Discovery of Helicobacter pylori has changed the life cycle of peptic ulcer disease (PUD). However, PUD does not completely disappear after elimination of H. pylori. Some ulcers recur even after successful eradication of H. pylori in non-users of non-steroidal anti-inflammatory drug (NSAID). In addition, the incidence of H. pylori-negative, non-NSAID PUD (idiopathic PUD) is reported to increase with time. Moreover, H. pylori-positive ulcers are not always H. pylori-induced ulcers because there are two paradoxes of the H. pylori myth: the existence of H. pylori-positive non-recurring ulcer and recurring ulcer after cure of H. pylori infection. Taken together, H. pylori is not the only cause of peptic ulcer disease. Therefore, it is still necessary to seriously consider the pathophysiology and the management of the ulcers, which may exist after elimination of H. pylori.

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  Objective  Obstructive sleep apnea syndrome (OSAS) is associated with increased cardiovascular morbidity and mortality. We investigated the values of brachial-ankle pulse wave velocity as an indicator of atherosclerosis in obstructive sleep apnea syndrome patients.
  Materials and Methods  Brachial-ankle pulse wave velocity (baPWV) was measured in 104 OSAS patients and 104 healthy control subjects matched for age, sex, and body mass index (BMI). BaPWV values were compared in both groups and investigated with respect to the number of risk factors for atherosclerosis, including hypertension, hypercholesterolemia, impaired glucose tolerance, smoking, and obesity. Comparisons were also made between 48 OSAS group cases and 90 control group cases free from hypertension, which has a major impact on baPWV.
  Results  As compared to the control group, the OSAS group had significantly higher baPWV (1, 645±349 cm/s vs 1, 436±278 cm/s, p<0.0001), and values obtained for baPWV were significantly higher in the OSAS group than in the control group even in groups free from hypertension (1, 453±216 cm/s vs 1, 374±213 cm/s, p<0.05). In both groups, baPWV rose as the number of risk factors for atherosclerosis increased, but baPWV was higher in the OSAS group than in the control group even in a comparison of individuals entirely free from risk factors (1, 400±200 cm/s vs 1, 198±79 cm/s, p<0.05).
  Conclusion  The condition of OSAS itself is considered a possible risk factor for atherosclerosis. We believe that the usefulness of baPWV as an index of atherosclerosis merits further study in the frequently observed cases of OSAS complicated by cardiovascular disease.

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  Objectives  Thymectomy has become recognized as an integral element in the treatment of patients with myasthenia gravis (MG). Although the incidence of elderly-onset MG has recently been increasing, there is little data demonstrating the efficacy and complications of thymectomy in this population. To clarify this point, we divided the thymectomized patients with MG into young and elderly groups, and retrospectively examined their clinical features and therapeutic outcomes.
  Patients and Methods  We reviewed 57 MG patients who had been admitted to our hospital between 1988 and 2002. The patients were classified into young (younger than 60) and elderly (60 or older) groups according to the age of onset, and the therapeutic outcomes of thymectomy were evaluated using myasthenic severity scales and the duration from operation to discharge.
  Results  Myasthenic severity scales significantly improved after thymectomy in the elderly group both with (p<0.005) and without thymoma (p<0.05) compared with before. With regard to the duration from thymectomy to discharge, no significant difference could be found between the young and elderly groups, irrespective of associated thymoma. There were no serious complications during and after thymectomy in either the young or the elderly group.
  Conclusions  Despite various possible complications due to aging, thymectomy should be actively considered also in the treatment of elderly MG patients because it can reliably and safely improve myasthenic symptoms in combination with immunosuppressive agents, including corticosteroid.

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Congenital absence of the portal vein (CAPV) is a rare malformation that is often accompanied by other anomalies such as cardiac and skeletal malformations and/or hepatic tumors. We describe here a case of CAPV complicating hepatic tumors in a 16-year-old Japanese girl. Abdominal ultrasonography revealed a hyperechoic tumor in the liver and dilatation of the portal vein that appeared to be connected directly with the inferior vena cava. Subsequent abdominal computed tomography (CT) revealed tumors and magnetic resonance angiography confirmed that the portal vein entered directly into the inferior vena cava just above the liver. In addition, there was absence of the right portal vein and the left intrahepatic branch except for the presence of left portal vein only within the porta hepatis. These findings led to a diagnosis of CAPV complicated hepatic tumors. Careful monitoring of these hepatic tumors is ongoing due to the possibility of malignant transformation.

