HIV阴性儿童播散性马尔尼菲青霉病致乳糜腹水1例报告

<正>马尔尼菲青霉病(penicilliosis marneffei,PSM)是由马尔尼菲青霉菌(penicillium marneffei,,PM)引起的一种少见的机会性真菌感染。马尔尼菲青霉菌是条件致病菌,主要感染免疫功能缺陷或免疫功能抑制者,尤其是艾滋病患者。关于马尔尼菲青霉菌感染的HIV阴性患者病例报道不多,儿童更为少见。现报告1例HIV阴性儿童播散性马尔尼菲青霉病致乳糜腹水病例,并结合相关文献进行复习,以提     

播散性马尔尼菲青霉菌感染二例报道并文献复习

目的 探讨儿童播散性马尔尼菲青霉菌感染的实验室检查、临床表现和治疗策略.方法报道分析播散性马尔尼菲青霉菌感染患儿临床表现,病原学检查,影像学资料和治疗结果.结果病例1,男,1岁,发热、咳嗽1个月;骨髓、血培养:马尔尼菲青霉菌;伏立康唑治疗后好转.病例2,女,8岁,发热1个月,烦躁1d;血培养:马尔尼菲青霉菌.结论儿童播散性马尔尼菲青霉菌感染可累及中枢神经系统.伏立康唑可作为静脉-口服序贯治疗策略的选择.     

幼儿反复发热、肝脾大、嗜酸性粒细胞增多

患儿,男,2岁,因咳嗽、发热第6次入院。患儿2月龄时发现腋下淋巴结结核,多次因发热、咳嗽住院,并发现嗜酸性粒细胞增多,以及多次免疫全套提示IgG、IgA、IgM降低。入院后予以抗结核、抗细菌、抗真菌治疗,仍反复发热、肝脾进行性增大,入院第35天出现果酱样大便、B超提示左侧腹局部肠管横断面呈“同心圆征”,于全麻下行剖腹探查、肠套叠复位+回盲部成形术以及肠壁结节、肠系膜淋巴结活检、肝活检术,术后患儿出现肝功能衰竭、DIC、电解质紊乱,救治无效死亡。入院后血培养及肝活检均发现马尔尼菲青霉菌;并进一步对患儿、患儿父母及弟弟进行免疫缺陷病相关基因检测,提示患儿存在CD40L基因半合突变 （IVS1-3T→G）,确诊为高IgM综合征。患儿母亲为此拼接点错误基因的携带者、父亲正常,患儿弟弟与患儿有相同的CD40L基因突变。     

播散性马尔尼菲青霉病致死一例

马尔尼菲青霉病国内文献已屡有报道，但仍常有漏诊或误诊。漏诊原因是临床表现像肺结核、败血症或恶性网织细胞增多症等；误诊原因是它主要侵犯肺、肝、脾、淋巴结和骨髓及组织中的菌体形态、大小与主要位于巨噬细胞内等特点酷似荚膜组织胞浆菌。主要鉴别是该菌特有的腊肠样长形菌体及个别菌可见横隔。马尔尼菲青霉病散发流行于我国南方和东南亚各国。各种原因的免疫力低下易感染该病，并已成为东南亚各国获得性免疫缺陷综合征（ＡＩＤＳ）的常见并发症。治疗深部真菌有效的药物均适用治疗本病，但要持续治疗半年以上方免复发。今介绍播散性马尔尼菲青霉病一例，以帮助读者认识及诊断马尔尼菲青霉病。     

儿童原发性免疫缺陷合并马内菲青霉菌病1例并文献复习

目的提高儿童马内菲青霉菌病的临床诊断水平。方法回顾分析1例原发性免疫缺陷病合并马内菲青霉菌病患儿的临床资料并进行相关文献复习。结果反复发热、"肺炎"、肝脾进行性肿大、皮疹、免疫功能低下等为马内菲青霉菌病的基本特征,骨髓细胞形态学检查见马内菲青霉菌及血液真菌培养发现马内菲青霉菌生长是确诊依据。结论认识马内菲青霉菌病的临床特征,避免漏诊、误诊。     

儿童马尔尼菲青霉菌感染的诊治进展

马尔尼菲青霉菌(PM)是青霉菌中唯一的温度双相型真菌,可引起全身播散性马尔尼菲青霉菌病(PSM)。儿童PM感染多见于有免疫缺陷的宿主,由于缺乏特异性临床表现,易误诊为肺组织胞浆菌病、肺结核或侵袭肺曲霉病等。播散性的PSM进展快,并发症多,如未能及时给予有效治疗,死亡率高,故早期诊断和治疗十分重要。诊断主要包括免疫功能检...     

