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https://www.readbyqxmd.com/read/29783824/-clinical-analysis-of-seven-cases-of-rare-hemolytic-disease-of-the-newborn
#1
S Gu, H X Wang, C Y Yang, X F Yang, Y Lin, Y Zhong, J P He, Y J Wang
Objective: To summarize the clinical features of 7 rare cases of hemolytic disease of newborn (HDN), and to improve the understanding of rare HDN. Methods: Data of clinical information, laboratory findings, treatments and outcomes were collected and analyzed for four cases with HDN due to anti-M, two cases due to anti-Kidd, and one case due to anti-Duffy. All of them were admitted to the Department of Neonatology, Beijing Children's Hospital Affiliated to Capital Medial University from July 2007 to June 2017...
May 2, 2018: Zhonghua Er Ke za Zhi. Chinese Journal of Pediatrics
https://www.readbyqxmd.com/read/29782390/suspecting-hyperferritinemic-sepsis-in-iron-deficient-population-do-we-need-a-lower-plasma-ferritin-threshold
#2
Swarup Ghosh, Arun K Baranwal, Prateek Bhatia, Karthi Nallasamy
OBJECTIVES: Hyperferritinemia is being suggested to identify patients with sepsis-induced macrophage activation syndrome for early intervention. However, data among iron-deficient children are scarce. This study was planned to explore the biological behavior of plasma ferritin in children from communities with a high frequency of iron deficiency with septic shock and its association with the outcome. DESIGN: Prospective observational study. SETTING: Tertiary care teaching hospital in a low-middle income economy of South Asia...
May 18, 2018: Pediatric Critical Care Medicine
https://www.readbyqxmd.com/read/29781568/diastolic-dysfunction-is-associated-with-exercise-impairment-in-patients-with-sickle-cell-anemia
#3
Tarek Alsaied, Omar Niss, Adam W Powell, Robert J Fleck, James F Cnota, Clifford Chin, Punam Malik, Charles T Quinn, Michael D Taylor
BACKGROUND: Left ventricular diastolic dysfunction (DD) is an independent risk factor for mortality in sickle cell anemia (SCA) and is associated with increased extracellular volume (ECV) on cardiac MRI (CMR). Exercise impairment is common in SCA, but its causes and prognostic value are not well understood. OBJECTIVE: To study the effects of DD and ECV on cardiopulmonary exercise test (CPET) in patients with SCA. METHODS AND RESULTS: As part of a prospective study to characterize the cardiomyopathy of SCA (NCT02410811), 20 children and adults with SCA underwent CMR, echocardiography, and cycle ergometer CPET (age range 8-43 years)...
May 21, 2018: Pediatric Blood & Cancer
https://www.readbyqxmd.com/read/29778393/fertility-preservation-in-pediatric-female-cancer-patients
#4
Sara E Arian, Rebecca L Flyckt, Richard Herman, Hadi Erfani, Tommaso Falcone
OBJECTIVE: To describe and demonstrate ovarian tissue cryopreservation (OTC) as an emerging fertility preservation technique DESIGN: Video presentation. SETTING: University hospital. PATIENT(S): A 6 year-old female patient diagnosed with aplastic anemia with plan for bone marrow transplantation underwent laparoscopic unilateral oophorectomy in conjunction with surgical procedure for port placement by the pediatric surgeon, followed by cryopreservation of ovarian tissue...
May 2018: Fertility and Sterility
https://www.readbyqxmd.com/read/29773497/indications-for-red-cell-transfusions-in-pediatric-patients
#5
REVIEW
Jill M Cholette, Suzie A Noronha, Jerard Seghatchian, Neil Blumberg
Red cell transfusions are amongst the most common therapeutic procedures in seriously ill children, particularly in the inpatient setting. This is despite the fact that there is no evidence base for most clinical settings, with the exception of patients with hemoglobinopathies, particularly thalassemia and sickle cell anemia. Obviously exsanguinating hemorrhage and life threatening anemia are urgent indications for which no other therapeutic approach is currently available. Most transfusions are, however, given prophylactically to prevent the complications of hypoxia or hemodynamic stability, based upon expert opinion and a faith in the oxygen carrying capacity and beneficial hemodynamic properties of transfused red cells...
