Acta paediatrica Hungarica最新文献

Cerebrospinal fluid and plasma sodium, potassium, chloride, calcium and glucose concentration were measured parallel in 14 pathological newborn babies of gestational age and birthweight of 36.3 +/- 4.3 wks and 2410 +/- 890 g, respectively, at the age of 37.8 +/- 4.4 wks postconceptionally. Whilst potassium, calcium and glucose level is much lower in the cerebrospinal fluid than in the plasma, similar sodium and higher chloride concentration was found in the cerebrospinal fluid. The significant positive correlation between plasma and cerebrospinal fluid glucose and sodium levels proves the lack of a functioning barrier for these compounds. On the other hand, cerebrospinal potassium level varied within a narrow range, independently of the plasma-concentration and the maturity of the studied babies. Pathophysiological implications of the results are further discussed in short.

Linked marker haplotype analysis of 16 cystic fibrosis (CF)-affected children, 3 fetuses, 1 healthy child and their parents was performed by restriction fragment length polymorphism (RFLP) for J3.11, Met H, Met D, XV-2c, KM.19 markers. Polymerase chain reaction (PCR) to detect the main mutation of CF chromosome, a specific 3 base pair (bp) deletion (delta F508) was also performed in 17 CF patients. Allelic frequencies on analysed CF chromosomes were: J3.11/Taq I 1.0, 0.0, J3.11/Msp I 0.44, 0.56, Met H/Taq I 0.73, 0.27, Met H/Msp I 0.80, 0.20, Met D/Taq I 0.75, 0.25, XV-2c/Taq I 0.85, 0.15, KM.19/Pst I 0.17, 0.83 for allele 1 and 2, respectively. Two markers, Met H and KM.19 were found to be in strong association with the CF mutation. The frequency of the delta F508 mutation on all 34 CF chromosomes was 0.65 (of which 0.73 was homozygous and 0.27 heterozygous for this deletion).

Hundred children of 64 fathers with testicular tumour treated from 1979 on at the National Institute of Oncology, Budapest were studied. Three groups were formed on the basis of the time of conception. 59 children were born before the illness of the fathers, 19 during the 9 pretreatment months and 22 during or after combined chemotherapy. Family anamnesis, perinatal and gestational data were listed, thereafter physical, laboratory, immunological, psychiatric, and, if required, radiological examinations were made. No difference was detectable in the somatic and psychiatric status of the three groups, development was well balanced, corresponding to age. Protocols of the combined chemotherapy applied and incidence of anomalies, malformations, malignancies and other diseases were recorded. Their incidence was similar in all three groups though frequently this was higher than that of the normal population. Often cumulated incidence of severe congenital malformations was found in the group conceived after concluded therapy where twice as many girls were born as boys. The interval between conception and the end of therapy was established in the case of children conceived during and after therapy. This was shortest in the case of healthy children, the number of healthy children conceived during cytostatic treatment was also remarkable. Further compilation of data and individual evaluation of case reports is recommended.

Serum sex hormone binding globulin (SHBG) concentration of children with obesity was measured and relationships between SHBG level and body mass index (BMI), waist hip ration (WHR), serum insulin, C-peptide, thyroid hormones (thyroxine--T4, triiodothyronine--T3/ sexual hormones (testosterone--T, oestradiol--E2) were investigated. Significant negative correlations were found between SHBG concentration and BMI, serum insulin, C-peptide concentration; significant positive concentrations were found between BMI and serum insulin, C-peptide concentration. Thyroid hormone and sexual hormones did not associate with SHBG levels. These results suggest that insulin hypersecretion has an important role in determining the reduction of SHBG production in obesity.

Ki-1 positive (anaplastic, large cell) lymphoma is a subgroup of non-Hodgkin lymphomas identified recently by Ki-1 (or BER-H2) (CD 30) monoclonal antibody. The clinicopathological features of two such pediatric cases of lymph node origin described here, and also the available literature emphasize the heterogenous nature of Ki-1 positive lymphomas, in almost every respect. Nevertheless, the Ki-1 antibody serves as an important diagnostic tool to differentiate lymphomas from other anaplastic, large malignancies.

Thirty-one 3 to 14 years old offspring of parents who had an acute myocardial infarction before age of 45 and 42 healthy children without any family history of cardiovascular disease were investigated. A significant increase in plasma free serotonin concentration was seen in endangered children. From these results it may be supposed that vascular endothelium is dysfunctional in offspring of parents suffering from premature coronary arterial disease.

The head is a very rare primary site for Ewing's sarcoma which occurs most often in the long bones of the extremities and in the pelvis. This report describes an unusual case of Ewing's sarcoma arising from the occipital bone in a seven year old girl. The tumour compressed the venous sinuses, thus lowering the intracranial pressure resulted in temporary recovery which made the diagnosis difficult.

60 children with acute lymphoblastic leukemia were sequentially randomized at the time of diagnosis: Immunoglobulin (Endobulin, Immuno) was administered intravenously to 30 patients at a dose 100 mg/kg/week during the first 3 months, followed by 2 x 200 mg/kg/month immunoglobulin during the 4., 5., 6. months. No immunoglobulin was administered to the control patients. We studied the effect of immunoglobulin prophylaxis on the number of days with fever, number of cases with bacteriologically proved infections, length and frequency of antibiotic therapy. Our data confirm the efficacy of immunoglobulin prophylaxis during the intensive phase of leukemia therapy in children.

The erythrocyte glutathione metabolism of 11 children with acute celiac disease (CD), 11 children under gluten free diet with CD and 5 children with cow's milk allergy was compared to that of 11 children with nutritive iron deficiency and to 22 healthy children as controls. Erythrocyte glutathione (GSH) content of celiac children was elevated and the glutathione disulfide (GSSG) level was significantly decreased as compared to normal controls. Erythrocyte GSSG/GSH ratio in acute CD differed also from the one in iron deficiency. In vitro oxidative load of acetylphenylhydrazine proved the impaired glutathione stability of the erythrocytes in acute CD and cow's milk allergy. A parallel rise of methemoglobin and hemichrome level of blood cells was seen. Further on, the selenium content of the red blood cells of CD patients decreased. All alterations of the erythrocyte tended to normalize during the dietetic period. These data suggest a reduced protective capacity of erythrocytes in CD and in cow's milk allergy in childhood against oxidizing stresses.

Insulin resistance was investigated in three obese boys with acanthosis nigricans and their results were compared to those obtained in non-acanthotic obese patients. Blood glucose immune reactive serum insulin and C-peptide during oral glucose tolerance test and 125I-insulin binding investigated. Obese patients with acanthosis nigricans were more insulin resistant than simple obese controls. Insulin binding studies performed in two acanthotic patients suggested that one of them had insulin resistance type A, and the second patient had insulin resistance type B. According to the results acanthosis nigricans can serve as a marker for severe insulin resistance in obesity.