Last update 01 Nov 2024

Alport Syndrome-Like Hereditary Nephritis

Last update 01 Nov 2024

Basic Info

Synonyms

Alport Syndrome-Like Hereditary Nephritis, Alport syndrome-like hereditary nephritis, Alport syndrome-like hereditary nephritis (ASLHN, ASLN)

+ [2]

Introduction-

Drugs associated with Alport Syndrome-Like Hereditary Nephritis

VAR-200

Target-

Mechanism-

Active Org.

Ege Universitesi [+1]

Originator Org.

L&F Co., Ltd.

Active Indication

Diabetic Nephropathies [+1]

Inactive Indication

Alport Syndrome-Like Hereditary Nephritis [+1]

Drug Highest PhasePhase 2

First Approval Ctry. / Loc.-

First Approval Date20 Jan 1800

100 Clinical Results associated with Alport Syndrome-Like Hereditary Nephritis

100 Translational Medicine associated with Alport Syndrome-Like Hereditary Nephritis

0 Patents (Medical) associated with Alport Syndrome-Like Hereditary Nephritis

Literatures (Medical) associated with Alport Syndrome-Like Hereditary Nephritis

01 Jul 2001·The Israel Medical Association journal : IMAJQ4 · MEDICINE

Autosomal dominant nephritis with renal failure of non-Alport type: clinical and molecular studies.

Q4 · MEDICINE

Article

Author: Magal, N ; Tobar, A ; Halpern, G J ; Shohat, T ; Shohat, M ; Ilan, T ; Yahav, M ; Rechavi, G

BACKGROUNDFamilial nephritis is a heterogeneous group of disorders caused by several genetic conditions such as Alport syndrome, glomerulonephritic syndromes, and unclassified nephritis without deafness or ocular defects.OBJECTIVESTo describe a family of Iraqi Jewish origin, several of whose members suffer from non-syndromic renal failure without deafness or ocular defects and where transmission is by autosomal dominant inheritance. We present the case histories of four family members and describe the molecular analysis performed in order to seek a possible linkage to one of the genes causing Alport or Alport-like syndromes.METHODSWe investigated all family members over the age of 18 for evidence of renal failure. We also extracted DNA and carried out molecular linkage analysis with polymorphic markers in each of the known loci involved in Alport and Alport-like syndromes.RESULTSHistology of the renal biopsy specimens showed non-specific findings. Linkage was excluded for all the Alport and Alport-like syndrome loci.CONCLUSIONSThe condition suffered by several members of this family seems to represent a unique autosomal dominant type of progressive hereditary nephritis, characterized by hypertension and progressive renal failure without significant hematuria or proteinuria. The main histological changes are non-specific in the early stage of the disease. Our study rules out all the currently known genes that cause Alport syndrome as being responsible for the basic defect in this type of nephritis.

28 Jul 1997·Pediatric NephrologyQ3 · MEDICINE

Renal problems in black South African children *

Q3 · MEDICINE

Review

Author: Thomson, P D

Black South African children have an increased prevalence of acute post-streptococcal glomerulonephritis, focal glomerulosclerosis, hepatitis B-associated membranous nephropathy, congenital syphilis, congenital nephrotic syndrome with Alport-like basement membrane changes and Takayasu's arteritis, but a paucity of reflux nephropathy, polycystic kidney disease and non-shigella-induced haemolytic uraemic syndrome. However, in recent years, the haemolytic uraemic syndrome has become more prevalent in black children; this is usually due to Shigella dysenteriae type 1, and could indicate a different emphasis in the pathogenic mechanism. Focal glomerulosclerosis is the commonest reason for renal failure requiring transplantation in black children with rapidly progressive glomerulonephritis (usually streptococcal induced) being second. Transplantation has been somewhat problematic with a scarcity of kidneys at times, many mismatches and poor compliance in the lower socio-economic groups. The association of tuberculosis with focal glomerulosclerosis and Takayasu's arteritis in black South African children is intriguing. The progression of focal glomerulosclerosis is accelerated in those patients with evidence of previous tuberculosis, and in Takayasu's arteritis the association is almost absolute as experienced locally, with the data suggesting an autoimmune reaction.

17 Jul 1995·American Journal of Medical Genetics

Skewed X‐inactivation in a tumor tissue from a female patient with leiomyomatosis

Letter

Author: Y. Jinno ; T. Hashida ; N. Niikawa ; T. Kishino

Leiomyomatosis (multiple leiomyomas) is characterized by benign smooth muscle cell proliferations in the esophagus, tracheobronchial tree, and female genital tract. At least 3 genetically different hereditary leiomyomatoses have been identified. Among them, an X-linked leiomyomatosis is often associated with an Alport syndrome-like nephropathy. It has remained obscure whether the leiomyomata occur monoclonally or polyclonally. The clonality of various malignancies has been examined by analysis of X-inactivation patterns in female patients heterozygous for polymorphic alleles of X-linked genes. We examined the clonality of a leiomyoma in a female patient by a polymerase chain reaction (PCR)-based X-inactivation assay. 6 refs., 1 fig.

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Alport Syndrome-Like Hereditary Nephritis

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