Impact of pulmonary vascular resistances in heart transplantation for congenital heart disease.

Congenital heart disease is one of the major diagnoses in pediatric heart transplantation recipients of all age groups. Assessment of pulmonary vascular resistance in these patients prior to transplantation is crucial to determine their candidacy, however, it is frequently inaccurate because of their abnormal anatomy and physiology. This problem places them at significant risk for pulmonary hypertension and right ventricular failure post transplantation.

Perioperative management of the pediatric cardiac transplantation patient.

The major diagnoses carried by children undergoing cardiac transplantation worldwide are congenital heart defects, cardiomyopathies, and retransplantation. The leading diagnosis in infancy is congenital heart disease, whereas cardiomyopathy predominates in older children. In view of this wide spectrum of diagnoses, the perioperative management of these children requires medical, interventional, and surgical expertise in treatment of complex congenital heart defects, end-stage heart failure, and cardiac transplantation.

Mechanical circulatory support of the critically ill child awaiting heart transplantation.

The majority of children awaiting heart transplantation require inotropic support, mechanical ventilation, and/or extracorporeal membrane oxygenation (ECMO) support. Unfortunately, due to the limited pool of organs, many of these children do not survive to transplant. Mechanical circulatory support of the failing heart in pediatrics is a new and rapidly developing field world-wide.

Case of hypercalcemia secondary to hypervitaminosis a in a 6-year-old boy with autism.

Vitamin A intoxication secondary to over-the-counter nutritional supplements and from its use in acne treatment has been described. However, there have been very few case reports of chronic hypervitaminosis A leading to hypercalcemia in the pediatric population. This paper describes a boy with hypercalcemia secondary to chronic vitamin A intoxication in the context of vitamin A usage for therapy of autism.

Mild traumatic brain injury in children.

Head injury occurs frequently in childhood and results in approximately 500,000 emergency department visits and over $1 billion in costs annually. Nearly 75% of these children are ultimately diagnosed with mild traumatic brain injury (MTBI), a misnomer because many will have radiographically identified intracranial injuries and long-term consequences. Identification of the brain at risk and prevention of secondary injury is associated with the largest reduction in head trauma morbidity and mortality.

Radiation therapy for pilocytic astrocytomas of childhood.

Purpose: Though radiation therapy is generally considered the most effective treatment for unresectable pilocytic astrocytomas in children, there are few data to support this claim. To examine the efficacy of radiation therapy for pediatric pilocytic astrocytomas, we retrospectively reviewed the experience at our institution.

Methods And Materials: Thirty-five patients 18 years old or younger with unresectable tumors and without evidence of neurofibromatosis have been treated since 1982.

Hypoplastic left heart associated with scimitar syndrome.

A child with the unique combination of hypoplastic left heart syndrome (HLHS) and scimitar syndrome is presented. Her HLHS was diagnosed in utero, and her scimitar syndrome was discovered during her immediate newborn period. She underwent a successful Norwood operation complicated by supraventricular tachycardia given her Wolf-Parkinson-White syndrome.

Biventricular assist devices as a bridge to heart transplantation in small children.

Background: Experience with the use of biventricular assist device (BiVAD) support to bridge small children to heart transplantation is limited.

Methods And Results: We used BIVAD support (Berlin EXCOR) in 9 pediatric heart transplant candidates from 4/05 to 7/07. The median patient age was 1.

Genital trauma in children and adolescents.

Traumatic wounds of the female genitalia include accidental straddle injuries or impalement, chemical or thermal burns, insufflation injuries, blunt trauma, or crush injuries. Children and adolescents may be victims of rape, sexual abuse, and female genital mutilation. Information is provided on epidemiology, pathophysiology, and management.

Atrial arrhythmia surgery in congenital heart disease.

Atrial reentrant tachycardias are a common source of morbidity in children with significant structural heart disease, especially following cardiac surgery. Preexisting atrial geometry combined with the hemodynamic effect of a congenital cardiac defect and electrophysiological alterations caused by surgical lesions can create large anatomic-functional barriers to conduction, allowing reentrant wavefronts to flourish. Elucidation of the genesis of reentrant arrhythmias in children has led to catheterization and surgical therapies.

