目的:研究分析青岛大学附属医院1例儿童木村病合并肾脏损害的临床特征,诊断,治疗及预后,旨在对儿童木村病的诊疗提供一定的经验。方法:回顾性分析我院一名5岁男孩临床表现为肘部和腋窝淋巴结肿大,实验室检查表现为嗜酸性粒细胞和免疫球蛋白IgE显著增加,根据淋巴结病理切片,诊断为木村病。治疗起初用醋酸泼尼松治疗2周后,肿大的淋巴结完全消退,在泼尼松减量过程中,该患儿出现木村病复发,改用环孢菌素A,在随后的1年随访期间,症状逐渐显著改善。结论:针对木村病的治疗包括手术切除皮下肿块和药物治疗。尽管糖皮质激素是药物治疗的首选药物,但在糖皮质激素不敏感或不耐受的儿童中,免疫抑制剂如环孢菌素A可能是首选的替代药物。
Objective: To analyze the clinical features, diagnosis, treatment and prognosis of a child with Mucun disease and kidney damage in a hospital affiliated to Qingdao University, aiming to provide some experience in the diagnosis and treatment of children’s Mucun disease. Methods: Retrospective analysis of a 5-year-old boy in our hospital showed clinical manifestations of elbow and axillary lymphadenopathy. Laboratory tests showed a significant increase in eosinophils and immunoglobulin IgE. According to the pathological section of the lymph nodes, the diagnosis was Mucun disease. After treatment for 2 weeks with prednisone acetate, the swollen lymph nodes completely resolved. During the prednisone reduction, the patient developed a recurrence of Mucun disease and switched to cyclosporin A for follow-up in the following 1 year. During the period, the symptoms gradually improved significantly. Conclusion: Treatment for Kimura disease includes surgical removal of subcutaneous masses and medication. Although glucocorticoids are the drug of choice for drug therapy, immunosuppressive agents such as cyclosporin A may be the preferred alternative in children with glucocorticoid insensitivity or intolerance.
患儿男,5岁,2016年12月因“右腋窝和肘部大量肿块4个月”就诊于我院(青岛大学附属医院)。体格检查见右侧腋窝3 cm × 2 cm的肿块,无肿胀和疼痛,触摸柔软。B超发现数枚肿大的淋巴结,范围从0.8 cm × 1.0 cm到1.5 cm × 0.8 cm,有明显的皮层和髓质边界,位于右耳和右腋窝区域。右上臂有一个2.8 cm × 1.5 cm低回声结节,具有明显的边界和不规则的形状。手术切除后,送检病理检查,肿块含有不同的细胞成分,包括小的成熟淋巴细胞,原始和不成熟的淋巴细胞,以及少量嗜中性粒细胞,嗜酸性粒细胞和内皮细胞。诊断为慢性反应性淋巴结炎。在9个月后(2017年9月),患者在手术切口部位出现新的肿块,表现为瘙痒,多发性皮疹和局部皮肤色素沉着增厚(见图1(C)),再次入住我院。体格检查见0.5 cm × 0.5 cm的肿块位于手术切口发生的肘窝右侧,双侧腹股沟处还有两个肿块(见图1(A)~(B))。复查患儿病理切片发现多个淋巴滤泡,具有不同的生发中心,淋巴滤泡和滤泡间区域的嗜酸性粒细胞浸润,以及滤泡间区和血管周围的嗜酸性脓肿,纤维组织和血管增生(见图2(A)~(D)),该患者被诊断为木村氏病。
全天文,宋爱琴. 儿童木村病合并肾脏损害病例报告并文献复习A Child with Kimura’s Disease Had Multiple Lymph Nodes Enlargement as the Initial Sign: A Case Report and Literature Review[J]. 临床医学进展, 2020, 10(06): 1039-1044. https://doi.org/10.12677/ACM.2020.106157
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