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Biomedical subjects

H Devlieger

Publications and source records attributed to H Devlieger.

At least 37 records · Page 2Linked to original sources
PubMed1997

Use of external drainage for posthemorrhagic hydrocephalus in very low birth weight premature infants.

To treat progressive posthemorrhagic hydrocephalus we used early external ventricular drainage (EVD) in 14 premature infants. We think it is important that the catheters in these critically ill infants be inserted in the neonatal intensive care unit, allowing us to keep the infants in an extremely stable environment. Only after prolonged external ventricular drainage (on average 38.4 days) is a ventriculoperitoneal shunt considered, preferably when the child has reached a body weight of 2000 g. There were no infections or other severe drainage-related problems. We report mean daily EVD volumes (which are related to body weight) and EVD duration. The 14 patients included 9 who required permanent shunting. Comparing the mortality, morbidity and follow-up data to at least 3 months of age in this group with similar data for an earlier cohort treated with lumbar punctures and late permanent shunting, we demonstrate the safety of the policy we have recently adopted.

Cerebral Hemorrhage↗
PubMed1997

Management with a staged approach of the premature hydropic fetus due to complete congenital heart block.

UNLABELLED: The management of the preterm fetus with hydrops due to complete congenital heart block is difficult. The outcome is frequently associated with significant morbidity and mortality. Two fetuses presented at the post menstrual age of 29 and 30 weeks respectively with severe hydrops due to complete heart block. The following staged approach was adopted: (1) enhance fetal lung maturation with maternal corticosteroids and thyroid releasing hormone for 48 h; (2) elective Caesarean section; (3) classical neonatal management consisting of intubation and ventilation, drainage of all cavities with effusions; (4) increase neonatal heart rate by administration of i.v. isoprenaline, by bipolar trans-oesophageal pacing or epicutaneo-oesophageal pacing; (5) after the regression of the hydrops, start epicardial pacing after implantation of 2 or 3 temporary epicardial 3/0 pacemaker; (6) implantation of a permanent abdominal pacing system with steroid epicardial tip once the threshold exceeds 20 mA or when the baby weighs more than 1500 g. In these patients a permanent pacing system was implanted at the ages of 8 weeks (2045 g) and 4 weeks (1560 g) respectively. No major complications occurred; the cardiac outcome with 37 and 34 months of follow up is excellent. CONCLUSION: This proposed staged approach with temporary epicardial leads can improve the outcome of hydropic fetuses due to complete congenital AV block.

Adult↗
PubMed1997

The effect of behavioural states on cerebral oxygenation during endotracheal suctioning of preterm babies.

Near infrared spectroscopy (NIRS) was used to investigate the effect of behavioural states on changes of oxygenated (O2Hb), deoxygenated haemoglobin (HHb) and total haemoglobin (tHb), during endotracheal suctioning. In an open prospective design, NIRS measurements have been done during 20 suctioning episodes in 13 preterm neonates. Heart rate, arterial oxygen saturation, and carbon dioxide tension were monitored continuously. Behavioural state (BS) observations were made and documented as well. The statistical analysis showed that in patients who were active, with crying periods during suctioning (behavioural states 4-5), changes of oxygenated (p < 0.005) and deoxygenated haemoglobin (p < 0.05), as well as of arterial oxygen saturation (p < 0.05) and heart rate (p < 0.05) were significantly greater than in patients who were quiet with predominant behavioural state 1, 2 and 3. These results underline the influence of behavioural states on the physiological answers to endotracheal suctioning. NIRS proved to be a valuable tool to evaluate possible harmful effects of different suctioning techniques.

Cerebrovascular Circulation↗
PubMed1997

Submicroscopic deletion in chromosome 22q11 in trizygous triplet siblings and their father. Clinical variability of 22q11 deletion.

