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Pandya N.K.,University of California at San Francisco | Edmonds E.W.,Rady Childrens Hospital San Diego
Journal of Pediatric Orthopaedics | Year: 2012

Background: Flexible nailing has become the preferred implant for pediatric patients with tibial shaft fractures that require operative fixation. Immediate definitive fracture fixation with flexible nails in patients with high-energy, open fractures has not been examined. The purpose of our study was to determine if immediate flexible nailing of open pediatric tibial shaft fractures is safe and efficacious from a bone healing, wound, and infectious standpoint. Methods: A retrospective review of 26 tibial shaft fractures consecutively treated with flexible nailing at our institution from 2003 to 2010 was performed. Age, mechanism of injury, associated injuries, presence of compartment syndrome, antibiotic administration, systemic insults, time to union, as well as bone healing (nonunion, delayed union, malunion, leg length discrepancy, growth arrest), wound, and infectious complications were collected. Comparisons were made between patients with open fractures and those with closed fractures. Results: We identified 14 patients with open fractures and a control group of 12 patients with closed injuries who underwent flexible nailing. Patients with open fractures were more likely to have polytraumatic injuries (71.0% vs. 25.0%, P=0.04). There was no difference (P=1.0) in the rates of compartment syndrome (open=14.0%, closed=17.0%) between the 2 groups. Systemic complications (pulmonary compromise and increased intracranial pressure) were noted in 2 patients who underwent immediate nailing of their open fractures; both of whom had closed head injuries. There was no difference (P=1.0) in the rates of wound/infectious complications between the open (7.0%) and closed (4.0%) fractures groups, with no cases of wound breakdown or osteomyelitis. There was an increased rate (P=0.02) of bone healing complications in the open fracture group (21.0% vs. 4.0%); all in patients with Gustilo type 2 or 3 injuries. All patients achieved radiographic union at final follow-up. Conclusions: Immediate flexible nailing of open pediatric tibial shaft fractures can be safely performed with minimal risk of wound or infectious complications. Clinicians should understand that prolonged bone healing (particularly in Gustilo type 2 or 3 injuries) should be expected in patients who undergo immediate flexible nailing of their open fractures. Open tibial shaft fractures are high-energy injuries, and should be seen as surrogate markers of polytrauma in the pediatric population. The risk of compartment syndrome is high regardless of whether a patient has a closed or open tibia fracture, and caution should be used in performing flexible nailing in patients who may have closed head injury due to a risk of systemic complications. Level of Evidence: Level III, therapeutic study, retrospective cohort. Copyright © 2012 by Lippincott Williams & Wilkins.

Pandya N.K.,Rady Childrens Hospital San Diego | Namdari S.,University of Pennsylvania | Hosalkar H.S.,Rady Childrens Hospital San Diego
Journal of the American Academy of Orthopaedic Surgeons | Year: 2012

There is an increasing trend toward stabilization and fixation of markedly displaced midshaft clavicle fractures in adolescents. Recent studies in the adult literature have shown a greater prevalence of symptomatic malunion, nonunion, and poor functional outcomes after nonsurgical management of displaced fractures. Fixation of displaced midshaft clavicle fractures can restore length and alignment, resulting in shorter time to union. Symptomatic malunion after significantly displaced fractures in adolescents may be more common than previously thought. Adolescents often have high functional demands, and their remodeling potential is limited. Knowledge of bone biology and the effects of shortening, angulation, and rotation on shoulder girdle mechanics is critical in decision making in order to increase the likelihood of optimal results at skeletal maturity. Selection of fixation is dependent on many factors, including fracture type, patient age, skeletal maturity, and surgeon comfort. Copyright © 2012 by the American Academy of Orthopaedic Surgeons.

