Monatsschrift fur Kinderheilkunde最新文献

57 premature and full-term newborn infants suffering from a perinatal asphyxia were neurologically and computertomographically examined during their first two weeks of life. No pathognomonical morphological patterns were found comparing the neurological syndromes with the computertomographic results. However, characteristical morphologic findings were obtained related to gestational age and typical neurological syndromes of the newborn period. The cranial computerized tomography permits in many cases a localized topical diagnosis and early prognosis. These morphological findings can have therapeutical consequences.

Enuresis is a frequent disease in childhood--entailing personal misery, shame, and discredit for the children concerned as well as irritation and a bigger workload for the responsible adults. Parents and teachers often tend to react to this uncontrolled urinating as to a personal provocation. The following study is meant to summarize different aetiological approaches from which therapeutic strategies are derived and classified according to pragmatic considerations. By resorting to common psychotherapeutic methods pediatricians can successfully treat 2/3--3/4 of all cases.

A 4.5 months old girl was suspected to have Glycogenosis type I because of hepatomegalie and recurrent hypoglycemia. Liverbiopsy revealed a normal glycogen content and a normal in vitro activity of glucose-6-phosphatase. We then examined the carbohydrate metabolism and could demonstrate that in vitro the transfer of glucose-6-phosphate to glucose was blocked. We therefore conclude that a normal in vitro activity of glucose-6-phosphatase does not rule out the diagnosis of Glycogenosis type I. Evaluation of carbohydrate metabolism is an important tool in marking the diagnosis. We suggest to use the term Glycogenosis type I B, which some institutions already use for this disorder.

A hemolytic uremic syndrome (HUS) reoccurred 13 months after the first episode in an 8 years old girl. Both episodes were treated by peritoneal dialysis. They had all signs of HUS: renal failure, severe anemia with fragmented erythrocytes and thrombocytopenia. Interesting was the transient thrombocytosis following the primary thrombocytopenia. 2.5 years since the second episode the girl is healthy with intact renal functions.

The condition of varicocele is rare in childhood. Usually it is not observed before puberty, and can cause infertility if not operated upon in time. With the usual examination of the children in supine position the varicocele can be overlooked. The primary idiopathic type with insufficiency of the spermatic vein is distinguished from the rare secondary symptomatic form, mainly caused by renal tumours. A renal mass should be excluded by an intravenous urography in every case. The percutaneous selective phlebography of the spermatic vein--results in two boys with primary idiopathic varicocele are described--is indicated, if clinical symptoms are still existing despite high ligature of the vein. Further open branches of the spermatic vein and collateral circulation can be shown by this method, the way and type of the second operation better planned, and the patients saved from the threatening infertility.

Measurements of T4, T3, rT3, T3U, and TSH (before and after TRH stimulation) were performed by RIA. 182 children, apparently euthyroid, age 2/12--14 years, were investigated in a cross sectional study. Free T4RIA and free T3RIA Indices and the ratio rT3/T3 and T4/T3 were calculated. Statistical analysis showed the following results: 1. Geometric mean serum concentrations of T4, T3, rT3 and TSH (basal) show no age related differences; T4 showed a not significant negative slope with age.--2. The ratio rT3/T3 and T4/T3 remains constant.--3. TRH induced TSH release (indicating the activity of the regulatory system) is unchanged from 2/12 to 14 years. Geometric means values, standard deviations and normal ranges are given.

In two different families each time two siblings head a congenital defect of the diaphragm, in 3 cases of the posterolateral type, in 1 case an almost complete aplasia of the diaphragm. Usually, the occurrence of defects of the diaphragm is sporadic. However, familial occurrence has been reported in 15 cases. Multifactorial inheritance is most likely the explanation for this. With this hypothesis a risk of 2% can be calculated for the recurrence of this malformation in a family with already one affected child.