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Pediatric In Review

Pouya Entezami, M Reid Gooch, Jonathan Poggi, Eric Perloff, Melissa Dupin, Matthew A Adamo
OBJECTIVES: Pediatric Chiari Type 1 Malformations (CM1) are commonly referred for neurosurgical opinion. The ideal management in children regarding surgical and radiographic decision making is not clearly delineated. PATIENTS AND METHODS: We retrospectively reviewed our cohort of patients age 18 years and younger referred to a single neurosurgeon for CM1. Baseline MRIs of the spine were obtained. Non-operative patients had repeat imaging at 6-12 months. Patients who underwent an operation (decompression with/without duraplasty) had repeat imaging at 6 months...
December 10, 2018: Clinical Neurology and Neurosurgery
Katherine E Bates, William T Mahle, Lauren Bush, Janet Donohue, Michael G Gaies, Susan C Nicolson, Lara Shekerdemian, Madolin Witte, Michael Wolf, Judy A Shea, Donald S Likosky, Sara K Pasquali
BACKGROUND: The Pediatric Heart Network Collaborative Learning Study (PHN CLS) increased early extubation after infant Tetralogy of Fallot (TOF) and coarctation repair overall at participating sites through implementing a clinical practice guideline (CPG). We evaluated variability across sites in CPG implementation and outcomes. METHODS: Patient characteristics and outcomes [time to extubation, length of stay (LOS)] were compared across sites, including pre- to post-CPG changes...
December 14, 2018: Annals of Thoracic Surgery
Darius Ebrahimi-Fakhari, Clara Van Karnebeek, Alexander Münchau
There are several hundred single-gene disorders that we classify as inborn errors of metabolism. Inborn errors of metabolism are often rare and highly heterogeneous multisystem diseases with non-neurological and neurological manifestations, commonly with onset during childhood. Movement disorders are among the most common neurological problems in inborn errors of metabolism, but, in many cases, remain poorly defined. Although movement disorders are usually not the only and often not the presenting symptom, their recognition can facilitate a diagnosis...
December 17, 2018: Movement Disorders: Official Journal of the Movement Disorder Society
Elizabeth T Kennedy, Sheree Chapman York
No abstract text is available yet for this article.
January 2019: Pediatric Physical Therapy
Ana Clara Maia Palhano, Lenise Jihe Kim, Gustavo A Moreira, Fernando Morgadinho Santos Coelho, Sergio Tufik, Monica Levy Andersen
Narcolepsy is a sleep disorder characterized by excessive daytime sleepiness, cataplexy, sleep paralysis and hypnagogic and hypnopompic hallucinations. The onset of the symptoms usually occurs in childhood, and previous studies have reported an association between narcolepsy and other endocrine diseases in the pediatric population, such as obesity and precocious puberty. The incidence of overweight or obesity ranges from 25% to 74% in patients with narcolepsy type I, while precocious puberty is present in 17% of children with narcolepsy with cataplexy...
December 2018: Pediatric Endocrinology Reviews: PER
Eric J Yang, Kristen M Beck, Sahil Sekhon, Tina Bhutani, John Koo
BACKGROUND: Atopic dermatitis (AD) is an extremely common childhood disease, with considerable impact on the quality of life of affected children and their families. While pruritus is the hallmark symptom of this disease, AD has been well-documented to impact patients beyond physical symptoms, resulting in behavior problems, mood disorders, and sleep disturbance. OBJECTIVE: This literature review outlines how atopic dermatitis impacts the quality of life of families of children affected by AD...
December 16, 2018: Pediatric Dermatology
Kailyn Anderson, James Cnota, Jeanne James, Erin M Miller, Ashley Parrott, Valentina Pilipenko, Kathryn Nicole Weaver, Amy Shikany
OBJECTIVE: To evaluate the prevalence of Noonan spectrum disorders (NSD) in a pediatric population with valvar pulmonary stenosis (vPS) and identify the clinical characteristics that differentiate those with NSD from those without NSD. DESIGN: A retrospective chart review of 204 patients diagnosed with vPS between 9/1/2012 and 12/1/2016 at a pediatric medical center was performed. The quantitative features of vPS, genetic diagnosis information, and phenotypic characteristics of Noonan syndrome were collected...
