Jaundice with hypertrophic pyloric stenosis: a possible manifestation of Gilbert syndrome.
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Biomedical subjects
Publications and source records attributed to A Statz.
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The levels of the individual proteins albumin, caeruloplasmin, immunoglobulin G, and alpha 2-macroglobulin were determined by Laurell's electroimmunoassay in the serum and urine of 127 children. Their age ranged from 29 gestational weeks to 13 years. Highest levels of the proteins in the urine were found during the first days of life. The values did not depend on gestational age. The urine concentrations are influenced by glomerular filtration and the reabsorption of proteins and water. The steady state of these functions can be characterized by the serum/urine concentration ratios and the clearance of the proteins. In the first days after birth the permeability of the renal barrier is maximal, the concentration ratios are low and increase markedly up to the end of the first three months; after that, they continue to rise only slightly. The clearances of all the examined individual proteins decrease between term and the third month of life, thereafter increasing until late childhood. The selectivity of the glomerular filtration also shows its highest rate of development during the first days after term.
The condition of the blood-CSF barrier can be evaluated by the simultaneous quantitation of marker proteins in serum and cerebrospinal fluid. The concentration ratios of albumin and alpha 2-macroglobulin, plotted versus the hydrodynamic radii are used as permeability parameter. There are wide ranging barrier disturbances during the early stages of meningitis. In mumps meningitis only slight disturbances were found, meanwhile in bacterial meningitis the barrier permeability was strongly increased. In both disease groups one may detect secretory fractions of both immunglobulins G and A. In some cases of mumps meningitis a prolonged humoral immune reaction was found.
A 3 6/12 years old girl with micromelic type of spondylo-meta-epiphyseal dysplasia combined with hepatosplenomegaly and muscular hypotonia is described.
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The clinical and radiological findings of Larsen's syndrome are illustrated by one case and the differential diagnosis is discussed. The condition is characterised by multiple congenital subluxations, particularly of the knees; there is a characteristic facial appearance and deformities of the fingers and feet, as well as a number of less constant features. The literature is shown in a table.
In short infusion series run over a 5day period, a fat emulsion on the basis of soya oil with egg lecithin as emulsifier, has been applied to rabbits by the intravenous route in a dosage of 2 and 4 g/kg body-weight/day. The results changes to the liver morphology have been analysed histologically by light-microscope in a systematic manner; using standard intervals of one to fifteen weeks. The most conspicuous phenomenon of the changed liver morphology is a fatty degeneration of the Kupffer's cells of the liver, whose different degree of severity was determined semiquantitatively by determination of the so-called index of fatty degeneration. The loading of the reticulo-endothelial system (RES) of the liver is very heavy with the higher dose; it is lower with a dose of 2 g fat/kg body-weight. The histological changes recede only slowly. A simultaneous parenteral administration of carbohydrates did not result in any provable inferior loading of the RES.
In short infusion series run over 5 days, a fat emulsion on the basis of soya oil with egg lecithin used as emulsifier has been infused to rabbits by the intravenous and intraportal routes in a dosage of 2 and 4 g/kg bodyweight/day. By a light-microscopic analysis of the liver morphology after one and three weeks, mainly a fatty degeneration of the Kupffer's cells of the liver was found. It was considerably less conspicuous after endo-portal supply than after peripheric-intravenous supply--findings which became more obvious with the higher dose.
The presented case report deals with the clinical course of a congenital toxoplasmosis in dicygotic twins. The variability of the clinical course was proofed, because one of the twins remained unaffected with clinical signs and was detected only by the conversion of the seroreactions. On the other hand the second twin showed the picture of an acute meningoencephalitis resulting in a neurological defect syndrome. The reasons for the different clinical course, which is more pronounced in dicygotic twins than monocygotic, remain unknown.