Acta paediatrica Hungarica最新文献

Of 1038 index patients with multiple congenital abnormalities, 34 were twins. This 3.3 per cent is higher than the Hungarian birth rate of about 2.1 per cent. However, after the exclusion of cases with congenital abnormality association of low birth weight newborn infants and with genital anomalies of the male, the twin birth rate was 1.8 per cent. Thus, the unidentified multiple congenital abnormalities have no common cause with twinning.

Ninety-three cases born from women who attempted suicide by taking large doses of chemicals and eight cases who had mothers affected by a chemical poisoning as accident during pregnancy were studied. Data of birth weight and gestational time, congenital anomalies and postnatal disorders were obtained in cases and matched controls (sociological twins). Test examinations were performed in both cases--controls and their mothers in the home of families. The mothers of index children had a lower socioeconomic status and more were unmarried than the Hungarian pregnant population at large. The behaviour of index and control mothers showed some obvious differences. The bivariate analyses indicated some possible teratogenic effect of maternal poisoning, but it was not confirmed by multivariate analysis except lower birth weight. Thus differences between cases and control were explained mainly by the maternal behavioural factors.

206 non-selected children who had wheezy bronchitis before two years of age were observed in the first follow-up (1985) about 9 years after their clinical wheezy episode. Among them 31 patients (15%) showed bronchial hyperreactivity (B.H.) after acetylcholine challenge and 9 children became asthmatic. In the second follow-up (1988) 28 children who had B.H. and randomly 17 children who did not have B.H. participated. The prognosis of wheezy bronchitis is good, there was no new asthmatic child at this time. The B.H. was found to diminish only in 7 cases. During the last 3 years wheezy episodes developed only in 5 asthmatic patients. The skin prick test (SPT) positivity did not change (26% versus 29%). The familial atopy in the group B.H. is higher (43%) than in the group non B.H. (23%) but the difference is not significant (X2 = 1.72). No significant correlation was found between the familial atopy and SPT positivity. The familial smoking is higher in the group B.H. (78% versus 64%) but no significant influence on the B.H. (X2 = 1.03) could be detected.

The author evaluates the usefulness of clinical features and some supplementary investigations in diagnosis of tuberous sclerosis in children. 52 children ranging in age from 1 month to 14 years took part in the study. Usually depigmented naevi were the first sign of tuberous sclerosis and were seen in 98% of affected children. Epileptic seizures occurred in 96% of patients, mostly during the first year of life. Axial computed tomography of brain seems to be very helpful in diagnosis of tuberous sclerosis in every age. Multiple periventricular calcifications were found in 98% of children. Two-dimensional echocardiography was especially useful in infants and children below 2 years of age. Retinal hamartomas were found in 25% of children with tuberous sclerosis. In some cases these changes were revealed during the first year of age. Abnormal EEGs were found in 90% of recordings. The EEGs tended to improve with increasing age. In 25% of children one of parents was affected by tuberous sclerosis. The careful skin examination of both parents can be especially helpful in children with doubtful diagnosis of tuberous sclerosis.

The study was undertaken to assess the influence of thyroxine given to improve respiratory adaptation in asphyxiated neonates on the recovery of compromised renal functions. Two groups of infants with perinatal asphyxia were selected for the study. Group I consisted of 8 infants treated conventionally, while Group II included 7 infants who in addition to standard therapy were administered 50 micrograms thyroxine at admission and repeated 24 hours later. Their respective mean gestational ages were 38.7 weeks (range: 34-42 weeks) and 37.4 weeks (range: 34-41 weeks). The studies were performed on days 1, 7 and 14 and the results compared to those obtained in 13 healthy neonates with the gestational age of 39.2 weeks (range: 38-41 weeks) (Group III). Asphyxiated neonates had significantly higher plasma uric acid, xanthine, hypoxanthine and creatinine levels (p < 0.05), while their GFR proved to be markedly reduced (p < 0.01) when compared to the values of healthy controls. Moreover, there was a significant elevation of urinary excretion of NAGA (p < 0.001), urine osmolality (p < 0.05), PENa, FECa, RFI (p < 0.05) in infants presenting with perinatal asphyxia. Renal tubular responsiveness to aldosterone measured as TTKG was also found to be depressed (p < 0.025). In response to thyroxine therapy renal functional recovery appeared to be accelerated as indicated by the lower plasma creatinine level, lower rate of fractional electrolyte and urinary NAGA excretion and improved reactivity to aldosterone on days 7 and/or 14 as compared to those obtained in neonates presenting with asphyxia but without thyroxine therapy. The results seem to suggest that thyroid hormones may have an important role in the recovery of renal functions in newborn infants suffering from perinatal asphyxia.

This study was performed as a part of a longer research programme on urinary tract smooth muscle layer in children. All the children whose samples were investigated underwent surgery for urinary tract malformations. Specimens were taken from different segments of upper urinary tract during surgical intervention. Specimens were investigated by either in vitro motility tests or electron microscopy or both of them. Basic patterns of tissue strips were recorded after incubation of varying duration and then tested by administering neurotransmitter agents like noradrenaline and acetylcholine-bromide. Microstructure of samples were examined electron microscopically. Investigations were performed in order to find correlation between microarchitecture and motility patterns of urinary muscle wall. Factors influencing urinary muscle motility, characteristic features of impaired musculature and its possible regeneration are discussed too. Microhistological deteriorations inhibit spontaneous smooth muscle motility but muscle contractility proved by administering noradrenaline and acetylcholine-bromide remained in some extent. Taking into consideration that smooth muscle is able to regenerate and rebuild close contacts pediatric surgeon and urologist should spare kidney parenchyma as far as it is possible.

This paper presents two brothers with incomplete expression of the Smith-Lemli-Opitz syndrome. A definite diagnosis, made after the birth of the second child, could only be reached when the clinical features of both patients were combined.

The ALL-BFM 88 RF greater than 1.7 treatment of high malignancy ALL patients in remission was monitored at the 4th week of Protocol I. The qualitative and quantitative processing of parameters indicating cellular transformations by light and electronmicroscopy was performed in lymphocytes separated from peripheral blood drawn at scheduled times of therapy. The immunophenotype of sample lymphocytes was determined by indirect immunofluorescent techniques, by listing the percentual level of CD2, CD3, CD4, CD8, CD19 surface antigens. The incidence of infections, confirmed clinically and bacteriologically, was recorded. The parameters of the patients were compared to those of healthy children. In the group of patients with high malignancy in the intensive period of ALL-BFM 88 RF greater than 1.7 therapy the incidence of cells rich in organelles, dense granules and vacuoles was significantly (p less than 0.05) higher compared to the control cells, poor in structures. During the weeks of induction and after the consolidating combination with VM-26+ARA-C the expression of surface antigens of the lymphocytes was reduced. That means that up to the introduction of maintenance treatment the presence of surface antigens remained below the range of normal deviation. These data obtained during monitoring revealed that the morphological and functional integrity of cells was damaged in patients with high malignancy ALL during intensive therapy, though only temporarily.

Authors analysed in an infant with primary intestinal lymphangiectasia the number of intraepithelial lymphocytes and the distribution of T lymphocyte subpopulations in the jejunal mucosa with immunohistochemical methods. It was established that the number of intraepithelial lymphocytes and of the cells belonging to the various T lymphocytes markedly decreased in the patient compared to controls, however the decrease of the CD4 cells was less than that of CD8 cells, thus the CD4/CD8 ratio increased. Authors conclude that the increase of CD4/CD8 ratio in the jejunal mucosa may explain the absence of frequent gut infections in intestinal lymphangiectasia.