Gharaibeh Huda, Barqawi Moussa A, Al-Awamreh Khetam, Al Bashtawy Mohammed
Maternal-Child Health and Midwifery Department, Faculty of Nursing, Jordan University of Science and Technology.
Department of Hematology, Princess Rahama Teaching Hospital, Irbid.
J Pediatr Hematol Oncol. 2018 Apr;40(3):182-187. doi: 10.1097/MPH.0000000000001104.
β-thalassemia major (BTM) is an inherited blood disorder leading to severe anemia. A better understanding of BTM complications can be considered an important factor in developing effective health care provision.
A descriptive exploratory design was used to identify the clinical burden of BTM from affected children's perspective. A convenience sample of 45 patients with BTM, accompanied by a family member, was recruited from a governmental hospital during April-May 2015.
The most reported clinical burden was facial deformity 86.9%, followed by systematic infection (48.8%), growth delay (44.4%), and liver problems (39.9%). Patient age was significantly associated with clinical burdens such as bone pain and facial deformity. The number of blood transfusions received was associated with growth delay and bone pain.
This study highlights the clinical burdens of thalassemia on affected children, in terms of physical appearance, growth delay and other burdens.
重型β地中海贫血（BTM）是一种导致严重贫血的遗传性血液疾病。更好地了解BTM并发症可被视为发展有效医疗保健服务的一个重要因素。
采用描述性探索性设计，从受影响儿童的角度确定BTM的临床负担。2015年4月至5月期间，从一家政府医院招募了45名BTM患者及其一名家庭成员作为便利样本。
报告最多的临床负担是面部畸形（86.9%），其次是系统性感染（48.8%）、生长发育迟缓（44.4%）和肝脏问题（39.9%）。患者年龄与骨痛和面部畸形等临床负担显著相关。输血次数与生长发育迟缓和骨痛相关。
本研究从外貌、生长发育迟缓及其他负担方面，突出了地中海贫血对受影响儿童的临床负担。
