Biomedical subjects
Publications and source records attributed to H Devlieger.
Two children with Joubert syndrome are reported. Patient one is the first case with Joubert syndrome where CT-findings are confirmed by autopsy. Until now only three cases with necropsy findings were reported. Patient two shows a remarkable clinical outcome not previously mentioned.
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Serum thyroglobulin (Tg) levels were determined in preterm neonates. Very high values were found at birth. A significant negative correlation was observed between Tg levels and gestational age. A longitudinal study indicates that a very sharp decrease in Tg levels occurred after birth. Three weeks after birth, the values were close to the values of full-term neonates. These high hTg values could result either from an increased turnover of hTg or from a lower clearance rate of this iodoprotein.
A prospective study was conducted in order to evaluate thyroid function in 20 healthy and 18 sick preterm infants with postmenstrual ages of 31 weeks or less. The clinical condition of both groups was compared using a "Neonatal Special Care Evolution Score". The effect of thyroid hormone treatment, given from D10 on to the sick infants, was also studied. TSH, thyroid hormone levels (TG, T4, T3, rT3, FT4 and FT3) and TBG were measured by radioimmunoassays at D0, D10, D20, D30 and D40. Healthy preterm infants on D0 have a median TSH level of 22 microU/ml and a high TG level of 200 ng/ml; thereafter, median serum levels decrease to 6 microU/ml and 35 ng/ml respectively. During the same period, median serum T4 is maintained at a low level of about 6-8 micrograms/dl, median serum T3 gradually increases from 80 ng/dl on D0 to 150 ng/dl on D40, and median serum rT3 decreases beyond D10 from a plateau of 200 ng/dl to about 100 ng/dl. In the sick preterm infants before treatment, serum TSH is as in the control group but serum T4, T3 and rT3 on D10 are well below the control values (P = 0.005). In all conditions, there is a significant correlation between serum T4 and FT4, and between serum T3 and FT3. Thyroxine, given to the sick preterm infants from D10 on, brings median serum T4 values closely to the ones of the control group whereas serum levels of TSH and TG are unaffected and similar to those of the healthy preterm infants. Furthermore, thyroxine brings serum rT3 within the range of the control group but leaves median serum T3 at a low level of about 50 ng/dl. On T3 treatment, serum T3 normalizes but rT3 and particularly T4 tend to decline further. In the conditions of this study a significant difference in TBG level is not proven. Although an untreated sick group was not enrolled in the study, thyroid hormone treatment brought the "Neonatal Special Care Evolution Score" of the treated sick infants closer to that of the healthy preterm infants. In the sick preterm infant with failure to thrive on D10, there is an impaired thyroid discharge of T4 in spite of serum TSH values not different from those of the control group.(ABSTRACT TRUNCATED AT 400 WORDS)
The value of a nursing questionnaire in the evaluation of preterm infants during their stay in the neonatal special care unit was studied. The questions were related to feeding, crying, sleeping, motoric process, tremor and stress, state control, position, sensory capabilities and parental attitudes. In general, questions were answered in 95%, but the questions related to sensory capabilities were only answered in 30%. Evaluation by the nurses of feeding, crying, motoric process, tremor and parental attitudes correlated well with observational data and with the results of a neurological examination. It was concluded that evaluation by the nurses using a questionnaire can be of value in neonatal units, especially when detailed observations or a systematic neurological examination are not feasible.
Behavioural states are easy to recognise in healthy neonates with a gestational age of 36 weeks or more. In younger neonates stable behavioural states, as seen at full-term, are not present, but activity cycles exist. In utero stable behavioural states can also be recognised from 36 to 38 weeks onwards. The existence of awake states in utero remains controversial. A close relationship exists between neural mechanisms regulating the behavioural state cycling and those modulating breathing. Our understanding of these is still very limited. The absence of behavioural states or of activity cycles is a very sensitive but non-specific sign of nervous system dysfunction in human perinatal life.
