Objective Fulminant hepatitis is a rare but fatal disease. In the present study, we examined the changes in etiology and prognosis of fulminant hepatitis in Nagasaki Prefecture, Japan between 1980 to 1999.
Methods Eighty-one patients with fulminant hepatitis admitted to our hospitals from 1980 to 1999 were examined with respect to the etiology and prognosis.
Results Fulminant hepatitis was due to hepatitis A virus in 2 (12%) cases, hepatitis B virus in 18 (22%) cases, unknown etiology in 50 (62%) cases, and drug-induced in 11(14%) cases. The number of cases in the first half of the study (1980-1989) was 47 and that of the latter half (1990-1999) was 34 cases. The incidence of fulminant hepatitis type B also decreased from 14 cases (30%) to 4 cases (12%) during these periods. The overall survival rate of fulminant hepatitis was 32%; it was equal in fulminant hepatitis type B, fulminant hepatitis of unknown etiology and fulminant drug-induced hepatitis. The survival rate of fulminant hepatitis type A was 100%, though only two cases were identified. Retrospectively, the survival rate in patients with a pre-encephalopathy period of ≤10 days and aged ≤39 years was significantly higher than in patients ≥40 years of age (p<0.01). There was no difference between the two age groups when pre-encephalopathy period was ≥11 days.
Conclusions The incidence of fulminant hepatitis especially that of fulminant hepatitis type B in Nagasaki Prefecture has decreased in recent years. The survival rate is significantly higher in younger patients with a short preencephalopathy period.
(Intenal Medicine 40: 5-8, 2001)

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Objective There is an increasing number of accidents by erroneous ingestion of button batteries in recent years; the batteries arouse the interest of infants because of their attractive shape and luster. The batteries remaining in the gastrointestinal tract and discharging electric current over a long period of time may induce ulceration or perforation, thus must be carefully considered the selection of appropriate treatment.
Methods We remove erroneously ingested button batteries with two tubes with ferrite magnets nearly the same size as the button batteries themselves.
Patients Four cases of erroneous ingestion of button batteries.
Results We easily removed button batteries from the stomach within 5 minutes in all cases with two magnet-attached tubes.
Conclusion We present this battery removal device together with a literature review, because it seems convenient and useful.
(Internal Medicine 40: 9-13, 2001)

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Objective The aim of this study was to clarify the trend of the HTLV-1 seroprevalence rate among blood donors.
Methods Samples were screened by the particle agglutination test for HTLV-1 antibodies.
Materials Serum from blood donors was screened.
Results The positive rate of HTLV-1 antibodies among blood donors decreased from 13.14 to 0.81% over the years from 1928 to 1983.
Conclusion A decline over the years in the positive rate of HTLV-1 antibodies among blood donors was revealed.
(Internal Medicine 40: 14-17, 2001)

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A 54-year-old man with a complaint of dysphagia was found to have a prominent stricture in the proximal esophagus. A biopsy of the stenotic area indicated sarcoma, leading to subtotal esophagectomy. The surgically removed esophagus demonstrated a well-defined intramural mass, consisting of a mixture of fibroblastic cells with bland cy tological appearances and inflammatory cells. Reflux esophagitis which was present distal to the stricture seemed to play a role in the development of this inflammatory pseudotumor.
(Internal Medicine 40: 18-22, 2001)

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We report a case of chronic gastric volvulus in which ultrasonography (US) was useful. An 81-year-old woman was hospitalized due to vomiting, and upper gastroduodenoscopy revealed that the stomach was spirally twisted and constricted. An upper gastrointestinal barium study demonstrated an organoaxial-mesenteroaxial combined type gastric volvulus. US showed constriction between the dilated upper stomach body and the lower stomach body similar to a "peanut". Thereafter, the patient's vomiting stopped and follow-up US demonstrated that the constriction of the stomach was loosened. Therefore, we believe that this characteristic US sign paralleled the symptoms of the patient.
(Internal Medicine 40: 23-27, 2001)

