儿童马尔尼菲青霉菌感染2例临床和病理分析

目的　探讨儿童马尔尼菲青霉菌感染的临床特点和病理特征。方法　通过淋巴结活检和尸体解剖的肺、肝、脾、淋巴结的病理片观察真菌的形态 ;用酶免疫法和免疫印迹法检测病例 1患儿血清人类免疫缺陷病毒 (HIV)抗体 ;用流式细胞仪检测病例 1患儿的CD3、CD4、CD8和CD1 5 5 6。结果　例 1为艾滋病合并马尔尼菲青霉菌感染 ,例 2为播散型马尔尼菲青霉菌感染。例 1的CD4显著降低 ,HIV ELISA、免疫印迹法检测阳性 ,淋巴结活检可见马尔尼菲青霉菌的腊肠状细胞和桑葚小体。对例 2进行尸体解剖 ,肝、脾、淋巴结和双肺中均发现青霉菌的典型的腊肠状细胞和桑葚小体。结论　儿童马尔尼菲青霉菌感染是机会性感染 ,主要继发于先天性免疫缺陷和艾滋病 ,早期诊断、早期治疗对预后的影响很大。     

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.     

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。     

婴儿播散性马内菲青霉感染一例

患儿男,10个月.因反复发热20余天入院.体检:精神萎靡,体温38.5℃,心率:140次/min.双侧颌下淋巴结蚕豆大小,活动.口腔黏膜见白色凝乳状斑片,附着紧密.咽部充血明显,双侧扁桃体不大.叩诊心界不大,听诊律齐,各瓣膜区未闻及杂音.双肺未闻及干湿哕音及哮鸣音.腹软,肝右肋下10 cm,脾左肋下8 cm.     

非HIV相关马尔尼菲篮状菌感染23例临床分析

目的 探讨非HIV马尔尼菲篮状菌感染患儿的临床特征和诊治方案，以提高儿童马尔尼菲篮状菌感染的诊疗水平。方法 回顾性分析23例马尔尼菲篮状菌感染的非HIV患儿的临床资料，总结其人口学特征、临床表现、实验室检查、并发症、治疗及转归。结果 23例患儿中男15例、女8例，年龄3个月～13岁(中位年龄22个月)。最常见的临床特征为发热(22/23，95.7%)、咳嗽(18/23，78.3%)和肝肿大(18/23,78.3%)等，此外皮肤受累9例(39.1%)。常见的严重并发症包括脓毒性休克(13/23，56.5%)、噬血细胞综合征(12/23，52.2%)、急性呼吸窘迫综合征(11/23,47.8%)、多器官功能障碍综合征(10/23，43.5%)等。7例患儿IgG下降，6例IgM升高，9例IgE升高，13例NK细胞计数下降。对11例患儿进行基因检测，发现6例有原发免疫缺陷病。87.0%患儿(20/23)经血培养证实马尔尼菲篮状菌感染，其中9例同时经骨髓培养证实，另2例分别通过皮肤活检和肺泡灌洗液高通量测序技术(NGS)确诊。20例患儿给予抗真菌治疗。最终12例(52.2%)死亡，其中9例已接受...     

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。     

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??Objective??To analyze the characteristics of disseminated penicillium marneffei??PSM?? in children and to deepen the understanding of PSM in the context of non-HIV. Methods??The clinical data??treatment program and prognosis of 15 children were retrospectively analyzed??who were diagnosed with non-HIV disseminated PSM in the First Affiliated Hospital of Guangzhou Medical University from Jan. 2005 to June 2016. Results??The 15 children??male??female??9??6?? had a median age of 23 months with a range of 3 months to 4 years and 10 months of age. All of them had clinical manifestations of fever and hepatomegaly on admission??which were often associated with cough??tachypnea??splenomegaly and lymphadenectasis. ESR was elevated by 93.3%??14/15?? in laboratory tests??80%??8/10?? was positive in fungal G tests??and 87.5%??7/8?? in fungal GM tests. Chest imaging studies revealed that the lungs were all involved and showed various forms. Bone marrow culture and lymph node biopsy showed the highest positive rate of PM??more than 90%. The prognosis was related to the duration of the disease and anti-fungal treatment. The duration of the death group??n??7?? was significantly longer than that of the cured group??n??8????P??0.05??. The duration of anti-fungal treatment for death groups was less than 2 weeks with the main death reason of septic shock and multiple organ failure. The cured group was given amphotericin B or voriconazole intravenously 2-4 weeks and later it was changed to itraconazole for oral maintenance??there was no recurrence after six months of follow-up. Conclusion??Non-HIV disseminated PSM in children occurs more often in infants under 3 years of age??and clinical and laboratory diagnosis lack specificity. Multi-site culture or biopsy??especially bone marrow culture and lymph node biopsy?? can help confirm the diagnosis. Patients with long course of disease without timely anti-fungal treatment are associated with infectious shock and multiple organ failure??which are the main cause of death.     

