The use of fluid boluses to safely perform extracorporeal photopheresis (ECP) in low-weight children: a novel procedure.

Apheresis procedures in small children are technically challenging and require special planning with attention to extracorporeal volume. Discontinuous procedures such as extracorporeal photopheresis (ECP) require additional consideration. Alternative methods to perform ECP have been utilized in small children that require manipulation of mononuclear cells outside the standard closed-loop system.

Pedicled thoracodorsal artery perforator flap in breast reconstruction: clinical experience.

Background: The thoracodorsal artery perforator (TDAP) flap has been described for reconstruction of the head and neck, trunk and extremities. Yet, its use as a pedicled flap in breast reconstruction has not gained wide popularity and has not been widely documented, especially not for complete breast reconstruction or in combination with expanders or permanent implants. The authors present their clinical experience with the thoracodorsal artery perforator flap in breast reconstruction.

Intraperitoneal bladder rupture as an isolated manifestation of nonaccidental trauma in a child.

Nonaccidental trauma is a significant cause of morbidity and mortality in children. We describe a case of a child who presented with intraperitoneal bladder rupture after sustaining blunt abdominal trauma. As per confession, the injury was apparently the result of the child being forcibly pulled into her caretaker's knee while the child had a full bladder.

Recent developments related to the laboratory diagnosis of primary immunodeficiency diseases.

Purpose Of Review: The rapid increases in newly recognized primary immunodeficiency diseases (PIDs), including their clinical, genetic and laboratory-associated abnormalities, make staying abreast of the latest developments a challenge. This review provides an overview of current information directly and indirectly related to the laboratory diagnosis of PIDs.

Recent Findings: The latest classification and several prevalence studies provide the framework for understanding the breadth, categories and incidence rates of over 120 recognized disease entities.

Searching for the cause of Kawasaki disease--cytoplasmic inclusion bodies provide new insight.

Kawasaki disease (KD) has emerged as the most common cause of acquired heart disease in children in the developed world. The cause of KD remains unknown, although an as-yet unidentified infectious agent might be responsible. By determining the causative agent, we can improve diagnosis, therapy and prevention of KD.

RNA-containing cytoplasmic inclusion bodies in ciliated bronchial epithelium months to years after acute Kawasaki disease.

Background: Kawasaki Disease (KD) is the most common cause of acquired heart disease in children in developed nations. The KD etiologic agent is unknown but likely to be a ubiquitous microbe that usually causes asymptomatic childhood infection, resulting in KD only in genetically susceptible individuals. KD synthetic antibodies made from prevalent IgA gene sequences in KD arterial tissue detect intracytoplasmic inclusion bodies (ICI) resembling viral ICI in acute KD but not control infant ciliated bronchial epithelium.

New strategies and decision making in the management of neuromuscular scoliosis.

The outcome of spinal fusion in neuromuscular scoliosis demonstrates a consistently high satisfaction rate among parents and caretakers. It is somewhat more difficult to determine the functional benefits in individual patients, especially in those with the most severe involvement. Even in those children, a predicted 70% survival rate at 11 years has been documented following surgery.

Etiology of stridor in infants.

Objectives: We undertook to identify data that facilitate determination of an accurate diagnosis of the cause of stridor in infants and to develop a framework to conceptualize the problem.

Methods: We reviewed medical records of patients less than 1 year of age with the presenting symptom of stridor who were initially evaluated in the outpatient setting of a tertiary children's hospital. Infants with obvious congenital syndromes, cerebral palsy, or hypotonia were excluded.

Evolving anatomic and electrophysiologic considerations associated with Fontan conversion.

The principles of Fontan conversion with arrhythmia surgery are to restore the cardiac anatomy by converting the original atriopulmonary connection to a total cavopulmonary artery extracardiac connection and treat the underlying atrial arrhythmias. Successful outcomes of this procedure are dependent on a thorough understanding of several factors: the patient's fundamental diagnosis of single-ventricle anatomy, the resultant cardiac configuration from the original atriopulmonary Fontan connection, right atrial dilatation that leads to atrial flutter or fibrillation, and associated congenital cardiac anomalies. The purpose of this article is to present some of the more challenging anatomic and electrophysiologic problems we have encountered with Fontan conversion and arrhythmia surgery and the innovative solutions we have used to treat them.

New developments in the search for the etiologic agent of Kawasaki disease.

Purpose Of Review: The aim of this article is to review recent developments in the search for the etiologic agent of Kawasaki disease.