December 16, 2018: Congenital Heart Disease
Fady Hannah-Shmouni, Constantine A Stratakis
Pituitary adenomas in children and adolescents are rare tumors that often result from a tumor predisposition syndrome. Several inherited causes for pituitary adenomas have been identified in the last few years, including multiple endocrine neoplasia type 1 and 4, Carney's complex, Tuberous sclerosis, DICER1 syndrome, neurofibromatosis type 1, McCune Albright syndrome, familial isolated pituitary adenoma, and pituitary adenoma association due to defects in succinate dehydrogenase genes. Recently, our group discovered X-linked acrogigantism (X-LAG), a new pediatric disorder that is caused by an Xq26...
August 2018: Current opinion in endocrine and metabolic research
Branden M Engorn, Christopher J L Newth, Margaret J Klein, Elizabeth A Bragg, Rebecca D Margolis, Patrick A Ross
Background: Pediatric Critical Care Medicine Fellowship trainees need to acquire skills to perform procedures. Over the last several years there have been advances that allowed for less invasive forms of interventions. Objective: Our hypothesis was that over the past decade the rate of procedures performed by Pediatric Critical Care Medicine Fellowship trainees decreased. Methods: Retrospective review at a single institution, tertiary, academic, children's hospital of patients admitted from July 1, 2007-June 30, 2017 to the Pediatric Intensive Care Unit and Cardiothoracic Intensive Care Unit...
2018: Frontiers in Pediatrics
Franziska Di Pauli, Thomas Berger
Inflammatory demyelinating CNS syndromes include, besides their most common entity multiple sclerosis (MS), several different diseases of either monophasic or recurrent character-including neuromyelitis optica spectrum disorders (NMOSDs) and acute disseminated encephalomyelitis (ADEM). Early diagnostic differentiation is crucial for devising individual treatment strategies. However, due to overlapping clinical and paraclinical features diagnosis at the first demyelinating event is not always possible. A multiplicity of potential biological markers that could discriminate the different diseases was studied...
2018: Frontiers in Immunology
Mohammad Abdallah, Hassan A Abdallah, Ziad A Memish
Burkholderia cepacia complex (Bcc) is a Gram-negative bacterium commonly found in moist environments and soil. Bcc species are associated with many outbreaks in intensive care units (ICUs). In this review, we describe the sources of Bcc outbreaks among non-cystic fibrosis (CF) patients in various ICUs that include neonatal intensive care units, pediatric intensive care units and adult ICUs. Also we summarize the risk factors and outcome predictors of Bcc infection or colonization in non-CF critically ill patients...
December 1, 2018: Le Infezioni in Medicina
P Corkum, S Weiss, W Hall, C Brown, C Chambers, E Constantin, R Godbout, A Hanlon-Dearman, O Ipsiroglu, G J Reid, S Shea, I M Smith, R Stremler, M Witmans
OBJECTIVES/BACKGROUND: This paper outlines the current state of Canadian training, clinical services, research, and advocacy initiatives related to non-respiratory sleep disorders, with a specific focus on insomnia, the most common sleep problem in children. METHODS: Information for this narrative review was collected from peer-reviewed publications, web-resources, and personal communications and experiences. RESULTS: It is estimated that approximately one-third of Canadian children and youth present with insomnia, and that this is impacting their physical and mental health, as well as learning in school...
November 26, 2018: Sleep Medicine
H Kaziz, M A Triki, T Mouelhi, K Bouattour, N Naouar, M L Ben Ayeche
INTRODUCTION: Septic elbow arthritis is a rare disease, especially in children. The aim of this study was to report the functional outcome of our pediatric population with both demographic and microbiological features. METHODS: We retrospectively reviewed all our cases of pediatric septic elbow arthritis between 2007 and 2017. Demographic, microbiological, biological, and radiological features were analyzed. Functional outcome was evaluated according to the Morrey Elbow Performance Score (MEPS)...
December 13, 2018: Archives de Pédiatrie: Organe Officiel de la Sociéte Française de Pédiatrie
Tyler A Janz, Ramamoorthy Nagasubramanian, Julie L Wei
OBJECTIVE: To examine pediatric head and neck fibrosarcoma cases and review the demographics, management, and survival for these patients. METHODS: Pediatric patients in the Surveillance, Epidemiology, and End Results (SEER) database were included from 1973 to 2014 based on a diagnosis of a head and neck fibrosarcoma using ICD-O-3 head and neck primary sites and histology codes. Patients were included from birth-18 years of age. Additionally, a pediatric case of a head and neck infantile fibrosarcoma treated at the Nemours Children's hospital in Orlando, Florida is presented...
