Hepatobiliary tuberculosis

Tuberculosis is known to involve the liver in different ways. The term hepatobiliary tuberculosis refers to the localized form of hepatic tuberculosis as a distinct clinical entity, with signs and symptoms related to the hepatobiliary tract. Its clinical features and the different diagnostic aids used in its diagnosis are reviewed. Plain abdominal radiographs showing diffuse hepatic calcifications seen in approximately 50% of cases are almost diagnostic for hepatobiliary tuberculosis. Liver biopsies obtained either by ultrasound, computed tomography or laparoscopy, showing caseating granuloma usually establish the diagnosis. In the absence of caseation necrosis, a positive acid-fast bacillus (AFB) or culture for Mycobacterium tuberculosis is needed to establish the diagnosis. A polymerase chain reaction assay for the identification of Mycobacterium tuberculosis in liver biopsy specimens is a new development. Treatment is similar to that used for pulmonary tuberculosis. Quadruple therapy (using four anti-tuberculosis drugs) is recommended, generally for 1 year. For patients with obstructive jaundice, in addition to anti-tuberculous treatment, biliary decompression should be performed either by stent insertion during endoscopic retrograde cholangiopancreatology, by percutaneous transhepatic biliary drainage or by surgical decompression whenever feasible.     

原发性肺隐球菌病52例临床及影像学表现和病理特征

目的 探讨原发性肺隐球菌病(pulmonary cryptococcosis,PC)的临床表现、病理特征、影像学特点及术前误诊原因.方法 回顾分析1998-2008年上海市中山医院收治的52例PC患者的临床、影像学及病理资料.结果 52例PC患者中,男36例,女16例,年龄17～80岁,中位年龄48岁.临床症状较轻,首发症状依次为咳嗽、咳痰及胸痛等.52例中13例既往有慢性疾病或肿瘤病史,其中肝癌并肝移植术后、糖尿病、结核及慢性活动性肝炎各2例,垂体瘤及结节病各1例,糖尿病合并肝痛及肺结核、糖尿病合并结核性胸膜炎及糖尿病合并高血压各1例.45例患者行胸部CT扫描,其中单发或多发结节团块37例,空洞4例,大叶实变3例,弥漫混合性病变1例;CT诊断为恶性肿瘤27例,肺炎15例,结核3例.49例光镜下表现为肉芽肿性病变,3例表现为纤维结缔组织病灶,Grocott六胺银及黏液卡红染色病灶内均可见新型隐球菌.结论 免疫功能正常者亦有发生PC的可能;临床及影像学表现缺乏特征性,且临床症状与影像学表现不符,易误诊为肺癌、肺炎或肺结核;诊断有赖于病理组织学检杳;CT引导下经皮肺穿刺等非开胸肺切除的方法有助于该病的早期诊断.     

Tubercular biliary hilar stricture: A rare case report

Localized hepatic tuberculosis (TB) with or without bile duct involvement is a rare form of hepatobiliary tuberculosis; accounting for less than 1% of all tuberculous infections. We report an uncommon case of cholestatic jaundice with disseminated TB in an immunocompetent male who presented with simultaneous involvement of liver and biliary system.     

Patterns of calcifications and cholangiographic findings in hepatobiliary tuberculosis

The radiologic findings on conventional examinations (plain films and cholangiograms) in a large group of patients with proven hepatobiliary tuberculosis are reviewed. The plain film findings of large "chalky" and confluent hepatic calcifications or nodal-type calcifications along the course of the common bile duct are suggestive of hepatobiliary tuberculosis. Small, discrete, scattered calcifications may be mimicked by histoplasmosis but can be differentiated from hepatobiliary tuberculosis. Obstructing defects seen on cholangiography are indicative of tuberculosis when adjacent calcifications are present. The patterns of liver calcifications could provide a clue to the diagnosis of hepatobiliary tuberculosis and its differentiation from liver calcifications of various other etiologies.     

