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Markers of severe vaso-occlusive painful episode frequency in children and adolescents with sickle cell anemia.

ABSTRACT: OBJECTIVE:To identify factors associated with frequent severe vaso-occlusive pain crises in a contemporary pediatric cohort of patients with sickle cell anemia (SCA) enrolled in a prospective study of pulmonary hypertension and the hypoxic response in sickle cell disease. STUDY DESIGN:Clinical and laboratory characteristics of children with SCA who had ?3 severe pain crises requiring health care in the preceding year were compared with those of subjects with <3 such episodes. RESULTS:Seventy-five children (20%) reported ?3 severe pain episodes in the preceding year, and 232 (61%) had none. Frequent pain episodes were associated with older age (OR, 1.2; 95% CI, 1.1-1.3; P < .0001), ?-thalassemia trait (OR 3.5; 1.6-6.7; P = .002), higher median hemoglobin (OR 1.7; 95% CI: 1.2-2.4; P < .003), and lower lactate dehydrogenase concentration (OR 1.82; 95% CI: 1.07-3.11; P = .027). Children with high pain frequency also had an increased iron burden (serum ferritin, 480 vs 198 ?g/L; P = .006) and higher median tricuspid regurgitation jet velocity (2.41 vs 2.31 m/s; P = .001). Neither hydroxyurea use nor fetal hemoglobin levels were significantly different according to severe pain history. CONCLUSIONS:In our cohort of children with SCA, increasing age was associated with higher frequency of severe pain episodes as were ?-thalassemia, iron overload, higher hemoglobin and lower lactate dehydrogenase concentration, and higher tricuspid regurgitation velocity.

SUBMITTER: Darbari DS

PROVIDER: S-EPMC3258348 | biostudies-literature | 2012 Feb

REPOSITORIES: biostudies-literature

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Markers of severe vaso-occlusive painful episode frequency in children and adolescents with sickle cell anemia.

Darbari Deepika S DS Onyekwere Onyinye O Nouraie Mehdi M Minniti Caterina P CP Luchtman-Jones Lori L Rana Sohail S Sable Craig C Ensing Gregory G Dham Niti N Campbell Andrew A Arteta Manuel M Gladwin Mark T MT Castro Oswaldo O Taylor James G JG Kato Gregory J GJ Gordeuk Victor V

The Journal of pediatrics 20110903 2

<h4>Objective</h4>To identify factors associated with frequent severe vaso-occlusive pain crises in a contemporary pediatric cohort of patients with sickle cell anemia (SCA) enrolled in a prospective study of pulmonary hypertension and the hypoxic response in sickle cell disease.<h4>Study design</h4>Clinical and laboratory characteristics of children with SCA who had ≥3 severe pain crises requiring health care in the preceding year were compared with those of subjects with <3 such episodes.<h4>R ...[more]

PMID: 21890147

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Project description:Background and purpose Sickle cell disease (SCD) is a collection of rare inherited blood disorders affecting approximately 100,000 people in the U.S. and 20–25 million people globally. Individuals with SCD experience recurrent episodes of severe and unpredictable pain that are caused by vaso-occlusive crises (VOCs), a hallmark of the disease. VOCs are the primary cause of hospitalization in SCD, result in missed workdays and school days, and decrease quality of life (QoL). Although VOCs cause significant burden in the lives of individuals with SCD, there is no synthesis on the frequency of VOCs in the real world. This systematic literature review sought to identify literature describing the frequency of VOCs experienced by individuals with SCD in real-world settings. Methods MEDLINE and 6 congresses were searched (date range: January 1, 2000 to June 30, 2020). Studies were reviewed independently by two researchers. Studies assessing frequency or prevalence of VOCs or VOC-related outcomes were included. Results Of 1438 studies identified in the search, 52 met pre-specified inclusion and exclusion criteria. Reported frequency of VOCs varied widely ranging from a mean or median of 0 VOCs/year to 18.2 VOCs/year. The proportion of patients experiencing ≥ 3 VOCs/year ranged from 4 to 67% and the proportion of patients experiencing ≥ 5 VOCs/year ranged from 18 to 59%. Measures of VOC severity were limited, with 13 studies considering frequency of complicated VOCs and only 1 study reporting duration of VOC episodes. Conclusions This is the first study to systematically assess published evidence pertaining to VOCs in real-world settings. Reported VOC frequency in real-world settings varied widely, with a majority of studies only considering VOCs managed in an inpatient or outpatient setting. Studies that considered VOCs managed at home reported a higher frequency of VOCs, suggesting that many studies may underestimate the frequency of VOCs. This systematic literature review (SLR) highlights the need for consistent reporting of (1) self-reported VOCs, including those managed at home, (2) definitions of VOCs, (3) complicated VOCs, and (4) duration of VOC episodes in literature. Supplementary Information The online version contains supplementary material available at 10.1186/s13023-021-02096-6.

Dataset Information

Markers of severe vaso-occlusive painful episode frequency in children and adolescents with sickle cell anemia.

Publications

Markers of severe vaso-occlusive painful episode frequency in children and adolescents with sickle cell anemia.

Similar Datasets

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