220例不明原因长期发热病因分析

目的探讨不明原因长期发热(FUO)的病因及诊断方法。方法对我院2004年1月～2007年1月期间收治的220例FUO患者的病例资料进行回顾性分析。结果220例FUO中,明确诊断212例,其中感染性疾病占74.55%(164/220),非感染性疾病占21.82%(48/220)。病因未明者占3.64%(8/220)。结论根据临床经过和必要的辅助检查可以明确FUO中的大多数病例的病因诊断。     

An unexpected cause of a febrile patient with huge splenomegaly

We report an unexpected cause of a febrile patient with huge splenomegaly. A 32-year-old patient with fever and huge splenomegaly was admitted to our hospital. Diagnostic splenectomy revealed that the enlarged spleen adhered strongly to the abdominal organs. Pathologically, the splenic parenchyma showed no malignant cells, and the soft tissue adjacent to the splenic hilum showed a proliferation of fibroblastic or myofibroblastic spindle cells with fibrosis and lymphoplasmacytic infiltration. These findings lead to a diagnosis of peritoneal fibrosis, and an administration of 50 mg/day of prednisolone alleviated all the symptoms. The differential diagnosis of huge splenomegaly with fever usually includes hematolymphoid malignancies and infectious diseases; however, our case was diagnosed as idiopathic retroperitoneal fibrosis. Our case suggests that when we see patients with fever and huge splenomegaly, differential diagnosis should include retroperitoneal fibrosis.     

First Indications of Omsk Haemorrhagic Fever Virus beyond Russia

Omsk haemorrhagic fever virus (OHFV) is the agent leading to Omsk haemorrhagic fever (OHF), a viral disease currently only known in Western Siberia in Russia. The symptoms include fever, headache, nausea, muscle pain, cough and haemorrhages. The transmission cycle of OHFV is complex. Tick bites or contact with infected small mammals are the main source of infection. The Republic of Kazakhstan is adjacent to the endemic areas of OHFV in Russia and febrile diseases with haemorrhages occur throughout the country—often with unclear aetiology. In this study, we examined human cerebrospinal fluid samples of patients with suspected meningitis or meningoencephalitis with unknown origins for the presence of OHFV RNA. Further, reservoir hosts such as rodents and ticks from four Kazakhstan regions were screened for OHFV RNA to clarify if this virus could be the causative agent for many undiagnosed cases of febrile diseases in humans in Kazakhstan. Out of 130 cerebrospinal fluid samples, two patients (1.53%) originating from Almaty city were positive for OHFV RNA. Screening of tick samples revealed positive pools from different areas in the Akmola region. Of the caught rodents, 1.1% out of 621 were positive for OHFV at four trapping areas from the West Kazakhstan region. In this paper, we present a broad investigation of the spread of OHFV in Kazakhstan in human cerebrospinal fluid samples, rodents and ticks. Our study shows for the first time that OHFV can not only be found in the area of Western Siberia in Russia, but can also be detected up to 1.600 km away in the Almaty region in patients and natural foci.     

Adult-onset Still's disease—pathogenesis,clinical manifestations,and new treatment options

Abstract

Adult-onset Still's disease (AOSD), a systemic inflammatory disorder, is often considered a part of the spectrum of the better-known systemic-onset juvenile idiopathic arthritis, with later age onset. The diagnosis is primarily clinical and necessitates the exclusion of a wide range of mimicking disorders. AOSD is a heterogeneous entity, usually presenting with high fever, arthralgia, skin rash, lymphadenopathy, and hepatosplenomegaly accompanied by systemic manifestations. The diagnosis is clinical and empirical, where patients are required to meet inclusion and exclusion criteria with negative immunoserological results. There are no clear-cut diagnostic radiological or laboratory signs. Complications of AOSD include transient pulmonary hypertension, macrophage activation syndrome, diffuse alveolar hemorrhage, thrombotic thrombocytopenic purpura and amyloidosis. Common laboratory abnormalities include neutrophilic leukocytosis, abnormal liver function tests, and elevated acute-phase reactants (ESR, CRP, ferritin). Treatment consists of anti-inflammatory medications. Non-steroidal anti-inflammatory drugs have limited efficacy, and corticosteroid therapy and disease-modifying anti-rheumatic drugs are usually required.

Recent advances have revealed a pivotal role of proinflammatory cytokines such as tumor necrosis factor-α (TNF-α), interleukin (IL)-1, IL-6, IL-8, and IL-18 in disease pathogenesis, giving rise to the development of novel targeted therapies aiming at optimal disease control.

The review aims to summarize recent advances in pathophysiology and potential therapeutic strategies in AOSD.     

