Birth mode is associated with earliest strain-conferred gut microbiome functions and immunostimulatory potential.

The rate of caesarean section delivery (CSD) is increasing worldwide. It remains unclear whether disruption of mother-to-neonate transmission of microbiota through CSD occurs and whether it affects human physiology. Here we perform metagenomic analysis of earliest gut microbial community structures and functions.

Colonization and Succession within the Human Gut Microbiome by Archaea, Bacteria, and Microeukaryotes during the First Year of Life.

Perturbations to the colonization process of the human gastrointestinal tract have been suggested to result in adverse health effects later in life. Although much research has been performed on bacterial colonization and succession, much less is known about the other two domains of life, archaea, and eukaryotes. Here we describe colonization and succession by bacteria, archaea and microeukaryotes during the first year of life (samples collected around days 1, 3, 5, 28, 150, and 365) within the gastrointestinal tract of infants delivered either vaginally or by cesarean section and using a combination of quantitative real-time PCR as well as 16S and 18S rRNA gene amplicon sequencing.

Antiviral Drug-Resistance Typing Reveals Compartmentalization and Dynamics of Acyclovir-Resistant Herpes Simplex Virus Type-2 (HSV-2) in a Case of Neonatal Herpes.

A neonate suffering from herpes simplex virus type 2 disease with central nervous system involvement developed an early recurrence under acyclovir therapy. Isolates from the cerebrospinal fluid and skin lesions were acyclovir resistant, while viruses from blood and trachea were not. Acyclovir combined with foscavir followed by long-term suppressive acyclovir therapy supported normal neurological development.

Early myoclonic epilepsy, hypertrophic cardiomyopathy and subsequently a nephrotic syndrome in a patient with CoQ10 deficiency caused by mutations in para-hydroxybenzoate-polyprenyl transferase (COQ2).

Background: Primary coenzyme Q10 (CoQ10) deficiencies are heterogeneous autosomal recessive disorders. CoQ2 mutations have been identified only rarely in patients. All affected individuals presented with nephrotic syndrome in the first year of life.

[Life-threatening macroglossia following cleft palate palatoplasty].

The case of a 13-month-old child who developed a life-threatening macroglossia with airway obstruction following palatoplasty for a cleft palate is reported. As direct laryngoscopy was not feasible a laryngeal mask (LM) was inserted to secure the airway. Under fiber optic guidance an endotracheal tube was then introduced via the LM.

Balloon gastrostomy buttons in pediatric patients: evaluation with respect to size, lifetime in patients, and parent acceptance.

Background And Objective: To evaluate the experience with "balloon" gastrostomy buttons in pediatric patients. Distributions of the shaft lengths and the longevity of the balloon tubes were examined. Parents' and caregivers' opinion about this type of tube were analysed.

Congenital cystic adenomatoid malformation type 0-a rare cause of neonatal death.

Objective: We give the first account of failure of extracorporeal membrane oxygenation therapy secondary to congenital cystic malformation of the lung (CCAM) type 0.

Design: Case report.

Setting: Pediatric intensive care unit.

Multiple types of cardiac arrhythmias in a child with head injury and raised intracranial pressure.

Arrhythmias occur as a life-threatening complication in adults with severe head injuries. A wide spectrum of brady- and tachyarrhythmias and different pathogenetic mechanisms have been described. We report an 8-year-old boy with traumatic brain injury who developed a variety of independent types of arrhythmias during the course of his illness, including supraventricular and ventricular extrasystoles, prolonged QT duration and ventricular fibrillation, accelerated junctional rhythm, and reentry tachycardia.

[Management of septic shock and acquired respiratory distress syndrome in pediatric cancer patients].

Septic shock occurs in 6 % of paediatric cancer patients with neutropenia and fever. The mortality of the septic shock is 40 % in BMT patients and 5 % in others. One third of paediatric ARDS cases affect immunocompromised individuals with a total mortality of 45 % and 80 % after BMT.

Successful use of the new immune-suppressor sirolimus in IPEX (immune dysregulation, polyendocrinopathy, enteropathy, X-linked syndrome).

IPEX (immune-dysregulation, polyendocrinopathy, enteropathy, X-linked) syndrome is an autoimmune disorder with an often lethal outcome in spite of immunosuppressive therapy. We report the successful use of sirolimus in 3 patients with IPEX. The efficacy of sirolimus is probably due to its different mode of action compared to calcineurin-dependent agents.

[Protein-loosing enteropathy: report of four cases and review of etiology, diagnostic work-up and treatment].

The protein-loosing enteropathy (PLE) may result from a broad variety of underlying diseases. These conditions are of systemic nature or locally affecting the gastrointestinal tract. Major symptoms are oedema due to low plasma protein levels.

Autoimmune enteric leiomyositis: a rare cause of chronic intestinal pseudo-obstruction with specific morphological features.

