胰腺脂肪增多症一例

<正>胰腺脂肪增多症,是一种罕见疾病,是指胰腺脏器内脂肪沉积增多,包括实质细胞内脂肪沉积及实质组织被脂肪组织替代,又称胰腺脂肪替代、多脂肪性胰腺萎缩、胰腺脂肪浸润等。自1993年首次提出胰腺脂肪增多症概念以来,相关报道有所增多,发现与其相关的疾病越来越多,包括肥胖、糖尿病、非酒精性脂肪性肝病等多种代谢性疾病,但对其发病机制仍不清楚,诊断仍未形成统一的共识。目前主要依据影像学检查辅助诊断。目前国内外     

高原红细胞增多症的研究进展

高原红细胞增多症是常见的一种慢性高原病,多在海拔3 000m以上地区发病,严重危害着高原人群的身体健康。近年来,国内外对高原红细胞增多症发病率、发病机制、治疗、预防等方面做了较多的研究,现就其相关研究进展阐述如下。     

小儿朗格汉斯细胞组织细胞增生症胸部影像诊断分析

目的 探讨小儿朗格汉斯细胞组织细胞增生症的临床及胸部影像特征。方法 回顾性分析经病理证实的2例朗格汉斯细胞组织细胞增生症患儿临床及胸部影像学资料。结果 小儿朗格汉斯细胞组织细胞增生症影像学表现典型:双肺弥漫分布小结节和气囊影,胸腺增大及钙化。结论 小儿朗格汉斯细胞组织细胞增生症胸部影像学表现典型,影像学检查诊断该病有较高的临床价值。     

儿童朗格汉斯细胞组织细胞增生症临床病理分析

目的探讨儿童朗格汉斯细胞组织细胞增生症临床病理特征,总结该病的诊疗要点。方法采用SP免疫组化法,应用多种抗体对4例儿童朗格汉斯细胞组织细胞增生症进行标记,观察其表达特点。结果4例儿童朗格汉斯细胞组织细胞增生症镜下形态和免疫标记有相同之处,病灶内均见大量朗格汉斯细胞、嗜酸性粒细胞,朗格汉斯细胞CD1a、CD68及S-100呈强阳性表达。结论儿童朗格汉斯细胞组织细胞增生症的诊断和治疗需结合临床与病理综合评定。     

肌少症与肝硬化的研究进展

肌少症是肝硬化的一种常见合并症,发病率为20％～70％.肌少症暂无诊断金标准,CT为常用的诊断方法.肝硬化发生肌少症的机制是多样的,尽早对合并肌少症者进行干预可改善预后.本文将从肌少症特点、诊断方法、发病机制、治疗与干预方面详细阐述,以期为后续研究提供借鉴.     

成人脊柱朗格汉斯细胞组织细胞增生症误诊1例

脊柱朗格汉斯细胞组织细胞增生症发病率低,临床表现无特异性,且病理组织获得难度大,因此临床误诊及漏诊率高。本文对1例开始误诊为脊柱结核最终通过手术病理活检确诊为脊柱朗格汉斯细胞组织细胞增生症的病例进行报道,以提高对本病的认识,减少误诊率。     

腰椎管狭窄症骶管内注射疗法72例

自从1954年 verbiest 氏提出腰椎管狭窄症以来,国内外有关报告陆续增多。对其病因、发病机制、诊断及治疗的认识也逐渐提高。在本症的治疗中,各家不一。本组自1979年6月～1981年8月应用普鲁卡因氟美松骶管内注射疗法72例中,而无效的19例中,10例又进行了手术治疗。现将普鲁卡因氟美松骶管注射疗法的操作、疗效及其作用机制,介绍如     

嗜酸细胞性肺炎

1　概　述嗜酸细胞性肺炎是一种与肺嗜酸细胞浸润有关的变态反应性疾病 ,病因与寄生虫感染、真菌感染、药物过敏等有关 ,但很多病因不很明确 ,发病机制亦不清楚。本病亦可称为 :嗜酸细胞肺浸润、嗜酸细胞浸润性肺炎、肺嗜酸细胞增多症、嗜酸细胞增多综合征等 ,这有待今后进一步统一。根据其临床特点本病可分为以下五型 :(1)单纯型嗜酸细胞性肺炎 (Loffler综合征 )。 (2 )慢性或迁延型嗜酸细胞性肺炎。 (3)哮喘型嗜酸细胞性肺炎。(4)热带型嗜酸细胞性肺炎。 (5 )过敏性肉芽肿性血管炎 (Churg -Strauss综合征 )。2　临床特点2 .1　病因　嗜…     

黏附在子宫内膜异位症发生发展中作用的研究现状

子宫内膜异位症(简称内异症)是日趋增多的生育年龄妇女的常见病,其发病机制不清,易于浸润和复发。主导理论是经血反流,种植和体腔上皮化生学说。内膜的种植与生长要完成黏附,侵袭和血管形成“三部曲”。故黏附是内膜异位病灶形成的第一步,是内异症形成和发展的基础。随着分子生物学研究的进展,发现许多因子在黏附过程中起着重要作用,本文就与黏附相关因子的研究作一综述。1细胞黏附分子与子宫内膜异位症细胞黏附分子是由细胞产生的、存在于细胞表面介导细胞与细胞之间,细胞和细胞外基质(extracelluar m atrix)之间相互接触和结合的一类分子,…     

