未经批准:木村病(KD),也被称为嗜酸性粒细胞增生性淋巴肉芽肿,是一种罕见的良性慢性炎症,其特点是无痛的进行性肿块位于头颈部的皮下区域,外周血嗜酸性粒细胞升高,血清免疫球蛋白E(IgE)水平升高。KD在临床实践中并不常见,尤其是儿童发病率低,因此,它经常导致儿科患者的误诊或漏诊。
UNASSIGNED:回顾性分析作者机构中11例小儿KD患者的临床资料。
UNASSIGNED:总共有11名KD患儿入组,其中男性患者9例,女性患者2例(性别比4.5:1)。诊断时的中位年龄为14岁(范围5-18岁),所有患者的初始症状包括无痛性皮下肿块和局灶性肿胀,症状持续时间从1个月到10年不等,平均病程为24.45个月。六个病人有单个病灶,5例多发病变。病变部位比例最高的是腮腺(n=5,31.3%)和耳后(n=5,31.3%),其次是颈部淋巴结(n=4,25%),和其他(n=2,12.5;肘部n=1;背部n=1)。所有患者的嗜酸性粒细胞绝对计数升高,范围为0.71×109/L至10.35×109/L(正常值0.02-0.52×109/L)。所有7例接受血清免疫球蛋白检查的患者的IgE水平均升高(正常范围<100IU/mL)。3例患者接受口服皮质类固醇治疗,2例复发。3例患者接受手术切除联合口服糖皮质激素治疗,没有病人复发.其他3例患者接受手术和放疗,手术联合糖皮质激素、环孢素和糖皮质激素联合来氟米特,没有病人复发.
未经评估:在研究的基础上,发现木村病是罕见的,在儿科患者中可能有不典型的症状,建议联合治疗以减少复发,应进行长期随访。
UNASSIGNED: Kimura disease (KD), also known as eosinophilic hyperplastic lymphoid granuloma, is a rare benign chronic inflammatory condition, which is featured with the painless progressive mass located in the subcutaneous area of the head and neck region, elevated peripheral blood eosinophils, and raised serum immunoglobulin E (IgE) levels. KD is uncommon in clinical practice, especially with low incidence in children, so it often leads to misdiagnosis or missed diagnosis in pediatric patients.
UNASSIGNED: The clinical data of 11 pediatric patients with KD in the authors\' institution were retrospectively analyzed.
UNASSIGNED: There were 11 pediatric patients with KD enrolled in total, including 9 male patients and 2 female patients (sex ratio 4.5:1). The median age at diagnosis stood at 14 years (range 5-18 years), the initial symptoms in all patients included painless subcutaneous masses and focal swelling, the duration of symptoms ranged from 1 month to 10 years, and the average duration was 24.45 months. Six patients had single lesions, and 5 had multiple lesions. The highest proportion of lesion regions were parotid gland (n = 5, 31.3%) and retroauricular (n = 5, 31.3%), followed by cervical lymph nodes (n = 4, 25%), and others (n = 2,12.5; elbow n = 1; back n = 1). The eosinophil absolute count elevated in all patients, ranging from 0.71×109/L to 10.35 ×109/L (normal range 0.02-0.52×109/L). IgE levels were increased in all 7 patients who underwent serum immunoglobulin examination (normal range <100 IU/mL). Three patients received oral corticosteroid treatment while 2 relapsed. Three patients received surgical resection combined with oral corticosteroid treatment, and no patient relapsed. The other 3 patients received surgery and radiotherapy, surgery combined with corticosteroid and cyclosporin and corticosteroid combined with leflunomide respectively, and no patient relapsed.
UNASSIGNED: Base on the study, it is found that Kimura disease is rare and may have the atypical symptoms in pediatric patients, combination therapy is recommended to reduce recurrence, and long-term follow-up should be performed.