Cook Childrens Hospital

Fort Worth, TX, United States

Cook Childrens Hospital

Fort Worth, TX, United States
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Uya A.,Children's Medical Center Dallas | Uya A.,University of Houston | Spear D.,Children's Medical Center Dallas | Patel K.,Children's Medical Center Dallas | And 4 more authors.
Academic Emergency Medicine | Year: 2012

Background: No single confirmatory device can accurately distinguish between endotracheal, endobronchial, and esophageal intubation. Bedside ultrasound (US) shows promising potential for endotracheal tube (ETT) verification. Image acquisition depends on the approach used and the experience of the sonographer. Air within the trachea remains a challenge for interpretation of US images. Insufflation of the ETT cuff with saline helps overcome this difficulty and allows easy visualization of the cuff. This novel approach has not been studied in ETT verification among novice sonographers. Objectives: The objective was to evaluate the accuracy of novice sonographers in identifying proper ETT location and depth using US visualization of a saline-filled cuff. Methods: Eight pediatric emergency medicine (PEM) fellows without prior training in airway bedside US participated in this prospective pilot study. Baseline US knowledge was assessed using a pretraining questionnaire. Fellows received a 20-minute didactic training session focused on airway US, followed by a 30-minute practice session. Using a linear US probe placed at the suprasternal notch, fellows identified the saline-filled cuff of an ETT in both the trachea and the esophagus. Following training, the ETT was placed in either the esophagus or the trachea of the cadaver model by the principal investigator. ETT depth (adequacy) was confirmed by chest radiograph. Each PEM fellow, blinded to the placement of the ETT, used bedside US to determine ETT location and depth. If placement was determined to be tracheal, the fellow was asked to comment on adequacy of tube placement. Adequate placement was defined as complete visualization of the ETT cuff within the trachea at the suprasternal notch. This was used as a surrogate for correct depth. This study sequence was repeated five times for each trainee, following varying placement of the ETT in the trachea or esophagus. Results: The PEM fellows displayed limited baseline knowledge of US prior to receiving the training module (average score of 50% on pretest questionnaire). None had any prior airway bedside US experience. Following training, PEM fellows correctly identified ETT location in 39 of 40 scans, with a sensitivity of 96% (23 of 24) for identifying tracheal location. The tube depth was correctly identified in 22 of 23 scans identified as tracheal intubations. Conclusions: PEM fellows, lacking formal airway bedside US training, were able to identify the location and depth of a saline-filled ETT above or at the suprasternal notch in an adult cadaver model following a 50-minute teaching module. Filling the ETT cuff with saline allowed novice sonographers to accurately visualize the ETT within the trachea. © 2012 by the Society for Academic Emergency Medicine.

Honeycutt J.H.,Cook Childrens Hospital
Seminars in Plastic Surgery | Year: 2014

Over the last decade, endoscopy has been increasingly utilized in craniosynostosis surgery. In 2006, the author added endoscopy followed by helmet therapy to the treatment of young craniosynostosis patients. Since then, 73 children have been successfully treated utilizing endoscopic techniques with a transfusion rate of 23%. Most children are discharged on the first postoperative day; helmet therapy begins one week later. A helmet is worn for 4 to 6 months with one helmet replacement. Complications were limited to three reoperations to address suboptimal results, and one reoperation for a persisting skull defect. One sagittal sinus injury was addressed successfully, with resolution of a small intrasinus thrombus and no adverse brain sequelae. Although not applicable to every craniosynostosis patient, properly applied endoscopic-assisted craniosynostosis surgery is safe and effective, adding another option to the treatment armamentarium for craniosynostosis. © 2014 by Thieme Medical Publishers, Inc., 333 Seventh Avenue, New York.

