Protein kinase A translocation and insulin secretion in pancreatic beta-cells: studies with adenylate cyclase toxin from Bordetella pertussis.

Activation of protein kinase A (cAMP-dependent protein kinase; PKA) triggers insulin secretion in the beta-cell. Adenylate cyclase toxin (ACT), a bacterial exotoxin with adenylate cyclase activity, and forskolin, an activator of adenylate cyclase, both dose-dependently increased insulin secretion in the presence, but not the absence, of glucose in insulin-secreting betaTC3 cells. The stimulation of cAMP release by either agent was dose-dependent but glucose-independent.

Interleukin-1beta stimulation of c-Jun NH(2)-terminal kinase activity in insulin-secreting cells: evidence for cytoplasmic restriction.

Cytokines have been shown to have dramatic effects on pancreatic islets and insulin-secreting beta-cell lines. It is well established that cytokines such as interleukin-1beta (IL-1beta), tumor necrosis factor-alpha (TNF-alpha), and gamma-interferon (IFN-gamma) inhibit beta-cell function and are cytotoxic to human and rodent pancreatic islets in vitro. Despite the pleiotropic effects of cytokines on beta-cells, the specific signal transduction pathways and molecular events involved in beta-cell dysfunction remain largely unresolved.

Biofeedback methodology: does it matter how we teach children how to relax the pelvic floor during voiding?

Purpose: Biofeedback is a noninvasive treatment that has been documented to be helpful for children with daytime wetting and/or urinary tract infection secondary to voiding dysfunction. We wish to determine the effectiveness of biofeedback in a large population of children presenting with voiding dysfunction, and evaluate differences between 2 different methods with regard to resolution of symptoms, improvement of objective measurements and patient satisfaction.

Materials And Methods: The charts of 102 consecutive patients treated with biofeedback were reviewed.

Recurrent arch obstruction after repair of isolated coarctation of the aorta in neonates and young infants: is low weight a risk factor?

Background: Repair of aortic coarctation is often delayed in small infants because of the belief that such patients are at risk of recurrent arch obstruction and that growth will decrease this risk. To determine whether low weight was a risk factor for recurrent arch obstruction, we reviewed our experience with coarctation repair via left thoracotomy in infants less than 3 months of age.

Methods: From 1990 to 1999, 103 patients less than 3 months of age underwent repair of aortic coarctation through a left thoracotomy.

Respiratory distress after intratracheal bleomycin: selective deficiency of surfactant proteins B and C.

Intratracheal bleomycin in rats is associated with respiratory distress of uncertain etiology. We investigated the expression of surfactant components in this model of lung injury. Maximum respiratory distress, determined by respiratory rate, occurred at 7 days, and surfactant dysfunction was confirmed by increased surface tension of the large-aggregate fraction of bronchoalveolar lavage (BAL).

Jagged1 mutations in alagille syndrome.

We have summarized data on 233 Alagille syndrome patients reported with mutations in Jagged1 (JAG1). This data has been published by seven different laboratories in Europe, the United States, Australia, and Japan. Mutations have been demonstrated in 60-75% of patients with a clinically confirmed diagnosis of Alagille syndrome.

Necrotizing enterocolitis in neonates with congenital heart disease: risk factors and outcomes.

Objective: Necrotizing enterocolitis (NEC) is primarily a disease of the premature infant. Among children born at term, however, congenital heart disease may be an important predisposing factor for this condition. To determine risk factors for NEC in patients with congenital heart disease, we conducted a case-control study of neonates with cardiac disease admitted to the cardiac intensive care unit at our center during the 4-year period from January 1995 to December 1998.

Salvage of a fetus with congenital high airway obstruction syndrome by ex utero intrapartum treatment (EXIT) procedure.

A fetus with congenital high airway obstruction syndrome (CHAOS) due to complete tracheal atresia was referred at 31 weeks of gestation after 12 weeks of massive hydrops. The fetus was delivered by the ex utero intrapartum treatment procedure allowing sufficient time while on placental support for bronchoscopy to confirm tracheal atresia and tracheostomy to secure the airway. His postnatal course was complicated by severe capillary leak syndrome secondary to hydrops, diaphragmatic paralysis, tracheobronchial malacia, and the need for chronic ventilatory support.

Vascular anomalies causing symptomatic tracheobronchial compression.

Objectives/hypothesis: To review the clinical presentation and diagnostic evaluation of patients with symptomatic congenital vascular anomalies causing tracheobronchial compression and to establish the short- and long-term results of surgical intervention with respect to postoperative complications, persistent symptoms, and ventilator and tracheostomy dependence.

Study Design: Retrospective review.

Methods: Chart review and telephone follow-up.

Effectiveness of treatments for nocturnal enuresis in a heterogeneous population.

The objective of this study was to determine the effectiveness of various treatments for nocturnal enuresis in a large, diverse population of children. A retrospective cohort review of patients with nocturnal enuresis was undertaken. All patients selected treatment after a single visit that included a history, examination, and demonstration of treatments.

Hospital costs of pediatric intensive care.

Objective: To characterize hospital costs of pediatric intensive care and to determine which demographic and disease characteristics are associated with cost.

Design: Prospective cohort study.

Setting: A 20-bed pediatric intensive care unit (PICU) in an urban university-affiliated teaching children's hospital.

Congenital airway abnormalities in patients requiring hospitalization.

Objective: To determine the cause of congenital airway abnormalities in pediatric patients requiring hospitalization for their respiratory status.

Design And Setting: Case series in a tertiary care center.

Patients: A 5-year retrospective chart review was conducted at our institution.

Relationship between cell surface carbohydrates and intrastrain variation on opsonophagocytosis of Streptococcus pneumoniae.

Streptococcus pneumoniae undergoes spontaneous phase variation between a transparent and an opaque colony phenotype, the latter being more virulent in a murine model of sepsis. Opaque pneumococci have previously been shown to express lower amounts of C polysaccharide (cell wall teichoic acid) and in this study were shown to have a higher content of capsular polysaccharide by immunoelectron microscopy. This report then examined the relationship between expression of these two cell surface carbohydrate structures and their relative contribution to the increased virulence of opaque variants.

Detection of HCV RNA in Serum by Reverse Transcriptase-PCR and Radiolabeled Liquid Hybridization.

Hepatitis C virus (HCV) possesses a single-stranded, positive-sense RNA that is 9.4 kb in length. The complete HCV genome has been cloned and sequenced and encodes for a nucleocapsid, an envelope, and five nonstructural proteins (1,2).

VESAS: a solution to seasonal fluctuations in emergency department census.

Study Objective: To design and implement a plan for emergency department staffing and additional space to reduce waiting time and the rate of patients leaving without being seen during the viral epidemic season.

Methods: The study was conducted in the ED of a tertiary care children's hospital. We compared 24,657 children who presented for care between November 1996 and March 1997 (VESAS plan enacted) with 24,012 children who presented for care during the same period in the preceding year.

Interaction between tacrolimus and chloramphenicol in a renal transplant recipient.

Background: The metabolism of tacrolimus is influenced by several medications when they are given concurrently. We report the interaction between tacrolimus and chloramphenicol in a renal transplant recipient.

Methods: An adolescent with vancomycin-resistant Enterococcus was given standard doses of chloramphenicol.