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https://www.readbyqxmd.com/read/29561985/review-of-celiac-disease-presentation-in-a-pediatric-tertiary-centre
#1
Gracinda Nogueira Oliveira, Rajiv Mohan, Andrew Fagbemi
BACKGROUND: Celiac disease is an immune-mediated disorder with a multiform presentation and therefore a challenging diagnosis. OBJECTIVE: Our purpose is to identify the epidemiological, clinical, laboratory and histologic characteristics of children with celiac disease at diagnosis and on follow-up. METHODS: Children with previously established or newly diagnosed celiac disease, admitted in a tertiary centre in a two-year period (2014-2016) were recruited...
January 2018: Arquivos de Gastroenterologia
https://www.readbyqxmd.com/read/29479942/recent-perspectives-of-pediatric-%C3%AE-thalassaemias
#2
Qi An, Conghai Fan, Shumei Xu
β-thalassaemia is a potentially lethal hereditary anaemia, caused by reduced or absent expression of HBB polypeptide chains of adult haemoglobin (HbA: α2β2). Current curative treatments options are limited to few patients, while alternative, chronic palliative therapy consisting of frequent transfusions coupled with iron chelation therapy, are costly. The above treatments also affect quality of life of patients. A search was conducted in the electronic databases like medline, pubmed, etc. for screening studies reporting various aspects including gene therapy, prevention strategies, blood, transfusion and chelation therapy for the management of β-thalassaemia...
February 23, 2018: Minerva Pediatrica
https://www.readbyqxmd.com/read/29451224/hypogonadism-in-male-thalassemia-major-patients-pathophysiology-diagnosis-and-treatment
#3
Vincenzo De Sanctis, Ashraf T Soliman, Mohamed A Yassin, Salvatore Di Maio, Shahina Daar, Heba Elsedfy, Nada Soliman, Christos Kattamis
Failure of pubertal growth, delay or absence of sexual development, infertility and sexual dysfunction due to hypogonadism and defective spermatogenesis are frequent and well recognized disturbances among male patients with transfusion dependent (TD) thalassaemia major (β-thal). These problems are attributed mainly to the damage caused by chronic anaemia and the deposition of excess iron in the pituitary gland and testicles. This is a short review of male pubertal disorders in patients with β-thal written by pediatric endocrinologists and haematologists with an interest and active involvement, in the diagnosis and management of these complications in this group of patients...
February 16, 2018: Acta Bio-medica: Atenei Parmensis
https://www.readbyqxmd.com/read/29447888/pathogenesis-of-chronic-rhinosinusitis-in-patients-affected-by-%C3%AE-thalassemia-major-and-sickle-cell-anaemia-post-allogenic-bone-marrow-transplant
#4
F Martino, R Di Mauro, K Paciaroni, J Gaziev, C Alfieri, L Greco, R Floris, S Di Girolamo, M Di Girolamo
OBJECTIVES: Sickle cell anemia (SCA) and β -thalassemia major are well-recognized beta-globin gene disorders of red blood cells associated to mortality and morbidity included bone morbidities due to ineffective erythropoiesis and bone marrow expansion, which affect every part of the skeleton. While there are an abundance of described disease manifestations of the head and neck, the manner of paranasal sinuses involvement and its relations to β-thalassemia and SCA process was not studied yet...
March 2018: International Journal of Pediatric Otorhinolaryngology
https://www.readbyqxmd.com/read/29378711/population-pharmacokinetics-of-cefotaxime-and-dosage-recommendations-in-children-with-sickle-cell-disease
#5
Elsa Maksoud, Berengere Koehl, Aude Facchin, Phuong Ha, Wei Zhao, Florentia Kaguelidou, Malika Benkerrou, Patricia Mariani, Albert Faye, Mathie Lorrot, Evelyne Jacqz-Aigrain
The pharmacokinetic profile of most drugs is dependent on the patient's covariates and may be influenced by the disease. Cefotaxime is frequently prescribed in pediatric patients with sickle cell disease (SCD), characterized by vaso-occlusive complications, chronic hemolytic anemia, and a defective immunological function predisposing the individual to severe infection. Data on the impact of the disease on the disposition of cefotaxime are missing. In the present study, our aims were to determine cefotaxime pharmacokinetics when prescribed to children with SCD for suspected or proven bacterial infection, identify significant covariates, and perform Monte Carlo simulations to optimize the drug dosage...
