Diffuse Lewy body disease (DLBD )has been studied from various viewpoints, and although clinical diagnostic criteria for DLBD have been proposed, the diagnosis remains difficult. It has been reported that DLBD is the second most frequent degenerative dementia amongthe elderly, following Alzheimer-type dementia. Many DLBD cases, however, are clinically misdiagnosed. Therefore, the search for diagnostic markers for DLBD must continue. Very recently, "dementia with Lewy bodies" (DLB) was proposed as a generic term including DLBD and similar disorders. Cortical Lewy bodies are the most important pathological marker for diagnosis of DLB.At this time, however, the mechanism of cortical Lewy body formation is yet to be disclosed.
(Internal Medicine 37: 6-10, 1998)

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Cirrhotic patients frequently manifest neutropenia and are predisposed to bacterial infections. We examined neutrophil apoptosis to determine if neutrophil survival in cirrhotic patients is shortened. Neutrophils isolated from 10 cirrhotic patients and 10 healthy volunteers were cultured for 24 hours. The time course of neutrophil viability was assessed by the trypan blue dye exclusion test and apoptosis was determined morphologically by light and electron microscopy. Apoptotic cells were also confirmed by terminal deoxynucleotidyl transferase-mediated deoxyuridine triphosphate nick and labeling (TUNEL)and DNA gel electrophoresis. Fas expression of neutrophils was examined by flow cytometry. Viabilities were significantly decreased in liver cirrhosis (p<0.0001). Neutrophils from cirrhotic patients exhibited significantly greater apoptosis. Fas expression of neutrophils was significantly reduced for cirrhotic patients (p=0.0001). Neutrophils from cirrhotic patients exhibited markedly accelerated apoptosis in vitro. Shortening of neutrophil survival via apoptosis mayexplain in part the mechanism of neutropenia in cirrhotic patients.
(Internal Medicine 37: 11-17, 1998)

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Based on an anonymous questionnaire obtained from 102 Japanese in-patients at a rural Japanese hospital who underwent coronary angiography, the patients understanding of information about the procedure, their perception of consent and their attitude toward it were investigated. The patients were able to recall 63.8 %of the contents of the information. There was a statistically significant correlation between the patients' educational status and the recall test score of the information. Older (65 years old and above) patients had a tendency to entrust decision making to their physicians. Only 19.6 %of the patients made the decision to undergo a coronary angiography by themselves. After being informed of the risks 40.2% of the patients felt more anxious. In conclusion, our patients could not recall the contents of the information sufficiently. There still existed a tendency to entrust important decisions to the physician especially in older patients. The patients had a tendency not to face difficult problems solely by themselves but with the support of family members.
(Internal Medicine 37: 18-20, 1998)

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Twelve patients with stable angina and 12 patients with unstable angina underwent rest iodine-123-β-methyl-iodophenyl-pentadecanoic acid (BMIPP), dipyridamole stress thallium scintigraphy and delayed thallium scintigraphy. In stable angina, sensitivity for detecting ischemic segments was higher in dipyridamole thallium (74 % ) than rest BMIPP (48 % ) images (p<0.05), but there was no significant difference between the 2 images in unstable angina. In unstable angina, the incidence of segments with higher defect scores on BMIPP images than on delayed thallium images and the opposite pattern was 27 and 5 (p<0.01). In stable angina, there was no difference. The mean defect score on BMIPP(6.3 ± 5.6) was higher than that on delayed thallium scintigraphy (2.9 ± 2.7) and it was almost the same as on the initial dipyridamole stress test (6.5 ± 5.2). In conclusion, BMIPP scintigraphy is safer and may be as useful in detecting myocardial ischemia in patients with unstable angina as thallium scintigraphy.
(Internal Medicine 37: 21-26, 1998)