May 10, 2018: Transfusion and Apheresis Science
https://www.readbyqxmd.com/read/29772478/bacillus-thuringiensis-cry5b-protein-as-a-new-pan-hookworm-cure
#6
Yan Hu, Thanh-Thanh Nguyen, Alice C Y Lee, Joseph F Urban, Melanie M Miller, Bin Zhan, David J Koch, Jason B Noon, Ambily Abraham, Ricardo Toshio Fujiwara, Dwight D Bowman, Gary R Ostroff, Raffi V Aroian
Hookworms are intestinal nematode parasites that infect nearly half a billion people and are globally one of the most important contributors to iron-deficiency anemia. These parasites have significant impacts in developing children, pregnant women and working adults. Of all the soil-transmitted helminths or nematodes (STNs), hookworms are by far the most important, with disease burdens conservatively estimated at four million DALYs (Disability-Adjusted Life Years) and with productivity losses of up to US$139 billion annually...
May 4, 2018: International Journal for Parasitology, Drugs and Drug Resistance
https://www.readbyqxmd.com/read/29767699/impact-of-double-fortified-salt-with-iron-and-iodine-on-hemoglobin-anemia-and-iron-deficiency-anemia-a-systematic-review-and-meta-analysis
#7
María J Ramírez-Luzuriaga, Leila M Larson, Venkatesh Mannar, Reynaldo Martorell
Double-fortified salt (DFS) containing iron and iodine has been proposed as a feasible and cost-effective alternative for iron fortification in low- and middle-income countries (LMICs). We conducted a systematic review and meta-analysis from randomized and quasi-randomized controlled trials to 1) assess the effect of DFS on biomarkers of iron status and the risk of anemia and iron deficiency anemia (IDA) and 2) evaluate differential effects of DFS by study type (efficacy or effectiveness), population subgroups, iron formulation (ferrous sulfate, ferrous fumarate, and ferric pyrophosphate), iron concentration, duration of intervention, and study quality...
May 1, 2018: Advances in Nutrition
https://www.readbyqxmd.com/read/29747664/adverse-effects-in-children-exposed-to-maternal-hiv-and-antiretroviral-therapy-during-pregnancy-in-brazil-a-cohort-study
#8
Adriane M Delicio, Giuliane J Lajos, Eliana Amaral, Fernanda Cavichiolli, Marina Polydoro, Helaine Milanez
BACKGROUND: Antiretroviral therapy (ART) in pregnancy was associated with a drastic reduction in HIV mother-to-child transmission (MTCT), although it was associated with neonatal adverse effects. The aim of this study was to evaluate the neonatal effects to maternal ART. METHODS: This study was a cohort of newborns from HIV pregnant women followed at the CAISM/UNICAMP Obstetric Clinic from 2000 to 2015. The following adverse effects were evaluated: anemia, thrombocytopenia, liver function tests abnormalities, preterm birth, low birth weight and congenital malformation...
May 10, 2018: Reproductive Health
https://www.readbyqxmd.com/read/29744996/hematopoietic-stem-cell-transplantation-without-in-vivo-t-cell-depletion-for-pediatric-aplastic-anemia-a-single-center-experience
#9
Sidan Li, Bin Wang, Lingling Fu, Yilin Pang, Guanghua Zhu, Xuan Zhou, Jie Ma, Yan Su, Maoquan Qin, Runhui Wu
For young patients, HLA-MRD HSCT is the first-line treatment of SAA. However, due to China's birth control policy, few patients could find suitable sibling donors and HLA-MUD. More and more transplantation centers have used Haplo-D as the donor source for young adult and pediatric patients. However, studies with larger amount of pediatric patients are rare. We retrospectively analyzed the data of children with AA who were treated with allogeneic HSCT and compared the therapeutic efficacy of Haplo-HSCT and MRD/MUD group...
May 10, 2018: Pediatric Transplantation
https://www.readbyqxmd.com/read/29729108/high-prevalence-of-anemia-in-10-month-old-japanese-infants-with-breastfeeding
#10
Masahiko Kimura, Youichi Kurozawa, Yumi Saito, Hiroshi Watanabe, Ayame Kobayashi, Takeshi Taketani
BACKGROUND: Anemia in infancy is still prevalent in developing countries. Commercial iron-fortified complementary foods or iron drops are not available in Japan and breastfed infants have a higher risk of anemia. We studied anemia screening in infants in 10-month old infants and evaluated whether breastfeeding is a risk factor for anemia. METHODS: Anemia screening was performed during a regular health check of 10-month children at four local pediatric clinics in Shimane prefecture, Japan...
