University Childrens Hospital Berne

Bern, Switzerland

University Childrens Hospital Berne

Bern, Switzerland
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Studer M.,University Childrens Hospital Berne | Studer M.,University of Bern | Boltshauser E.,University of Zürich | Mori A.C.,Childrens Hospital Aarau | And 16 more authors.
Neurology | Year: 2014

Objective: We examined cognitive performance in children after stroke to study the influence of age at stroke, seizures, lesion characteristics, neurologic impairment (NI), and functional outcome on cognitive outcome. Methods: This was a prospectively designed study conducted in 99 children who sustained an arterial ischemic stroke (AIS) between the age of 1 month and 16 years. All children underwent cognitive and neurologic follow-up examination sessions 2 years after the insult. Cognitive development was assessed with age-appropriate instruments. Results: Although mean cognitive performance was in the lower normative range, we found poorer results in subtests measuring visuoconstructive skills, short-term memory, and processing speed. Risk factors for negative cognitive outcome were young age at stroke, seizures, combined lesion location (cortical and subcortical), as well as marked NI. Conclusions: We recommend that all children with a history of AIS undergo regularly scheduled neuropsychological assessment to ensure implementation of appropriate interventions and environmental adjustments as early as possible.© 2014 American Academy of Neurology.

Teuffel O.,University Childrens Hospital Berne | Sung L.,Hospital for Sick Children
Current Opinion in Pediatrics | Year: 2012

Purpose of review: To describe and discuss the most recent advances in the management of low-risk febrile neutropenia in children with cancer. Recent findings: Several risk stratification tools for children with febrile neutropenia have been developed, although none of these tools have been directly compared and few have been validated in independent populations. However, there is good evidence that, for pediatric patients with febrile neutropenia at low risk for severe infection, outpatient management is a well tolerated and efficacious alternative to inpatient care. Moreover, major progress has been made in obtaining and understanding perceived quality of life and preferences for outpatient management in pediatric cancer patients. Many parents prefer inpatient management although child quality of life is, in general, anticipated to be higher with outpatient intravenous therapy. Finally, outpatient strategies are more cost-effective as compared with traditional management in hospital. Summary: Outpatient management is a well tolerated and cost-effective strategy for low-risk febrile neutropenia in children with cancer, although parental preferences are highly variable for outpatient versus inpatient management. Future research should examine the effectiveness of outpatient strategies through conduct of large cohort studies. Other future work could focus on development of decision aids and other tools to facilitate ambulatory approaches. © 2012 Wolters Kluwer Health | Lippincott Williams & Wilkins.

Ooi C.Y.,Hospital for Sick Children | Ooi C.Y.,University of Toronto | Dorfman R.,Hospital for Sick Children | Cipolli M.,Cystic Fibrosis Center | And 14 more authors.
Gastroenterology | Year: 2011

Background & Aims: Different mutations in the cystic fibrosis gene (CFTR) are associated with different functional status of the exocrine pancreas. We investigated whether CFTR genotypes determine the risk of pancreatitis in patients with cystic fibrosis (CF). Methods: Patients with pancreatic-sufficient CF were identified from 2 CF population-based databases (N = 277; 62 with pancreatitis and 215 without pancreatitis); patients' genotypes and clinical characteristics were analyzed. The loss of pancreatic function associated with each CFTR genotype was determined based on the pancreatic insufficiency prevalence (PIP) score. Results: Patients with pancreatitis were more likely to have genotypes associated with mild (70%) than moderate-severe (30%) PIP scores (P = .004). The cumulative proportion of patients who developed pancreatitis through to the age of 50 years was significantly greater for genotypes associated with mild (50%) than moderate-severe (27%) PIP scores (P = .006). The genotype associated with mild PIP scores had a hazard ratio of 2.4 for pancreatitis (95% confidence interval, 1.34.5; P = .006). Patients with pancreatitis were diagnosed with CF at an older median age than those without pancreatitis (14.9 years [interquartile range, 9.527.7] vs 9.3 years [interquartile range, 1.521.4]; P = .003) and had lower mean levels of sweat chloride than patients without pancreatitis (74.5 ± 26.2 mmol/L vs 82.8 ± 25.2 mmol/L; P = .03). Conclusions:: Specific CFTR genotypes are significantly associated with pancreatitis. Patients with genotypes associated with mild phenotypic effects have a greater risk of developing pancreatitis than patients with genotypes associated with moderate-severe phenotypes. This observation provides further insight into the complex pathogenesis of pancreatitis. © 2011 AGA Institute.