A submicroscopic deletion of chromosome 22q11 was demonstrated in three triplets and in their father. Two children had the typical DiGeorge sequence with at least three of the four cardinal features: conotruncal heart disease, hypoplastic thymus and typical facial features. Hypoparathyroidism was present in one of them. The third child had features of both DiGeorge and velo-cardio-facial syndrome (VCFS). The father presented with features compatible with VCFS. This observation further illustrates the wide variability in expression of a submicroscopic deletion of 22q11, even within one family.

Abnormalities, Multiple↗
PubMed1997

Use of methohexital for elective intubation in neonates.

The effectiveness and safety of a short acting barbiturate, methohexital, was assessed for its use at the time of elective intubation in 18 newborn infants with severe respiratory or cardiac conditions. Evaluation included the speed of action and the degree of relaxation, sedation, and sleep in the first five minutes after administration. All newborn infants were intubated in a fully relaxed and somnolent state. In most infants recovery was completed within five minutes. A slight to moderate oxygen saturation drop was observed during the period of intubation, especially in patients with cyanotic heart disease. The side effects of the drug were twitching and a slight drop in blood pressure. In conclusion, methohexital seems to be a useful drug for short term anaesthesia in neonates, during which, short procedures like elective intubation can be safely performed.

Anesthesia Recovery Period↗
PubMed1997

L-thyroxine treatment of preterm newborns: clinical and endocrine effects.

Preterm newborns have low serum thyroxine (T4) levels compared with late-gestational fetuses. Low thyroid hormone levels are associated with increased severity of neonatal illness and neurodevelopmental dysfunction. We assessed the endocrine and clinical effects of increasing serum T4 levels in preterm newborns with a gestational age <31 wk. Forty newborns were randomized in a double blind protocol: 20 infants received a daily dose of 20 microg/kg L-T4 for 2 wk, whereas 20 control infants received saline. Serum concentrations of T4, triiodothyronine (T3), reverse T3 (rT3), thyroglobulin (TG), and TSH were measured weekly as well as serum levels of GH, prolactin, and IGF-I. After 2 wk, a TSH-releasing hormone (TRH) test was performed. Neonatal illness and outcome was evaluated by noting heart rate, oxygen requirement, duration of ventilation, development of chronic lung disease, oral fluid intake, and weight gain; a Bayley score was done at the corrected age of 7 mo. L-T4 administration induced a marked increase in serum T4 without apparent change in T3 levels, whereas the postnatal decline in serum rT3 was more gradual. L-T4 treatment was associated with a decrease in serum TG and TSH levels. TRH injection induced a definite rise in serum TSH and T3 in controls, but not in L-T4 treated newborns. Neither L-T4 treatment, nor TRH administration appeared to alter circulating levels of prolactin, GH, or IGF-I. In contrast to the pronounced endocrine effects, no clinical effects of L-T4 administration were detected.

Dopamine↗
PubMed1996

Clinical and molecular genetic features of congenital spinal muscular atrophy.

A neonate presented with the fetal hypokinesia sequence and signs of spinal muscular atrophy (SMA). Severe pathological changes including ballooned neurons and neuronophagia were found not only in the motor nerve nuclei but also in the thalamic, cerebellar, and brainstem nuclei as well as in the dorsal root ganglia. Direct DNA analysis showed the presence of a chimeric SMN gene, with a rearrangement occurring between exon 7 of the centromeric SMN gene and exon 8 of the telomeric SMN gene. Circumstantial evidence suggests that only a single copy of this gene is present, with transcriptional characteristics of a centromeric SMN gene. In addition, a homozygous deletion in the NAIP genes was demonstrated. This observation demonstrates that at least some cases with fetal hypokinesia and SMA may represent the severe end of a spectrum of disorders caused by deletions in the SMA locus on chromosome 5q13. In addition, these findings are compatible with a modifying role for the centromeric SMN genes and the NAIP genes in the severity of the SMA phenotype.

Autoantigens↗
PubMed1996

The 13C-octanoic acid breath test: a noninvasive technique to assess gastric emptying in preterm infants.

