Vermindering van diagnostiek en overbehandeling bij RS-virus-bronchiolitis na geprotocolleerde behandeling.

PURPOSE: Evaluating the guideline 'Diagnosis and treatment of respiratory syncytial (RS) virus bronchiolitis' on the number of chest X-rays, C-reactive proteïn (CRP) counts, leukocyte counts, and antibiotic prescriptions in infants admitted to hospital with RS bronchiolitis. DESIGN: Retrospective 'before-after' cohort study. LOCATION: Canisius-Wilhelmina Hospital, Nijmegen, The Netherlands. PATIENTS: Infants admitted with proven RS virus infection. METHODS: Guidelines, including sound restriction of performance of X-rays, CRP and leukocytes, were introduced in February 2003. Data from infants admitted with RS virus infection during 1997- 1999 (cohort A) were compared with those admitted from 2003- April 2006 (cohort B)Results: There were 155 infants in cohort A and 170 in cohort B. Implementation of guidelines led to significant reductions of CRP and leukocyte determinations: 49.0% and 48.2%, respectively (both p<0.001) and X-rays: 30.3% (p=0.020). Numbers of antibiotic prescriptions decreased with 55% (p<0.001). The chance of antibiotic prescription increased significantly when X-rays (OR=5.2), CRP (OR=5.4), or leukocytes (OR=4.2) were done. After implementation of the guidelines, the median stay in hospital decreased significantly from 8.0 to 6.0 days (p<0.001; ranges 1-13 days and 2-23 days, respectively). Performing X-ray, CRP or leukocytes, or antibiotic prescription did not significantly alter the total duration of hospital stay. CONCLUSION: Implementation of the guidelines led to significant decreases in numbers of X-rays, CRP and leukocytes determinations, and antibiotic prescriptions. Our data support the restrictive use of chest X-rays, CRP and leukocyte determinations in infants, admitted to hospital with RS virus bronchiolitis.

Effects of continuing paediatric education in interpersonal communication skills.

UNLABELLED: Paediatric care places great demands on interpersonal communication skills, especially as regards the handling of psychosocial issues. Recent shifts in paediatric morbidity and increases in patient empowerment furthermore emphasize the need for continuing paediatric education in communication skills. It is, however, debatable, whether after residency paediatric education can influence paediatrician performance. This study evaluated the effects of a 5-day experiential communication training by means of a pretest/post-test control group design. A total of 21 consulting paediatricians (10 experimental, 11 control group paediatricians) videotaped two series of consecutive outpatient encounters. Compared with the untrained control group, trained paediatricians asked more psychosocial questions and looked at the patients and their parents more often. In addition, they gave patients and parents more room to talk. No difference was found in the length of the outpatient visits. CONCLUSION: Paediatric education after residency influences paediatricians' verbal and nonverbal communication skills positively, especially as regards handling psychosocial issues. On the basis of these findings, it appears worthwhile to encourage continuing paediatric education in interpersonal communication skills.

Immunological tolerance in an HLA non-identical chimeric twin.

Blood group chimeric twins offer a unique opportunity to study immunological tolerance in humans. Although this condition is not as rare as previously considered, detailed immunological studies of blood group chimeras are lacking. We describe here a case of secondary chimerism in a dizygotic twin of opposite gender. The karyotypes of the cultured fibroblast confirmed the sex of each twin, all cells in the boy were 46, XY and all cells in the girl were 46, XX. Molecular HLA typing on fibroblasts revealed HLA-DR, DQ and DP disparities between the two siblings. Mixed lymphocyte culture (MLC) revealed a mutual absence of alloreactivity.

[Acute pancreatitis in children]. / Acute pancreatitis bij kinderen.

Acute pancreatitis is probably commoner in children than was previously thought. In children it is most commonly associated with trauma or viral infection. The presentation may be subtler than in adults, requiring a high index of suspicion in the clinician. In three children, two boys aged 4 and 10 and a girl of 15 years, acute pancreatitis was suspected because of the findings at ultrasonography and endoscopic retrograde cholangiopancreatography performed when the disease recurred (the boy aged 4), apathy and immobility without dehydration or other obvious causes (the boy aged 10), and severe abdominal pain in combination with vomiting (the girl). All three patients had severely increased (urinary) amylase levels. Most often, acute pancreatitis in children tends to be a self-limiting disease which responds well to conservative treatment.

The role of parents in end-of-life decisions in neonatology: physicians' views and practices.

