SURGICAL TREATMENT OF ATIPICAL FORMS OF CONGENITAL HYPERINSULINISM
نویسندگان
چکیده
منابع مشابه
Sirolimus treatment of severe congenital hyperinsulinism
Sirolimus treatment reduced dependence on octreotide and frequent feeding in 4 infants with CHI in a recent study [1]. We report our experience in a 1 year-old boy with presumed diffuse disease due to a de novo heterozygous ABCC8 missense mutation (p.D1506E). This macrosomic infant (term birth weight 5.676kg) presented with hypoglycaemia in the first hours of life. Critical sample results were ...
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چکیده ندارد.
15 صفحه اولSurgical Management of Congenital Hyperinsulinism in a Resource-Limited Setting
Laboratory investigations revealed hemoglobin 14.6 g/dL, total leukocyte count 12X109/L, and platelet count 290X109/L. C-reactive protein (CRP) was negative. During the hospital stay, the infant had repeated generalized and focal clonic seizures during documented hypoglycemic episodes requiring glucose infusion rate (GIR) up to 14 mg/kg/min. There was no ketosis, hyperammonemia, or lactic acido...
متن کاملCongenital Hyperinsulinism: Diagnosis and Treatment Update
Pancreatic β-cells are finely tuned to secrete insulin so that plasma glucose levels are maintained within a narrow physiological range (3.5-5.5 mmol/L). Hyperinsulinaemic hypoglycaemia (HH) is the inappropriate secretion of insulin in the presence of low plasma glucose levels and leads to severe and persistent hypoglycaemia in neonates and children. Mutations in 12 different key genes (ABCC8, ...
متن کامل[Congenital hyperinsulinism].
In the last five years, our knowledge about the heterogenous syndrome of congenital hyperinsulinism (HI) has expanded explosively. HI may be familiar or sporadic, mild or severe, transitory or persistent, and histologically focal or diffuse. At least 63 disease-causing mutations have been found in the genes for the beta cell's ATP-dependent potassium channel, whose elements are the sulphonylure...
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ژورنال
عنوان ژورنال: Russian Journal of Pediatric Surgery
سال: 2020
ISSN: 2412-0677,1560-9510
DOI: 10.18821/1560-9510-2020-24-2-83-88