Çekiç Şükrü, Özgür Taner, Karalı Yasin, Özkan Tanju, Kılıç Sara Şebnem
Divisions of Pediatric Immunology, Uludağ University Faculty of Medicine, Bursa, Turkey.
Pediatric Gastroenterology, Department of Pediatrics, Uludağ University Faculty of Medicine, Bursa, Turkey.
Turk J Pediatr. 2019;61(6):937-940. doi: 10.24953/turkjped.2019.06.016.
Çekiç Ş, Özgür T, Karalı Y, Özkan T, Kılıç SŞ. Vedolizumab treatment in a patient with X-linked agammaglobulinemia, is it safe and efficient? Turk J Pediatr 2019; 61: 937-940. The loss of inflammatory regulation resulting from the absence of B-lymphocytes leads to a risk for autoimmune and autoinflammatory complications. There is no data on the use of Vedolizumab in patients with X-linked agammaglobulinemia (XLA) as well as children with another primary immunodeficiency (PID) diseases. A 4-year-old boy was admitted to our clinic with a history of recurrent respiratory tract infections. He was diagnosed with XLA based on extremely low immunoglobulins, very low level of B cells, genetic mutation of BTK gene, and family history. At the age of 8, he suffered from intermittent fever attacks, abdominal pain, weakness, oral aft, and weight loss. His clinical and laboratory features were consistent with inflammatory bowel disease. Histopathological examination of the biopsy material obtained from terminal ileum, colon and cecum showed Crohn`s disease. Initially, he was treated with prednisolone and infliximab. Because of the lack of response, infliximab treatment was switched to adalimumab. Terminal ileum was resected to relieve obstruction complication. Although he had been treated with adalimumab, a significant improvement was not observed. Vedolizumab (Entyvio™), a humanized monoclonal antibody α4β7 integrin-receptor antagonist, was commenced. After treatment with vedolizumab, his fever and abdominal pain attacks reduced, his total daily calorie intake increased and weight gain improved. He began to walk again and continued his school education properly. No side effects were observed in 18 months. This is the first immunocompromised child treated with vedolizumab. The symptoms of the patient receded and no side effect were seen during the treatment.
切基奇·Ş、奥兹居尔·T、卡拉利·Y、奥兹坎·T、基利奇·SŞ。维得利珠单抗治疗一名X连锁无丙种球蛋白血症患者，是否安全有效？《土耳其儿科学杂志》2019年；61: 937 - 940。由于B淋巴细胞缺失导致炎症调节丧失会引发自身免疫和自身炎症并发症的风险。目前尚无关于维得利珠单抗在X连锁无丙种球蛋白血症（XLA）患者以及患有其他原发性免疫缺陷（PID）疾病儿童中使用的数据。一名4岁男孩因反复呼吸道感染病史入住我们的诊所。基于极低的免疫球蛋白、极低水平的B细胞、BTK基因的基因突变以及家族病史，他被诊断为XLA。8岁时，他出现间歇性发热发作、腹痛、虚弱、口腔溃疡和体重减轻。他的临床和实验室特征与炎症性肠病相符。从末端回肠、结肠和盲肠获取的活检材料的组织病理学检查显示为克罗恩病。最初，他接受泼尼松龙和英夫利昔单抗治疗。由于缺乏反应，英夫利昔单抗治疗改为阿达木单抗。切除末端回肠以缓解梗阻并发症。尽管他接受了阿达木单抗治疗，但未观察到明显改善。开始使用维得利珠单抗（恩特维尤™），一种人源化单克隆抗体α4β7整合素受体拮抗剂。使用维得利珠单抗治疗后，他的发热和腹痛发作减少，每日总热量摄入增加且体重增加情况改善。他又开始走路并正常继续学业。18个月内未观察到副作用。这是首例接受维得利珠单抗治疗的免疫功能低下儿童。患者症状消退且治疗期间未出现副作用。
