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Biomedical subjects

D Tibboel

Publications and source records attributed to D Tibboel.

At least 145 records · Page 8Linked to original sources

Characterization of HNK-1 antigens during the formation of the avian enteric nervous system.

During vertebrate embryogenesis, interaction between neural crest cells and the enteric mesenchyme gives rise to the development of the enteric nervous system. In birds, monoclonal antibody HNK-1 is a marker for neural crest cells from the entire rostrocaudal axis. In this study, we aimed to characterize the HNK-1 carrying cells and antigen(s) during the formation of the enteric nervous system in the hindgut. Immunohistological findings showed that HNK-1-positive mesenchymal cells are present in the gut prior to neural crest cell colonization. After neural crest cell colonization this cell type cannot be visualized anymore with the HNK-1 antibody. We characterized the HNK-1 antigens that are present before and after neural crest cell colonization of the hindgut. Immunoblot analysis of plasma membranes from embryonic hindgut revealed a wide array of HNK-1-carrying glycoproteins. We found that two HNK-1 antigens are present in E4 hindgut prior to neural crest cell colonization and that the expression of these antigens disappears after neural crest colonization. These two membrane glycoproteins, G-42 and G-44, have relative molecular masses of 42,000 and 44,000, respectively, and they both have isoelectric points of 5.5 under reducing conditions. We suggest that these HNK-1 antigens and the HNK-1-positive mesenchymal cells have some role in the formation of the enteric nervous system.

Animals↗

[Results of prenatal echographic studies in the diagnosis of pediatric surgical abnormalities].

In 180 newborns with congenital anatomical anomalies, admitted to the Department of Paediatric Surgery, Sophia Children's Hospital, Rotterdam, we retrospectively determined whether ultrasound studies had been performed during the second and third trimesters of pregnancy. The correlation between prenatal and postnatal diagnoses was investigated as well. Ultrasound study had been performed in 86 if the 180 pregnant mothers (48%). In 41 cases the anomaly of the newborn had not been diagnosed prenatally. In 45 cases anomaly had been diagnosed prenatally. The ultimate diagnosis was the same as the prenatal diagnosis in 35 patients and was different in 10. We conclude that routine prenatal ultrasound study in the second or third trimester of pregnancy, aimed at detecting congenital anatomical anomalies to be treated by the (general) paediatric surgeon, provided little certainty about the health of the baby. Permanent education of obstetricians, and at the slightest doubt referral to one of the University Hospital Centres for specific ultrasound study, are mandatory in order to be able to correctly inform an increasing number of parents about the prospects of their unborn child.

Adult↗

[Parents' reactions to the birth of a child with a congenital anatomical abnormality: an exploratory study].

In an exploratory study of 12 couples the emotional reactions to the birth of a child with extensive anatomical malformation were investigated retrospectively, using semistructured interviews (on average 18 months after the birth). The couples were selected from the group of parents of children admitted between January 1986 and August 1988 to the Paediatric Intensive Care Unit of the Sophia Hospital, Rotterdam. The couples were divided into three groups: those who had had no ultrasonography during the pregnancy (n = 4), those in whom ultrasonography during pregnancy had revealed a foetal abnormality (n = 4) and those in whose children no such diagnoses had been made at ultrasonography during pregnancy (n = 4). After the birth of a child with congenital abnormalities, most parental couples displayed a shock reaction, denial, anger and hurt. In general there was no difference in emotional reaction between the three groups. In all couples the mourning process appeared to have begun only after the birth. Parents of a child with clearly visible abnormalities appeared to have more problems. Two-thirds of the couples had accepted the child's anomaly after some time. Persistent anger, feelings of guilt and anxiety are probably due to uncertainty regarding complications in the child or another pregnancy. Teams treating a child with a congenital anomaly should be aware of the consequences for the parents and should regard support of the parents as an essential part of their task.

Adaptation, Psychological↗

Evaluation of mortality in surgical neonates over a 10-year period: nonpreventable, permissible, and preventable death.