儿童艾滋病三例分析

目的 报道3例儿童艾滋病，以提高对儿童艾滋病的认识。方法 1999年1月－2001年7月，北京儿童医院收治的3例艾滋病患儿均经免疫层析法、酶联免疫ELISA双抗原夹心法筛查及蛋白印记法确认抗人类免疫缺陷病毒（HIV）抗体阳性，诊断为艾病病。结果 3例中男性2例，女性1例，年龄为4－6岁，均以长期反复咳嗽为主诉，病史长达3个月至2年，全身临床表现有长期间断发热（3例）、明显消瘦（3例），慢性腹泻（2例）等，在身体各器官中，以肺、脑、肾的表现较为突出：肺部以反复急、慢性感染为主要表现，X线及CT表现各异。2例头颅CT示脑萎缩，其中1例程度较重，临床表现为明显的智力发育落后及进行性运动退化；2例有肾脏改变，1例为双贤弥漫性纤维化，另1例表现为双肾饱满、尿检异常。1例合并有丙肝病毒感染，胆肝功能正常。实验室检查3例均显示不同程度的贫血和免疫功能低下，尤其以CD4明显降低为主要特点，3例中2例有多次输血史，1例的父母为HIV感染者。该3例入院后曾被诊断为反复呼吸道感染、营养不良、免疫缺陷病等，因怀疑艾滋病而做HIV抗体检测，确诊为艾滋病，达到北京市佑安医院进一步治疗。结论 提高对儿童艾滋病的认识，重视影响感染及发病的危险因素，对临床可疑病例及时查血HIV抗体，提高临床检出率。     

儿童获得性免疫缺陷综合征临床特点

目的 探讨儿童获得性免疫缺陷综合征(AIDS)的病因、临床特征、诊治经过及预后,以提高对该病的认识.方法 对2001年以来在广西医科大学第一附属医院儿科住院的12例儿童AIDS或疑似AIDS的临床及实验室检查资料进行回顾性分析,以总结其临床特点.结果 12例患儿中男9例,女3例;年龄45 d-9岁;11例患儿父母血清均为人类免疫缺陷病毒(HIV)抗体阳性或疑似因AIDS死亡者;临床表现为生长发育迟缓并不同程度营养不良12例(100%),长期不规则发热、间断性咳嗽及肝脾大各10例(83.3%),迁延性腹泻及淋巴结大各8例(66.7%).全身性皮炎6例(50%),大脑发育不全4例(33.6%),口腔念珠菌感染3例(26.7%),中耳炎2例(16.7%);实验室检查:12例2次血清HIV抗体检测均呈阳性,CD4 T淋巴细胞的百分比均下降,CD4 /CD8 细胞比值均降低,肝功能检查转氨酶均升高,3例血清巨细胞病毒抗体阳性,4例血培养及1例骨髓培养出马尔尼菲青真菌,大便涂片及培养4例找到真菌.死亡1例,余病例对症治疗效果差.结论 儿童AIDS潜伏期相对较短,临床表现多样化,细胞免疫功能低下,对症治疗效果差.提高对该病的认识,可早期诊断、早治疗,延长患儿生命.     

