Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0023890 (cirrhosis)
 42,195 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

We report here on a case of non-Hodgkin's lymphoma in which liver involvement was the predominant clinical manifestation. A healthy 44-year-old man presented with upper abdominal pain, hepatosplenomegaly, thrombocytopenia, elevated AST, ALT and bilirubin, and marked elevation of lactate dehydrogenase and alkaline phosphatase. The abdominal CT scan showed only diffuse hepatosplenomegaly and uneven contrast enhancement of the spleen without any definite mass of the liver and spleen. US-guided aspiration biopsy of liver and the histologic examination confirmed a diagnosis of non-Hodgkin's lymphoma, the diffuse large B cell type. Bone marrow biopsy showed the infiltration of malignant lymphoma cells. PET-CT showed an increased FDG uptake of the liver, spleen and long bones. The patient was treated with combination regimen of cyclophosphamide, doxorubicin, vincristine and prednisone chemotherapy. Even in the absence of a mass lesion or lymphadenopathy, primary hepatic or hepatosplenic lymphoma should be considered in differential diagnosis of hepatitis or liver cirrhosis, especially for patients with diffuse hepatosplenomegaly and markedly elevated LDH.
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PMID:[A case of primary hepatic lymphoma mimicking hepatitis]. 1617 55

Hepatocellular carcinoma will continue to be one of the most common malignancies worldwide. Improved survival occurs following resection or liver transplantation. The appropriate pre-operative stratification and staging of these patients is essential. CT and MRI will undoubtedly continue to play a major role in the detection and diagnosis of HCC. These imaging techniques should be optimized for the evaluation of suspected HCC. The radiology report from the CT or MRI examination should include a comprehensive review of key diagnostic information for appropriate staging. This includes lesion size and number. Also to be noted are segmental and vascular involvement, regional and distant adenopathy as well as metastases, and finally, the presence of ascites, varices and cirrhosis.
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PMID:Hepatocellular carcinoma: MRI and CT examination. 1740 24

Hepatic fibrolamellar carcinoma (FLC) is an uncommon tumour that differs from hepatocellular carcinoma (HCC) in demographics, condition of the affected liver, tumour markers, and prognosis. FLC characteristically manifests as a large hepatic mass in adolescents or young adults with female predominance (mean age 23 years). Cirrhosis, elevated alpha-fetoprotein levels, and risk factors for HCC such as viral hepatitis are typically absent. FLC is usually associated with serum tumour markers such as vitamin B12 binding protein, and neurotensin. FLC is characterized pathologically by cords of tumour cells surrounded by abundant collagenous fibrous tissue arranged in a parallel or lamellar distribution. FLC usually appears on radiologic images as a lobulated heterogeneous mass with a central scar in an otherwise normal liver. The clinical presentation of patients with FLC is variable. These patients commonly have pain, and palpable right upper quadrant abdominal mass. An uncommon presenting sign is gynaecomastia in men. Use of percutaneous biopsy (FNAB) is beneficial if there is diagnostic uncertainty about the radiologic diagnosis (US, CT MRI). Although FLC is frequently recurrent, patients have a better prognosis than those with HCC, and aggressive surgical liver resection with extended lymphadenectomy or liver transplantation may be indicated. The presence of advanced-stage disease, direct invasion of adjacent organs, lymphadenopathy, or limited metastasis does not preclude attempts at curative resection. In inoperable cases, the patient may benefit from chemotherapy, permitting in up to 50% of these cases a curative resection. The case is reported of a 18-year-old man with bilateral gynecomastia secondary to an unknown hepatic fibrolamellar carcinoma producing oestrogens. Serum alpha-fetoprotein was negative; des-gamma-carboxy prothrombin (DCP) level was elevated. CT scan and MRI showed a solid hepatic tumour (theta 10 cm) without evidence of extrahepatic spreading. By a needle biopsy a fibrolamellar carcinoma was diagnosed. On March 1995 a right hemihepatectomy was performed. The postoperative course was uneventful and the patient recovered. Specimen's histologic examination confirmed the preoperative diagnosis. Intracellular (hepatocytes) oestrogens were found, but oestrogen and androgen receptors were negative. After surgery DCP and oestradiol levels rapidly decreased and gynaecomastia disappeared. A follow-up program was established. On April 2000 a probable recurrence within the caudate lobe was discovered by a liver CT scan without evidence of extrahepatic spreading. Tumour markers, FNAB, and bone scintigraphy were negative. On July 2000 the patient underwent second look laparotomy. Only a coeliac lymphadenopathy was found and a lymphadenectomy performed. Specimen's histologic examination showed a metastatic lymph nodal disease (FLC). The postoperative course was uneventful and the patient recovered. He is currently alive without evidence of recurrence 5 years after the second operation.
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PMID:[Surgical therapy of hepatic fibrolamellar carcinoma]. 1751 32