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We report a 67-year-old Japanese man who presented with worsening heart failure with asymptomatically transient ischemic ST-segment depression. Left ventriculography showed diffuse hypokinesis; asymptomatic coronary artery spasm was evoked by the acetylcholine provocation test. Endomyocardial biopsy exhibited hypertrophic cardiomyocytes and scattered microscopic focal myocardial necrosis with amyloid-deposition. Transient ST-segment depression improved after treatment with a calcium antagonist, but cardiac contraction was still impaired. We hypothesize that asymptomatic coronary spasm may cause irreversible cardiac damage and heart failure with amyloid-deposition; the presence or absence of coronary spasm in heart failure patients should be clarified in order to determine therapeutic strategy.

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We describe a rare case of Becker’s muscular dystrophy (BMD) in a 28-year-old man complicated by rapidly progressing heart failure without apparent clinical signs of neuromuscular disease. He showed rhabdomyolysis, which repeatedly occurred causing acute renal failure as heart failure worsened. His serum creatine kinase (CK) level was generally below 300 IU/l. However, it exceeded more than 10,000 IU/l at the time of myoglobinuria. This suggests that the worsening of heart failure could induce rhabdomyolysis in a BMD patient. Gene analysis for BMD should be considered when the elevation of serum CK is noted in heart failure.

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A 76-year-old man with autosomal dominant polycystic kidney disease (ADPKD) was admitted complaining of severe edema of bilateral lower extremities. Computed tomography (CT) of his abdomen revealed an enlarged hepatic cyst with intra-cystic hemorrhage, and massive thrombosis in the inferior vena cava (IVC). The extrinsic mechanical stress on the IVC seemed to induce thrombosis within the IVC, and the thrombosis was likely the cause of severe edema in lower extremities. In this report, we present a rare case of IVC thrombosis due to an enlarged cyst in ADPKD successful treated with anticoagulant therapy.

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A 66-year-old man had a progressive increase in the pacing threshold over a one-year period, resulting from chronic myocarditis. Following steroid therapy, the pacing threshold decreased and became stabilized, and was accompanied by a decrease in the serum creatine kinase, cardiac myosin light chains and pro-collagen III peptide values, but cardiac function did not improve. Endocardial biopsy showed that there was no progression in the fibrosis. The pacing failure improved, but the cardiac function did not. It was believed that the steroid therapy suppressed the progression of the inflammation and fibrosis caused by the chronic myocarditis.

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Familial hypokalemic periodic paralysis is an autosomal dominant genetic muscle disease characterized by periodic attacks of muscle weakness associated with a decrease in serum potassium. There are two major missense mutation sites in the calcium channel α1 subunit (CACNA1S) gene in these patients. We recently encountered a 13-year-old Japanese boy who had collapsed following exercise and was found to have a low serum potassium level. Clinical and genetic studies including exploration of his family tree proved that he and his maternal relatives had the disease with the missense mutation, Arg528His (CGC→CAC). However, his mother and grandmother had no symptoms of the disease, indicating reduced penetrance in female carriers. Sexual difference in the penetrance of this disease and the association between the clinical symptoms and the types of genetic defects are discussed.

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Alpha-1 antitrypsin (AAT) deficiency is a hereditary disorder characterized by an early onset of emphysema. While this disease is common in the Caucasian population, it is quite rare in Japan. To date, only 15 traits have been reported and it can be speculated that many cases of this genetic deficiency may have been overlooked. We report an additional case of AAT deficiency with severe emphysema that is genetically determined as S_iiyama variant by allele-specific polymerase chain reaction (PCR) analysis.

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A 19-year-old man visited our hospital complaining of cough, sputum and low-grade fever. Chest radiograph and computed tomography findings suggested that he was suffering from pulmonary Langerhans’ cell histiocytosis (PLCH). Bronchoscopy revealed a whitish elevated lesion at the bifurcation of the right upper lobe bronchus, and a specimen of this lesion showed the same pathological findings as pulmonary parenchymal lesions. Although there have been only a few reports of endobronchial LCH without pulmonary parenchymal lesions, this is, to our knowledge, the first case of PLCH with an endobronchial lesion, which was confirmed by bronchoscopy, and disappeared several months later.

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Clarithromycin (CAM) has been widely used for the treatment of respiratory infection. Macrolides are generally well tolerated and their adverse reactions are rare. An 80-year-old woman with nontuberculous mycobacterium infection was treated with combined chemotherapy, including isoniazid, rifampicin, and ethambutol. She developed a fever and peripheral blood eosinophilia, and new subpleural consolidations were observed on chest radiography three days after add-on therapy with CAM. The symptoms and clinical findings improved with the withdrawal of CAM. Histopathologic examinations confirmed the diagnosis of eosinophilic pneumonia. This is the first report of CAM-induced eosinophilic pneumonia.