Lin A.E.,MassGeneral Hospital for Children | Traum A.Z.,MassGeneral Hospital for Children | Sahai I.,MassGeneral Hospital for Children | Keppler-Noreuil K.,U.S. National Institutes of Health | And 4 more authors.
American Journal of Medical Genetics, Part A | Year: 2013

Sensenbrenner syndrome, also known as cranioectodermal dysplasia, is a rare multiple anomaly syndrome with distinctive craniofacial appearance, skeletal, ectodermal, connective tissue, renal, and liver anomalies. Dramatic advances with next-generation sequencing have expanded its phenotypic variability and molecular heterogeneity. We review 39 patients including two new patients, one with compound heterozygous novel mutations in WDR35 and a previously unreported multisutural craniosynostosis that may be a part of Sensenbrenner syndrome. In 14 of 25 (56.0%) patients pathogenic mutations have been identified in 4 different genes that regulate (intraflagellar) cilia transport. We compared Sensenbrenner syndrome to asphyxiating thoracic dystrophy-Jeune syndrome (ATD-JS) and other ciliopathies. Our analyses showed that the high anterior hairline, forehead bossing and dolichocephaly (accompanied by sagittal craniosynostosis in more than half of the patients) occur in almost all patients with Sensenbrenner syndrome. Metaphyseal dysplasia with narrow thorax, proximal limb shortness, and short fingers are typical of Sensenbrenner syndrome and ATD-JS. Respiratory complications have been reported in both syndromes, usually less severe with Sensenbrenner syndrome. Proposed diagnostic criteria for Sensenbrenner syndrome include the distinctive craniofacial appearance, ubiquitous brachydactyly and ectodermal anomalies, and sagittal craniosynostosis. Mild heart defects have been noted, but there have been no atrioventricular canal or heterotaxy defects that are common in Ellis-Van Creveld syndrome. We anticipate that the steady identification of molecularly defined patients may allow correlation of phenotype and genotype. Additional natural history data will improve genetic counseling and current guidelines. © 2013 Wiley Periodicals, Inc.

Akers L.J.,Cook Childrens Hospital | Fang W.,University of Texas M. D. Anderson Cancer Center | Levy A.G.,Md Anderson Cancer Center Orlando | Franklin A.R.,University of Texas M. D. Anderson Cancer Center | And 2 more authors.
Leukemia Research | Year: 2011

Rapidly proliferating solid tumor cells are often dependent on glycolysis for ATP production even in normoxia (the Warburg effect), however it is not yet clear whether acute leukemias have a similarly increased dependence on aerobic glycolysis. We report that all acute leukemia subtypes (pre-B ALL, T-ALL and AML) demonstrated growth arrest and cell death when treated the novel glycolysis inhibitor 3-BrOP. Potentiated ATP depletion and pro-apoptotic effects were seen for 3-BrOP combinations with the cytochrome-c-reductase inhibitor antimycin A and the mTOR inhibitor rapamycin. These results reveal a potential role for glycolysis inhibition in acute leukemia subtypes and suggest potential combinations. © 2011 Elsevier Ltd.

Brooks M.R.,Cook Childrens Hospital | Golianu B.,Stanford University
Paediatric Anaesthesia | Year: 2016

Children with chronic pain often undergo surgery and effective perioperative management of their pain can be challenging. Identification of the pediatric chronic pain patient preoperatively and development of a perioperative pain plan may help ensure a safer and more comfortable perioperative course. Successful management usually requires multiple different classes of analgesics, regional anesthesia, and adjunctive nonpharmacological therapies. Neuropathic and oncological pain can be especially difficult to treat and usually requires an individualized approach. © 2016 John Wiley & Sons Ltd

Lawrence R.,Washington University in St. Louis | Inder T.,Washington University in St. Louis | Inder T.,Cook Childrens Hospital
Seminars in Pediatric Neurology | Year: 2010

Seizures are more prevalent during the neonatal period than at any other time in the human lifespan. During early development, neonates are developmentally predisposed to excitatory neuronal activity increasing their susceptibility to seizures. Status epilepticus is poorly defined in this subpopulation with a lack of a consensus definition. In this review, we discuss the common etiologies of recurrent seizures in the newborn in addition to current trends on monitoring and treatment. Finally, we discuss the current evidence in both animal and human studies that indicate that neonatal seizures may be harmful to the immature brain with adverse long-term neurodevelopment outcomes. © 2010 Elsevier Inc.

