Atherosclerosis-deafness-diabetes-epilepsy-nephropathy syndrome

Orpha code: 1192OMIM code: 209010

Definition

A rare, severe, circulatory system disease characterized by premature, diffuse, severe atherosclerosis (including the aorta and renal, coronary, and cerebral arteries), sensorineural deafness, diabetes mellitus, progressive neurological deterioration with cerebellar symptoms and photomyoclonic seizures, and progressive nephropathy. Partial deficiency of mitochondrial complexes III and IV in the kidney and fibroblasts (but not in muscle) may be associated. There have been no further descriptions in the literature since 1994.

Disease data
Classification

Malformation syndrome

Synonyms
Atherosclerosis-hearing loss-diabetes-epilepsy-nephropathy syndrome
Zespół Feigenbauma, Bergerona i Richardsona
Feigenbaum-Bergeron-Richardson syndrome
ORPHA code
1192
OMIM code
209010
ICD10 code
I70.9
ICD11 code
LD2H.Y

No additional description.

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