Age of first pain crisis and associated complications in the CASiRe international sickle cell disease cohort.

Autor: Tartaglione I; Department of Women, Child and General and Specialized Surgery, University of Campania 'Luigi Vanvitelli', Naples, Italy., Strunk C; ProMedica Russell J. Ebeid Children's Hospital, Toledo, OH, USA., Antwi-Boasiako C; Department of Physiology, University of Ghana Medical School, University of Ghana, Accra, Ghana., Andemariam B; New England Sickle Cell Institute, Division of Hematology-Oncology, Neag Comprehensive Cancer Center, UCONN Health, University of Connecticut, Farmington, CT, USA., Colombatti R; Clinic of Pediatric Hematology Oncology, Department of Women's and Child Health, Azienda Ospedaliera-Università di Padova, Padova, Italy., Asare EV; Department of Hematology, Korle-Bu Teaching Hospital, Accra, Ghana., Piccone CM; Pediatric Hematology/Oncology, Rainbow Babies and Children's Hospital, Cleveland, OH, USA., Manwani D; Department of Pediatrics, Albert Einstein College of Medicine, Children's Hospital at Montefiore, Bronx, NY, USA., Boruchov D; Department of Pediatrics, University of Connecticut School of Medicine, Connecticut Children's Medical Center, Hartford, CT, USA., Tavernier F; Division of Pediatric Hematology/Oncology, University of Michigan, Ann Arbor, MI, USA., Farooq F; Division of Pediatric Hematology/Oncology, University of Michigan, Ann Arbor, MI, USA., Akatue S; Division of Pediatric Hematology/Oncology, University of Michigan, Ann Arbor, MI, USA., Oteng B; Division of Pediatric Hematology/Oncology, University of Michigan, Ann Arbor, MI, USA., Urbonya R; Division of Pediatric Hematology/Oncology, University of Michigan, Ann Arbor, MI, USA., Wilson S; Division of Pediatric Hematology/Oncology, University of Michigan, Ann Arbor, MI, USA., Owda A; Division of Pediatric Hematology/Oncology, University of Michigan, Ann Arbor, MI, USA., Bamfo R; Division of Pediatric Hematology/Oncology, University of Michigan, Ann Arbor, MI, USA., Boatemaa GD; Department of Physiology, University of Ghana Medical School, University of Ghana, Accra, Ghana., Rao S; Department of Child Health, University of Ghana Medical School Accra, Ghana., Zempsky W; Department of Pediatrics, University of Connecticut School of Medicine, Connecticut Children's Medical Center, Hartford, CT, USA., Sey F; Ghana Institute of Clinical Genetics, Korle Bu Teaching Hospital, Accra, Ghana., Inusa BP; Department of Pediatric Haematology, Evelina Children's Hospital, Guy's and St. Thomas NHS Trust, London, UK., Perrotta S; Department of Women, Child and General and Specialized Surgery, University of Campania 'Luigi Vanvitelli', Naples, Italy., Segbefia C; Department of Child Health, University of Ghana Medical School Accra, Ghana., Campbell AD; Division of Pediatric Hematology/Oncology, University of Michigan, Ann Arbor, MI, USA; Center for Cancer and Blood Disorders, Children's National Medical Center; George Washington University School of Medicine Health Sciences, Washington, DC, USA. Electronic address: acampbell@childrensnational.org.
Jazyk: angličtina
Zdroj: Blood cells, molecules & diseases [Blood Cells Mol Dis] 2021 May; Vol. 88, pp. 102531. Date of Electronic Publication: 2021 Jan 02.
DOI: 10.1016/j.bcmd.2020.102531
Abstrakt: Pain is a hallmark of Sickle Cell Disease (SCD) affecting patients throughout their life; the first pain crisis may occur at any age and is often the first presentation of the disease. Universal newborn screening identifies children with SCD at birth, significantly improving morbidity and mortality. Without early screening, diagnosis is generally made after disease manifestations appear. The Consortium for the Advancement of Sickle Cell Research (CASiRe) is an international collaborative group evaluating the clinical severity of subjects with SCD using a validated questionnaire and medical chart review, standardized across 4 countries (United States, United Kingdom, Italy and Ghana). We investigated the age of first pain crisis in 555 sickle cell subjects, 344 adults and 211 children. Median age of the first crisis in the whole group was 4 years old, 5 years old among adults and 2 years old among children. Patients from the United States generally reported the first crisis earlier than Ghanaians. Experiencing the first pain crisis early in life correlated with the genotype and disease severity. Early recognition of the first pain crisis could be useful to guide counseling and management of the disease.
(Copyright © 2020 Elsevier Inc. All rights reserved.)
Databáze: MEDLINE
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