Vichinsky E.,Childrens Hospital Oakland Research Institute | Torres M.,Cook Childrens Hospital | Minniti C.P.,U.S. National Institutes of Health | Barrette S.,Sainte Justine Hospital | And 3 more authors.
American Journal of Hematology | Year: 2013

We report a prospective, randomized, Phase II study of deferasirox and deferoxamine (DFO) in sickle cell disease patients with transfusional iron overload, with all patients continuing on deferasirox after 24 weeks, for up to 2 years. The primary objective was to evaluate deferasirox safety compared with DFO; long-term efficacy and safety of deferasirox was also assessed. We also report, for the first time, the safety and pharmacokinetics of deferasirox in patients concomitantly receiving hydroxyurea. Deferasirox (n=135) and DFO (n=68) had comparable safety profiles over 24 weeks. Adverse events (AEs) secondary to drug administration were reported in 26.7% of patients in the deferasirox cohort and 28.6% in the DFO cohort. Gastrointestinal disorders were more common with deferasirox, including diarrhea (10.4% versus 3.6%) and nausea (5.2% versus 3.6%). The most common AE in the DFO group was injection-site pain irritation, which occurred in 7% of patients. Acute renal failure occurred in one patient on deferasirox who was continued on medication despite progressive impairment of renal function parameters. Serum ferritin levels were reduced in both treatment groups. Patients continuing on deferasirox for up to 2 years demonstrated an absolute median serum ferritin decrease of -614 ng/mL (n=96). Increasing deferasirox dose was associated with improved response and a continued manageable safety profile. Concomitant hydroxyurea administration (n=28) did not appear to influence the efficacy, safety (including liver and kidney function), and pharmacokinetic parameters of deferasirox. © 2013 Wiley Periodicals, Inc.

Wirrell E.C.,Mayo Medical School | Shellhaas R.A.,University of Michigan | Joshi C.,University of Iowa | Keator C.,Cook Childrens Hospital | And 2 more authors.
Epilepsia | Year: 2015

Summary Objective To prospectively evaluate the etiology of new-onset infantile spasms and evaluate the yield of genetic and metabolic investigations in those without obvious cause after initial clinical evaluation and magnetic resonance imaging (MRI). Methods Twenty-one U.S. pediatric epilepsy centers prospectively enrolled infants with newly diagnosed West syndrome in a central database. Etiology and investigations performed within 3 months of diagnosis were documented. Results From June 2012 to June 2014, a total of 251 infants were enrolled (53% male). A cause was identified in 161 (64.4%) of 250 cases (genetic,14.4%; genetic-structural, 10.0%; structural-congenital, 10.8%; structural-acquired, 22.4%; metabolic, 4.8%; and infectious, 2.0%). An obvious cause was found after initial clinical assessment (history and physical examination) and/or MRI in 138 of 161, whereas further genetic and metabolic studies were revealing in another 23 cases. Of 112 subjects without an obvious cause after initial evaluation and MRI, 81 (72.3%) had undergone genetic testing, which showed a causal abnormality in 23.5% and a variant of unknown significance in 14.8%. Although metabolic studies were done in the majority (serum, 79.5%; urine, 69.6%; and cerebrospinal fluid [CSF], 38.4%), these revealed an etiology in only five cases (4.5%). No correlation was found between type of health insurance (public vs. private) and either genetic or metabolic testing. Significance Clinical evaluation and MRI provide a specific diagnosis in 55% of children presenting with West syndrome. We propose that a cost-effective workup for those without obvious cause after initial clinical evaluation and MRI includes an array comparative genomic hybridization (aCGH) followed by an epilepsy gene panel if the microarray is not definitive, serum lactate, serum amino acids, and urine organic acids. © Wiley Periodicals, Inc. © 2015 International League Against Epilepsy.

