Rhodococcus equi pneumonia in AIDS: a case report

We describe the first case, in Campania, of Rhodococcus equi pneumonia in an HIV positive subject. The clinical symptoms resolved after antibiotic therapy. The pulmonary lesion at 10 months of radiological follow up results just partially reduced in its diameters.     

Camurati-Engelmann's disease: a case report

Camurati-Engelmann's disease is a rare condition worldwide. No cases have been documented in Uganda. A 26 year old female presented with a history of grinding pain in the limbs for over 20 years. Strong painkillers would temporally relieve the pain. She had an asthenic stature with generalised reduction in muscle bulk. Plain x-rays revealed the characteristic symmetrical thickening and sclerosis of the diaphyses of the appendicular skeleton and skull base, which is pathognomonic of Camurati-Engelmann's disease. Involvement of the metaphyses of these long bones as well as the metacarpal bones makes this an unusual case.     

弥漫型胃黏膜皱襞肥大症一例

患者男,55岁。2个月前无明显诱因出现双下肢水肿同时稍感上腹部不适,进食后尤其明显,食欲减退,于200年6月19日入院。入院前于外院CT检查示:胃壁平滑肌瘤可能性大,胃腔变小,胃镜检查未见明显异常。上消化道造影提示:胃癌、右侧胸膜肥厚。外科查体:腹平坦,全腹无压痛、反跳痛及肌紧张,左上腹可触及一8cm×9cm×15cm大小的肿块,无触痛,双下肢出现凹陷性水肿。实验室检查:白蛋白25g/L。病理检查:全胃组织,大弯长39cm,小弯长21cm,胃体直径12cm,胃角直径6.5cm,网膜面积为23cm×16cm,沿胃大弯剪开胃组织,可见弥漫性黏膜皱襞粗大,弥漫至胃底、大小…     

Encephalopathy as a presentation of pediatric AIDS: case report

Fifty to ninety percent of pediatric AIDS cases are complicated by neurologic dysfunction. We present a case of a 5-year-old black female with AIDS encephalopathy and Mycobacterium avium intracellulare. Her initial presentation was that of neuroencephalopathy with loss of developmental milestones, pyramidal tract signs, and subsequent evidence of cortical atrophy. Her initial CT scan at the time of frank encephalopathy was normal, whereas 18 months into the clinical course of her encephalopathy, her CT scan of the head demonstrated typical ventricular dilatation and severe cortical atrophy consonant with her clinical picture. She subsequently developed Mycobacterium avium intracellulare documented by gastric aspirate culture and other opportunistic infections including Candida esophagitis. Her neuroencephalopathy plateaued with continued evidence of immune dysfunction and mycobacterium by gastric aspirate, despite triple antibiotic therapy with INH, streptomycin, Pyrazinamide with later addition of Rifampin and final substitution of the investigational congener Rifabutin. AIDS encephalopathy and Mycobacterium intracellulare are discussed in terms of their prognosis and therapy, particularly in view of new reports of the application of AZT and immunoglobulin therapy.     

Fabry病一例

患者女,41岁。因发现蛋白尿1个月于2006年3月15日入院。患者1个月前因腰痛在当地医院就诊,尿常规检查提示蛋白＋＋,当时未予特殊处理,后多次查尿常规检查均提示有蛋白尿,为进一步诊治入本院肾内科。调查家族史,其父死于心脏病,其母、兄弟姐妹及儿女未发现类似病患。体检：各器官未见异常,全身皮肤末见皮疹。[第一段]     

Von Hippel-Lindau disease: a case report

Von Hippel Lindau (VHL) disease is a rare autosomal dominant condition manifested by central nervous system hemangioblastoma, retinal angiomas, cysts of pancreas, kidney and epididymis, pheochromocytomas and renal cell carcinoma. We present such a case in a 45 years old male patient.     

Erdheim-Chester disease: a case report.

A 42-year-old man with Erdheim-Chester disease (EC) is presented. This is the first case of this disease reported in Korea. The patient complained of knee pain and plain roentgenogram of the bilateral legs revealed diffusely increased density, coarsened trabecular pattern, and cortical thickening in the diaphysis, and metaphysis as well as epiphysis. Magnetic resonance imaging revealed that the lesions showed low signal intensity on T1-weighted images and heterogeneously low and high signal intensity on T2-weighted images. Histological examination of the biopsy specimen showed a xanthogranulomatous lesion consisting aggregations of foamy histiocytes and Touton-type giant cells. Immunohistochemical staining showed positive reaction to anti-S-100 and lysozyme in the cytoplasm of the giant cells.     

肾小球囊肿病一例

患者女,46岁.因体检发现左肾囊性占位性病变2年于2010年1月入院.患者2008年体检发现左肾囊肿3 cm×3 cm,现囊肿增大,无尿频、尿急、腰腹痛、血尿、发热等病史.超声示左肾中极囊性占位约4 cm×5 cm.     

Niemann Pick disease A: a case report

The Niemann Pick disease is a rare lysosomal storage disease responsible for numerous cytological abnormalities of blood cells and bone marrow. The diagnosis requires enzymatic dosages, which can be long and difficult. In this context, the detection of inconstant cytologic anomalies in blood and bone marrow smears, allowing a rapid screening, is an important step in the diagnostic approach. We report the case of a 6?year-old child who presents with abdominal distension; medullogram was performed and revealed the presence of vacuolated cells overload. Correlated with clinical and biochemical data, medullogam results confirmed the diagnosis of type A?Niemann Pick disease.     

