Hepatic and splenic mycosis in children with acute leukemia.

Three cases of hepatic and/or splenic mycosis in children with acute leukemia are reported. Patients presented with fever not responding to broad spectrum antibiotics during or after prolonged and profound neutropenia. Noteworthy, in 1 case no abnormality in liver function tests was detectable at diagnosis. CT scan showed focal hepatic lesions in all patients. In 1 patient ultrasounds failed to detect hepatic lesions and showed only splenic lesions, while CT scan detected hepatic and not splenic lesions. Definitive diagnosis was performed by open liver biopsy and histological demonstration of fungal invasion. Cultures were negative in all cases. All patients survived with prolonged amphotericin B treatment (from 36 to 40 mg/kg), combined with flucytosine in two cases. In conclusion, this clinical entity should be taken into account by physicians taking care of neutropenic patients, even in the absence of abnormalities in liver function. CT seems to be the method of choice for diagnosis, although ultrasounds might be useful for detecting concomitant splenic lesions.     

Peripheral T-cell lymphoma presenting as predominant liver disease: a report of three cases

Three cases of a peculiar form of peripheral T-cell lymphoma presenting as predominant hepatic disease with splenomegaly are reported. The three patients had marked liver enlargement without lymphadenopathy; white blood cell count was normal, and modifications of hepatic tests were mild. In the three cases, the diagnosis of the lymphoma was mainly based on the results of hepatic morphological changes. Liver involvement was histologically characterized by a predominantly sinusoidal infiltration by tumor cells in the three cases, associated with perisinusoidal fibrosis in two of them; portal infiltration was noted in two patients. Immunopathological study showed that tumor cells were T-lymphoid cells that were different from normal T-lymphocytes by the lack of expression of one T-cell membrane antigen, i.e., Leu-1. These findings suggest that a distinct clinical, pathological and immunopathological entity might be individualized within the large group of T-cell lymphomas.     

先天性肝纤维化——附12例临床分析

目的分析先天性肝纤维化的临床特征。方法对12例先天性肝纤维化患者临床表现、实验室检查及病理组织学特点进行回顾性分析。结果12例患者均有明显临床症状,10例有典型门脉高压症状,11例就诊时肝功能异常,肝脏病理主要表现为：肝组织内呈现宽大致密且炎症不明显的胶原纤维间隔,或纤维束弥漫穿插于固有的肝小叶内,无典型的假小叶结构。结论对于不明原因肝功能异常的门脉高压症患者,应尽可能进行肝组织活检病理检查以协助诊断及指导治疗。     

A Case of Inflammatory Pseudotumor of the Gallbladder and Bile Duct

A rare cause of obstructive jaundice is presented. A 43-yr-old man developed jaundice with fever and weight loss, and showed lymphadenopathy, reticular shadows on chest roentgenogram, deranged liver function tests, eosinophilia, low values of complement proteins, and hypergammaglobulinemia. There was a stricture throughout the bilateral hepatic ducts and common hepatic and bile ducts with dilated intrahepatic bile ducts on imagings. The walls of the gallbladder and common bile duct were thickened due to diffuse granulomatous lesions of unknown etiology, composed mainly of plasma cells, lymphocytes, and fibroblasts identical with inflammatory pseudotumor. The same histological findings also were observed in the lung and lymph nodes. Jaundice disappeared, with reduced thickening of the common bile duct wall. All clinical and laboratory abnormalities subsided after high-dose prednisolone therapy, starting when dyspnea developed. There is no similar case in the literature.     

83例肝病患者临床与肝组织病理学分析

目的通过对83例肝病患者的临床与肝组织病理学检查的对比研究,以提高临床诊断的准确性。方法用全自动生化分析仪进行血清生化指标检测,ELISA法检测HBV血清标志物,同时进行肝组织病理检查,检测肝组织HBsAg和HBcAg的表达。结果51例血清HBsAg阳性者肝细胞中均有HBsAg和/或HBcAg表达,32例血清HB-sAg阴性者有9例(28.1%)肝组织中有HBsAg和/或HBcAg表达。75例慢性肝病患者中ALT在各炎症分级组间差异无统计学意义(P>0.05),AST和TBIL在不同的肝脏炎症分级组间差异有统计学意义(P<0.05),且炎症分级越高,AST和TBIL升高越明显;ALT、AST、TBIL值在肝脏纤维化S2期最高。以病理诊断为标准,临床慢性肝炎轻度和中度的诊断准确率分别为61.9%(13/21)和62.5%(20/32),肝硬化的临床诊断准确率为40%。结论以肝组织病理检查为金标准,肝病临床诊断的准确率仍较低。为提高慢性肝病的临床确诊率,应尽可能行肝组织病理学检查。     

