Gurion R.,Rainbow Hospitals |
Lehman T.J.A.,Cornell University |
Moorthy L.N.,University of New Brunswick
International Journal of Inflammation | Year: 2012
Systemic juvenile idiopathic arthritis (sJIA) constitutes a small part of juvenile idiopathic arthritis (JIA), yet has a disproportionally higher rate of mortality. Despite being grouped under JIA, it is considered to be a multifactorial autoinflammatory disease. The objective of this paper is to review the epidemiology, pathogenesis, genetics, clinical manifestations, complications, therapy, prognosis, and outcome of sJIA. The presentation and clinical manifestations of sJIA have not changed much in the past several decades, but the collective understanding of the pathogenesis and the development of new targeted therapies (particularly the biologic agents) have transformed and improved the disease outcome for children with sJIA. © 2012 R. Gurion et al.
Bodas P.,Akron Children’s Hospital |
Rotz S.,Rainbow Hospitals
Journal of Pediatric Hematology/Oncology | Year: 2014
OVERVIEW: Stroke is a common sequela of sickle cell disease (SCD). Patients with SCD who undergo hematopoietic stem cell transplantation (HSCT) with successful engraftment will not experience sickling. This ameliorates one aspect of stroke risk; however, the significance of preexisting cerebrovascular abnormalities remains unclear. METHODS: We performed a literature search for neurological outcomes following HSCT for SCD. We searched for relevant neuroimaging and neurosurgical protocols. We identified 4 unique studies encompassing 196 patients. Of these, 81 had a history of a stroke, transient ischemic attack (TIA), cognitive dysfunction or cerebrovascular abnormalities identified by pretransplant neuroimaging, achieved stable engraftment, and had long-term follow-up. RESULTS: Of the 81 patients, 1 had peritransplant (10 days prior transplant to 50 days posttransplant) TIA. One had posttransplant TIA within 36 to 72 months. None had strokes. Forty-five underwent cerebral imaging at nonuniform intervals. Among this group, 32 (71%) had stable cerebrovascular abnormalities on imaging, 6 (13%) had improvement, and 7 (16%) showed worsening. CONCLUSIONS: Cerebrovascular abnormalities identified on neuroimaging may stabilize, improve, or worsen in patients after successful HSCT. Some patients may have neurological events such as TIA. Neurological outcomes in children with SCD post-HSCT have been inadequately studied. © 2014 by Lippincott Williams & Wilkins.
Griswold K.J.,Case Western Reserve University |
Fanaroff J.M.,Case Western Reserve University |
Fanaroff J.M.,Rainbow Hospitals
Pediatrics | Year: 2010
Before the delivery of a premature infant, a prenatal consultation between parents and physicians provides the opportunity to establish a trusting relationship and create a supportive environment for decision-making concerning neonatal resuscitation. The ideal consult enables physicians to educate parents about preterm delivery and potential outcomes for their infant while providing parents with the time to ask questions and express their values. The uncertainty that surrounds many decisions in the treatment and resuscitation of infants born at the limits of viability creates a situation in which joint responsibility for decision-making between parents and physicians is vital. In this review we examine ethical considerations regarding the resuscitation of infants born at the limits of viability and present the current policies established by the Neonatal Resuscitation Program and the American Academy of Pediatrics. The parental and physician perspectives regarding the consultation experience are presented also. Finally, a model for the prenatal consultation is introduced with suggestions for the incorporation of morbidity and mortality data as well as the structure and approach to discussion with parents with threatened preterm delivery.
Deakins K.M.,Rainbow Hospitals
Respiratory Care | Year: 2015
Asthma continues to be recognized as a well-known respiratory disease requiring complex management. Asthma is assessed and treated by clinicians across the continuum. The interest in evidence- based recommendations for diagnosis, treatment, and long-term management is ongoing and essential for aligning clinical practice with its changes. The purpose of this review is to provide updates from recent literature on asthma for clinicians. © 2015 Daedalus Enterprises.
