Acta paediatrica Hungarica最新文献

Serum lipids and lipoproteins were investigated in the premature myocardial infarction (PMI) risk families before 45 years of age with the aid of screening for hyperlipemia and hyperlipoproteinemia (HLP): in the case of 174 persons from Csongrád County from the Departments of Internal Medicine I and II and of 42 patients (fathers) suffering from PMI and their 79 "high risk" children from Heves County. In the investigated three groups of "high risk" children the genetically determined antiatherogenic HDL-Ch level diminished in 34.8, 52.3, 40.5 per cent. Significant negative correlation was detected between the serum HDL-Ch and beta-lipoprotein; significant positive correlations were found between the HDL-Ch and the serum lipase activity; between the beta-lipoprotein and the phospholipid level; significant negative correlation was proved between the HDL-Ch and the phospholipid level in the group of PMI patients and their offsprings. The Ch/Tg, and the HDL-Ch ratios were significantly diminished in the PMI patients' group against the risk children' group, while the Ch/HDL-Ch rate was significantly elevated.

To test the hypothesis that prostaglandin (PG) E2 is a respiratory depressant in the newborn lamb, 12 chronically catheterized, unanesthetized lambs (age 2 to 6 days) were infused with progressively increasing doses of PGE2 (0.1, 0.5, 1.0 and 5.0 ug/kg/min: 30 min for each dose) into the ascending aorta. PGE2 caused significant, progressive decrease in ventilation (due to decreased tidal volume and breathing rate) heart rate, blood pressure and percent of the time spent in low voltage electrocortical activity (LVA). PGE2 also caused respiratory acidosis, hypoxemia and increased frequency and duration of apneic events (greater than 3 sec). During the infusion, there was a dose related increase in plasma concentration of PGE2. At 30 min post-infusion, all measured variables showed recovery, although arterial pH carbon dioxide tension and plasma PGE2 remained significantly different from control values and the percent time in LVA was even higher than during control. Infusion of the vehicle alone (n = 5) caused no significant changes in any of the measured variables. The results, taken in combination with previous fetal studies, indicate that PGE2 has marked inhibitory effects on breathing movements both before and after birth.

"In vitro" sensitivity of lectin (PHA, Con A)-stimulated lymphocytes to antitumor drugs (ARA-C, ADR, VM26, MTX, CP, VCR, Vepesid, ACLA) and the clinical efficiency of the complex therapy was compared in 7 patients with ALL and 2 patients with NHL. H3-thymidin incorporation of lymphocytes labelled prior to the drug exposure was used as "in vitro" method. A fairly good correlation was found between the "in vitro" test and the clinical response to the drug administered. These results suggest that this "in vitro" test is useful in choosing the drugs to be administered in case of malignancies of children.

Ultrastructural findings of biopsy materials of four gipsy first cousin infants suffering from late infantile type of ceroid lipofuscinosis (Jansky-Bielschowsky) were investigated. The diagnostic significance of the conjunctival biopsy is emphasized. The pericytes and the vascular smooth muscle cells of the arterioles proved to be the main inclusion storing cells.

Head injuries of children between 4-16 years in the first hour after the trauma have been studied. The neurological examination was completed by CT-scan. Glasgow Coma Score (GCS) as well as Glasgow Outcome Scale (GOS) were also applied and proved to be very helpful. Our findings show a significantly frequent occurrence of epidural haematomas. Their treatment in patients with GCS above 8 was in 90% successful.

A population-based aetiological study was carried out on 6 to 14 years-old severely visually handicapped children in Hungary. Of the 547 recorded cases 491 (90%) were included in the analysis. Eleven aetiological groups were separated: isolated cataracts (16.7%), congenital abnormalities of the eye (15.1%), high myopia +/- retinal detachment and other cases (13.4%), retinopathy of premature (11.0%), choroidoretinal degenerations (10.0%), syndromes (9.6%), nystagmus and/or hypermetropia (9.0%), isolated and complicated optic atrophy (6.7%), postnatal causes (4.9%), retinoblastoma (1.8%), prenatal causes (1.8%). A significantly higher rate of previous induced abortions was found in the group of retinopathy of premature. Perinatal damage syndrome and Mendelian monogenic defects are the two most common aetiological categories in the origin of severe visual handicaps in Hungary.

1320 students of two high schools were examined in Holon. Their physical and behavioral problems are summarized. These data serve to build the model of the medical profile of the adolescent in Israel, as a background to medical screening in school, which should be done at least every two years.

This study has been performed to assess the effect of methyldopa (MD) therapy in pregnancy hypertension on the neonatal adaptation. Infants born to mothers on MD for several weeks prior to delivery and presenting with excessive tremor and irritability were evaluated according to the dose of maternal MD. Pregnancy hypertension and high dose MD was associated with impaired placental perfusion, compromised function of fetoplacental unit and more frequent surgical delivery. Infants of mothers on high (1.25-2.0 g/day) or low (less than 1 g/day) MD had gestational age, head circumference, acid-base balance, Apgar score and blood pressure similar to those born to healthy control mothers. The birth weight of infants of the high MD group, however, were significantly lower than in the low-dose or control groups. MD therapy resulted in a dose-dependent increase in plasma levels of prolactin, thyrotropin and triiodthyronine indicating decreased dopaminergic inhibition of pituitary hormone release. Plasma thyroxine concentration, however, decreased significantly. Cerebrospinal fluid noradrenaline was found to be markedly depressed after maternal MD showing disturbed central nervous system monoamine metabolism. It is suggested that MD administration to mothers presenting with pregnancy hypertension interferes with cerebral monoamine metabolism of the neonate and induces alterations in some endocrine functions under dopaminergic control. The possible role of chronic fetal distress frequently associated with pregnancy hypertension should also be considered.

61 infants with critical valvular pulmonary stenosis (21 cases) or pulmonary atresia with intact ventricular septum (40 cases) were operated on between 1975-1989 in the Semmelweis University Medical School. Right ventricular volume, area, outflow tract dimension and tricuspid anulus diameter was measured by angiocardiography and echocardiography to evaluate right ventricular size, and to predict the operative outcome using these data. Our results suggested, that if the normalized right ventricular volume was less than 3 ml/m2, the normalized right ventricular area was less than 2.5 cm2/m2, and the normalized right ventricular area was less than 2.5 cm2/m2 and the normalized tricuspid anulus diameter was less than 1.2 cm2/m2/3 only a systemopulmonary shunt procedure is needed. In all other cases pulmonary valvotomy is necessary to decompress the right ventric and to help increase the right ventricular size. Where a different part of the right ventricle is hypoplastic a systemopulmonary shunt procedure is needed too. In the follow up period the right ventricular dimension and tricuspid valve diameter was measured by echocardiography. This noninvasive assessment can predict the preoperative diagnosis, postoperative outcome and demonstrates an adequate growth of the right ventricle after pulmonary valvotomy.

The cranio-carpo-tarsal or "whistling face" syndrome was first described by Freeman and Sheldon in 1938. More than 60 cases with great variability of expression are known till now and autosomal dominant as well as recessive inheritance and sporadic cases suggest a genetic heterogeneity. We review 60 well-documented cases of the literature and present a patient with a severe form, who died of bronchopneumonia at the age of 9 months. The facial stigmata of his mother and the ulnar deviations of his maternal grandfather support the autosomal inheritance of the syndrome.