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Hydroxyurea (hydroxycarbamide) for sickle cell disease
CONCLUSIONS: There is evidence to suggest that hydroxyurea may be effective in decreasing the frequency of pain episodes and other acute complications in adults and children with sickle cell anaemia of HbSS or HbSβºthal genotypes and in preventing life-threatening neurological events in those with sickle cell anaemia at risk of primary stroke by maintaining transcranial Doppler velocities. However, there is still insufficient evidence on the long-term benefits of hydroxyurea, particularly with regard to preventing chronic complications of SCD, or recommending a standard dose or dose escalation to maximum tolerated dose. ...
Source: Cochrane Database of Systematic Reviews - September 1, 2022 Category: General Medicine Authors: Angela E Rankine-Mullings Sarah J Nevitt Source Type: research

Clinical Characteristics of Pediatric Patients with Congenital Erythrocytosis: A Single-Center Study
AbstractAlthough congenital erythrocytosis (CE), an inherited disorder, impairs pediatric quality of life, physicians often overlook high hemoglobin (Hgb) levels and its symptoms due to lack of knowledge of age-adjusted pediatric Hgb levels and CE ’s rarity. In a retrospective, single-center study, data from hospital records of pediatric patients diagnosed with CE were evaluated. Twenty-six patients from 25 families (80.8% male) had been diagnosed with CE in 20 years, at a mean age of 14.9 ± 2.8 years (8.3–17.8) and with a mean Hgb level of 17.36 ± 1.44 g/dL (14.63–22.1). No serum erythropoietin levels e...
Source: Indian Journal of Hematology and Blood Transfusion - August 25, 2021 Category: Hematology Source Type: research

Blood transfusion for preventing primary and secondary stroke in people with sickle cell disease.
CONCLUSIONS: There is no evidence for managing adults, or children who do not have HbSS sickle cell disease. In children who are at higher risk of stroke and have not had previous long-term transfusions, there is moderate quality evidence that long-term red cell transfusions reduce the risk of stroke, and low quality evidence they also reduce the risk of other sickle cell disease-related complications. In primary and secondary prevention of stroke there is low quality evidence that switching to hydroxyurea with phlebotomy has little or no effect on the liver iron concentration. In secondary prevention of stroke there is lo...
Source: Cochrane Database of Systematic Reviews - July 26, 2020 Category: General Medicine Authors: Estcourt LJ, Kohli R, Hopewell S, Trivella M, Wang WC Tags: Cochrane Database Syst Rev Source Type: research

Interventions for preventing silent cerebral infarcts in people with sickle cell disease.
CONCLUSIONS: We identified no trials for preventing silent cerebral infarcts in adults, or in children who do not have HbSS SCD. Long-term red blood cell transfusions may reduce the incidence of silent cerebral infarcts in children with abnormal TCD velocities, but may have little or no effect on children with normal TCD velocities. In children who are at higher risk of stroke and have not had previous long-term transfusions, long-term red blood cell transfusions probably reduce the risk of stroke, and other SCD-related complications (acute chest syndrome and painful crises). In children and adolescents at high risk of str...
Source: Cochrane Database of Systematic Reviews - April 5, 2020 Category: General Medicine Authors: Estcourt LJ, Kimber C, Hopewell S, Trivella M, Doree C, Abboud MR Tags: Cochrane Database Syst Rev Source Type: research

Red blood cell transfusion to treat or prevent complications in sickle cell disease: an overview of Cochrane reviews.
CONCLUSIONS: This overview provides support from two high-quality Cochrane Reviews for the use of RBC transfusions in preventing stroke in children and adolescents at high risk of stroke (abnormal TCDs or SCI) and evidence that it may decrease the risk of SCI in children with abnormal TCD velocities. In addition RBC transfusions may reduce the risk of ACS and painful crisis in this population.This overview highlights the lack of high-quality evidence in adults with SCD and the number of reviews that have no evidence for the use of RBC transfusions across a spectrum of SCD complications. Also of concern is the variable and ...
Source: Cochrane Database of Systematic Reviews - August 1, 2018 Category: General Medicine Authors: Fortin PM, Hopewell S, Estcourt LJ Tags: Cochrane Database Syst Rev Source Type: research

