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El Saleeby C.M.,Harvard University | El Saleeby C.M.,University of Memphis | El Saleeby C.M.,nheur Childrens Medical Center | El Saleeby C.M.,The Childrens Foundation Research Center | And 8 more authors.
Journal of Pediatrics | Year: 2010

Objective: To examine whether genetic variations within the surfactant protein A2 (SP-A2) gene are associated with respiratory syncytial virus (RSV) disease severity in infected children. Study design: Naturally infected children aged ≤24 months were prospectively enrolled in 3 RSV seasons. SP-A2 genotyping was performed. Independent clinical predictors of disease severity were analyzed. The association of SP-A2 genetic diversity and disease severity was tested by using multivariate logistic regression models and 4 levels of disease gradation as outcome measures. Results: Homozygosity of the 1A0 allele was protective against hospitalization (odds ratio [OR] = 0.15, P = .0010). This remained significant in African American patients (OR = 0.24, P = .042) and Caucasian patients (OR = 0.05, P = .021) after adjustment for other co-variates. Hospitalized children with the 1A2 allele demonstrated significant protection from severe disease with univariate analyses, but only a trend for protection with multivariate analyses. Patients homozygous or heterozygous for an asparagine at amino acid position 9 were twice or more likely to need intensive care unit admission (OR = 2.15, P = .022), require intubation (OR = 3.04, P = .005), and have a hospitalization lasting ≥4 days (OR = 1.89, P = .02) compared with children homozygous for a threonine at this position. Conclusions: SP-A2 polymorphisms are associated with the severity of RSV infection in infants. © 2010 Mosby, Inc. All rights reserved.


Jabara H.H.,Childrens Hospital | Jabara H.H.,Harvard University | Ohsumi T.,Massachusetts General Hospital | Ohsumi T.,Harvard University | And 24 more authors.
Journal of Allergy and Clinical Immunology | Year: 2013

Background: Combined immunodeficiency (CID) is characterized by severe recurrent infections with normal numbers of T and B lymphocytes but with deficient cellular and humoral immunity. Most cases are sporadic, but autosomal recessive inheritance has been described. In most cases, the cause of CID remains unknown. Objective: We wanted to identify the genetic cause of CID in 2 siblings, the products of a first-cousin marriage, who experienced recurrent bacterial and candidal infections with bronchiectasis, growth delay, and early death. Methods: We performed immunologic, genetic, and biochemical studies in the 2 siblings, their family members, and healthy controls. Reconstitution studies were performed with T cells from mucosa-associated lymphoid tissue lymphomatranslocation gene 1-deficient (Malt1-/-) mice. Results: The numbers of circulating T and B lymphocytes were normal, but T-cell proliferation to antigens and antibody responses to vaccination were severely impaired in both patients. Whole genome sequencing of 1 patient and her parents, followed by DNA sequencing of family members and healthy controls, showed the presence in both patients of a homozygous missense mutation in MALT1 that resulted in loss of protein expression. Analysis of T cells that were available on one of the patients showed severely impaired IkBa degradation and IL-2 production after activation, 2 events that depend on MALT1. In contrast to wild-type human MALT1, the patients' MALT1 mutant failed to correct defective nuclear factor-κB activation and IL-2 production in MALT1-deficient mouse T cells. Conclusions: An autosomal recessive form of CID is associated with homozygous mutations in MALT1. If future patients are found to be similarly affected, they should be considered as candidates for allogeneic hematopoietic cell transplantation. © 2013 American Academy of Allergy, Asthma &Immunology.


Sansgiri R.K.,nheur Childrens Medical Center | Neel M.D.,St Jude Childrens Research Hospital | Soto-Fourier M.,St Jude Childrens Research Hospital | Kaste S.C.,St Jude Childrens Research Hospital | Kaste S.C.,University of Memphis
American Journal of Roentgenology | Year: 2012

