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pediatrics, thrombocytopenia

Kriti Mohan
BACKGROUND: Malaria is an important cause of death and illness in children worldwide. Most cases of neonatal malaria are misdiagnosed because of the lack of specific symptoms and a general lack of awareness. Nothing much is known in literature about the haematological changes during malaria infection and outcome of disease in neonates. Neonatal malaria is an underdiagnosed entity. So this hospital based observational study aims to assess diagnostic features of neonatal malaria. METHODS: From August 2004 to August 2013, information of all slide positive for malaria cases aged 0 to 28 days admitted to our pediatric hospital was collected and analysed...
October 20, 2016: Infectious Disorders Drug Targets
Osman Yeşilbaş, Hasan Serdar Kıhtır, Hamdi Murat Yıldırım, Nevin Hatipoğlu, Esra Şevketoğlu
BACKGROUND: Leptospirosis is a zoonotic infectious disease caused by pathogenic spirochetes of the genus Leptospira. Although it is usually asymptomatic and self-limited, severe potentially fatal illness accompanied by multi-organ failure may occur. CASE REPORT: Here we report an unusual case of severe leptospirosis successfully treated with continuous venovenous hemofiltration (CVVHF) and therapeutic plasma exchange (TPE). The patient presented with pericardial tamponade, renal failure and macrophage activation syndrome, and later suffered prolonged jaundice and sclerosing cholangitis during hospitalization in the pediatric intensive care unit (PICU)...
September 2016: Balkan Medical Journal
Fabio de Araujo Motta, Libera Maria Dalla-Costa, Marisol Dominguez Muro, Andrea Lenzi, Gledson Luiz Picharski, Marion Burger
BACKGROUND: Candida species are the primary cause of invasive fungal infection in hospitalized children. There are few data on risk factors for postoperative candidemia in pediatric patients with congenital heart defects. This study aimed to identify risk factors for candidemia in patients with congenital heart defects who underwent cardiac surgery. METHODS: This was a case-control study conducted in patients admitted to a pediatric cardiology intensive care unit from January 2006 to December 2013...
November 2016: Pediatric Infectious Disease Journal
Anne-Sylvia Sacri, Annelyse Bruwier, Geneviève Baujat, Sylvain Breton, Stéphane Blanche, Tracy A Briggs, Brigitte Bader-Meunier
Childhood-onset chronic and refractory cytopenias are rare and may be genetic in etiology. We report three pediatric cases of severe autoimmune thrombocytopenia or anemia associated with growth retardation and spastic diplegia with intracranial calcification. The identification of platyspondyly and metaphyseal lesions suggested a potential diagnosis of spondyloenchondrodysplasia (SPENCD), which was confirmed with the identification of biallelic ACP5 mutations. Two patients demonstrated elevated serum interferon alpha levels...
October 8, 2016: Pediatric Blood & Cancer
Andrew D J Pearson, Sara M Federico, Isabelle Aerts, Darren R Hargrave, Steven G DuBois, Robert Iannone, Ryan D Geschwindt, Ruixue Wang, Frank G Haluska, Tanya M Trippett, Birgit Geoerger
PURPOSE: Ridaforolimus is an investigational, potent, selective mTOR inhibitor. This study was conducted to determine the recommended phase 2 dose (RP2D), maximum tolerated dose, safety, pharmacokinetics, and antitumor activity of oral ridaforolimus in children with advanced solid tumors. EXPERIMENTAL DESIGN: In this phase 1, multicenter, open-label study in children aged 6 to <18 years with advanced solid tumors, ridaforolimus was administered orally for 5 consecutive days/week in 28-day cycles until progression, unacceptable toxicity, or consent withdrawal...
October 4, 2016: Oncotarget
Thomas Kühne
Evidence-based medicine is growing in immune thrombocytopenia (ITP), but solid clinical data are still lacking in many areas. A majority of children has self-limited ITP, but chronic symptomatic ITP exists also in pediatrics. Management includes a watch-and-wait strategy for children with newly diagnosed ITP and no or mild bleeding, and immunoglobulins and corticosteroids, if more bleeding and mucous membrane involvement is present. Treatment endpoints differ in clinical research and in clinical practice. The requirement of platelet enhancing drugs needs to be better defined in guidelines...
October 4, 2016: Hämostaseologie
Yingkang Jin, Li Huang, Huifeng Fan, Gen Lu, Yi Xu, Zhiyuan Wu
Hemophagocytic lymphohistiocytosis (HLH) is a life-threatening immune disorder that may be inherited or secondary to infection, malignancy or rheumatological disease. The aim of the present study was to highlight the clinical features of scrub typhus-associated HLH in children. A retrospective study was performed on 6 pediatric patients with scrub typhus-associated HLH. For each patient, medical records were reviewed and analyzed, and demographic, clinical and laboratory data and outcomes were collected. The duration of fever prior to admission ranged between 4 and 12 days...