Epstein-Barr virus infection rapidly progressing to monoclonal lymphoproliferative disease in a child with selective immunodeficiency

We report on a 30-month-old previously healthy Turkish boy who presented with fever, hepatosplenomegaly and generalized lymphadenopathy. He died 4 months after admission in spite of treatment with steroids, acycloguanosine and cyclophosphamide. Epstein-Barr virus (EBV) DNA was detected in the patient's bone marrow and in a lymph node biopsy. Cells from the lymph node biopsy showed monoclonal rearrangements of immunoglobulin heavy chain genes but no rearrangements of T-cell receptor -chain genes or immunoglobulin kappa chain genes. Serological data indicated chronic active EBV infection. There was a slight increase of CD8 positive cells in peripheral blood and a normal response to T-cell mitogens. However, T-cell lines established with interleukin 2 from lymph node biopsy completely failed to kill autologous EBV-transformed B-cells and K 562 target cells. Moreover, in regression tests the patient's peripheral blood mononuclear cells completely failed to limit outgrowth of autologous EBV infected B-cells. We conclude that the patient's selective immunodeficiency had led to the rapid development of EBV-associated monoclonal lymphoproliferation.     

Serum immunoglobulin E levels in human immunodeficiency virus‐infected children with pneumonia

Elevated serum immunoglobulin E (IgE) levels have been reported in association with human immunodeficiency virus (HIV) infection in adults, but there is little information in children. The aim of the present study was to compare serum IgE levels in HIV‐positive and ‐negative children hospitalized with pneumonia in South Africa and to investigate whether IgE may be useful as a marker of specific infections or prognosis in HIV‐infected children. History, examination, blood tests, and induced sputum or bronchoalveolar lavage were carried out. Of 122 children [45% female, median age 8 months (3–20 months)], 81 were infected with HIV. A history of allergy or asthma was present in three children (two of whom were HIV positive). Serum IgE was higher in HIV‐infected children [83 (33–147) vs. 29 (6–113) IU/l; p = 0.011] as was immunoglobulin G (IgG) [49 (37–63) vs. 27.5 (23–34) g/l; p < 0.001]. CD4 lymphocytes [600 (330–1210) vs. 1900 (1500–3030) cells/µl], percentage CD4 cells [13.6 (9.4–20.3) vs. 40.1 (31.1–44.9)] and CD4 : CD8 ratio [0.3 (0.2–0.4) vs. 2 (1.4–2.8)] were lower in HIV‐positive children (p < 0.001 for all). Bacteremia occurred in 12 (10%) children; other specific pathogens identified included Mycobacterium tuberculosis in eight (7%) and Pneumocystis carinii in nine (7%). There was no correlation with CD4 count, CD4 : CD8 ratio, or the presence of specific pathogens, and IgE level. In‐hospital mortality (11%) did not correlate with IgE levels. HIV‐infected children with pneumonia have higher serum IgE compared with seronegative patients. In HIV‐positive children, IgE levels did not correlate with the degree of immunosuppression or with outcome.     

Acyclovir-resistant varicella infection with atypical lesions in a non-HIV leukemic infant

An HIV-negative infant presented with VZV primary infection during the maintenance therapy for megakaryoblastic leukaemia. The lesions were initially vesicular and necrotic but became verrucous and hyperkeratotic. A clinical resistance to acyclovir was suspected and confirmed by histologic and virologic studies. The patient was successfully treated by foscarnet. CONCLUSION: resistance of VZV to acyclovir may occur after a short treatment in a non-AIDS patient.     