We describe a 20-year-old woman with autoimmune hepatitis (AIH) with cirrhosis who developed Kikuchi-Fujimoto's disease (KFD) and de novo minor features of systemic lupus erythematosus (SLE). This is the first report of a patient with histologically confirmed AIH developing KFD (histiocytic necrotizing lymphadenitis). One previous case described KFD after AIH (diagnosed clinically but without biopsy). KFD is a rare condition of unknown aetiology, first described in 1972, characterized by fever and cervical adenopathy and has a self-limiting course. KFD is associated with SLE, and SLE in turn can be associated with abnormal liver function tests, which in a minority of cases may be due to AIH. The association of AIH, KFD, and SLE in our patient suggests an autoimmune pathogenesis of KFD.
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PMID:Autoimmune hepatitis associated with Kikuchi-Fujimoto's disease. 1809 Sep 96

Melioidosis is endemic in Taiwan. It is caused by infection with Burkholderia pseudomallei. A prolonged course of oral eradication therapy to avoid relapse after an intensive intravenous therapy is recommended to treat melioidosis. Melioidosis with cardiac involvement is rare and is often combined with septicemia, for which the mortality rate is 20-60%. The initial clinical presentations of melioidosis mimic Mycobacterium tuberculosis infection, which is the most common etiology of bacterial pericarditis in Taiwan. We present a case of non-septicemic melioidosis that presented as non-suppurative cardiac tamponade and left subcarinal lymphadenopathy. Underlying diseases included hepatitis B-related liver cirrhosis and hepatocellular carcinoma. The patient was successfully treated with 2 weeks of intravenous ceftazidime and 12 weeks of oral doxycycline, trimethoprim-sulfamethoxazole, and amoxicillin/clavulanate. Melioidosis-related pericarditis should be considered in the differential diagnoses of bacterial pericarditis in Taiwan.
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PMID:Non-septicemic melioidosis presenting as cardiac tamponade. 1878 43

Here we report a case of a 25-year-old woman with mesenteric and hepatic sarcoidosis without lung involvement complicated by severe noncirrhotic portal hypertension. In 1992, at the clinical presentation, she had abdominal pain, asthenia, and weight loss. Splenomegaly, signs of flogosis, sideropenic anemia, and cholestasis were observed. Laparoscopic abdominal exploration and histological analysis demonstrated noncaseating granulomas of the liver, abdominal lymph nodes, and mesenteric connective tissue. The clinical course was severe with episodic remissions and recrudescences characterized by ascites (mild or moderate), elevation of bilirubin levels (mean: 1.1 mg/dl; range: 0.9-3.5 mg/dl), reduction of albumin levels (mean: 4 g/dl; range: 3.4-4.2 g/dl), and prolongation of elevated international normalized ratio (mean: 1; range: 0.9-1.4). In 1997, the patient had variceal bleeding. Complete hemostasis was obtained with band ligation. Liver function was preserved, and until 2000 the disease remained stable. In 2001, the patient became pregnant. At the 36th week of gestation, the patient delivered a healthy female infant and afterwards remained in clinical remission. This report stresses that sarcoidosis can have a hepatic and mesenteric involvement in absence of thoracic lymphadenopathy. Portal hypertension may be severe, and in absence of cirrhosis it may be associated with portal thrombosis. Finally, portal hypertension in patients with hepatic sarcoidosis and preserved liver function should not be considered as an absolute contraindication to pregnancy.
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PMID:Hepatic and mesenteric sarcoidosis without thoracic involvement: a case of severe noncirrhotic portal hypertension and successful pregnancy. 1878 74

