Acta paediatrica Hungarica最新文献

Authors report on long term follow up of 12 patients operated with critical valvular aortic stenosis. They could perform control echocardiography in 11 patients 4-83 (mean 29) months after valvulotomy. The size and function of the left ventricle was found to be satisfactory, with elevated ejection fraction. The cause of the significant pressure gradient between the left ventricle and the aorta is discussed emphasizing the importance of echocardiography in determining the optimal time for valve replacement or homograft implantation.

In the course of the follow-up of 206 previously obstructive bronchitis children, the effect of parental smoking upon the occurrence of respiratory diseases, the yearly frequency of wheezing episodes and the age until the obstructive episodes used to return have been investigated. Familial and maternal smoking was more frequent in this group compared to the control group. In spite of this, however, no correlation could be detected between familial smoking and frequency of respiratory diseases, as well as the above mentioned characteristics of obstructive bronchitis. The familial smoking did not seem to influence the bronchial hyperreactivity challenged with acetylcholine, although the prevalence was higher.

Burkitt's lymphoma of a ten-year-old boy with specific 8;14 and variant 2;8 translocations is reported. The post mortem diagnosis of Burkitt's lymphoma was based on histological picture and the cytogenetic findings of the tumor biopsy. The child died four days after clinical admission. Because of the rapid progression of the disease immunological and serological investigations could not be performed. Therefore several questions remained unclarified. It is supposed that in the patient's B-lymphocytes multiple transformation events occurred leading to the development of the polyclonal lymphoma, similarly to that described in transplant-associated lymphoproliferations.

The authors report on the convulsive activity provoking effect of Promazine as shown on EEG during sleep. It has successfully been used in infants and children who do not tolerate sleep deprivation. Promazine had smaller effect on the EEG-pattern than other related compounds or barbiturates. The greatest efficacy could be reached in generalised epilepsy, but the method was also helpful in other epilepsies. It is emphasized that in cases of acute neurological symptoms of not epileptical origin the compound is helpful to exclude epilepsy since Promazine induced sleep was never associated with convulsive activity on EEG in these cases.

A number of published papers have dealt with the comparison of birth prevalences of congenital cardiovascular malformation (CCVMs). The feasibility of meaningful intercountry comparison was explored during the visiting fellowships to Dr Andrew Czeizel of Charlotte Ferencz and Dr Francine Lys. Data from three Hungarian studies, one USA and one Belgian study are presented here for selected CCVMs. Differential perinatal and diagnostic circumstances which lead to possible causes of bias involve four main domains: study population, ascertainment of cases, categorization of CCVMs and diagnostic definitions. Unique features are highlighted as a descriptive framework which will promote the comparability of epidemiologic data from various regions.

Peripheral blood lymphocyte subset levels were analyzed in 28 patients with active, polyarticular, juvenile chronic arthritis and in 12 healthy control children. The patients with a similar treatment were divided into two groups, at the beginning of disease and after five years' course. Ten children with juvenile chronic arthritis were treated by glucocorticosteroid. After five years the percentage of active T cells, T suppressor cells and active/total T cells ratio were significantly elevated and there was a decrease in the T4/T8 ratio. Glucocorticosteroids increased the proportion of active T cells but did not change the percentage of other lymphocyte subsets. It is concluded that during the course of disease a drug-induced or spontaneous process appears which corrects the initial immunological disturbance.

Six-hour cultures of unstimulated peripheral blood cells from patients with various types of childhood leukemias were examined for chromosome karyotypes. It was found that this method was suitable for the detection of characteristic chromosomal abnormalities in two cases of acute nonlymphoblastic leukemias (ANLL; FAB types M3 and M6) and in a case of chronic myelogenous leukemia (CML), but not in acute lymphoblastic leukemias (ALL). The results suggest the usefulness of this simple method (possibly in combination with the thymidine boost technique of Yunis) in the cytogenetic diagnosis of some types of leukemias.

Twenty out of 303 ventilated low birth weight infants, hospitalized in the Beilinson Medical Center's Intensive Care Unit, during the years 1984-1986, developed pulmonary interstitial emphysema. Eighteen infants had very low birth weight (less than 1500 grams) and 17 were less than 30 weeks' gestation. The mean birth weight was 987 +/- 311 and mean gestational age 27.7 +/- 2.5. Eight infants survived the pulmonary disease. There were no significant differences in the neonatal parameters between infants who died or survived. However, the survivors had a significantly lower maximal peak inspiratory pressure and FiO2 on the first day of ventilation. The incidence of pneumothorax and asphyxia was the same in both groups.

A total of 29 preterm infants maintained on mixed enteral nutrition (50% pooled human milk, 50% formula daily) were studied over a 15 days period. 16 of them received L-carnitine supplemented formula during the first seven consecutive days (600 nmol/ml, as added supplement), 13 infants served as controls. In response to enhanced dietary intake, the plasma levels and urinary excretion rates of carnitines were increased by the 7th day of study. The plasma carnitines then returned to the initial values, whilst the urinary excretion remained elevated at the 14th day of study. The elevated daily urinary excretion of carnitines was accompanied by increased clearance and decreased relative reabsorption rates in the supplemented group. In the control group the plasma carnitine levels remained unchanged throughout the observations, while the daily excretion of free carnitine decreased by the end of the study. In the supplemented group statistically significant decrease was found in the daily excreted ammonia and urea with a decrease of plasma alanine and glutamine levels by the 7th day of study. The plasma levels of beta-hydroxybutyrate, glucose and creatinine remained unchanged in both groups.