PubMed | University Hospital Berne, University of Zürich, University Childrens Hospital Berne, Spital Tiefenau Berne and 5 more.
Type: Journal Article | Journal: Clinical and experimental immunology | Year: 2015

The Swiss National Registry for Primary Immunodeficiency Disorders (PID) was established in 2008, constituting a nationwide network of paediatric and adult departments involved in the care of patients with PID at university medical centres, affiliated teaching hospitals and medical institutions. The registry collects anonymized clinical and genetic information on PID patients and is set up within the framework of the European database for PID, run by the European Society of Immunodeficiency Diseases. To date, a total of 348 patients are registered in Switzerland, indicating an estimated minimal prevalence of 42 patients per 100 000 inhabitants. Distribution of different PID categories, age and gender are similar to the European cohort of currently 19 091 registered patients: predominantly antibody disorders are the most common diseases observed (n=217/348, 62%), followed by phagocytic disorders (n=31/348, 9%). As expected, predominantly antibody disorders are more prevalent in adults than in children (78 versus 31%). Within this category, common variable immunodeficiency disorder (CVID) is the most prevalent PID (n=98/217, 45%), followed by other hypogammaglobulinaemias (i.e. a group of non-classified hypogammaglobulinaemias) (n=54/217, 25%). Among phagocytic disorders, chronic granulomatous disease is the most prevalent PID (n=27/31, 87%). The diagnostic delay between onset of symptoms and diagnosis is high, with a median of 6 years for CVID and more than 3 years for other hypogammaglobulinaemias.

Jorg R.,University of Bern | Milani G.P.,Pediatric Emergency Unit | Simonetti G.D.,University of Bern | Bianchetti M.G.,University of Bern | Simonetti B.G.,University Childrens Hospital Berne
American Journal of Hypertension | Year: 2013

Background Signs of nervous system dysfunction such as headache or convulsions often occur in severe systemic hypertension. Less recognized is the association between severe hypertension and peripheral facial nerve palsy. The aim of this study was to systematically review the literature on the association of peripheral facial palsy with severe hypertension. Methods Systematic review of Medline, Embase, Web of Science, and Google Scholar from 1960 through December 2011 and report of two cases. Results The literature review revealed 24 cases to which we add two cases with severe hypertension and peripheral facial palsy. Twenty-three patients were children. Palsy was unilateral in 25 cases, bilateral in one case, and recurred in nine. The time between the first facial symptoms and diagnosis of hypertension was a median of 45 days (range, 0 days-2 years). In five case series addressing the complications of severe hypertension in children, 41 further cases of peripheral facial palsy were listed out of 860 patients (4.8%). Conclusions The association between severe hypertension and peripheral facial palsy is mainly described in children. Arterial hypertension is diagnosed with a substantial delay. Outcome is favorable with adequate antihypertensive treatment. The pathophysiology is still debated. © 2013 American Journal of Hypertension, Ltd 2013. All rights reserved.

Teuffel O.,University Childrens Hospital Berne | Leibundgut K.,University Childrens Hospital Berne | Lehrnbecher T.,Goethe University Frankfurt | Alonzo T.A.,University of Southern California | And 4 more authors.
British Journal of Haematology | Year: 2013

This systematic review and meta-analysis compared the efficacy of different anthracyclines and anthracycline dosing schedules for induction therapy in acute myeloid leukaemia in children and adults younger than 60 years of age. Twenty-nine randomized controlled trials were eligible for inclusion in the review. Idarubicin (IDA), in comparison to daunorubicin (DNR), reduced remission failure rates (risk ratio (RR) 0·81; 95% confidence interval (CI), 0·66-0·99; P = 0·04), but did not alter rates of early death or overall mortality. Superiority of IDA for remission induction was limited to studies with a DNR/IDA dose ratio <5 (ratio <5: RR 0·65; 95% CI, 0·51-0·81; P < 0·001; ratio ≥5: RR 1·03; 95% CI, 0·91-1·16; P = 0·63). Higher-dose DNR, compared to lower-dose DNR, was associated with reduced rates for remission failure (RR 0·75; 95% CI, 0·60-0·94; P = 0·003) and overall mortality (RR 0·83; 95% CI, 0·75-0·93; P < 0·001), but not for early death. Comparisons of several other anthracycline derivates did not reveal significant differences in outcomes. Survival estimates in adults suggest that both high-dose DNR (90 mg/m2 daily × 3 or 50 mg/m2 daily × 5) and IDA (12 mg/m2 daily × 3) can achieve 5-year survival rates of between 40 and 50 percent. © 2013 Blackwell Publishing Ltd.

