Klaus A. Zuppinger

Endocrine function of the pancreas in cystic fibrosis: Evidence for an impaired glucagon and insulin response following arginine infusion*

Pancreatic glucagon and insulin were determined in 10 children with cystic fibrosis (C.F.) and in a control group following stimulation by an arginine infusion. In controls the infusion of arginine induced a prompt threefold elevation in plasma pancreatic glucagon and about a sixfold increase in plasma immunoreactive insulin. In patients with C.F. the average response of both hormones was considerably less, and the base-line concentrations for glucagon were lower. The data support the hypothesis that in C.F. both alpha- and beta-cell function are affected as a result of the underlying process.

The significance of intermediate plasma growth hormone levels in growth-retarded children

The plasma growth hormone response to insulin-induced hypoglycemia has been evaluated in 60 children with growth relardation. Peak plasma GH values of more than 7 ng. per milliliter were found in 31 patients with consitutional growth retardation, and peak values less than 2 ng. per milliliter were found in 21 patients with GH defiency. eight children who had an intermediate peak plasma GH value between 2.8 and 5.3 ng. per milliter could be divided into three categories: normal plasma GH during a repeat test; consistently intermediate plasma GH values with good response to human GH, as in GH deficiency; and consistently intermediate plasma GH values with intermediate effect of human GH on growth and on nitrogen retention, indicating partial GH deficiency.

Defects of Growth Hormone Genes

Human growth hormone (GH) is a 191-amino-acid polypeptide hormone whose production and release by somatotropes of the anterior pituitary is under complex hormonal control. Production of GH is increased by glucocorticoid, thyroid hormone, and growth-hormone-releasing factor (GRF) at least in part by stimulation of transcription of the GH gene.(1) Release of GH into the peripheral circulation is controlled by at least two hypothalamic factors: GRF, which stimulates, and somatostatin, which inhibits its release.(2–5) In turn, these hypothalamic factors are modulated by various influences originating in the central nervous system (CNS).(6) Because of the complexity of these interactions which control the synthesis and release of GH, GH deficiency could be caused by disorders at the level of the CNS, the hypothalamus, or the anterior pituitary. Thus, a wide variety of disorders including CNS developmental defects such as septooptic dysplasia, head trauma, histiocytosis X, tumors, or irradiation can cause GH deficiency.(6,7)

Kohlenhydratstoffwechsel in der perinatalen Periode

Das Neugeborene ist wahrend der ersten Lebenstage weitgehend auf die wahrend der Schwangerschaft angereicherten Energielieferanten angewiesen. Es wird allgemein angenommen, das die notwendige Energie fur die Entfaltung vitaler Funktionen hauptsachlich aus Kohlenhydraten gewonnen wird.