parenchymatous liver disease characterized by diffuse benign transformation of the hepatic parenchyma into multiple small nodules (composed of regenerating hepatocytes) and that is usually asymptomatic but can lead to the development of non-cirrhotic portal hypertension and its complications, including esophageal variceal bleeding, hypersplenism and ascites. It is often associated with rheumatologic, autoimmune, hematologic, and myeloproliferative disorders as well as various immune deficiency states and exposure certain drugs and toxins.


autoimmune hepatitis type 1

description: Autoimmune hepatitis characterized by the presence of antinuclear antibody (ANA) and antismooth-muscle antibody (ASMA).


intestinal infectious disease

description: An infectious disease involving a pathogenic inflammatory response in the intestinal mucosa.


name: membranous obstruction of inferior vena cava


anal canal adenocarcinoma

description: An anal adenocarcinoma arising from the anal canal mucosa. Morphologically, it resembles the adenocarcinoma which arises from the colorectal glandular epithelium. Symptoms include anal pruritus, discomfort when sitting, pain, change in bowel habit, and bleeding.


name: carcinoma of esophagus, salivary gland type


digestive system carcinoma

description: A malignant neoplasm that arises from the epithelium of any part of the digestive system. Representative examples include colorectal carcinoma, esophageal carcinoma, and pancreatic carcinoma.


rectum adenoma

description: An adenoma that arises from the rectum. The group of rectal adenomas includes tubular, villous, and tubulovillous adenomas, traditional serrated adenomas, sessile serrated adenomas/polyps, and familial adenomatous polyposis.


porphyria cutanea tarda

description: The most common form of chronic hepatic porphyria. It is characterized by bullous photodermatitis.


hepatic angiomyolipoma

description: An angiomyolipoma arising from the liver.


gallbladder adenosquamous carcinoma

description: A carcinoma that arises from the gallbladder. It is characterized by the presence of glandular and squamous malignant epithelial components.


name: autoimmune enteropathy and endocrinopathy - susceptibility to chronic infections syndrome

description: Autoimmune enteropathy and endocrinopathy-susceptibility to chronic infections syndrome is an extremely rare, autosomal dominant immunological disorder characterized by variable enteropathy, endocrine disorders (e.g. type 1 diabetes mellitus, hypothyroidism), immune dysregulation with pulmonary and blood-borne bacterial infections, and fungal infections (chronic mucocutaneous candidiasis) developing in infancy. Other manifestations include short stature, eczema, hepatosplenomegaly, delayed puberty, and osteoporosis/osteopenia.


rectal neoplasm

description: A benign or malignant neoplasm that affects the rectum. Representative examples of benign neoplasms include lipoma and leiomyoma. Representative examples of malignant neoplasms include carcinoma, lymphoma, and sarcoma. Rectal adenomas always exhibit epithelial dysplasia and are considered premalignant neoplasms.


pyloric gland adenoma

description: A rare neoplastic polyp that arises from the stomach. It is characterized by the presence of gastric epithelial differentiation and pyloric gland-type tubular structures, which are closely packed.


lipoatrophic diabetes

description: A rare syndrome characterized by almost complete absence of body fat, accentuated muscularity, insulin-resistant diabetes, hyperlipidemia, hepatomegaly, and hypermetabolism.


name: hereditary neuroendocrine tumor of small intestine

description: An instance of neuroendocrine tumor of the small intestine that is caused by an inherited modification of the individual's genome.


fibrolamellar hepatocellular carcinoma

description: A distinctive type of liver cell carcinoma that arises in non-cirrhotic livers and is seen predominantly in young patients. The tumor cells are polygonal and deeply eosinophilic, and are embedded in a fibrous stroma. The prognosis is similar to classical hepatocellular carcinoma that arises in non-cirrhotic livers, and better than hepatocellular carcinoma that arises in cirrhotic livers.


intrahepatic bile duct cancer

description: A cancer that involves the intrahepatic bile duct.


name: small intestinal neuroendocrine tumor G1

description: A well differentiated, low grade neuroendocrine tumor (carcinoid tumor) that arises from the small intestine. The mitotic count is less than 2 per 10 HPF and/or the Ki67 index is equal to or less than 2 percent.