Objective The diagnostic criteria of autoimmune hepatitis (AIH) were recently modified by the International Autoimmune Hepatitis Group. This study was performed to assess the impact of the revised scoring system on the diagnosis of AIH.
Patients and Methods We re-analyzed the clinical features of 89 patients diagnosed as AIH in Nagasaki Prefecture, Japan, using the revised scoring system, and compared the scores and final diagnosis with our previously published results using the original system.
Results Of the 89 patients with AIH, 40 (45%) were classified using the new system as "definite" AIH, 41 (46%) as "probable" AIH, and 8 (9%) patients were categorized as "others". Of these, 37 (42%), 35 (39%), and 4 (4%) patients who were classified as "definite", "probable", and "others" by the original system remained in the same category by the revised system, respectively. However, 3, 4, and 6 patients were re-categorized as "definite" from "probable", "others" from "probable", and "probable" from "definite", respectively. The difference in aggregate scores between the above two systems ranged from -5 to +2. The main contributing factors to the changes in aggregate AIH score were "other autoimmune disease(s)" and "interface hepatitis without lobular involvement and bridging necrosis on liver histology". However, the main contributing factors to the demotions from "definite" to "probable" and form "probable" to "others" were those related to the characteristics of biliary diseases, i.e., antimitochondrial antibody positive, biliary changes in liver histology, and alkaline phosphatase: aspartate aminotransferase ratio between 1.5 and 3.0. Moreover, two patients who had no histological evidence of AIH were both re-categorized as "others" from "probable" AIH.
Conclusion Our results indicated that the diagnosis, whether based on the revised or original system, was the same in the majority of AIH patients, but the revised scoring system excluded cases who had features suggestive of biliary diseases from "definite" AIH, and also confirmed that a diagnosis of "definite" AIH should not be made without liver histology.
(Internal Medicine 39: 1008-1012, 2000)

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Objective In hypertensive subjects, exercise training is a therapeutic modality that not only lowers blood pressure but also corrects metabolic abnormality, such as hyperinsulinemia. Insulin causes sympatho-excitation via the modification of baroreflex, norepinephrine release, or central sympathetic outflow. However, the link between neural and metabolic changes by exercise training in hypertensive patients remains unknown. The aim of this study was to examine whether or not the blood pressure lowering effect of exercise training is associated with the improvement of insulin sensitivity in conjunction with the inhibition of sympathetic tone in hypertensive patients.
Methods We evaluated plasma insulin levels, arterial baroreflex function and humoral parameters before and after exercise training. Twenty-nine patients with essential hypertension under hospitalization participated in the study. Before and after three weeks of exercise training (75% max VO₂, 6 min, q.i.d.), 24-hour blood pressure recordings, arterial baroreflex function testing and 75 g glucose tolerance tests were conducted. Area under the curve of insulin (∑ insulin) to glucose load was calculated as an index of hyperinsulinemia.
Results Three weeks of exercise training decreased the 24-hour mean arterial pressure, heart rate and ∑ insulin, and improved baroreflex function. There was a significant correlation between the reduction of arterial pressure and the change in ∑ insulin. Furthermore, the reduction of ∑ insulin was correlated with the improvement of baroreflex function and with the decrease in heart rate.
Conclusions Exercise training lowered the arterial pressure, with parallel changes in heart rate, baroreflex function and insulin resistance. The correction of sympathetic overactivity was closely associated with the amelioration of hyperinsulinemia. Our results suggest that the improvement of neuro-metabolic factors may be involved in the depressor effect caused by exercise training.
(Internal Medicine 39: 1013-1019, 2000)