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To survey the prognoses of interferon-alpha (IFNα)-induced thyroid dysfunction, a total of 100 patients (49 males and 51 females) with biopsy-proven chronic active hepatitis C were studied. Either during or after IFNα therapy, 29 patients (33.7 %) revealed suppression/elevation of thyroid stimulating hormone (TSH) or both, transient thyrotoxicosis (TSH less than 0.1 μU/ml) or transient hypothyroidism (TSH 5.0-190.95 μU/ml). However, the thyroid function normalized without supplementation of the thyroid hormone in the follow-up period. In the same period, one of the 14 control patients (7.1 %) developed thyroid dysfunction. Thyroid abnormalities developed significantly more in patients with IFNα therapy than in those without IFNα therapy. The findings suggest that the occult autoimmune disorder becomes overt with IFNαtreatment in patients with pre-existent autoimmune thyroid disease. IFNα-induced thyroid dysfunction is transient, reversible and self-limited. It is not necessary to discontinue IFNα therapy when thyroid dysfunction develops.
(Internal Medicine 37: 27-31, 1998)

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To clarify the clinical significance of elevation of serum aminotransferase levels in anorexia nervosa, we analyzed the relationships of serum aminotransferase levels to other serum biochemistry and physical conditions before and during refeeding therapy in 101 patients with anorexia nervosa. Before refeeding therapy, body mass index (BMI) was distributed from 9.9 to 16.4 kg/m² (13.2 ± 1.3, mean ± SD), and 29 patients (28.7 %) showed abnormally high aminotransferase levels. Among 17 patients with a BMI of less than 12 kg/m², the aminotransferase level was abnormally high in 13 patients (76 %). Incidence and severity of serum aminotransferase elevation were greater in the patients with lower BMI. The groups with high serum aminotransferase levels had a lower body temperature, lower pulse rate, and higher incidence of other biochemical abnormalities than the group with normal serum aminotransferase levels. These findings indicate that aminotransferase elevation develops at a high incidence in anorectic patients with a critically life-threatening condition, and it is a sign of multiorgan failure requiring urgent calorie repletion. This type of aminotransferase elevation is to be distinguished from refeeding-induced aminotransferase elevation.
(Internal Medicine 37: 32-39, 1998)

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The characteristics of urate metabolism in renal hypouricemic patients with hematuria were studied to clarify the risk factors for hematuria in patients with renal hypouricemia. In 16 Japanese patients with isolated renal hypouricemia, urate metabolism was measured using the urate clearance study and the subtype of renal hypouricemia [defective presecretory reabsorption (Pre), defective postsecretory reabsorption (Post), enhanced tubular secretion (Secretion) and defective presecretory and postsecretory reabsorption (Pre&Post)] were determined by the pharmacological tests. Hematuria was seen in 7 out of the 16 patients (44%), all of whom were females (58%). Serum urate and urinary urate concentrations were significantly higher in the group with hematuria (Sur = 1.76 ± 0.31 mg/dl and Uur/Ucr = 0.75 ± 0.12: p<0.05) than in the group without hematuria (Sur = 1.44 ± 0.46 mg/dl and Uur/Ucr = 0.56 ± 0.04), although there was no difference in the urate excretion rate between the two groups. Hematuria was more likely to be accompanied by Post (75 % ) and Secretion (75 %), which showed significantly higher urinary urate concentration (Uur/Ucr = 0.75 ± 0.1 and 0.69 ± 0.13, respectively) than by Pre (25%) and Pre&Post (0%), which showed lower urinary urate concentration (0.61 ± 0.06 and 0.62 ± 0.05, respectively). The risk factors for hematuria in patients with renal hypouricemia are the elevation of urinary urate concentration and the subtypes of Post and Secretion.
(Internal Medicine 37: 40-46, 1998)

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Intermittent intestinal bleeding persisted in a 77-year-old male, who had undergone grafting for abdominal aortic aneurysm. Each attack lasted for a few weeks and spontaneously resolved. Only a minute abnormality was found in the third portion of the duodenum; barium studies showed a segmental narrowing, but endoscopy disclosed only a small erosion in that portion. Massive and fatal gastrointestinal hemorrhage broke out 6 months after the onset of bleeding. Autopsy revealed an adhesion area with a small fistula formation between the duodenum and aorta. Evenslight endoscopic findings should be considered suggestive of aortoenteric fistula in patients after aortic surgery.
(Internal Medicine 37: 47-50, 1998)