Zimmermann P.,University Childrens Hospital | Berlinger L.,University of Bern | Berlinger L.,Bioanalytica AG | Liniger B.,University Childrens Hospital | And 5 more authors.
BMC Infectious Diseases | Year: 2012

Background: Actinobaculum schaalii was first described as a causative agent for human infection in 1997. Since then it has mainly been reported causing urinary tract infections (UTI) in elderly individuals with underlying urological diseases. Isolation and identification is challenging and often needs molecular techniques. A. schaalii is increasingly reported as a cause of infection in humans, however data in children is very limited.Case presentation: We present the case of an 8-month-old Caucasian boy suffering from myelomeningocele and neurogenic bladder who presented with a UTI. An ultrasound of the urinary tract was unremarkable. Urinalysis and microscopy showed an elevated leukocyte esterase test, pyuria and a high number of bacteria. Empiric treatment with oral co-trimoxazole was started.Growth of small colonies of Gram-positive rods was observed after 48 h. Sequencing of the 16S rRNA gene confirmed an A. schaalii infection 9 days later. Treatment was changed to oral amoxicillin for 14 days. On follow-up urinalysis was normal and urine cultures were negative.Conclusions: A.schaalii is an emerging pathogen in adults and children. Colonization and subsequent infection seem to be influenced by the age of the patient. In young children with high suspicion of UTI who use diapers or in children who have known abnormalities of their urogenital tract, infection with A. schaalii should be considered and empiric antimicrobial therapy chosen accordingly. © 2012 Zimmermann et al.; licensee BioMed Central Ltd.

PubMed | University of Zürich, University of Bonn, University Hospital Berne and University Childrens Hospital Berne
Type: Journal Article | Journal: European journal of radiology | Year: 2015

Femoro-patellar dysplasia is considered as a significant risk factor of patellar instability. Different studies suggest that the shape of the trochlea is already developed in early childhood. Therefore early identification of a dysplastic configuration might be relevant information for the treating physician. An easy applicable routine screening of the trochlea is yet not available. The purpose of this study was to establish and evaluate a screening method for femoro-patellar dysplasia using 3D ultrasound. From 2012 to 2013 we prospectively imaged 160 consecutive femoro-patellar joints in 80 newborns from the 36th to 61st gestational week that underwent a routine hip sonography (Graf). All ultrasounds were performed by a pediatric radiologist with only minimal additional time to the routine hip ultrasound. In 30 flexion of the knee, axial, coronal, and sagittal reformats were used to standardize a reconstructed axial plane through the femoral condyle and the mid-patella. The sulcus angle, the lateral-to-medial facet ratio of the trochlea and the shape of the patella (Wiberg Classification) were evaluated. In all examinations reconstruction of the standardized axial plane was achieved, the mean trochlea angle was 149.1 (SD 4.9), the lateral-to-medial facet ratio of the trochlea ratio was 1.3 (SD 0.22), and a Wiberg type I patella was found in 95% of the newborn. No statistical difference was detected between boys and girls. Using standardized reconstructions of the axial plane allows measurements to be made with lower operator dependency and higher accuracy in a short time. Therefore 3D ultrasound is an easy applicable and powerful tool to identify trochlea dysplasia in newborns and might be used for screening for trochlea dysplasia.

Zimmermann P.,University Childrens Hospital Berne | Zimmermann P.,Royal Melbourne Hospital | Ritz N.,University of Basel | Ritz N.,University of Melbourne | Stranzinger E.,University Childrens Hospital
Pediatric Infectious Disease Journal | Year: 2016

Odontoid osteomyelitis is a rare disease which is frequently misdiagnosed as torticollis, leading to a delay in diagnosis. We present 2 illustrative cases and a systematic literature review summarizing previously reported cases. Odontoid osteomyelitis should be considered in children presenting with decreased head movements and with elevated erythrocyte sedimentation rate, particularly without improvement while receiving antiinflammatory treatment. Plain radiographs can be misleading, and magnetic resonance imaging should be performed for better visualization. © 2016 Wolters Kluwer Health, Inc.

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