Mulyana Aep Maulid, Rakhmawati Windy, Pramukti Iqbal, Lukman Mamat, Wartakusumah Riki, Mediani Henny Suzana
Faculty of Nursing, Universitas Padjadjaran, Bandung, West Java, 40132, Indonesia.
Department of Pediatric Nursing, Faculty of Nursing, Universitas Padjadjaran, Bandung, West Java, 40132, Indonesia.
J Multidiscip Healthc. 2024 Jul 9;17:3235-3246. doi: 10.2147/JMDH.S475371. eCollection 2024.
Children with sickle cell disease (SCD) are more likely to have deficient serum levels of vitamin D for bone metabolism. However, appropriate interventions are essential to prevent its progression and alleviate symptoms.
The aim of this study is to determine interventions for managing bone disease in children with SCD.
This study uses a scoping review. A literature review was conducted using PubMed, CINAHL, ScienceDirect, Scopus, and Google Scholar search engines. The study was eligible for inclusion if it included articles published from 2013 to 2023, full-text, and original study design. Study quality was assessed using the Joanna Briggs Institute (JBI) appraisal tool.
This review identified six studies and 114 children with SCD, including 57 boys (50%) and 57 girls (50%). The majority of SCD types experienced were HbSS (86.84%), HbS-B Thal (7.01%), HbSC (5.27%), and the HbSS Arab-Indian haplotype (0.88%). Bone disease problems that often arise in children with SCD include Avascular Necrosis (AVN) (78.08%), Osteonecrosis of the Femoral Head (ONFH) (18.42%), and other bone problems (3.50%). Meanwhile, four types of intervention findings were used in managing bone disease among children with SCD: 1). Surgical procedures 53 (41.09%) included total hip arthroplasty (THA), Osteotomy, and Multiple epiphyseal drilling with Autologous Bone Marrow Implantation (AMBI); 2). Invasive procedures 67 (51.93%) included intravenous bisphosphonates, hydroxyurea (HU), and core decompression (CD) with bone marrow aspirate concentrate injection: 3). Oral pharmacological or Vitamin D3 (cholecalciferol) 4 (3.10%); 4). Non-pharmacology or physical therapy 5 (3.88%).
Our findings highlight that surgical, invasive, pharmacological, and physical therapy interventions positively impact increasing bone mineral density (BMD) and functional improvement of bone disease among children with SCD. The interventions provided excellent functional outcomes with minimal complications and no life-threatening side effects.
镰状细胞病（SCD）患儿血清中用于骨代谢的维生素D水平更有可能不足。然而，适当的干预措施对于预防其进展和缓解症状至关重要。
本研究的目的是确定SCD患儿骨病的管理干预措施。
本研究采用范围综述。使用PubMed、CINAHL、ScienceDirect、Scopus和谷歌学术搜索引擎进行文献综述。如果研究包括2013年至2023年发表的文章、全文和原始研究设计，则符合纳入条件。使用乔安娜·布里格斯研究所（JBI）评估工具评估研究质量。
本综述确定了六项研究和114名SCD患儿，其中包括57名男孩（50%）和57名女孩（50%）。经历的大多数SCD类型为HbSS（86.84%）、HbS-B地中海贫血（7.01%）、HbSC（5.27%）和HbSS阿拉伯-印度单倍型（0.88%）。SCD患儿中经常出现的骨病问题包括无血管坏死（AVN）（78.08%）、股骨头骨坏死（ONFH）（18.42%）和其他骨问题（3.50%）。同时，在管理SCD患儿的骨病方面使用了四种干预措施：1）。手术程序53例（41.09%），包括全髋关节置换术（THA）、截骨术和自体骨髓植入多骨骺钻孔术（AMBI）；2）。侵入性程序67例（51.93%），包括静脉注射双膦酸盐、羟基脲（HU）和骨髓抽吸浓缩液注射的核心减压术（CD）：3）。口服药物或维生素D3（胆钙化醇）4例（3.10%）；4）。非药物或物理治疗5例（3.88%）。
我们的研究结果表明，手术、侵入性、药物和物理治疗干预对提高SCD患儿的骨矿物质密度（BMD）和改善骨病功能有积极影响。这些干预措施提供了良好的功能结果，并发症最少，没有危及生命的副作用。