May 5, 2018: Pediatrics International: Official Journal of the Japan Pediatric Society
https://www.readbyqxmd.com/read/29712865/mechanism-for-survival-of-homozygous-nonsense-mutations-in-the-tumor-suppressor-gene-brca1
#11
Aaron Seo, Orna Steinberg-Shemer, Sule Unal, Silvia Casadei, Tom Walsh, Fatma Gumruk, Stavit Shalev, Akiko Shimamura, Nurten Ayse Akarsu, Hannah Tamary, Mary-Claire King
BRCA1 is essential for repair of DNA double-strand breaks by homologous recombination, and hence for survival. Complete loss of its function is lethal during early embryonic development. Patients who are compound heterozygous for BRCA1 truncating mutations and missense alleles that retain some DNA repair capacity may survive, albeit with very high risk of early onset breast or ovarian cancer and features of Fanconi anemia. However, a mechanism enabling survival of patients homozygous for BRCA1 truncating mutations has not been described...
April 30, 2018: Proceedings of the National Academy of Sciences of the United States of America
https://www.readbyqxmd.com/read/29702541/novel-variations-of-fanca-gene-provokes-fanconi-anemia-molecular-diagnosis-in-a-special-chinese-family
#12
Niu Li, Aiyun Song, Lixia Ding, Hua Zhu, Guoqiang Li, Yan Miao, Jian Wang, Benshang Li, Jing Chen
Fanconi anemia (FA) is a rare autosomal recessive or X-linked disorder with highly variable clinical manifestations and an incidence of ∼1 to 5 in 1 million births. To date, 15 bona fide FA genes have been reported to be responsible for the known FA complementation groups and the FANCA gene accounts for almost 60%. In the present study, we report a special Chinese family, which has 2 children with classic FA characteristics. Via 2-step analysis of the whole-exome sequencing data and verification using multiplex ligation-dependent probe amplification test, one child was found to have a novel compound heterozygous mutation of a splicing variant (c...
April 26, 2018: Journal of Pediatric Hematology/oncology
https://www.readbyqxmd.com/read/29695301/health-in-yemen-losing-ground-in-war-time
#13
Charbel El Bcheraoui, Aisha O Jumaan, Michael L Collison, Farah Daoud, Ali H Mokdad
BACKGROUND: The effect of the ongoing war in Yemen on maternal and child health (MCH) has not been comprehensively assessed. Providing a situational analysis at the governorate level is critical to assist in planning a response and allocating resources. METHODS: We used multiple national- and governorate-level data sources to provide estimates of 12 relevant MCH indicators in 2016 around child vaccination, and child and maternal nutritional status, and the change in these estimates for the period 2013-2016 based on shock variables including change in gross domestic product, burden of airstrikes per 1000 population, change in access to untreated water sources and unimproved toilets, and change in wheat flour prices...
April 25, 2018: Globalization and Health
https://www.readbyqxmd.com/read/29685474/the-evaluation-and-management-of-rocky-mountain-spotted-fever-in-the-emergency-department-a-review-of-the-literature
#14
Michael Gottlieb, Brit Long, Alex Koyfman
BACKGROUND: Rocky Mountain spotted fever (RMSF) is potentially deadly and can present subtly with signs and symptoms overlapping with other clinical conditions. Delayed diagnosis can be fatal. OBJECTIVE: This review provides an evidence-based summary of the current data for the evaluation and management of RMSF in the emergency department. DISCUSSION: RMSF occurs through transmission of Rickettsia rickettsii by an infected tick. Exposure in the United States occurs most commonly from April to September, and high-risk locations include wooded, shrubby, or grassy areas...
April 20, 2018: Journal of Emergency Medicine
https://www.readbyqxmd.com/read/29677086/hematologic-adverse-events-associated-with-prolonged-valganciclovir-treatment-in-congenital-cytomegalovirus-infection
#15
Liron Ziv, Joanne Yacobovich, Joseph Pardo, Havatzelet Yarden-Bilavsky, Jacob Amir, Micky Osovsky, Efraim Bilavsky
BACKGROUND: Valganciclovir (2/d) therapy for 6 months in neonates with symptomatic congenital cytomegalovirus infection improves hearing and neurodevelopmental outcome. The only reported adverse event was neutropenia. Since 2009, our protocol for symptomatic congenital cytomegalovirus infection was a 1-year treatment of 2/d for the first 3 months followed by 9 months of 1/d. METHODS: A retrospective study. Infants with congenital cytomegalovirus treated with valganciclovir for 1 year were recruited...
