Hanneke IJsselstijn

Can we improve outcome of congenital diaphragmatic hernia

This review gives an overview of the disease spectrum of congenital diaphragmatic hernia (CDH). Etiological factors, prenatal predictors of survival, new treatment strategies and long-term morbidity are described. Early recognition of problems and improvement of treatment strategies in CDH patients may increase survival and prevent secondary morbidity. Multidisciplinary healthcare is necessary to improve healthcare for CDH patients. Absence of international therapy guidelines, lack of evidence of many therapeutic modalities and the relative low number of CDH patients calls for cooperation between centers with an expertise in the treatment of CDH patients. The international CDH Euro-Consortium is an example of such a collaborative network, which enhances exchange of knowledge, future research and development of treatment protocols.

Prospective evaluation of surfactant composition in bronchoalveolar lavage fluid of infants with congenital diaphragmatic hernia and of age-matched controls.

OBJECTIVES Infants with congenital diaphragmatic hernia may have biochemically immature lungs. However, normal lecithin/sphingomyelin ratios and phosphatidylglycerol concentrations have been reported in the amniotic fluid of congenital diaphragmatic hernia patients. We hypothesized that if the lungs of congenital diaphragmatic hernia patients are surfactant deficient, that this condition would be reflected in an altered surfactant composition in the bronchoalveolar lavage fluid compared with that composition in age-matched controls. DESIGN Prospective, controlled study. SETTING Surgical intensive care unit in a Level III pediatric university hospital. PATIENTS Four groups were studied: two groups of congenital diaphragmatic hernia patients (conventionally ventilated, n = 13; treated with extracorporeal membrane oxygenation, n = 5); and two control groups (conventionally ventilated, n = 13; extracorporeal membrane oxygenation, n = 6). INTERVENTIONS Bronchoalveolar lavage, using a blind, standardized technique, was performed in conventionally ventilated congenital diaphragmatic hernia patients, extracorporeal membrane oxygenation-treated congenital diaphragmatic hernia patients, age-matched conventionally ventilated controls without pulmonary abnormalities, and extracorporeal membrane oxygenation-treated infants without congenital diaphragmatic hernia. MEASUREMENTS AND MAIN RESULTS The concentrations of different surfactant phospholipids and the fatty acid composition of phosphatidylcholine in bronchoalveolar lavage fluid were measured. No significant differences between the concentrations of phosphatidylcholine and phosphatidylglycerol, and the lecithin/sphingomyelin ratios, were found between the four groups. The fatty acid composition of phosphatidylcholine in conventionally ventilated patients showed a median percentage of palmitic acid within the normal range for age in both groups: 68% in congenital diaphragmatic hernia patients and 73% in controls (p < .001). CONCLUSIONS Our findings indicate that the concentrations of different phospholipids are similar in congenital diaphragmatic hernia patients and controls without congenital diaphragmatic hernia. A primary surfactant deficiency is unlikely in infants with congenital diaphragmatic hernia. However, secondary surfactant deficiency after respiratory failure may be involved.

Enteral Nutrition in Children with Short-Bowel Syndrome: Current Evidence and Recommendations for the Clinician

The optimal enteral feeding regimen in children with short-bowel syndrome (SBS) is debated by clinicians. The purpose of this article is to present an overview of published data on feeding strategies in children with SBS. A structured literature search (years 1966 through 2007) was done to identify human studies in children directly addressing nutrition (or specified nutrients) in relation to SBS. Eight relevant studies retrieved were graded by seven experts according to the Scottish Intercollegiate Guidelines Network criteria. This grading system is based on the study design and methodological quality of individual studies. Recommendations were made based on the outcome according to the Scottish Intercollegiate Guidelines Network if appropriate and on expert opinion otherwise. The most important recommendations are: Enteral nutrition should be initiated as soon as possible after bowel resection to promote intestinal adaptation. Enteral nutrition should be administered in a continuous fashion. Breast milk or standard polymeric formula (depending on the childs age) is recommended as preferred type of nutrition. Bottle-feeding (small volumes) should be started as soon as possible in neonates to stimulate the suck and swallow reflexes. Solid food can be introduced at the age of 4 to 6 months (corrected for gestational age if necessary) to stimulate oral motor activity and to avoid feeding aversion behavior. The team of experts concluded that high-quality research on the preferred types of enteral and oral nutrition in children with SBS is scarce. Multicenter prospective studies on the effects of feeding strategies on bowel adaptation, fecal production, linear growth, and clinical outcome are required to find the optimal feeding regimen in children with SBS.