April 2018: Antimicrobial Agents and Chemotherapy
https://www.readbyqxmd.com/read/29166891/pediatric-hospital-admissions-in-indigenous-children-a-population-based-study-in-remote-australia
#6
Philippa J Dossetor, Alexandra L C Martiniuk, James P Fitzpatrick, June Oscar, Maureen Carter, Rochelle Watkins, Elizabeth J Elliott, Heather E Jeffery, David Harley
BACKGROUND: We analysed hospital admissions of a predominantly Aboriginal cohort of children in the remote Fitzroy Valley in Western Australia during the first 7 years of life. METHODS: All children born between January 1, 2002 and December 31, 2003 and living in the Fitzroy Valley in 2009-2010 were eligible to participate in the Lililwan Project. Of 134 eligible children, 127 (95%) completed Stage 1 (interviews of caregivers and medical record review) in 2011 and comprised our cohort...
November 22, 2017: BMC Pediatrics
https://www.readbyqxmd.com/read/28596707/red-cell-distribution-width-rdw-as-a-biomarker-for-respiratory-failure-in-a-pediatric-icu
#7
Tom Schepens, Jozef J De Dooy, Walter Verbrugghe, Philippe G Jorens
BACKGROUND: The red cell distribution width (RDW) is a widely available, inexpensive, and highly reproducible test that reflects the range of the red cell sizes. Any process that releases reticulocytes in the circulation will result in an increase in RDW. Elevated RDW values are linked to worsened pulmonary function in the adult population. We performed a retrospective cohort study to describe the association between RDW and respiratory failure in critically ill children in a in a pediatric intensive care unit (PICU) in a tertiary university hospital...
2017: Journal of Inflammation
https://www.readbyqxmd.com/read/27774886/the-clinico-hematological-features-and-management-outcome-in-neonatal-malaria-a-nine-years-analysis-from-north-india
#8
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
https://www.readbyqxmd.com/read/27086352/phase-1-study-of-clofarabine-in-pediatric-patients-with-relapsed-refractory-acute-lymphoblastic-leukemia-in-japan
#9
Katsuyoshi Koh, Chitose Ogawa, Yasuhiro Okamoto, Kazuko Kudo, Jiro Inagaki, Tsuyoshi Morimoto, Hideya Mizukami, Evelyne Ecstein-Fraisse, Atsushi Kikuta
A phase 1 study was conducted to evaluate the safety, pharmacokinetics (PK), efficacy and pharmacogenetic characteristics of clofarabine in seven Japanese pediatric patients with relapsed/refractory acute lymphoblastic leukemia (ALL). Patients in Cohort 1 received clofarabine 30 mg/m(2)/day for 5 days, followed by 52 mg/m(2)/day for 5 days in subsequent cycles. Cohort 2 patients were consistently treated with 52 mg/m(2)/day for 5 days. No more than six cycles were performed. Every patient had at least one ≥Grade 3 adverse event (AE)...
August 2016: International Journal of Hematology
https://www.readbyqxmd.com/read/26692024/chronic-haemodialysis-in-small-children-a-retrospective-study-of-the-italian-pediatric-dialysis-registry
#10
MULTICENTER STUDY
Fabio Paglialonga, Silvia Consolo, Carmine Pecoraro, Enrico Vidal, Bruno Gianoglio, Flora Puteo, Stefano Picca, Maria Teresa Saravo, Alberto Edefonti, Enrico Verrina
BACKGROUND: Chronic haemodialysis (HD) in small children has not been adequately investigated. METHODS: This was a retrospective investigation of the use of chronic HD in 21 children aged  <2 years (n = 12 aged <1 year) who were registered in the Italian Pediatric Dialysis Registry. Data collected over a period of >10 years were analysed. RESULTS: The median age of the 21 children at start of HD was 11.4 [interquartile range (IQR) 6...
