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Ulkem Kocoglu Barlas, Hasan Serdar Kıhtır, Nilufer Goknar, Melike Ersoy, Nihal Akcay, Esra Sevketoglu
BACKGROUND: Hemolytic uremic syndrome (HUS) is a clinical syndrome characterized by hemolytic anemia, thrombocytopenia, and acute kidney injury. Atypical hemolytic uremic syndrome (aHUS) is a devastating disease with significant mortality and high risk of progression to end-stage kidney disease. It is mostly caused by dysregulation of the alternative complement pathway. Cobalamin C (Cbl C) defect is a genetic disorder of cobalamin metabolism and is a rare cause of HUS. CASE-DIAGNOSIS/TREATMENT: We present a 6-month-old male infant who was admitted to the pediatric intensive care unit (PICU) due to restlessness, severe hypertension, anemia, respiratory distress, and acute kidney injury...
March 20, 2018: Pediatric Nephrology: Journal of the International Pediatric Nephrology Association
Caroline C Swift, Meryle J Eklund, Jacqueline M Kraveka, Adina L Alazraki
Neuroblastoma is an embryonic tumor of the peripheral sympathetic nervous system. It is the most common extracranial solid tumor of childhood and accounts for up to 15% of all pediatric cancer fatalities. The manifestation of neuroblastoma is variable depending on the location of the tumor and on the presence or absence of paraneoplastic syndromes. The prognosis of neuroblastoma is also highly variable, ranging from spontaneous regression to widespread metastatic disease that is unresponsive to treatment. The age of the patient, stage of disease, histopathologic results, and multiple biologic factors contribute to the presurgical and pretreatment risk stratification of a patient with neuroblastoma...
March 2018: Radiographics: a Review Publication of the Radiological Society of North America, Inc
Xiaolin Li, Zhangning Zhao, Xiaomin Liu, Gaoting Ma, Mei-Jia Zhu
INTRODUCTION: Propofol infusion syndrome (PRIS) is a rare but potentially fatal complication of propofol infusion. It is clinically characterized by metabolic acidosis, refractory bradycardia, rhabdomyolysis, renal failure, hyperlipidemia, and hepatomegaly. Brain lesion was only reported once in a pediatric patient. We present the 1st adult case with colon polyp and cancer who was diagnosed with PRIS. Her brain magnetic resonance imaging (MRI) and computed tomography (CT) scans reveal prominent bilateral brain lesions, matching with the proposed pathophysiologic mechanism of the syndrome...
January 2018: Medicine (Baltimore)
Samaneh Salehi, Modjtaba Emadi-Baygi, Majdaddin Rezaei, Roya Kelishadi, Parvaneh Nikpour
BACKGROUND: Metabolic syndrome (MetS) is a complex and multifactorial disorder characterized by insulin resistance, dyslipidaemia, hyperglycemia, abdominal obesity, and elevated blood pressure. The apolipoprotein A5 (APOA5) gene variants have been reported to correlate with two major components of MetS, including low levels of high density lipoprotein cholesterol (HDL-C) and high levels of triglyceride. In the present study, we explored the associations between five single nucleotide polymorphisms (SNPs) of APOA5 gene and the MetS risk...
February 2018: Diabetes & Metabolism Journal
Rahul Badheka, Narendra Kumar Barad, Charulata Savant Sankhla
Pediatric movement disorders are commonly encountered clinical entities in the pediatric outpatient department. These disorders are a heterogenous group of disorders and may represent an underlying genetic disorder, a metabolic disorder or a hypoxic-ischemic insult during the perinatal period. Hyperkinetic movement disorders are more common as compared to hypokinetic disorders. This is unlike the situation in adult movement disorders where hypokinetic disorders are more often seen. A child's nervous system is more prone to hypoxic-ischemic insults due to its higher metabolic demands and the presence of an immature blood-brain barrier...
March 2018: Neurology India
Kristen Sgambat, Sarah Clauss, K Y Lei, Jiuzhou Song, Shaik O Rahaman, Margaret Lasota, Asha Moudgil
As originally published, this article contained errors owing to oversights in typesetting. The article has now been amended accordingly.
March 2, 2018: Pediatric Nephrology: Journal of the International Pediatric Nephrology Association
Akash Deep, Romit Saxena, Bipin Jose
Acute kidney injury (AKI) is a common accompaniment in patients with liver disease. The causes, risk factors, manifestations and management of AKI in these patients vary according to the liver disease in question (acute liver failure, acute-on-chronic liver failure, post-liver transplantation or metabolic liver disease). There are multiple causes of AKI in patients with liver disease-pre-renal, acute tubular necrosis, post-renal, drug-induced renal failure and hepatorenal syndrome (HRS). Definitions of AKI in liver failure are periodically revised and updated, but pediatric definitions have still to see the light of the day...
