Wolframův syndrom [Wolfram Syndrome]

tematický
8
Termíny

DIDMOAD syndrom
Wolframův syndrom 1

 

Diabetes Insipidus and Mellitus with Optic Atrophy and Deafness
Diabetes Insipidus, Diabetes Mellitus, Optic Atrophy, and Deafness
DIDMOAD
DIDMOAD Syndrome
DIDMOADUD
Wolfram Syndrome 1

Perzistentní odkaz   https://www.medvik.cz/link/D014929
Definice

Autozomálně recesivní spojení diabetes mellitus a diabetes insipidus, s atrofií optiku a hluchotou; občas se připojuje atonie měchýře a močovodu. Onemocnění začíná ve školním věku větš. cukrovkou. Též DIDMOAD syndrom (diabetes insipidus diabetes mellitus optic atrophy deafness). (cit. Velký lékařský slovník online, 2016 http://lekarske.slovniky.cz/)

A hereditary condition characterized by multiple symptoms including those of DIABETES INSIPIDUS; DIABETES MELLITUS; OPTIC ATROPHY; and DEAFNESS. This syndrome is also known as DIDMOAD (first letter of each word) and is usually associated with VASOPRESSIN deficiency. It is caused by mutations in gene WFS1 encoding wolframin, a 100-kDa transmembrane protein.

DUI
D014929 MeSH Prohlížeč
CUI
M0022993
Předchozí užití
Deafness (1966-1985); Diabetes Insipidus (1966-1985); Diabetes Mellitus, Insulin-Dependent (1984-1985); Optic Atrophy (1966-1985)
Historická pozn.
86
Veřejná pozn.
86

C Nemoci
C09.218 nemoci ucha 315
C09.218.458 poruchy sluchu 1 289
C09.218.458.341 nedoslýchavost 203
C09.218.458.341.186 hluchota 434
C09.218.458.341.186.500 hluchoslepota 65
C09.218.458.341.186.500.250 CHARGE syndrom 1
C09.218.458.341.186.500.500 Usherovy syndromy 8
C09.218.458.341.186.500.750 Wolframův syndrom 8
C10.292.700.225 atrofie optického nervu 51
C10.292.700.225.500.400 Leberova atrofie zrakového nervu 24
C10.292.700.225.500.980 Wolframův syndrom 8
C10.574.500.662.980 Wolframův syndrom 8
C10.597.751 poruchy senzitivity 93
C10.597.751.418 poruchy sluchu 1 289
C10.597.751.418.341 nedoslýchavost 203
C10.597.751.418.341.186 hluchota 434
C10.597.751.418.341.186.500 hluchoslepota 65
C10.597.751.418.341.186.500.250 CHARGE syndrom 1
C10.597.751.418.341.186.500.500 Usherovy syndromy 8
C10.597.751.418.341.186.500.750 Wolframův syndrom 8
C10.597.751.941 poruchy zraku 703
C10.597.751.941.162 slepota 424
C10.597.751.941.162.625 hluchoslepota 65
C10.597.751.941.162.625.250 CHARGE syndrom 1
C10.597.751.941.162.625.500 Usherovy syndromy 8
C10.597.751.941.162.625.750 Wolframův syndrom 8
C11 oční nemoci 1 485
C11.270.564.980 Wolframův syndrom 8
C11.640.451.451.980 Wolframův syndrom 8
C11.966 poruchy zraku 703
C11.966.075 slepota 424
C11.966.075.375 hluchoslepota 65
C11.966.075.375.250 CHARGE syndrom 1
C11.966.075.375.500 Usherovy syndromy 8
C11.966.075.375.750 Wolframův syndrom 8
C12.050.351.968 urologické nemoci 834
C12.050.351.968.419 nemoci ledvin 3 059
C12.050.351.968.419.135 diabetes insipidus 173
C12.050.351.968.419.135.500 nefrogenní diabetes insipidus 19
C12.050.351.968.419.135.750 centrální diabetes insipidus 23
C12.050.351.968.419.135.875 Wolframův syndrom 8
C12.200.777 urologické nemoci 834
C12.200.777.419 nemoci ledvin 3 059
C12.200.777.419.135 diabetes insipidus 173
C12.200.777.419.135.500 nefrogenní diabetes insipidus 19
C12.200.777.419.135.750 centrální diabetes insipidus 23
C12.200.777.419.135.875 Wolframův syndrom 8
C12.950.419 nemoci ledvin 3 059
C12.950.419.135 diabetes insipidus 173
C12.950.419.135.500 nefrogenní diabetes insipidus 19
C12.950.419.135.750 centrální diabetes insipidus 23
C12.950.419.135.875 Wolframův syndrom 8
C16.131 vrozené vady 1 740
C16.131.077.299 hluchoslepota 65
C16.131.077.299.250 CHARGE syndrom 1
C16.131.077.299.500 Usherovy syndromy 8
C16.131.077.299.750 Wolframův syndrom 8
C16.320.290.564.980 Wolframův syndrom 8
C16.320.400.630.980 Wolframův syndrom 8
C18.452 metabolické nemoci 1 196
C18.452.394.750 diabetes mellitus 9 524
C18.452.394.750.124 diabetes mellitus 1. typu 3 001
C18.452.394.750.124.960 Wolframův syndrom 8
C19.246 diabetes mellitus 9 524
C19.246.267 diabetes mellitus 1. typu 3 001
C19.246.267.960 Wolframův syndrom 8
C19.700 nemoci hypofýzy 126
C19.700.159 diabetes insipidus 173
C19.700.159.875 Wolframův syndrom 8