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Nephritis, Hereditary

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Definition: A group of inherited conditions characterized initially by HEMATURIA and slowly progressing to RENAL INSUFFICIENCY. The most common form is the Alport syndrome (hereditary nephritis with HEARING LOSS) which is caused by mutations in genes for TYPE IV COLLAGEN and defective GLOMERULAR BASEMENT MEMBRANE.      Other names Nephritis, Familial; Alport's Syndrome; X-Linked Alport Syndrome; Syndromes, Alport; Syndrome, X-Linked Alport; Syndrome, Alport's; Syndrome, Alport; Nephritides, Hereditary; Nephritides, Familial; Hereditary Nephritis; Hereditary Nephritides; Familial Nephritis; Familial Nephritides; Alports Syndrome; Alport Syndromes; Alport Syndrome, X Linked; Alport Syndrome, X-Linked; Alport Syndrome, Autosomal Recessive; Alport Syndrome, Autosomal Dominant; Alport Syndrome
 
SubstanceCAS Registry & nameCategoriesSource
Hemorrhagic hereditary nephritis  0   *Hematuria *Nephritis, Hereditary.
Salcedo syndrome  0   *Nail-Patella Syndrome *Nephritis, Hereditary.
Leiomyomatosis, esophageal and vulval, with nephropathy  0   *Nephritis, Hereditary *Leiomyomatosis.
Fechtner syndrome  0   *Hearing Loss, Sensorineural *Nephritis, Hereditary *Thrombocytopenia.
Alport syndrome, recessive type  0   *Nephritis, Hereditary.
Alport syndrome, dominant type  0   *Nephritis, Hereditary.
Deafness nephritis ano rectal malformation  0   *Abnormalities, Multiple *Anus, Imperforate *Hearing Loss, Central *Nephritis, Hereditary Anal Canal/abnormalities Rectum/abnormalities.
Daentl Towsend Siegel syndrome  0   *Hydrocephalus *Nephritis, Hereditary *Scleral Diseases.
Epstein syndrome  0   *Nephritis, Hereditary *Thrombocytopenia.
C3 protein, human  0   *Complement C3 Nephritis, Hereditary. J Biol Chem 1983;258(11):7200

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Sources: NLM Medical Subject Headings, NIH UMLS, Drugs@FDA, FDA AERS original data copyright United States Government. No endorsement implied. Last modified 6/6/2012
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