The mortality pattern and causes of death in surgical neonates were evaluated over the period of 1980 through 1990 in order to assess whether any deaths might have been prevented. In the study period 1,010 neonates were admitted, 104 of whom died (10%). These 104 neonates formed our study population. The Clinical Classification System (CCS) and the Therapeutic Intervention Scoring System (TISS) were used to assess the severity of illness. All 104 nonsurvivors were seriously ill (CCS 3 and 4; TISS greater than 20). The mortality pattern was classified in three groups. Group A (nonpreventable death) included 56 deaths, 48 of which were due to the underlying disease. The other 8 patients died of a nonpreventable complication of treatment. Eleven of the patients in group B (permissible death) had (multiple) congenital anomalies associated with chromosomal anomalies. Three of them were not treated and in the other 8 treatment was initially started but later withdrawn. In most of the other 14 patients further treatment was withdrawn because of serious postoperative complications or inoperable cardiac anomalies. Group C (preventable death) consisted of 23 patients. Six of 10 had irreversible brain damage due to prolonged hypoxemia. In the other 13 patients of group C, death was due to sepsis acquired in the postoperative period and treated inadequately. Staphylococcus epidermidis was the most frequent isolated pathogen (7/13).

Cause of Death↗

[The pediatrician and the surgical patient].

Knowledge of the specific reaction pattern of the developing organ system on surgical interventions is essential for the management of surgical problems in infants and children. Pediatricians and surgeons should combine the knowledge of their own field for successful treatment. The pediatrician is able to give an essential contribution to the treatment of the infant with congenital anomalies, underlying illnesses interfering with surgery, total parenteral nutrition and perioperative fluid therapy.

Child↗

[Children and accidents: decreasing mortality, different care].

In order to explore the demand for trauma care facilities for children with severe injuries in the University Hospital of Rotterdam, the departments of Paediatric Surgery and Epidemiology initiated a study of mortality, morbidity and patterns of trauma care. The standardized mortality rate in the region declined by 40% between 1979 and 1986, as did the clinical prevalence of trauma injuries, expressed by the number of days spent in hospital per 100,000 children. This was in agreement with the national epidemiological data. The number of hospital admissions for childhood injuries in the University Hospital of Rotterdam stabilized in recent years, which may be considered a relative increase. Moreover the number of children with severe injuries admitted to Intensive Care Units increased by 46%, which was mainly due to Intensive Care admissions of children living outside Rotterdam. Obviously, another pattern of referral of these patients has developed in the south-west of The Netherlands. The decrease of the number of severe injuries in childhood together with the complexity of trauma care and the increasing number of physicians raises questions on the maintenance of skills. This is an argument for centralization of trauma care for severely injured children, which to some extent has already been implemented in this region.

Accidents↗

Pulmonary hypoplasia secondary to oligohydramnios with very premature rupture of fetal membranes.

Over a 3-year-period, pulmonary hypoplasia was diagnosed in 14 cases out of a group of 150 infants born prematurely after prolonged rupture of fetal membranes and prolonged amniotic fluid leakage of whom 30 died. Review of these patients revealed pulmonary hypoplasia to be the main single cause of neonatal death (13/30). In contrast to literature data, infection was only a minor complication in this series. Lung hypoplasia was clearly correlated with long-standing rupture of membranes with oligohydramnios. It is concluded that upon premature rupture of fetal membranes, prenatal care should focus on oligohydramnios, because if this condition is prolonged, the risk of pulmonary hypoplasia increases accordingly.

Amniotic Fluid↗

Total parenteral nutrition associated cholestasis: a predisposing factor for sepsis in surgical neonates?

Of 496 neonates and infants less than 1 year of age admitted to the paediatric surgical intensive care unit (PSICU) over a 5 year period (1983-1987), 94 required total parenteral nutrition (TPN) for more than 14 consecutive days, generally due to congenital anomalies of the digestive tract. Cholestasis occurred in 15 of them and 12 of these patients developed sepsis. In contrast, of the 79 patients on TPN that remained free from cholestasis, only 23 developed sepsis. The mortality rate for the TPNAC-group was substantially higher than for the group without TPNAC. It is suggested that development of TPNAC might lead to impairment of non-specific cellular immunity in neonates.

Bacterial Infections↗

Nitrofen-induced diaphragmatic hernias in rats: an animal model.

In embryological terms, pathogenesis of congenital diaphragmatic hernia (CDH) associated with pulmonary hypoplasia is still unclear. However, it is known since 1971 that Nitrofen (2,4-dichloro-phenyl-p-nitrophenyl ether) can induce anatomical malformations in rats including diaphragmatic hernias. On order to establish an animal model of the embryogenesis of CDH, the effect of Nitrofen on the developing diaphragm was studied. Thirty-three pregnant female rats were exposed to Nitrofen. Five unexposed pregnant rats served as controls. In the first set of experiments, single doses of Nitrofen were given between the 9th and 13th day of pregnancy. In the second set of experiments, dosages of 50, 100, and 150 mg per animal were given on day 11 of pregnancy only. Postnatally the litters (469 newborn rats) were dissected to record the incidence of diaphragmatic malformations. The results were: (1) most hernias occurred after administration of 100 mg Nitrofen on day 9 (42%) and 11 (59%); (2) left-sided hernias were observed only after exposure to Nitrofen on day 9; (3) after exposure on day 10 or later all hernias were on the right side; and (4) Fifty-nine percent of the newborn rats exposed on day 11 had CDH. These results show that this model is suitable for further embryological investigations on the development of CDH.

