Pathophysiology and management of opioid-induced pruritus.

Pruritus occurs frequently following opioid use, particularly after neuraxial administration. Although not life threatening, pruritus is discomforting and may decrease patient satisfaction. Even though the mechanism of opioid-induced pruritus is not yet fully understood, there is increasing evidence of the important role played by micro opioid receptors.

Interlobular bile duct loss in pediatric cholestatic disease is associated with aberrant cytokeratin 7 expression by hepatocytes.

The objective of this study was to determine whether aberrant hepatic expression of cytokeratin 7 (CK7) and/or other putative stem cell markers is seen in pediatric cholestatic diseases. Eighteen liver biopsies and 14 liver explants from pediatric patients with extrahepatic biliary atresia (EHBA), Alagille syndrome (AGS), primary sclerosing cholangitis (PSC), inborn errors of bile acid synthesis, and progressive familial intrahepatic cholestasis (PFIC) were examined along with 5 histologically normal control liver biopsies. Immumohistochemical stains (CK7, CD56, and OV6) were performed on paraffin-embedded tissue.

Hamartomatous tongue lesions in children.

The incidence and spectrum of tongue lesions in children, in particular tongue hamartomas, is relatively unknown. We report a retrospective review of all tongue lesions seen at a major tertiary care children's hospital over an 18-year period with an emphasis on describing tongue hamartomas. A total of 135 tongue lesions were identified.

A contemporary comparison of the effect of shunt type in hypoplastic left heart syndrome on the hemodynamics and outcome at stage 2 reconstruction.

Objective: We compare the hemodynamics and perioperative course of shunt type in hypoplastic left heart syndrome at the time of stage 2 reconstruction and longer-term survival.

Methods: We retrospectively reviewed the echocardiograms, catheterizations, and hospital records of all patients who had a stage 1 reconstruction between January 2002 and May 2005 and performed a cross-sectional analysis of hospital survivors.

Results: One hundred seventy-six patients with hypoplastic left heart syndrome and variants underwent a stage 1 reconstruction with either a right ventricle-pulmonary artery conduit (n = 62) or a modified Blalock-Taussig shunt (n = 114).

Neuroblastoma.

The clinical hallmark of neuroblastoma is heterogeneity, with the likelihood of cure varying widely according to age at diagnosis, extent of disease, and tumour biology. A subset of tumours will undergo spontaneous regression while others show relentless progression. Around half of all cases are currently classified as high-risk for disease relapse, with overall survival rates less than 40% despite intensive multimodal therapy.

Midface growth in patients with ectrodactyly-ectodermal dysplasia-clefting syndrome.

Background: Ectrodactyly-ectodermal dysplasia-clefting syndrome is an autosomal dominant disorder involving abnormalities of the hands, feet, skin, and teeth and clefts of the lip and palate. Patients with this syndrome have abnormal facial features--namely, clefts of the lip and palate--and abnormal dentition, which could affect the growth of their midface region. The purpose of this study was to describe characteristics of midface growth in patients with this syndrome.

Effect of maternal betamethasone administration on prenatal congenital cystic adenomatoid malformation growth and fetal survival.

Objective: To evaluate the effect of prenatal steroid treatment on the growth of congenital cystic adenomatoid malformations (CCAM) and survival in affected fetuses not amenable to other percutaneous ultrasound-guided prenatal interventions.

Methods: A retrospective review of patients with a CCAM or hybrid lesion treated with two maternal prenatal betamethasone injections was performed. Patients receiving cyst aspiration or thoracoamniotic shunting at the time of or after steroid administration were excluded.

Incidence, complications, and risk factors for prolonged stay in children hospitalized with community-acquired influenza.

Objectives: Few studies have examined the characteristics and clinical course of children hospitalized with laboratory-confirmed influenza. We sought to (1) estimate the age-specific incidence of influenza-related hospitalizations, (2) describe the characteristics and clinical course of children hospitalized with influenza, and (3) identify risk factors for prolonged hospitalization.

Patients And Methods: Children < or = 21 years of age hospitalized with community-acquired laboratory-confirmed influenza at a large urban children's hospital were identified through review of laboratory records and administrative data sources.

Multiple simultaneous perineural infusions for postoperative analgesia in adolescents in an outpatient setting.