对肝肺综合征诊治的76例临床分析

目的:探讨肝肺综合征(HPS)的临床特点及诊断方法,提高对其认识。方法:回顾性分析76例HPS患者的临床资料,并与无HPS的133例肝硬化患者在肺功能、血气分析等方面进行比较。结果:在HPS组中除4例为慢性酒精性肝损害基础上的急性药物性肝炎外,72例均为肝硬化患者。临床表现为呼吸困难、紫绀、杵状指及蜘蛛痣。据Child-Pugh分级标准,肝功能B级和C级者59例(77.6%),平均PaO2为(79.93±11.29)mm Hg(1 mm Hg=0.133 kPa),对照组为(92.48±13.53)mm Hg,2组比较有统计学意义(P0.01)。HPS组中76例中有48例直立性缺氧10%(63.1%)。不同门静脉内径、有无蜘蛛痣、不同肝功能分级的PaO2和SaO2值比较,均有统计学意义(P0.01)。影像学检查结果示肺内血管扩张。结论:肝肺综合征的发生与肝功能不全有明显的关系,该病患者常有呼吸困难、紫绀、杵状指、蜘蛛痣及直立性缺氧等典型表现,患者低氧血症发生率高,而肺功能存在弥散功能障碍,肺血管有明显的扩张。     

Hepatic abscess: sensitivity of imaging tests and clinical findings

The imaging studies of 63 patients with hepatic abscess were evaluated to determine the sensitivity of specific imaging tests and define causes of false-negative test results. Computed tomography (CT) detected 57 of 59 (97%) separate episodes of hepatic abscess. The two false-negative CT examinations were in patients with a diffuse low-density pattern throughout the liver. The radionuclide (RN) examination detected 16 of 20 (80%) cases, missing abscesses less than 2 cm in diameter. Ultrasound detected 33 of 42 (79%) cases, missing abscesses in the dome of the liver, small abscesses, and 2 large early abscesses. For all three imaging modalities, a specific diagnosis of abscess was possible only in those patients in whom CT scans demonstrated abscess gas (15%). The results of the imaging studies were correlated with the patients' clinical condition and laboratory findings. Thirty-one percent of patients were afebrile while 23% had normal white blood cell counts. Thirteen percent had totally normal liver function tests. We conclude that the clinical absence of fever, leukocytosis, or elevated liver function tests does not permit exclusion of the diagnosis of hepatic abscess. A CT scan is highly specific in excluding the diagnosis of hepatic abscess in the absence of diffuse liver disease. In this retrospective study CT was the most sensitive imaging modality available for the detection of hepatic abscess.     

4例肝淀粉样变性的临床特点和分析

目的对已确诊病例的临床特点进行总结和分析,以提高对本病的认识,减少误诊率。方法经肝、肾组织学检查及特异性组织学染色明确诊断为肝淀粉样变性的4例住院患者。住院期间检测肝功、血脂、尿常规等化验,腹部彩超、腹部CT或腹部核磁,肝脏、肾脏的活体组织病理检查及特异性组织学染色（刚果红染色）。结果患者均有肝大,肝功生化检查AKP及r-GT明显增高,低蛋白血症,高脂血症。肝脏彩超和CT显示肝脏增大,肝密度低,回声细密,类似脂肪肝的表现。肝穿及肾穿病理见大量粉红色淀粉样物质沉积,刚果红染色（＋）,病理结论为肝、肾淀粉样变性。结论对临床上高度怀疑为肝淀粉样变性的患者,作肝肾活体组织检查及特异性组织学染色（刚果红染色）,可证实为肝淀粉样变性。     

甲状腺功能亢进症初诊患者肝功能异常的临床分析

探讨甲状腺功能亢进症(甲亢)初诊患者肝功能异常的发生率、临床特点及相关因素。将428例甲亢患者根据肝功能是否异常分为两组,分析其临床表现、肝功能指标与血清甲状腺激素水平以及甲状腺自身抗体的水平的关系。初诊甲亢患者肝功能异常的发生率为30．1％,肝功能损害最常见的是碱性磷酸酶(ALP)、丙氨酸氨基转移酶(ALT)增高,其甲状腺激素水平显著高于无肝功能异常者。而TSH、甲状腺微粒体抗体(TMA)、甲状腺球蛋白抗体(TGA)则无明显差异。甲亢初诊患者合并肝功能异常较常见,肝功能损害以ALP、ALT增高为最常见,且与甲状腺激素水平关系密切。     

The clinical features of hepatic angiosarcoma: a report of four cases and a review of the English literature.

