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Biomedical subjects

G Wolff

Publications and source records attributed to G Wolff.

At least 199 records · Page 11Linked to original sources
PubMed1979

Clinical and biochemical investigations on patients with partial deficiency of placental steroid sulfatase.

We report on three independent cases with a partial deficiency of placental steroid sulfatase (E.C.3.1.6.2). Upon routine pregnancy monitoring these patients were detected on the basis of low estriol excretion and failing induction of labor. In all three cases a male was delivered and subsequently the diagnosis of partial deficiency of placental steroid sulfatase was confirmed enzymatically in placenta homogenates. In one case, fibroblast cultures were established from skin explants of mother and son. In fibroblasts of the child, as in placental tissue, the activity of steroid sulfatase was only 34% of normal. Similar values were obtained for arylsulfatase C, though this enzyme is clearly separable from steroid sulfatase by electrophoresis. In cells of the mother, enzyme activities were unremarkable.

Arylsulfatases↗
PubMed1979

High-dose propranolol therapy in the management of supraventricular tachycardia.

Five patients, age 3 weeks to 11 years, presented with supraventricular tachycardia that remained uncontrolled following adequate digitalization. Four of these patients underwent invasive electrophysiologic studies to determine the mechanism of the arrhythmias. Of these four patients, three had concealed Wolff-Parkinson-White syndrome, and one patient had evidence of dual A-V nodal pathways. Propranolol was added to the medical treatment and was administered orally in doses ranging from 7 to 14 mg/kg/day (average 9 mg/kg/day). All five children remain free of their tachycardia except for one patient who occasionally has supraventricular tachycardia with febrile illnesses. No adverse reactions to these high doses of propranolol were encountered.

Child↗
PubMed1979

Right-to-left atrial shunt in cardiac dislocation following extensive pneumonectomy.

Previous reports dealing with cardiac herniation following intrapericardial pneumonectomy illustrate the critical and often lethal hemodynamic sequelae of this complication. In the case presented here, the first and nearly exclusive sign of cardiac herniation after left-sided pneumonectomy with extensive resection of the pericardium was systemic arterial hypoxemia. Subsequent investigations suggested inter-atrial right-to-left shunt in the presence of a patent foramen ovale, caused by slight right-ventricular outflow obstruction with consecutively reversed pressure relationships at atrial level. This explanation was supported by the operative findings, and reversibility was achieved by pericardial reconstruction with parietal pleura. When the patient died 8 months later due to general progression of a mucoepidermoid carcinoma, autopsy confirmed a large patent foramen ovale.

Adenocarcinoma, Mucinous↗
PubMed1979

[Psychosocial intellectual development of children with infantile cystinosis and cerebral atrophy (author's transl)].

The psychosocial and intellectual development of 4 boys with nephropathic cystinosis and brain atrophy documented by cranial computerized tomography was investigated by use of biographical data and psychological tests (HAWIK, Deutscher Rechtschreibtest). Inspite of the brain atrophy the patients showed low-normal intellectual capacities and mainly average school performance. There were no psychosocial abnormalities correlated to the primary metabolic disease. However, renal dwarfism led to mascotism requiring psychotherapy.

Atrophy↗
PubMed1978

Preferential X inactivation in human placenta membranes: is the paternal X inactive in early embryonic development of female mammals?

In placenta membranes of newborn girls carrying electrophoretically distinguishable G6PD alleles, the maternally derived isozyme is expressed preferentially. This phenomenon cannot be explained by allelic differences in enzyme activity or by somatic selection directed against cells with particular G6PD phenotypes. Instead, it may be that in this tissue X inactivation is nonrandom. Preferential expression of the maternal X chromosome, as has been shown in marsupials and in extraembryonic membranes of rodents and now in man, may reflect the state of activity of the X chromosomes in the early stages of female embryonic development.

Female↗
PubMed1978

Distribution of G6PD phenotypes in red blood cells of Southern African Negroids: evidence for somatic selection.

In a recent population study, we observed a striking deficit of G6PD heterozygotes among Southern African Negroid females. This finding was interpreted tentatively as evidence for a small number of hematopoetic stem cells in man. In a follow-up study we examined peripheral blood and cord blood in 547 mothers and in their newborn offspring. In mothers and sons, the frequencies of the G6PD alleles are apparently quite different. When the allele frequencies determined in sons are used for calculation of the expected phenotype frequencies in mothers and daughters, there is a large deficit of maternal G6PD AB phenotypes, and an equivalent surplus of G6PD homozygotes. However, no relevant heterozygote deficit is observed in newborn daughters. This discrepancy may be explained by the assumption that in peripheral blood of heterozygotes carrying the GdA- allele, G6PD-deficient cells progressively become eliminated during development from birth to adulthood. In other words, the large heterozygote deficit observed in adult females may be due to somatic selection rather than to a small pool of hematopoetic cells at the time of X differentiation.

Alleles↗
PubMed1978

A rationale for epidural analgesia in the treatment of multiple rib fractures.

Thoracic epidural analgesia (EA) is described as an alternative to controlled ventilation in patients presenting with multiple rib fractures. Lung mechanics were especially studied in 6 patients selected from a total of 49. The average ICU stay for this group was 4.5 days (2-11) and the mean age 55.7 years. The EA group was compared with 51 patients primarily ventilated who had an average stay in the ICU of 9.8 days and a mean age of 44.7 years. Mean number of rib fractures of the ventilated group at 6.5 was almost equal to the mean of 6.8 in the EA group. There was a difference in the number of associated fractures, 98 in the ventilated group compared to 35 in the EA group. Severe pulmonary and cerebral contusion were the two most important factors in enforcing the need to ventilate. The success of the method is evidenced by the increase in functional residual capacity (FRC), dynamic lung compliance (Cdyn), vital capacity (VC), the decrease of airway resistance (R) and a significantly increase of PaO2 (p less than 0,001) for the EA group with a balanced fluid therapy. All this accounts for the clinical observation of diminishing paradoxical movement of the flail segment.

Adult↗
PubMed1978

Glucagon, insulin, cortisol, and growth hormone levels following major surgery: their relationship to glucose and free fatty acid elevations.

Circulating hormone and substrate levels were measured in 7 patients at regular intervals before, during and after pulmonary surgery. During surgery, cortisol and growth hormone were significantly elevated, pancreatic glucagon was unchanged and insulin was depressed. One and two days after surgery, growth hormone had almost returned to preoperative fasting values, but cortisol, insulin and glucagon levels were significantly increased. The mean insulin:glucagon molar ratio declined from a preoperative fasting value of 3.2 +/- 0.5 (+/- SEM) to 1.7 +/- 0.4 during operation but was within normal limits 1 and 2 days after surgery due to a parallel rise and fall in plasma insulin and glucagon. Plasma glucose was elevated both during operation and for several days thereafter, whereas free fatty acid levels were increased only during operation. Thus, there was no consistent relation between insulin:glucagon ratio or any of the hormone levels and the observed elevations in plasma glucose and free fatty acids. It is concluded that neither any of the hormones assayed nor the insulin:glucagon ratio was the primary determinant of plasma glucose and free fatty acid responses to surgery. Rather, fuel homeostasis appeared to result from the combined effects of glucagon, insulin, growth hormone, cortisol and adrenergic activity.

Adult↗