Goldenhar 综合征家系的临床表型和遗传学分析

目的分析1组Goldenhar综合征家系的临床表现及遗传学特征。方法我们随访到1组4代33人的Goldenhar综合征家系，对目前存活的29人进行了临床表型和遗传学的初步分析。结果家系内有Goldenhaar综合征患者5人．临床表现具高度多样性，累及眼、耳、脊柱、颜面、口腔等多个器官和系统的发育不良，在遗传方式上属于常染色体显性遗传。从细胞遗传学水平对家系中成员进行染色体检查，未发现核型异常。结论该Goldenhar综合征家系属常染色体显性遗传，染色体检查未发现核型异常。     

老年精神分裂症合并肺炎临床分析

目的：通过对精神分裂症合并肺炎患者和老年肺炎患者进行对照观察分析，探索老年精神分裂症合并肺炎的临床特点，为临床诊治提供资料。方法：对35例CCMD-2-R精神分裂症合并肺部感染的患者及35例同期老年肺炎患者对照分析。结果：发现老年精神分裂症合并肺炎患者与老年肺炎患者在神经精神症状和消化系统症状方面差异有显著性。结论：对老年精神分裂症合并肺炎患者更要细致观察，精心护理，早发现、早诊断、早治疗，以降低其死亡率。     

Epidemiology and clinical manifestation of HIV infection in Northern Zaire

1275 patients were evaluated for HIV-1+2 seroprevalence and its association with clinical symptoms of HIV infection. Of 667 apparently healthy subjects, 8.2% had anti-HIV-1 antibodies. In 465 patients with clinical signs of AIDS, 39.4% were seropositive. 143 patients with miscellaneous symptoms had positive predictive values for HIV infection between 67% (vaginal ulcerations) and 20% (profound pyogenic abscesses). The WHO definition for AIDS had a specificity of 78.3%, a sensitivity of 72.2% and a predictive value of 61.6%.     

Oral manifestation of chronic mucocutaneous candidiasis: seven case reports

BACKGROUND: Chronic mucocutaneous candidiasis (CMC) is a rare disorder characterized by persistent or recurrent candidal infections of the skin, nails and mucous membranes or by a variable combination of endocrine failure as well as immunodeficiency. Oral clinicopathological features of CMC have seldom been described in detail. METHODS: Seven patients with CMC were reported in the study. The clinical and histological findings, etiological Candida species, immunological evaluation, and therapeutic pattern of oral lesions, were analyzed. RESULTS: Long-standing whitish hyperplastic and nodule-like lesions with exaggerated deep fissure were the typical and characteristic oral manifestations presented by all patients. The tongue was the most common site affected. Histologically, no obvious distinction was found between CMC and other forms of candidal infection. Abnormal proportions of T-lymphocyte subsets and positive titers of autoantibody were observed in three subjects (42.9%) and one patient (14.3%) respectively. Meanwhile, four subjects (57.1%) showed decreased albumin and increased globulin, three cases (42.9%) had high levels of ESR. But no iron deficiency was found. Candida albicans was the microorganism isolated from these patients. CONCLUSIONS: Multiple and widespread candidal infectious lesions can be observed on the oral cavity of CMC patients. Hyperplastic and nodule-like lesion with irremovable whitish patches and deep fissure are the most common oral manifestations of these patients. Dentists, otolaryngologists and pediatricians should be familiar with the clinical appearances of CMC to make an accurate diagnosis. Potential systemic disorders should be concerned to avoid the reoccurrence of oral candidiasis.     

脊椎转移瘤的MRI探讨

目的 观察ＭＲＩ各序列对脊椎转移瘤的诊断价值并分析其ＭＲＩ征象。方法 对２２例脊椎转移瘤病人行Ｔ１ＷＩ，Ｔ２ＷＩ，Ｐ（Ｎ）Ｉ，小翻转角准Ｔ２Ｗ等序列检查并对病变信号的清晰度分级。     

Neurological Manifestations of COVID-19 Feature T Cell Exhaustion and Dedifferentiated Monocytes in Cerebrospinal Fluid

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老年性系统性红斑狼疮的特点(附48例临床分析)

目的　探讨老年性系统性红斑狼疮患者的临床特征。方法　总结 48例老年性系统性红斑狼疮患者临床表现及自身抗体分布特点 ,并与中青年患者对比分析。结果　老年组蝶形红斑、盘状红斑、光过敏、脱发、雷诺氏现象等皮肤表现及肾损害较中青年组明显少见 (P<0 .0 5 ) ,浆膜炎、肺部病变、肌痛和肌无力、疲乏、血液系统损害明显高于中青年组 ,而狼疮的两项特异性抗体 (抗ds-DNA和抗Sm抗体 )、非特异性抗体 (抗SS -A和抗SS -B抗体 )以及补体C3 下降的阳性率都低于中青年组 (P <0 .0 5 )。结论　老年性与中青年性系统性红斑狼疮相比在临床表现、免疫学检查等方面有其自身特点 ,在临床诊断中应给予足够重视。     

伴嗜酸性粒细胞增多性血管淋巴样增生的临床病理分析

目的：探讨伴嗜酸性粒细胞增多性血管淋巴样增生的临床病理学特征。方法：对我科1950-1999年期间所诊治的伴嗜酸性粒细胞增多性血管淋巴样增生的7名患者的病理标本重新切片,进行病理学上的分析。结果：伴嗜酸性粒细胞增多性血管淋巴样增生在病理学上有其特征,形态学上的改变主要表现在以下3个方面：①真皮内大量毛细血管增生;②血管内皮细胞增生肿大,似“墓碑”状突入血管腔;③病变处有淋巴细胞、嗜酸性粒细胞混合浸润。结论：伴嗜酸性粒细胞增多性血管淋巴样增生是一种良性局限性血管增生,其病因及发病机制尚不清楚。熟悉这一疾病的组织学改变对避免误诊为其他类拟疾病有重要意义。     

Hantavirus Dobrava infection with pulmonary manifestation

Dobrava virus infection was diagnosed serologically by enzyme-linked immunosorbent and immunofluorescence assays. To determine which hantavirus serotype was involved, sera were analyzed by a focus reduction neutralization test. The clinical data indicated that only pulmonary manifestation was present. Our data support the presence of Dobrava virus infection outside the Balkan region. In conclusion, a previously healthy adult with unexplained pulmonary perfusion failure should be investigated for hantavirus infection. Received: 15 March 1999