Langerhans' cell histiocytosis of the liver in adults

Among adults, liver involvement is relatively frequent in Langerhans' cell histiocytosis (LCH), even though it is often overlooked. In fact, the liver involvement may be missed in apparently localized LCH or when it is the sole site of involvement. We present 23 cases of liver involvement in LCH out of a cohort study of 85 adult patients included in the French Histiocytosis Study Group Registry. The most frequent clinical setting was multiorgan involvement (87% of our cases). The main histological pattern in liver LCH was sclerosing cholangitis (56% of the cases). The symptoms included hepatomegaly (48%) and/or liver biochemistry abnormalities (61%, including cholestasis associated with increased transaminases levels in 35% of cases, cholestasis only in 22% and increased transaminases levels only in 4% of the cases). Particularly suggestive of the diagnosis was the observation of biliary tree abnormalities through magnetic resonance imaging (MRI). The natural history of liver LCH fits into two stages: early infiltration by histiocytes and late sclerosis of the biliary tree. We found that liver involvement had a significant impact on survival. Thus we suggest that clinical and biological liver evaluation must be performed regularly onwards to screen every LCH adult patient from the time of the initial diagnosis. MRI and liver biopsy should be considered as soon as the data point to a possible liver localization. If this diagnosis is confirmed, we suggest a treatment with ursodesoxycholic acid, as in other cholestatic diseases, together with treatments specifically directed towards LCH. However, the ideal treatment of liver LCH remains to be found, and in advanced cases transplantation is the sole option.     

Clinical profile of primary sclerosing cholangitis in Singapore

BACKGROUND AND AIM: Primary sclerosing cholangitis (PSC) is a rare chronic disease in Singapore and its epidemiological profile has not been described previously. The present study aimed to define the demographic and clinical profile of patients with PSC in Singapore. METHODS: The case records of patients with PSC seen at Changi General Hospital were analyzed in terms of demographic profile, clinical presentation, clinical course, treatment and complications. RESULTS: Ten cases of PSC were diagnosed over a 10-year period. The male:female ratio was 9:1. The median age of diagnosis was 49.5 years (mean: 50.9 years; range: 35-63 years). With regards to clinical presentation, seven patients had hepatobiliary sepsis, two patients had asymptomatic liver biochemistry abnormalities while one patient had cholestatic jaundice. Prevalence rate of perinuclear antineutrophil cytoplasmic antibody (pANCA) was 20%. Symptomatic inflammatory bowel disease (IBD) was diagnosed in 20% of PSC cases. Eight patients (80%) had intrahepatic ductal involvement while two patients (20%) had combined intrahepatic and extrahepatic ductal involvement on endoscopic retrograde cholangiopancreatography (ERCP). The prevalence rate of recurrent cholangitis was 30% while that of recurrent liver abscess, cirrhosis and common bile duct stricture were all 10%. The mean duration of follow up was 6.6 years with one death from liver failure. CONCLUSION: The clinical profile of patients with PSC in Singapore appears to differ with other published data, with a greater number presenting with hepatobiliary sepsis and less frequent association with IBD and pANCA. It is hypothesized that this may be related to differences in environmental triggers and genetic susceptibility.     

肝结核5例诊治分析

[目的]分析肝结核的临床表现、影像学特点及治疗措施,提出诊治对策,提高诊断能力.[方法]回顾性 分析5例肝结核患者的病历资料,以期提高对肝结核的诊断及认识.[结果]5例中,以脐周痛、发热为主要表现1例,以右上腹痛表现1例,以右下胸痛为主要表现1例,以肝功能损害为主要表现1例,以腹胀、多浆膜腔积液为主要表现1例；其中3例经病理及影像学检查确诊,另2例为临床诊断考虑肝结核,给予抗结核治疗有效.[结论]肝结核可表现为腹痛、腹胀,也可无明显腹部症状,或仅有肝功能损害及表现为多浆膜病变；腹部CT表现为肝脏结节,增强后可强化,或有钙化表现；病理活检是金标准,表现为肉芽肿性炎.对于有肺结核病史,近期出现发热、纳差、腹痛、肝脏肿大伴触痛、血沉增高、肝功能异常尤其是γ-GT增高、贫血、甲胎蛋白正常,结合腹部CT及B超表现需考虑肝结核的可能.     