March 1, 2018: Pediatric Nephrology: Journal of the International Pediatric Nephrology Association
Nathalie Voide, Matthieu P Robert
PURPOSE: Although less frequent than consecutive exotropia, consecutive esotropia is a well-known type of strabismus when it follows the surgical correction of an exotropia. Spontaneous conversion from initial constant, large-angle exotropia beyond the age of 3 months to esotropia or orthophoria, however, is not common. We describe a series of infants who presented a spontaneous evolution from a large-angle infantile exotropia to either an orthophoria or a spontaneously consecutive esotropia...
March 2, 2018: Eye
Yael Lebenthal, Sigal Levy, Efrat Sofrin-Drucker, Nessia Nagelberg, Naomi Weintrob, Shlomit Shalitin, Liat de Vries, Ariel Tenenbaum, Moshe Phillip, Liora Lazar
Objective: Patients with Turner syndrome (TS) are at increased risk for metabolic disorders. We aimed to delineate the occurrence and evolution of metabolic comorbidities in TS patients and to determine whether these differ in 45,X monosomy and other karyotypes. Methods: A longitudinal and cross-sectional retrospective cohort study was conducted in a tertiary pediatric endocrine unit during 1980-2016. Ninety-eight TS patients, 30 with 45,X monosomy were followed from childhood to early adulthood...
2018: Frontiers in Endocrinology
Amanda Willig, Lauri Wright, Theresa A Galvin
Nutrition is an integral component of medical care for people living with human immunodeficiency virus (HIV)/autoimmune deficiency syndrome (AIDS) (PLWHA). The Academy of Nutrition and Dietetics supports integration of medical nutrition therapy into routine care for this population. Fewer PLWHA experience wasting and undernutrition, while the prevalence of obesity and other chronic diseases has increased significantly. Improved understanding of HIV infection's impact on metabolism and chronic inflammation has only increased the complexity of managing chronic HIV infection...
March 2018: Journal of the Academy of Nutrition and Dietetics
Pooneh Angoorani, Ramin Heshmat, Hanieh-Sadat Ejtahed, Mohammad Esmaeil Motlagh, Hasan Ziaodini, Majzoubeh Taheri, Tahereh Aminaee, Azam Goodarzi, Mostafa Qorbani, Roya Kelishadi
PURPOSE: The purpose of this study was to determine the cut-off values of triglycerides and glucose (TyG) index as one of the indirect indices for metabolic syndrome (MetS) in a pediatric population. METHODS: This national study was conducted in 2015 on 14400 students, aged 7-18 years. They were selected by random cluster sampling from 30 provinces of our country during the fifth survey of a national school-based surveillance program. MetS was defined based on the Adult Treatment Panel III (ATP III) criteria modified for the pediatric age group...
February 16, 2018: Eating and Weight Disorders: EWD
Ana Carolina G Bermudes, Werther B de Carvalho, Patricia Zamberlan, Giovana Muramoto, Raul C Maranhão, Artur F Delgado
OBJECTIVES: Limited knowledge exists regarding the lipid profiles of critically ill pediatric patients with systemic inflammatory response syndrome. The aim of this study was to evaluate the relationship between the intensity of the inflammatory response and changes in the lipid profiles of critically ill pediatric patients admitted to a pediatric intensive care unit (PICU) with severe sepsis/septic shock. METHODS: This was a prospective and observational study at a 15-bed PICU at a public university hospital...
March 2018: Nutrition
Rebecca D Ganetzky, Marni J Falk
BACKGROUND: Intravenous (IV) arginine has been reported to ameliorate acute metabolic stroke symptoms in adult patients with Mitochondrial Encephalopathy with Lactic Acidosis and Stroke-like Episodes (MELAS) syndrome, where its therapeutic benefit is postulated to result from arginine acting as a nitric oxide donor to reverse vasospasm. Further, reduced plasma arginine may occur in mitochondrial disease since the biosynthesis of arginine's precursor, citrulline, requires ATP. Metabolic strokes occur across a wide array of primary mitochondrial diseases having diverse molecular etiologies that are likely to share similar pathophysiologic mechanisms...
February 2, 2018: Molecular Genetics and Metabolism
Kristen Sgambat, Sarah Clauss, Asha Moudgil
Children are at increased risk of developing metabolic syndrome (MS) after kidney transplantation, which contributes to long-term cardiovascular (CV) morbidities and decline in allograft function. While MS in the general population occurs due to excess caloric intake and physical inactivity, additional chronic kidney disease and transplant-related factors contribute to the development of MS in transplant recipients. Despite its significant health consequences, the interplay of the individual components in CV morbidity in pediatric transplant recipients is not well understood...
