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PubMed2006

Management of a cadaveric orthotopic liver transplantation in a pediatric patient with complex congenital heart disease.

Pediatric orthotopic liver transplantations (OLT) are commonly performed nowadays. Two primary reasons for OLT in children are complications from either extrahepatic biliary atresia (EHBA) or inborn errors of metabolism. However, congenital liver disease may be associated with significant other congenital abnormalities. We present a case of a successful OLT in a pediatric patient with a history of EHBA, situs inversus, and complex congenital heart disease. The cardiac anomalies include dextrocardia, absence of the atrial septum (single atrium), single atrioventricular valve (a-v canal), and an incomplete ventricular septum. Prior surgery include a Kasai procedure for EHBA, banding of the proximal main pulmonary artery, and Broviac catheter placement. We present the anesthesia concerns and management for this complicated case.

Abnormalities, Multiple↗
PubMed2005

TNF promoter polymorphisms and modulation of growth retardation and disease severity in pediatric Crohn's disease.

OBJECTIVES: Delayed growth is common in pediatric Crohn's disease (CD). Multiple factors have been shown to affect growth in this situation, the most prominent being the presence and severity of inflammation and inadequate nutritional intake. Inflammation, anorexia, and weight loss are all manifestations of circulating TNF-alpha, which is elevated in CD. The ability to secrete TNF-alpha may be affected by polymorphisms in the TNF-alpha promoter. The aim of our study was to determine whether growth retardation and disease severity in pediatric onset CD are affected by TNF promoter genotype. METHODS: Genotyping for TNF-alpha and NOD2/CARD15 single nucleotide polymorphisms was performed in 87 patients with detailed growth records. Parameters including disease location and disease severity were recorded, and the effect of these polymorphisms on Z-scores for height and weight at disease onset and during follow-up were analyzed. RESULTS: Lower age of onset was linked to more height retardation, while the presence of colonic disease and the absence of ileal disease were more likely to predict the absence of growth retardation. The presence of two polymorphisms thought to decrease circulating TNF-alpha was associated with higher mean Z-scores for height and a trend toward less growth retardation. Two other polymorphisms were modestly associated with disease severity. CONCLUSION: Polymorphisms in the TNF-alpha promoter may independently modulate growth and disease severity in pediatric onset CD. The effect of these polymorphisms does not appear to be mediated via weight loss, and is relatively modest.

Adenine↗
PubMed1989

Compliance with treatment regimens for pediatric rheumatic diseases.

Children with pediatric rheumatic diseases (PRDs) are required to comply with long-term treatment regimens that may not have immediate beneficial effects or may be required primarily to prevent further morbidity. As with other chronic disease regimens, compliance can be a significant problem that interferes with treatment efficacy and adequate clinical outcomes. This paper reviews (1) the prevalence and types of compliance problems in the management of PRDs; (2) patient/family, disease, and regimen factors that may be associated with treatment noncompliance; and (3) strategies for improving compliance with regimens for PRDs.

Adolescent↗
PubMed1998

Immunization guidelines for pediatric renal disease.

It is imperative that pediatric nephrologists monitor the immunization status of pediatric chronic renal insufficiency, dialysis and transplantation patients closely to reduce the risk of vaccine-preventable disease. Pediatric patients with chronic renal insufficiency and those on dialysis should receive all the standard immunizations according to the schedule as deliniated by the Red Book. In addition to these standard vaccines, these patients will also benefit from influenza and pneumococcal vaccine. Pediatric renal transplant recipients should also be immunized with standard and special vaccines; however, all live viral vaccines should be avoided in this population. Because patients with renal disease may not respond optimally to all immunizations, it is important to study antibody response to MMR and varicella in patients before transplantation. If these patients are unprotected, they should be immunized before transplantation. It seems that pediatric dialysis and transplantation patients may not respond optimally to hepatitis B vaccine. Therefore, if at all possible, this vaccine should be administered before these therapies. Doubling the recommended dose of hepatitis B vaccine may improve response. Antibody levels to hepatitis B should be monitored every other year, and this vaccine should be readministered when the antibody level decreases to less than 10 mIU/mL. Hopefully the morbidity and mortality associated with vaccine-preventable disease can be reduced in this population by ensuring that pediatric patients with chronic renal disease are adequately immunized.

Adolescent↗
PubMed1996

Congenital and pediatric interstitial disease.

Congenital and pediatric interstitial lung disease (ILD) is set in the context of the maturing lung and immune system, and thus differs completely from its adult equivalents in presentation, therapy, and outcome. We first establish the background by briefly reviewing normal maturational changes and then describe recent advances in diagnosis. We then highlight three specific topics: drugs and the lung (treatment and iatrogenic ILD); the histiocytic disorders of the lung; and congenital ILD (specifically congenital surfactant protein deficiency).

Adult↗
PubMed1995

Use of Tl-201 to detect untreated pediatric Hodgkin disease.

PURPOSE: To evaluate the usefulness of thallium-201 scintigrams in depiction of supradiaphragmatic lymphadenopathy associated with newly diagnosed Hodgkin disease in pediatric patients. MATERIALS AND METHODS: Thallium-201 and gallium-67 scans of the chest and neck were obtained in 33 consecutive patients. Mediastinal, cervical, supraclavicular, and axillary lymph node activity was evaluated by independent raters, and results were compared in a blinded fashion with those on chest computed tomographic (CT) scans. RESULTS: Gallium and thallium scans demonstrated similar accuracy in depiction of CT-defined mediastinal masses. The kappa statistic indicated moderate to strong agreement with CT results. Similarly, there was no statistically significant difference between the accuracy of thallium and gallium scans in depiction of extramediastinal lymphadenopathy. CONCLUSION: Thallium scintigraphy is effective for depiction of lymphadenopathy in pediatric patients with untreated Hodgkin disease. Serial thallium studies may provide an alternative to gallium scans in monitoring response to treatment.