56例结核性腹膜炎患者CT影像分析

目的 探讨结核性腹膜炎（TBP）特征性CT影像表现及治疗中影像动态变化的特点。方法 回顾性分析56例临床及病理确诊的TBP患者,观察分析CT影像特征,包括腹腔积液、腹膜（壁腹膜、肝包膜、大网膜及肠系膜）及腹腔淋巴结。56例均行CT平扫+增强扫描。治疗过程中收集动态复查CT影像。结果 腹腔积液45例,大量1例,中少量44例,限制性38例,CT值>20 HU者34例。壁腹膜增厚46例,肝脏边缘部腹膜均有受累,均匀增厚17例,扁丘状凸起或腹膜结核瘤29例,增强扫描结核瘤边缘强化,肝包膜或肝脏受累15例;网膜增厚35例,结节及斑片34例,网膜饼1例,增强扫描网膜饼呈轻度不均匀强化;肠系膜增厚41例,表现为斑片、结节及线状、星芒状条索状高密度影,与肠袢粘连,增强扫描肠系膜血管包埋其内,走形扭曲。腹腔及腹膜后淋巴结增大19例,伴钙化5例,簇集状排列,增强扫描环状或蜂窝状强化。17例完整的动态CT图像,腹腔积液吸收最快,网膜及肠系膜病变吸收稍慢,腹膜结核瘤缩小速度最慢。结论 TBP的CT诊断为多种征象的综合,中少量腹腔积液伴壁腹膜、网膜及肠系膜的增厚粘连是最为常见的影像表现;CT影像动态变化对临床评价疗效具有重要意义。     

成人血行播散性肺结核202例临床及影像分析

目的通过对血行播散性肺结核临床资料的分析,提高对该病的认识。方法回顾性分析1998—2008年收治的成人血行播散性肺结核202例的临床资料、影像学特点、误诊情况及治疗反应。结果（1）中青年患者占80.2%, 老年患者占19.8%。（2）痰涂片查抗酸杆菌（AFB)阳性率为20.7%;31例AFB阴性患者中35.5%纤维支气管镜检抗酸杆菌阳性。（3）12.9%X线胸片早期表现为肺间质磨玻璃样改变,51.5%表现为大小、密度、分布均匀的粟粒结节。（4）胸部高分辨CT显示55.0%急性血行播散型表现双肺弥漫分布的大小、密度均匀的粟粒结节;另48例亚急性和慢性血行播散性肺结核表现以上中肺野为主的3～7mm大小、密度及分布不均匀的结节。46.8%的肺野内可见斑片、结节、纤维条索状影;43.1%伴纵隔和/或肺门淋巴结肿大。（5）20例活组织检查60%病理阳性。 （6）44.6%合并肺外结核,常见于脑膜、浆膜腔、淋巴结、脑、肝、脾、骨等。（7）42.1%入院前被误诊为其他疾病。（8）除8例外其余患者抗结核治疗后体温在3d到12周内降至正常。（9）79.7%在抗结核治疗2个月后胸片显示病灶不同程度吸收。结论痰涂片、HRCT、纤维支气管镜及器官组织活检是早期诊断的关键。     

The role of hepatobiliary scintigraphy in cystic fibrosis

This was a prospective open study that examined the quantitative and qualitative analysis of hepatobiliary scintigraphy (DISIDA) in detecting liver involvement in cystic fibrosis (CF). Forty-four adult and pediatric patients (median age, 12.1 years; range, 1.1-36.3 years) were divided into three groups: group 1, no evidence of liver involvement (n = 8); group 2, biochemical evidence of liver involvement on two or more occasions (n = 26); and group 3, clinical evidence of liver disease (n = 10). In groups 1 and 2, the most common qualitative scintigraphic finding was focal intrahepatic retention of tracer (26/34 patients, 12 of whom had normal findings on ultrasonography). This finding corresponds to focal cholestasis and may warrant treatment with the choleretic agent ursodeoxycholic acid (UDCA). In the group 3 patients, the abnormal qualitative scintigraphic appearances (heterogeneous uptake of tracer and nodular liver outline) added little to the findings on ultrasonography; however, these patients had a prolonged mean hepatic clearance time compared with those in groups 1 and 2 (one-way ANOVA; P < .015). It is proposed that scintigraphy with DISIDA has a role in the detection of early liver involvement in cystic fibrosis. (Hepatology 1996 Feb;23(2):281-7)     

