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Biomedical subjects

H Devlieger

Publications and source records attributed to H Devlieger.

At least 73 records · Page 4Linked to original sources

[Familial occurrence of esophageal atresia].

Two siblings with esophageal atresia are reported, and literature data as to familial occurrence and heredity have been reviewed. Until now, only statistical data can be given: the recurrence risk for sibs after one affected child is around 0.5-2% and after two affected children around 20%. The risk for the children of a parent affected by congenital esophageal atresia is estimated at around 3-4%. This is compatible with the recurrence risk of a multifactorial condition in first degree relatives. However, two patients have been described in which the possibility of autosomal dominant transmission has been raised.

Chromosome Aberrations↗

Unilateral thalamic haemorrhage in the pre-term and full-term newborn.

One full-term and three premature newborns with a unilateral thalamic haemorrhage are reported. The lesion was diagnosed using cranial ultrasonography and confirmed in three using magnetic resonance imaging. Severe perinatal asphyxia occurred in all infants. The neonatal clinical history, subsequent neuro-developmental outcome and neuroimaging findings are discussed. As the prognosis appears to be much better than for infants with bilateral thalamic densities, it is important that this type of lesion is recognised as a separate entity.

Cerebral Hemorrhage↗

Pathogenesis of congenital cystic adenomatoid malformation of the lung.

Congenital cystic adenomatoid malformation is a rare developmental abnormality of the lung. In most earlier reported cases, the anatomy of the bronchial tree was poorly documented. We describe four cases studied following autopsy. Post-mortem bronchography or serial microscopical examination showed segmental bronchial absence or atresia in each of them. Our observations provide further evidence pointing to bronchial atresia as being the primary defect leading to the development of congenital cystic adenomatoid malformation. The morphology of the lesion, i.e. the type of malformation, is determined by the extent of dysplastic lung growth beyond the atretic segment. The aetiology of the bronchial atresia is probably heterogeneous and may either represent a primary malformation, or be the result of vascular disruption.

Autopsy↗

Effect of prenatal betamethasone/thyrotropin releasing hormone treatment on somatosensory evoked potentials in preterm newborns.

The maternal administration of betamethasone and thyrotropin releasing hormone (TRH) to accelerate the maturation of the fetus is an increasingly adopted strategy to prevent neonatal morbidity in preterm infants. The effect of this prenatal treatment on the neural maturation of the infant was assessed by measuring somatosensory evoked potentials (SEP) in preterm infants (gestational age 29-36 wk) on the 1st postnatal day, at the age of 1 wk, and before discharge. The N1 latency values of the SEP obtained in 14 infants who were exposed prenatally to betamethasone/TRH were compared with the N1 latencies measured in 12 control infants. On the 1st postnatal day, the N1 latencies in the betamethasone/TRH-treated infants were strikingly shorter (p less than 0.01) than in the controls. However, at the age of 1 wk and at discharge, the N1 latency values of both groups were similar. In conclusion, the present study provides the first solid evidence for the concept that the prenatal exposure to betamethasone/TRH accelerates the SEP-assessed neural maturation of the human fetus, that this prenatal acceleration is followed by a compensatory relative deceleration during the early neonatal period, and that the subsequent SEP-assessed neural maturation proceeds at a normal velocity.

Betamethasone↗

Pulsatile and sexually dimorphic secretion of luteinizing hormone in the human infant on the day of birth.

Experimental evidence indicates that the hypothalamic-pituitary-gonadal axis is operational and sexually dimorphic in the mammalian fetus and newborn. We examined the dynamics of human luteinizing hormone (LH) secretion in five male and three female infants on the day of birth, after 34-41 wk of gestation. The infants were polycythemic, and blood samples were obtained every 20 min for 160 to 360 min during a therapeutic, standardized, isovolumetric, partial exchange transfusion. Serum LH was measured by an immunoradiometric assay that does not cross-react with human chorionic gonadotropin. The serum profiles of LH presented a striking sex dimorphism with elevated LH levels in male compared with female newborns. Deconvolution analysis of all male LH profiles was consistent with a high-frequency, pulsatile secretory pattern. Testosterone, measured in a pooled serum sample of each infant, was 10-fold higher in male than in female newborns. These results document pulsatile and sexually dimorphic secretion of LH in the human infant as early as the first day of postnatal life. It is possible that the augmented LH secretion in the male newborn participates in the neonatal rise of the serum testosterone concentration.

Estradiol↗

Laryngeal atresia sequence as part of the DiGeorge developmental field defect.

The subject of the present report is a male newborn with laryngeal atresia (LA) type I, giving rise to a malformation sequence consisting of overdistended polyalveolar lungs and nonimmune fetal hydrops with massive ascites. The infant was chromosomally normal and the first child of consanguineous parents. Retention of liquid secreted by the fetal lungs in utero was the pathogenetic mechanism, responsible for the LA sequence. The LA was part of a complex constellation of anomalies, compatible with the DiGeorge developmental field defect.

Asphyxia Neonatorum↗

[Stridor in infants: sometimes not so innocent].

Stridor is a frequent symptom in the neonate. Often it consists of a discrete inspiratory stridor in an alert child or of a transient symptom of a viral respiratory infection. In both cases no specific therapy is required. Whenever stridor occurs in the first months of life, or when it is severe or chronic, or when stridor also persists throughout expiration, an underlying anatomic anomaly frequently exists. The following steps are important to the diagnostic management: besides a detailed and complete history and physical examination, an X-ray of the airways and, if necessary, laryngo/bronchoscopy, angio(cardio)graphy or computerized tomography of the chest and the cervical region. Subsequently, appropriate therapeutic management can be initiated by proficient and accomplished physicians.