儿童非人类免疫缺陷病毒感染相关播散性马尔尼菲青霉菌病15例临床回顾分析

目的　总结非人类免疫缺陷病毒（HIV）感染相关儿童播散性马尔尼菲青霉菌病（penicillium marneffei,PSM）特点,提高对其的认识。 方法　回顾性分析广州医科大学附属第一医院儿科2005年1月至2016年6月诊断的15例非HIV感染相关播散性PSM患儿的临床资料、治疗方案及转归。 结果　15例患儿中,男∶女＝9∶6,中位年龄23个月（3个月至4岁10个月）。入院时均有发热、肝大,多伴有咳嗽气喘、脾大、淋巴结肿大。实验室检查红细胞沉降率升高93.3%（14/15）,真菌G试验阳性80.0%（8/10）,真菌GM试验阳性87.5%（7/8）。胸部影像学检查提示15例肺部均有累及,表现形态多样。骨髓培养和淋巴结组织活检马尔尼菲菌阳性率最高（＞90%）。预后与病程长短及抗真菌治疗疗程相关,死亡的7例患儿病程明显长于治愈的8例（P＜0.05）,抗真菌治疗时间均＜2周,主要死亡原因是感染性休克及多脏器衰竭。治愈患儿采取两性霉素B或伏立康唑静滴2～4周后改为伊曲康唑口服维持,随访半年以上无复发。结论　儿童非HIV感染相关播散性PSM好发于婴幼儿,临床及实验室诊断缺乏特异性。同时进行多部位体液培养或组织活检（尤其是骨髓培养和淋巴结活检）有助于明确诊断。病程偏长、未进行及时抗真菌治疗的患儿容易合并感染性休克及多脏器衰竭,是导致死亡的主要原因。     

儿童艾滋病17例临床报告

目的通过对17例艾滋病患儿的临床分析,初步了解儿童艾滋病的临床表现、并发症及传播途径。方法回顾分析1999-01—2006-05北京儿童医院收治的17例艾滋病患儿的临床资料。17例患儿均经免疫层析法及酶联免疫(ELISA)双抗原夹心法筛查及蛋白印记法确认人类免疫缺陷病毒(HIV)抗体阳性,确诊儿童艾滋病。结果17例患儿年龄8个月至11岁,平均7·8岁,其中男13例,女4例。籍贯为12例来自于河南,2例来自山西,1例来自甘肃,1例来自山东,1例来自四川。病程最短20d,最长2年。14例出现间断发热,11例有咳嗽,其中2例有咯血,8例出现腹泻。12例有明显消瘦,体重低于同龄正常儿童2个标准差,3例表现为皮肤反复瘀点、瘀斑,6例患儿肝脏肿大,7例白细胞降低,肝功能异常9例,15例CD4+T淋巴细胞明显下降,平均13%,胸片提示8例有肺部异常表现,其中1例为肺部结核,1例为支气管扩张,1例疑为机会性感染,其余为肺内非特异性炎症,腹部B超提示6例肝脏肿大。6例合并肺部感染,2例败血症,1例结核,1例支气管扩张,2例鹅口疮,1例灰指甲,1例真菌性肠炎,1例新型隐球菌脑膜炎。17例患儿中12例有输新鲜血、血浆及血液制品史,2例患儿母亲HIV抗体阳性,3例无输血及血制品史,父母HIV抗体未查。结论儿童艾滋病常以发热伴反复咳嗽或发热伴迁延性腹泻为就诊表现;真菌感染可能是儿童艾滋病主要并发症;儿童艾滋病早期可表现为皮肤瘀点、瘀斑;儿童艾滋病发病的地域性与成人艾滋病一致;血源性传播仍然是我国儿童艾滋病主要传播途径。     

Clinical utility of liver biopsy in children with acquired immunodeficiency syndrome

BACKGROUND: Little is known about hepatic histology in children with AIDS, although the liver is frequently involved in the course of HIV infection. The clinical utility of liver biopsy in these patients is not well-defined. We reviewed retrospectively the results of this procedure in a group of infected children better to delineate its indications. PATIENTS AND METHODS: Eighteen children with AIDS underwent liver biopsy in our institution. The indications were unexplained fever in eight children, six of whom had an elevated erythrocyte sedimentation rate and clinical suspicion of mycobacterial infection; jaundice in four; suspicion of drug toxicity (dideoxyinosine) in two; discussion of treatment for chronic hepatitis B in three; suspicion of cytomegalovirus infection in one who had also AIDS cholangiopathy. RESULTS: Of the six children thought to have mycobacterial infection, two had the disease on biopsy, both of whom had abnormal liver enzymes. The children with unexplained fever had nonspecific findings, except for one with lymphoid interstitial pneumonitis who had a dense lymphoid infiltrate. Of the four with jaundice two had extensive necrosis caused by adenovirus infection in one and suspected herpes simplex infection in the other. The other two with jaundice had unexplained findings, severe necrosis and fibrosis in one case and hemophagocytosis in the other one; both improved clinically. Both children with suspected dideoxyinosine hepatotoxicity had nonspecific findings. The three children with chronic hepatitis B had mild lesions that were not an indication for treatment. CONCLUSIONS: Liver biopsy appeared to be useful in two groups of selected children with AIDS: when there is strong clinical suspicion of mycobacterial infection; and when the child is jaundiced.     