Congenital diaphragmatic hernia with(out) ECMO: impaired development at 8 years.

Objective To evaluate developmental and social-emotional outcomes at 8 years of age for children with congenital diaphragmatic hernia (CDH), treated with or without neonatal extracorporeal membrane oxygenation (ECMO) between January 1999 and December 2003. Design Cohort study with structural prospective follow-up. Setting Level III University Hospital. Patients 35 children (ECMO: n=16; non-ECMO: n=19) were assessed at 8 years of age. Interventions None. Main outcome measures Intelligence and motor function. Concentration, behaviour, school performance, competence and health status were also analysed. Results Mean (SD) intelligence for the ECMO group was 91.7 (19.5) versus 111.6 (20.9) for the non-ECMO group (p=0.015). Motor problems were apparent in 16% of all participants and differed significantly from the norm (p=0.015) without differences between treatment groups. For all participants, problems with concentration (68%, p<0.001) and with behavioural attention (33%, p=0.021) occurred more frequently than in reference groups, with no difference between treatment groups. School performance and competence were not affected. Conclusions Children with CDH—whether or not treated with neonatal ECMO—are at risk for long-term morbidity especially in the areas of motor function and concentration. Despite their impairment, children with CDH have a well-developed feeling of self-competence.

Interdisciplinary management of infantile short bowel syndrome: resource consumption, growth, and nutrition

BACKGROUND/PURPOSE To date, there are hardly any data on the treatment costs of infantile short bowel syndrome (SBS), despite growing interest in evidence-based and cost-effective medicine. Therefore, the aim of the study was to evaluate resource consumption and costs, next to studying nutritional and growth outcomes, in children with SBS who were treated by an interdisciplinary short bowel team. METHODS Data were collected for 10 children with infantile SBS (<or=1 year of age) born between 2002 and 2007. Data included demographic and medical data of the first admission and data on resource consumption, growth, and type of nutrition for the total follow-up period. Real economic costs were calculated in Euro (euro) and US dollar (

Exercise capacity in Dutch children: New reference values for the Bruce treadmill protocol

). RESULTS Seven of the 10 patients were discharged with home parenteral nutrition. Total follow-up varied between 9 months and 5.5 years (median, 1.5 years). Six patients could be weaned off parenteral nutrition and 5 patients off enteral tube feeding, resulting in full oral intake. Seven patients had normal growth. Median duration of initial hospital admission was 174 days, and average costs of initial admission amounted to euro166,045 (

Congenital Diaphragmatic Hernia: Long-term Risk of Gastroesophageal Reflux Disease

218,681). Average total costs were euro269,700 (

Motor-function and exercise capacity in children with major anatomical congenital anomalies: an evaluation at 5 years of age.

355,195), reaching to a maximum of euro455,400 (

Early developmental assessment of children with major non-cardiac congenital anomalies predicts development at the age of 5 years

599,762). These costs mainly comprised hospital admissions (82%), followed by nutrition (12%), surgical interventions (5%), and outpatient visits (1%). CONCLUSIONS This study is among the first to describe resource consumption and costs in infants with SBS, examining real economic costs and extending beyond the initial hospitalization. Treatment of SBS requires considerable resource consumption, especially when patients depend on parenteral nutrition. Because the costs mainly comprise those of hospital admissions, early home parenteral nutrition could contribute to costs reduction. Interdisciplinary teams have the potential to facilitate early home parenteral nutrition and thus may reduce health care costs.

Neuropsychological Follow-up After Neonatal ECMO

The Bruce treadmill protocol is suitable for children 4 years of age and older. Dutch reference values were established in 1987. We considered that childrens exercise capacity has deteriorated due to changes in physical activity patterns and eating habits. We determined new reference values and evaluated determinants of exercise capacity. Healthy Dutch children (n=267) aged 6–13 years participated in this cross‐sectional observational study. The maximal endurance time on the treadmill was the criterion of exercise capacity. Furthermore, we obtained data on anthropometry, smoking habits, socioeconomic status, ethnicity, sports participation, and school transport habits. The maximal endurance time for children aged up till 10 was lower (up to 1.6 and 1.4 min in girls and boys, respectively) than previously published. Body mass index was negatively, and intense sports participation was positively associated with endurance time (β=−0.412 and 0.789, respectively; P<0.001). In conclusion, exercise capacity seems to have deteriorated in Dutch children aged up till 10 years whereas the values from the older children are remarkably similar to those from the previous study.