Literature DB >> 9173210

[Alström syndrome--a rare disease of diabetic association].

B Weichenhain1, J Stemplinger, A G Ziegler, W Rabl, E Standl, H Stiegler.   

Abstract

BACKGROUND: Alström's disease is a rare hereditary multiple-system illness, whereas a second-messenger defect can be assumed. CASE REPORT: We describe a case-the first in Germany of 15 known cases in the world literature-, who suffers from all clinical features, such as non-insulin-dependent diabetes mellitus, retinitis pigmentosa, pancochlear damage of the ears, hypogonadism, obesity and chronic nephropathy, with the exception of acanthosis nigricans.
CONCLUSION: Because of the multiplicity of affected organs the diagnosis of Alström's disease is difficult.

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Year:  1997        PMID: 9173210     DOI: 10.1007/BF03043276

Source DB:  PubMed          Journal:  Med Klin (Munich)        ISSN: 0723-5003


  8 in total

1.  Retinal degeneration combined with obesity, diabetes mellitus and neurogenous deafness: a specific syndrome (not hitherto described) distinct from the Laurence-Moon-Bardet-Biedl syndrome: a clinical, endocrinological and genetic examination based on a large pedigree.

Authors:  C H ALSTROM; B HALLGREN; L B NILSSON; H ASANDER
Journal:  Acta Psychiatr Neurol Scand Suppl       Date:  1959

2.  The Alström syndrome. Report of three cases with further delineation of the clinical, pathophysiological, and genetic aspects of the disorder.

Authors:  J L Goldstein; P J Fialkow
Journal:  Medicine (Baltimore)       Date:  1973-01       Impact factor: 1.889

3.  The syndrome of Laurence-Moon-Bardet-Biedl and allied diseases in Switzerland. Clinical, genetic and epidemiological studies.

Authors:  D Klein; F Ammann
Journal:  J Neurol Sci       Date:  1969 Nov-Dec       Impact factor: 3.181

4.  Familial syndrome of primary testicular insufficiency with normal virilization, blindness, deafness and metabolic abnormalities.

Authors:  R L Weinstein; B Kliman; R E Scully
Journal:  N Engl J Med       Date:  1969-10-30       Impact factor: 91.245

5.  A new familial syndrome characterized by pigmentary retinopathy, hypogonadism, mental retardation, nerve deafness and glucose intolerance.

Authors:  J A Edwards; P K Sethi; A J Scoma; R M Bannerman; L A Frohman
Journal:  Am J Med       Date:  1976-01       Impact factor: 4.965

Review 6.  Banting lecture 1988. Role of insulin resistance in human disease.

Authors:  G M Reaven
Journal:  Diabetes       Date:  1988-12       Impact factor: 9.461

7.  Ophthalmologic and systemic manifestations of Alström's disease.

Authors:  R H Millay; R G Weleber; J R Heckenlively
Journal:  Am J Ophthalmol       Date:  1986-10-15       Impact factor: 5.258

Review 8.  Mitochondrial mutation commonly associated with Leber's hereditary optic neuropathy observed in a patient with Wolfram syndrome (DIDMOAD).

Authors:  D Pilz; O W Quarrell; E W Jones
Journal:  J Med Genet       Date:  1994-04       Impact factor: 6.318

  8 in total

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