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Objective Long-term nitrate therapy for ischemic heart disease may cause drug tolerance which diminishes its beneficial effects; consequently, intermittent administration of nitrates is recommended. With this regimen, however, the potential occurrence of rebound angina during the nitrate-free intervals is a source of concern.
Subjects and Methods We carried out a retrospective study of 606 patients to determine whether rebound angina occurred when conventional continuous nitrate administration was replaced by intermittent administration as part of a long-term therapy protocol for prior myocardial infarction. The subjects were receiving treatment for myocardial infarction and included 293 patients treated with nitrates (Nitrate group) and 313 patients who were not (Nonitrate group). The former included 186 patients who received intermittent nitrate administration (Intermittent group) and 107 patients who received continuous administration (Continuous group). The mean period of observation was 4.3±1.6 months.
Results There were no cases of rebound angina in the Intermittent group. Cardiac events occurred in one case in the No-nitrate group (0.3%), in 4 cases in the Continuous group (3.7%) and in 2 cases in the Intermittent group (1.1%). The incidence of cardiac events was thus significantly increased in the Continuous group compared to the No-nitrate group (p<0.05; odds ratio 9.06; 95% CI 1.41-58.28). The Intermittent group did not significantly differ from the No-nitrate group in the incidence of cardiac events.
Conclusion It is concluded that intermittent administration of nitrates does not cause rebound angina and is therefore safe. A randomized controlled trial is needed to find the long-term effect on cardiac events.
(Internal Medicine 39: 1020-1026, 2000)

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Methods We evaluated the clinical efficacy of transdermal nitroglycerin (NTG-TTS), a patch application of a nitrate, in the treatment of 27 patients with angina pectoris who had asymptomatic myocardial ischemic (SMI) attacks, using a double-blind cross-over method. Evaluation was made using Holter ECG and patient activity data.
Results In frequency and duration of continuation of SMI episodes, no significant differences were noted between the observation and placebo treatment periods, while the values of both these parameters were decreased significantly in the active drug treatment period compared with those in the observation and placebo treatment periods. Critical heart rate, the heart rate at the initiation of ST-segment depression, was significantly higher during the period of active drug treatment than during the placebo treatment and observation periods. In SMI frequency index, which was determined by adjusting the SMI frequency for the number of steps taken, there were no significant differences between the 3 periods. The SMI time index was significantly lower in the active drug treatment period than in the observation period.
Conclusions These results indicated that the clinical evaluation of the efficacy of anti-anginal drugs against SMI should take into consideration individual patient activity data.
(Internal Medicine 39: 1027-1037, 2000)

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Objective The differences in the clinical findings of patients with acute pulmonary thromboembolism (acute-PTE) and chronic pulmonary thromboembolism (chronic-PTE) were compared, and the association between the clinical findings and prognosis in the two groups was analyzed.
Patients This study included 204 patients with PTE, 139 patients with acute-PTE and 65 patients with chronic-PTE.
Results There were significant differences between acute-PTE and chronic-PTE in the predisposing factors of post operation (19.4 versus 1.5%, p=0.0003), and electrocardiographic signs such as sinus tachycardia (73.7 versus 50.9%, p=0.007), ST-depression (25.3 versus 9.4%, p=0.03), right ventricular hypertrophy (20.0 versus 47.2%, p=0.0007), and right axis deviation (3.2 versus 22.6%, p=0.0003). Thirty-one of the 139 acute-PTE patients died from PTE, as did 17 of the 65 chronic-PTE patients. The prognosis was poor in patients older than 70 years old (p=0.01), with stroke (p=0.008), syncope (p=0.01), shock (p=0.0006), hypocapnia (Paco₂≤25 torr; p=0.0006) and an elevation in total pulmonary resistance (TPR >1, 000 dyne • sec • cm^-5 (p=0.02)) in acute-PTE, and in those with syncope (p=0.03), shock (p=0.008), and right ventricular hypertrophy on electrocardiogram (p=0.03) in chronic-PTE.
Conclusion The results of this study indicate a relationship between the clinical features of patients with acute-PTE and chronic-PTE, and the predictors of in-hospital mortality.
(Internal Medicine 39: 1038-1043, 2000)

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Objective To study the adverse reactions and therapeutic effects of fluoroquinolones to investigate whether they can be used for the treatment of patients with typhoid fever and paratyphoid fever.
Methods The adverse reactions and therapeutic effects of fluoroquinolones were studied retrospectively in patients with typhoid fever and paratyphoid fever.
Patients 58 patients (54 Japanese) with typhoid fever, 42 patients (41 Japanese) with paratyphoid fever, and 1 Japanese patient with both typhoid fever and paratyphoid fever, who were admitted in hospitals in Tokyo, Kawasaki, Yokohama, Kyoto, and Osaka from 1995 to 1998 and treated with fluoroquinolones.
Results Almost 80% of the patients were treated with tosufloxacin (TFLX) and the remaining 20% were treated with norfloxacin, ciprofloxacin, levofloxacin, or sparofloxacin. Side effects (nausea, urticaria, aphthous stomatitis) and elevation of serum amylase were found in 3.6% and 8.3% of patients treated with TFLX, respectively, but these adverse reactions disappeared in all of these cases either with or without a change in the drug used. No adverse reactions were found in patients treated with the other fluoroquinolones. The clinical and bacteriological effects of these drugs were adequate.
Conclusion Though further studies still need to be performed on the fluoroquinolones other than TFLX, we can preliminarily conclude that fluoroquinolones are safe drugs and they can be recommended for the initial therapy of patients with typhoid fever and paratyphoid fever.
(Internal Medicine 39: 1044-1048, 2000)

