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Giang-Kim T Nguyen, Angela Lewis, Carol Goldener, Brenda Reed, Robin Yates Dulman, Elizabeth Yang
Folic acid (FA) is commonly prescribed for patients with sickle cell anemia, but evidence for the efficacy of this practice is lacking. We stopped FA supplementation and measured red blood cell folate levels after discontinuation of FA in 72 patients with clinically severe forms of sickle cell disease. We compared hemoglobin and reticulocyte counts before and after FA discontinuation in 51 of those patients, the majority of whom were on hydroxyurea. No patients had red blood cell folate levels below normal and no significant difference in hemoglobin levels (P=0...
August 26, 2016: Journal of Pediatric Hematology/oncology
Rachel T Clarke, Ann Van den Bruel, Clare Bankhead, Christopher D Mitchell, Bob Phillips, Matthew J Thompson
OBJECTIVE: Leukaemia is the most common cancer of childhood, accounting for a third of cases. In order to assist clinicians in its early detection, we systematically reviewed all existing data on its clinical presentation and estimated the frequency of signs and symptoms presenting at or prior to diagnosis. DESIGN: We searched MEDLINE and EMBASE for all studies describing presenting features of leukaemia in children (0-18 years) without date or language restriction, and, when appropriate, meta-analysed data from the included studies...
October 2016: Archives of Disease in Childhood
Ilham Youssry, Samuel Makar, Rania Fawzy, Manal Wilson, Ghada AbdAllah, Eman Fathy, Happy Sawires
BACKGROUND: Given the burden and poor outcome of end-stage renal disease in sickle cell disease (SCD), early markers of sickle cell nephropathy (SN) are desirable. Disordered angiogenesis underlies many complications of SCD. We aimed to determine the relationship between serum FMS-like tyrosine kinase-1 (sFLT-1) and other biomarkers of renal damage for the early diagnosis of SN. METHODS: Forty-seven SCD patients and 49 healthy controls were enrolled. Microalbuminuria was determined in patient urine samples...
December 2015: Pediatric Nephrology: Journal of the International Pediatric Nephrology Association
Alexander Shimabukuro-Vornhagen, Boris Böll, Matthias Kochanek, Éli Azoulay, Michael S von Bergwelt-Baildon
Answer questions and earn CME/CNE The increasing prevalence of patients living with cancer in conjunction with the rapid progress in cancer therapy will lead to a growing number of patients with cancer who will require intensive care treatment. Fortunately, the development of more effective oncologic therapies, advances in critical care, and improvements in patient selection have led to an increased survival of critically ill patients with cancer. As a consequence, critical care has become an important cornerstone in the continuum of modern cancer care...
June 27, 2016: CA: a Cancer Journal for Clinicians
Raffaella Colombatti, Laura Sainati, Daniele Trevisanuto
Neonatal anemia is a frequent occurrence in neonatal intensive care units. Red blood cell transfusion criteria in case of blood loss are clearly defined but optimal hemoglobin or hematocrit thresholds of transfusion for anemia due to decreased production or increased destruction are less evident. This review focuses on the causes of anemia in the newborn period and the most recent evidence-based treatment options, including transfusion and erythropoiesis-stimulating agents.
February 2016: Seminars in Fetal & Neonatal Medicine
Lise J Estcourt, Patricia M Fortin, Sally Hopewell, Marialena Trivella, Ian R Hambleton, Gavin Cho
BACKGROUND: Sickle cell disease is a genetic haemoglobin disorder, which can cause severe pain, significant end-organ damage, pulmonary complications, and premature death. Sickle cell disease is one of the most common severe monogenic disorders in the world, due to the inheritance of two abnormal haemoglobin (beta globin) genes. The two most common chronic chest complications due to sickle cell disease are pulmonary hypertension and chronic sickle lung disease. These complications can lead to morbidity (such as reduced exercise tolerance) and increased mortality...
2016: Cochrane Database of Systematic Reviews
Melanie A Villanueva, Keith J August
BACKGROUND: Fever and neutropenia (FN) is a common complication of pediatric oncology therapy and accounts for a large number of hospital admissions. Standard therapy for FN includes hospital admission and empiric antibiotics. Strict adherence to this practice leads to prolonged hospitalizations that may be unnecessary for patients at low risk of having an underlying significant infection. PROCEDURE: Children admitted with FN could be discharged after a minimum of 48 hr with no further antibiotic therapy once they had been afebrile for 24 hr with negative blood cultures from initial presentation, regardless of their neutrophil count...
October 2016: Pediatric Blood & Cancer
Alem Mehari, Elizabeth S Klings
Sickle cell disease (SCD), the most common genetic hemolytic anemia worldwide, affects 250,000 births annually. In the United States, SCD affects approximately 100,000 individuals, most of African descent. Hemoglobin S (HbS) results from a glutamate-to-valine mutation of the sixth codon of the β-hemoglobin allele; the homozygous genotype (HbSS) is associated with the most prevalent and severe form of the disease. Other SCD genotypes include HbSC, composed of one HbS allele and one HbC (glutamate-to-lysine mutation) allele; and HbS-β-thalassemia(0) or HbS-β-thalassemia(+), composed of one HbS allele and one β-thalassemia allele with absent or reduced β-chain production, respectively...