Hepatitis tuberculosis presenting as tumoral disease on ultrasonography

Macronodular involvement of the liver is a rare manifestation of hepatitis tuberculosis. Two cases of this pseudotumoral form are reported on ultrasonography, demonstrating multiple hypoechoic nodules distributed throughout the liver. The authors state the difficulty in differentiating this form in its atypical presentation from lymphomatous or secondary malignancies. They stress the importance of the bacteriological and/or histological diagnosis which can be performed with fine-needle percutaneous biopsy under ultrasound guidance and which allows effective therapy.     

24例细支气管肺泡细胞癌外科治疗效果

目的总结细支气管肺泡细胞癌（BAC）的临床特点及外科治疗效果。方法回顾性分析我院2000年1月至2006年12月经手术治疗的24例BAC的临床资料，并随访其生存情况。结果本组男性11例（46％），女性13例（54％），平均年龄59．1（47～72）岁；其中孤立结节型13例，肺炎实变型7例和多发结节型4例。全组均手术切除。术后TNM分期：Ⅰ期8例（ⅠA期4例、ⅠB期4例），Ⅱ期4例（ⅡA期1例、ⅡB期3例），Ⅲ期11例（ⅢA期7例、ⅢB期4例），Ⅳ期1例。随访22例，平均随访时间42月（12月～90月）；10例死亡，死亡者术后中位生存期14月。结论BAC是一种特殊类型腺癌，呈女性多发趋势。早期BAC的手术治疗可以取得满意的长期生存，没有淋巴结转移的多发结节型BAC仍可采取手术治疗，肺炎实变型BAC手术治疗效果差。     

Abdominal tuberculosis in children. Report of 10 cases

BACKGROUND: Abdominal tuberculosis is a rare location of this infection with a lot of diagnostic difficulties. The aim of this study was to review our experience of pediatric abdominal tuberculosis with that of the literature data. PATIENTS AND METHODS: A retrospective study was conducted over a 7-year period; 10 cases of abdominal tuberculosis in children were reviewed (6 girls and 4 boys, mean age: 11 years, extremes 4-14). Eight patients enrolled in this study satisfied the following criteria: a positive culture for mycobacterium tuberculosis on samples of ascites fluid, sputum, urine, abscess puncture and/or caseating granulomas on histologic examination of biopsies obtained by endoscopy, percutaneous aspiration or needle biopsy or exploratory laparotomy. Two other patients (patients No 1 & 5) had a favorable response within 10 weeks of antituberculous therapy. RESULTS: We observed 8 cases of peritoneal tuberculosis and 2 cases of intestinal tuberculosis. Extra-abdominal tuberculosis was found in 4 of those patients. Two patients had a contact with a tuberculosis positive individual. Nine children had BCG immunization. The main clinical features were abdominal distension (6 cases) and abdominal pain (6 cases). Abdominal ultrasonography visualized mesenteric lymphadenopathies (6 cases), an abdominal mass (5 cases), free (1 case) and localized ascites (1 case). Barium enema and small bowell series showed small bowel stenosis (1 case) and ileal ulcerations (1 case). Exploratory laparotomy, performed in 3 patients, showed whitish granulations and peritoneal abscesses with caseous necrosis on histology. Quadruple therapy with tuberculostatic agents was prescribed in all patients, associating isoniazid, rifampicin, pyrazinamide combined at the start of the treatment with streptomycin (7 cases) and ethambutol (3 cases). Short term outcome was favorable with no deaths. The long term outcome was complicated by persistent ascites (1 case) and the development of portal hypertension (1 case). CONCLUSION: Abdominal tuberculosis remains a rare localization with a lot of diagnostic difficulties.     