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Ursodeoxycholic acid (UDCA) treatment-for primary sclerosing cholangitis (PSC) has been considered a rational therapy, though its effectiveness in the clinical course is still open to discussion. In this report, we describe a 22-year-old man with PSC at an early stage, which was associated with ulcerative colitis (UC). He showed progressive strictures of bile ducts over a 1.5-year period in spite of an improvement in the biochemical parameters by UDCA treatment. Therefore, care should be taken in interpreting the effectiveness of UDCA, because the biochemical parameters may not change in parallel with the clinical course of PSC.
(Internal Medicine 39: 1049-1053, 2000)

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A 70-year-old woman with rheumatoid arthritis was admitted to our hospital because of gastric ulcers. Abdominal echography incidentally revealed that the patient's gallbladder was multiseptate, an extremely rare anomaly. The diagnosis was confirmed by endoscopic retrograde cholangiography. This is the 26th case of multiseptate gallbladder in the world literature, but the patient is the oldest yet recorded with this condition. Furthermore, unlike most patients with multiseptate gallbladder, she had no biliary symptoms with concomitant cholelithiasis.
(Internal Medicine 39:1054-1059, 2000)

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Radiation therapy is a powerful method for the control of cancer. The utilization of abdominal or pelvic radiation has been extended, and the incidence of radiation enteritis appears to be increasing. The majority of the induced lesions is in the distal ileum, sigmoid colon, or rectum. Reported here is an unusual case of radiation enteritis which caused a severe sequelae of stricture in the transverse colon as a long-term effect of therapeutic irradiation for uterine cancer, and required a surgical resection.
(Internal Medicine 39:1060-1063, 2000)

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A 24-year-old, nulliparous woman in her 30th week of pregnancy was admitted due to threatened premature delivery. Ritodrin chloride relieved the premature contraction of the uterus but jaundice and drowsiness appeared 7 weeks later. Laboratory data revealed disseminated intravascular coagulation (DIC) with intrahepatic cholestasis, and ultrasound examination showed fatty liver. The patient was diagnosed with acute fatty liver of pregnancy (AFLP). Emergency delivery by Caesarean section was performed at 37 weeks of pregnancy and the liver function and DIC improved immediately. Liver biopsy 13 days after delivery showed nuclear swelling and cytoplasmic ballooning with mild fatty deposition. These findings were relatively compatible with acute AFLP. Higher magnification and electron microscopy revealed intracytoplasmic bacteria and fungus in the residual stage. The bacterial infection could be considered related to AFLP.
(Internal Medicine 39: 1064-1067, 2000)

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A 17-year-old man was admitted to our hospital because of severe acute hepatitis. Serologic studies were negative for A, B, C and G hepatitis viruses. Later, he was found to be positive for transfusion-transmitted virus (TTV) DNA. He was discharged after normalization of liver function tests. Four months after the onset of hepatitis, he was readmitted because of pancytopenia. Bone marrow findings were consistent with aplastic anemia. The anemia responded to steroid therapy. In this case, TTV was probably involved in the development of aplastic anemia.
(Internal Medicine 39: 1068-1070, 2000)

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A 32-year-old man with lung cancer involving pericarditis carcinomatosa underwent pericardiotomy, using an Inoue balloon dilating catheter, to create a non-surgical pericardial window. The procedure was performed from the thoracic wall to the left pleural effusion and parietal pericardium under local anesthesia. The effects of non-surgical pericardial window had been maintained until this patient died from his primary disease. It is concluded that percutaneous balloon pericardiotomy is helpful in the management of massive pericardial effusions particularly in patients with malignancies and poor clinical condition.
(Internal Medicine 39: 1071-1074, 2000)

