Case of mixed connective tissue disease associated with autoimmune hepatitis

Summary A 56-year-old female with mixed connective tissue disease (MCTD) who developed autoimmune hepatitis is described. Hepatitis was controlled effectively by the corticosteroid therapy. Biopsy of the liver revealed swelling and hydropic degeneration of hepatocytes, accompanied by Councilman's body formation and focal necrosis. These histological findings differ from those in three previously described cases. A relationship between MCTD and liver involvement appears possible.     

Role of Active Oxygen Species and Lipid Peroxidation in Mepirizole-Induced Duodenal Ulcers in Rats

The role of active oxygen species and lipidperoxidation in the pathogenesis of duodenal ulcersinduced by mepirizole was investigated in rats. Oraladministration of mepirizole (200 mg/kg) resulted in ulcer lesions in the proximal duodenum.Thiobarbituric acid-reactive substances (TBA-reactivesubstances), an indicator of lipid peroxidation, alsosignificantly increased in the duodenal mucosa.Myeloperoxidase (MPO) activity in the duodenal mucosa, a signof polymorphonuclear leukocyte (PMN) accumulation,significantly increased. Combination treatment withpolyethylene glycol-modified Serratia Mn-SOD andcatalase significantly decreased the size of the ulcersand TBA-reactive substances in the duodenal mucosa.Allopurinol, a xanthine oxidase inhibitor, also reducedthe size of duodenal ulcers. Both the size of the ulcers and the increase in TBA-reactivesubstances in the duodenal mucosa were significantlylower in PMN-depleted rats. Mepirizole increased thesurface expression of adhesion molecule CD18 on PMNs in vitro. These results suggest that lipidperoxidation, mediated by active oxygen speciesgenerated from xanthine oxidase and PMNs, plays animportant role in the pathogenesis of duodenal ulcersinduced by mepirizole.     

Thrombopoietin-producing hepatocellular carcinoma

A 66-year-old man with hepatocellular carcinoma (HCC) showed marked thrombocytosis (110.7 x 10(4)/microl). Bone marrow (BM) aspirates demonstrated an increase of mature megakaryocytes (MgK). The serum thrombopoietin (TPO) level was increased to about 100-fold that of the normal level in the terminal stage. However, the platelet count gradually decreased to 13.5 x 10(4)/microl. The autopsy specimen revealed normoplastic BM with decreased MgK, mainly consisting of the immature type, and it was negative for tumor cells. Liver specimen showed markedly fatty metamorphosis. Immunohistochemical staining of TPO demonstrated that hepatocytes were weakly stained and HCC cells strongly stained, suggesting TPO-producing HCC.     

Acute pancreatitis occurring in gastric aberrant pancreas treated with surgery and proved by histological examination

We describe a case of gastric aberrant pancreas with acute pancreatitis. Barium meal examination, endoscopic examination and computed tomography of a 32-year-old man with abdominal pain revealed a submucosal tumor, about 3.5 cm in diameter, at the angulus of his stomach. Endoscopic ultrasonography revealed a hypoechoic mass with anechoic capillary areas. His serum amylase level was high at 262 IU/l. Laparoscopy-assisted local resection was carried out. The resected tumor revealed pancreatic tissue with extensive neutrophil infiltration in the gastric wall and fat necrosis in the subserosa. There are few cases of histologically proven acute pancreatitis in gastric aberrant pancreatic tissue.     

Increased Levels of Human Hepatocyte Growth Factor in Serum and Peritoneal Fluid After Partial Hepatectomy

Objective/Method : It has been reported that inflammatory cytokines up-regulate human hepatocyte growth factor synthesis in vitro. lo demonstrate the relation of this growth factor to interleukin-6 and tumor necrosis factor a, the changes in the levels of these cytokines were measured in serum and peritoneal fluid in 22 patients after partial hepatectomy. Results : Serum and fluids levels of cytokines showed a maximum within 3 days after surgery. Cytokines concentrations were much higher in fluid than in serum (p < 0.05). The maximum serum levels of human hepatocyte growth factor were significantly correlated with those of interleukin-6, intraoperative blood loss, and operating time (p < 0.05) but not with resected liver weights. In fluid level, the growth factor was also correlated with interleukin-6 (p < 0.05) but not with tumor necrosis factor α. Conclusions : These results suggest that human hepatocyte growth factor might be locally produced in the injured tissue associated with interleukin-6 and independently of resected liver weights.     

Thallium scintigraphy for the prognosis of idiopathic dilated cardiomyopathy

OBJECTIVES: This study evaluated the significance of perfusion defects demonstrated by thallium-201 and age in the prognosis of patients with idiopathic dilated cardiomyopathy. METHODS: Seventy-four dilated cardiomyopathy patients underwent thallium scintigraphy as well as clinical and hemodynamic examination. RESULTS: Abnormal perfusion defects were present in 23 of 38 patients aged < 60 years (61%) and in 26 of 36 elderly patients aged > or = 60 years (72%; NS). Univariate analysis showed that such perfusion defects were a significant predictor of cardiac death only in patients aged < 60 years (p = 0.015). Stepwise discriminant analysis also revealed that perfusion defects were a significant predictor in patients aged < 60 years (Wilks' lambda 0.499, chi-square test 20.2, p = 0.003). Perfusion defects were not more important than the history of syncope or stroke in elderly dilated cardiomyopathy patients. Twenty-one patients died of disease-related causes during 58 +/- 43 months. The five-year survival rate was better in patients aged < 60 years without than in those with perfusion defects (100% vs 58.4%, respectively), but not affected in patients aged > or = 60 years (66.7% vs 62.2%). CONCLUSIONS: Thallium scintigraphy is valuable for the prognosis of patients with dilated cardiomyopathy aged < 60 years who are usually candidates for heart transplantation. Absence of thallium perfusion defects may indicate good long-term prognosis.     

Pulmonary artery obstruction caused by thoracic aortic dissection: a case with unique pathological findings.

Pulmonary artery obstruction is a rare complication of acute thoracic aortic dissection. A 74-year-old woman was admitted to hospital with respiratory distress. Computed tomography scan showed right pulmonary artery occlusion and a thoracic aortic dissection of the Stanford A type. Lung perfusion scan revealed a defect in the entire right lung field. These findings mimicked acute pulmonary thromboembolism accompanying aortic dissection. On the other hand, pulmonary angiography revealed a round smooth defect of the right pulmonary artery, indicating an extrinsic compression. The patient finally died of cardiac tamponade. Autopsy disclosed that the right pulmonary artery was compressed by a hematoma in the adventitial space around the pulmonary artery. Such a mechanism of pulmonary artery obstruction caused by acute aortic dissection is unique, and is distinct from that caused by chronic non-dissecting aortic aneurysms, which themselves compress the pulmonary arteries. This complication inevitably follows aortic rupture; therefore, emergency operation to repair the dissected aorta must be performed to avoid the following catastrophic event.     

Granulocyte Colony-stimulating Factor-induced Aortitis with Lung Injury,Splenomegaly, and a Rash During Treatment for Recurrent Extraosseous Mucinous Chondrosarcoma

We herein report a case of aortitis induced by granulocyte colony-stimulating factor (G-CSF) that coincided with lung injury, splenomegaly, and cutaneous manifestations during treatment for recurrent extraosseous mucinous chondrosarcoma. Computed tomography revealed large-vessel vasculitis, splenomegaly, and pulmonary interstitial changes. Treatment with prednisolone was successful. Because sarcoma is a rare disease, this case is valuable for showing clinicians that G-CSF preparations could cause aortitis regardless of the patient''s underlying diseases or therapeutic pharmacological backgrounds.