Liver involvement in hereditary hemorrhagic telangiectasia (HHT)

Liver involvement in hereditary hemorrhagic telangiectasia (HHT) consists of extensive intrahepatic vascular malformations associated with blood shunting (arteriovenous, arterioportal and/or portovenous). It is a rare disorder that nevertheless can result in significant systemic and hepatobiliary abnormalities. Although hepatic vascular malformations are present in a majority of patients with HHT, symptoms occur in a only a minority with a clear predominance for the female gender. Symptoms from liver vascular malformations are often misdiagnosed and this can lead to potentially harmful interventions. In this review article, clinical findings of liver involvement in HHT and their pathophysiology are discussed as well as diagnostic methodologies, therapies used and their outcome. Data presented is based on a review of the literature performed in October 2006 using the following MEDLINE search terms: (hereditary hemorrhagic telangiectasia [ALL] OR Rendu-Osler-Weber [ALL]) AND (liver OR hepatic [ALL]). Papers were considered if they were published in English and if they included specific cases that were sufficiently described.     

The incidence and clinical characteristics of Mycobacterium tuberculosis infection among systemic lupus erythematosus and rheumatoid arthritis patients in Korea

OBJECTIVES: The aim of this study was to describe the incidence and clinical characteristics of Mycobacterium tuberculosis infection in SLE and RA patients in Korea where the prevalence rate of active pulmonary tuberculosis in a general population is relatively higher than in Western countries. PATIENTS: We reviewed the medical records of 283 SLE and 284 RA patients retrospectively and then assessed the incidence, risk factors, and clinical characteristics of active tuberculous infection. We then compared the results for the two different groups. RESULTS: Tuberculosis was documented in 15 SLE and 7 RA patients with an incidence rate of 7.9/1,000 patient-years and 2.3/1,000 patient-years, respectively (p = 0.003). SLE-associated tuberculosis cases included 3 of miliary tuberculosis, 7 of pulmonary tuberculosis (including 1 case of diffuse pulmonary involvement with meningitis) predominantly involving two or more lobes at the mid-/lower lungfield, and 5 extra-pulmonary forms (joint, bone, kidney, larynx, pleura). All of the RA-associated tuberculosis cases were pulmonary forms with the majority being localized to single lobe, and only one case had a past history of tuberculosis, whereas a past history of tuberculosis and a longer duration of the underlying disease were significantly correlated with the development of tuberculosis in the SLE patients. Major organ involvement, the mean daily dosage of prednisolone, and a history of over 30 mg of daily prednisolone were not related to the development of tuberculosis. However, when we took only those patients taking corticosteroid until the diagnosis of tuberculosis for analysis, SLE patients with tuberculosis showed a higher daily dosage of prednisolone than those without tuberculosis. CONCLUSION: Taken together, the characteristics of tuberculosis in SLE patients were: (1) a higher incidence rate, (2) more frequent extra-pulmonary involvement, (3) more extensive pulmonary involvement, and (4) a higher relapse rate than in rheumatoid arthritis. Thus, the contributory role of M. tuberculosis infection in the morbidity and mortality of patients with SLE must be emphasized, especially in areas in which this bacteria is endemic.     

Interferon gamma/IL10 ratio defines the disease severity in pulmonary and extra pulmonary tuberculosis

Several cytokines (IFN gamma, TNF alpha, IL10 and IL6) show an association with either disease localization or dissemination in tuberculosis. There are also reports of involvement of extra-pulmonary sites in tuberculosis with differential clinical severity. However, no comparative study of biomarkers across the disease severity spectrum is available. This was the purpose of the current study. Cytokines (IFN gamma, TNFalpha, IL10 and IL6) secreted in response to a panel of stimulants (PHA, LPS or mycobacterial antigens) in whole blood were determined in eighty-two tuberculosis patients. WHO criteria was applied for stratification of patients according to disease severity: disseminated and or severe disease (EPTB1; N=29); disease localized to lung parenchyma (PTB; N=32) and disease localized to peripheral sites without lung involvement (EPTB2; N=21). Mycobacterial antigens induced IFN gamma/IL10 ratio showed a direct relationship with disease severity ranking (median ratios: EPTB1=0.21; PTB=0.85; EPTB2=7.7) and the highest correlation (Spearman Rank; rho=0.673, p<0.000001). IFN gamma/IL10 ratio also rank ordered clinical severity as it relates to anatomic sites. IFN gamma/IL10 ratio may therefore provide a useful objective marker of disease severity in both pulmonary and extra-pulmonary tuberculosis.     