Kerr D.S.,Rainbow Hospitals
Molecular Genetics and Metabolism | Year: 2010
While many treatments for mitochondrial electron transport (respiratory) chain disorders have been suggested, relatively few have undergone controlled clinical trials. This review focuses on the recent history of clinical trials of dichloroacetate (DCA), arginine, coenzyme Q10, idebenone, and exercise in both primary (congenital) disorders and secondary (degenerative) disorders. Despite prior clinical impressions that DCA had a positive effect on mitochondrial disorders, two trials of diverse subjects failed to demonstrate a clinically significant benefit, and a trial of DCA in MELAS found a major negative effect of neuropathy. Arginine also has been used to treat MELAS with promising effects, although a controlled trial is still needed for this potentially toxic agent. The anti-oxidant coenzyme Q10 is very widely used for primary mitochondrial disorders but has not yet undergone a controlled clinical trial; such a trial is now underway, as well as trials of the co-Q analogue idebenone for MELAS and LHON. Greater experience has accumulated with multi-center trials of coenzyme Q10 treatment to prevent the progression of Parkinson disease. Although initial smaller trials indicated a benefit, this has not yet been confirmed in subsequent trials with higher doses; a larger Phase III trial is now underway. Similarly, a series of trials of idebenone for Friedreich ataxia have shown some benefit in slowing the progression of cardiomyopathy, and controlled clinical trials are now underway to determine if there is significant neurological protection. Uncontrolled trials of exercise showed an increase of exercise tolerance in patients with disorders of mitochondrial DNA, but did not selectively increase the percentage of normal mtDNA; a larger partially controlled trial is now underway to evaluate this possible benefit. In summary, none of the controlled trials so far has conclusively shown a benefit of treatment with the agents tested, but some promising therapies are currently being evaluated in a controlled manner. These experiences underscore the importance of controlled clinical trials for evaluation of benefits and risks of recommended therapies. Application of such clinical trials to future more effective therapies for mitochondrial disorders will require multi-center collaboration, organization, leadership, and financial and advocacy support. © 2009 Elsevier Inc. All rights reserved.
Chandrasekharam V.V.S.,Rainbow Hospitals
Journal of Pediatric Urology | Year: 2013
Laparoscopic pyeloplasty is being performed successfully in children with results comparable to open pyeloplasty. Pediatric laparoscopic pyeloplasty, however, remains a technically demanding procedure. Some of the most difficult, time-consuming, and at the same time critical steps of the procedure are ureteral spatulation and intracorporeal suturing of the anastomosis [1,2,3]. It is also preferable to avoid grasping the ureter with any instrument. The difficulty of ureteral spatulation has led to the development of some technical modifications [1,2], including an ex-vivo spatulation technique . In our own practice, we tried some modifications to overcome this challenging part of the procedure. We have finally developed a technique that simplifies this surgical step. This technique can be performed with simple conventional laparoscopic instruments and is easily reproducible. © 2012 Journal of Pediatric Urology Company. Published by Elsevier Ltd. All rights reserved.
Shahid A.,Rainbow Hospitals
Epilepsia | Year: 2013
Tuberous sclerosis complex (TSC) is an autosomal dominant, neurocutaneous disorder causing benign noninvasive lesions in multiple organ systems. Cognitive delays and neuropsychiatric disorders such as autism are common. Epilepsy is very common and is associated with single or multiple epileptogenic cortical tubers. More than half of these individuals will become medically refractory over time. For these patients, epilepsy surgery with resection of the epileptogenic tuber or tubers not only can render a select patient population seizure-free, but also improves IQ and cognitive behaviors over time. © 2013 International League Against Epilepsy.
