hrp0086p1-p199 | Diabetes P1 | ESPE2016

Anthropometry and Glucose Homeostasis in a Patient with Donohue Syndrome (Homozygous Insulin Receptor Mutation): Effect of Continuous s.c. rIGF-I Therapy

Plamper Michaela , Schreiner Felix , Gohlke Bettina , Wolfle Joachim

Background: Donohue syndrome (DS) is caused by autosomal-recessive loss of function mutations of the insulin receptor gene. DS is associated with diabetes mellitus unresponsive to conventional insulin therapy due to severe insulin resistance. Patients exhibit IUGR and postnatal failure to thrive. They develop a characteristic facies, hypertrichosis and acanthosis nigricans. Most patients die within the first two years of life because of respiratory infections. To date, no caus...

hrp0082p1-d2-151 | Growth (1) | ESPE2014

Sequential Measurements of IGFI Serum Concentrations in Patients With Severe Primary IGFI Deficiency (SPIGFD) and Growth Failure Treated With Recombinant IGFI (Increlex®)

Bettendorf Markus , Kapelari Klaus , Kneppo Carolin , Muller Hermann L , Schnabel Dirk , Wolfle Joachim

Introduction: Increlex® was approved as an orphan drug for treatment of growth failure in children and adolescents with SPIGFD in 2007 with relatively little data available. Therefore sequential measurements of serum IGFI, glucose, insulin and potassium were performed in SPIGFD patients treated with Increlex® to evaluate their significance in safety and efficacy.Design: Blood samples were taken after meals before and 30, 6...

hrp0082p2-d1-321 | Diabetes | ESPE2014

Interaction of Pubertal Development and Metabolic Control in 1303 Adolescents with Diabetes Mellitus Type 1

Hamm Michaela , Gohlke Bettina , Wolfle Joachim , Fink Katharina , Konrad Katja , Rohrer Tilman , Hofer Sabine , Holl Reinhard

Background: T1DM may influence growth and pubertal development and vice versa. Delayed pubertal development and reduced final height are known to be associated with inadequate metabolic control. Many factors including insulin resistance during puberty and insufficient adherence may be responsible for increasing HbA1c.Objective: Is pubertal growth spurt associated with an increase of HbA1c? Are there gender differences in metabolic control during...