Cardiopulmonary resuscitation: special considerations for infants and children with cardiac disease.

Pulseless cardiac arrest, defined as the cessation of cardiac mechanical activity, determined by unresponsiveness, apneoa, and the absence of a palpable central pulse, accounts for around one-twentieth of admissions to paediatric intensive care units, be they medical or exclusively cardiac. Such cardiac arrest is higher in children admitted to a cardiac as opposed to a paediatric intensive care unit, but the outcome of these patients is better, with just over two-fifths surviving when treated in the cardiac intensive care unit, versus between one-sixth and one-quarter of those admitted to paediatric intensive care units. Children who receive chest compressions for bradycardia with pulses have a significantly higher rate of survival to discharge, at 60%, than do those presenting with pulseless cardiac arrest, with only 27% surviving to discharge.

Imaging and treatment strategies for children after first urinary tract infection.

Purpose Of Review: To highlight recent controversies regarding the rationale and effectiveness of imaging and treatment strategies for children who experience a first urinary tract infection.

Recent Findings: The yield of renal ultrasound for children who have had a first urinary tract infection is relatively low, and the most commonly identified abnormalities are of unclear clinical significance. If concerned about renal ultrasound abnormalities, clinicians should not be reassured by a normal late trimester prenatal ultrasound because its negative predictive value is not sufficiently high.

Targeting Notch signaling in autoimmune and lymphoproliferative disease.

Patients with autoimmune lymphoproliferative syndrome (ALPS) and systemic lupus erythematosis (SLE) have T-cell dysregulation and produce abnormal, activated T lymphocytes and an atypical peripheral T-cell population, termed double negative T cells (DNTs). T-cell functions, including DNT transition in T-cell development and T-cell activation, are critically dependent on Notch signaling. We hypothesized that inhibiting Notch signaling would be effective in ALPS and SLE by reducing the production of abnormal DNTs and by blocking aberrant T-cell activation.

Child passenger injury risk in motor vehicle crashes: a comparison of nighttime and daytime driving by teenage and adult drivers.

Introduction: To examine the association between child passenger injury risk, restraint use, and crash time (day vs. night) for children in crashes of vehicles driven by teenage versus adult drivers.

Methods: Cross-sectional study involving telephone interviews with insured drivers in a probability sample of 6,184 crashes involving 10,028 children.

Exercise performance in children and adolescents after the Ross procedure.

Objectives: The Ross procedure is increasingly utilized in the treatment of aortic valvar disease in children and adolescents. Our purpose was to compare pre- and post-operative exercise state in this population.

Methods: We included patients who underwent the Ross procedure at our institution between January, 1995, and December, 2003, and in whom we had performed pre- and post-operative exercise stress tests.

Neonatal lupus erythematosus with microvascular hemolysis.

A female, term newborn born to a mother with a history of idiopathic thrombocytopenic purpura and antinuclear antibodies, single-stranded A antibody, and IgM anticardiolipin antibodies presented with immune thrombocytopenia, disseminated intravascular coagulation (DIC), microangiopathic hemolytic anemia, and a characteristic lupus rash in the periorbital areas. She responded to combined treatment with dexamethasone and intravenous immunoglobulin (IVIG). At age 9 months, she was readmitted with severe thrombocytopenia, DIC, and microangiopathic hemolytic anemia.

Talo-navicular arthrodesis for residual midfoot deformities of a previously corrected clubfoot.

Triangular navicular, dorsal-lateral subluxation of the talo-navicular (TN) joint with a secondary forefoot cavovarus deformity, and degenerative changes of the TN joint are frequent causes of residual clubfoot deformity and pain in the midfoot after surgical correction. This study investigates the usefulness of TN arthrodesis to correct these deformities and to resolve symptoms resulting from these deformities. During the period from 1991-1996, the senior author performed 19 TN fusions (16 patients) for the above residual clubfoot deformities involving a painful TN joint.

Brain abscess following delayed endoscopic removal of an initially asymptomatic esophageal coin.

Brain abscesses are rare occurrences in pediatric patients, and making their diagnosis can be difficult. The two most commonly cited risk factors are otorhinologic infections and cyanotic congenital heart disease (CCHD). We present a 13-month-old child with a brain abscess who, 2 weeks prior, underwent rigid endoscopy for the extraction of a coin from the esophagus.

Adenoviral-mediated overexpression of platelet-derived growth factor-B corrects ischemic impaired wound healing.

Chronic wounds represent a major clinical problem with significant morbidity and healthcare expenditures, but no effective therapies. Topical platelet-derived growth factor-BB trials have required large and repeated doses to achieve only a modest effect. We examined the ability of an adenovirus containing the platelet-derived growth factor-B transgene to improve the rate of wound healing through induction of platelet-derived growth factor-B overexpression in cells participating in the wound healing response.

Frequency of the Fc gamma RIIIA-158F allele in African American patients with systemic lupus erythematosus.