肺脏Rosai-Dorfman病一例

患者男,44岁.因间断性发热4个月于2006年6月20日入院治疗.无咳嗽、咳痰及咯血等症状.体检:体温37.2～38.6℃,双侧颈部及全身浅表淋巴结未触及肿大,双肺呼吸音粗,未闻及干湿性哕音,肝脾未触及肿大.薄层靶扫CT表现:左肺下叶外侧基底段见一类圆形软组织密度影,边缘清楚,密度均匀,邻近支气管血管束呈推压移位改变,未见确切侵袭征象.     

A case of Penicillium marneffei infection in an AIDS patient: the first case in Japan.

A 38-year-old Japanese AIDS patient developed papular lesions which rapidly increased in number, eroded and crusted, and spread over not only skin but also the mucosal surface. High fever, sore throat, malaise and hepatosplenomegaly were also noted, and he died despite 2 months of intensive treatment. An autopsy revealed numerous histiocytes infected with Penicillium marneffei in the lymph nodes, liver, spleen, bone marrow, skin, and mucosal surface of the oral cavity to the pharynx. This case is thought to be the first Japanese case of penicilliosis marneffei.     

Hydatid disease of the spine: a case report

Hydatid disease of the spine is rare and has a poor prognosis, presenting both diagnostic and therapeutic challenges. The diagnosis is based on epidemiological, clinical, biological and radiological arguments. Treatment is primarily surgical. In this observation we report the case of a 45?years old man hospitalized for back pain with heaviness of lower limbs and upper sphincter disorders. Through this case we emphasize the role of the parasitological laboratory in the diagnosis and management of this disease.     

Peripheral neuropathy in Whipples disease: a case report

Whipples disease is a chronic multisystem inflammatory disease with predominantly gastrointestinal manifestations due to Tropheryma whipplei infection. Typical neurological abnormalities include dementia, eye movement abnormalities, hypothalamic dysfunction and oculomasticatory myorhythmias. The literature on peripheral neuropathy in Whipples disease is sparse and the involvement of peripheral nerves in Whipples disease has not been documented convincingly so far. We present a case of Whipples disease presenting by axonal peripheral neuropathy without gastrointestinal involvement. The diagnosis was confirmed by a sural nerve biopsy and consequent PCR of the sample. All clinical signs disappeared progressively during the antibiotic therapy. Two years after the T. whipplei infection, the patient developed dopa-sensitive Parkinson's disease, although these two events seem to be unrelated. This case illustrates the value of peripheral nerve biopsy in cases of axonal neuropathy of unexplained origin and extends the clinical spectrum of Whipples disease to a new modality.     

Castleman's disease, intermediate type: a case report

Castleman's disease or giant lymph node hyperplasia is a distinct form of lymph node hyperplasia. Histologically, there are 2 variants, hyaline vascular variant which occurs in 90% of the cases and plasma cell type which is rare. We encountered a 70 year old man with Castleman's disease showing intermediate features consisting of both hyaline vascular and plasma cell elements presenting as cervical lymphadenopathy. Following surgical excision, the patient was relieved of his constitutional symptoms and had no recurrence even after 6 months of follow-up.     

复杂性紫绀型先天性心脏病1例

患者，男，8岁，发现紫绀7年，发热、咳嗽、反复发作，于2003年12月8日收入我院治疗。入院体检：体温36．5℃，脉搏96次／min，呼吸18次／min，血压14．65／8．66kPa，一般情况好。皮肤、巩膜无黄染。头颅无畸形，气管居中。双肺呼吸音清晰，未闻及干、湿罗音。心尖搏动在左第5肋间隙锁骨中线处，弥散，可扪及震颤，心率96次／min，律齐，心前区可闻及Ⅵ级收缩期杂音，以胸     

Weber-Christian disease presenting with proptosis: a case report

Weber-Christian disease (WCD) is a rare inflammatory disease of adipose tissue, which is characterized by painful cutaneous nodules and constitutional symptoms. Although any area of the body containing fat can be affected by WCD, the involvement of retrobulbar fat is uncommon and proptosis is a rare presenting manifestation. We report a case who presented with proptosis of the right eye which is accompanied by painful subcutaneous nodules, high fever and myalgia. Biopsies of retrobulbar tissue and suprapubic nodule showed lobular panniculitis with mixed cellular infiltration, mainly composed of histiocytes and lymphocytes. He responded well to high-dose glucocorticoid.     

Light-chain deposition disease of the kidney: a case report

A 41-year-old man was admitted for evaluation of nephrotic syndrome associated with microhematuria, hypertension, and moderate renal failure. In serum and urine samples, monoclonal IgG-lambda was detected. Bone marrow examination showed normal representation of all cell lines with normal range of plasma cells. Renal biopsy demonstrated diabetes-like nodular glomerulosclerosis. Immunofluorescence failed to demonstrate the presence of kappa or lambda light chains in the kidney. Electron microcopy showed granular electron-dense deposits along the glomerular basement membranes and in the mesangial nodules. The patient was diagnosed as having light-chain deposition disease (LCDD) without evidence of plasma cell dyscrasia. This report was designed to stress the significant challenges that remain in the diagnosis of LCDD-related glomerulopathy. The salient morphological features that help in making an accurate diagnosis are discussed.