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Biomedical subjects

H Devlieger

Publications and source records attributed to H Devlieger.

At least 127 records · Page 7Linked to original sources
PubMed1988

The syndrome of diaphragmatic hernia, abnormal face and distal limb anomalies (Fryns syndrome): report of two sibs with further delineation of this multiple congenital anomaly (MCA) syndrome.

We describe 2 sibs with the syndrome of diaphragmatic hernia, abnormal face, and distal limb anomalies. Both infants died shortly after birth with severe respiratory distress. Postmortem examination showed gross internal anomalies: Dandy-Walker malformation, ventricular septal defect, and renal cystic dysplasia. This combination of anomalies, also termed the Fryns syndrome, appears to be a distinct MCA syndrome with variable expression and probable autosomal recessive inheritance. Prenatal ultrasonographic diagnosis was successful in both patients.

Abnormalities, Multiple↗
PubMed1988

Feeding, behavioural state and cardiorespiratory control.

The aim of the present study was to examine whether immaturity of cardiorespiratory control corresponds to a less mature behavioural state pattern and/or to less efficient feeding behaviour. Fifty-four infants were observed and data polygraphically recorded for 6 hours; a feeding session was included. It was found that infants with immature cardiorespiratory control spent more time in REM-sleep, less time in the active awake state, and were more likely to be inefficient feeders. In addition, 100 infants were observed for risk signs of sudden infant death syndrome and their parents were asked to answer a questionnaire on the sleeping and feeding behaviour of their infants. The majority of the infants with immature cardiorespiratory control were described as bad feeders but good sleepers. We conclude that gathering information about sleeping and feeding behaviour is useful when screening for immaturity of cardiorespiratory control.

Apnea↗
PubMed1988

[Tracheobronchial stenosis of the premature infant. Treatment by balloon dilatation].

The case of a 30th week preterm newborn infant, ventilated because of hyaline membrane disease and presenting with localized stenoses of the carina involving both main-stem bronchi is described. The diagnosis was made on bronchoscopy and bronchography after a period of recurrent atelectases and lobar emphysema. Due to severe broncho-pulmonary dysplasia, surgical reconstruction was impossible. A balloon dilatation was successfully performed while the infant was still ventilated. He died however at the age of 180 days.

Bronchial Diseases↗
PubMed1988

[Paroxysmal cardiorespiratory attacks in infants].

In a group of 46 fullterm infants, with a history of paroxysmal cardiorespiratory attacks, the anamnestic characteristics of the attacks were found to be heterogeneous. A 48 hours polygraphic recording was carried out in order to detect signs of immature cardiorespiratory control. Thirty seven percent of these infants were classified as having an immature cardiorespiratory control defined as abnormally long apneic episodes of 15 seconds or more, bradycardia or a high percentage (more than 10% of the time) of periodic breathing. In a group of normal control infants, those signs of immature cardiorespiratory control were significantly less frequently observed (8%). From this it is concluded that those symptoms might be considered as indicators of an increased risk for sudden infant death.

Apnea↗
PubMed1987

Congenital eventration of the diaphragm: an unusual cause of intractable neonatal respiratory distress with variable etiology.

We describe two infants dying neonatally of respiratory failure despite all attempts at resuscitation. The most striking finding at autopsy was eventration and reduced muscle content of the diaphragm. Microscopic examination of the skeletal muscles, in combination with retrospective evaluation of the family history, disclosed severe X-linked centronuclear myopathy in the first patient and congenital myotonic dystrophy in the second. These disorders are probably more frequent than reported before. Their identification is important, not only for genetic counseling of the involved families but also for providing the neonatologist a sufficient explanation for the failure of resuscitation.

Diaphragmatic Eventration↗
PubMed1987

X-linked centronuclear myopathy as a cause of floppy baby.

Two families with X-linked recessive centro-nuclear myopathy (XLR-CNM) are described. Evidence is accumulating that XLR-CNM forms a distinct entity, in contrast to the commoner later-onset forms. Family history often provides a clue to the diagnosis. Pregnancy is very often complicated by hydramnios and reduced fetal movements. We describe two families with five affected male babies who all died. An isolated case with severe fetal brady-arrhythmias in combination with acute increase in polyhydramnios is mentioned. The neonatal mortality in all recorded cases is 80 percent, with respiratory insufficiency as the cause of death. An attempt is made to establish early prenatal diagnosis and the possibility of carrier detection is briefly discussed.

Adult↗
PubMed1987

Electrolyte composition of the amniotic fluid in Bartter syndrome.

In three patients with neonatal Bartter syndrome associated with polyhydramnios, analysis of the amniotic fluid showed normal sodium, normal-to-low potassium, but high chloride concentrations. This finding clearly suggests a renal chloride reabsorption defect as the primary cause of the neonatal form of Bartter syndrome. It is suggested that whenever polyhydramnios occurs, the electrolyte composition of the amniotic fluid should first be analysed in order to establish the diagnosis of Bartter syndrome.

Adult↗
PubMed1986

Nutritive and non-nutritive sucking in preterm infants.

Nutritive and non-nutritive sucking was studied in 9 preterm infants with postmenstrual ages ranging from 28 to 33 weeks and postnatal ages ranging from 0 to 8 weeks. During nutritive sucking, sucking bursts were longer than sucking pauses. During non-nutritive sucking the opposite was seen. The sucking rate was lower during nutritive sucking. During nutritive sucking the respiratory rate was higher during the pauses than during the bursts. During non-nutritive sucking the respiratory rate was higher during sucking. It is concluded that non-nutritive sucking cannot serve as a model for studying feeding mechanisms in the preterm infant.

Electromyography↗
PubMed1985

Variability of outcome in Joubert syndrome.

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.

Cerebellum↗
PubMed1984

Serum thyroglobulin levels in preterm neonates.

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.

Gestational Age↗
PubMed1984

The thyroid-system function in preterm infants of postmenstrual ages of 31 weeks or less: evidence for a "transient lazy thyroid system".

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)

Age Factors↗
PubMed1984

Neonatal evaluation by nurses in a neonatal special care unit.

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.

Behavior↗
PubMed1984

The behavioural state in human perinatal life.

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.

Aging↗
PubMed1983

Thrombosis of the right umbilical artery, presumably related to the shortness of the umbilical cord: an unusual cause of fetal distress.

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.

Adult↗
PubMed1983

Diagram for easy volume setting of an infant ventilator.

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.

Bronchopulmonary Dysplasia↗
PubMed1983

[Neonatal hyperammonemia].

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.

Amino Acid Metabolism, Inborn Errors↗