May 2016: Chest
Chaima Abdelhafidh Sahli, Amina Bibi, Faida Ouali, Sondess Hadj Fredj, Boutheina Dakhlaoui, Rym Othmani, Naouel Laouini, Latifa Jouini, Fekria Ouenniche, Hajer Siala, Imed Touhami, Mariem Becher, Slaheddine Fattoum, Nour El Houda Toumi, Taieb Messaoud
BACKGROUND: In Tunisia, thalassemia and sickle cell disease represent the most prevalent monogenic hemoglobin disorders with 2.21% and 1.89% of carriers, respectively. This study aims to evaluate the diagnosis reliability of a series of red blood cell indices and parameters in differentiation of beta-thalassemia trait (β-TT) from iron deficiency anemia (IDA) and between homozygous sickle cell disease (SS) and sickle cell-thalassemia (ST). METHODS: The study covered 384 patients divided into three groups...
November 2013: Clinical Chemistry and Laboratory Medicine: CCLM
Samuel Ademola Adegoke, Umar Abdullahi Shehu, Lasisi Oluwafemi Mohammed, Yunusa Sanusi, Oyeku Akibu Oyelami
BACKGROUND AND OBJECTIVES: The pain in sickle cell anemia (SCA) is often triggered by dehydration, acidosis, and fever that are usually due to malaria. Intake of lime juice was recently demonstrated to facilitate clearance of the malaria parasite. It was therefore sought to determine whether regular intake of lime juice will ameliorate crisis, especially recurrent bone pain. DESIGN: In this preliminary, open-labeled, randomized study, the effects of lime juice on the clinical and some laboratory characteristics of children with SCA were tested...
June 2013: Journal of Alternative and Complementary Medicine: Research on Paradigm, Practice, and Policy
Stephen P Hunger
No abstract text is available yet for this article.
April 28, 2016: Blood
Michael Sze Yuan Low, Joanna Speedy, Claire E Styles, Luz Maria De-Regil, Sant-Rayn Pasricha
BACKGROUND: Iron-deficiency anaemia is highly prevalent among non-pregnant women of reproductive age (menstruating women) worldwide, although the prevalence is highest in lower-income settings. Iron-deficiency anaemia has been associated with a range of adverse health outcomes, which restitution of iron stores using iron supplementation has been considered likely to resolve. Although there have been many trials reporting effects of iron in non-pregnant women, these trials have never been synthesised in a systematic review...
April 18, 2016: Cochrane Database of Systematic Reviews
W Barcellini, B Fattizzo
Several hemolytic markers are available to guide the differential diagnosis and to monitor treatment of hemolytic conditions. They include increased reticulocytes, an indicator of marrow compensatory response, elevated lactate dehydrogenase, a marker of intravascular hemolysis, reduced haptoglobin, and unconjugated hyperbilirubinemia. The direct antiglobulin test is the cornerstone of autoimmune forms, and blood smear examination is fundamental in the diagnosis of congenital membrane defects and thrombotic microangiopathies...
2015: Disease Markers
Françoise Bernaudin, Suzanne Verlhac, Cécile Arnaud, Annie Kamdem, Isabelle Hau, Emmanuella Leveillé, Manuela Vasile, Florence Kasbi, Fouad Madhi, Christine Fourmaux, Sandra Biscardi, Eliane Gluckman, Gérard Socié, Jean-Hugues Dalle, Ralph Epaud, Corinne Pondarré
Stroke risk in sickle cell anemia (SCA), predicted by high transcranial Doppler (TCD) velocities, is prevented by transfusions. We present the long-term follow-up of SCA children from the Créteil newborn cohort (1992-2012) detected at risk by TCD and placed on chronic transfusions. Patients with normalized velocities and no stenosis were treated with hydroxyurea, known to decrease anemia and hemolytic rate. Trimestrial Doppler was performed and transfusions restarted immediately in the case of reversion to abnormal velocities...
April 7, 2016: Blood
Simon Mantha, Martin S Tallman, Gerald A Soff
PURPOSE OF REVIEW: Acute promyelocytic leukemia (APL) is associated with a complex coagulopathy. In spite of substantial recent improvements in treatment regimens, hemorrhagic death remains the main cause of induction failure. In this review, we delineate recent understanding of the pathophysiology and management of the hemorrhagic diathesis of APL. RECENT FINDINGS: Laboratory and clinical data suggest that the malignant leukocytes mediate the hemorrhagic diathesis associated with APL through multiple mechanisms which lead to a combination of consumptive coagulopathy and primary hyperfibrinolysis...
March 2016: Current Opinion in Hematology
Stanley L Schrier
In this issue of Blood, Moretti et al provide data that challenge the entrenched oral treatment of iron deficiency anemia. The paper shows how the newer understanding of hepcidin and iron metabolism in general can lead to very practical improvements in the management of iron deficiency anemia, a disorder that may affect as many as 1 billion people.
October 22, 2015: Blood
Clara Camaschella
New England Journal of Medicine, Volume 372, Issue 19, Page 1832-1843, May 2015.
May 7, 2015: New England Journal of Medicine
Richard M Kaufman, Benjamin Djulbegovic, Terry Gernsheimer, Steven Kleinman, Alan T Tinmouth, Kelley E Capocelli, Mark D Cipolle, Claudia S Cohn, Mark K Fung, Brenda J Grossman, Paul D Mintz, Barbara A O'Malley, Deborah A Sesok-Pizzini, Aryeh Shander, Gary E Stack, Kathryn E Webert, Robert Weinstein, Babu G Welch, Glenn J Whitman, Edward C Wong, Aaron A R Tobian
BACKGROUND: The AABB (formerly, the American Association of Blood Banks) developed this guideline on appropriate use of platelet transfusion in adult patients. METHODS: These guidelines are based on a systematic review of randomized, clinical trials and observational studies (1900 to September 2014) that reported clinical outcomes on patients receiving prophylactic or therapeutic platelet transfusions. An expert panel reviewed the data and developed recommendations using the Grading of Recommendations Assessment, Development and Evaluation (GRADE) framework...
February 3, 2015: Annals of Internal Medicine
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