Resistance to leptin-replacement therapy in Berardinelli–Seip congenital lipodystrophy: an immunological origin
نویسندگان
چکیده
منابع مشابه
Resistance to leptin-replacement therapy in Berardinelli-Seip congenital lipodystrophy: an immunological origin.
CONTEXT Recently, in a 4-month proof-of-concept trial, beneficial metabolic effects were reported in non-diabetic children with Berardinelli-Seip congenital lipodystrophy (BSCL); this information prompted us to hypothesize that long-term leptin-replacement therapy might improve or reverse the early complications of the disease in these patients. PATIENTS AND METHODS A 28-month trial was imple...
متن کاملCongenital leptin deficiency: diagnosis and effects of leptin replacement therapy.
To describe our 10-year experience in treating leptin-deficient humans. Three adults and one boy presented with childhood-onset morbid obesity, hypogonadism and family history of obesity and early death. Serum leptin was inappropriately low. A recessive C105T leptin gene mutation was identified. Metabolic and endocrine assessments were conducted, before and while on and off leptin. The adults' ...
متن کاملEffects of Leptin Replacement Therapy on Pancreatic b-Cell Function in Patients With Lipodystrophy
In a prospective, open-label, currently ongoing study we studied the effects of leptin replacement on b-cell function in 13 patients with congenital or acquired lipodystrophy. Insulin secretory rate (ISR) was calculated by C-peptide deconvolution from plasma glucose and C-peptide levels measured during oral glucose tolerance tests (OGTTs) performed at baseline and after 16–20 weeks of leptin re...
متن کاملEffects of Leptin Replacement Therapy on Pancreatic β-Cell Function in Patients With Lipodystrophy
OBJECTIVE Leptin administration is known to directly modulate pancreatic β-cell function in leptin-deficient rodent models. However, human studies examining the effects of leptin administration on β-cell function are lacking. In this study, we examined the effects (16-20 weeks) of leptin replacement on β-cell function in patients with lipodystrophy. RESEARCH DESIGN AND METHODS In a prospective,...
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Molecular genetic studies have pointed to a relationship between congenital lipodystrophy syndromes and some cardiac disorders. For instance, mutations in LMNA cause either lipodystrophy or cardiomyopathy, indicating that different mutations in the same gene can produce these clinical syndromes. The present authors describe a 10-year-old female with Berardinelli-Seip congenital complete lipodys...
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ژورنال
عنوان ژورنال: European Journal of Endocrinology
سال: 2010
ISSN: 0804-4643,1479-683X
DOI: 10.1530/eje-09-1027