October 2016: Experimental and Therapeutic Medicine
Shahrzad Bakhtiar, Frank Ruemmele, Fabienne Charbit-Henrion, Eva Lévy, Frédéric Rieux-Laucat, Nadine Cerf-Bensussan, Peter Bader, Ulrich Paetow
Monogenic primary immunodeficiency syndromes can affect one or more endocrine organs by autoimmunity during childhood. Clinical manifestations include type 1 diabetes mellitus, hypothyroidism, adrenal insufficiency, and vitiligo. Lipopolysaccharide (LPS)-responsive beige-like anchor protein (LRBA) deficiency was described in 2012 as a novel primary immunodeficiency, predominantly causing immune dysregulation and early onset enteropathy. We describe the heterogeneous clinical course of LRBA deficiency in two siblings, mimicking an autoimmune polyendocrine disorder in one of them in presence of the same underlying genetic mutation...
2016: Frontiers in Pediatrics
Philippe Cavailler, Arnaud Tarantola, Yee Sin Leo, Andrew A Lover, Anne Rachline, Moniboth Duch, Rekol Huy, Ai Li Quake, Yuvatha Kdan, Veasna Duong, Jeremy L Brett, Philippe Buchy
BACKGROUND: Dengue is endemic throughout Cambodia, a country faced with significant health and economic challenges. We undertook a clinical study at the National Paediatric Hospital in Phnom Penh to evaluate clinical diagnostic parameters for dengue and predictors of disease outcome. METHODS: Between September 2011 and January 2013, all consecutive inpatients aged between 1 and 15 years and presenting with suspected dengue were enrolled. They were clinically assessed using both the 1997 and 2009 WHO dengue classifications...
2016: BMC Infectious Diseases
Xin Yao, Chengrong Li, Jun Yang, Guobing Wang, Changgang Li, Yu Xia
OBJECTIVE: This study aims to investigate the role of T follicular helper (TFH) cells in the immunopathogenesis of pediatric immune thrombocytopenia (ITP), as well as differences in TFH expansion and its regulation between newly diagnosed ITP (nITP) and chronic pediatric ITP (cITP). METHODS: Eighty-five children with ITP and 20 age-matched healthy controls were enrolled into this study. TFH cell frequencies and TFH cell-associated regulatory factors before and after treatment were analyzed by flow cytometry, RT-PCR and ELISA...
October 2016: Blood Cells, Molecules & Diseases
Pooja Khandelwal, Chie Emoto, Tsuyoshi Fukuda, Alexander A Vinks, Lisa Neumeier, Christopher E Dandoy, Javier El-Bietar, Sharat Chandra, Stella M Davies, Jacob J Bleesing, Michael B Jordan, Parinda A Mehta, Sonata Jodele, Michael S Grimley, Ashish Kumar, Kasiani C Myers, Rebecca A Marsh
We describe a single-center prospective study of alemtuzumab as a second-line agent for steroid-refractory (SR) acute graft-versus-host disease (aGVHD) in pediatric and young adult allogeneic hematopoietic stem cell transplant recipients. Alemtuzumab was administered for grades II to IV aGVHD if patients did not improve within 5 days or worsened within 48 hours after corticosteroids. Interim analyses of alemtuzumab levels and response were performed after every 5 patients enrolled, resulting in 3 dosing cohorts, as follows: (1) ...
September 21, 2016: Biology of Blood and Marrow Transplantation
Rose M Ayoob, Andrew L Schwaderer
The most common acute glomerulonephritis in children is poststreptococcal glomerulonephritis (PSGN) usually occurring between 3 and 12 years old. Hypertension and gross hematuria are common presenting symptoms. Most PSGN patients do not experience complications, but rapidly progressive glomerulonephritis and hypertensive encephalopathy have been reported. This paper reports 17 patients seen in 1 year for PSGN including 4 with atypical PSGN, at a pediatric tertiary care center. Seventeen children (11 males), mean age of 8 years, were analyzed...
2016: International Journal of Nephrology
Daniel C Moreira, Christopher J Ng, Ralph Quinones, Xiayuan Liang, Dominic W Chung, Jorge Di Paola
Idiopathic systemic capillary leak syndrome (SCLS) is a rare process characterized by acute and recurrent episodes of vascular leakage with severe hypotension, hypoalbuminemia, hemoconcentration and edema. Anemia and thrombocytopenia are not part of this syndrome, but here we present the case of a pediatric patient with a clinical presentation consistent with SCLS who subsequently developed microangiopathic hemolytic anemia at a time when she had significant fluid loss and anasarca. Based on serial ADAMTS-13 levels, we propose that the anemia in this patent developed as a result of ADAMTS-13 loss in the third-space fluid, a novel mechanism for acquired microangiopathic hemolytic anemia...