皮质醇增多症的诊断和治疗现状

皮质醇增多症是因肾上腺过度分泌糖皮质激素引起的临床综合征 ,1912年由HarveyCushing首先报道 ,故又称库欣综合征 (Cushingsyndrome)。皮质醇增多症可在任何年龄发病 ,但多发于 2 0～ 4 5岁 ,女性多于男性 ,男女比例为 1:3～ 8。1　皮质醇增多症的诊断1.1　皮质醇增多症的分类　皮质醇增多症可分为促肾上腺皮质激素 (ACTH)依赖性和非依赖性两大类。ACTH依赖性皮质醇增多症是因下丘脑 -垂体或垂体外的肿瘤组织分泌过量ACTH或促肾上腺皮质激素释放激素 (CRH) ,引起双侧肾上腺皮质增生并分泌过量的皮质醇 ,最常见的为垂体分泌过量ACT…     

Evaluation of Cheek Edema in an Infant Reveals Langerhans Cell Histiocytosis

BackgroundLangerhans cell histiocytosis is a rare hematological disorder. Skin rash is the typical early feature, and bony involvement is the second most common presentation.MethodsWe present a case of a 5-month-old female infant with left hemifacial swelling, initially treated for infection with antibiotics. However, due to persistence of swelling and new onset fever, further evaluation with ultrasonography, CT scan, FDG PET/CT and eventually biopsy was performed.ResultsImaging methods revealed mandibular osteolysis indicative of either osteomyelitis or histiocytosis X. Tissue biopsy was diagnostic for Langerhans cell histiocytosis.ConclusionLangerhans cell histiocytosis may present in infancy with a variety of symptoms, included an isolated bony lesion. Langerhans cell histiocytosis, despite its rarity, should be included in the differentiated diagnosis, when bone osteolysis is found.     

MRI findings in pituitary-hypothalamic axis Langerhans cell histiocytosis

Pituitary-hypothalamic axis Langerhans cell histiocytosis is an uncommon entity. It is a rare disease in adults. The CT and MR study provides us the best anatomo-topographic evaluation and determine the precise size of the lesion which are necessary to the treatment. We report a case of hypothalamic involvement by Langerhans cell histiocytosis accompanied by lesions in bone affecting a 31-year-old woman. The clinical, histiotological and CT/MR findings of histiocytosis X are described in this article.     

A neonate with a skin rash post partum

A term-born boy presented with a rash immediately post partum, consisting of erosions, crusts and a few vesicles. Skin biopsy showed dermal infiltration of S100 and CD1a immunopositive histiocytes. The diagnosis was 'congenital Langerhans cell histiocytosis of the skin'.     

21例儿童髂骨骨质破坏性病变的临床分析

目的 总结儿童髂骨骨质破坏性病变的临床特点.方法 回顾性分析2013年1月至2015年12月,西安市儿童医院收治的髂骨骨质破坏患儿的临床资料.对21例患儿术后通过切除的病灶标本确定病变性质,分析其临床特点.结果 患几年龄为24天～11.5岁,中位年龄3.5岁;男∶女=2.5∶1.朗格汉斯细胞增生症11例(9例多发,2例单发),骨髓炎3例,尤文肉瘤2例,多发性纤维结构不良2例,骨软骨瘤、臀部横纹肌肉瘤和急性淋巴细胞白血病各1例;有12例患儿在髂骨病灶之外还有多处病灶.所有病例影像学均表现为髂骨不同形态的骨质破坏,发生在髂骨翼19例,髋臼2例.8例伴有大小不等的软组织肿块,5例见骨膜反应.以髋部疼痛为首发症状9例,以下肢跛行为首发症状6例.经99锝全身骨扫描阳性率仪占57.1％,多病灶者准确率为66.7％.结论 儿童髂骨骨质破坏以朗格汉斯细胞增生症最多见,常合并多处骨病灶.临床表现特异性不高,确诊仍需病理检查.     

Adult Langerhans’cellhistiocytosis with multisystem bone and lung involvement : About a case

Introduction: Langerhans cell histiocytosis (LCH) is a rare systemic disease characterized by the abnormal overproduction of histiocytes that tend to infiltrate single or multiple organ systems leading to significant tissue damage. Although LCH can involve various organs including bone, skin, and lymph nodes, multisystem involvement of LCH is rare in adults Case presentation: We report the case of a 31-year-old man with LCH involving his lungs and bone. The initial radio-clinical presentation was rather in favor of pulmonary tuberculosis. Through this observation we draw attention to this rare pathology and we discuss the diagnostic elements and the therapeutic management of this pathology. Conclusion: Although it is occasionally difficult to discriminate LCH from other disorders, systemic evaluation might be helpful for differential diagnosis. As LCH isn’t infiltrating malignant cells, strong chemotherapy regimen is not recommended in order to avoid severe toxic and side effects.     

Rare association of rare diseases: Langerhans-cell histiocytosis and lymphangioleiomyomatosis in the lung]

This is a case history of a 24-year-old female patient in whom two rare pulmonary diseases occurred: Langerhans cell histiocytosis and four years later lymphangioleiomyomatosis were diagnosed. Both diseases were verified by the examination of the lung tissue removed by the surgery for pneumothorax. The patient's symptoms were characterised by coughing, dyspnoe, repeated pneumothorax. The authors summarised the characteristics and treatment of these diseases. This case history is worth of reporting for its unique rareness.     

Langerhans' cell histiocytosis in an adult patient manifested as recurrent skull lesions and Diabetes Insipidus

We describe a 57-year old female with the diagnosis of skull and meningeal Langerhans' cell histiocytosis who was treated with the combination of azathioprine and methotrexate. Although the skull lesions improved considerably on this regimen, the patient developed diabetes insipidus while the anterior pituitary function remained intact.     

The role of parental and perinatal characteristics on Langerhans cell histiocytosis: characterizing increased risk among Hispanics

Purpose

Potential roles of inherited and environmental risk factors in pathogenesis of Langerhans cell histiocytosis (LCH), a myeloid neoplastic disorder, are undefined. We therefore evaluated the role of parental and perinatal factors on the risk of this childhood cancer.