Pardee P.E.,University of California at San Diego | Dunn W.,University of Kansas Medical Center | Schwimmer J.B.,University of California at San Diego | Schwimmer J.B.,Rady Childrens Hospital San Diego
Alimentary Pharmacology and Therapeutics | Year: 2012

Background: Non-alcoholic fatty liver disease (NAFLD) is the most common cause of chronic liver disease in children. Liver disease can be a cause of low bone mineral density. Whether or not NAFLD influences bone health is not known. Aim To evaluate bone mineral density in obese children with and without NAFLD. Methods Thirty-eight children with biopsy-proven NAFLD were matched for age, gender, race, ethnicity, height and weight to children without evidence of liver disease from the National Health and Nutrition Examination Survey. Bone mineral density was measured by dual energy X-ray absorptiometry. Age and gender-specific bone mineral density Z-scores were calculated and compared between children with and without NAFLD. After controlling for age, gender, race, ethnicity and total per cent body fat, the relationship between bone mineral density and the severity of histology was analysed in children with NAFLD. Results Obese children with NAFLD had significantly (P < 0.0001) lower bone mineral density Z-scores (-1.98) than obese children without NAFLD (0.48). Forty-five per cent of children with NAFLD had low-bone mineral density for age, compared to none of the children without NAFLD (P < 0.0001). Among those children with NAFLD, children with NASH had a significantly (P < 0.05) lower bone mineral density Z-score (-2.37) than children with NAFLD who did not have NASH (-1.58). Conclusions The NAFLD was associated with poor bone health in obese children. More severe disease was associated with lower bone mineralisation. Further studies are needed to evaluate the underlying mechanisms and consequences of poor bone mineralisation in children with NAFLD. © 2011 Blackwell Publishing Ltd.

Mulligan C.K.,University of California at San Diego | Trauner D.A.,University of California at San Diego | Trauner D.A.,Rady Childrens Hospital San Diego
Journal of Autism and Developmental Disorders | Year: 2014

Autism spectrum disorders (ASD) are associated with an increased incidence of epilepsy and of epileptiform discharges on electroencephalograms. It is unknown whether epileptiform discharges correlate with symptoms of ASD. We completed a retrospective chart review of 101 patients with ASD who had overnight electroencephalograms. We looked for a relationship between epileptiform abnormalities and diagnosis, history of regression, communication skills, and other features associated with ASD. There was a higher incidence of epileptiform activity in children with stereotypies and aggressive behavior. The incidence of epileptiform abnormalities was significantly lower in Asperger's compared with more severe forms of autism. Results suggest that increasing severity of autistic symptoms may be associated with higher likelihood of epileptiform abnormalities. Whether treatment alters outcome is unknown. © Springer Science+Business Media 2013.

Perry J.C.,Rady Childrens Hospital San Diego
Pediatric Cardiology | Year: 2012

A key component of recognizing sudden cardiac death (SCD) risk in the adult congenital heart disease (ACHD) patient is the recognition of heart failure risk for each physiology. The risk of SCD is an accrued phenomenon, representing the influences of anatomy, genetics, surgical and catheter interventions, and long-term sequelae of residual hemodynamic issues. These all lead to a substrate for tachyarrhythmia. It is beneficial in thinking about all of the potential combinations of CHD anatomy and physiologies to categorize SCD risk for the ACHD patient in terms of systemic left-ventricular failure, systemic right-ventricular failure, subpulmonary ventricular failure, the dyssynchronous contractility states due to bundle branch block, and single-site ventricular pacing. This article reviews important issues in arrhythmogenesis for ACHD patients with all of these physiologies and discusses potential cardiac rhythm device-management needs. © Springer Science+Business Media, LLC 2012.

Isaacs Jr. H.,Rady Childrens Hospital San Diego | Isaacs Jr. H.,University of California at San Diego
Journal of Pediatric Surgery | Year: 2010

Purpose: Few studies have focused on the behavior of rhabdoid tumor (RT) in the fetus and neonate. The purpose of this review is to show that perinatal RTs are associated with unusual findings and a poor prognosis. Methods: The author conducted a 40-year systematic review of the literature. Clinical presentation, pathology, management, and outcome of 72 fetuses and neonates with RTs are discussed. Results: Seventy-two fetuses and neonates presented with RTs detected prenatally (n = 12) and during the neonatal period (n = 60). The review consisted of 3 main groups: extrarenal noncentral nervous system (CNS) RT, renal RT, and CNS RT. There were some group differences in survival: extrarenal non-CNS RT (3/33 or 9.1%), renal RT (2/27 or 7.4%), and CNS RT (2/12 or 16.7%). Metastatic RT was present at diagnosis in more than half the patients (41/72 or 57%) who had a survival of 2.3%. The overall survival was 9.7%. For statistical results, there was no significant difference in survival among the 3 groups by type of tumor (P = .692). χ 2 analysis for survival with and without metastases was not valid due to small sample size. Conclusions: The review shows that extrarenal RT was more common than either renal RT or CNS RT groups that is different than that observed in older individuals. Concomitant brain tumors were found in almost a third of fetuses and neonates. The CNS involvement occurred more often in patients with renal RT than in those with extrarenal RT. Metastatic disease at diagnosis was noted in more than half of the patients. Higher stage and presence of a CNS tumor were significant determinants in survival. © 2010 Elsevier Inc. All rights reserved.