Autoimmune enteric leiomyositis is an extraordinary rare cause of acquired chronic intestinal pseudo-obstruction in children. We report a 5-year-old girl who developed chronic intestinal pseudo-obstruction 3 years after an autoimmune hepatitis. Mucosal biopsies of the upper gastrointestinal tract and colon showed minimal inflammatory changes.

Human metapneumovirus RNA in encephalitis patient.

We describe a fatal case of encephalitis that might be correlated with primary human metapneumovirus (HMPV) encephalitis. Postmortem HMPV RNA was detected in brain and lung tissue samples from the patient. Furthermore, HMPV RNA was found in culture fluids from cells coincubated with lung tissue.

Incidence of acute respiratory distress syndrome in German children and adolescents: a population-based study.

Objective: The objective of this study was to determine the epidemiology of acute respiratory distress syndrome (ARDS) in children and adolescents aged 1 mo to 18 yrs.

Design: The authors conducted a population-based prospective multicenter survey from February 1 to 28,1997, June 1 to 30, 2001, and April 1 to 30, 2004.

Setting: This study was conducted at 94 intensive care units (ICUs) in 1997, 92 ICUs in 2001 and 2004 in the district of Cologne, Germany with a population of 4.

Phenotypic and genetic heterogeneity in congenital generalized lipodystrophy.

Congenital generalized lipodystrophy (CGL) is a rare autosomal recessive disorder characterized by near complete absence of adipose tissue from birth. Recently, mutations in 1-acylglycerol-3-phosphate O-acyltransferase 2 (AGPAT2) and Berardinelli-Seip congenital lipodystrophy 2 (BSCL2) genes were reported in pedigrees linked to chromosomes 9q34 and 11q13, respectively. There are limited data regarding phenotypic differences between the various subtypes of CGL.

Treatment with bovine surfactant in severe acute respiratory distress syndrome in children: a randomized multicenter study.

Objective: To determine whether bovine surfactant given in cases of severe pediatric acute respiratory distress syndrome (ARDS) improves oxygenation.

Design: Single-center study with 19 patients, followed by a multicenter randomized comparison of surfactant with a standardized treatment algorithm. Primary endpoint PaO(2)/FIO(2) at 48 h, secondary endpoints: PaO(2)/FIO(2) at 2, 4, 12, and 24 h, survival, survival without rescue, days on ventilator, subgroups analyzed by analysis of variance to identify patients who might benefit from surfactant.

Further case of Cantú syndrome: exclusion of cryptic subtelomeric chromosome aberrations.

Cantú syndrome consists of hypertrichosis, osteochondrodysplasia, and cardiomegaly, and has been reported in 18 patients to date. We report an infant with Cantú syndrome. In addition to typical findings, he had relatively mild radiological and cardiological manifestations.

Induction of adipocyte differentiation by a thiazolidinedione in cultured, subepidermal, fibroblast-like cells of an infant with congenital generalized lipodystrophy.

Congenital generalized lipodystrophy (CGL) is characterized by the absence of adipose tissue from birth due to a hypothetical differentiation block. The genetic causes of CGL are still not completely understood. Subepidermal, fibroblast-like cells were prepared from the sc tissue of an infant with CGL.

Plasma levels of 24S-hydroxycholesterol reflect the balance between cerebral production and hepatic metabolism and are inversely related to body surface.

We have previously presented evidence that most of the 24S-hydroxycholesterol present in the circulation originates from the brain and that most of the elimination of this oxysterol occurs in the liver. Plasma 24S-hydroxycholesterol levels decline by a factor of about 5 during the first decades of life. The concentration of the enzyme cholesterol 24S-hydroxylase in the brain is, however, about constant from the first year of life, and reduced enzyme levels thus cannot explain the decreasing plasma levels during infancy.

[Surveillance of nosocomial infections: prospective study in a pediatric intensive care unit. Background, patients and methods].

Unlabelled: BACKGROUND, PATIENTS AND METHODS: From November 1997 through May 1998, the incidence of nosocomial infections was studied prospectively in a 10-bed multidisciplinary pediatric intensive care unit in Germany. A standardized surveillance [SEKI] system based on the National Nosocomial Infection Surveillance [NNIS] System of the Centers for Disease Control and Prevention [CDC] was used. The CDC definitions for nosocomial infections were adapted to the current practice of pediatric intensive care in Germany.

Dual versus triple therapy of Helicobacter pylori infection: results of a multicentre trial.

Objective: To compare dual therapy (omeprazole and amoxicillin) with triple therapy (omeprazole, amoxicillin, and clarithromycin) in the treatment of Helicobacter pylori infection. The efficacy of 1 mg/kg/day omeprazole was randomly compared with 2 mg/kg/day.

Study Design: 252 patients (median age, 11.