Neonatal subgaleal hematoma: presentation and outcome--radiological findings and factors associated with mortality.

To describe the presentation and outcome of infants who develop subgaleal hematoma (SGH), we compared perinatal factors, clinical and head imaging findings, and outcome in a cohort (N = 34) of all infants admitted to Saint Louis Children's Hospital neonatal intensive care unit with SGH from January 1991 to June 2003. All except three of the infants admitted with SGH had instrumental deliveries (31 of 34; 91.2%): 21 vacuum, eight vacuum followed by forceps, two forceps).

Rituximab: a promising therapy in systemic lupus erythematosus.

Several trials of new immunologic agents in systemic lupus erythematosus (SLE) have recently been undertaken. Rituximab, a chimeric antibody directed against CD20 on B lymphocytes, has emerged as a promising therapy. Based upon preliminary data, clinical efficacy of rituximab has been documented in both pediatric and adult-onset SLE patients.

Review: Accounting for prematurity in developmental assessment and the use of age-adjusted scores.

Objective: This review summarizes the literature to date concerning age adjustment in developmental assessment and illustrates relevant issues for clinicians and researchers in this area.

Methods: Pubmed, Medline, Premedline, and PsycInfo databases were used to search the following terms: assessment, prematurity, age adjustment, and adjusted age. Additional sources were obtained through the references listed in the primary articles.

Acute normovolemic hemodilution.

Patients and physicians continue to be motivated to find methods to reduce the use of allogeneic blood. Even though donor screening has increased the safety of donated blood products, autologous blood is the most desirable source of red cells during the perioperative period. The methods commonly used to obtain autologous blood during the perioperative period can be initiated prior to the operative procedure (autologous preoperative donation, acute normovolemic hemodilution) or during surgery (cell scavenging).

Matrilysin (MMP-7) expression in renal tubular damage: association with Wnt4.

Background: Matrilysin, a secreted matrix metalloproteinase and target gene of Wnt signaling, functions in epithelial repair and host defense, but no role in renal injury has been described.

Methods: Matrilysin expression was assessed in human kidney specimens by immunohistochemistry, and in experimental renal injury in mice by immunohistochemistry, Northern blotting, and RNase protection assays (RPA). A relationship to Wnt4, which is also induced in renal injury, was determined by RPA and in situ hybridization.

Performance evaluation of the Sysmex XT-2000i automated hematology analyzer.

The Sysmex XT-2000i automated hematology analyzer was evaluated at Saint Louis Children's Hospital (SLCH), St. Louis, MO, USA. Complete blood count results from the Sysmex XT-2000i were compared to results from the Sysmex XE-2100 for 114 pediatric and adult patient samples.

High dose weekly oral prednisone improves strength in boys with Duchenne muscular dystrophy.

Daily prednisone improves strength in boys with Duchenne muscular dystrophy, but side effects are almost universal. We used a different dosing regimen of prednisone to determine if benefit to boys with Duchenne muscular dystrophy might be maintained with fewer side effects. Twice weekly oral prednisone was given each Friday and Saturday (5mg/kg/dose).

Expression profiling of the developing mouse lung: insights into the establishment of the extracellular matrix.

We have undertaken a comprehensive gene expression profiling of the entire process of murine lung development using oligonucleotide-based microarrays. Our data reveals the expression pattern of approximately 11,000 genes throughout the morphologic stages of lung development. This includes known genes with unappreciated pulmonary expression and novel genes with undefined functions.

Childhood Migraine Headache Syndromes.

The treatment of migraine headache in children depends on the following: a) defining the underlying cause; b) the frequency of the attacks; and c) the severity of the disability produced by the pain. Any medication taken to relieve pain is most effective if taken at maximum dose at the onset of the headache. The dose should be the maximum recommended by weight or age.

Anti-tubulin autoantibodies in acquired demyelinating polyneuropathies.

Selective high-titer anti-tubulin autoantibodies are associated with Guillain-Barré syndrome and chronic inflammatory demyelinating polyneuropathy (CIDP). Low-titer anti-tubulin autoantibodies are a normal component of some human sera. Analysis of 7 human sera with monoclonal anti-tubulin autoantibodies showed that the epitopes recognized by these antibodies are within central, conserved regions of tubulin.