OBJECTIVE: End-of-life decisions for newborn infants are usually made with the consent of parents as well as physicians, but may occasionally involve disagreement about which decision is in the best interest of the child. Our study was aimed at providing an empirical background for the ethical discussion on the parent's versus the physician's role in decision-making. METHODS: We conducted face-to-face interviews with a stratified sample of pediatricians. The response rate was 99%. The most recent decisions in newborn infants to hasten death or not prolong life and the most recent cases in which such decisions were not made because either the parents or the physician objected were comprehensively discussed. RESULTS: Decisions to hasten death or not prolong life were usually made after discussing it with parents and did not occur while parents were known to disagree. Situations in which an end-of-life decision was not made because parents did not consent predominantly involved infants with complications of prematurity (24%) or perinatal asphyxia (40%), whereas situations in which parents requested an end-of-life decision that was not acceded to by the pediatrician involved Down syndrome as the main diagnosis in 43% and as a concurrent diagnosis in 21%. Pediatricians afterwards often expressed feelings of discontent about situations in which there had been disagreement with parents. CONCLUSIONS: The opinion of parents about which medical decision is in the best interest of their child is for pediatricians only decisive in case it invokes the continuation of treatment. The principle of preserving life is abandoned only when the physician feels sufficiently sure that the parents agree that such a course of action is in the best interest of the child.

Medical end-of-life decisions made for neonates and infants in the Netherlands.

BACKGROUND: Advances in neonatal intensive care have lowered the neonatal death rate. There are still some severely ill neonates and infants, however, for whom the application of all possible life-prolonging treatment modalities may be questioned. METHODS: We did two studies in the Netherlands. In the first we sent questionnaires to physicians who had attended 338 consecutive deaths (August-November, 1995) within the first year of life (death-certificate study), and in the second we interviewed 31 neonatologists or paediatric intensive-care specialists and 35 general paediatricians. The response rates were 88% and 99%, respectively. FINDINGS: In the death-certificate study, 57% of all deaths had been preceded by a decision to forgo life-sustaining treatment; this decision was accompanied by the administration of potentially life-shortening drugs to alleviate pain or other symptoms in 23%, and by the administration of drugs with the explicit aim of hastening death in 8%. A drug was given explicitly to hasten death to neonates not dependent on life-sustaining treatment in 1% of all death cases. No chance of survival was the main motive in 76% of all end-of-life decisions, and a poor prognosis was the main motive in 18%. The interview study showed that parents had been involved in making 79% of decisions. The physicians consulted colleagues about 88% of decisions. Most paediatricians favoured formal review of medical decisions by colleagues together with ethical or legal experts. INTERPRETATION: Death among neonates and infants is commonly preceded by medical end-of-life decisions. Most Dutch paediatricians seem to find prospects for survival and prognostic factors relevant in such decisions. Public control by a committee of physicians, paediatricians, ethicists, and legal experts is widely endorsed by paediatricians.

Adenylosuccinase deficiency: an inborn error of purine nucleotide synthesis.

Clinical and biochemical data are presented on eight children with adenylosuccinase deficiency. This newly discovered inborn error of purine metabolism is characterized by an accumulation in body fluids of succinyladenosine (S-Ado) and succinylaminoimidazole carboxamide riboside (SAICA riboside), the dephosphorylated derivatives of the two substrates of adenylosuccinase. Six living children (three boys and three girls) and one deceased sibling displayed severe psychomotor retardation. Epilepsy was documented in five cases, autistic features in three, and growth retardation associated with muscular wasting in a brother and sister. In the cerebrospinal fluid, plasma and urine of these patients, the S-Ado/SAICA riboside ratio was between 1 and 2. In striking contrast, the eighth patient (a girl) was markedly less mentally retarded. Most noteworthy, the S-Ado/SAICA riboside ratio in her body fluids was around 5, suggesting that her milder psychomotor retardation was causally linked to this higher ratio. Adenylosuccinase deficiency was demonstrated in the liver of all seven living children, in the kidney of three patients in whom the enzymatic activity was measured, and in the muscle of three patients, including the two with muscular wasting. In fibroblasts of the six severely retarded patients, adenylosuccinase activity was reduced to approximately 40% of normal; in the patient with the higher S-Ado/SAICA riboside ratio, it reached only 6% of normal. The clinical heterogeneity of adenylosuccinase deficiency justifies systematic screening for the enzyme defect in unexplained neurological disease.

Bone marrow transplantation in children with severe aplastic anemia: reconstitution of cellular immunity.

Cellular immune functions were evaluated longitudinally in seven children with severe aplastic anemia, who were successfully transplanted with bone marrow cells from an HLA-identical, mixed lymphocyte culture (MLC)-negative sibling. Several parameters were followed: the number of lymphocytes and E rosette-forming cells in the peripheral blood and the lymphocyte reactivity toward various mitogens, antigens, and allogeneic lymphocytes. Some patients already displayed decreased in vitro lymphocyte reactivity before transplantation, especially with regard to the response to pokeweed mitogen (PWM). After transplantation, a severe cellular immunodeficiency developed in all patients, with low numbers of T cells and markedly impaired responsiveness to mitogens, antigens, and allogeneic lymphocytes. Variations between patients were substantial, both with regard to the severity and duration of the immunodeficiency and to the pattern of the recovery of lymphocyte responses to mitogens and antigens. This variability might be attributable to an imbalanced proliferation of different lymphocyte subsets and/or the sequence of appearance of receptors for mitogens on the cell surface.