OBJECTIVE. Osteonecrosis is a potential complication of glucocorticoid chemotherapy in children surviving leukemia. Early diagnosis may allow effective interventions to minimize or ameliorate joint deterioration and obviate surgical intervention. We investigated the significance of MRI signal changes that precede the currently recognized "double-line"changes, which are considered pathognomic of osteonecrosis. MATERIALS AND METHODS. We retrospectively reviewed MRI scans acquired during prospective screening and follow-up of pediatric patients with leukemia for osteonecrosis. RESULTS. Of 481 patients, we identified 21 cases (4.3%; 12 boys; median age at leukemia diagnosis, 12.8 years) with subtle poorly defined geographically delineated MRI signal abnormalities in knees or hips, or both, that progressed over a median of 4 months (range, 1.6-18.5 months) to florid MRI signs of osteonecrosis. Articular surface collapse developed in three hips (two patients) and three knees (three patients). Three patients subsequently underwent surgical intervention (one bilateral total hip arthroplasty and one bilateral and one unilateral hip core decompression). The median duration of follow-up was 27 months (range, 1.9-90.7 months). CONCLUSION. The MRI signal abnormalities described here appear to herald extensive osteonecrosis and precede the typical MRI findings of osteonecrosis previously reported in the literature. © American Roentgen Ray Society.


Sawyer J.R.,University of Memphis | Ivie C.B.,University of Memphis | Huff A.L.,nheur Childrens Medical Center | Wheeler C.,nheur Childrens Medical Center | And 3 more authors.
Journal of Pediatric Orthopaedics | Year: 2010

BACKGROUND: The purpose of this review was to determine when and why pediatric patients with cast complaints return to the emergency room (ER). If this could be determined, educational and treatment strategies may help decrease the number of these visits and the cost of care. METHODS: Retrospective chart review of patients initially seen in a busy urban pediatric orthopaedic clinic identified those who had an ER visit because of a cast-related problem over a 5-year period. Patients were included only if they were seen for their initial visit and cast application in our fracture clinic. RESULTS: Of 168 ER visits made by 155 children treated with cast immobilization, 29% were because of a wet cast; 10%, a damaged cast; 23%, a tight cast; 13%, a loose cast; and 10%, pain. In addition to wet and damaged casts, compliance issues included a missed clinic appointment (5%) and being told by medical personnel to return to the ER for a cast check (8%). Several groups with a high risk for return to the ER were identified: the younger the patient, the more likely that the cast was too loose or wet, and the older the patient, the more likely the cast was too tight. Cast type also played a role: a significantly higher rate of return to the ER was found with long arm, long leg, and hand casts. There were no major complications and no child required hospitalization. CONCLUSIONS: All 168 ER visits required only a cast change or reassurance, which could have been done during regular fracture clinic hours; no child required hospitalization or surgery. From these results, a program has been instituted that includes patient education, triage, and follow-up in our fracture clinics to not only improve the quality of patient care but to decrease the financial burden on physicians and the healthcare system. LEVEL OF EVIDENCE: Economic and decision analysis, Level III. Copyright © 2010 by Lippincott Williams & Wilkins.


Reed B.N.,University of North Carolina at Chapel Hill | Caudle K.E.,University of Tennessee Health Science Center | Caudle K.E.,nheur Childrens Medical Center | Rogers P.D.,Le Bonheur Childrens Medical Center
Annals of Pharmacotherapy | Year: 2010

OBJECTIVE: To evaluate the literature regarding the use of fluconazole prophylaxis in high-risk neonates. DATA SOURCES: Literature was accessed through MEDLINE (February 2001-August 2009) using the search terms fluconazole and prophylaxis, with limits for age group (ie, birth-18 y). Reference citations from identified articles were also reviewed. DATA SELECTION AND DATA EXTRACTION: All prospective and retrospective studies in English identified from MEDLINE were evaluated. DATA SYNTHESIS: Critically ill neonates possess a number of risk factors that predispose them to fungal colonization with Candida spp. In many cases, colonization may progress to invasive systemic infections despite efforts aimed at early diagnosis and treatment. Because of its success among immunocompromised patients, fluconazole prophylaxis has been suggested as a possible approach for reducing the rates of both colonization and invasive fungal infections among at-risk neonates. To date, 4 prospective randomized controlled trials and 8 retrospective cohort studies have examined fluconazole prophylaxis in neonates. Although fluconazole prophylaxis appears to reduce the rates of colonization and invasive fungal infections, no trial in this review was able to demonstrate a significant difference in long-term morbidity or mortality. Concerns also remain regarding the adverse effects associated with prolonged exposure to fluconazole therapy. Lack of standardized study designs and treatment regimens also limit widespread recommendation for the use of fluconazole prophylaxis in clinical practice. CONCLUSIONS: While it may be beneficial for critically ill neonates with certain predisposing risk factors (eg, central venous access, sustained exposure to broad-spectrum antibiotics, or units with significantly high incidence of invasive fungal infections), existing research does not support the use of fluconazole prophylaxis based on birth weight or gestational age alone. Multifactor analysis evaluating the effect of fluconazole prophylaxis is necessary to establish which neonates would benefit from this practice.


