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 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.
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.
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.
Authors report on a Bicarnesine replacement therapy in an infant girl patient suffering from carnitine deficient myopathy diagnosed at 1 year of age. The hypotonic patient's motoric functions improved and she became able to walk as a result of therapy applied, but the pathological process generalized to encephalomyopathy. Free and esterified carnitine were determined from the serum and muscle biopsymaterial. After the Bicarnesine-supplementation the serum carnitine fractions elevated.
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.
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.
Two groups of mothers with infants between sixty and eighty-nine days of age, from the same Health Area of the city of Havana were studied. The first group's age was nineteen years or less and members of the second one were twenty to twenty-five years old. The sociocultural level of the older group was significantly more favourable. The proportion of mothers exclusively breast-feeding was lower among the teenagers, and the frequency of complete weaning by the end of the third month was higher in their group. Duration of breast-feeding, either exclusive or combined with bottle-feeding was also shorter in the group of adolescent mothers, and frequency of diarrheal episodes was higher among their infants; body weight for age and body weight for length showed a shift towards lower percentile channels in these infants. Such differences were evident despite of that both groups of mothers had the same opportunity to receive information about the advantages of breast-feeding and there were no differences in the frequency and quality of prenatal care.
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.
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.
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.
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 data of first 1000 non-malformed, mature (greater than or equal to 2500 g) singletons of participants in the Hungarian Family Planning Programme were evaluated. The mean maternal weight gain during pregnancy was 13 kg which was modified by the body weight of women. Maternal weight gain exceeded 13 kg in 54% of pregnant women. There was a positive correlation between maternal weight gain and birth weight which was calculated as 26.6 g/kg.
To assess the relationship between severe hypoglycaemias and autonomic dysfunction, five cardiovascular tests (resting heart rate, hyperventilatory arrhythmia, standing/lying heart rate ratio, orthostatic decrease in blood pressure, and increase in blood pressure during sustained handgrip) were performed in a 1-yr prospective study of 34 insulin-dependent diabetic children treated with intensified conventional insulin therapy (ICIT). There were twelve severe episodes in 7 diabetic children, and the remaining 27 patients had no severe hypoglycaemia. The hypoglycaemic group had a longer duration of diabetes than the nonhypoglycaemic group (5.4 SD 2.5 years vs. 2.8 SD 2.2 years, p less than 0.02). The hyperventilatory arrhythmia in the hypoglycaemic group in comparison with the nonhypoglycaemic group was significantly decreased (before ICIT: 16.1 SD 3/min vs. 24.4 SD 5/min, p less than 0.01; 1 yr thereafter: 17.3 SD 3/min vs. 26.0 SD 5/min, p less than 0.01). The hypoglycaemic group showed a pronounced orthostatic decrease in blood pressure compared to the nonhypoglycaemic group (before ICIT: 13.2 SD 4 mmHg vs. 6.0 SD 4 mmHg, p less than 0.01; 1 yr thereafter: 12.3 SD 4 mmHg vs. 5.6 SD 4 mmHg, p less than 0.01). Three or more abnormal cardiovascular test results were found in patients of the hypoglycaemic group who showed abnormal hyperventilatory arrhythmia and abnormal orthostatic decrease in blood pressure simultaneously, whereas such a coexistence was not found in the nonhypoglycaemic group. These observations may support the view that diabetic children and adolescents with autonomic dysfunction are susceptible to severe hypoglycaemia during ICIT.
Applying proton-induced X-ray emission authors investigated the hair trace element contents in 10 children with acute celiac disease after 3 to 6 and 12 months long gluten-free diet; in 9 children with cystic fibrosis and in a control group (6 children) of the same age. There was no difference in Cu, Fe, Ca, Cl values between the examined groups. The Zn contents of the hair are significantly low in the group with acute celiac disease after a short-term diet and also in the group with cystic fibrosis, the data approach the normal range only after a year's diet. The significant rise of hair potassium contents is well indicated in patients with acute celiac disease and this rise may be due to the destruction of cell cuticles. In case of cystic fibrosis there is no significant rise of hair potassium value.
A retrospective analysis of the HLA-DR antigens was performed at the DR beta DNA locus by the means of restriction fragment length polymorphism (RFLP) in the case of a two-times kidney transplanted paediatric patient and in 16 adult kidney donor recipient pairs in order to prove the importance of DNA molecular analysis in those cases where the serological identification is poor. The child and her grafts (first from her mother, the second from a cadaver donor) carried the DRw6 antigen which serologically can very poorly be defined. According to DR serotyping before transplantation both the child and the cadaver kidney proved to be DR5, 6, while the DNA analysis revealed mismatches; the child possessed the two subtypes: 13b1 and 14a of the DRw6 antigen only and none of the DR5, the cadaver kidney proved to belong to the DR4 antigen group instead of DR5, and furthermore to a different subtype of the DRw6 (13a3) than the recipient. The DNA analysis of other 16 adult donor-recipient pairs also underlined the importance of the DR beta RFLP analysis in cases where the transplantation antigens could be poorly defined.