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We report a 70-year-old woman with hypothyroidism and severe hyponatremia. Her plasma antidiuretic hormone (ADH) level was inappropriately high for her low plasma osmolality. Her low serum sodium level was gradually corrected by water restriction and sodium supplementation prior to the initiation of thyroid hormone replacement. After a diagnosis of Hashimoto's thyroiditis had been made, the patient was treated with levothyroxine. Following this treatment, the patient's serum sodium level increased drastically. It is suggested that the elevated plasma ADH level played an important role in the development of hyponatremia in this case.
(Internal Medicine 39: 1075-1078, 2000)

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We examined the histological changes of the pancreas in a 75-year-old diabetic woman who was treated with sulphonylurea for 10 years and required insulin treatment for the subsequant 10 years. She was positive for anti-glutamic acid decarboxylase antibody (GAD Ab) 20 years after the diagnosis of diabetes. Her autopsied pancreas revealed disfigured islets and no β cells. These changes were indistinguishable from those of the long-sustained type 1 diabetes mellitus, and insulitis was not found. This case suggests that diabetic patients positive for the GAD Ab, even if oral hypoglycemic agents are effective, may develop complete plete β cell destruction over a long period.
(Internal Medicine 39: 1079-1082, 2000)

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In a 40-year-old man, a mediastinal hemangioma was discovered intially as a compression of the esophagus by upper gastrointestinal endoscopy. Furthermore, perirenal hemangioma and inferior vena cava (IVC) malformation were stimultaneously found. Hemangiomas, which occur in the mediastinal and perirenal area, are extremely uncommon and congenital IVC malformation, like the present case, has not been reported. We review the literature of these vascular abnormalities.
(Internal Medicine 39: 1083-1087, 2000)

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The esophagorespiratory fistula is difficult to treat, and the patients' quality of life is generally poor due to suffering from dysphagia and dyspnea. We performed stent therapy in four cases of the esophagorespiratory fistula associated with esophageal cancer. Three of four patients showed improved symptoms, enabling oral liquid or food intake, although one died of dyspnea despite the therapy. The findings suggest that stent therapy is an effective method to close the esophagorespiratory fistula and to improve the patients' quality of life, although it is temporary and not a radical treatment.
(Internal Medicine 39: 1088-1093, 2000)

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A 61-year-old woman who had undergone an operation for thymoma 17 years previously suddenly became dyspneic and showed bilateral pulmonary infiltrates on a chest radiograph. In the bronchoalveolar lavage fluid cells contained characteristic cytomegalic inclusion bodies, as well as cytomegalovirus DNA demonstrated by a polymerase chain reaction. Immunological findings included hypogammaglobulinemia, deficient numbers of circulating B cells, and impaired blast transformation of peripheral blood T cells in response to mitogens in vitro. Considering all of the findings, the patient was diagnosed with Good's syndrome presenting with cytomegalovirus pneumonia.
(Internal Medicine 39: 1094-1096, 2000)

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Amiodarone is a useful drug for the treatment of lifethreatening cardiac arrhythmias. However, amiodarone can induced pulmonary toxicity (APT) and may cause lifethreatening lung damage. APT can be difficult to diagnose, but early diagnosis is important. Here, in a 51-year-old man with APT, the high serum KL-6 level was correlated with the severity of symptoms and chest X-ray findings, and it was inversely correlated with PaO₂ and diffusion capacity for carbon monoxide levels. The findings suggest that the serum KL-6 level may be increased in APT and that therefore it's the determination of serum KL-6 may provide a useful indicator and/or monitoring marker of APT. KL-6 is believed to be produced and secreted by type II pneumocytes. Typical pathological findings of APT include proliferation of type II pneumocytes which may produce KL-6, and result in increased serum KL-6 levels.
(Internal Medicine 39: 1097-1100, 2000)

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We report a case of Crow-Fukase (POEMS) syndrome associated with pulmonary hypertension (PH). In this case, the concentration of vascular endothelial growth factor (VEGF) was extremely high in the serum, and the levels of IL-1β, IL-6, TNF-α, and thiamine, which were thought in past reports to be mediators of PH in Crow-Fukase syndrome, were normal. After prednisolone therapy, PH disappeared with a dramatic decrease in serum VEGF. Our results suggest that VEGF is closely correlated with PH in Crow-Fukase syndrome.
(Internal Medicine 39: 1101-1104, 2000)

