Rolf P. Zurbrügg

Long-Term Treatment of Infantile Nephropathic Cystinosis with Cysteamine

CYSTINOSIS is a rare autosomal recessive metabolic disorder of children and adults, characterized biochemically by intracellular accumulation of free, nonprotein cystine crystals in the reticuloend...

EVALUATION OF THE EFFECTIVENESS OF TREATMENT ON ADULT HEIGHT PROGNOSIS IN DISORDERS WITH ADVANCED AND RETARDED BONE AGE Height and Height Velocity related to Chronological or to Bone Age

Different growth parameters and their graphical presentation in disorders with advanced and retarded bone age are discussed. Their usefulness for the evaluation of the effect of treatment on growth and maturation is compared. In precocious puberty, more information is obtained when height is plotted against bone age instead of chronological age. It is more reasonable from a physiological standpoint to plot height velocity against bone age rather than chronological age. For the evaluation of the efficiency of treatment on adult height prognosis in precocious puberty, two possibilities are proposed: height velocity expressed as height increment per bone age year, or the quotient height age increment/bone age increment. However, where skeletal maturation is severely retarded, as in hypopituitary growth retardation, these growth parameters should be related in the conventional way to chronological age, rather than to bone age.

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.