Four cases of hepatic angiosarcoma are reported with a review of 99 other cases in the English literature. Angiosarcoma of the liver is associated with chronic exposure to thorotrast, vinyl chloride, arsenicals, radium and possibly copper and with chronic idiopathic hemochromatosis. Although 40% of patients have hepatic fibrosis or cirrhosis at autopsy, the nature of the association between chronic liver disease and hepatic angiosarcoma is unknown. The clinical presentation of hepatic angiosarcoma is nonspecific with abdominal pain, weakness and weight loss common complaints and with hepatomegaly, ascites and jaundice common findings. Liver function tests are usually abnormal but there is no one liver function test or set of tests specific for the tumor. The occurrence of thrombocytopenia and disseminated intravascular coagulation is characteristic of hepatic angiosarcoma and may be related to local consumption of clotting factors and formed blood elements in the tumor. Catastrophic intraabdominal bleeding is also characteristic and occurs in one-fourth of all cases. This complication is likely related to the high incidence of clotting abnormalities and the vascular nature of the neoplasm. Selective hepatic arteriogram and open liver biopsy are the foundations of diagnostic evaluation. Percutaneous liver biopsy should be avoided. Failure to appreciate the possibility of hepatic angiosarcoma in the proper clinical setting, leading to blind percutaneous biopsy, may result in failure to make the diagnosis at the cost of significant morbidity and mortality. Survival of patients with hepatic angiosarcoma is brief; only 3% live longer than 2 years. Treatment of the tumor to date is empirical. There are probably a few patients who might benefit from radical surgery with curative intent. For all others chemotherapy is indicated. Adriamycin is active against hepatic angiosarcoma, but optimal dose and mode of administration require further investigation. Further study is also required to delineate the cause of hepatic angiosarcoma in the 60% of cases without definite epidemiologic association.     

Portal hypertension syndrome

The clinical and laboratory manifestations of the following groups of patients were compared: 45 cases of schistosomal fibrosis, 30 cases of hepatic cirrhosis with schistosomiasis, and 40 cases of hepatic cirrhosis alone. The patients with schistosomal fibrosis had the following characteristics which distinguished them from those in the other two groups: (1) lower average age; (2) marked splenomegaly; (3) less frequent signs of liver failure and milder alterations in biochemical tests of liver function; (4) large gradients between intrasplenic pressure and wedged hepatic vein pressure; and (5) lower mortality of operations for portal hypertension.     

肝窦阻塞综合征八例临床分析

目的探讨肝窦阻塞综合征（SOS）的临床诊治方法。方法回顾性分析8例SOS的临床资料,并进行随访。结果8例SOS主要临床表现为腹胀（8例）、肝区疼痛（7例）、腹水征（8例）及肝肿大（7例）等。8例的肝功能损害程度大多较轻,血清-腹水白蛋白梯度均大于11．1g／L,血清与腹水CA125均显著升高。超声检查均见肝脏肿大、胆囊壁水肿或增厚、门静脉增宽且流速缓慢、肝静脉变细以及腹腔积液等;MRI表现为门脉期及延迟期肝实质不均匀片状强化,肝叶、段静脉腔内造影剂充盈不良。经皮肝穿刺活检均见肝窦扩张、淤血及肝细胞变性、坏死,3例发现小静脉管腔狭窄、管壁增厚伴纤维组织增生。8例中1例行肝移植术后痊愈,4例经内科治疗后逐渐康复,3例死亡。结论SOS的临床表现以突出的门脉高压症为特点,CA125常显著升高,超声及MRI对本病的诊断与鉴别诊断有重大价值,而经皮肝穿刺活检的价值有限,联合应用影像学方法与病理活检可提高诊断正确率。早期应用抗凝药物是治疗本病的关键,严重病例可行肝移植术。     

