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1.
Diabetes Obes Metab ; 18(7): 693-7, 2016 07.
Article in English | MEDLINE | ID: mdl-26584826

ABSTRACT

Recombinant methionyl human leptin (metreleptin) therapy was shown to improve hyperglycaemia, dyslipidaemia and insulin sensitivity in patients with lipodystrophic syndromes, but its effects on insulin secretion remain controversial. We used dynamic intravenous (i.v.) clamp procedures to measure insulin secretion, adjusted to insulin sensitivity, at baseline and after 1 year of metreleptin therapy, in 16 consecutive patients with lipodystrophy, diabetes and leptin deficiency. Patients, with a mean [± standard error of the mean (s.e.m.)] age of 39.2 (±4) years, presented with familial partial lipodystrophy (n = 11, 10 women) or congenital generalized lipodystrophy (n = 5, four women). Their mean (± s.e.m.) BMI (23.9 ± 0.7 kg/m(2) ), glycated haemoglobin levels (8.5 ± 0.4%) and serum triglycerides levels (4.6 ± 0.9 mmol/l) significantly decreased within 1 month of metreleptin therapy, then remained stable. Insulin sensitivity (from hyperglycaemic or euglycaemic-hyperinsulinaemic clamps, n = 4 and n = 12, respectively), insulin secretion during graded glucose infusion (n = 12), and acute insulin response to i.v. glucose adjusted to insulin sensitivity (disposition index, n = 12), significantly increased after 1 year of metreleptin therapy. The increase in disposition index was related to a decrease in percentage of total and trunk body fat. Metreleptin therapy improves not only insulin sensitivity, but also insulin secretion in patients with diabetes attributable to genetic lipodystrophies.


Subject(s)
Diabetes Mellitus, Type 1/drug therapy , Hypoglycemic Agents/therapeutic use , Insulin/metabolism , Leptin/analogs & derivatives , Lipodystrophy/genetics , Adult , Diabetes Mellitus, Type 1/metabolism , Female , Glycated Hemoglobin/metabolism , Humans , Hyperglycemia/chemically induced , Hypolipidemic Agents/therapeutic use , Insulin/administration & dosage , Insulin Resistance/physiology , Insulin Secretion , Lamin Type A/genetics , Leptin/deficiency , Leptin/therapeutic use , Lipodystrophy/drug therapy , Male , Mutation/genetics , Syndrome , Triglycerides/metabolism
2.
Ann Intern Med ; 134(9 Pt 1): 721-8, 2001 May 01.
Article in English | MEDLINE | ID: mdl-11329229

ABSTRACT

BACKGROUND: Maternally inherited diabetes and deafness (MIDD), which is seen in 0.5% to 2.8% of patients with type 2 diabetes mellitus, is related to a point mutation at position 3243 of mitochondrial (mt) DNA. Its clinical description is incomplete. OBJECTIVE: To study the clinical presentation and complications of diabetes in patients with MIDD and to identify clinical characteristics that may help select diabetic patients for mtDNA mutation screening. DESIGN: Multicenter prospective descriptive study. SETTING: 16 French departments of internal medicine, diabetes and metabolic diseases, or both. PATIENTS: 54 patients with type 2 diabetes mellitus and the mtDNA 3243 mutation. MEASUREMENTS: Characteristics of diabetes, metabolic control (glycosylated hemoglobin level), complications of diabetes, and involvement of other organs. RESULTS: On average, patients with MIDD were young at diabetes onset and presented with a normal or low body mass index. None were obese. Seventy-three percent of probands had a maternal family history of diabetes. Diabetes was non-insulin-dependent at onset in 87% of patients; however, 46% of patients had non-insulin-dependent disease at onset but progressed to insulin therapy after a mean duration of approximately 10 years. Neurosensory hearing loss was present in almost all patients. Eighty-six percent of patients who received an ophthalmologic examination had macular pattern dystrophy (a specific retinal lesion). Forty-three percent of patients had myopathy, 15% had cardiomyopathy, and 18% (9 of 51) had neuropsychiatric symptoms. Although the prevalence of diabetic retinopathy was 8% among patients who received an ophthalmologic examination, lower than expected after a mean 12-year duration of diabetes, prevalence of kidney disease was 28%. This suggests that a specific renal involvement was the result of mitochondrial disease. CONCLUSIONS: Maternally inherited diabetes and deafness has a specific clinical profile that may help identify diabetic patients for mtDNA testing.


Subject(s)
Deafness/genetics , Diabetes Mellitus, Type 1/genetics , Diabetes Mellitus, Type 2/genetics , Adolescent , Adult , Age of Onset , Aged , Analysis of Variance , Body Mass Index , Child , DNA, Mitochondrial/genetics , Deafness/complications , Diabetes Mellitus, Type 1/complications , Diabetes Mellitus, Type 1/pathology , Diabetes Mellitus, Type 2/complications , Diabetes Mellitus, Type 2/pathology , Diabetic Angiopathies/epidemiology , Diabetic Angiopathies/pathology , Female , Humans , Macula Lutea/pathology , Male , Middle Aged , Neuromuscular Diseases/complications , Point Mutation , Prospective Studies , Statistics, Nonparametric , Surveys and Questionnaires
3.
Arch Anat Cytol Pathol ; 41(5-6): 245-50, 1993.
Article in French | MEDLINE | ID: mdl-8135587

ABSTRACT

The clinical investigations carried out in a 58 years woman complaining of malaise led to the discovery of an hypoglycaemia resulting from a secreting pancreatic insulinoma. In addition, a chronic pancreatitis, an endocrine hyperplasia (possible nesidioblastosis) and a villous adenomatosis of the pancreatic duct were diagnosed on two biopsies. The immunohistological tests performed on the insulinoma showed insulin, calcitonin and gastrin labelled cells. Electron microscopy displayed numerous neurosecretory granules. The peritumoral endocrine hyperplasia contained intermingled B, A and D cells respectively labelled by insulin, glucagon and somatostatin. Following the operation, the patient recovered without recurrence of the hypoglycaemia (three year follow-up). Factors which may explain such a rare pathological association are discussed.


Subject(s)
Adenoma, Villous/pathology , Insulinoma/pathology , Pancreatic Neoplasms/pathology , Pancreatitis/pathology , Adenoma, Villous/complications , Chronic Disease , Female , Follow-Up Studies , Humans , Insulinoma/complications , Insulinoma/surgery , Middle Aged , Pancreatic Ducts/pathology , Pancreatic Neoplasms/complications , Pancreatic Neoplasms/surgery , Pancreatitis/complications
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