Diagnostic Imaging↗

Outcome of respiratory distress syndrome at 28 days: a prospective longitudinal study.

Fifty eight newborn infants with respiratory distress syndrome (RDS) were prospectively studied, in order to determine clinical variables prognostic of poor outcome at 28 days. Twenty six infants survived without bronchopulmonary dysplasia (BPD), 13 had Type 1 BPD, 4 had Type 2 BPD and 15 infants died before 28 days. Survivors without BPD had higher birthweights and gestational ages. Among the other infants, severity of initial lung disease was the best discriminator between outcome groups: Type 1 BPD infants had the best lungs at onset, and the nonsurvivors had the worst lungs. Stepwise multiple logistic regression identified gestational age and the ventilatory index number 1 (VI1) (= respirator frequency x maximal inspiratory pressure) at day 3 as the most useful variables to predict "poor outcome" (nonsurvival or Type 2 BPD). Ninety five percent of the infants were correctly classified using a cut-off probability of 0.5. We conclude that RDS outcome at 28 days is determined at a very early stage and that poor outcome can be predicted with reasonable accuracy at three days of age.

Birth Weight↗

[Neonatal listeriosis].

During the period november 1988-december 1989 eight cases of early onset neonatal Listeria monocytogenes infection were registered in the 'Neonatal Intensive Care Unit' of the University Hospital in Leuven (Belgium). Before bacteriological proof was available, diagnosis could be made in 7 cases, based on a characteristic clinical picture: a maternal flu-like syndrome leading to preterm labour with intact membranes, meconium stained amniotic fluid, perinatal asphyxia and respiratory distress of the neonate. In 5 infants a maculopapulovesicular skin eruption was present. Laboratory findings were non-specific. An interesting finding was the predominance of monocytes in the endotracheal aspirate of infected infants. In 5 out of 8 patients meningitis and intraventricular hemorrhage were present; two of them died, three developed severe neurological sequelae.

Cerebral Hemorrhage↗

Possible platelet contribution to pathogenesis of transient neonatal hyperammonaemia syndrome.

The pathogenesis of the transient neonatal hyperammonaemia syndrome is largely unknown. The role of platelet activation was investigated in three preterm infants with this syndrome by non-invasive methods. In all three infants, urinary concentrations of beta-thromboglobulin and 11-dehydrothromboxane B2 levels were much higher during the hyperammonaemia than those in ten control preterm infants. It is possible that transient platelet activation occurs in the portal system of these infants, thereby causing the hyperammonaemia.

Ammonia↗

The effects of the addition of sufentanil to 0.125% bupivacaine on the quality of analgesia during labor and on the incidence of instrumental deliveries.

In a double-blinded, randomized, prospective multi-center study of 695 women, we investigated whether epidural injection of sufentanil added to 0.125% bupivacaine with epinephrine (1:800,000) reduces the total amount of local anesthetic required, resulting in less motor blockade and reduced incidence of instrumental deliveries, and improves the quality of analgesia provided by this low concentration of local anesthetic without jeopardizing the safety of the baby. In addition, other potential benefits of sufentanil (such as decrease in the incidence of shivering) and side effects were examined. It was found that adding incremental doses of 10 micrograms sufentanil up to a maximum of 30 micrograms reduced the incidence of instrumental deliveries from 36 to 24% (P less than 0.01) and significantly improved quality and duration of analgesia without depressing the neurobehavioral status of the baby. No other benefits from adding sufentanil were found. The only side effect that occurred more frequently after sufentanil was pruritus. We conclude that epidural injection of 10-30 micrograms sufentanil added to 0.125% bupivacaine with epinephrine (1:800,000) improved the quality of analgesia during labor and reduced the incidence of instrumental deliveries without jeopardizing the safety of the baby.

Adult↗

Different patterns of pulmonary sequelae after hyaline membrane disease: heterogeneity of bronchopulmonary dysplasia? A clinicopathologic study.

In an autopsy study of 37 infants who suffered from hyaline membrane disease (HMD), two distinct patterns of pulmonary lesions could be defined. One pattern, characterized by marked interstitial fibrosis in the absence of airway abnormalities, was labelled 'interstitial-type' histology. A second type, labelled 'bronchiolar-type' histology, showed marked airway lesions and alveolar emphysema. Nine patients of each type were analysed. Nineteen other patients had a 'mixed-type' histology and were not further studied. Retrospectively, the clinical and radiological characteristics of both series were analysed. No differences in birth weight, gestational age and initial radiological HMD grade could be demonstrated. The pulmonary function of the bronchiolar-type patients however was significantly worse at the onset, and these infants needed more oxygen and ventilatory support during the first 10 days of life. They all died from respiratory failure whereas 4 patients from the interstitial group had non-pulmonary fatalities. In the bronchiolar group chest X-rays showed atelectasis and pulmonary interstitial emphysema in the early stages, and hyperinflation with bullae and irregular streaky densities in the later. Patients in the interstitial group had no or minor emphysema with more homogeneously spread densities over both lungs. We conclude that both types of evolution represent two extremes of the bronchopulmonary dysplasia spectrum.

Bronchopulmonary Dysplasia↗