January 2019: International Journal of Pediatric Otorhinolaryngology
Christian R Francom, Cameron A Best, Ryan G Eaton, Victoria Pepper, Amanda J Onwuka, Christopher K Breuer, Meredith N Merz Lind, Jonathan M Grischkan, Tendy Chiang
BACKGROUND: Endoscopic airway measurement (EAM) combines optical endoscopic instruments with open source image processing to accurately obtain airway dimensions. Preclinical models have demonstrated EAM as an accurate technique of airway measurement with the added advantage of characterizing multilevel stenosis, non-circular lesions, and distal obstruction. The aim of this prospective clinical study was to compare EAM to airway measurements obtained from endotracheal tube approximation (ETTA) during pediatric aerodigestive evaluation and to evaluate reproducibility of EAM across practitioners...
January 2019: International Journal of Pediatric Otorhinolaryngology
M Shannon Fracchia, Gillian Diercks, Anna Cook, Cheryl Hersh, Stephen Hardy, Marina Hartnick, Christopher Hartnick
OBJECTIVE/HYPOTHESIS: Multi-disciplinary aero-digestive centers provide high quality health care through improved outcomes and treatment costs over separate sub specialty clinics. These outcomes are often the result of a common investigative tool known as triple endoscopy: a rigid bronchoscopy performed by an otolaryngologist, flexible bronchoscopy and lavage obtained by a pulmonologist, and an endoscopy with guided biopsies performed by a gastroenterologist. Combining such procedures into one 'triple endoscopy' allows for diagnoses which otherwise might have been missed with just one procedure...
January 2019: International Journal of Pediatric Otorhinolaryngology
Hayley Born, Andre Wineland, Michael J Rutter
Acquired laryngomalacia in the pediatric population is rare, especially from a neurogenic cause. This case report describes a pediatric patient who developed laryngomalacia following a neurologic insult. A proposed physiologic pathway is reviewed. A thorough literature review was performed to identify cases of acquired laryngomalacia ascribed to a neurologic cause and are compared to this case.
January 2019: International Journal of Pediatric Otorhinolaryngology
Emily Savoca, Saral Mehra, Erik H Waldman
Cervicofacial actinomyces is an uncommon infection, especially in children. Notoriously low culture sensitivity and malignant appearance make diagnosis challenging. Treatment requires a prolonged antibiotic course often in conjunction with surgical debridement or drainage. We report an amorphous anterior neck mass in a 10-year-old girl due to actinomyces. Diagnosis required open biopsy after non-diagnostic fine needle aspirate and core needle biopsies. The patient responded well to six-week course of parenteral penicillin followed by six months of convalescent therapy with oral penicillin...
January 2019: International Journal of Pediatric Otorhinolaryngology
Anna C Bitners, William B Burton, Christina J Yang
OBJECTIVES: To compare the rates of skin-related complications and accidental decannulation in pediatric patients who received Velcro® ties versus twill ties during the early postoperative period following tracheotomy. The rates of skin-related complications and accidental decannulation in patients with Velcro® ties was hypothesized to differ from those in patients with twill ties. METHODS: Medical records of 109 patients ≤18 years old who underwent elective tracheotomy were reviewed: 70 received twill ties and 39 received Velcro® ties...
January 2019: International Journal of Pediatric Otorhinolaryngology
Diane W Chen, Yi-Chun Carol Liu
OBJECTIVE: To evaluate the feasibility and the outcomes for step-down (SD) unit admission as an alternative to intensive care unit (ICU) admission after supraglottoplasty in the pediatric patient. METHODS: A review of 98 patients who underwent supraglottoplasty from 2012 to 2017 at a tertiary referral pediatric hospital was performed. An SD unit had 1-to-3 nurse-to-patient ratio with noninvasive positive pressure ventilation capability. Data variables included demographics, comorbidities, preoperative and postoperative respiratory requirements, and length of stay...
January 2019: International Journal of Pediatric Otorhinolaryngology
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