Tendulkar R.D.,Cleveland Clinic | Pai Panandiker A.S.,St Jude Childrens Research Hospital | Wu S.,St Jude Childrens Research Hospital | Kun L.E.,St Jude Childrens Research Hospital | And 3 more authors.
International Journal of Radiation Oncology Biology Physics | Year: 2010

Purpose: To report the outcome using radiation therapy (RT) for pediatric patients with high-grade spinal cord tumors. Methods and Materials: A retrospective chart review was conducted that included 17 children with high-grade spinal cord tumors treated with RT at St. Jude Children's Research Hospital between 1981 and 2007. Three patients had gross total resection, 11 had subtotal resection, and 3 underwent biopsy. The tumor diagnosis was glioblastoma multiforme (n = 7), anaplastic astrocytoma (n = 8), or anaplastic oligodendroglioma (n = 2). Seven patients received craniospinal irradiation (34.2-48.6 Gy). The median dose to the primary site was 52.2 Gy (range, 38-66 Gy). Results: The median progression-free and overall survivals were 10.8 and 13.8 months, respectively. Local tumor progression at 12 months (79% vs. 30%, p = 0.02) and median survival (13.1 vs. 27.2 months, p = 0.09) were worse for patients with glioblastoma multiforme compared with anaplastic astrocytoma or oligodendroglioma. The median overall survival was shorter for patients when failure included neuraxis dissemination (n = 8) compared with local failure alone (n = 5), 9.6 vs. 13.8 months, p = 0.08. Three long-term survivors with World Health Organization Grade III tumors were alive with follow-up, ranging from 88-239 months. Conclusions: High-grade spinal cord primary tumors in children have a poor prognosis. The propensity for neuraxis metastases as a component of progression after RT suggests the need for more aggressive therapy. © 2010 Elsevier Inc.


Tran N.P.,nheur Childrens Medical Center | Vickery J.,nheur Childrens Medical Center | Blaiss M.S.,nheur Childrens Medical Center | Blaiss M.S.,University of Tennessee Health Science Center
Allergy, Asthma and Immunology Research | Year: 2011

Rhinitis is a global problem and is defined as the presence of at least one of the following: congestion, rhinorrhea, sneezing, nasal itching, and nasal obstruction. The two major classifications are allergic and nonallergic rhinitis (NAR). Allergic rhinitis occurs when an allergen is the trigger for the nasal symptoms. NAR is when obstruction and rhinorrhea occurs in relation to nonallergic, noninfectious triggers such as change in the weather, exposureto caustic odors or cigarette smoke, barometric pressure differences, etc. There is a lack of concomitant allergic disease, determined by negativeskin prick test for relevant allergens and/or negative allergen-specific antibody tests. Both are highly prevalent diseases that have a significant economic burden on society and negative impact on patient quality of life. Treatment of allergic rhinitis includes allergen avoidance, antihistamines (oral and intranasal), intranasal corticosteroids, intranasal cromones, leukotriene receptor antagonists, and immunotherapy. Occasional systemic corticosteroidsand decongestants (oral and topical) are also used. NAR has 8 major subtypes which includes nonallergic rhinopathy (previously known as vasomotor rhinitis), nonallergic rhinitis with eosinophilia, atrophic rhinitis, senile rhinitis, gustatory rhinitis, drug-induced rhinitis, hormonal-induced rhinitis, and cerebral spinal fluid leak. The mainstay of treatment for NAR are intranasal corticosteroids. Topical antihistamines have also been found to be efficacious. Topical anticholinergics such as ipratropium bromide (0.03%) nasal spray are effective in treating rhinorrhea symptoms. Adjunct therapy includes decongestants and nasal saline. Investigational therapies in the treatment of NAR discussed include capsaicin, silver nitrate, and acupuncture. © 2011, The Korean Academy of Asthma, Allergy and Clinical Immunology.


