Genetic characteristics and long-term follow-up of 11 patients with congenital hyperinsulinism followed in a single center.

Background: Congenital hyperinsulinism (CHI) is a rare disease with an estimated incidence of 1:40,000 live births. Here, we characterize 11 patients treated at Munich Children's Hospital Schwabing.

Methods: We analyzed data on birth, treatment and laboratory results including genetic testing and evaluated the long-term course with a follow-up visit.

Current use of metformin in addition to insulin in pediatric patients with type 1 diabetes mellitus: an analysis based on a large diabetes registry in Germany and Austria.

Background: With increasing obesity in childhood and adolescence, weight gain, and insulin resistance become also more frequent in patients with type 1 diabetes mellitus (T1DM). Especially during puberty, insulin therapy often has to be intensified and higher insulin doses are necessary. Some studies point to a beneficial effect of metformin in addition to insulin in these patients.

Severe pretreatment cerebral edema in newly diagnosed type 1 diabetes.

Introduction: Cerebral edema (CE) is a rare and dangerous complication of diabetic ketoacidosis. In typical cases, it may develop during several hours after the beginning of ketoacidosis therapy. Nevertheless, CE sometimes occurs before the start of any therapy - as for the patient in this report here.

GAD65 antigen therapy in recently diagnosed type 1 diabetes mellitus.

Background: The 65-kD isoform of glutamic acid decarboxylase (GAD65) is a major autoantigen in type 1 diabetes. We hypothesized that alum-formulated GAD65 (GAD-alum) can preserve beta-cell function in patients with recent-onset type 1 diabetes.

Methods: We studied 334 patients, 10 to 20 years of age, with type 1 diabetes, fasting C-peptide levels of more than 0.

GCK-MODY (MODY 2) Caused by a Novel p.Phe330Ser Mutation.

Maturity onset diabetes of the young (MODY) is a monogenic form of diabetes inherited as an autosomal dominant trait. The second most common cause is GCK-MODY due to heterozygous mutations in the GCK gene which impair the glucokinase function through different mechanisms such as enzymatic activity, protein stability, and increased interaction with its receptor. The enzyme normally acts as a glucose sensor in the pancreatic beta cell and regulates insulin secretion.

Parenteral fat emulsions based on olive and soybean oils: a randomized clinical trial in preterm infants.

Objective: To evaluate in premature infants a new parenteral lipid emulsion based on olive and soybean oils (ratio 4:1), with less polyunsaturated fatty acids (PUFA) and more alpha-tocopherol than standard soybean oil emulsion.

Study Design: Premature infants (gestational age, 28-<37 weeks) were randomized to receive one of the two emulsions within the first 72 hours of life. The triglyceride dose was increased to 2 g/kg/day within 3 days.