Sarcoidosis is a multisystem disease characterized by noncaseating granulomas in the affected organs, including skin, heart, nervous system, and joints. Diagnosis of sarcoidosis is generally based upon a compatible history, demonstration of granulomas in at least two different organs, negative staining and culture for acid fast bacilli, absence of occupational or domestic exposure to toxins, and lack of drug-induced disease. Involvement of the hollow organs is rare. Rather than being due to sarcoidosis, some reported mucosal lesions may simply have incidental granulomas. Extrinsic compression from lymphadenopathy can occur throughout the gastrointestinal tract. The stomach, particularly the antrum, is the most common extrahepatic organ to be involved, while the small bowel is the least common. Liver involvement frequently occurs and ranges from asymptomatic incidental granulomas to portal hypertension from granulomas in the portal triad, usually with relatively preserved liver function. CT scans show hepatosplenomegaly and adenopathy, followed in frequency by focal low-attenuation lesions of the liver and spleen. Ascites is usually a transudate from right heart failure (because of pulmonary hypertension) or portal hypertension (because of biliary cirrhosis). Rarely, an exudative ascites may occur from studding of the peritoneum with nodules. Pancreatic involvement presents as a mass, usually in the head or a diffusely firm, nodular organ. Corticosteroids should be instituted when organ function is threatened, usually lungs, eyes, and central nervous system. Their role in the treatment of hepatic sarcoidosis is unclear. The overall prognosis is good although most patients will have some permanent organ impairment. Cardiac and pulmonary diseases are the main causes of death.
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PMID:Gastrointestinal and hepatic manifestations of sarcoidosis. 1936 62

A 51-year-old man with a known history of hepatic cirrhosis and a right lung mass presented with hemoptysis and widespread bone pain. Sputum cytology demonstrated atypical cells. A CT scan of the chest demonstrated a 3-cm spiculating mass in the right lower lobe, in addition to nodules in both upper lobes and lymphadenopathy within the mediastinum and both hila. Skeletal scintigraphy was performed to assess for metastatic bone disease, and demonstrated increased tracer uptake along the cortex of the distal femurs bilaterally. There was also low-grade cortical uptake in the mid femur and tibia bilaterally on planar imaging. SPECT/CT was able to improve the specificity of the planar scintigraphic findings, by confirming tracer uptake was localized to the periosteum as expected for hypertrophic pulmonary osteoarthropathy, thereby excluding the presence of skeletal metastases.
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PMID:Hypertrophic pulmonary osteoarthropathy demonstrated on SPECT/CT. 1969 32

Evidence of immune stimulation has been noted in opiate dependent patients for many decades. Documented changes have included lymphadenopathy, round cell infiltration of the hepatic portal triads, diffuse peri-bronchitis, hyperglobulinaemia, lymphocytosis, monocytosis, systemic cytokine stimulation, and cytokine and chemokine activation within the neuraxis. A parallel literature describes an elevated list of chronic degenerative disease as common in such patients including neurodegenerative conditions, atherosclerosis, nephrosclerosis, hepatic fibrosis and cirrhosis, chronic obstructive and fibrotic lung disease, osteoporosis, chronic periodontitis, various cancers, hair greying, and stem cell suppression. All of these disorders are now known to have an important immunological role in their pathogenic pathways. The multisystem nature of these myriad changes strongly suggest that the ageing process itself is stimulated in these patients. The link between the immunostimulation on the one hand and the elevated and temporally advanced nature of the chronic degenerative diseases on the other appears not to have been made in the literature. Moreover as immunostimulation is also believed to be an important, potent and principal contributor to the ageing process it appears that experimental and studies of this putative link are warranted. Verification of such an hypothesis would also carry management implications for dose and duration of chronic pain and addiction treatment, pharmacotherapeutic selection, and novel treatments such as long term naltrexone implant therapy and heroin trials.
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PMID:Chronic immune stimulation as a contributing cause of chronic disease in opiate addiction including multi-system ageing. 2080 Mar 62

Primary Effusion Lymphoma (PEL) is a rare form of Non-Hodgkin lymphoma that involves serous body cavities (pleural, pericardial, or peritoneal) with lymphomatous effusions in the absence of lymphadenopathy or organomegaly. Although it was seen mostly in HIV-positive patients, it has recently been reported in HIV-negative patients with chronic Hepatitis B or C infections. PEL is associated with human herpes virus type-8 infection, often presents with rapidly progressive effusions and generally has a poor prognosis. This is a case of a 65-year-old HIV-negative man with Hepatitis C cirrhosis, who presented with abdominal pain. An F-18 FDG PET/CT showed marked ascites and pleural effusions with increased F-18 FDG uptake in the pleura and peritoneum on the left side. Analysis of the cells in the peritoneal fluid revealed a human herpes virus 8-positive PEL of the peritoneum. As a result the patient was no longer considered a liver transplant candidate and died 2 weeks after the diagnosis.
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PMID:Hepatitis C-related primary effusion lymphoma of the pleura and peritoneum, imaged with F-18 FDG PET/CT. 2083 90


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