组织细胞增生症X伴脊椎破坏：附14例报告

本文介绍了北京儿童医院近年来确诊的组织细胞增生症X110例中伴有脊椎破坏者14例。其中病理检查确诊5例,余8例除脊椎破坏外尚有组织细胞增生症X的一般表现而确诊,另1例仅有胸椎破坏经抗结核治疗无效改用化疗后明显缓解确诊。脊椎破坏可出现脊神经受压迫症状,表现为肢体麻木、疼痛、无力和麻痹。X线常见2～3个以上不相邻的椎体破坏,受压变形呈扁平椎,椎体不自溶,椎间隙多正常,可见有椎弓破坏。临床易误诊为脊椎结核,椎体骨软骨瘤,脊椎转移癌,尤文氏瘤等。病变早期应用化疗可尽快缓解,合并肢体瘫痪者配合手术治疗。病变广泛加用放疗效果满意。     

组织细胞增生症X35例

组织细胞增生症Ｘ３５例浙江医科大学附属儿童医院（３１０００３）胡春燕，姜节安，沈文娟组织细胞增生症Ｘ（ＨＸ）目前又称郎罕细胞组织细胞增生症（Ｌａｎｇｅｒｈａｎｓｃｅｌｌｈｉｓｔｏｃｙｔｏｓｌｓ，ＬＣＨ），小儿少见。现分析３５例。临床资料对象均为住院患...     

组织细胞增生症X1例报告

患儿,男,5岁。维吾尔族。因发热伴肝大2月于1982年6月24日入院。患儿于2月前,无明显诱因高热40℃,轻咳,呕吐,当地县医院检查发现肝大,拟诊“肝炎”住院治疗,查Hb 4g%,输血150ml     

组织细胞增生症X误诊2例报告

例1,男,1岁。右耳后局限性隆起1月余,局部无红肿及压痛。检查发现右外耳道内少量脓性分泌物,以“右耳后骨膜下脓肿”入院。入院后全身检查未见病变。右耳廓向前下方移位,耳后上方可见6×4.5×3cm大小囊状隆起,皮肤无红肿。包块穿     

组织细胞增生症X的研究进展

组织细胞增生症X(HX)是一种以郎罕细胞异常增生为病理特征的疾病,据此,已提出用LCH的名称代替HX。它在临床上具有极大的异质性,包含了婴儿急性弥慢性致死性疾病和相对良性的成人孤立性骨病变等不同性质的疾病。免疫学异常的发现支持HX是一种免疫紊乱性疾病,但也有证据表明可能部分病人具有恶性性质。局限性疾病治疗常采用外科局部刮除术、局部注射肾上腺皮质激素和放疗;全身性病变仍多用化疗,VP-16被认为是目前较好的药物。应用抑素等的免疫治疗取得了有意义的结果,但还需更加严密设计的临床研究加以证实。     

噬血细胞性淋巴组织细胞增生症21例

目的　探讨噬血细胞性淋巴组织细胞增生症 (HLH)的临床表现、病因、治疗及预后。方法　回顾分析 1998年 1月～2 0 0 3年 9月我科收治 2 1例HLH的临床资料 ,总结其特点。结果　 1.临床表现主要为发热 ,肝、脾、淋巴结大 ,肝功能损害、黄疸等 ;2 .实验室检查 :外周血常表现为三系减少 ;骨髓增生活跃 ;高三酰甘油和 (或 )纤维蛋白原降低。 3 .病因以感染诱发多见 ,其中EB病毒 5例 ,CMV 3例 ,MP、HIV各 2例。 4.治疗及转归 :2 1例中 8例放弃治疗 ,4例未用激素及人血丙种球蛋白 ,该 12例预后不良 ;9例在病因治疗的基础上加用激素及人血丙种球蛋白 ,其中 7例治愈 ,另 2例预后不良。结论　HLH多由感染诱发 ,为致死性疾病 ,激素及人血丙种球蛋白治疗有望改善预后     

组织细胞增生症 X(附16例报告及文献复习)

本文报告16例小儿组织细胞增生症X,其中勒雪氏病(LS)10例,韩薛柯氏病(HSC)5例及介于两者之间的中间型1例。发病年龄LS 1个月～2岁3个月,HSC 1岁11个月～12岁,中间型为5岁。主要临床表现LS 有发热、皮疹、肝、脾和淋巴结肿大,HSC 有头部肿块或缺损、突眼和尿崩症,中间型有发热和肝脾肿大。LS 和HSC 各有4例以临床及X 线为诊断依据,病理检查确诊LS6例,HSC 及中间型各1例。复习有关文献对本病的病因、发病年龄、特殊类型、临床表现、X 线检查、诊断、治疗和预后等作了简要的讨论。     

郎格罕组织细胞增生症诊治疗研讨：附87例分析

本文报道郎格罕组织细胞增生症８７例，计ＬＳ２４例（２７．０％），ＨＳＣ１６（１８．３％），间型１４例（１０．１％）及ＥＧＢ３３例（３８．０％）。除对各型的临床表现、实验室检查、Ｘ线型象等特点作了比较和归纳，并提出在诊断时需注意的某重要线索及提高诊断可信度的措施，还结合对本病的治疗体会作了初步的研讨。对其中５６例作了长期随访，总有效率８５．７％，死亡８例（１４．３％），均属重症ＬＳ；疗效较１９７６年     

儿童颅骨嗜酸性肉芽肿

目的 探讨颅骨嗜酸性肉芽肿的临床表现、诊断和治疗.方法 对2003年1月至2010年12月间我科收治的儿童颅骨嗜酸性肉芽肿48例进行回顾性分析.所有病例术前均行头颅CT检查,其中12例行头颅MRI检查,均行长骨和扁骨的X线片检查,均行手术治疗和病理检查.结果 顶骨为好发骨.1例多发,1例合并锁骨病变.所有病例均行手术全切,术后均未行放疗.15例因局部硬脑膜、帽状腱膜受浸,术后应用吲哚美辛治疗6周.随访6个月至7年(平均3.2年),均无复发.结论 颅骨嗜酸性肉芽肿为良性病变,在诊断时需警惕多发病灶.手术切除是单发病灶的最佳治疗方法,术后不需要侵袭性化疗和放疗,预后好.     