脂肪肝临床病理分析

目的 总结10年来肝穿刺标本中,脂肪肝的发生率及其与基础肝病的关系,探讨其临床及病理表现特征,了解脂肪肝炎的病因及危险因素。方法在１９８８－１９９７年,６５８例肝穿刺病例中,共有脂肪肝７１便,其中非酒精性脂肪肝68例,按性别、年龄匹配、选取非脂肪肝155例作对照,观察肝功能、血脂、血糖及肝炎病毒标记,并评其肝穿刺病理学特征。结果 行肝穿刺检查的病例中,脂肪肝的发生率为10．8％,脂肪肝组丙氨酸氨基转移酶（ALT）、门冬氨酸氨基转移酶（AST）、总胆红素（TB）、结合胆红素（CB）均明显低于非脂肪肝组（P＜0．05）,血三酰甘油高于非脂肪肝组（P＜0．05）。脂肪肝的脂肪变性主要分布在小叶周围,且以大泡型为主,35例（51．5％）有肝细胞坏死,46例（67．6％）有炎性细胞浸润,19例（27．9％）有不同程度的纤维化。结论 脂肪肝与高脂血症之间关系密切,对肝功能异常的无症状患者,肝组织活检是目前唯一的确诊方法。     

The structural and functional status of the liver in tropical sprue

Summary Liver biopsy and function tests were performed on 8 patients with tropical sprue. Although mild hepatic siderosis and lipofuscin deposition were revealed in addition to some minimal abnormalities in liver function tests, no evidence of significant hepatic injury was found.     

Galactose elimination capacity as a prognostic index in patients with fulminant liver failure.

In 25 patients with fulminant hepatic failure the prognostic value of a quantitative liver function test, the galactose elimination capacity, was assessed and comapred with routine liver function tests and clinical features. The galactose elimination capacity was significantly higher (P less than 0-05) in the five patients who survived than in the 20 patients who died. None of the other liver function tests, was significantly different. The values of the galactose elimination capacity overlapped considerably between survivors and non-survivors, but all patients with a galactose elimination capacity below 12-8 mumol galactose/min and kg body weight died. The disease among most patients who died having a galactose elimination capacity greater than 13 mumol ran a subacute course. It is suggested that quantitative liver function tests be included when new treatments of fulminant hepatic failure are investigated.     

Serum hyaluronate as a marker of hepatic derangement in acute liver damage

Twenty patients with paracetamol(acetaminophen)-induced acute liver damage of varying severity were studied longitudinally with assessment of clinical state, standard liver function tests and radiometric hyaluronate (HYA) assay (Pharmacia). In patients (n = 6) who developed coma, HYA rose rapidly with clinical deterioration to reach a median value of 27,510 micrograms/l, 7 days post-ingestion, which was significantly higher (p less than 0.005) than in patients (n = 7) who exhibited only marked derangement of liver function tests without evidence of encephalopathy, HYA median value of 3240 micrograms/l. These peak values showed no correlation to the peak values of serum alanine aminotransferase (ALT). A third group of patients (n = 7) who were treated with N-acetyl cysteine, did not exhibit any evidence of liver failure and showed no significant rise in levels of HYA or ALT. The data demonstrate that HYA is a rapidly changing marker of liver derangement which appears to follow the clinical course of the patient. The increase to extremely high levels in patients with hepatic encephalopathy, suggests that there is a reversible defect in the hepatic endothelial cell HYA receptor, possibly due to endothelial cell damage or release of toxins from the necrotic liver.     

Chemotherapeutic evaluation of pulmonary tuberculosis patients with a complication of hepatic dysfunction

A retrospective study on the evaluation of antituberculous drugs for patients with hepatic dysfunction was undertaken to clarify treatment regimens. The values of GOT, GPT and T. Bil were used as indicators of liver function, and the "deterioration" in the liver function was defined as a level greater than 1.5 times the initial value. Of total 538 cases of active pulmonary tuberculosis, 103 cases (19.1%) had abnormalities in liver functions before chemotherapy, and 21 of 103 cases showed the deterioration in their liver functions during chemotherapy. There was little relationship between the initial status of liver functions and the incidence of their deteriorations after chemotherapy. Nine of 21 cases did not exceed the 3 times of normal ranges of liver functions. These nine cases could tolerate the same regimens of chemotherapy, and showed satisfactory clinical responses to chemotherapy with two exceptions: one died of tuberculosis and the other cerebrovascular disease. On the other hand, of 12 cases with elevated values of hepatic function greater than 3 times the normal limits, 6 cases discontinued chemotherapy. One case, however, died of hepatic failure related to chronic active hepatitis. Eight of 12 cases showed the rapid improvement of liver dysfunctions. These results suggest that antituberculous drugs are acceptable to patients with hepatic dysfunction as long as the elevations of GOT, GPT and T. Bil stay within 3 times of normal limits. Further chemotherapy could be continued under careful monitoring of liver functions even if the cases exhibit elevated levels of liver functions greater than 3 times the normal ranges.     