Sankar W.N.,Children's Hospital of Philadelphia | Schoenecker J.G.,Monroe Hospital | Mayfield M.E.,Cook Childrens Hospital | Kim Y.-J.,Childrens Hospital of Boston | Millis M.B.,Childrens Hospital of Boston
Journal of Pediatric Orthopaedics | Year: 2012

BACKGROUND: The management of the unstable Down syndrome hip is challenging, and there is controversy about the anatomic factors that may contribute to the instability. It has been our observation that children with Down syndrome often have a deficient posterior acetabular wall. This is different from other congenital acetabular dysplasia where the anatomic deficiency is typically anterolateral. These observations suggest that the acetabulum in Down syndrome hip dysplasia may be relatively retroverted. The purpose of this study was to determine the acetabular version in children with Down syndrome and compare this with matched controls from both normal and developmental dysplasia of the hip (DDH) populations. METHODS: A cohort of Down patients treated surgically for acetabular dysplasia and/or hip instability was matched by age, sex, and side to a group of normal controls and compared with a cohort of patients who had undergone periacetabular osteotomy for DDH. For all patients, preoperative computed tomography scans were used to measure acetabular version through the joint center. Statistical differences were determined using analysis of variance with α=0.05. RESULTS: We identified 16 subjects in each cohort. The average acetabular version in the normal control group was 13±5 degrees and in the DDH cohort was 21±7 degrees. In contrast, the mean version in the group of patients with Down syndrome was 2±11 degrees, indicating increased acetabular retroversion; this result was significantly different from both the normal group (P=0.02) and those with DDH (P<0.001). According to the criteria described by Tönnis for computed tomography measured retroversion, 10/16 patients with Down syndrome were severely retroverted compared with only 3/16 normal controls and 1/16 patients with DDH (P=0.002). CONCLUSIONS: Patients with Down syndrome and hip instability seem to have more retroverted acetabula than normal controls and patients with DDH. In patients with Trisomy 21, axial imaging should be performed to evaluate acetabular version when planning the optimal corrective osteotomy for instability and/or acetabular deficiency. LEVEL OF EVIDENCE: Level III (prognostic, retrospective case-control). Copyright © 2012 by Lippincott Williams & Wilkins.

Brna P.M.,Dalhousie University | Dooley J.M.,Dalhousie University | Esser M.J.,Alberta Childrens Hospital | Perry M.S.,Cook Childrens Hospital | Gordon K.E.,Dalhousie University
Epilepsy and Behavior | Year: 2013

The internet has become the first stop for the public and patients to seek health-related information. Video-sharing websites are particularly important sources of information for those seeking answers about seizures and epilepsy. Because of the widespread popularity of YouTube, we sought to explore whether a seizure diagnosis and classification could reliably be applied. All videos related to "seizures" were reviewed, and irrelevant videos were excluded. The remaining 162 nonduplicate videos were analyzed by 4 independent pediatric neurologists who classified the events as epilepsy seizures, nonepileptic seizures, or indeterminate. Videos designated as epilepsy seizures were then classified into focal, generalized, or unclassified. At least 3 of the 4 reviewers agreed that 35% of the videos showed that the events were "epilepsy seizures", at least 3 of the 4 reviewers agreed that 28% of the videos demonstrated that the events were "nonepileptic seizures", and there was good agreement that 7% of the videos showed that the event was "indeterminate". Overall, interrater agreement was moderate at k. = 0.57 for epilepsy seizures and k. =0.43 for nonepileptic seizures. For seizure classification, reviewer agreement was greatest for "generalized seizures" (k. =0.45) and intermediate for "focal seizures" (k. =0.27), and there was no agreement for unclassified events (k. =0.026, p. =0.2). Overall, neurology reviewer agreement suggests that only approximately one-third of the videos designated as "seizures" on the most popular video-sharing website, YouTube, definitely depict a seizure. Caution should be exercised in the use of such online video media for accessing educational or self-diagnosis aids for seizures. © 2013 Elsevier Inc.

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