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A 66-year-old woman with an altered mental status caused by hypoglycemia was referred to Akita City Hospital. Abdominal ultrasonography (US) and endoscopic US revealed an isoechoic mass measuring 25 mm in the head of the pancreas. Endoscopic retrograde Cholangiopancreatography (ERCP) revealed that the main pancreatic duct and its branches were displaced around the mass in the head of the pancreas. On arteriography, a poorly vascularized tumor was observed. Dynamic contrast-enhanced computed tomography (CT) showed a low-attenuation mass in the head of the pancreas. A pylorus-preserving pancreaticoduodenectomy was performed. Microscopically, the diagnosis was malignant insulinoma.
(Internal Medicine 40: 28-31, 2001)

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We describe a Japanese case of neonatal hyperinsulinism due to a de novo mutation (Gly446Asp) in glutamate dehydrogenase gene (GLUD1). A boy suffered from hypoglycemic coma with relative hyperinsulinemia on day 1 after birth, and received subtotal pancreatectomy. Examination of the resected pancreas revealed a diffuse increase in endocrine cells, consistent with 'nesidioblastosis'. He is now 15 years old and has exhibited mild but persistent hyperammonemia, which is a very unique feature of the disorder caused by GLUD1 activating mutations. He has also been suffering from seizures and mental retardation. Thus, GLUD1 mutations can be a cause of congenital hyperinsulinisni in Japanese.
(Internal Medicine 40: 32-37, 2001)

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A female with von Hippel-Lindau (VHL) disease type 2A first presented with erythrocytosis at the age of 9 years. This patient revealed multiple paragangliomas at age 22. After the removal of tumors, a retinal hemangioblastoma developed. Our diagnosis of VHL disease type 2 A was confirmed. Moreover, systemic examination showed a duodenal somatostatinoma. Frequent and long-term monitoring is important for patients with pheochromocytomas or paragangliomas, and a screening for VHL disease and other hereditary cancer syndromes is recommended. Recognition of neuroendocrine tumors as a manifestation of VHL disease permits earlier diagnosis and improves prognosis.
(Intenal Medicine 40: 38-43, 2001)

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A case of Basedow's disease, that developed after successful treatment of ulcerative colitis with a total colectomy, is presented, along with a review of the Japanese literature on the coexistence of hyperthyroidism and ulcerative colitis. A 26-year-old man was referred to our department, complaining of general fatigue, appetite loss, and palpitation. At age 14, blood was discovered in his stool and a diagnosis of ulcerative colitis was made. Since then, he has been treated with salazosulfapyridine and prednisolone. On examination, mild exophthalmos and thyroid swelling were observed. Both serum free T₃ and T₄ levels were increased along with a positive TSH receptor antibody, while TSH was decreased. Scintigraphic and ultrasonographic examinations of the thyroid gland showed diffuse enlargement. Treatment with thiamazole relieved the symptoms and normalized the thyroid function. Although a high incidence of autoimmune thyroid diseases in association with ulcerative colitis has been suggested, only 6 cases of hyperthyroidism coexisting with ulcerative colitis have been reported in Japan. A common immunological process has been suggested to be implicated in the pathogenesis of this association, however, the exact mechanism remains unclear.
(Internal Medicine 40: 44-47, 2001)

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In a 35-year-old woman diagnosed with insulinoma selective intraarterial calcium injection was positive for proximal gastroduodenal artery and inferior pancreaticoduodenal artery involvement preoperatively. Although non-invasive imaging studies were negative, a mass was detected at the pancreatic uncus by intraoperative ultrasonography. Gastroduodenal artery calcium injection was performed after enucleation of the tumor. Following calcium injection, the insulin level was inappropriately increased and further pancreaticoduodenectomy was performed. After excision, another tumor was detected at the head of the pancreas by histopathological examination. As shown, selective intraarterial calcium injection is useful to localize tumors preoperatively and intraoperatively.
(Internal Medicine 40: 48-51, 2001)