Acute pancreatitis associated with varicella infection in an immunocompetent child

Acute pancreatitis associated with varicella-zoster infection is a rare event, particularly in immunocompetent children. We report on a case of acute pancreatitis in a 6-year old girl presenting with acute abdominal pain less than 72 hours after the onset of a typical vesicular rash. The diagnosis was confirmed through hyperamylasaemia, ultrasonographic findings of oedematous pancreatitis and acute seroconversion to varicella-zoster virus, after excluding more common causes of acute pancreatitis. Conservative treatment was initiated, with complete resolution of symptoms and absence of local or systemic complications on follow up. A review of the literature revealed only three previously reported cases, with very different outcomes, highlighting the need to consider this potentially fatal complication in every child presenting with acute abdominal pain during the course of varicella disease.     

Transient global amnesia in a young child

Unprovoked and unexplained sudden loss of memory and inappropriate behaviour in a 5 year old child is described as fitting into the clinical picture of 'transient global amnesia'. The likely pathophysiology of this condition is discussed with some support for Fisher's suggestion of 'hippocampal-fornical' dysfunction.     

Familial high factor VIII level in a child with necrotizing fasciitis complicating primary varicella infection

This article describes an unusual association of familial high plasma factor VIII level and necrotizing fasciitis in a 4-year-old girl with primary varicella infection.     

Multicentric castleman disease in a child with primary immunodeficiency

Multicentric Castleman disease is a rare lymphoproliferative disorder mostly seen in adults with HIV. It presents with fever and systemic symptoms and is extremely uncommon in children. We describe a novel case of multicentric Castleman disease associated with primary immunodeficiency (common variable immunodeficiency) and discuss pathophysiologic mechanisms and recent advances in understanding this disease. Pediatr Blood Cancer. 2010;55:1198–1200. © 2010 Wiley‐Liss, Inc.     

Hemolytic uremic syndrome related to cryptosporidium infection in an immunocompetent child

Hemolytic‐uremic syndrome (HUS) is characterized by the clinical and laboratory manifestations of acute renal failure, thrombocytopenia and microangiopathic hemolytic anemia. In children, the majority of cases occur after an infectious diarrhea mainly associated with the serotype Escherichia coli O157:H7. We present a case of a 5‐year‐old boy with post‐diarrhea HUS due to cryptosporidium. The child remained on peritoneal dialysis for 24 days. However, he had a full recovery of his renal function and 6 months later, his overall condition was still very good. This is a particularly interesting case, not only due to the exceptionally rare cause of HUS, that is, the cryptosporidium, but also because of the serious gastroenteritis caused by the cryptosporidium in an immunocompetent child. It seems that in cases of post‐diarrhea HUS, apart from E. coli O157:H7, even the rarest causes of gastroenteritis should be investigated.     

Recurrent pneumococcal meningitis in a patient with transient IgG subclass deficiency

We report the case of a 3 year old boy who exhibited recurrent serious infections with a transient imbalance of IgG subclass in the second year of life. He suffered from pneumococcal meningitis at 3 months, hepatitis at 9 months, and purulent arthritis at 11 months of age. The second episode of pneumococcal meningitis occurred at 14 months. Serum IgG level was normal for age. Low level of IgG2, undetectable level of IgG4 and negligible level of pneumococcus-specific IgG1-G2 antibodies were found. No other primary immunodeficiency was apparent. Serum IgG2-G4 levels but not pneumococcus-specific IgG1-G2 titers increased by the age of 30 months. At that time, he was inoculated with a polyvalent pneumococcal vaccine along with acellular diphtheria-pertussis-tetanus vaccine. He acquired the immunity against these agents, and had no episodic infections in the following 2 years. This observation stresses the existence of transient IgG subclass deficiency associated with delayed development of the anti-polysaccharide antibody response.     

Malignant insulinoma in a child

Insulinomas are rare tumors with an estimated incidence of one per 250,000 person‐years. Most insulinomas are benign with less than 10% demonstrating malignant behavior, the vast majority of which occur in adults. A systemic review of the literature revealed only nine cases of malignant insulinomas occurring in children. Herein, we present a case of metastatic malignant insulinoma in a 12‐year‐old child. The occurrence of this diagnosis in a child, its unusual pattern of metastases and the challenging management of severe hypoglycemia make this case worth reporting. Pediatr Blood Cancer. 2010;55:1423–1426. © 2010 Wiley‐Liss, Inc.