We report a case series of five adolescents who were managed with dual simultaneous perineural infusions after orthopaedic surgery on an outpatient basis. Adolescents undergoing orthopaedic procedures involving more than one extremity or nerve distribution can be managed after operation with multiple catheters. Use of low concentrations of local anaesthetic infusions along with good patient and family education and close follow-up by a dedicated paediatric pain management team can make this feasible in an outpatient setting.

T-cell-receptor-dependent actin regulatory mechanisms.

Following stimulation, T cells undergo marked changes in actin architecture that are required for productive immune responses. T-cell-receptor-dependent reorganization of the actin cytoskeleton is necessary for the formation of the immunological synapse at the T-cell-antigen-presenting-cell contact site and the distal pole complex at the opposite face of the T cell. Convergence of specific signaling molecules within these two plasma membrane domains facilitates downstream signaling events leading to full T-cell activation.

Neurologic complications in children hospitalized with influenza: characteristics, incidence, and risk factors.

Objective: To determine the characteristics, incidence, and risk factors for influenza-related neurologic complications (INC).

Study Design: A retrospective cohort study of INC in children hospitalized with laboratory-confirmed influenza infection (LCI) from June 2000 to May 2004 was conducted. Systematic chart review was performed to identify clinical characteristics and outcomes.

Low-dose intrathecal morphine for postoperative analgesia in children.

Background: We evaluated the efficacy and safety profile of low-dose (4-5 mcg/kg) intrathecal morphine for postoperative pain management after various surgical procedures in children.

Methods: We reviewed the pain management service database and the medical records of patients who received low-dose intrathecal morphine for postoperative analgesia at The Children's Hospital of Philadelphia between October 2003 and March 2006. Patients had been prospectively followed for 24-48 h after the intrathecal morphine administration.

Sleep and sleep disorders in children and adolescents.

Pediatric sleep disorders are common, affecting approximately 25% to 40% of children and adolescents. Although there are several different types of sleep disorders that affect youth, each disorder can have a significant impact on daytime functioning and development, including learning, growth, behavior, and emotion regulation. Researchers are only beginning to uncover the interaction between sleep and psychiatric disorders in children and adolescents, including depression, attention-deficit/hyperactivity disorder, and autism.

Ethical challenges in neonatal research: Summary report of the ethics group of the newborn drug development initiative.

Background: The Newborn Drug Development Initiative (NDDI) was established to address the lack of substantive data supporting efficacy and safety of drugs in the neonate.

Objective: This commentary summarizes some of the ethical issues involved in neonatal drug development.

Methods: At the NDDI workshop held March 29 and 30, 2004, in Baltimore, Maryland, members of the Ethics Group were dispersed among the subspecialty groups before convening to discuss common ethical themes.

Congenital muscular dystrophies and the extracellular matrix.

During the past decade, considerable progress in the field of congenital muscular dystrophies (CMDs) had led to the identification of a growing number of causative genes. This genetic progress has uncovered crucial pathophysiological concepts and has been instrumental in redefining clinical phenotypes. Important new pathogenic mechanisms include the disorders of O-mannosyl-linked glycosylation of alpha-dystroglycan as well as the involvement of a collagen type VI in the pathogenesis of congenital disorders of muscle.

Interleukin 1 receptor antagonist to treat cytophagic histiocytic panniculitis with secondary hemophagocytic lymphohistiocytosis.

Cytophagic histiocytic panniculitis (CHP) is a rare, usually fatal disease of childhood; it typically presents with daily high spiking fevers and severe panniculitis. Hemophagocytosis from macrophage activation during a cytokine storm can result in pancytopenia and disseminated intravascular coagulopathy. We describe a 14-year-old girl with long-standing CHP who developed severe hemophagocytic lymphohistiocytosis, which responded to treatment with a combination of high dose corticosteroids, cyclosporine, and the interleukin 1 receptor antagonist, anakinra.

The new pentavalent rotavirus vaccine composed of bovine (strain WC3) -human rotavirus reassortants.

Background: Infantile gastroenteritis caused by human rotaviruses is a prevalent disease throughout the world, causing dehydration and hospitalization in all countries. In developing countries, it is associated with a high mortality. A licensed vaccine against rotavirus was withdrawn because of a causal association with intussusception.