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We treated a female patient suffering from immunoglobulin A (IgA) nephropathy and congenital deficiency of the ninth component of the complement system (C9). She was admitted with hematuria and proteinuria, and the C9 deficiency was diagnosed based on the low hemolytic activity of 50% of the hemolytic unit of the complements (CH50) and the normal C3 level in the plasma. Renal biopsy revealed mild mesangial proliferation, and immunofluorescence examination revealed mild mesangial deposits of IgA and C3 with the same distribution. We discuss the pathogenesis of IgA nephropathy and the role of the complements in its progression.
(Internal Medocine 40: 52-55, 2001)

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We describe a case of solitary papilloma of the bronchus and provide a review of 38 similar cases reported in Japan. A 70-year-old man complained of cough and sputum. Chest X-rays and CT scans revealed atelectasis of the right middle lobe. On bronchoscopy, a polypoid tumor was found at the orifice of the bronchus of the right middle lobe. The tumor was histologically diagnosed as a squamous papilloma with moderate atypia. Because of elevated tumor markers and the reported high incidence of malignant changes in papillomas, the tumor was endoscopically resected by electrosurgical snare. While this procedure resulted in improvement of atelectasis, the chest CT taken subsequently revealed a mass adjacent to the resected polypoid tumor in the middle lobe bronchus. Percutaneous needle biopsy followed by histopathological examination confirmed the tumor to be a squamous cell carcinoma. Only three cases of malignant changes in papillomas have been previously reported in Japan. Electrosurgical snare, which allows the identification of tissue at the tumor base, should be the treatment of choice rather than YAG laser surgery.
(Internal Medicine 40: 56-60, 2001)

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We report two cases of severe bronchopneumonia due to influenza A (H3N2) virus. The severity of the disease necessitated initiation of empiric therapy based on the present illness and clinical data on admission. Both patients were improved by artificial ventilation with positive end-expiratory pressures and administration of broad spectrum antibiotics and corticosteroids before confirming the diagnosis of viral bronchopneumonia using viral culture and serological tests. Within 24 hours, influenza A (H3N2) virus was identified by amplification of the pathogen genes by reverse transcription polymerase chain reaction (RT-PCR) using the stored bronchoalveolar lavage (BAL) fluids of both cases. This suggests that a combination of detection methods of pathogens using RT-PCR and BAL fluid will facilitate determination of rational treatment aimed at influenza A virus.
(Internal Medicine 40: 61-67, 2001)

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Behçet's disease (BD) is a chronic relapsing systemic vasculitis in which orogenital ulceration is a prominent feature. The disease affects many systems and causes hypercoagulability. We present a 27-year-old male patient who exhibited widespread great vessel thrombosis including right atrial and ventricular thrombi in the setting of right-sided infectious endocarditis and orogenital aphthous ulcerations and erythema nodosum due to BD. We reviewed the enigmatic prothrombotic state of BD, and discuss our prior experiences in this field.
(Internal Medicine 40: 68-72, 2001)

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This is the first case of a limited form of systemic sclerosis (ISSc) associated with acute congestive heart failure (CHF) and primary biliary cirrhosis (PBC). A 58-year-old woman with ISSc was admitted because of a sudden onset of CHF. The intravenous administration of nitroglycerine and furosemide ameliorated the symptoms of CHF within 24 hours. She had both anticentromere antibodies and antip25 doublet/triplet antibodies to intrahepatic microsomes. Thallium scintigraphy at rest demonstrated significant perfusion defects in both the anteroseptal and inferior myocardium. A coronary angiogram revealed normal coronary arteries and no vasospasm was provoked by the intracoronary administration of acetylcholine. The present case indicates that minute care should thus be taken for the prevention of acute CHF even in patients with a limited form of SSc when thallium perfusion defects are identified.
(Internal Medicine 40: 73-76, 2001)

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