Outcome of esophageal atresia in Germany.

The aim of this study was to evaluate the outcome of esophageal atresia in Germany in a retrospective observational study of a large cohort. Data from the major health insurance company in Germany, which covers approximately 30% of German patients, were analyzed. All patients born and registered between 2009 and 2013 with a diagnosis of esophageal atresia at first admission to the hospital were included. Mortality was analyzed during the first year of life. We identified 287 patients with esophageal atresia, including 253 with and 34 without tracheoesophageal fistula. Associated anomalies were found in 53.7% of the patients; the most frequent were cardiac anomalies (41.8%), anomalies of the urinary tract (17.4%), and atresia of the colon, rectum, and anus (9.4%). Forty-one patients (14.3%) had a birth weight <1500 g. Seventeen patients (5.9%) died before surgery. Gastrostomy was performed during the index admission in 70 patients (25.9%). The reconstruction of the esophageal passage was performed in 247 patients (93.9%). Forty-eight percent of the patients who underwent an operation required dilatation. The mortality rate in the patients who underwent an operation was 10.4%. These results from Germany correspond to the international results that have been reported. The number of dilatations was in the middle of the range of those reported in the literature; the overall mortality rate was in the upper portion of the range of the international rates. Efforts should be made to establish a clinical registry to measure and improve the quality of care for this and other rare conditions.

Percentiles of Lymphocyte Subsets in Preterm Infants According to Gestational Age Compared to Children and Adolescents.

Preterm newborns show an increased susceptibility to infections, conceivably related to their immature immune system. To gain further knowledge about the immune development in early preterm infants, we aimed to establish references for lymphocyte subsets and compare the maturation process during hospitalization to healthy term-born children and adolescents. For this purpose, peripheral blood samples (n = 153) were collected from 40 preterm infants, gestational age (GA) 26-30 week between 2nd and 6th day of life, and were monitored in intervals of every 2 or rather 4 weeks until the end of hospitalization. Furthermore, we analysed single sample controls of 10 term neonates. We compared these data with results of a study in healthy children and adolescent (n = 176). Flow cytometry of immune cell subsets was performed as single-platform analysis using 10-colour flow cytometry. Based on preterm's age, our percentile model allows readout of absolute cell count for lymphocytes, B cells, T cells, NK cells, T8 and T4 cells. The median (minimum) value of T-, B- and NK cells after birth was 2800 (600), 790 (120) and 140 (20) cells/µl, respectively. Major differences were found in absolute cell numbers of B cells, and in the frequency of regulatory T cells, most pronounced in the earliest preterm infants (GA 26). Compared to healthy children and adolescents, preterm infants reached lymphocyte counts in between the 5th and 50th percentile when discharging the hospital. This prospective observational study provides reference percentiles for lymphocytes subsets of preterm infants. These data are conducive to interpret immunological capability of preterm infants with possible immune disorders appropriate.

Prenatal management of diaphragmatic rhabdomyosarcoma presenting with fetal hydrops.

Fetal malignant tumors are rare. We present a case of intrauterine diagnosis of a diaphragmatic tumor presenting with fetal hydrops at 32 weeks' gestation. The sonographic findings were bilateral pleural effusion, ascites and skin edema. A large right-sided diaphragmatic tumor was identified. Owing to the findings on ultrasound and magnetic resonance imaging a solid malignant tumor was suspected. The pleural effusions were drained and malignant cells identified. Because of rapid tumor progression Cesarean section was performed and a hydropic female newborn was delivered at 34 + 0 weeks' gestation. There was no sign of metastatic disease. Postnatally tumor biopsy revealed an alveolar rhabdomyosarcoma. Therapy included chemotherapy and secondary surgical intervention. After a good primary response with complete remission after 6 months, the rhabdomyosarcoma relapsed at 12 months with cerebral metastasis. The prognosis was poor.

Diagnostic approaches for immunocompromised paediatric patients with pulmonary infiltrates.

