Hepatic Tumor Rupture in Other Iatrogenic Immunodeficiency-Associated Lymphoproliferative Disorders of the B-cell Type in a Patient With Chronic Rheumatoid Arthritis.

A 75-year-old woman on tumor necrosis factor inhibitors for rheumatoid arthritis presented with hematemesis and a gastric biopsy revealed diffuse large B-cell lymphoma with possible bulky left liver tumor involvement. On the second day of treatment with rituximab, cyclophosphamide, doxorubicin, vincristine, and prednisone, the patient experienced abdominal pain followed by shock vitals. A contrast-enhanced computed tomography scan revealed a ruptured liver. Transcatheter arterial embolization (TAE) was performed to stop the bleeding. This is the first case of hepatic tumor rupture secondary to an iatrogenic immunodeficiency-associated lymphoproliferative disorder of the B-cell type that was successfully treated with TAE to achieve hemostasis.

Premature Onset Aortic Stenosis in Systemic Sclerosis: A Report of a Series of Cases.

Although cardiovascular involvement is a well-known complication correlated with a poor prognosis in patients with systemic sclerosis, there are few reports on valvular heart disease. Forty patients with systemic sclerosis were retrospectively analyzed. Valvular heart disease was found in six patients, five of whom had severe tri-leaflet aortic stenosis. Three of these 5 patients were ≤71 years old. Two frail elderly patients who underwent transcatheter aortic valve replacement died within two years. Premature-onset aortic stenosis is not uncommon in patients with systemic sclerosis. When considering mechanical intervention, the evaluation of frailty is important.

[Shewanella algae bacteremia in patients with biliary tract malignancy: report of three cases].

We encountered three cases of Shewanella algae bacteremia in patients with biliary tract malignancy. Shewanella species are mainly found in seawater and other watery environments. Human infections caused by Shewanella species have been rare. However, reports of Shewanella infections are increasing, although only a few have been from Japan. The three patients we encountered had common features, including hepatobiliary malignancy, male sex, and age over 60 years. These features are similar to those in reports from elsewhere. Shewanella infection bears consideration in patients with those characteristics who have a history of exposure to sea or fresh water or ingestion of seafood. Prompt treatment is needed as the mortality rate of this infection is high.

Phenotypes and Chronic Organ Damage May Be Different among Siblings with Wilson's Disease.

Background and Aims: Cloning of ATP7B provided evidence that Wilson's disease is a hepatic copper toxicosis with a variety of extrahepatic complications. Affected siblings with the same genetic background and exposure to similar environmental factors may be a good model for the study of genotype-phenotype correlation. Methods: Twenty-three affected siblings in 11 families were selected from a database. The first phenotypes were determined according to the international proposal. The final types of chronic organ damage were re-evaluated for life-long management. Results: Phenotypes were identical in 5 of the families and different in 6 of the families. The acute hepatic phenotype H1 was found in 3 younger siblings and 1 older sibling. All survived an acute episode of hemolysis with underlying chronic liver disease. One also presented complication with neurological disease. The neurological phenotype N1 with neuropsychiatric symptoms and hepatic disease was found in 2 aged siblings of 1 family, in an older sibling in 3 families and in the oldest sibling in 1 family. Phenotypes in siblings were mainly split by either H1 occurring in random order or age-dependent N1. Types of chronic organ damage were identical in 8 of the families and different in 3 of the families. The same combination of chronic liver disease was found in 6 families and chronic liver disease complicated with neurological disease in 2 families. Split organ damage in siblings was found when an older sibling was complicated by neurological disease. There was no reverse combination of a younger sibling being complicated by neurological disease in any of the families. Conclusion: Phenotype combinations of siblings were mainly modified by externally-induced hemolytic episodes, while chronic organ damage in siblings was split by age-dependent neurological complications.

Focal Intramucosal Adenocarcinoma Occurring in Gastric Hyperplastic Polyps: Two Case Reports.

Gastric hyperplastic polyps are generally considered benign lesions, although rare cases of adenocarcinoma have been reported. Two cases of intramucosal adenocarcinoma originating from gastric hyperplastic polyps that were successfully removed by endoscopic mucosal resection or endoscopic submucosal dissection are reported. On pathological examination, adenocarcinoma limited to the hyperplastic foveolar epithelial mucosa of the gastric hyperplastic polyps was observed.

[A case of adult-onset type II citrullinemia in an elderly patient].

A 72-year-old man presented with consciousness disturbance. The results of brain magnetic resonance imaging and cerebrospinal fluid examination were normal, but triphasic waves were noted on electroencephalography. His plasma ammonia level was elevated due to which encephalopathy secondary to hyperammonemia was suspected. However, his liver function was normal, and no evidence of cirrhosis or portal-systemic shunt was noted. The patient's medical history revealed that he had a tendency to excessively consume pulse products since childhood, and an amino acid analysis showed elevation of citrulline and arginine levels. Thus, we diagnosed the patient with an extremely rare case of adult-onset type II citrullinemia, which was triggered by cessation of the intake of pulse foods (soybeans and peanuts) due to dental problems.

Complications of radiofrequency ablation for hepatocellular carcinoma in a multicenter study: An analysis of 16 346 treated nodules in 13 283 patients.

AIM: We surveyed multiple centers to identify types and frequency of complications and mortality rate associated with radiofrequency ablation (RFA) of hepatocellular carcinoma (HCC). METHODS: We distributed a questionnaire developed by members of the Chugoku-Shikoku Society for the Local Ablation Therapy of Hepatocellular Carcinoma to 20 centers and analyzed types and frequency of complications and mortality rate. RESULTS: In total, 16 346 nodules were treated in 13 283 patients between January 1999 and November 2010. Five patients (0.038%) died: two from intraperitoneal hemorrhage, and one each from hemothorax, severe acute pancreatitis and perforation of the colon. In 16 346 treated nodules, 579 complications (3.54%) were observed, including 78 hemorrhages (0.477%), 276 hepatic injuries (1.69%), 113 extrahepatic organ injuries (0.691%) and 27 tumor progressions (0.17%). The centers that treated a large number of nodules and performed RFA modifications, such as use of artificial ascites, artificial pleural effusion and bile duct cooling, had low complication rates. CONCLUSION: This study confirmed that RFA is a low-risk treatment for HCC and that sufficient experience and technical skill can reduce complications.

[Fulminant Japanese spotted fever--the second fatal case in Japan].

A 77-year-old woman who have no past history, was admitted in a local hospital in Muroto City, Kochi, Japan, after several days of fever and severe general fatigue and generalized skin erythema. She was suspected to have Japanease spotted fever, which was a local pandemic disease. She was treated with minocycline immediately. The next day, she had consciousness disturbance and low blood pressure. Laboratory findings indicated disseminated intravascular coagulation (DIC) and multiple organ failure. She was referred to our hospital. An eschar was identified in the back of It. femur. Treatment included minocycline, ciprofloxain, gabexate mesilate, methylprednisolone, hemodialysis and mechanical ventilation. In spite of the avobe treatment, she died 3 days after admission of the local hospital. Though the serological test showed no positive antibody titer against Rickettsia japonica, Rickettsia japonica was isoleted from blood culture, to confirm Japanese spotted fever, Japanese spotted fever is generaly a curative disease with early diagnosis and minocycline. In this case, the patient died 3 days after proper diagnosis and treatment was started. We reported the second fatal Japanese spotted fever case in Japan.