The atonic stomach: a dangerous condition prior to scoliosis surgery.

A dilated atonic stomach as part of neuromuscular or syndromic disorders can have devastating results after scoliosis surgery. Patients can be asymptomatic preoperatively and non-clinical signs can be easily overlooked. Awareness of the condition, however, can prevent severe complications such as aspiration.

Outcome of invasive mechanical ventilation after pediatric allogeneic hematopoietic SCT: results from a prospective, multicenter registry.

Exact data on prognosis of children receiving invasive mechanical ventilation (IMV) after allogeneic hematopoietic SCT (HSCT) is lacking. We therefore started a prospective registry in four European university HSCT centers (Leiden, Paris, Prague and Utrecht) and their pediatric intensive care units (PICUs). The registry started in January 2009. In January 2013, the four centers together had treated a total of 83 admissions with IMV. The case fatality rate in these patients was 52%. Mortality 6 months after PICU discharge was 45%. There were significant differences between centers in the proportion of children who received IMV after HSCT (6-23%, P<0.01), in severity of disease on admission to PICU (predicted mortality 14-37%, P<0.01), in applying noninvasive ventilation before IMV (3-75% of admissions, P<0.01) and in the use of renal replacement therapy (RRT) (8-58% of admissions, P<0.01). Severe impairment in oxygenation, use of RRT and CMV viremia were independent predictors of mortality. Our study shows that mortality in children receiving IMV after HSCT remains high, but has clearly improved compared with older studies. Patient selection and treatment in PICU differed significantly between centers, which underscores the need to standardize and optimize the PICU admission criteria, ventilatory strategies and therapies applied in PICU.

Systematic review: clinical efficacy of chelator agents and zinc in the initial treatment of Wilson disease.

BACKGROUND: No consensus is available on the optimal initial treatment in Wilson disease. AIM: To assess systematically the available literature of treatment in newly presenting patients with a presymptomatic, hepatic or neurological presentation of Wilson disease. METHODS: A systematic literature search of the MEDLINE, EMBASE and COCHRANE databases was performed. Original studies on clinical efficacy of D-penicillamine, trientine, tetrathiomolybdate or zinc monotherapy as initial treatment in Wilson disease were included. A descriptive analysis of the relevant published data was performed. RESULTS: One randomized trial and 12 observational studies met the inclusion criteria. These studies were quite heterogeneous and generally of low validity. Nevertheless, according to currently available data, patients with hepatic presentation of Wilson disease are probably most effectively treated by D-penicillamine. Zinc seems to be preferred above d-penicillamine for treatment of presymptomatic and neurological patients, as in these subgroups, the tolerance profile is in favour of zinc, while no obvious differences in clinical efficacy could be observed. CONCLUSIONS: There is lack of high-quality evidence to estimate the relative treatment effects of the available drugs in Wilson disease. Therefore, multicentre prospective randomized controlled comparative trials are necessary.

[The practice guideline 'Volume suppletion in critically-ill neonates and children up to the age of 18 years' of the Dutch Paediatric Association]. / Richtlijn 'Volumesuppletie bij kritisch zieke pasgeborenen en kinderen tot 18 jaar' van de Nederlandse Vereniging voor Kindergeneeskunde.

Hypovolaemia is the most common cause of circulatory failure in children. Treatment consists of volume suppletion with a crystalloid or colloid solution; which agent is the best in children is not clear. This evidence-based practice guideline formulates recommendations as to which fluid should be used for volume suppletion in critically-ill neonates and children up to the age of 18 years with hypovolaemia. Before the guideline development first-choice fluid for volume resuscitation was in 50% a colloid and in 50% a crystalloid solution for both neonatologists and paediatric intensivists. The neonatologists used human albumin as a priority, and the paeditric intensivists predominantly used a synthetic colloid. The guideline was developed on the basis of a comprehensive search and analysis of the literature according to the principles of evidence-based guideline development. The recommendations were formulated by a committee based on evidence from the literature and, when evidence from the literature was insufficient, on consensus after discussion in the committee. Since colloids are much more expensive than crystalloids and can give an anaphylactic reaction, their added value over crystalloids must be proven. In sick neonates and children, insufficient clinical trials have been done to reach the conclusion that colloids are more effective than crystalloids in hypovolaemia. A number of meta-analyses in adults revealed excess mortality in the group treated with albumin, but one recent, large, randomised study showed no difference in mortality. No added value could be demonstrated for the administration of synthetic colloids. On the basis of data from the literature and considerations regarding the applicability of evidence in adults to children and neonates, the side effects of resuscitation fluids, pathophysiology and costs, the first-choice fluid for neonates and children with hypovolaemia is isotonic saline. Albumin should not be used for the treatment of hypovolaemia. The volume to be administered and the infusion rate depend on the severity of the hypovolaemia and should be determined on an individual basis.

A drug use review study in patients with obstructive lung disease. Assessment of the quality of drug therapy.

This study presents the results of an analysis of the pharmacy records of 778 patients with asthma or chronic obstructive lung diseases. The high percentage of patients taking oral corticosteroids was striking. Inhaled beta-agonists for use as needed have been prescribed to only a minority of patients using these agents. Only half of the patients on beta-agonists used inhaled corticosteroids prophylactically. Drug interactions capable of causing changes in plasma theophylline concentrations appeared in only a small number of patients. The results from studies like the one presented here can provide valuable data to be used for further discussion between physicians and pharmacists about rational drug therapy.