April 19, 2018: Pediatric Infectious Disease Journal
https://www.readbyqxmd.com/read/29672357/refugee-children-s-participation-in-the-women-infants-and-children-supplemental-nutrition-wic-program-in-massachusetts-1998-2010
#16
Laura Smock, Thinh Nguyen, Elizabeth Metallinos-Katsaras, Hema Magge, Jennifer Cochran, Paul L Geltman
OBJECTIVES: To (1) describe prevalence of growth abnormalities and anemia in refugee children; (2) describe the proportion of age-eligible refugee children enrolled in Special Supplemental Nutrition Program for Women, Infants, and Children (WIC); and (3) identify risk factors for lack of enrollment in WIC. DESIGN: Data were collected from 1731 health screenings for refugee children younger than 5 years in Massachusetts in 1998-2010 and matched to WIC program records...
April 18, 2018: Journal of Public Health Management and Practice: JPHMP
https://www.readbyqxmd.com/read/29668982/improved-medical-treatment-and-surgical-surveillance-of-children-and-adolescents-with-ulcerative-colitis-in-the-united-kingdom
#17
Marcus Karl-Keinz Auth, Su K Bunn, Aimee Leanne Protheroe, Linda Jane Williams, John M Fell, Rafeeq Muhammed, Nicholas Michael Croft, R Mark Beattie, Anne Willmott, Christine Spray, Babu Vadamalayan, Astor Rodrigues, John Puntis, Anna Jane Pigott, David C Wilson, Sally Mitton, Mark Furman, Charlie Charlton, Sonny K F Chong, Richard K Russell
Background: Pediatric ulcerative colitis (UC) presents at an earlier age and increasing prevalence. Our aim was to examine morbidity, steroid sparing strategies, and surgical outcome in children with active UC. Methods: A national prospective audit was conducted for the inpatient period of all children with UC for medical or surgical treatment in the United Kingdom (UK) over 1 year. Thirty-two participating centers recruited 224 children in 298 admissions, comparisons over 6 years were made with previous audits...
April 13, 2018: Inflammatory Bowel Diseases
https://www.readbyqxmd.com/read/29668553/glucose-homeostasis-markers-in-beta-thalassemia
#18
Sunil Gomber, Anjali Bagaria, Sri V Madhu, Pooja Dewan
BACKGROUND: Glycosylated hemoglobin (HbA1c) has been a well-recognized marker of glucose homeostasis among thalassemics. Recently some studies have proposed the role of fructosamine as a better marker as compared with HbA1c. Hence, the study was carried out to find out which marker holds promise among Indian beta-thalassemic children. METHODS: In this case-control study, 60 diagnosed cases of beta-thalassemia major and equal number of normal controls who were ≥8 years of age were enrolled...
April 17, 2018: Journal of Pediatric Hematology/oncology
https://www.readbyqxmd.com/read/29664198/hemoglobin-a-clearance-in-children-with-sickle-cell-anemia-on-chronic-transfusion-therapy
#19
Marianne E M Yee, Cassandra D Josephson, Anne M Winkler, Jennifer Webb, Naomi L C Luban, Traci Leong, Sean R Stowell, John D Roback, Ross M Fasano
BACKGROUND: Chronic transfusion therapy for sickle cell anemia reduces disease complications by diluting sickle-erythrocytes with hemoglobin A (HbA)-containing erythrocytes and suppressing erythropoiesis. Minor antigen mismatches may result in alloimmunization, but it is unknown if antigen mismatches or recipient characteristics influence HbA clearance posttransfusion. STUDY DESIGN AND METHODS: Children with sickle cell anemia on chronic transfusion therapy were followed prospectively for 12 months...
April 17, 2018: Transfusion
https://www.readbyqxmd.com/read/29663621/neurodevelopmental-outcomes-of-young-children-born-to-hiv-positive-mothers-in-rural-yunnan-china
#20
Jie Wu, Jing Li, Yan Li, Kek Khee Loo, Haixia Yang, Qiong Wang, Rufei Duan, Xia Xiao, Xiaoxiao Song, Siwei Yang, Lin Sun
BACKGROUND: Children born to HIV-infected mothers are at risk for neurodevelopmental delay. Little is known about neurodevelopmental outcomes of infants and toddlers who were born to HIV-positive mothers but were not themselves infected by HIV, especially in poor rural areas. This study was conducted to compare developmental outcomes between young children who were HIV-exposed but uninfected (HEU) and their HIV-unexposed and uninfected (HUU) peers in rural Yunnan, China. METHODS: A total of 250 HEU children aged 6-36 months and 250 HUU children matched on age, gender and residency were recruited from rural Yunnan, China...
April 16, 2018: Pediatrics International: Official Journal of the Japan Pediatric Society
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