End-of-life decisions and practices for very preterm infants in the Wallonia-Brussels Federation of Belgium.

BACKGROUND: Very preterm birth (24 to < 32 week's gestation) is a major public health issue due to its prevalence, the clinical and ethical questions it raises and the associated costs. It raises two major clinical and ethical dilemma: (i) during the perinatal period, whether or not to actively manage a baby born very prematurely and (ii) during the postnatal period, whether or not to continue a curative treatment plan initiated at birth. The Wallonia-Brussels Federation in Belgium counts 11 neonatal intensive care units. METHODS: An inventory of key practices was compiled on the basis of an online questionnaire that was sent to the 65 neonatologists working in these units. The questionnaire investigated care-related decisions and practices during the antenatal, perinatal and postnatal periods, as well as personal opinions on the possibility of standardising and/or legislating for end-of-life decisions and practices. The participation rate was 89% (n = 58). RESULTS: The results show a high level of homogeneity pointing to overall agreement on the main principles governing curative practice and the gestational age that can be actively managed given the current state of knowledge. There was, however, greater diversity regarding principles governing the transition to end-of-life care, as well as opinions about the need for a common protocol or law to govern such practices. CONCLUSION: Our results reflect the uncertainty inherent in the complex and diverse situations that are encountered in this extreme area of clinical practice, and call for qualitative research and expert debates to further document and make recommendations for best practices regarding several "gray zones" of end-of-life care in neonatology, so that high quality palliative care may be granted to all neonates concerned with end-of-life decisions.

Congenital diaphragmatic eventration and bilateral uretero-hydronephrosis in a patient with neonatal Marfan syndrome caused by a mutation in exon 25 of the FBN1 gene and review of the literature.

UNLABELLED: Neonatal Marfan syndrome, the most severe presentation of Marfan syndrome phenotypes (MIM 154700), is characterised mainly by joint contractures, arachnodactyly, loose skin, crumpled ears, severe atrioventricular valve dysfunction and pulmonary emphysema. Death usually occurs within the first 2 years of life from congestive heart failure. We describe here a newborn male with many typical characteristics of neonatal Marfan syndrome associated with a diaphragmatic eventration and a bilateral uretero-hydronephrosis with bladder dilatation. He died from cardiac failure due to severe tricuspid and mitral regurgitation at 62 h of age. CONCLUSION: Molecular analysis showed a heterozygous missense mutation at nucleotide 3165 (3165T>G) in exon 25 of the FBN1 gene, resulting in the substitution of cysteine for tryptophan (C1055W).

Surgical necrotizing enterocolitis: are intestinal lesions more severe in infants with low birth weight?

PURPOSE: This study examines whether the intestinal lesions of necrotizing enterocolitis (NEC) in infants undergoing surgery are more severe in patients with extremely low birth weight (BW). METHODS: Between 1980 and 2000, 128 infants underwent laparotomy for NEC: 90 in the acute phase, and 38 for secondary stenosis. Resections were limited to areas of transparietal bowel necrosis and to secondary stenoses. The authors studied the extent of initial bowel lesions at initial laparotomy, and, in the survivors, the extent of bowel resections and the existence of digestive sequelae, with a median follow-up of 24 (range, 1 to 247) months. Children with BW < or =1,000 g (group 1, 22 patients) and greater than 1,000 g (group 2, 103 patients) were compared by using chi(2) and t test. RESULTS: Patients' survival rate was 87%: 68% and 91% in the groups 1 and 2, respectively (P =.01). No significant difference between the 2 groups was seen: (1) for the rate of patients with panintestinal lesions at initial surgery (12%); (2) in the survivors, the ratio of remaining to total length of jejuno-ileum (mean 88%), the number of colonic segments resected (mean 1.2), the rate of survivors without distal ileum (34%), ileo-caecal valve (39%), or right colon (29%); and (3) for the existence of digestive symptoms, even minor, at last follow-up (25%). CONCLUSIONS: Although the prognosis of surgical NEC was worse in infants with extremely low birth weight, the intestinal lesions were not found more severe in these patients.

Early treatment with erythropoietin beta ameliorates anemia and reduces transfusion requirements in infants with birth weights below 1000 g.

OBJECTIVE: To investigate whether recombinant erythropoietin (rhEPO) reduces the need for transfusion in extremely low birth weight (ELBW) infants (birth weight 500-999 g) and to determine the optimal time for treatment. METHODS: In a blinded multicenter trial, 219 ELBW infants were randomized on day 3 to one of 3 groups: early rhEPO group (rhEPO from the first week for 9 weeks, n = 74), late rhEPO group (rhEPO from the fourth week for 6 weeks, n = 74), or control group (no rhEPO, n = 71). All infants received enteral iron (3-9 mg/kg/day) from the first week. The rhEPO beta dose was 750 IU/kg/week. Success was defined as no transfusion and hematocrit levels never below 30%. RESULTS: Success rate was 13% in the early rhEPO group, 11% in the late rhEPO group, and 4% in the control group (P =.026 for early rhEPO versus control group). Median transfusion volume was 0.4 versus 0.5 versus 0.7 mL/kg/day (P =.02) and median donor exposure was 1.0 versus 1.0 versus 2.0 (P =.05) in the early rhEPO group, the late rhEPO group, and the control group, respectively. Infection risk was not increased and weight gain was not delayed with rhEPO beta. CONCLUSION: Early rhEPO beta treatment effectively reduces the need for transfusion in ELBW infants.