Animals↗

Formation and malformation of the enteric nervous system in mice: an organ culture study.

Despite some progress in the treatment of congenital malformations of the enteric nervous system, there is no knowledge about the pathogenesis. The study of the normal formation of the enteric nervous system is hampered by the difficulty of manipulating and culturing mammalian embryos and their organs. Three methods to culture bowel explants of murine embryos, (chorioallantoic membrane grafting, organotypic tissue culture, and renal subcapsular space grafting) were compared. The three-dimensional cytoarchitecture of the bowel developed almost normally in the renal subcapsular space cultures. Using this culture system, it was found that neural crest cells colonize the murine bowel in distinct phases. The distal bowel was colonized at the 13th day of development. In a spontaneous mouse mutant model for intestinal aganglionosis, the lethal spotted mouse, the colonization of the distal 2 mm of the bowel did not occur at E13.

Animals↗

Carnitine deficiency in surgical neonates receiving total parenteral nutrition.

Carnitine plays a key role in the oxidation of fatty acids. Most solutions for parenteral nutrition do not contain carnitine. Because endogenous carnitine synthesis is insufficient in newborns, they are prone to developing a carnitine deficiency when they are dependent on total parenteral nutrition (TPN). Stimulated by the clinical observation of manifest clinical symptoms of carnitine deficiency in one patient, a study of 13 consecutive neonates who received TPN for over 2 weeks was begun. Their plasma carnitine levels before and during carnitine supplementation were determined. All patients had a carnitine intake far below the recommended minimal need of 11 mumol/kg per day. Although only three of them clearly showed clinical symptoms described as carnitine deficiency, carnitine supplementation for all neonates receiving TPN for over 2 weeks is recommended.

Carnitine↗

Experimentally induced congenital diaphragmatic hernia in rats.

Experiments to induce congenital diaphragmatic hernia (CDH) in rats, by means of administering a single dose of 2,4-dichlorophenyl-P-nitrophenyl (Nitrofen) on the 10th day of gestation, are reported here. Previously, congenital diaphragmatic hernia has been induced in sheep late in fetal development, and in mice early in gestation. The rat model, including a control group, was used to evaluate lung development and the presence of lung hypoplasia by morphometrical analysis. It was found that the single dose of Nitrofen, given 5 days before the normal closure of the diaphragm in the rat, leads to a high incidence of diaphragmatic hernia, mainly on the right side, and highly abnormal lung development (hypoplasia) comparable to the human situation. Both the lung weight/body weight index as well as the radial alveolar count were significantly lower in animals with CDH (P less than .05). This animal model offers a good opportunity to study abnormal lung development in relation to ventilatory capacity and pulmonary vascular reactivity.

Animals↗

Congenital diaphragmatic hernia: impact of prostanoids in the perioperative period.

A prospective study of 10 neonates with congenital diaphragmatic hernia and five controls to determine the importance of prostanoid concentrations perioperatively and the relation with persistent pulmonary hypertension (PPH) is reported. In neonates with congenital diaphragmatic hernia postoperative concentrations of the vasoconstrictor thromboxane B2 rose significantly and were higher during episodes of PPH; this rise may provoke PPH and subsequent right to left shunting.

6-Ketoprostaglandin F1 alpha↗

[Psychosocial aspects of the so-called short-bowel syndrome in children].

In a retrospective study of 16 patients with (congenital) bowel pathology, aged 2 to 12, attention was paid to the psychosocial aspects of the short bowel syndrome. Parents were interviewed and parents' and teachers' reports of children's behavioural and emotional problems were obtained. Parents as well as children appeared to have reached a reasonable level of adjustment. Parents' emotions concerning the period of hospitalisation still appeared to be strikingly strong. Although most children did not show obvious psychopathology, problems were observed in the areas of behaviour and attention, interfering with optimal school functioning in the case of some children.

Adaptation, Psychological↗