Focal liver lesions detection and characterization: The advantages of gadoxetic acid-enhanced liver MRI

Since its clinical introduction, several studies in literature have investigated gadolinium ethoxybenzhyl diethylenetriaminepentaacetic acid or gadoxetic acid(Gd-EOB-DTPA) properties. Following contrast injection, it provides dynamic vascular phases(arterial, portal and equilibrium phases) and hepatobiliary phase, the latter due to its uptake by functional hepatocytes. The main advantages of Gd-EOB-DTPA of focal liver lesion detection and characterization are discussed in this paper. Namely, we focus on the possibility of distinguishing focal nodular hyperplasia(FNH) from hepatic adenoma(HA), the identification of early hepatocellular carcinoma(HCC) and the pre-operative assessment of metastasis in liver parenchyma. Regarding the differentiation between FNH and HA, adenoma typically appears hypointense in hepatobiliary phase, whereas FNH is isointense or hyperintense to the surrounding hepatic parenchyma. As for the identification of early HCCs, many papers recently published in literature have emphasized the contribution of hepatobiliary phase in the characterization of nodules without a typical hallmark of HCC. Atypical nodules(no hypervascularizaton observed on arterial phase and/or no hypovascular appearance on portal phase) with low signal intensity in the hepatobiliary phase, have a high probability of malignancy. Finally, regarding the evaluation of focal hepatic metastases, magnetic resonance pre-operative assessment using gadoxetic acid allows for more accurate diagnosis.     

骨化性气管支气管病的临床特征(附16例分析)

目的探讨骨化性气管支气管病(tracheobronchopathia osteochondroplastica,TO)的临床特征。方法收集2017年1月至2019年8月武汉市肺科医院医院经支气管镜和病理诊断的16例TO患者的临床、影像、支气管镜及病理资料。结果16例TO患者中,男性12例,女性4例。年龄27~73岁,平均年龄(47.8±15.3)岁。15例合并其他疾病,包括8例肺结核,3例支气管结核,2例肺炎,1例肺腺癌和1例支气管扩张。最常见的症状为咳嗽(10/16),其他初始症状包括咯血(3/16)、发热(3/16)、胸闷(2/16)和胸痛(2/16)。胸部CT扫描显示6例(6/16)患者有气管壁的钙化小结节,10例未见明显异常。支气管镜显示典型的局灶或弥漫的软骨或骨性小结节,部分结节融合。TO分期:I期7例,Ⅱ期7例,Ⅲ期2例。病理组织学特征为气道黏膜下骨或软骨组织结节状增生。结论TO是一种少见的良性病变,症状不特异,CT诊断阳性率低,支气管镜联合组织病理学检查可以确诊,医生对该病的认识是早期诊断和治疗的关键。     

"Solitary" necrotic nodule of the liver: an enigmatic entity mimicking malignancy

Aim The aim of the study is to further investigate the clinicopathological features of solitary necrotic nodules. Material and methods Twenty-three archived cases of solitary necrotic nodule of the liver, which were preoperatively misdiagnosed as liver metastases were studied. The pathological findings were correlated with the clinical data of the patients. Results The nodules were solitary in 20 cases and multiple (2) in three cases, and measured from 0.5–1.5 cm in diameter. Twenty-one cases were located in the right lobe of the liver (91.6%) and two in the left lobe (8.69%). Twenty nodules were found in the subcapsular region (86.95%), while three nodules were located within the hepatic parenchyma (13.04%). More then half of our cases (12) were accompanied by extended calcification. Granulomatous tissue resembling “burnt-out” parasitic granulomas was found in three cases. Conclusions Solitary necrotic nodules of the liver often mimic malignancy in abdominal imaging. Thus, permanent histopathology of the operative specimen remains the only accurate method of diagnosis. Their pathogenesis is most likely variable, and most reported cases are linked either to a parasitic or a vascular origin. Despite the designation of these lesions as “solitary” they may occasionally be multiple. In addition to the standard histological criteria of solitary necrotic nodules, the relatively small size (15 mm or less) and the frequent presence of calcifications seem to further characterize this enigmatic entity. Solitary necrotic nodule should be included in the differential diagnosis of small liver lesions with extensive necrosis.