Cianci Paola, Salvatore Silvia, Moretti Alex, Berardinelli Angela, Salvatoni Alessandro, Marinoni Maddalena, Agosti Massimo
Woman and Child Department, Ospedale "F. Del Ponte," University of Insubria, Varese, Italy.
IRCCS Mondino National Neusrological Foundation, Pavia, Italy.
J Pediatr Neurosci. 2019 Jan-Mar;14(1):38-41. doi: 10.4103/jpn.JPN_132_18.
Chronic inflammatory demyelinating polyneuropathy (CIDP) is a chronically progressive or relapsing sensorimotor disorder presumably due to antibody-mediated reactions. It is a rare condition in children, with estimated prevalence as 0.48 per 100,000 among patients younger than 20 years of age. Recommended treatments include immune modulators, intravenous immunoglobulins (IVIgs), steroids, and plasmapheresis. Management of pediatric CIDP is challenging because of the lack of evidence-based efficacy of the current therapies in children. Because of the rarity of this condition, there are no double-blind randomized studies to support the therapeutic choice as well as to identify the optimal first-line therapeutic regimen. IVIgs are widely used but the intravenous administration is usually uncomfortable, especially for children. Subcutaneous immunoglobulins (SCIgs) have proven to be effective in adults with CIDP and in children affected by antibody deficiencies and other different immune and inflammatory disorders. Herein, we described the case of a 7-year-old boy, affected by CIDP who clinically responded to IVIg but was dependent on this therapy. In order to improve his quality of life, we switched to SCIg with excellent result.
慢性炎性脱髓鞘性多发性神经病(CIDP)是一种慢性进行性或复发性感觉运动障碍,可能由抗体介导的反应引起。它在儿童中是一种罕见疾病,在20岁以下患者中的估计患病率为每10万人0.48例。推荐的治疗方法包括免疫调节剂、静脉注射免疫球蛋白(IVIg)、类固醇和血浆置换。由于目前的疗法在儿童中缺乏基于证据的疗效,小儿CIDP的管理具有挑战性。由于这种疾病的罕见性,没有双盲随机研究来支持治疗选择以及确定最佳的一线治疗方案。IVIg被广泛使用,但静脉给药通常不舒服,尤其是对儿童而言。皮下免疫球蛋白(SCIg)已被证明对患有CIDP的成人以及受抗体缺陷和其他不同免疫和炎症性疾病影响的儿童有效。在此,我们描述了一名7岁男孩的病例,他患有CIDP,对IVIg有临床反应,但依赖于这种治疗。为了改善他的生活质量,我们改用SCIg,效果极佳。