Good综合征诊治一例

Good综合征（伴胸腺瘤的免疫缺陷症）是一种成人免疫缺陷病,特点是合并胸腺瘤及低丙种球蛋白血症。该病有细胞和体液免疫双重缺陷,患者常表现为反复感染。该文报道1例62岁AB型胸腺瘤女性患者,反复细菌、真菌和病毒感染,血清Ig低下,B淋巴细胞缺如、CD4⁺ T淋巴细胞下降、CD4⁺/CD8⁺ T淋巴细胞比值下降,诊断为Good综合征。该患者同时有粒细胞缺乏、慢性病性贫血,进一步骨髓宏基因组测序发现KIT与ARID1A基因突变,予补充Ig及抗感染治疗,患者症状好转出院。随访患者仍有Ig下降,予定时复查血常规、Ig,并适时补充Ig。Good综合征相对罕见,临床表现复杂,容易延误诊断,临床医师需引起重视。     

Successful recovery of MERS CoV pneumonia in a patient with acquired immunodeficiency syndrome: A case report

Middle East Respiratory Syndrome Coronavirus (MERS CoV) may cause severe pneumonia with significant morbidity and mortality, particularly in patients with multiple comorbid condition. MERS CoV pneumonia has not been previously reported in patients with Human Immunodeficiency Virus (HIV). Herein, we report a case of MERS CoV pneumonia with a successful outcome in a patient recently diagnosed with HIV.     

HIV/AIDS‐related beliefs among Israeli Arab‐Palestinian women

Studies show that women are more vulnerable to human immunodeficiency virus (HIV) transmission than men because of biological and sociocultural risk factors. Israeli Arab‐Palestinians are the largest ethnic minority group in Israel. Unofficial data suggest that merely one percent of the tests conducted in Israel to diagnose HIV/acquired immune deficiency syndrome (AIDS) are conducted by this population and that Israeli Arab‐Palestinians are typically diagnosed late. Arab communities in Israel were found to be predominately patriarchal, characterised by conservative gender roles and structures, and the policing of overt sexual behaviours, particularly those of women. In order to advance our understanding of the HIV/AIDS phenomenon in different sociocultural contexts, as well as to design effective cultural‐sensitive interventions, we must first understand how HIV/AIDS is constructed in different cultural sites. Against this backdrop, this exploratory study examines HIV/AIDS‐related beliefs among non‐infected, Israeli Arab‐Palestinian women. We conducted semi‐structured interviews with 12 women. Thematic content analysis was used in order to analyse the data. Five themes emerged from the analysis, in the first, personal responsibility and blame were assigned to people with HIV/AIDS for their illness. In the second theme, HIV/AIDS was constructed as a sexually transmitted disease, associated with ‘deviant’ sexuality and sexual conduct in the Arab culture. In the third theme, HIV/AIDS was believed to be transmitted by multiple, mostly mistaken, modes of contact. In the fourth theme, people with HIV/AIDS were believed to be subjected to severe negative social responses. In the fifth theme, individuals with HIV/AIDS were depicted as a symbol of physical and psychological malaise. In conclusion, the findings imply that Israeli Arab‐Palestinian women hold negative beliefs concerning people with HIV/AIDS and are misinformed regarding HIV transmission. This suggests a need to design culturally appropriate interventions to address stigma‐related beliefs and misconceptions concerning transmission, among Israeli Arab‐Palestinian women.     

Multi-system infection – tuberculosis or melioidosis?

Melioidosis is a multi-system disorder which is often mistaken for tuberculosis in the Indian sub-continent. It is caused by the gram-negative bacterium Burkholderia pseudomallei. The imaging findings of melioidosis often mimic many other bacterial infections and the differentiation is sometimes difficult. The clinical manifestations of melioidosis range from asymptomatic and subclinical to acute localized forms, acute septicemia and chronic forms. We report a case of melioidosis presenting with multi-visceral abscesses (with adrenal involvement).     

Pathomorphology of humoral,cellular and combined primary immunodeficiencies

Summary Histologic, immunohistologic and electron microscopic findings in three children with primary immunodeficiencies are reported. Classical X-linked infantile agammaglobulinemia Bruton was present in case 1 (, aged 16 years), selective cellular immunodeficiency with thrombopenia in case 2 (, aged 2 1/2 years) and non-lymphopenic severe combined immunodeficiency in case 3 (, aged 1 3/4 years). At autopsy, all three cases exhibited unusual types of pneumonia. In case 2 a generalized cytomegalovirus infection was present. Case 3 disclosed panmyelopathia and chronic liver lesions due to severe GvH-reaction subsequent to bone marrow transplantation. A detailed morphologic study of the immune system revealed distinct alterations in the thymus, spleen, and lymph nodes and the lymphatic tissues of the gastrointestinal tract characteristic of an immunodeficiency state, either humoral (case 1), cellular (case 2) or combined (case 3).Dedicated to Prof. Dr. Chr. Hedinger for his 60th birthdayThis paper was presented in part at the 7th Fall Meeting of the German Society for Pathology in Mainz, Oct. 29–31, 1976     

故意性途径传播艾滋病现象浅析

目的:调查故意性途径传播艾滋病现象的现状及危害,分析原因,讨论可行的解决方案。方法:通过网络与人类免疫缺陷病毒(human immunodeficiency virus,HIV)感染者、获得性免疫缺陷综合征(acquired immuno deficiency syndrome,AIDS)患者交流及查阅文献,并调查2014年湖北省大冶市新发HIV/AIDS患者婚外性行为、性伙伴数等数据佐证。结果:经故意性途径感染HIV的人群客观存在,老年和青少年尤甚,而湖北省大冶市2014年新发137例HIV/AIDS患者中100%经性途径感染,有婚外性行为者占89%,有2个及以上性伙伴者占81.8%。结论:性途径传播已经成为艾滋病主要感染方式,其中故意传播现象客观存在,加强艾滋病知识宣传,完善相关法律法规,重视故意性途径传播现状,保护易感人群,加大对故意性途径传播者的打击力度,重视受害人群心理干预、安抚受害者,避免其成为下一个传播者是重要的社会问题。     