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A rare case of peliosis hepatis associated with idiopathic restrictive cardiomyopathy is reported. A 75-year-old man was admitted for evaluation of marked edema and jaundice. Serum total bilirubin was elevated above 20 mg/dl. The liver biopsy under laparoscopy revealed marked sinusoidal dilatation and retention of red blood cells, which was consistent with a diagnosis of peliosis hepatis. Cardiac catheterization revealed right ventricular filling disturbance without specific findings on endomyocardial biopsy, suggesting idiopathic restrictive cardiomyopathy. The level of serum total bilirubin decreased in association with improvement of edema after drip infusion of furosemide therapy.
(Internal Medicine 37: 51-55, 1998)

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Coronary ostial stenosis in otherwise normal coronary vessels, is a rare complication of syphilitic aortitis, and most of the cases are found at autopsy. We report here a case in which bilateral coronary ostial stenosis and aortic regurgitation due to syphilitic aortitis was diagnosed; coronary artery bypass graft and aortic valve replacement were then performed. The macroscopic finding and the histopathological examination of the ascending aorta revealed the presence of syphilitic aortitis. It is important to note that syphilis is one of the causes of coronary ostial stenosis in young adults associated with aortic regurgitation.
(Internal Medicine 37: 56-59, 1998)

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During early embryogenesis, anastomoses are formed between the carotid artery and the basilar or the vertebral artery, and subsequently, these anastomoses regress. In some cases, these anastomoses remain as persistent carotid-basilar or carotid-vertebral anastomoses. Atrial septal defect (ASD), a communication between the atria at the septal level, is a congenital heart anomaly. Intrahepatic venous shunts between the portal and hepatic veins are very rare and only some are considered congenital. We present the first case report of a patient with an ASD, a persistent primitive hypoglossal artery, and congenital portahepatic shunts.
(Internal Medicine 37: 60-64, 1998)

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We could detect recruitable backward collateral flow in systole and diastole by using a Doppler guide wire during coronary artery spasm in a 42-year-old female among 20 patients with documented variant angina. The electrocardiogram (ECG) showed no ST-segment deviation, although she had chest pain and her coronary angiography showed a totally occluded right coronary artery. After administration of isosorbide dinitrate (ISDN) the collateral flow disappeared and the flow pattern recovered to normal. The good collateral flow pattern in patient with totally occluded coronary artery due to spasm was indicated to relieve myocardial ischemia, even when the patient felt chest pain.
(Internal Medicine 37: 65-68, 1998)

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A 30-year-old woman was admitted to our hospital because of recurrent ureterolithiasis. She was suspected of having adenine phosphoribosyltransferase (APRT) deficiency based on the presence of 2, 8-dihydroxyadenine (DHA) crystals in her urinary sediment, infrared spectrophotometric analysis of the excreted stone, and then the definitive diagnosis by gene analysis. A pedigree study indicated only a slight possibility of this disease in the family. From these results, we consider that urinary sediment and stone analysis should be used for screening while gene analysis should be employedfor definitive diagnosis of APRT deficiency, so that the complications of this condition can be prevented.
(Internal Medicine 37: 69-72, 1998)

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A 72-year-old man who suffered from recurrent fever was found to have enlarged bilateral adrenal glands on computed tomographic scanning, combined with subclinical adrenal insufficiency. Based on the pathology of bone marrow aspiration, he was diagnosed to have an angiotropic large cell lymphoma (ALCL). Soon after the treatment with a combination chemotherapy, he achieved complete remission of ALCL and size and function of the adrenal glands were apparently normalized. ALCL should be included in the list of differential diagnoses of non-functioning bilateral adrenal swelling with non-specific symptoms such as fever.
(Internal Medicine 37: 73-76, 1998)