This case report concerns a late pregnancy complication, clinically apparent as severe variable decelerations in the first stage of labor. Emergency cesarean section delivered a mildly asphyxiated full-term newborn infant. Examination of the umbilical cord revealed a thrombus of the right umbilical artery, near the fetal side, confirmed by histological examination. The total length of the umbilical cord, only 30 cm, was below the limit necessary for uncomplicated delivery of the fetus near term. Transient stretching during fetal descent is thought to be responsible for constriction of the umbilical arteries, blood flow sludging and thrombosis. Although only a few cases have been reported, thrombosis of the umbilical vessels has to be considered whenever the fetal heart rate pattern shows unexplained variable decelerations.
A pressure-volume diagram with isocompliance lines allows quick, accurate volume setting of the piston-driven infant ventilator Bourns LS. A line parallel with the machine compliance line and an intercept on the ordinate equal to the tidal volume, serves as a guideline for initial volume setting and subsequent adaptation according to the patient's lung compliance. This diagram is particularly useful when the lung compliance is low; changes consistently during controlled ventilation.
Experience with severe neonatal hyperammonaemia in the Newborn Intensive Care Unit of the Leuven University Hospital is reported and compared with the literature data. Among eleven patients seven presented with the transient neonatal hyperammonaemia-syndrome and four with a urea cycle defect. Prompt recognition of the transient neonatal hyperammonaemia syndrome is important because its prognosis is much better than that of the primary urea cycle defects presenting in the neonatal period.
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In 100 bottle-fed preterm infants feeding efficiency was studied by quantifying the volume of milk intake per minute and the number of teat insertions per 10 ml of milk intake. These variables were related to gestational age and to number of weeks of feeding experience. Feeding efficiency was greater in infants above 34 weeks gestational age than in those below this age. There was a significant correlation between feeding efficiency and the duration of feeding experience at most gestational ages between 32 and 37 weeks. A characteristic adducted and flexed arm posture was observed during feeding: it changed along with feeding experience. A neonatal feeding score was devised that allowed the quantification of the early oral feeding behavior. The feeding score correlated well with some aspects of perinatal assessment, with some aspects of the neonatal neurological evaluation and with developmental assessment at 7 months of age. These findings are a stimulus to continue our study into the relationships between feeding behaviour and other aspects of early development, especially of neurological development.
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A girl presented with an important growth retardation, hepatomegaly, fasting hypoglycemia, lactic acidosis, increased serum cholesterol, triglycerides and uric acid, and increased liver glycogen (7.5%). There was no rise in blood glucose after IV galactose or fructose, but glucagon gave a delayed response. Type Ib glycogen storage disease was suggested by the low normal activity of glucose-6-phosphatase (G-6-Pase) which reached 1.8 units/g (normal, 2 to 10 units/g) and the normal activity of other glycogenolytic enzymes, measured in homogenates prepared in H2O (mean +/- S.E. in control subjects: 59% +/- 7; in type Ia GSD: 92% +/- 3). The activity of G-6-Pase measured as described above increased to 3.8 units/g of liver 1 year after PCS and 7.85 units/g of liver after 3 years. At that time, a simultaneous assay of the enzyme in a fresh, previously not frozen liver biopsy, homogenized in 0.25 M sucrose, revealed only about 29% of the activity of the same sample prepared in H2O (mean +/- S.E. in three controls: 95.8% +/- 8.9.
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A mother, treated with indomethacin because of premature labour, delivered a pair of monozygous twins at a gestational age of 33 weeks. Because of foetal transfusion syndrome, the first twin was polycythaemic and the second anaemic. The latter was also hydropic, suffered from anuria requiring peritoneal dialysis, and showed signs of severe pulmonary hypertension, probably as a consequence of intra-uterine constriction of the ductus arteriosus. The possible interaction between foetal transfusion syndrome, anuria, premature constriction of the ductus arteriosus and the prenatal administration of indomethacin is discussed. In general, indomethacin should cautiously be used as a tocolytic agent.