May 2016: Pediatric Nephrology: Journal of the International Pediatric Nephrology Association
https://www.readbyqxmd.com/read/25847050/hydroxyurea-lowers-transcranial-doppler-flow-velocities-in-children-with-sickle-cell-anaemia-in-a-nigerian-cohort
#11
COMPARATIVE STUDY
IkeOluwa Lagunju, Biobele J Brown, Olugbemiro Sodeinde
BACKGROUND: Sickle cell anaemia (SCA) is the leading genetic disorder in Nigeria. Elevated velocities ≥170 cm/sec occur in about a third of Nigerian children with SCA. Chronic blood transfusion for stroke prevention is faced with a myriad of challenges in our practice. OBJECTIVES: To evaluate the effectiveness of hydroxyurea (HU) in reducing flow velocities in a cohort of Nigerian children with SCA and elevated velocities treated with HU. METHODS: An observational study was carried out on a cohort of Nigerian children with SCA and elevated velocities identified on routine transcranial Doppler (TCD) screening...
September 2015: Pediatric Blood & Cancer
https://www.readbyqxmd.com/read/25682760/oral-leukoplakia-in-patients-with-fanconi-anaemia-without-hematopoietic-stem-cell-transplantation
#12
Laura Grein Cavalcanti, Karine Fátima Lyko, Renata Lins Fuentes Araújo, José Miguel Amenábar, Carmem Bonfim, Cassius Carvalho Torres-Pereira
BACKGROUND: Fanconi anaemia is a genetic disease characterized by congenital abnormalities, progressive bone marrow failure, and a higher predisposition of oral squamous cell carcinoma. The purpose of this study was to evaluate the prevalence of oral mucosa lesions in patients with Fanconi anaemia without hematopoietic stem cell transplantation (HSCT). PROCEDURE: Patients with Fanconi anaemia who had not undergone HSCT was cross-sectional evaluated for the presence of oral lesions...
June 2015: Pediatric Blood & Cancer
https://www.readbyqxmd.com/read/25628902/sirolimus-based-immunosuppression-as-gvhd-prophylaxis-after-bone-marrow-transplantation-for-severe-aplastic-anaemia-a-case-report-and-review-of-the-literature
#13
Katia Perruccio, Elena Mastrodicasa, Francesco Arcioni, Ilaria Capolsini, Carla Cerri, Grazia Gurdo, Maurizio Caniglia
Congenital or acquired severe aplastic anaemia (SAA) is cured by bone marrow transplantation (BMT) from a histocompatible leukocyte antigen- (HLA-) identical sibling. The best conditioning regimen is cyclophosphamide (CTX) with or without antithymocyte globulin (ATG), followed by short-term methotrexate (MTX) and cyclosporine A (CsA) to prevent graft-versus-host disease (GvHD). In our pediatric oncology-hematology unit, a 5-year-old girl with SAA was treated with two BMT from the same HLA-identical sibling donor...
2015: Case Reports in Hematology
https://www.readbyqxmd.com/read/25326048/effectiveness-and-safety-of-ferric-carboxymaltose-treatment-in-children-and-adolescents-with-inflammatory-bowel-disease-and-other-gastrointestinal-diseases
#14
Martin W Laass, Simon Straub, Suki Chainey, Garth Virgin, Timothy Cushway
BACKGROUND: The treatment of iron deficiency anemia in children with inflammatory bowel disease is a particular challenge and often insufficient. Absorption of orally given iron may be impaired by intestinal inflammation and treatment with oral iron may aggravate intestinal inflammation. This retrospective study is the first to describe the use of intravenous ferric carboxymaltose (FCM) in the pediatric setting. METHODS: All subjects who had received at least one dose of FCM intravenously in the observation period were included in this analysis with data collected for up to 3 months post last FCM dose...
2014: BMC Gastroenterology
https://www.readbyqxmd.com/read/25142757/outcomes-of-human-immunodeficiency-virus-infected-children-after-anti-retroviral-therapy-in-malaysia
#15
Fong Siew Moy, Paul Fahey, Nik K Nik Yusoff, Kamarul A Razali, Revathy Nallusamy
AIMS: To describe outcome and examine factors associated with mortality among human immunodeficiency virus (HIV)-infected children in Malaysia after anti-retroviral therapy (ART). METHODS: Retrospective and prospective data collected through March 2009 from children in four different states in Malaysia enrolled in TREAT Asia's Pediatric HIV Observational Database were analysed. RESULTS: Of 347 children in the cohort, only 278 (80.1%) were commenced on ART...