Gastric emptying (GE) is difficult to evaluate properly in preterm infants because of the lack of safe and reliable noninvasive methods. The 13C-octanoic acid breath test, a noninvasive method to assess GE, was validated in adults. The aim of this study was to adapt the methodology of the 13C-octanoic acid breath test regarding test meal and sampling methods and to define normal values for healthy preterm infants. We tested 11 clinically stable preterm infants who demonstrated normal fetal growth. The infants mean gestational age at birth was 33 weeks, mean birth weight was 1754 g, mean postnatal age at the day of study was 26 days, and mean weight was 2296 g. After a fasting period of 3 h, the subject was fed a test meal with low and stable 13C background activity mixed with 50 microliters of 13C-labeled octanoic acid and 1 g polyethylene glycol 3350. Breath samples were collected using a nasal prong in basal conditions and after the test meal. CO2 production according to weight and age was used in the calculations for 13CO2 enrichment of exhaled air. Results were expressed as percentage of 13C dose excretion per hour and percentage of cumulative 13C after 4h. gastric emptying coefficient (GEC), and gastric half-emptying time (t1/2b). The values for percent of cumulative 13C after 4 h ranged from 30.7 to 52.6% (mean, 40.2%), GEC ranged from 2.7 to 3.4 (mean, 3.0), and the values for t1/2b ranged from 17 to 100 min (mean, 57 min). We conclude that the 13C-octanoic acid breath test can be adapted to preterm infants to allow the study of GE in various conditions.

Breath Tests↗
PubMed1995

Diaphragmatic hernia in Denys-Drash syndrome.

We report on a newborn infant with male pseudohermaphroditism and glomerular lesions (Denys-Drash syndrome) but without Wilms tumor. A constitutional heterozygous mutation in the WT1 gene (366Arg to His) was identified. In addition the child had a large diaphragmatic hernia, so far not described in Denys-Drash syndrome. The expression of the WT1 gene in pleural and abdominal mesothelium and the occurrence of diaphragmatic hernia in transgenic mice with a homozygous WT1 deletion strongly suggests that the diaphragmatic hernia in this patient is part of the malformation pattern caused by WT1 mutations.

Animals↗
PubMed1995

Dopamine suppresses thyroid-stimulating hormone secretion in neonatal hypothyroidism.

The infusion of dopamine, a hypophysiotropic catecholamine, which inhibits release of thyroid stimulating hormone (TSH), is the inotropic therapy of first choice in neonatal intensive care. Newborns with primary hypothyroidism are at increased risk of cardiocirculatory morbidity and are screened by measuring serum TSH concentrations. In an infant with both congenital heart disease and neonatal hypothyroidism, withdrawal of dopamine infusion was documented to evoke a doubling of serum TSH levels within 40 min, a finding suggestive of an inhibitory effect of dopamine administration on neonatal TSH hypersecretion. As a result, dopamine therapy may be a pitfall in TSH screening for neonatal hypothyroidism.

Congenital Hypothyroidism↗
PubMed1995

The prenatal role of thyroid hormone evidenced by fetomaternal Pit-1 deficiency.

The role of thyroid hormone in the human fetus is uncertain; a significant amount of T4 is transferred from the maternal to the fetal circulation. A mother-infant pair was found to be heterozygotic for a point mutation in codon 271 of the gene encoding Pit-1, a pituitary-specific transcription factor regulating somatotrope, lactotrope, and thyrotrope function. At birth, serum T4 was undetectable in mother and infant. The newborn presented with a striking delay of respiratory, cardiovascular, neurological, and bone maturation. Despite replacement therapy since birth, neurological development of the infant is impaired. Fetomaternal Pit-1 deficiency resulted in unmitigated fetal hypothyroidism that unmasked thyroid hormone as a potent endogenous drive of fetal maturation and revealed placental transfer of maternal T4 as a rescue mechanism for infants with congenital hypothyroidism, preventing fetal and neonatal symptoms of thyroid deficiency and safeguarding developmental potential.