肥厚性硬脊膜炎的诊断与治疗

肥厚性硬脊膜炎罕见，我院近年来收治２例，均经手术和病理证实。本病致瘫率高，预后差，临床表现不典型，容易误诊。复习国外有关文献，认为对无明显病因而发生脊髓压迫症，Ｘ线片上无异常改变者，应注意排除本病。脊髓造影和ＭＲＩ对本病的诊断颇有帮助。本病一旦确诊应及时手术治疗，以免脊髓发生不可逆的损害。     

Percutaneous vertebroplasty for eosinophilic granuloma of the cervical spine in a child

We report a case of eosinophilic granuloma at the fourth cervical vertebra in a 10-year-old girl presenting with a 1-month history of cervical pain and stiffness. This lesion was histologically diagnosed by needle biopsy and then treated by percutaneous vertebroplasty. After the procedure, the cervical pain and stiffness resolved rapidly. The height of the vertebral body remained stable without further collapse over a 6-month follow-up period.     

Congenital intraspinal neuroblastoma with intraspinal calcification in the neonatal period: Report of a case with a 32-year follow-up

Punctate and amorphous intraspinal calcification associated with interpediculate widening in a neonate with lower extremity paresis is probably pathognomonic for congenital calcified intraspinal neuroblastoma.     

Deep Granuloma Annulare (Pseudorheumatoid Nodule) in Children: Clinicopathologic Study of 35 Cases

Deep granuloma annulare (DGA) is one of several lesions of skin and superficial soft tissues whose histologic character is a palisading granuloma with a small central focus of necrosis or necrobiosis. Unlike the other palisading necrobiotic lesions, DGA has a predilection for children in the first 5 to 6 years of life. A painless subcutaneous nodule(s) in the lower anterior tibial region or foot and the scalp, typically in the occiput, was the most common presenting feature in this study of 35 cases. Additional or recurrent lesions were reported in approximately 70% of cases with clinical follow-up. All lesions showed the presence of necrobiosis; however, one of the characteristic features was the multinodular character of the predominantly mononuclear cellular aggregates. The presence of vascular spaces at the periphery of the nodular profiles served as a clue to the diagnosis of DGA. The palisading arrangement of the mononuclear cells was evident only in those foci with central necrobiosis. A histiocytic disorder or fibrohistiocytic process was a common consideration in the differential diagnosis, especially in those cases with less apparent foci of necrosis. Palisading histiocytes with prominent eosinophilic cytoplasm and some nuclear atypism were problematic with regard to possible epithelioid sarcoma. Our study failed to identify any underlying or predisposing factors in the development of DGA. Despite the fact that DGA is a well-documented lesion in children, it occurs sufficiently infrequently that it is often not considered clinically when it presents as a subcutaneous mass or masses in a child. Its recognition by the pathologist is especially important as the occurrence of additional lesions in a high proportion of children can be anticipated without undue concern. Received June 16, 1997; accepted October 28, 1997.     

Herniation of calcifying discopathy in childhood: Report of three cases

Herniation of the calcified nucleus pulposus through the fibrous annulus is unusual in childhood. We present three cases and have surveyed 32 cases in the literature. Most herniations are located in the lower cervical and upper thoracic spine. Herniation is usually symptomatic. Usually calcification decreases after some months. For most children conservative therapy with bedrest and analgesics is sufficient.     

小儿精索静脉曲张11例报告

报告１１例小儿精索静脉曲张，年龄５～１２岁。根据局部体征该症诊断并不困难，但多因无临床症状而被忽视。１１例均行精索内静脉高位结扎术。术中测量静脉断面直径平均３．２０ｍｍ；７例顺行性静脉造影，４例显示血管变异；９例病理组织学观察，发现在小儿睾丸已发生损害。为减少不育症，主张早期手术治疗。     

Limp as unusual presentation of Langerhans' cell histiocytosis

An isolated eosinophilic granuloma involving the posterior elements of a lumbar vertebra is reported in a 3-year-old boy presenting with progressive limp. Radiological investigations revealed osteolysis of the L5 right pedicle. MRI showed a well-defined homogeneous mass with nonspecific signal intensity. An unusual feature was the paravertebral muscular location of the largest part of the tumour indicating a possible soft tissue origin. Immunohistochemical studies were typical for Langerhans' cell histiocytosis. Conclusion A limp can be due to lumbar and paravertebral muscular location of Langerhans' cell histiocytosis. Received: 16 June 1998 / Accepted in revised form: 7 October 1998     

小儿先天性淋巴水肿48例诊治体会

目的：通过病例分析，研究先天性淋巴水肿几种治疗方法的优劣与指征。方法：复习４８例行天性淋巴水肿，其中３例术前核林巴显像确诊。本组８例地真皮瓣埋藏术，３７例行病变切除如游离植皮，１例行淋巴静脉吻合术，２例未手术。结果：８便行真皮瓣埋藏术者６例效果满意，３７例行病变切除加游离植皮者效果均满意。结论：核素淋巴显像是一种诊断淋巴水肿的可靠客观方法。本交轻者行真皮瓣埋藏术效果较好，较重者宜行病变切除加自体植