Acute hepatic failure due to Plasmodium falciparum liver injury

Nine patients with acute liver failure due to Plasmodium falciparum liver injury admitted to the Rajgarhia Liver Unit of the All-India Institute of Medical Sciences during 1982-84 are presented. The liver was palpable in all the patients, and eight had splenomegaly. Investigations revealed mild to moderate abnormality in liver function tests. All were negative for the markers of acute infection due to hepatitis A and B viruses. Blood film examination showed P. falciparum alone in seven and along with P. vivax in the remaining two patients. Liver histology, which was identical in all eight patients where liver biopsy was done, showed centrizonal necrosis and hyperplastic Kupffer cells loaded with malarial pigment. All the patients recovered with specific anti-malarial and supportive treatment. Our observations suggest that malaria due to P. falciparum may present as jaundice and encephalopathy which stimulates acute hepatic failure due to fulminant hepatitis.     

Serum T3 and Reverse T3 Levels in Hepatic Cirrhosis: Relation to Hepatocellular Damage and Normalization on Improvement in Liver Dysfunction

Liver is one of the major sites of T4 metabolism. Several studies have reported low serum T3 concentrations and elevated reverse T3 (rT3) levels in hepatic cirrhosis. This study examined the influence of degree of the hepatocellular damage and the effect of improvement in clinical state on thyroid hormone concentrations in 44 cirrhotic patients. Low serum T4 and T3 as well as raised rT3 were observed in cirrhotic patients with advanced liver dysfunction alone. T3 resin uptake was increased in some of these patients suggesting decrease in serum thyroid-binding globulin concentration. In patients with histological changes but with normal liver function tests, serum T4, T3, and rT3 were not altered. Serum T3 and rT3 correlated significantly with liver function tests. T4, T3, and rT3 normalized on improvement in clinical status and liver function tests. Lowest levels of T4 and T3 with extremely high rT3 were seen in patients with extremely advanced liver dysfunction. In these patients, the mortality was high. Therefore, in hepatic cirrhosis, 1) T4 metabolism is altered with lowering of T4 and T3 and a rise in rT3. 2) These changes may be dependent on the degree of hepatocellular damage and reverse on improvement in liver function. 3) T4, T3, and rT3 levels are useful prognostic indices.     

Liver involvement in sickle cell disease

In an effort to clarify the features of hepatic dysfunction in sickle cell disease, we obtained serial tests of liver function in 100 consecutive patients with sickle cell anemia and in 30 consecutive patients with hemoglobinopathy SC during a five-year period. There were 32 patients with chronic abnormalities in tests of liver function. These abnormal tests were explained by a variety of lesions in 30 cases, and the liver disease remained unexplained in only 2 patients who declined liver biopsy. The diagnoses in these 30 patients included hepatitis, chronic passive congestion, common duct obstruction, alcoholic liver disease, pregnancy, collagen-vascular disease, and sarcoidosis. Evidence for hepatitis B infection was present in 19 of those with sickle cell anemia and in 6 of those with hemoglobinopathy SC. The bilirubin levels in sickle cell anemia appeared to have a trimodal distribution, with six patients exhibiting markedly elevated levels of indirect bilirubin suggesting a difference in bilirubin metabolism. There was no evidence of liver disease in 72 patients with sickle cell anemia, nor in 24 patients with hemoglobinopathy SC, as these patients exhibited only mild elevation of their serum indirect bilirubin levels owing to chronic hemolysis. Intrasinusoidal sickling and Kupffer cell erythrophagocytosis were nearly universal findings at liver biopsy, irrespective of the clinical disorder, and were not related to the degree of liver test abnormalities. Liver and biliary tract dysfunction in sickle cell disease have been attributed to anoxia secondary to sinusoidal obstruction by sickled erythrocytes and Kupffer cell erythrophagocytosis. However, some causes of liver disease in sickle cell patients can be explained by clinical disorders other than the hemoglobinopathy alone.(ABSTRACT TRUNCATED AT 250 WORDS)