Adult↗
PubMed1995

Birth weight and early lung compliance as predictors of short-term outcome in premature infants with respiratory distress syndrome.

In addition to birth weight (BW), respiratory mechanics during the first week of life have been reported to predict outcome in ventilated newborn infants with respiratory distress syndrome (RDS). Most measuring techniques are invasive, requiring the placement of an oesophageal tube or balloon. In the present study the compliance (Crs) and resistance (Rrs) of the total respiratory system were measured without an oesophageal tube , using a commercially available system (PEDS; MAS Inc., Hatfield, Pa.) . The Crs and Rrs were determined once, within 24 hours of birth, in 28 preterm infants requiring mechanical ventilation for RDS. Variables such as gestational age (GA) and BW were also evaluated for their predictive role in outcome. Poor outcome was defined as death from respiratory failure or the development of bronchopulmonary dysplasia (BPD) at 28 days. All non-survivors died of refractory respiratory failure, at a median of age of 6 days. The median Crs of the 21 survivors was 0.5 ml/cm H2O and of the non-survivors 0.21 ml/cm H2O (P = 0.01). Crs below 0.45 ml/cm H2O predicted 15 of the 16 infants who either developed BPD or died (positive predictive value 100%; negative predictive value 92%; sensitivity 94%; specificity 100%). Nine survivors, who subsequently developed B PD, had a median Crs of 0.38 ml/cm H2O. Their Crs was significantly lowe r than that of the infants without evidence of BPD (Crs = 0.61 ml/cm H2O ) (P = 0.01). All of the 12 babies without BPD who survived had median C rs values above 0.45 ml/cm H2O. The median Rrs of the 9 infants with BPD (96 cm H2O/l/s) was also significantly higher than the Rrs value of the non-BPD group (59 cm H2O/l/s) (P = 0.05). When stepwise multiple logistic regression was applied to predict outcome, the only variable that could be entered at a 0.05 level of significance was BW. Uncorrected compliance entered the second step, but did not reach statistical significance. We conclude that in premature infants with RDS, BW is a strong predictor of outcome. Although determination of the Crs within the first 24 hours after birth did not add significance to this predictive model, it was nevertheless a useful parameter to determine respiratory morbidity and mortality.

Birth Weight↗
PubMed1994

Intractable asphyxia at birth: analysis of the underlying conditions.

A review of clinical and pathological data on 10,000 consecutive deliveries revealed that the incidence of unexpected and intractable asphyxia at birth despite adequate resuscitation was 0.24%. Lung hypoplasia appeared to be the underlying non-viable condition in 22 out of 24 infants. Attention is drawn to congenital neuromuscular disorders which were found to be the cause of lung hypoplasia in at least 4 out of 22 infants. Some helpful hints for diagnosis are given which may contribute to the final decision making.

Asphyxia Neonatorum↗
PubMed1994

Pulmonary mechanics during respiratory distress syndrome in the prediction of outcome and differentiation of mild and severe bronchopulmonary dysplasia.

Pulmonary mechanics was prospectively and longitudinally studied in a cohort of 58 infants who suffered from respiratory distress syndrome. The aim was to determine if early compliance and resistance measurements had additional value to simple clinical variables in predicting poor outcome ie nonsurvival or severe bronchopulmonary dysplasia (BPD) at 28 days. Second, we wanted to determine whether and when the recently described type 1 (mild) BPD and type 2 (severe) BPD could be differentiated by means of lung function tests. In a logistic model, neither lung compliance nor pulmonary resistance at days 1 and 4 of life were selected as predictive variables. On the other hand, gestational age and the ventilatory index no. 1 (ventilator frequency x maximal inspiratory pressure) on day 3 were the best early predictors of poor outcome. Type 2 BPD was characterized by a lower lung compliance and a higher pulmonary resistance than type 1 BPD, although the differences were only significant at 28 days. In conclusion, pulmonary function tests were not helpful in the early prediction of poor outcome at 28 days. They might, however, be of value in the follow-up of BPD patients after 28 days.

Age Factors↗