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Two brothers with classical xanthinuria who lacked xanthine dehydrogenase activity were encountered. Their hypouricemia was caused by underproduction of uric acid. In their duodenal mucosa, no xanthine dehydrogenase (oxidase) activity was detected. The patients had no symptoms except for duodenal ulcer in one case. The conversion of allopurinol to oxipurinol during an allopurinol loading test for determining the type of classical xanthinuria revealed that the patients had classical type 1 xanthinuria, because aldehyde oxidase activity was present. Furthermore, the allopurinol loading test was conducted to determine the optimal examination times and specimens required for this test.
(Internal Medicine 37: 77-82, 1998)

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A 16-year-old girl with renal failure was transferred to our hospital for an extensive renal examination. Computerized tomography and ultrasonography showed multiple small cysts throughout the medulla of both kidneys. Histological findings revealed tubular atrophy and dilatation and marked periglomerular fibrosis, all of which were compatible with nephronophthisis. Her development and growth were normal. A prior urinalysis, as well as her symptoms and family history were not helpful for making a diagnosis. As early diagnosis of nephronophthisis is difficult in some cases, more detailed screening is needed for children and adolescents.
(Internal Medicine 37: 83-85, 1998)

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Amass of 8 cm in diameter was revealed in the right upper lung field of a 46-year-old female patient. The chest X-ray film taken one year previously revealed only a linear shadow in the same position, which was thought to be a vacant cyst. The levels of carbohydrate antigen (CA) 19-9 in cyst fluid and serum were elevated, at 410, 000 and 130 U/ml, respectively. After surgical resection, serum CA19-9 returned to normal. Pathologically, the cyst wall was lined with bronchial epithelium with no evidence of malignancy. Immunohistochemical study revealed CA19-9 positivity in the bronchial epithelium of the cyst wall.
(Internal Medicine 37: 86-90, 1998)

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A 21-year-old man with bronchial asthma who suffered from productive cough; his chest X-rays and computed tomographic (CT) scans revealed central atelectasis and pulmonary infiltrates with paratracheal and hilar lymphadenopathy. The serum CA19-9 level was elevated. He was suspected to have malignant neoplasms on admission, but he was diagnosed with allergic bronchopulmonary aspergillosis (ABPA) by Rosenberg's criteria. After steroid therapy, his symptoms and radiographic findings improved and the serum CA19-9 level decreased. ABPA should be considered in the differential diagnosis of asthmatic patients with or without lymphadenopathy and an elevated serum CA19-9 level.
(Internal Medicine 37: 91-93, 1998)

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A 43-year-old woman with rheumatoid arthritis (RA), renal amyloidosis and crescentic glomerulonephritis had severe abdominal pain, melena and progressive renal failure. Autopsy findings revealed vasculitis of small and middle size of vessels and there was a deposition of amyloid in the small intestines. Although there were no findings of vasculitis in the kidney, amyloid deposition was noticed and 70-80% of glomeruli showed a crescentic formation. No immunological abnormality was found in glomeruli. Although the immunological mechanisms of crescentic glomerulonephritis were not necessarily eliminated, amyloid deposition may play a role in crescent formation.
(Internal Medicine 37: 94-97, 1998)

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A 43-year-old patient with rice-induced bronchial asthma was first admitted to our hospital for developing bronchial asthma induced by mite and house dust. At that time, there was no sign of allergic response to rice. Two years later, he was readmitted in an emergency state, with a severe attack of bronchial asthma. This time, however, the result of immunoglobulin E (IgE)-radioallergosorbent test (RAST) for rice was positive, with those to mite and house dust were unexpectedly negative. The causative factors and pathological mechanisms of various kinds of food allergies still remain unknown. The patient is now followed up in an ambulatory setting, and has been eating hypoallergenic rice with no bronchial asthma-induced attack. The present case, being an adult patient with severe grain-induced bronchial asthma previously allergic to mite and house dust, seems to be rather rare in the literature.
(Internal Medicine 37: 98-101, 1998)

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