Childhood autoimmune cytopenia secondary to unsuspected common variable immunodeficiency

Immune thrombocytopenic purpura and autoimmune hemolytic anemia are typically idiopathic processes without underlying systemic illness. Four children with autoimmune cytopenia had low immunoglobulin levels that led to the diagnosis of common variable immunodeficiency. Routine screening of immunoglobulins is suggested for children with chronic or recurrent immune thrombocytopenic purpura and autoimmune hemolytic anemia.     

Local and cultural aspects of childhood idiopathic thrombocytopenic purpura: a summary of statements from the 12 countries worldwide

Idiopathic thrombocytopenic purpura (ITP) is a heterogeneous bleeding disorder. The Intercontinental Childhood ITP Study Group (ICIS) analyzed demographic data and outcome in a prospective registry of over 2,000 children with ITP. In addition, participating centers from 12 different countries worldwide were asked to describe their local and cultural aspects of ITP management to provide a summary statement of the variable diagnostic and management approaches for childhood ITP. The statements of the 15 reports from 12 countries are summarized under eight main areas to reflect the variability in approach to ITP among different countries. The management of ITP in children differs dramatically worldwide in terms of observation only, medical treatment, bleeding symptoms, acceptable platelet counts, need for hospitalization or outpatient treatment, and medical care climate. The majority of experimental treatments, surveys, and guidelines are not evidence-based. Although there is a great need for cooperative studies to learn more about diagnosis, management, and prognosis in the heterogeneous disorder of ITP, local and cultural variations must be considered in international investigations.     

Duplication of the cervical esophagus: a case report and review of the literature

This report describes a newborn with a cervical esophageal duplication cyst, a rare developmental anomaly of the neck. Only a few cases of this cystic entity have been described in the literature to date. This case is unique in that the patient had an air-fluid level within the lesion as a result of communication of the duplication cyst with the native esophagus.     

Localization of postresection EGF receptor expression using laser capture microdissection

Background/Purpose: Epidermal growth factor (EGF) and its receptor (EGFR) are key components in the genesis of adaptation after small bowel resection (SBR). Within intestinal homogenates, EGFR expression is increased after SBR; however, the exact cells responsible for altered EGFR expression are unknown. In this study, laser capture microdissection (LCM) microscopy was used to elucidate the specific cellular compartment(s) responsible for postresection changes in EGFR expression. Methods: Male ICR mice underwent a 50% proximal SBR or sham operation. After 3 days, frozen sections were taken from the remnant ileum. Individual cells from villi, crypt, muscularis, and mesenchymal compartments were isolated by LCM. EGFR mRNA expression for each cell compartment was quantified using real-time polymerase chain reaction (PCR). Results: EGFR expression was increased after SBR within the crypt (2-fold) and muscularis compartments (3-fold). There were no changes detected after SBR in the villus tips or mesenchymal compartments. Conclusions: Increased expression of EGFR in crypts directly correlates with the zone of cell proliferation and supports the hypothesis that EGFR signaling is crucial for the mitogenic stimulus for adaptation. The finding of increased EGFR expression in the muscular compartment is novel and may implicate a role for EGFR as a mediator of the muscular hyperplasia seen after massive SBR. J Pediatr Surg 38:440-445.     

Intelligibility of sentences recorded from the uterus of a pregnant ewe and from the fetal inner ear

The intelligibility of sentences recorded from the uterus of a pregnant ewe and from the near-term fetal sheep inner ear was judged by 30 listeners. Sentences were presented to the ewe at 95 and 105 dB SPL while sequential recordings of sound with a hydrophone and a cochlear microphonic (CM) with electrodes were made. Recordings were randomized and presented to listeners to judge the intelligibility of sentences processed through the ewe and fetal inner ear. Intelligibility scores were nearly 99% for air and uterus conditions, dropped to 73% for CM ex utero and to 41% for CM in utero. Results indicated that filtering provided by the tissues and fluids of the maternal abdomen did not affect sentence intelligibility significantly, but the filtering effects as the signal passed into the fetal inner ear resulted in much poorer intelligibility.     

D-serine facilitates the effectiveness of extinction to reduce drug-primed reinstatement of cocaine-induced conditioned place preference

Addiction is a disease that is characterized by compulsive drug-seeking and use despite negative health and social consequences. One obstacle in treating addiction is a high susceptibility for relapse which persists despite prolonged periods of abstinence. Relapse can be triggered by drug predictive stimuli such as environmental context and drug associated cues, as well as the addictive drug itself. The conditioned place preference (CPP) behavioral model is a useful paradigm for studying the ability of these drug predictive stimuli to reinstate drug-seeking behavior. The present study investigated the dose-dependent effects of D-serine (10?mg/kg, 30?mg/kg and 100?mg/kg) on extinction training and drug-primed reinstatement in cocaine-conditioned rats. In the first experiment, D-serine had no effect on the acquisition or development of cocaine-induced locomotor sensitization or CPP. In the second experiment, D-serine treatment resulted in significantly decreased time spent in the drug-paired compartment following completion of an extinction protocol. A cocaine-primed reinstatement test indicated that the combination of extinction training along with D-serine treatment resulted in a significant reduction of drug-seeking behavior. The third experiment assessed D-serine's long-term effects to diminish drug-primed reinstatement. D-serine treatment given during extinction was effective in reducing drug-seeking for more than four weeks of abstinence after the last cocaine exposure. These findings demonstrate that D-serine may be an effective adjunct therapeutic agent along with cognitive behavioral therapy for the treatment of cocaine addiction. This article is part of a Special Issue entitled 'Cognitive Enhancers'.     

Assay Formats: Recommendation for Best Practices and Harmonization from the Global Bioanalysis Consortium Harmonization Team

As part of the GBC (Global Bioanalysis Consortium), the L3 assay format team has focused on reviewing common platforms used to support ligand binding assays in the detection of biotherapeutics. The following review is an overview of discussions and presentations from around the globe with a group of experts from different companies to allow an international harmonization of common practices and suggestions for different platforms. Some of the major platforms include Gyrolab, Erenna, RIA, AlphaLISA, Delfia, Immuno-PCR, Luminex, BIAcore, and ELISAs. The review is meant to support bioanalysts in taking decisions between different platforms depending on the needs of the analyte with a number of recommendations to help integration of platforms into a GLP environment.