Medical end-of-life decisions : experiences and attitudes of Belgian pediatric intensive care nurses

Objective: To investigate Belgian pediatric intensive care nurses' involvement in and attitudes toward medical end-of-life decisions with a possible or certain life-shortening effect. Methods: Questionnaires were distributed to 141 nurses working in 5 of the 7 pediatric intensive care units in Belgium. Nurses were asked to recall the last child in their care whose treatment involved an end-of-life decision and to describe anonymously their involvement in the decision. Attitudes were ascertained by means of statements and a Likert scale. Results: Questionnaires were completed by 89 nurses (63%). During the preceding 2 years, 76 (85%) had cared for at least 1 child for whom a medical end-of-life decision had been made. Nurses were involved in initiating the decision in 17% of cases, participated in decision making in 50%, and played a role in carrying out the decision in 90%. Only 6% of nurses found it always ethically wrong to hasten the death of a child by administering lethal drugs; most nurses (78%) reported they were prepared to cooperate in administering life-ending drugs in some cases. Most (89%) favored adapting the law, making life termination of children legally possible in certain cases. Conclusions: Belgian pediatric intensive care nurses are often involved in carrying out medical end-of-life decisions, including administration of life-ending drugs, whereas their participation in decision making is more limited. Most think that the current euthanasia law should be extended to minors so that administering life-ending drugs would be possible for terminally ill children in specific circumstances

Using A Sociological Model to Analyze Access to Pediatric HIV/AIDS Care in Rural Sub-Saharan Africa

The decentralization of pediatric HIV/AIDS-treatment programs to primary health care centers in rural Africa has lagged behind. In order to guide an analysis of current access to care, a sociological conceptual framework was developed. This framework focused on conditions of seeking pediatric HIV care among community members and initiating pediatric HIV care by primary health care workers (PHCWs). The use of the sociological conceptual framework helped in determining basic research questions and current gaps in knowledge (e. g. the effectiveness and long-term impact of Western counseling models in rural African settings), exploring the need for healthcare level specific research and policy (e. g. in infant HIV-testing), identifying potential pitfalls in decentralizing pediatric HIV treatment programs to rural African communities (e. g. lack of self-confidence in HIV counseling among PHCWs). Consequently, the use of the sociological model is helpful in maximizing efforts and resources allocated to such roll-out. A renewed appreciation for primary health care in general, however, remains crucial for a successful decentralization of pediatric HIV/AIDS-treatment programs to rural Africa

A Spinal Arteriovenous Fistula in a 3-Year Old Boy

We present a case of a 3-year-old boy with neurodegeneration. Family history reveals Rendu-Osler-Weber disease. Magnetic resonance imaging (MRI) of the spinal cord and spinal angiography showed a spinal arteriovenous fistula with venous aneurysm, causing compression of the lumbar spinal cord. Embolisation of the fistula was executed, resulting in clinical improvement. A week after discharge he was readmitted with neurologic regression. A second MRI scan revealed an intraspinal epidural haematoma and increase in size of the aneurysm with several new arterial feeders leading to it. Coiling of the aneurysm and fistulas was performed. Postoperative, the spinal oedema increased despite corticoids, causing more extensive paraplegia of the lower limbs and a deterioration of his mental state. A laminectomy was performed and the aneurysm was surgically removed. Subsequently, the boy recovered gradually. A new MRI scan after two months showed less oedema and a split, partly affected spinal chord. This case shows the importance of excluding possible arteriovenous malformations in a child presenting with progressive neurodegeneration. In particular when there is a family history for Rendu-Osler-Weber disease, scans should be performed instantly to rule out this possibility. The case also highlights the possibility of good recovery of paraplegia in paediatric Rendu-Osler-Weber patients

Endothelial progenitor cells and endothelial microparticles are independent predictors of endothelial function

Objective: To examine the degree of microvascular endothelial dysfunction in relation to classical cardiovascular risk factors, arterial stiffness, and numbers of circulating endothelial progenitor cells (EPCs) and endothelial microparticles (EMPs), in obese and normal-weight children. Study design: Cross-sectional study with 57 obese (15.2 +/- 1.4 years) and 30 normal-weight children (15.4 +/- 1.5 years). The principal outcome was microvascular endothelial function measured with peripheral arterial tonometry. Fasting blood samples were taken for biochemical analysis and EMPs (CD31(+)/CD42b(-) particles) and EPCs (CD34(+)/KDR+/CD45dim/(-) cells) flow cytometry. Characteristics between groups were compared by use of the appropriate independent samples test; a stepwise multiple regression analysis was used to determine independent predictors of microvascular endothelial function. Results: Microvascular endothelial function was significantly impaired in obese children and inversely correlated with body mass index Z scores (r = -0.249; P = .021) and systolic blood pressure (r = -0.307; P = .004). The number of EPCs was significantly lower in obese children and correlated with endothelial function (r = 0.250; P = .022), and the number of EMPs was significantly greater in obese children and correlated inversely with endothelial function (r = -0.255; P = .021). Multivariate analysis revealed that systolic blood pressure and numbers of circulating EPCs and EMPs are important determinants of endothelial function. Conclusion: Obese children demonstrate impaired endothelial microvascular function, increased arterial stiffness, fewer EPCs, and more EMPs. Besides systolic blood pressure, EPC and EMP counts independently predict the presence of microvascular endothelial dysfunction

Inborn errors in RNA polymerase III underlie severe varicella zoster virus infections

Varicella zoster virus (VZV) typically causes chickenpox upon primary infection. In rare cases, VZV can give rise to life-threatening disease in otherwise healthy people, but the immunological basis for this remains unexplained. We report 4 cases of acute severe VZV infection affecting the central nervous system or the lungs in unrelated, otherwise healthy children who are heterozygous for rare missense mutations in POLR3A (one patient), POLR3C (one patient), or both (two patients). POLR3A and POLR3C encode subunits of RNA polymerase III. Leukocytes from all 4 patients tested exhibited poor IFN induction in response to synthetic or VZV-derived DNA. Moreover, leukocytes from 3 of the patients displayed defective IFN production upon VZV infection and reduced control of VZV replication. These phenotypes were rescued by transduction with relevant WT alleles. This work demonstrates that monogenic or digenic POLR3A and POLR3C deficiencies confer increased susceptibility to severe VZV disease in otherwise healthy children, providing evidence for an essential role of a DNA sensor in human immunity