Pulmonary infiltrates in immunocompromised children often pose problems in terms of deciding on further diagnostic and therapeutic procedures, but few studies have evaluated the value of non-invasive and invasive diagnostic methods in paediatric populations. Both galactomannan ELISA and PCR protocols appear to be less useful in children than in adults. Invasive procedures, such as bronchoalveolar lavage or lung biopsy, can yield a pathohistological diagnosis and/or the isolation of a pathogen. Prospective studies in paediatric patients are needed urgently to assess the value of different diagnostic procedures and to define an effective and safe diagnostic strategy for the individual child.

[Dermatitis and eosinophilia in a 9-year-old girl from Congo: putative onchodermatitis]. / Dermatitis und Eosinophilie bei einem 9-jährigen Mädchen aus dem Kongo: Wahrscheinliche Onchodermatitis.

Onchocercosis or riverblindness, caused by the filaria ochnocerca volvulus, is endemic in many countries of central and Western Africa. Symptoms of the disease can occur years after the infection, chronic itching dermatitis is the first sign, without treatment blindness may develop after years. Onchodermatitis is a hyperreactive course of onchocercosis with massive eosinophilia and elevated IgE, which suppresses a microfilarial spread through the body. Here, we report about the case of an 9-year-old girl who immigrated from the republic of Congo at the age of seven and has been living in Germany for more than two years. Presumably she suffered from onchodermatitis. She presented papular, indurated and itching skin lesions with pigmentary changes, predominantly located at the limbs. Remarkable results of blood tests were 11,000/microl (60 %) eosinophils and IgE 28 000 KU/l, ECP > 200 mg/l, without a history of atopic diseases. HIV, Strongylosis and Loa Loa were excluded. Anti filaria antibodies were detected in a concentration of 51 AKE, microscopy of skin samples failed to detect the parasites. After a single dose of Ivermectin the dermatitis improved, after two weeks the itching was absent, results of repeated blood tests tend to normalize in the following months. Due to the long lifespan of filaria in humans, the disease occurs years after infection in endemic areas. The differential diagnosis for itching skin lesions with high eosinophils in children from developing countries should include onchocercosis.

Point mutations identify the glutamate binding pocket of the N-methyl-D-aspartate receptor as major site of conantokin-G inhibition.

Conantokin-G (Con-G), a gamma-carboxylglutamate (Gla) containing peptide derived from the venom of the marine cone snail Conus geographus, acts as a selective and potent inhibitor of N-methyl-D-aspartate (NMDA) receptors. Here, the effect of Con-G on recombinant NMDA receptors carrying point mutations within the glycine and glutamate binding pockets of the NR1 and NR2B subunits was studied using whole-cell voltage-clamp recording from cRNA injected Xenopus oocytes. At wild-type receptors, glutamate-induced currents were inhibited by Con-G in a dose-dependent manner at concentrations of 0.1-100 microM. Substitution of selected residues within the NR2B subunit reduced the inhibitory potency of Con-G, whereas similar mutations in the NR1 subunit had little effect. These results indicate a selective interaction of Con-G with the glutamate binding pocket of the NMDA receptor. Homology-based molecular modeling of the glutamate binding region based on the known structure of the glutamate binding site of the AMPA receptor protein GluR2 suggests how selected amino acid side chains of NR2B might interact with specific residues of Con-G.

Subunit-dependent inhibition of recombinant rodent N-methyl-D-aspartate receptors by a HIV-1 glycoprotein 120 derived peptide.

Considerable evidence suggests that low (picomolar) concentrations of the HIV-1 envelope glycoprotein gp120 induce neuronal cell death by stimulating the release of microglial toxins, which in turn activate N-methyl-D-aspartate (NMDA) receptors. Conversely, high (micromolar) concentrations of gp120 have been reported to directly inhibit NMDA receptor-mediated currents and do not induce neurotoxicity. Here we show that micromolar concentrations of a synthetic peptide corresponding to the V3-loop of gp120 (V3-pep) inhibited agonist responses of recombinant heteromeric rodent NMDA receptors expressed in Xenopus laevis oocytes by decreasing their apparent glycine affinity. Different combinations of NMDA receptor subunits displayed differential sensitivities to inhibition by V3-pep, with a potency rank order of NR1/2B > NR1/2D > NR1/2C > or = NR1/2A. Our observations may provide an explanation for the reduced neurotoxicity of high doses of gp120 in cell cultures and may be useful for the pharmacological discrimination of NMDA receptor subtypes.