Chandrasekharam V.V.S.,Rainbow Hospitals
Indian Journal of Urology | Year: 2013
Purpose: There are many techniques of harvesting vascularized skin flaps from penile skin for hypospadias repairs. Here, we review our experience with the use of longitudinal dorsal island flap (LDIF) for mid- and proximal hypospadias repairs and to assess the results and complications of onlay and tubularized repairs. Materials and Methods: We retrospectively reviewed the data of all children with hypospadias operated by a single surgeon using the LDIF technique. The severity of hypospadias, technique, complications, and follow-up were assessed. Results: Over a 9.4-year period, 102 children (mean age 4.2 years, range 6 months to 11 years) were operated for primary hypospadias using the LDIF technique. All children had poorly formed urethral plates and hence were considered unsuitable for tubularized incised plate repair. The hypospadias was classified as midpenile, proximal penile/penoscrotal or perineal in 29, 64, and 9 children respectively. Onlay LDIF repair was done in 85 children, while in 17 children, tubularized LDIF repair was performed. At a mean follow-up of 1.8 years, complications occurred in 12 (12%) children. Complications were more common after tubularized repairs than onlay repairs (24% vs. 9.5%). All glans dehiscences occurred after onlay repairs, while meatal stenosis and diverticulum occurred after tubularized repairs. No child had urethral stenosis after onlay repair, and uroflow studies in 16 children demonstrated normal curves and flow rates. Conclusions: We report the use of LDIF for single-stage mid and proximal hypospadias repair with good success and an acceptable complication rate. Onlay repairs had fewer complications than tubularized repairs. Our results indicate that the specific advantages and versatility of LDIF make it a good option to consider in cases of hypospadias with poorly developed urethral plates where onlay or substitution urethroplasty is indicated.
Allareddy V.,Harvard University |
Allareddy V.,Rainbow Hospitals |
Nalliah R.P.,Harvard University
Journal of Oral and Maxillofacial Surgery | Year: 2011
Purpose: Injuries resulting from accidents are a leading cause of mortality and morbidity. The objective of this study was to present epidemiologic estimates of hospital-based emergency department (ED) visits for facial fractures in the United States. Materials and Methods: The Nationwide Emergency Department Sample for 2007 was used. All ED visits with facial fractures were selected. Demographic characteristics of these ED visits, causes of injuries, presence of concomitant injuries, and resource use in hospitals were examined. All estimates were projected to national levels and each ED visit was the unit of analysis. Results: During 2007 in the United States, 407,167 ED visits concerned a facial fracture. Patients' average age for each ED visit was 37.9 years. Sixty-eight percent of all ED visits concerned male patients, and 85,759 ED visits resulted in further treatment in the same hospital. Three hundred fourteen patients died in EDs, and 2,717 died during hospitalization. Mean charge per each ED visit was $3,192. Total United States ED charges were close to $1 billion. Mean hospitalization charges (ED and inpatient charges) amounted to $62,414. Mean length of stay was 6.23 days, and total hospitalization time in the entire United States was 534,322 days. Frequently reported causes of injuries included assaults (37% of all ED visits), falls (24.6%), and motor vehicle accidents (12.1%). Conclusions: The management of maxillofacial fractures in EDs across the United States uses considerable resources. The public health impact of facial fractures is highlighted in the present study. © 2011 American Association of Oral and Maxillofacial Surgeons.
Dasenbrook E.C.,Rainbow Hospitals
Current Opinion in Pulmonary Medicine | Year: 2011
Purpose of Review: Respiratory infection is a major contributor to morbidity and mortality in cystic fibrosis (CF). One infection the CF community is particularly concerned about is methicillin-resistant Staphylococcus aureus (MRSA). Worldwide, the prevalence of MRSA has been rising and the impact on clinical outcomes and optimal prevention and treatment strategies are unclear. Recent Findings: Studies have demonstrated MRSA is independently associated with poor clinical outcomes, even after taking into account severity of illness. Additionally, characteristics of MRSA strains, such as small colony variants and borderline oxacillin-resistant S. aureus, may be important in predicting the subsequent clinical course. The treatment of MRSA has had variable results and emergence of resistance to commonly prescribed antibiotics is a concern. Summary: The evidence to date supports MRSA infection is independently associated with worse outcomes. The next step is to build upon the current research to expand the knowledge about the impact different strains of MRSA have on infection control strategies and MRSA treatment protocols. Interventions should balance patient safety, efficacy, and treatment burden to improve the quality and length of life in patients with CF. © 2011 Wolters Kluwer Health | Lippincott Williams & Wilkins.