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We describe a case of malignant lymphoma which presented in the body cavities without identifiable tumor masses. Malignant lymphoma cells showed strong atypia with prominent nuclei and basophilic cytoplasm containing vacuoles. The chromosomes showed diploidy and complex abnormalities including translocations and deletions. We diagnosed this patient with primary effusion lymphoma (PEL), even though she tested negative for human herpes virus-8 (HHV-8) which has been suggested to be causally related to PEL. Interestingly, the patient also showed complicated protein-losing enteropathy, and PEL occurred after repeated chylous ascites and chylothorax. The possible pathogenesis of this rare disease is discussed here.

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A 34-year-old man who had a history of ulcerative colitis (UC) was admitted to our hospital with complaints of arthralgia, erythema nodosum, recurrent oral aphthous ulcers and bloody stools. A colonoscopy revealed multiple aphthous ulcers on his cecum and colon and also revealed a transmural ulcer on his rectum consistent with a diagnosis of UC. The patient was HLA-B51 positive. Based on clinical evidence [recurrent oral ulcers, skin lesions (erythema nodosum), positivity for pathergy test] this patient was diagnosed as having Behçet’s disease with gastrointestinal involvement. We describe this rare case of Behçet’s disease with colitis and discuss the difficulties in making a differential diagnosis between Behçet’s disease and the inflammatory bowel diseases.

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A 53-year-old woman presented with oliguria, urinary frequency, abdominal pain and severe edema of the lower extremities. Her serum creatinine was 8.1 mg/dl. Computed tomographic and ultrasonographic studies showed a severely dilated urinary bladder, and bilateral hydroureteronephrosis. Examination of a urinary bladder biopsy specimen showed subepithelial edema and infiltration by lymphocytes and plasmacytes. However, the patient complainted of dry mouth and dry eyes. Ophthalmologically, the Schirmer test was positive. A biopsy of the minor salivary glands in the lip showed chronic sialoadenitis. A diagnosis of Sjögren’s syndrome complicated by interstitial cystitis was made. Since she had been anuric, secondary to urinary obstruction, intermittent self-catheterization was started. Combination of corticosteroid and cyclosporin therapy was initiated. Spontaneous urination began, and gradually the patient’s symptoms remitted. After 8 months of therapy, bladder capacity increased from 140 ml to 350 ml, and she voided approximately 1, 200 ml by herself and 600 ml by catheterization daily. This case suggests that when severe interstitial cystitis is associated with Sjögren’s syndrome, a therapeutic trial of corticosteroids and cyclosporin may be beneficial.

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We describe a patient with type I hereditary angioedema presenting recurrent episodes of skin swelling and abdominal pain. Laboratory examination showed reduced levels of CH50 and C4 with a normal C3 level. The C1 inhibitor was decreased to 7.0 mg/dl (normal, 10-25 mg/dl) with a remarkably reduced activity (<25%; normal, 80-125%). DNA analysis of the C1 inhibitor gene revealed a novel point mutation at the 3' acceptor mRNA splice site of the intron 5 (G→A at nucleotide 8722). This mutation may abolish the correct splicing of the intron 5 and create unstable mRNA.

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The association of leukocytoclastic vasculitis and renal cell carcinoma has been rarely documented. We report a patient who presented with leukocytoclastic vasculitis involving the skin and was diagnosed later as renal cell carcinoma. After the nephron-sparing surgery, the vasculitic lesions disappeared. We also briefly review cases of vasculitis and renal neoplasms.

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We report a series of five patients with autoimmune hepatitis (AIH) accompanied by systemic lupus erythematosus (SLE) (AIH-SLE overlap). Serologic tests showed that all patients were positive for antinuclear antibody and double-stranded DNA antibody. Histological examination of the liver showed that three of the patients had chronic hepatitis with severe activity. One of the other two had acute and severe hepatitis with submassive necrosis in both portal and lobular areas. The last patient already had liver cirrhosis. All patients had a mild form of SLE and showed a rapid response to corticosteroid. There was no serious involvement of organs other than the liver in any of the patients, and the prognoses were comparatively good in all patients.

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Four habitual drinking and smoking patients with pulmonary tuberculosis who were thought to have had no contact with one another were admitted to our hospital. During admission, we found that they were regular visitors of the same bar. To investigate the possibility of outbreak, we analyzed the tuberculosis isolates from them by restriction fragment length polymorphism. Such analysis showed identical chromosomal DNA restriction patterns of these 4 culture isolates. We concluded that these patients were considered to represent a mini-outbreak of pulmonary tuberculosis, although there was little, if any, contact among them while in or out of the bar.

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