February 2015: Journal of Paediatrics and Child Health
https://www.readbyqxmd.com/read/24771408/early-erythropoietin-for-preventing-red-blood-cell-transfusion-in-preterm-and-or-low-birth-weight-infants
#16
REVIEW
Arne Ohlsson, Sanjay M Aher
BACKGROUND: Low plasma levels of erythropoietin (EPO) in preterm infants provide a rationale for the use of EPO to prevent or treat anaemia. OBJECTIVES: To assess the effectiveness and safety of early initiation of EPO or darepoetin (initiated before eight days after birth) in reducing red blood cell (RBC) transfusions in preterm and/orlow birth weight infants. SEARCH METHODS: The Cochrane Library, MEDLINE, EMBASE, CINAHL, reference lists of identified trials and reviews, Pediatric Academic Societies Annual meetings 2000 to 2013 (Abstracts2View(TM)) and clinical trials registries (clinicaltrials...
April 26, 2014: Cochrane Database of Systematic Reviews
https://www.readbyqxmd.com/read/24760628/late-erythropoietin-for-preventing-red-blood-cell-transfusion-in-preterm-and-or-low-birth-weight-infants
#17
REVIEW
Sanjay M Aher, Arne Ohlsson
BACKGROUND: Low plasma levels of erythropoietin (EPO) in preterm infants provide a rationale for the use of EPO to prevent or treat anaemia. OBJECTIVES: To assess the effectiveness and safety of late initiation of erythropoietin (EPO) between eight and 28 days after birth, in reducing the use of red blood cell (RBC) transfusions in preterm and/or low birth weight infants. SEARCH METHODS: We searched the Cochrane Central Register of Controlled Trials (CENTRAL), MEDLINE, EMBASE, and CINAHL in July 2013...
April 23, 2014: Cochrane Database of Systematic Reviews
https://www.readbyqxmd.com/read/24719649/vitamin-b12-deficiency-presenting-as-pancytopenia-and-retinopathy-in-a-young-boy-helicobacter-pylori-a-novel-causative-agent
#18
Palaniyandi Anitha, Rajendraprasad Sasitharan, Thalavaisundaram Thambarasi, Prabaharan Krithika, Madhuvanthi Mohan, Paramasivam Venkataraman, Saji James, Ponnurangam Nagarajan Vinoth
Deficiency of vitamin B12 (cobalamin) is a well-known cause of megaloblastic anaemia. It is a reversible cause of bone marrow failure and demyelinating nervous system disorder, hence early detection and prompt treatment of vitamin B12 deficiency is essential. After diagnosing vitamin B12 deficiency, tracking down its root cause is important in individualising the treatment approach. Helicobacter pylorirelated (H. pylori) B12 deficiency presenting as pancytopenia in pediatric age groups has been reported. However, vitamin B12 deficiency presenting as retinopathy in paediatric age groups has been rarely reported in the medical literature...
2014: Australasian Medical Journal
https://www.readbyqxmd.com/read/24712262/-blood-volume-for-biochemistry-determinations-laboratory-needs-and-everyday-practice
#19
Krystyna Sztefko, Katarzyna Mamica, Jolanta Bugajska, Barbara Maziarz, Przemysław Tomasik
UNLABELLED: Blood loss due to diagnostic phlebotomy jest a very serious problem, especially for newborn, infants and critically ill patients on intensive care units. Although single blood loss can be easily tolerated in adults, in small babies and in patients who are frequently monitored based on laboratory tests iatrogenic anaemia can occur. AIM OF THE STUDY: To evaluate the blood volume drawn for routine biochemistry tests in relation to patient age and the number of parameters requested...
2014: Przegla̧d Lekarski
https://www.readbyqxmd.com/read/24516760/frequency-of-glomerular-dysfunction-in-children-with-beta-thalassaemia-major
#20
Basma A Ali, Ahmed M Mahmoud
OBJECTIVES: This study investigated the frequency of glomerular dysfunction in children with beta thalassaemia major (β-TM) by using different markers and correlating them with serum ferritin and iron chelation therapy. METHODS: The study, carried out between August 2011 and May 2012, included 100 patients with β-TM, in two groups. Group Ia (n = 62) received chelation therapy (deferoxamine). Group Ib (n = 38) received follow-up care at the Pediatric Hematology Outpatient Clinic, Minia University Children's Hospital, Egypt...
February 2014: Sultan Qaboos University Medical Journal
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