Morphologic findings in children with acquired immune deficiency syndrome: pathogenesis and clinical implications

The lesions observed in biopsy and autopsy material from children with the acquired immunodeficiency syndrome (AIDS) can be divided into three pathogenetic categories: primary lesions related to infection by human immunodeficiency virus (HIV) (e.g., lymphoreticular system and brain); lesions due to the sequelae of HIV infection (e.g., opportunistic infections, pulmonary lymphoid lesions, etc.); and lesions of undetermined pathogenesis (e.g., renal lesions, cardiomyopathy, etc.). The role of morphologic studies in AIDS in understanding the pathogenesis of the various lesions and their clinical implications are discussed by describing the following examples among others. Study of the thymus enabled us to distinguish AIDS from some congenital immune deficiency syndromes. Thymic injury contributes to immunodeficiency in AIDS. Its apparent irreversibility will have to be considered in the long-term management of children with AIDS when specific effective therapy for HIV becomes available. Demonstration of HIV--like particles in the characteristic giant cells in the brain has been instrumental in the recognition of HIV encephalopathy. Biopsy is helpful in the rapid diagnosis of opportunistic infections (OIs). Autopsy study of OIs has shown involvement of clinically unsuspected organs, such as the adrenals. Characterization of the pulmonary lymphoid lesions led to their inclusion as a diagnostic criterion for AIDS in children. Progression of pulmonary lymphoid lesions to a lymphoproliferative disorder was demonstrated at autopsy. Recognition of lesions such as cardiomyopathy and arteriopathy at autopsy should alert clinicians to suspect these disorders during life.     

Morphologic Findings in Children with Acquired Immune Deficiency Syndrome: Pathogenesis and Clinical Implications

The lesions observed in biopsy and autopsy material from children with the acquired immunodeficiency syndrome (AIDS) can be divided into three pathogenetic categories: primary lesions related to infection by human immunodeficiency virus (HIV) (e.g., lymphoreticular system and brain); lesions due to the sequelae of HIV infection (e.g., opportunistic infections, pulmonary lymphoid lesions, etc.); and lesions of undetermined pathogenesis (e.g., renal lesions, cardiomyopathy, etc.).

The role of morphologic studies in AIDS in understanding the pathogenesis of the various lesions and their clinical implications are discussed by describing the following examples among others.

Study of the thymus enabled us to distinguish AIDS from some congenital immune deficiency syndromes. Thymic injury contributes to immunodeficiency in AIDS. Its apparent irreversibility will have to be considered in the long-term management of children with AIDS when specific effective therapy for HIV becomes available. Demonstration of HIV—like particles in the characteristic giant cells in the brain has been instrumental in the recognition of HIV encephalopathy.

Biopsy is helpful in the rapid diagnosis of opportunistic infections (OIs). Autopsy study of OIs has shown involvement of clinically unsuspected organs, such as the adrenals. Characterization of the pulmonary lymphoid lesions led to their inclusion as a diagnostic criterion for AIDS in children. Progression of pulmonary lymphoid lesions to a lymphoproliferative disorder was demonstrated at autopsy. Recognition of lesions such as cardiomyopathy and arteriopathy at autopsy should alert clinicians to suspect these disorders during life.     

Disseminated adenovirus (type 19) infection in a neonate. Rapid detection of the infection by immunofluorescence

A case of fatal disseminated adenovirus infection in a neonate who suffered from severe keratoconjunctivitis and pneumonitis is reported. The diagnosis was made seven days after the onset of illness based on the detection of adenovirus antigen in the smears of the tracheal suction and conjunctival swab by immunofluorescence. Viral antigen was detected in the frozen or formalin-fixed autopsy specimens of the lungs, kidneys, spleen, liver and lymph nodes. Typical crystal arrangement of adenovirus virions was observed in the alveolar epithelial cells by electron microscopy. The isolated virus was identified to be of type 19 by a neutralization test. The IF examination using adenovirus group specific immune reagents on the smears of clinical specimens appears to be useful for rapid diagnosis of viral infections.     

