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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

Platelet count and mean volume in acute stroke: a systematic review and meta-analysis.
Authors: Sadeghi F, Kovács S, Zsóri KS, Csiki Z, Bereczky Z, Shemirani AH Abstract Changes of mean platelet volume (MPV) and platelet count (PC) could be a marker or a predictor of acute stroke (AS). We conducted a systematic review and meta-analysis of the published literature on the reporting of MPV and PC in AS. Studies were included in accordance with Patient Population or Problem, Intervention, Comparison, Outcomes, and Setting framework. The PRISMA strategy was used to report findings. Risk of bias was assessed with the Newcastle-Ottawa Scale. We included 34 eligible articles retrieved from the literature. ...
Source: Platelets - October 30, 2019 Category: Hematology Tags: Platelets Source Type: research

Validation of Sickle Cell Disease Severity Score in a Cohort of Hemoglobin SC Disease Patients
Conclusion: Despite having been derived from a SCD population that was 26% HbSC, the study was unable to validate the SSS within the cohort of HbSC patients. This may reflect the differences in patient population and/or therapeutic intervention between this cohort and the CSSCD cohort used in the construction of the SSS calculator. While SSS was found to correlate with 3 discrete markers of disease morbidity (TRJV, CVA, creatinine clearance), it appears that a new scoring system is required to accurately predict clinical mortality and morbidity in contemporary cohorts of adult HbSC disease patients.DisclosuresNo relevant c...
Source: Blood - November 21, 2018 Category: Hematology Authors: Rosart, R. M., Pestrin, O. A., Tomlinson, G. A., Ward, R., Pendergrast, J., Binding, A., Kuo, K. H. M. Tags: 903. Outcomes Research-Non-Malignant Hematology: Poster I Source Type: research

Therapeutic phlebotomy is safe in children with sickle cell anaemia and can be effective treatment for transfusional iron overload
Summary Serial phlebotomy was performed on sixty children with sickle cell anaemia, stroke and transfusional iron overload randomized to hydroxycarbamide in the Stroke With Transfusions Changing to Hydroxyurea trial. There were 927 phlebotomy procedures with only 33 adverse events, all of which were grade 2. Among 23 children completing 30 months of study treatment, the net iron balance was favourable (−8·7 mg Fe/kg) with significant decrease in ferritin, although liver iron concentration remained unchanged. Therapeutic phlebotomy was safe and well‐tolerated, with net iron removal in most children who completed 30�...
Source: British Journal of Haematology - January 22, 2015 Category: Hematology Authors: Banu Aygun, Nicole A. Mortier, Karen Kesler, Alexandre Lockhart, William H. Schultz, Alan R. Cohen, Ofelia Alvarez, Zora R. Rogers, Janet L. Kwiatkowski, Scott T. Miller, Pamela Sylvestre, Rathi Iyer, Peter A. Lane, Russell E. Ware, Tags: Short Report Source Type: research

Magnetic resonance imaging/angiography and transcranial Doppler velocities in sickle cell anemia: results from the SWiTCH trial
The Stroke With Transfusions Changing to Hydroxyurea (SWiTCH) trial compared standard (transfusions/chelation) to alternative (hydroxyurea/phlebotomy) treatment to prevent recurrent stroke and manage iron overload in children chronically transfused over 7 years before enrollment. Standardized brain magnetic resonance imaging/magnetic resonance angiography (MRA) and transcranial Doppler (TCD) exams were performed at entry and exit, with a central blinded review. A novel MRA vasculopathy grading scale demonstrated frequent severe baseline left/right vessel stenosis (53%/41% ≥Grade 4); 31% had no vessel stenosis on either ...
Source: Blood - August 7, 2014 Category: Hematology Authors: Helton, K. J., Adams, R. J., Kesler, K. L., Lockhart, A., Aygun, B., Driscoll, C., Heeney, M. M., Jackson, S. M., Krishnamurti, L., Miller, S. T., Sarnaik, S. A., Schultz, W. H., Ware, R. E., for the SWiTCH Investigators Tags: Pediatric Hematology, Sickle Cell Disease, Free Research Articles, Red Cells, Iron, and Erythropoiesis, Vascular Biology, Clinical Trials and Observations 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