Interventions for preventing silent cerebral infarcts in people with sickle cell disease.
CONCLUSIONS: We identified no trials for preventing silent cerebral infarcts in adults, or in children who do not have HbSS SCD.Long-term red blood cell transfusions may reduce the incidence of silent cerebral infarcts in children with abnormal TCD velocities, but may have little or no effect on children with normal TCD velocities. In children who are at higher risk of stroke and have not had previous long-term transfusions, long-term red blood cell transfusions probably reduce the risk of stroke, and other SCD-related complications (acute chest syndrome and painful crises).In children and adolescents at high risk of strok...
Source: Cochrane Database of Systematic Reviews - May 13, 2017 Category: General Medicine Authors: Estcourt LJ, Fortin PM, Hopewell S, Trivella M, Doree C, Abboud MR Tags: Cochrane Database Syst Rev Source Type: research

Hydroxyurea (hydroxycarbamide) for sickle cell disease.
CONCLUSIONS: There is evidence to suggest that hydroxyurea is effective in decreasing the frequency of pain episodes and other acute complications in adults and children with sickle cell anaemia of HbSS or HbSβºthal genotypes and in preventing life-threatening neurological events in those with sickle cell anaemia at risk of primary stroke by maintaining transcranial doppler velocities. However, there is still insufficient evidence on the long-term benefits of hydroxyurea, particularly in preventing chronic complications of SCD, recommending a standard dose or dose escalation to maximum tolerated dose. There is also insuf...
Source: Cochrane Database of Systematic Reviews - April 20, 2017 Category: Journals (General) Authors: Nevitt SJ, Jones AP, Howard J Tags: Cochrane Database Syst Rev Source Type: research

Double the Trouble: Acute Coronary Syndrome and Ischemic Stroke in Polycythemia Vera
Vague symptoms turned out to be life-threatening conditions. A 50 year-old man with a history of polycythemia vera, presented to hospital with epigastric discomfort and clumsiness of the right hand for 5 days. Polycythemia vera was diagnosed 1 year prior with good control of hematocrit by intermittent phlebotomy. He did not have any other medical history, and was not taking any medications. He denied chest pain, shortness of breath, nausea, headache, vision change, or speech difficulty.
Source: The American Journal of Medicine - March 12, 2017 Category: Journals (General) Authors: Hitomi Hosoya, Jeffrey J. Levine, David H. Henry, Sheldon Goldberg Tags: Diagnostic Dilemma Source Type: research

Blood transfusion for preventing primary and secondary stroke in people with sickle cell disease.
CONCLUSIONS: There is no evidence for managing adults, or children who do not have HbSS sickle cell disease.In children who are at higher risk of stroke and have not had previous long-term transfusions, there is moderate quality evidence that long-term red cell transfusions reduce the risk of stroke, and low quality evidence they also reduce the risk of other sickle cell disease-related complications.In primary and secondary prevention of stroke there is low quality evidence that switching to hydroxyurea with phlebotomy has little or no effect on the liver iron concentration.In secondary prevention of stroke there is low-q...
Source: Cochrane Database of Systematic Reviews - January 16, 2017 Category: Journals (General) Authors: Estcourt LJ, Fortin PM, Hopewell S, Trivella M, Wang WC Tags: Cochrane Database Syst Rev Source Type: research

Pain and other non‐neurological adverse events in children with sickle cell anemia and previous stroke who received hydroxyurea and phlebotomy or chronic transfusions and chelation: Results from the SWiTCH clinical trial
Conclusions: In children with SCA and prior stroke, monthly transfusions and daily iron chelation provided superior protection against acute vaso‐occlusive pain SAEs when compared to hydroxyurea and monthly phlebotomy.
Source: American Journal of Hematology - July 16, 2013 Category: Hematology Authors: Ofelia Alvarez, Nancy A. Yovetich, J. Paul Scott, William Owen, Scott T. Miller, William Schultz, Alexandre Lockhart, Banu Aygun, Jonathan Flanagan, Melanie Bonner, Brigitta U. Mueller, Russell E. Ware, Tags: Research Article Source Type: research