Multiple cardiac arrhythmias in a previously healthy child: a case of accidental digitalis intoxication?

We present a case of multiple arrhythmias in a 3-year-old child who was presented to the emergency department with emesis. Initial vital signs were significant for a heart rate from 40 to 60 beats per minute with stable blood pressure. An electrocardiogram showed complete atrioventricular block with a junctional escape rhythm of 40 to 55 bpm that subsequently progressed to atrial flutter/fibrillation and then to a junctional escape rhythm.

Ophthalmologic findings in Cornelia de Lange syndrome: a genotype-phenotype correlation study.

Objective: To evaluate individuals with Cornelia de Lange syndrome previously screened for mutations in the NIPBL gene for genotype-phenotype correlations with regard to severity of ophthalmologic findings.

Methods: Fifty-four patients with Cornelia de Lange syndrome (26 mutation positive and 28 mutation negative) with varying extent and severity of ophthalmologic findings participated in the study. We conducted a retrospective analysis of ophthalmologic data obtained through survey responses and medical records.

Jagged1 (JAG1) mutations in Alagille syndrome: increasing the mutation detection rate.

Alagille syndrome (AGS) is caused by heterozygous mutations in JAG1, and mutations have been previously reported in about 70% of patients who meet clinical diagnostic criteria. We studied a cohort of 247 clinically well-defined patients, and using an aggressive and sequential screening approach we identified JAG1 mutations in 94% of individuals. Mutations were found in 232 out of 247 patients studied and 83 of the mutations were novel.

Immune reconstitution and predictors of virologic failure in adolescents infected through risk behaviors and initiating HAART: week 60 results from the PACTG 381 cohort.

The responses to HAART in HIV-infected adolescents infected through risk behaviors are not well defined. PACTG 381 collected intensive immunologic and virologic data on youth naive to or with minimal exposure to antiretroviral therapy who began HAART. Subjects were evaluated according to their weeks 16-24 virologic response.

Molecular and immunohistochemical analyses of the focal form of congenital hyperinsulinism.

Congenital hyperinsulinism is a rare pancreatic endocrine cell disorder that has been categorized histologically into diffuse and focal forms. In focal hyperinsulinism, the pancreas contains a focus of endocrine cell adenomatous hyperplasia, and the patients have been reported to possess paternally inherited mutations of the ABCC8 and KCNJ11 genes, which encode subunits of an ATP-sensitive potassium channel (K(ATP)). In addition, the hyperplastic endocrine cells show loss of maternal 11p15, where imprinted genes such as p57(kip2) reside.

Structure-function studies of PANDER, an islet specific cytokine inducing cell death of insulin-secreting beta cells.

PANDER (pancreatic derived factor, FAM3B) is a novel cytokine, present in insulin secretory granules, that induces apoptosis of alpha and beta cells of mouse, rat, and human islets in a dose- and time-dependent manner, and may be implicated in diabetes. PANDER has the predicted secondary structure of 4 alpha-helical bundles with an up-up-down-down topology, and two disulfide bonds. Eleven mutated PANDERs were constructed and expressed in beta-TC3 cells to identify the essential region of PANDER involved in beta-cell death.

Injury pattern of the neonatal brain after hypothermic low-flow cardiopulmonary bypass in a piglet model.

Unlabelled: Low-flow cardiopulmonary bypass (LF-CPB) is a widely used modality in neonatal heart surgery. While facilitating surgical repair, it poses a risk of neurological injury caused by hypoperfusion. In the present study, we characterize the injury pattern and influencing factors in a piglet hypothermic LF-CPB model.

Class B scavenger receptor-mediated intestinal absorption of dietary beta-carotene and cholesterol.

There is now a general consensus that the intestinal absorption of water-insoluble, dietary lipids is protein-mediated, but the assignment of protein(s) to this function is still a matter of debate. To address this issue, we measured beta-carotene and cholesterol absorption in wild-type and SR-BI knockout mice and the uptake of these lipids in vitro using brush border membrane (BBM) vesicles. From the comparison of the in vivo and in vitro results we conclude that both BBM-resident class B scavenger receptors, SR-BI and CD36, can facilitate the absorption of beta-carotene and cholesterol.