Maternal tetrahydrobiopterin deficiency: the course of two pregnancies and follow-up of two children in a mother with 6-pyruvoyl-tetrahydropterin synthase deficiency.

No reports are available about the course of pregnancies in women with tetrahydrobiopterin (BH(4)) deficiencies or the effects of treatment with BH(4), L-dopa/carbidopa and 5-hydroxytryptophan (5-OHTrp) on fetal development. We present for the first time the case of a mother with late-diagnosed mild form of 6-pyruvoyl-tetrahydropterin synthase (PTPS) deficiency, the course of her two subsequent pregnancies and clinical evaluation with follow-up of two offspring. In both pregnancies neurotransmitter precursors, as well as BH(4) dosages were increased proportionally to the mother's weight gain. To prevent maternal phenylketonuria (MPKU) syndrome, special attention was paid to increasing BH(4) dosages. Both pregnancies were complicated by threatened premature labour, by the mother's nicotinism and additionally, in the first pregnancy, by gestational diabetes mellitus and vaginitis. The first child was born in the 31st week of pregnancy with the symptoms of moderate intrauterine growth retardation (IUGR) and brain malformation in the form of right sided closed-lip schizencephaly with absence of septum pellucidum. Although the girl demonstrates mild left-sided hemiparesis, her psychological development at the age of 8 years is above average. The second child was born in the 37th week of pregnancy without brain anomalies and at the age of 5 years his psychomotor development is appropriate for the age. As the cause of brain malformations resulting in physical impairment in the first child is unknown, more data are essential to verify conclusions about the influence of the mother's BH(4) deficiency and the safety of her treatment for fetal development.

[Abnormalities in neonatal brain ultrasound examination in relation to cord blood TNF and chorioamnionitis]. / Nieprawidlowosci obrazu ultrasonograficznego mózgowia u noworodków a stezenie TNF we krwi pepowinowej i zmiany zapalne blon plodowych.

The study was undertaken to determine whether PIVH and PVL in neonates are related to cord blood TNF levels and chorioamnionitis. The study material consisted of 54 neonates--31 preterm and 23 full-term. 39 of those babies were born to mothers with clinical signs of intrauterine infection, but only in 20 cases histologic chorioamnionitis were recognised. In the whole study newborns brain ultrasound examination was taken during the first 3 days of life. Abnormal head ultrasound (PIVH--17 cases or PVL--6 cases) was diagnosed in 23 babies. That was found that brain damage, recognised during neonatal period, was mainly connected with prematurity and signs of maternal infections but not with cord blood TNF levels. We conclude that prolongation of pregnancy as well as early recognition and proper treatment of intrauterine infection can prevent PIVH and PVL in neonates.

[Hypoglycemia in newborn of the diabetic mother in relation to some indicators of perinatal hypoxia]. / Wystepowanie hipoglikemii u noworodków matek z cukrzyca a wybrane wskazniki niedotlenienia okoloporodowego.

DESIGN: To examine whether perinatal hypoxia increases the risk of occurrence of hypoglycaemia--between first and second hour of life--in newborn of the diabetic mother. MATERIAL AND METHODS: The study material consisted of 151 newborns born to 58 pregestational and 93 gestational diabetes mothers. The occurrence of hypoglycaemia was examined in accordance with some perinatal hypoxia indicators such as: 1 and 5 minutes Apgar scores, umbilical arterial blood gas analysis and cord blood erythropoietin (EPO) level. RESULTS: Newborns of the diabetic mothers in whom hypoglycaemia was recognised had lower 1 minutes Apgar scores, lower pH values, higher pCO2 values and higher EPO levels than those, in whom normoglycaemia was recognised. CONCLUSIONS: Low 1 minutes Apgar scores and occurrence of even mild perinatal hypoxia are factors increasing the risk of hypoglycaemia in the group of newborns of the diabetic mothers in the time between first and second hour of life.

[Arterial blood pressure 3-8 years after EPH gestosis in mothers and their children]. / Cisnienie tetnicze krwi w 3-8 lat po przebytej gestozie EPH u matek i urodzonych przez nie dzieci.

The analysis covered 61 women with a history of EPH gestosis and 63 children born to them as well as 30 healthy women with normal arterial blood pressure during pregnancy and their 30 children aged 3-8 years. Hypertension exceeding 145/95 mm Hg was found in 26 mothers out of 61 at the moment of examination. In the other 35 patients from the EPH gestosis group arterial blood pressure was still within the norm, but diastolic blood pressure (DBP) values were significantly higher than in the control group. The values of DBP in children from mothers with EPH gestosis were higher than the values of DBP in children from the control group. However significantly higher values were observed only in children from mothers with constantly elevated blood pressure.

[Serological conflict: relation between antibody titer, Liley's zone and the condition of the fetus and newborn infant]. / Konflikt serologiczny: zwiazek miedzy mianem przeciwcial i strefa Lileya a stanem plodu i noworodka.

On the basis of analysis of 128 cases of serological incompatibility it was found that antibodies to Rh in titres of 1/8 or less constituted a good prognostic factor, and without evidence of fetal distress in USG examination this was not an indication to treatment undertaking. If the titre is from 1/16 to 1/128 the prognosis is worse and the degree of fetal haemolytic disease risk may be estimated on the basis of USG and Liley's zone. The prognosis is very bad in case of titre exceeding 1/128 and high Liley's zone, and without effective treatment of haemolytic disease in fetal life only few fetuses survive.