Rag1、Rag2基因缺陷小鼠的构建及在异种肿瘤移植中的应用

目的 建立重组激活基因,Rag1和Rag2基因缺陷小鼠模型,并对其表型和异种肿瘤移植的成瘤性进行分析.方法 通过传统的胚胎干细胞(ES)细胞打靶、囊胚注射方式,分别构建Rag1和Rag2基因缺陷小鼠模型;通过流式细胞术对缺陷小鼠外周血T/B淋巴细胞含量进行检测;通过外源异种肿瘤细胞移植实验,对移植肿瘤细胞的成瘤性进行评价.结果 建立了Rag1和Rag2基因缺陷小鼠模型,两种基因突变纯合子小鼠模型外周血中T、B淋巴细胞含量较野生型小鼠显著降低;人源肿瘤细胞A549在上述两种基因突变纯合子小鼠中较BALB/c-nu(裸小鼠)或(NOD-SCID)非肥胖糖尿病/重症联合免疫缺陷小鼠表现出更强的成瘤性.结论 分别成功建立了Rag1和Rag2基因缺陷小鼠模型,两种小鼠模型均发生了T、B淋巴细胞生成障碍,导致免疫系统严重缺陷,可作为异种外源肿瘤移植的受体,用于小鼠荷瘤模型的建立.     

Computed tomography scan assessment of lung disease in primary immunodeficiencies

A wide spectrum of lung disease can complicate primary immunodeficiencies and early recognition influences management and prognosis. Computed tomography (CT) especially high resolution computed tomography (HRCT) has been shown to detect lung disease in adult immunodeficient patients often when the chest radiograph (CXR) is normal, but this has not been studied in children. Twenty-five CT scans [10 HRCT] and CXRs were reviewed in 23 children [14 male, 9 female] with primary immunodeficiency. Eighteen [72%] of the CT scans were abnormal, bronchiectasis being the commonest finding present in eight CT scans in patients with antibody deficiency. In eight cases CT scan revealed changes not seen on CXR (bronchiectasis;interstitial changes; small parenchymal nodules; air trapping;and a small upper lobe cyst) which influenced treatment in six cases. Conclusion CT scans have a valuable role in assessing lung disease in children with primary immunodeficiencies and will detect important changes not visible on CXR. Received: 9 December 1997 / Accepted in revised form: 3 June 1998     

Clinical applications of flow cytometry and cell immunophenotyping to companion animals (dog and cat)

Clinical applications of flow cytometry to certain diseases of the dog and cat are now possible. The utility of such applications for diagnosis, prognosis and follow-up are illustrated here by a number of examples: feline AIDS resulting from FIV infection, Leukocyte Adhesion Deficiency in Irish setters, deep pyoderma in German shepherds, Immune-mediated Thrombocytopenia, canine Systemic Lupus Erythematosus and Leishmaniasis, Leukemia and Lymphoma.     

Clinical Characteristics and Treatment Outcomes of Patients with Hepatitis C Virus and Human Immunodeficiency Virus Coinfection: Experience at a Single Center in Korea

BackgroundBecause of the very low incidence of human immunodeficiency virus (HIV) coinfection in Korea, data on hepatitis C virus (HCV)/HIV coinfection are limited. This study aimed to investigate the clinical characteristics and treatment outcomes of patients with HCV/HIV coinfection in Korea.MethodsWe performed a retrospective cohort study of all HCV-monoinfected and HCV/HIV-coinfected patients treated with antivirals at National Medical Center in Seoul, Korea, between January 2009 and March 2020.ResultsWe enrolled 220 HCV-monoinfected and 23 HCV/HIV-coinfected patients treated with antivirals. The HCV/HIV-coinfected patients were younger (HCV vs. HCV/HIV: 57.3 ± 11.3 vs. 40.7 ± 10.1 years, P < 0.001) and had a higher proportion of men (HCV vs. HCV/HIV: 54.5% [n = 120] vs. 91.3% [n = 21], P < 0.001) than the HCV-monoinfected patients. Genotype 1b and 2 were most common in both HCV monoinfection and HCV/HIV coinfection groups. HCV-monoinfected patients had a higher incidence of genotype 1b and 2 than HCV/HIV-coinfected patients (HCV vs. HCV/HIV: 95.4% [n = 210] vs. 73.9% [n = 17], P < 0.001), while the HCV/HIV-coinfected patients had genotype 1a (HCV vs. HCV/HIV: 1.8% [n = 4] vs. 21.7% [n = 5], P < 0.001). The fibrosis-4 index was significantly lower in the HCV/HIV-coinfected patients than in the HCV-monoinfected patients (HCV vs. HCV/HIV: 3.81 ± 3.38 vs. 1.66 ± 1.10, P < 0.001). Among the direct-acting antivirals (DAA)-treated patients, the sustained viral response (SVR) rate did not differ significantly between both groups (HCV vs. HCV/HIV: 94.9% [93/99] vs. 90.9% [10/11], P = 0.480).ConclusionIn Korea, the HCV/HIV-coinfected patients who received antiviral treatment were younger, had higher proportion of men and incidence of genotype 1a, and had less advanced fibrosis than the HCV-monoinfected patients. In actual clinical settings, HCV/HIV-coinfected patients show excellent SVR to DAA treatment, similar to HCV-monoinfected patients.