September 13, 2016: Journal of Thrombosis and Haemostasis: JTH
Taro Yamazaki, Atsushi Shibuya, Saori Ishii, Nobuyuki Miura, Akira Ohtake, Nozomu Sasaki, Ryuichiro Araki, Yatio Ota, Mitsuhiro Fujiwara, Yuji Miyajima, Kimiaki Uetake, Keigo Hamahata, Koji Kato, Kiyoshi Kawakami, Hidemi Toyoda, Naohiko Moriguchi, Masahiko Okada, Masanori Nishi, Yoshiyasu Ogata, Tomohito Takimoto, Shouichi Ohga, Shigeru Ohta, Shin Amemiya
BACKGROUND: A nationwide, multicenter and observational study was retrospectively conducted to evaluate the clinical utility of cepharanthin (CEP) therapy for pediatric patients with chronic immune thrombocytopenia (ITP). METHODS: Clinical and laboratory data of 46 Japanese patients less than 16 years of age were analyzed, who were diagnosed as having chronic ITP in 14 hospitals during the 2001-2011 decade, and were treated with CEP for more than 12 months. RESULTS: The median daily dose of CEP was 1 mg/kg (0...
September 6, 2016: Pediatrics International: Official Journal of the Japan Pediatric Society
Heba M Gouda, Nermine M Kamel, Safa S Meshaal
BACKGROUND: DNA methylation is an epigenetic process that refers to chromatin-based mechanisms in the regulation of gene expression without DNA alternation. It is mediated by DNA methyltransferases (DNMTs). The DNA methyltransferase 3B (DNMT3B) gene contains a C-to-T single nucleotide polymorphism (SNP; rs2424913) in the Promotor region, 149 base pairs from the transcription start site, which is reported to significantly increase the Promotor activity. OBJECTIVE: To investigate the prevalance of rs2424913 single nucleotide polymorphism located in the DNMT3B gene Promotor...
November 2016: Laboratory Medicine
Nowneet Kumar Bhat, Vivek Ahuja, Minakshi Dhar, Sohaib Ahmad, Neerul Pandita, Vibha Gupta, Smita Chandra
Adult cases of visceral leishmaniasis (VL), predominantly males, have been reported in the past decade from natives of high altitude areas of North Indian state of Uttarakhand. We report 14 pediatric cases of VL, who were diagnosed and treated successfully over the past 7 years. All these children were born and brought up in this area and had never visited any of the endemic areas. High prevalence of pallor, splenohepatomegaly, thrombocytopenia and poor association with HIV are cardinal features of VL in this region...
August 30, 2016: Journal of Tropical Pediatrics
Hector R Wong, Natalie Z Cvijanovich, Nick Anas, Geoffrey L Allen, Neal J Thomas, Michael T Bigham, Scott L Weiss, Julie Fitzgerald, Paul A Checchia, Keith Meyer, Michael Quasney, Mark Hall, Rainer Gedeit, Robert J Freishtat, Jeffrey Nowak, Shekhar S Raj, Shira Gertz, Kelli Howard, Kelli Harmon, Patrick Lahni, Erin Frank, Kimberly W Hart, Trung C Nguyen, Christopher J Lindsell
OBJECTIVE: The Pediatric Sepsis Biomarker Risk Model (PERSEVERE), a pediatric sepsis risk model, uses biomarkers to estimate baseline mortality risk for pediatric septic shock. It is unknown how PERSEVERE performs within distinct septic shock phenotypes. We tested PERSEVERE in children with septic shock and thrombocytopenia-associated multiple organ failure (TAMOF), and in those without new onset thrombocytopenia but with multiple organ failure (MOF). DESIGN: PERSEVERE-based mortality risk was generated for each study subject (n = 660)...
November 2016: Critical Care Medicine
Geneviève Du Pont-Thibodeau, Marisa Tucci, Nancy Robitaille, Thierry Ducruet, Jacques Lacroix
OBJECTIVES: To characterize the determinants of platelet transfusion in a PICU and determine whether there exists an association between platelet transfusion and adverse outcomes. DESIGN: Prospective observational single center study, combined with a self-administered survey. SETTING: PICU of Sainte-Justine Hospital, a university-affiliated tertiary care institution. PATIENTS: All children admitted to the PICU from April 2009 to April 2010...
September 2016: Pediatric Critical Care Medicine
Michela Massoud, Francesca Del Bufalo, Anna Maria Caterina Musolino, Paolo Maria Schingo, Stefania Gaspari, Mara Pisani, Cinzia Orazi, Antonino Reale, Umberto Raucci
BACKGROUND: Low back pain is a common symptom in the pediatric population; approximately half of all children present with at least one episode of low back pain. The majority of cases are due to nonspecific causes such as musculoskeletal trauma with spontaneous regression. On some occasions, however, life-threatening diseases have to be considered. CASE REPORT: A 15-year-old girl presented to the Pediatric Emergency Department for a history of continuous 2-day duration of low back pain and transient paresthesia of the right gluteal area...
September 2016: Journal of Emergency Medicine
Saimun Singla, Debra L Canter, Timothy J Vece, Eyal Muscal, Marietta DeGuzman
BACKGROUND: Diffuse alveolar hemorrhage (DAH) is a devastating clinical syndrome characterized by a falling hematocrit, respiratory insufficiency, and radiographic evidence of pulmonary infiltrates. Literature regarding management of DAH in childhood-onset SLE (cSLE) is limited. METHODS: We reviewed the presentation, management, and outcome of DAH in a pediatric tertiary medical center with one of the largest cSLE cohorts in North America. During a 10 year period 7 of 410 children with cSLE had DAH...
August 2016: Hospital Pediatrics
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