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Cheiro-oral syndrome due to a parietal lesion has been reported in conjuction with a brain tumor, infarction and migraine. Only six reports of cheiro-oral syndrome due to a parietal infarction have been reported to date. We treated a 45-year-old woman with cheiro-oral syndrome due to a parietal infarction. Her sensory disturbance was characterized by paresthesia in the lower face and hand on the left side, and severe involvement of stereognosis and graphesthesia in the left hand. The pathogenesis of parietal cheiro-oral syndrome is discussed.
(Internal Medicine 39: 1105-1107, 2000)

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We report a 51-year-old woman with polymyositis accompanied by a high titer of antiacetylcholine receptor antibody. The patient presented with weakness of grip strength followed by rapidly progressive dyspnea, which required mechanical ventilation. She was treated with a glucocorticoid and came off the respirator one week later. Antiacetylcholine receptor antibody activity was elevated in the acute phase and decreased during recovery, although other signs of myasthenia gravis were negative. This patient suggested that in cases of rapidly progressive bulbar palsy and limb muscle weakness, it is necessary to include polymyositis associated with elevated antiacetylcholine receptor antibody activity in the differential diagnosis.
(1108 Internal Medicine 39: 1108-1110, 2000)

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We studied a Japanese patient who developed parkinsonian symptoms over 3 months before the diagnosis of acquired immunodeficiency syndrome. Brain MRI showed multiple lesions with mass effect and ring enhancement in the basal ganglia and subcortical white matter suggesting Toxoplasma infection. Anti-Toxoplasma therapy and highly active antiretroviral therapy for 6 months allowed improvement of parkinsonism, brain MRI findings, and immune system.
(Internal Medicine 39:1111-1114, 2000)

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We report the fourth case of simultaneous macroamylasemia and macrolipasemia. A 39-year-old woman had been treated for systemic lupus erythematosus from 1982 to 1993. She was found to have an unexplained increase in serum amylase and lipase activities since 1996. Immunoprecipitation assay showed that amylase was bound to IgA2-κ and IgA1-κ (IgA2>IgAl), whereas lipase was bound to IgAl-κ. During a follow-up period up to December 1999, the patient did not develop any additional autoimmune or lymphoproliferative disorders. In the situation of atypical clinical features associated with hyperamylasemia and hyperlipasemia, the possibility of macroenzymes should be considered.
(Internal Medicine 39:1115-1118, 2000)

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A 24-year-old-woman with mixed connective tissue disease (MCTD) developed multiple organ failure, disseminated intravascular coagulation (DIG), metabolic acidosis, and respiratory and renal failure resulting from visceral vasospasm, so-called visceral Raynaud's phenomenon. After plasmapheresis, the condition of multiple organ failure was markedly improved. The successful treatment with plasmapheresis was dependent upon the removal of immune complexes in serum and improvement of visceral circulation. Thus plasma exchange is recommended as a possible a treatment for multiple organ damage in MCTD.
(Internal Medicine 39:1119-1122, 2000)

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A 62-year-old man who had a 14-year history of diabetes complained of low-grade fever, general malaise, pain of bilateral femurs and hip girdle, and was admitted to our hospital. The diagnosis of polymyalgia rheumatica (PMR) was made from the clinical symptoms, elevated C-reactive protein and erythrocyte sedimentation rate. Electromyography revealed abnormalities that suggested diabetic peripheral neuropathy. However, the abnormalities were improved after starting treatment with corticosteroids (PSL). After stopping PSL, electric nerve conduction disturbance developed; therefore, it was suggested that peripheral nerve involvement due to PMR was improved by administration of PSL regardless of the existence of diabetic peripheral neuropathy.
(Internal Medicine 39:1123-1127, 2000)

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An 87-year-old woman died of rapidly progressive pneumonia due to Aeromonas hydrophila shortly after a near-drowning event. Autopsy showed necrotizing pneumonia and postmortem cultures of both blood and lung revealed the organism. Fulminant pneumonia should be considered in patients of a near-drowning event.
(Internal Medicine 39:1128-1130, 2000)

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