Literature DB >> 209163

Congenital lipodystrophy: An endocrine study in three siblings. I. Disorders of carbohydrate metabolism.

C Huseman, A Johanson, M Varma, R M Blizzard.   

Abstract

Three siblings with congenital lipodystrophy were studied extensively for endocrine abnormalities. A severe disturbance in carbohydrate metabolism was observed. Plasma concentrations of glucagon and insulin were markedly elevated both in the basal state and in response to provocative stimuli. In addition, marked resistance to exogenous insulin and a diabetic oral glucose tolerance test were demonstrated. Lipid metabolism, GH, and ACTH secretion were normal...

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Year:  1978        PMID: 209163     DOI: 10.1016/s0022-3476(78)80500-9

Source DB:  PubMed          Journal:  J Pediatr        ISSN: 0022-3476            Impact factor:   4.406


  4 in total

1.  Seip-Berardinelli syndrome.

Authors:  G P Das; L Mehta
Journal:  Indian J Pediatr       Date:  1991 Jul-Aug       Impact factor: 1.967

2.  Metabolic manifestations of insulin deficiency do not occur without glucagon action.

Authors:  Young Lee; Eric D Berglund; May-yun Wang; Xiaorong Fu; Xinxin Yu; Maureen J Charron; Shawn C Burgess; Roger H Unger
Journal:  Proc Natl Acad Sci U S A       Date:  2012-08-13       Impact factor: 11.205

3.  The development of hyperglycaemia in patients with insulin-resistant generalized lipoatrophic syndromes.

Authors:  J J Robert; B Rakotoambinina; I Cochet; V Foussier; J Magre; D Darmaun; D Chevenne; J Capeau
Journal:  Diabetologia       Date:  1993-12       Impact factor: 10.122

4.  Mutations in Gng3lg and AGPAT2 in Berardinelli-Seip congenital lipodystrophy and Brunzell syndrome: phenotype variability suggests important modifier effects.

Authors:  Mao Fu; Rasa Kazlauskaite; Maria de Fátima Paiva Baracho; Maria Goretti Do Nascimento Santos; José Brandão-Neto; Sandra Villares; Francesco S Celi; Bernardo L Wajchenberg; Alan R Shuldiner
Journal:  J Clin Endocrinol Metab       Date:  2004-06       Impact factor: 5.958

  4 in total

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