Dori Y.,Children's Hospital of Philadelphia | Sathanandam S.,nheur Childrens Medical Center | Glatz A.C.,Children's Hospital of Philadelphia | Gillespie M.J.,Children's Hospital of Philadelphia | Rome J.J.,Children's Hospital of Philadelphia
Catheterization and Cardiovascular Interventions | Year: 2014

Objectives The goal of this report is to describe a percutaneous approach to rerouting hepatic venous return in patients who developed progressive cyanosis due to unilateral pulmonary arteriovenous malformations (PAVM) after the total cavopulmonary connection (TCPC) operation. Background Unilateral PAVM can develop in patients after TCPC operation when there is unequal distribution of hepatic venous return between the two lungs. This often results in progressive cyanosis and the need for surgical re-intervention. A percutaneous based approach for rerouting hepatic venous return has never been described. Methods We retrospectively reviewed the clinical data on four patients who underwent percutaneous rerouting procedures. One patient with a misaligned TCPC underwent realignment of the circuit with a bare metal stent. In three patients a combination of bare metal and covered stents were needed to achieve the desired results. Results The rerouting procedures were successful in all patients with significant improvement in oxygen saturation from a median of 75% (range 55-80%) to a median of 90% (range 84-92%) (P = 0.02). There were no recorded short term or intermediate term complications with maximum follow-up time of 43 months. Conclusions Percutaneous rerouting of hepatic venous flow is feasible and should be considered when a surgical approach is not possible; this strategy may serve as a viable alternative to complex operative approaches in select cases. Furthermore studies are needed to determine the long-term efficacy of this procedure. © 2013 Wiley Periodicals, Inc.


PubMed | nheur Childrens Medical Center
Type: Journal Article | Journal: Allergy, asthma & immunology research | Year: 2011

RHINITIS IS A GLOBAL PROBLEM AND IS DEFINED AS THE PRESENCE OF AT LEAST ONE OF THE FOLLOWING: congestion, rhinorrhea, sneezing, nasal itching, and nasal obstruction. The two major classifications are allergic and nonallergic rhinitis (NAR). Allergic rhinitis occurs when an allergen is the trigger for the nasal symptoms. NAR is when obstruction and rhinorrhea occurs in relation to nonallergic, noninfectious triggers such as change in the weather, exposure to caustic odors or cigarette smoke, barometric pressure differences, etc. There is a lack of concomitant allergic disease, determined by negative skin prick test for relevant allergens and/or negative allergen-specific antibody tests. Both are highly prevalent diseases that have a significant economic burden on society and negative impact on patient quality of life. Treatment of allergic rhinitis includes allergen avoidance, antihistamines (oral and intranasal), intranasal corticosteroids, intranasal cromones, leukotriene receptor antagonists, and immunotherapy. Occasional systemic corticosteroids and decongestants (oral and topical) are also used. NAR has 8 major subtypes which includes nonallergic rhinopathy (previously known as vasomotor rhinitis), nonallergic rhinitis with eosinophilia, atrophic rhinitis, senile rhinitis, gustatory rhinitis, drug-induced rhinitis, hormonal-induced rhinitis, and cerebral spinal fluid leak. The mainstay of treatment for NAR are intranasal corticosteroids. Topical antihistamines have also been found to be efficacious. Topical anticholinergics such as ipratropium bromide (0.03%) nasal spray are effective in treating rhinorrhea symptoms. Adjunct therapy includes decongestants and nasal saline. Investigational therapies in the treatment of NAR discussed include capsaicin, silver nitrate, and acupuncture.


PubMed | nheur Childrens Medical Center
Type: Journal Article | Journal: AJR. American journal of roentgenology | Year: 2012

Osteonecrosis is a potential complication of glucocorticoid chemotherapy in children surviving leukemia. Early diagnosis may allow effective interventions to minimize or ameliorate joint deterioration and obviate surgical intervention. We investigated the significance of MRI signal changes that precede the currently recognized double-line changes, which are considered pathognomic of osteonecrosis.We retrospectively reviewed MRI scans acquired during prospective screening and follow-up of pediatric patients with leukemia for osteonecrosis.Of 481 patients, we identified 21 cases (4.3%; 12 boys; median age at leukemia diagnosis, 12.8 years) with subtle poorly defined geographically delineated MRI signal abnormalities in knees or hips, or both, that progressed over a median of 4 months (range, 1.6-18.5 months) to florid MRI signs of osteonecrosis. Articular surface collapse developed in three hips (two patients) and three knees (three patients). Three patients subsequently underwent surgical intervention (one bilateral total hip arthroplasty and one bilateral and one unilateral hip core decompression). The median duration of follow-up was 27 months (range, 1.9-90.7 months).The MRI signal abnormalities described here appear to herald extensive osteonecrosis and precede the typical MRI findings of osteonecrosis previously reported in the literature.

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