Vaibhav Divyansh, Panda Prem S, Debata Ipsita, Sinha Ashish K
Undergraduate Student, Pt J N M Medical College, Raipur, Chhattisgarh, India.
Department of Community Medicine, Kalinga Institute of Medical Sciences, Bhubaneswar, Odisha, India.
J Family Med Prim Care. 2024 May;13(5):1825-1829. doi: 10.4103/jfmpc.jfmpc_1490_23. Epub 2024 May 24.
Sickle cell disease (SCD) is a disorder marked by a single-point mutation in the beta-globin gene. Hydroxyurea is a globally accepted disease-modifying agent that sounds to be effective in managing clinically and probably preventing complications of SCD. The current study aims to document the morbidity pattern and impact of Hydroxyurea therapy in the Outpatient Department of Sickle Cell Institute, Raipur.
This cross-sectional study was conducted among randomly selected sixty-five patients (adults and children above six years). After obtaining informed consent, relevant data were collected in a predesigned pretested questionnaire. The appropriate statistical exercise was applied for the interpretation of results and inferences.
Acute febrile illness 54 (83%) and 53 (81.5%) reported pain crisis observed to have the most common morbidity among the study subjects, followed by 55.4% (36), 33 (50.8%) jaundice and difficulty breathing, respectively. Joint pain was the most commonly observed complaint, particularly at the knee joint (76.9%). Other complaints such as hand-foot syndrome (24.6%), epistaxis (27.7%), and acute chest syndrome (21.5%). Vaso-occlusive crisis (72.4%), difficulty in walking (60.0%) and eyesight (35.4%), leg ulcers (9.2%), and dactylitis (3.1%) were also documented as clinical manifestations among study participants. Less than half (44.46%) had an awareness about SCD. Hydroxyurea therapy was highly significant in improving the patient's clinical picture ( < 0.01), especially following the frequency of hospitalization and the requirement for blood transfusion.
Pain crisis is the most common morbidity among study participants with a low level of knowledge about SCD with febrile illness. Hydroxyurea therapy was found to be quite effective as a disease-modifying therapy, especially for reducing the frequency of blood transfusion and lowering hospitalization rates among SCD patients.
镰状细胞病（SCD）是一种由β-珠蛋白基因突变引起的单基因疾病。羟基脲是一种全球公认的病情改善药物，似乎对SCD的临床管理有效，可能还能预防并发症。本研究旨在记录赖布尔镰状细胞病研究所门诊部羟基脲治疗的发病模式及其影响。
本横断面研究选取了65例随机抽取的患者（成人及6岁以上儿童）。在获得知情同意后，通过预先设计并经过预测试的问卷收集相关数据。运用适当的统计方法对结果和推论进行解释。
在研究对象中，54例（83%）报告有急性发热性疾病，53例（81.5%）出现疼痛危机，是最常见的发病情况，其次分别是黄疸（55.4%，36例）、呼吸困难（33例，50.8%）。关节疼痛是最常见的症状，尤其是膝关节（76.9%）。其他症状如手足综合征（24.6%）、鼻出血（27.7%）和急性胸部综合征（21.5%）。血管闭塞性危机（72.4%）、行走困难（60.0%）和视力问题（35.4%）、腿部溃疡（9.2%）以及指（趾）炎（3.1%）也被记录为研究参与者的临床表现。不到一半（44.46%）的人了解SCD。羟基脲治疗在改善患者临床状况方面具有高度显著性（<0.01），尤其是在住院频率和输血需求方面。
在对SCD知识了解较少且伴有发热性疾病的研究参与者中，疼痛危机是最常见的发病情况。羟基脲治疗作为一种病情改善疗法相当有效，尤其是对于减少SCD患者的输血频率和降低住院率。
