IgA NEPHROPATHY, SUSCEPTIBILITY TO, 1

General Information (adopted from Orphanet):

Synonyms, Signs: BERGER DISEASE
IGAN
IGAN1
NEPHRITIS, IgA TYPE
Number of Symptoms 20
OrphanetNr:
OMIM Id: 161950
ICD-10:
UMLs:
MeSH:
MedDRA:
Snomed:

Prevalence, inheritance and age of onset:

Prevalence: No data available.
Inheritance: Autosomal dominant inheritance
Heterogeneous
[Omim]
Age of onset:

Disease classification (adopted from Orphanet):

Parent Diseases: No data available.

Symptom Information: Sort by abundance 

1
(HPO:0000093) Proteinuria 169 / 7739
2
(HPO:0000794) IgA deposition in the glomerulus 4 / 7739
3
(HPO:0000123) Nephritis 18 / 7739
4
(HPO:0012587) Macroscopic hematuria 2 / 7739
5
(HPO:0000790) Hematuria 106 / 7739
6
(HPO:0002907) Microscopic hematuria 27 / 7739
7
(HPO:0003774) Stage 5 chronic kidney disease 78 / 7739
8
(HPO:0003365) Arthralgia of the hip 10 / 7739
9
(HPO:0002829) Arthralgia rare [HPO:skoehler] 79 / 7739
10
(HPO:0005059) Arthralgia/arthritis 141 / 7739
11
(HPO:0000979) Purpura 27 / 7739
12
(HPO:0000822) Hypertension 224 / 7739
13
(OMIM) Immune complex nephritis 2 / 7739
14
(OMIM) Glomerular capillary-loop deposits (rare), seen on biopsy 2 / 7739
15
(HPO:0000006) Autosomal dominant inheritance 2518 / 7739
16
(OMIM) Hematuria, macroscopic and microscopic 2 / 7739
17
(OMIM) Serum circulating immune complexes contain galactose-deficient IgA1 2 / 7739
18
(OMIM) Galactose-deficient IgA1 deposits in the glomerular mesangium, with or without IgM and IgG 2 / 7739
19
(OMIM) Glomerular crescents (rare), seen on biopsy 2 / 7739
20
(HPO:0001425) Heterogeneous 132 / 7739

Associated genes:

ClinVar (via SNiPA)

Gene symbol Variation Clinical significance Reference

Additional Information:

Description: (OMIM) End-stage renal disease (ESRD) is a major public health problem, affecting 1 in 1,000 individuals and with an annual death rate of 20% despite dialysis treatment. IgA nephropathy (IgAN) is the most common form of glomerulonephritis, a principal ...
Clinical Description OMIM IgA nephritis was first described by Berger and Hinglais (1968).

Katz et al. (1980) reported 2 brothers with typical clinical and pathologic features of IgA nephritis. One presented with gross hematuria in association with an upper ...

Molecular genetics OMIM - Associations Affecting Progression to End-Stage Renal Disease

Yoshida et al. (1995) studied 53 patients with biopsy-proven IgA nephropathy in whom creatinine clearance had been monitored over 5 years. Studying polymorphism of the ACE gene consisting ...

Population genetics OMIM Katz et al. (1980) stated that the frequency of IgAN is said to be low in Britain and high in France, Australia, Hungary, and parts of North America.

McCoy et al. (1974) stated that the disorder ...