Objective: Defects in genes involved in immune complex clearance constitute one of the most common gene defects identified in patients with systemic lupus erythematosus (SLE). Defects in early complement components, complement receptors, and Fc receptors have all been implicated in the susceptibility to SLE. Recently, the role of functionally relevant Fc receptor polymorphisms in the etiology of SLE has been investigated.

The influence of chronic disease on resource utilization in common acute pediatric conditions. Financial concerns for children's hospitals.

Objectives: To estimate the resource utilization in hospitalizations for common pediatric conditions or procedures involving patients with chronic disease vs those with no chronic disease and to develop an economic model of hospital per-patient profit (or loss) when insurance contracts fail to account for the presence of chronic disease.

Setting And Design: A retrospective analysis of selected acute pediatric conditions found in the 1991 and 1992 MedisGroups National Comparative Data Base.

Patients: We studied 30379 pediatric admissions for common acute conditions, including concussion, croup, pneumonia, appendicitis, gastroenteritis, fractures, cellulitis, urinary tract infection, and viral illness.

Elevated levels of the IGF-binding protein protease MMP-1 in asthmatic airway smooth muscle.

We have previously demonstrated that the asthma-associated proinflammatory eicosanoid leukotriene D4 (LTD4) is co-mitogenic with insulin-like growth factors (IGFs) in airway smooth-muscle (ASM) cells in vitro. This synergistic effect of LTD4 and IGF on ASM cell growth involves proteolysis of ASM-produced IGF binding proteins (IGFBPs), which are cell growth-inhibitory proteins. We also identified this IGFBP protease to be the matrix metalloproteinase-1 (MMP-1), and showed that this enzyme had a significant role in modulating IGF action in ASM cells.

Comparison of pH-stat and alpha-stat cardiopulmonary bypass on cerebral oxygenation and blood flow in relation to hypothermic circulatory arrest in piglets.

Background: Deep hypothermic circulatory arrest is used in neonatal cardiac surgery. Recent work has suggested improved neurologic recovery after deep hypothermic arrest with pH-stat cardiopulmonary bypass (CPB) compared with alpha-stat CPB. This study examined cortical oxygen saturation (ScO2), cortical blood flow (CBF), and cortical physiologic recovery in relation to deep hypothermic arrest with alpha-stat or pH-stat CPB.

Pseudopapilledema and congenital disc anomalies in growth hormone deficiency.

Optic nerve hypoplasia is a congenital disc anomaly associated with growth hormone deficiency (GHD). Pseudotumor cerebri is an adverse event associated with growth hormone treatment (hGH) and manifested by increased intracranial pressure and papilledema. Pseudopapilledema is a generic ophthalmologic term encompassing several conditions, including congenital disc anomalies.

A database program for the management of staff scheduling in a radiology department.

Objective: Our objective is to report how an inexpensive computer database program (Filemaker Pro, version 3.0, for Macintosh) can be used to manage work schedules and optimize staff use in a radiology department.

Conclusion: Using this report in conjunction with the manufacturer's documentation, one can adapt this database program to any scheduling situation.

QT and JT dispersion in children with long QT syndrome.

Introduction: Abnormalities of ventricular repolarization leading to ventricular arrhythmias place children with long QT syndrome at high risk for sudden death. Dispersion of the QT (QTd) and JT (JTd) intervals, as markers of cardiac electrical heterogeneity, may be helpful in evaluating children with long QT syndrome and identifying a subset of patients at high risk for development of critical ventricular arrhythmias (ventricular tachycardia, torsades de pointes, and/or cardiac arrest).

Methods And Results: The QTd and JTd intervals in 39 children with long QT syndrome were compared to those of 50 normal age-matched children.

Diagnosis of Duchenne/Becker muscular dystrophy and quantitative identification of carrier status by use of entangled solution capillary electrophoresis.

Use of capillary electrophoresis, a new and useful analytical tool, offers a variety of advantages for nucleic acid analyses, including rapid analysis, automation, high resolution, qualitative and quantitative results, and low consumption of both sample and reagents. We report the first example of the use of entangled solution capillary electrophoresis (ESCE) and laser-induced fluorescence detection (LIF) for separation-based diagnostics in the quantitative analysis of multiplex PCR products for determination of carrier status of Duchenne/ Becker muscular dystrophy (DMD/BMD). This approach greatly improved the speed, resolution, and sensitivity of information needed for the diagnosis of DMD/BMD compared with that from conventional diagnostic methods, and is of general utility for diagnosis of genetic diseases.

Endoscopic findings in hypertrophic pyloric stenosis: appearance in classic and evolving disease.

Background: Hypertrophic pyloric stenosis (HPS) is the most common abdominal surgical disorder in infants. Although the majority of cases are diagnosed by ultrasound, equivocal cases may require endoscopy. This study was performed to assess the various endoscopic appearances of HPS in infants.