不明原因发热患者病因临床分析

目的探讨引起不明原因发热（FUO）的致病因素及其构成比。方法分析两年来符合不明原因发热诊断标准的198例患者的临床资料。结果198例中明确诊断者192例，确诊率为96．97％，未确诊者6例，占3．03％。确诊病例中，不明原因发热的病因依次为细菌感染性疾病（44．27％）、自身免疫性疾病（19．27％）、病毒感染性疾病（11．46％）。确诊的有效方法包括细菌学培养、病毒检测、病理检查、影像学检查。结论根据病史、临床经过和必要的辅助检查，可以明确大多数FUO病因诊断。感染性疾病和自身免疫性疾病是本组不明原因发热的主要病因。     

The role of F-FDG PET or PET/CT in the detection of fever of unknown origin

Even with the recent advance in diagnostic tools and techniques, fever of unknown origin (FUO) remains a clinical challenge. A wide range of diseases, mainly infections, autoimmune conditions (inflammatory diseases), malignancies and miscellaneous can cause FUO. Positron emission tomography (PET) or positron emission tomography/computed tomography (PET/CT) scanning makes a great contribution to the diagnosis and differential diagnosis of FUO due to the high sensitivity of pathological accumulation of ¹⁸F-FDG. The diagnostic yield of PET/CT is higher than traditional radiographic imaging and other nuclear medicine scanning. Owing to the numerous advantages of PET/CT including high sensitivity and the ability to perform whole-body scans, many rare diseases presenting with FUO can be detected and the spectrum of diseases that can exhibit FUO has been increasing. Recent studies utilizing FUO are discussed in this paper. However, there are limited data available about the role of ¹⁸F-FDG PET or PET/CT in evaluation of FUO.     

原因不明发热患者的骨髓像检查附366例分析

目的:通过对366例原因不明发热(FUO)患者骨髓像检查并结合临床进行分析,探讨骨髓像检查在FUO患者诊断中的意义。方法:对FUO患者进行血常规,骨髓像检查。结果:诊断血液系统疾病患者158例(43.2%),其中各种白血病75例,其它血液系统疾病83例;各种感染性疾病99例(27.0%),其中包括传染性单核细胞增多症11例,其余88例为非特异感染骨髓像(其中46例为结核病);骨髓像无特殊变化者109例(29.8%)。结论:提示骨髓检查是FUO患者查找病因的重要实验诊断技术之一,对FUO患者诊断应根据临床进行骨髓穿刺检查。     

Giant Hepatic Hemangioma Causing Prolonged Fever and Indicated for Resection

Hepatic hemangiomas are benign liver tumors, and most of them progress asymptomatically. We report a case of hepatic hemangioma considered the cause of fever. A 53-year-old woman had a fever of 40°C for about 3 months without infection. Hepatic hemangiomas with internal bleeding of 10 cm in size on liver S8/7 and S3/2 were observed. These were resected laparoscopically for diagnostic treatment. She was afebrile after the operation. The pathological diagnosis was hematoma inside cavernous hemangioma. It should be noted that a bleeding hepatic hemangioma may cause fever of unknown origin and be indicated for resection.     

Fever in hospitalized medical patients

The occurrence of fever and the clinical profile of febrile patients on the medical service of a teaching hospital were studied prospectively. Thirty-six per cent of 972 patients developed fever (temperature exceeding 38°C). Their 13% mortality rate and 13.2-day average hospital stay exceeded the 3% mortality and seven-day hospitalization for afebrile patients (p<0.0001 for both). Most fever episodes occurred during the first two hospital days. Approximately 30% of first and subsequent fever episodes were caused by bacterial infections; illnesses involving tissue necrosis (e.g., stroke, myocardial infarction) accounted for 20%. Five conditions comprised 53% of diagnoses: respiratory and urinary tract infections, neoplasm, myocardial infarction, and drug reaction. Only one patient had a fever of uncertain origin. Several clinical clues used frequently to identify bacterial infections were reevaluated. Patients with bacterial infections had higher temperatures on the first febrile day (mean 38.9°C) and were more likely to have had prior infections than those with other causes of fever (mean 38.3°C, p<0.001). Older patients (>75 years) had a lower febrile response to bacterial infections than younger patients. Fever in hospitalized medical patients is a common and important concomitant of increased mortality and length of hospitalization. Supported in part by grants from the National Center for Health Services Research (HS 02063 and HS 04066) and by a grant from the Henry J. Kaiser Family Foundation. The work was performed, in part, while Dr. Bor was a Henry J. Kaiser Fellow in General Medicine, Harvard Medical School.