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Critical illness following heart transplantation can include acute kidney injury (AKI). Study objectives were to define the epidemiology of, risk factors for, or impact on outcomes of AKI after pediatric heart transplant. Using data from a prospective study of 66 young children, we evaluated: (1) post-operative AKI rate (by pediatric modified RIFLE criteria); (2) pre, intra, and early post-operative AKI risk factors using stepwise logistic regression (3) effect of AKI on short-term outcomes (ventilation and length of pediatric intensive care unit (PICU) stay) using stepwise multiple regression. AKI occurred in 73 % of children. Pre-transplant ventilation and higher baseline estimated creatinine clearance (eCCl) were independent risk factors for AKI. Pre-operative inotrope use was associated with reduced risk of AKI. Tacrolimus level emerged as important in multivariable risk prediction. Children with AKI had a longer duration of ventilation and length of pediatric intensive care unit (PICU) stay, with AKI being an independent predictor. AKI was common after heart transplant and associated with more complicated early post-transplant course. Lower baseline eCCl was associated with lower incidence of AKI; this merits further investigation. The association of pre-operative inotropes with less AKI may reflect a pathophysiological mechanism or be a surrogate for clinical factors and management prior to transplant. Avoiding high tacrolimus levels may be a modifiable risk factor for AKI.

Otitis media represents one of the most common infections in childhood. Within the first 3 years of life, up to 80% of children experience at least one episode of otitis media. It is often resolved with supportive therapies and consequently not considered a worrisome problem. However, it may be an early manifestation of a severe underlying disease. Primary immunodeficiencies are rare congenital defects of the immune system that often remain unrecognized, or diagnosis can be delayed, sometimes resulting in fatal consequences for the child. Patients suffer from recurrent, prolonged, and/or unusual infections leading to local sequelae, failure to thrive, developmental delays, and systemic infections with severe courses. This review provides a brief insight into primary immunodeficiencies and an overview of leading findings that should result in further evaluation of the immune system in cases of otitis media. A stepwise diagnostic approach is proposed to facilitate early and accurate diagnosis and, consequently, effective and timely therapy to improve the patient's outcome and quality of life.

The Ullrich-Turner-Syndrome (UTS) is the most commonly occurring sex chromosome abnormality in females. Cardiac malformations are well known, but no data exist on specific electrocardiogram (ECG) patterns in children and adolescents. A prolongation of the QT interval on the ECG has been correlated to an increased risk for sudden cardiac death, and medical treatment is warranted in patients with long QT syndrome (LQTS). Moreover, several drugs of common use are contraindicated in LQTS because of their effects on myocardial repolarization.The ECG tracings of 86 UTS patients [mean age (+/-SD): 11.0+/-3.6 years; mean height-SD: -2.6+/-1.0] were analyzed and compared to age-matched control groups of 75 boys (mean age: 11.3+/-3.5 years; mean height-SD: -1.9+/-1.0) and 62 girls (mean age: 10.1+/-3.2 years; mean height-SD: -2.5+/-0.9) with short stature.Eighteen UTS patients (20.9%) had an abnormally prolonged QTc0.45 s in contrast to only one control subject (0.9%). The QTc interval of UTS patients was significantly prolonged compared to that of the controls (p0.01) and normal values.There is an increased prevalence of a long QT interval among UTS patients. This should be taken into account for the cardiovascular screening of such patients. Patients with UTS have an intrinsically prolonged QT interval and as such may be at higher risk for arrhythmias in the context of a QT-prolonging medication.

Hintergrund Patienten mit X-SCID weisen eine schwere Beeintrachtigung des zellularen und humoralen Immunsystems auf. Ohne spezifische Therapie versterben sie meist innerhalb des ersten Lebensjahrs. Als kurative Behandlung steht die Knochenmark- bzw. Stammzelltransplantation zur Verfugung. Eine kleine Patientenzahl wurde erfolgreich mit einer somatischen Gentherapie behandelt, bei der das betroffene Gen in vitro mittels Retroviren in patienteneigene Stammzellen transfiziert wird.