Recent Findings: Two recently proposed theories of Kawasaki disease etiology, the toxic shock syndrome toxin-1 hypothesis and the coronavirus NL-63 hypothesis, have been studied extensively and have been disproven. Surprisingly, IgA plasma cells infiltrate inflamed tissues in acute Kawasaki disease, including the coronary artery, and are oligoclonal, or antigen-driven.

Cloning the arterial IgA antibody response during acute Kawasaki disease.

Kawasaki disease (KD) is the most common acquired cardiac disease in children in developed nations. The etiology of KD is unknown but likely to be a ubiquitous microbial agent. Previously, we showed that oligoclonal IgA plasma cells infiltrate coronary arteries and other inflamed tissues in acute KD.

Development of a de novo cerebral arteriovenous malformation in a child with sickle cell disease and moyamoya arteriopathy. Case report.

The authors report the unique case of a 6-year-old African-American girl with sickle cell disease (SCD) and an associated moyamoya arteriopathy who developed a de novo arteriovenous malformation (AVM) of the cerebral circulation. Based on preoperative cerebral angiography, computerized tomography angiography, and magnetic resonance imaging, the incidentally discovered lesion was originally thought to be a direct arteriovenous fistula with an associated varix. At surgery, however, a 1.

Intracranial vascular abnormalities in patients with Alagille syndrome.

Objectives: To define the spectrum of intracranial events and cerebrovascular lesions in patients with Alagille syndrome using magnetic resonance imaging with angiography of the head and medical histories and to correlate the presence of lesions with the clinical outcome of bleeding or ischemic intracranial events.

Methods: 26 patients with Alagille syndrome underwent magnetic resonance imaging with angiography of the head; 22 had no symptoms and underwent study for screening purposes and 4 were symptomatic with neurologic changes. The results of studies and the history of ischemic intracranial events were reviewed.

Pertussis immunization in the global pertussis initiative North American region: recommended strategies and implementation considerations.

In North America, children currently receive 5 doses of a combined diphtheria-tetanus-acellular pertussis vaccine between the ages of 2 months and 6 years. Although this schedule has reduced the incidence of childhood pertussis, it has not led to the development of herd immunity in the total population, largely because pertussis immunity wanes with time. The time course over which immunity wanes is uncertain; however, high pertussis antibody titers in adolescents and adults indicate unrecognized infection in these groups.

Pasteurella aerogenes hamster bite peritonitis.

Pasteurella multocida has been reported to cause peritoneal dialysis-associated peritonitis after a cat bite or scratch to the catheter. We report a teenager with hamster bite peritonitis caused by P. aerogenes, an organism predominantly isolated from swine.

The history of Pediatric Infectious Diseases.

The history of Pediatric Infectious Diseases closely parallels the history of Pediatrics at least until the last century, because historically infections comprised the major causes of childhood morbidity and mortality, as they still do in the developing world. This history reviews developments in the field through the centuries and is written so that it does not overlap the contribution to this series by Baker and Katz entitled 'Childhood Vaccine Development in the United States.' Remarkable descriptions of selected pediatric infections existed long before the invention of printing, and early pediatric texts included many chapters devoted to various infections.

The etiology of neural tube defects: the role of folic acid.

Discussion: While the cause of neural tube defects in humans is considered to be multifactorial, it is apparent that folic acid can prevent 70% of open neural tube defects. Even in laboratory animals with known genetic defects, folic acid can prevent the genetic expression.

Conclusion: While some of the metabolic pathways for folic acid are known, the true effects of folic acid on closure of the neural tube have yet to be discovered.

The Chiari II malformation: cause and impact.

Introduction: It is the Chiari II malformation and its effects that determine the quality of life of the individual born with spina bifida.

Discussion: The cause of this malformation has been a source of debate for many years. Understanding the cause enables strategies for the management of problems created by this malformation to be developed.

Prevention and treatment of severe hemodynamic compromise in pediatric heart transplant patients.

Allograft rejection is a leading cause of severe hemodynamic compromise in pediatric heart transplant patients. A triple-drug immunosuppression regimen, which includes a calcineurin inhibitor, antiproliferative agent, and corticosteroid, suppresses the immune system at multiple different levels for optimal graft protection while minimizing the adverse effects of any one particular agent. Some pediatric centers also use induction therapy with anti-T cell antibodies immediately following transplantation as additional rejection prophylaxis.