Adolescent↗
PubMed2003

Safety and steroid-sparing experience using infliximab for Crohn's disease at a pediatric inflammatory bowel disease center.

OBJECTIVES: The published experience using infliximab (Remicade, Centocor, Malvern, PA) for the treatment of pediatric Crohn's disease is limited but suggests utility in the treatment of refractory disease. Experience using infliximab at a large pediatric center is reviewed. METHODS: A retrospective review of all infliximab infusions administered to patients with Crohn's disease (CD) was undertaken. Data were obtained from database and pharmacy records. Chart review and interviews with physicians, patients, and families were used to obtain missing data. RESULTS: A total of 432 infusions were administered to 82 patients (34 female and 48 male) with CD. The number of infusions each patient received ranged from one to 18, with a mean of 5.3 (SD 4.6) and median of 3. Of 33 patients, 19 (57.6%) became independent and remained free of corticosteroids. There was a statistically significant difference in the steroid dose between 0 and 4 wk and 0 and 8 wk. In all, 23 infusion reactions occurred (5.3%). Three patients developed herpes zoster, and one developed Listeria monocytogenes meningitis. No patients were documented to have delayed hypersensitivity reactions or malignancies. CONCLUSIONS: Infliximab is safe and effective for treating pediatric patients with CD. A steroid-sparing effect was demonstrated. The most common adverse reaction to infliximab was infusion reaction. These reactions did not preclude further use of the agent. Serious infections were seen in a small number of patients.

Adolescent↗
PubMed2001

Incidence of pediatric rheumatic diseases in a regional population in Austria.

OBJECTIVE: To establish a population based disease registry for pediatric rheumatology in a defined population of Austria; to describe the demographic and diagnostic classification of children referred to pediatric rheumatology clinics; and to estimate the incidence of pediatric rheumatic diseases in Eastern Austria. METHODS: For 2 years (1997-98) all pediatric rheumatology centers in the area contributed data on all new cases to a prospective multicenter patient registry. Diagnostic criteria defined the rheumatic disease cases, determined by a pediatric rheumatologist, and record linkage was carried out to avoid duplication of subjects. RESULTS: Rheumatic conditions were diagnosed in 107 subjects. Juvenile rheumatoid arthritis (JRA) was the most frequently encountered rheumatic condition (49.5%), followed by spondyloarthropathy (SpA, 33.6%) and systemic lupus erythematosus (SLE, 5.6%). The mean annual incidence of JRA, SpA, and SLE among children referred to pediatric rheumatology centers was 4.28, 2.9, and 0.48 per 100,000 children at risk, respectively. CONCLUSION: Establishment of a population based disease registry led to collection of descriptive epidemiologic data on a defined regional cohort of children with rare disorders. Our registry will provide data on pediatric rheumatic diseases in a European population and will allow more accurate comparisons between populations for future research. Our data also indicate that more resources should be designated for the care of pediatric rheumatic diseases in view of the relatively high incidences of these diseases.

Adolescent↗
PubMed2000

Pediatric sinonasal imaging: normal anatomy and inflammatory disease.

Pediatric sinonasal anatomy changes and develops from birth to adolescence. This article elucidates the normal anatomy and patterns of development in the pediatric population. Issues in pediaric sinusitis include indications for imaging, the nonspecificity of sinus opacification, and the importance of clinical information. Sinonasal physiology is briefly discussed to offer insight into the interpretation of radiographic findings. Cystic fibrosis, polyps, and choanal atresia, representing the spectrum of common pediatric sinonasal inflammatory disorders are illustrated, and the spectrum of orbital and intracranial complications of sinusitis is reviewed.

Adolescent↗
PubMed1989

Cerebral thromboembolic disease in pediatric and adult inflammatory bowel disease: case report and review of the literature.

Cerebral and retinal vascular disease are rare complications of inflammatory bowel disease. Most reports are of adult patients, with only seven instances described in children. The eighth case, a 14-year-old boy with ulcerative colitis and cerebral venous thrombosis is reported in whom the diagnosis was confirmed by magnetic resonance imaging. The adult and pediatric literature is also reviewed.

Adolescent↗
PubMed1991

Gallium-67 scans as an adjunct to computed tomography scans for the assessment of a residual mediastinal mass in pediatric patients with Hodgkin's disease. A Pediatric Oncology Group study.

This study determines the utility of gallium-67 (Ga-67) scintigraphs as an adjunct to computed tomography (CT) scans for the assessment of residual mediastinal masses in children and adolescents with advanced-stage Hodgkin's disease. At diagnosis 42 patients with CT scan-documented mediastinal disease had a Ga-67 scan performed. Thirty-four of 42 patients (81%) had gallium-avid mediastinal lesions, whereas in eight (19%), the Ga-67 scan was negative. At the completion of eight cycles of therapy of Mustargen (mechlorethamine), Oncovin (vincristine), procarbazine, prednisone (MOPP) alternating with doxorubicin, bleomycin, vinblastine, dacarbazine (ABVD), 21 of 34 patients with initially positive Ga-67 scans had them repeated; 18 of 21 converted to negative results, and three remained positive. In 11 of 18 patients, the loss of gallium avidity was consistent with a negative mediastinal CT scan. In seven, although the gallium scan was negative, the CT scan remained positive; all seven patients had a mediastinal biopsy of suspected residual disease and in all seven the biopsy results were negative for Hodgkin's disease. These preliminary results in a small cohort of patients demonstrate that Ga-67 scans may be of benefit in evaluating residual mediastinal masses in patients with Hodgkin's disease.

Adolescent↗