Disseminated Adenovirus (Type 19) Infection in a Neonate Rapid Detection of the Infection by Immunofluorescence

ABSTRACT. A case of fatal disseminated adenovirus infection in a neonate who suffered from severe keratoconjunctivitis and pneumonitis is reported. The diagnosis was made seven days after the onset of illness based on the detection of adenovirus antigen in the smears of the tracheal suction and conjunctival swab by immunofluorescence. Viral antigen was detected in the frozen or formalin-fixed autopsy specimens of the lungs, kidneys, spleen, liver and lymph nodes. Typical crystal arrangement of adenovirus virions was observed in the alveolar epithelial cells by electron microscopy. The isolated virus was identified to be of type 19 by a neutralization test. The IF examination using adenovirus group specific immune reagents on the smears of clinical specimens appears to be useful for rapid diagnosis of viral infections.     

FAMILIAL HEMOPHAGOCYTIC LYMPHOHISTIOCYTOSIS: AN AUTOPSY STUDY

We describe four classical cases of familial hemophagocytic lymphohistiocytosis (FHL), a macrophage-related, autosomal recessive fatal disorder. Parental consanguinity was present in three cases. All patients presented with fever, neurological involvement of varying degrees, hepatosplenomegaly, cytopenias, deranged liver function tests, and coagulogram, hypofibrinogenemia (three cases), and hyperlipidemia (one case). An antemortem diagnosis could not be made, although it was suspected in one case. Necropsy (done in three cases and postmortem liver biopsy in one case) revealed classical features of FHL. Florid lymphohistiocytic infiltrate exhibiting hemophagocytosis was seen in the bone marrow, liver, spleen, lymph nodes and brain (examined in two case). In addition to this, focal infiltrates were seen in the kidneys, lung, pancreas, testes, adrenals, and skin. Marked lymphoid depletion was seen in one case in the lymph nodes and spleen.     

Imaging features of Mycobacterium avium-intracellulare complex (MAC) in children with AIDS

Purpose. The purpose of this paper was to review the imaging features of Mycobacterium avium-intracellulare complex (MAC) in 16 pediatric patients with human immunodeficiency virus (HIV).¶Materials and methods. We reviewed the pertinent clinical records of 16 children diagnosed with MAC between January 1990 and June 1998. These 16 cases were blood- or biopsy-proven to have MAC infection. Their plain films, abdominal, and chest CT scans were then reviewed and the findings were analyzed with reference to the few reported cases of children with MAC.¶Results. Abdominal findings: all but one had retroperitoneal adenopathy, mesenteric adenopathy or both. Ten patients had hepatomegaly, while nine patients were found to have splenomegaly. Four patients had nonspecific thickened gallbladder wall, while intestinal wall thickening and thickened stomach folds were identified in six of ten patients. Necrotic, fluid-filled nodes were also found. Chest findings included mediastinal adenopathy, cystic/cavitary lesions and bronchiectasis. One patient developed a fistula between the mediastinal lymph nodes, esophagus, and bronchial tree.¶Conclusion. Pediatric patients with HIV who develop MAC infection may present with massive lymph-node enlargement. This can occur not only in mesenteric and retroperitoneal nodes but also in hilar and posterior mediastinal nodes as well. As in MTB infection, these nodes can break down with development of fistulous tracts to both esophagus and adjacent lung. The major differential diagnostic consideration besides MTB is lymphoma.     

Cerebral infarction in pediatric acquired immunodeficiency syndrome

Cerebral infarction is an uncommon complication of AIDS in pediatric patients. We have seen three HIV-infected children who developed acute neurological deficits due to stroke. Cerebral infarction must be considered in the work-up of a child with AIDS who presents with focal neurological deficit, seizure or mental status change.Stroke is a complication of HIV infection that occurs in approximately 1% of affected children [1]. At autopsy, evidence of cerebral infarction was documented in 10–30% of children with HIV infection [2]. We have seen three children with focal infarction who are HIV positive.