Schierholz E.,Rady Childrens Hospital San Diego
Advances in neonatal care : official journal of the National Association of Neonatal Nurses | Year: 2014

Therapeutic hypothermia as a neuroprotective strategy in neonates is an established standard of care for infants with hypoxic-ischemic encephalopathy (HIE) in tertiary care neonatal intensive care units (NICUs). To maximize the neuroprotective effect in infants with HIE, hypothermia is initiated as soon as possible after birth. Many infants who would benefit from therapeutic hypothermia are not born at centers that have intensive care units or offer therapeutic hypothermia and are thus transported to a tertiary care center with a NICU, offering specialty services of therapeutic hypothermia and pediatric neurology. The neonatal transport team plays a significant role in the management of these critically ill infants. Clinical research provides data for safe and effective management of these infants during therapeutic hypothermia in the NICU; however, there are no evidence-based clinical guidelines for management before and during transport. The establishment of evidence-based guidelines for cooling before and during transport will facilitate early recognition of infants who would benefit from therapeutic hypothermia therapy, and decrease delay in initiation of therapy. Careful assessment, monitoring, and intervention by the transport team are critical to provide appropriate care and ensure safe transport of these infants.

Chen T.S.,Rady Childrens Hospital San Diego | Eichenfield L.F.,Rady Childrens Hospital San Diego | Friedlander S.F.,Rady Childrens Hospital San Diego
Pediatrics | Year: 2013

Infantile hemangiomas (IHs) are the most common vascular tumors of childhood, affecting ~5% of all infants. Although most lesions proliferate and then involute with minimal consequence, a significant minority can be disfiguring, functionally significant, or, rarely, lifethreatening. Recent discoveries concerning hemangioma pathogenesis provide both an improved understanding and more optimal approach to workup and management. Important detrimental associations can be seen with IH, such as significant structural anomalies associated with segmental IH. Standards of care have dramatically changed evaluation and management of hemangiomas. The goal of timely recognition and therapy is to minimize or eliminate long-term sequelae. New modalities, such as oral propranolol, provide the caregiver with better therapeutic options, which can prevent or minimize medical risk or scarring, but the side effect profile and risk-benefit ratio of such interventions must always be evaluated before instituting therapy. Copyright © 2013 by the American Academy of Pediatrics.

Gunta S.S.,Rady Childrens Hospital San Diego | Mak R.H.,Rady Childrens Hospital San Diego
Pediatric Nephrology | Year: 2013

There is a rapid increase worldwide in the prevalence of obesity in adults and children. Obesity is not only a comorbidity for chronic kidney disease (CKD) but may also be a risk factor for CKD. Epidemiological correlations and pathophysiological changes have been observed associating obesity with CKD. Low birth weight may be associated with both obesity and low nephron mass, leading to CKD later in life. Elevated levels of adipokines, such as leptin and adiponectin, in obesity may be factors in CKD pathogenesis and progression. Furthermore, various other factors, such as hypertension, increased cardiovascular morbidity, insulin resistance, dyslipidemia, and lipotoxicity, may play significant roles in the pathogenesis of CKD in obesity. Reduction in obesity, which is a potentially modifiable risk factor, might help decrease the burden of CKD in the population. Apart from individualized options, community-based interventions have the potential to create a strong impact in this condition. © 2012 IPNA.

Rady Childrens Hospital San Diego | Date: 2014-10-14

A system and method for automated quality assurance including a quality assurance application server for managing quality assurance. The quality assurance application server includes an application configuration module which has a data configuration module configured to define configurable reference fields to be captured and reviewed during the processing of the records. The application configuration module also has a rules configuration module to receive, create or modify a set of rules where the configurable reference fields are selectable when the rules are defined. The system also includes a portal module for processing the received records and applying the set of rules to the record. The portal module includes a quality assurance records review module configured to evaluate and validate the received records and to generate an output utilized to validate the record to ensure compliance with a data recipient, for example payer billing, standards.

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