February 2018: Clinical Kidney Journal
T Ferraris, L Toselli, J Udaquiola, R Vagni, P Coccia, G Alonso, P Lobos, J Moldes, D Liberto
OBJECTIVES: To describe our initial experience in the treatment of hyperparathyroidism (HPP) of renal cause using total or subtotal parathyroidectomy, autoimplant and cryopreservation in pediatric patients. Secondary HPP is the increased function of the parathyroid hormone (PTH) due to an abnormal phosphocalcic metabolism in patients with chronic renal failure (CRF). This situation produces increased bone resorption resulting in osteodystrophy and endovascular calcifications. Surgical treatment is aimed to diminish the level of PTH in CRF patients, to avoid HPP complications...
February 1, 2018: Cirugía Pediátrica: Organo Oficial de la Sociedad Española de Cirugía Pediátrica
Kristen Sgambat, Sarah Clauss, K Y Lei, Juizhou Song, Shaik O Rahaman, Margaret Lasota, Asha Moudgil
Early signs of subclinical CV dysfunction can be detected by ultrasound for CIMT. Although A-A are at high risk for CV disease, CIMT of A-A kidney transplant recipients has not been previously investigated. The aim of this prospective, controlled, longitudinal study was to investigate determinants of CIMT in a multiracial pediatric kidney transplant population, with a focus on A-A. Transplant recipients (n = 42) had BMI, waist-to-height ratio, fasting glucose, lipid panel, HbA1c%, and CIMT measured at 1, 18, and 30 months post-transplant...
February 8, 2018: Pediatric Transplantation
Myrtis Katille de Assunção Bezerra, Eduardo Freese de Carvalho, Juliana Souza Oliveira, Eduarda Ângela Pessoa Cesse, Pedro Israel Cabral de Lira, Jonathan Galvão Tenório Cavalcante, Vanessa Sá Leal
BACKGROUND: The emergence of diseases such as dyslipidemia, systemic arterial hypertension, insulin resistance and metabolic syndrome in children and adolescents has brought about a change in the epidemiologic profile of the pediatric population. As action to promote health in the school environment is a useful tool for changing the pattern of health/disease in the young population, the present study aimed to identify schools that promote healthy eating and physical activity and to study the relationship between these practices and the prevalence of overweight, hypertension, insulin resistance and hypercholesterolemia in adolescents...
February 7, 2018: BMC Public Health
Lawrence J Hirsch, Nicolas Gaspard, Andreas van Baalen, Rima Nabbout, Sophie Demeret, Tobias Loddenkemper, Vincent Navarro, Nicola Specchio, Lieven Lagae, Andrea O Rossetti, Sara Hocker, Teneille E Gofton, Nicholas S Abend, Emily J Gilmore, Cecil Hahn, Houman Khosravani, Felix Rosenow, Eugen Trinka
We convened an international group of experts to standardize definitions of New-Onset Refractory Status Epilepticus (NORSE), Febrile Infection-Related Epilepsy Syndrome (FIRES), and related conditions. This was done to enable improved communication for investigators, physicians, families, patients, and other caregivers. Consensus definitions were achieved via email messages, phone calls, an in-person consensus conference, and collaborative manuscript preparation. Panel members were from 8 countries and included adult and pediatric experts in epilepsy, electroencephalography (EEG), and neurocritical care...
February 5, 2018: Epilepsia
Federica Zibordi, Zorzi Giovanna, Carecchio Miryam, Nardocci Nardo
The terms Pediatric Autoimmune Neuropsychiatric disorders associated with streptococcal infections (PANDAS), Pediatric acute-onset neuropsychiatric Syndrome (PANS), and Childhood Acute Neuropsychiatric Symptoms (CANS) have been used to describe certain acute onset neuropsychiatric pediatric disorders. This clinical characteristic was unusually abrupt onset of obsessive compulsive symptoms and/or severe eating restrictions and concomitant cognitive, behavioral or neurological symptoms. Because the CANS/PANS criteria define a broad spectrum of neuropsychiatric conditions, the syndrome is presumed to result from a variety of disease mechanisms and to have multiple etiologies, ranging from postinfectious autoimmune and neuroinflammatory disorders to toxic, endocrine or metabolic disorders...
February 1, 2018: European Journal of Paediatric Neurology: EJPN
Wei-Ting Lin, Pei-Chen Lin, Chun-Ying Lee, Yi-Ling Chen, Te-Fu Chan, Sharon Tsai, Hsiao-Ling Huang, Pei-Wen Wu, Yu-Ting Chin, Hui-Yi Lin, Chien-Hung Lee
OBJECTIVES: Retinol-binding protein 4 (RBP4) and insulin resistance (IR) are clinical parameters associated with cardiometabolic diseases. The mediating and modifying roles of IR on children's susceptibility to cardiometabolic disorders are undetermined. This study investigated the mediating and modifying effects of the homeostatic model assessment of IR (HOMA-IR) on the relationship between the serum RBP4 level and clustering of pediatric cardiometabolic risk factors. METHODS: We assessed the diet, physical activity, cardiometabolic risk factors, and clinical parameters of 272 randomly selected adolescents from a large-scale cross-sectional study (n = 2727)...
January 31, 2018: Pediatric Diabetes
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