Non-insulin-dependent diabetes mellitus (NIDDM) is one of the most common metabolic and endocrine disorders in developed countries. NIDDM is a multifactorial disorder in which genetic factors play a complex role in its development. Until recently, little has been known of the genetic determinants of NIDDM. Advances in molecular biological and molecular genetic approaches have now made it possible to begin to identify the diabetes susceptibility genes in NIDDM. This review describes the progress of recent research in the molecular genetic aspects of NIDDM.
(Internal Medicine 35: 347-355, 1996)

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Nitric oxide (NO) is present in the breath exhaled from healthy humans. The location and subtype of the NO synthase responsible for the expired NO are unknown. As dexamethasone inhibits the induction of NO synthase, we evaluated the effect of administering dexamethasone (4 mg/day for 2 days) on the amount of NO exhaled by eight healthy men. The amount of NO showed a significant linear correlation with the duration of exhalation, allowing the rate of NO release to be calculated. The rate of NO release was 0.047±0.023 nmol/s before drug administration. There was no significant change in the release rate at the end of the 2-day administration of drug or at 5 days after cessation. Serum concentrations of interferon-γ and interleukin-1β were unaffected by the administration of dexamethasone. These results suggest that the NO released from the human airway under normal conditions is not generated by the action of inducible NO synthase.
(Internal Medicine 35: 356-361, 1996)

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Although the influence of high dose inhaled steroids on hypothalamo-pituitary-adrenal (HP A) function in patients with asthma has been extensively studied worldwide, there has been limited information on Japanese asthmatics, especially in terms of a prospective analysis of HP A function in the course of time. We analyzed the changes in HPA function using 2 serial short tetracosactrin tests (STT) separated by an interval of one year in 11 Japanese asthmatics who were treated with high dose inhaled steroids alone [beclomethasone dipropionate (BDP); mean dose 982 μg/day] during the period between 2 STTs. Mean values of plasma cortisol before administration of ACTH, maximum cortisol and the rise in cortisol in response to ACTH in the 2 STTs were 7.8, 20.5 and 12.7 μg/dl for the 1st test, and 8.9, 23.6 and 14.7 μg/dl for the 2nd test, respectively. Overall, there was no significant change in the course of time in each of these 3 values. Although the results of the 1st STT proved to be abnormal in 3 patients who had been receiving systemic steroids before their 1st STT, they improved uniformly in their 2nd STT. In the remaining 8 patients, who had never received systemic steroids, 4 patients showed improvements while the other 4 showed deterioration in HPA function in their serial STTs over the course of time. The dose of BDP was 800 μg/day in the former 4 patients, while it was 1, 200 μg/day in the latter 4. Furthermore, only one patient, in whom BDP had been increased from 800 μg/day to 1, 200 μg/day between the 2 tests, developed an abnormal response in the 2nd STT. On the other hand, one patient whose BDP dose was increased from 800 μg/day to 1, 600 μg/day showed an improvement in HPA function in the 2nd test. These results indicate that the threshold dose of BDP which may cause HPA suppression in Japanese asthmatics lies between 800-1, 200 μg/day, although there is a large inter-individual variation in terms of such doses.
(Internal Medicine 35: 362-366, 1996)

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Anti-DNA antibody, especially high-affinity anti-DNA antibody (ADNA), is thought to have an important role in the pathogenesis of lupus nephritis. In this study, ADNA which binds to double-stranded DNA under a high concentration of sodium chloride was measured in patients who had received immunoadsorption (IA) therapy with a dextran-sulfate column. Titers of high-affinity ADNA in the cases with renal dysfunction tended to be higher than in those without renal dysfunction. The change in the titer of high-affinity ADNA paralleled the clinical course. These findings suggest that measurement of high-affinity ADNA is useful for follow-up of the clinical course of patients who have undergone IA therapy.
(Internal Medicine 35: 367-372, 1996)

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We report a case of obstructive jaundice caused by a large hepatic cyst which was successfully treated by percutaneous drainage and instillation of minocycline hydrochloride. An 88-year-old man presented with obstructive jaundice and upper abdominal fullness. Abdominal CT and ultrasonography revealed a large hepatic cyst and intrahepatic bile duct dilatation. After the percutaneous drainage of the cyst, minocycline hydrochloride was instilled as a sclerosant via a catheter into the cyst cavity. Liver enzyme levels returned to normal and the jaundice disappeared. Nine months after the treatment, the large cyst and dilatation of the intrahepatic bile duct had disappeared. The patient has remained healthy without symptoms.
(Internal Medicine 35: 373-375, 1996)

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We report a 67-year-old male patient with a known history of sarcoidosis in remission who had recurrent sarcoidosis following a five-month administration of interferon alpha (IFN-α) for chronic hepatitis C. He developed bilateral swelling of the parotid glands and bilateral diffuse reticulonodular pulmonary parenchymal opacities on chest roentgenograms. Serum angiotensin converting enzyme (ACE) levels and soluble IL-2 receptor levels were high and a transbronchial lung biopsy revealed noncaseating granulomas. The abnormalities on both laboratory data and chest roentgenograms were resolved after administration of oral prednisolone.
(Internal Medicine 35: 376-379, 1996)

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A 79-year-old male, admitted because of severe dyspnea on exertion, showed echocardiographic findings of a large tumor in the dilated right ventricle. The right ventricular outflow tract was nearly occluded by the tumor mass, and the mass was attached to the interventricular septum by a pedicle. The tumor removal operation was successful. The size of the tumor was 40 mm × 90 mm, and the weight 70 g. Microscopic findings showed typical myxomatous tissue with high cellularity, and no malignancy was observed. This is the oldest reported patient with right ventricular myxoma which was cured by operation.
(Internal Medicine 35: 380-382, 1996)

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Marked arteriosclerosis localized to the descending thoracic and abdominal aorta was observed in a 19-year-old girl with interruption of the aorta (type A) and a ventricular septal defect. She died from rupture of saccular aneurysm of the pulmonary trunk. The descending thoracic and abdominal aorta showed marked thrombogenic, lamellar thickening of the intima. In contrast, no significant arteriosclerosis was detected in the ascending aorta. Several factors including diffuse sludging of blood, mural thrombosis and hemodynamic changes, in close association with polycythemia, are hypothesized to be causative factors in the development of this lesion.
(Internal Medicine 35: 383-387, 1996)

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A 41-year-old woman and her 2 sons had Type V hyperlipoproteinemia. Interestingly, the patient and her younger son had higher levels of remnant-like particle (RLP)-cholesterol than her elder son; the former two had apolipoprotein E phenotype E3/E4, while the latter had E3/E3. Hyper-RLP-ch levels may be associated with the apolipoprotein E4 phenotype. Moreover, after administration of bezafibrate, the patient's triglyceride, RLP-cholesterol and RLP-triglyceride levels decreased markedly and the chylomicron fraction disappeared. Further studies may be necessary to determine if hypertriglyceridemic patients with or without apolipoprotein E4 show a greater reduction in serum TG levels with lipid-lowering agents.
(Internal Medicine 35: 388-391, 1996)

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We describe herein a case of parathyroid carcinoma accompanied with metastatic calcification identified by technetium-99m methylene diphosphonate (Tc-99m MDP) scintigraphy in the lungs, kidneys and stomach. Parathyroid carcinoma remains a rare disorder despite the increased prevalence of primary hyperparathyroidism. Metastatic calcification is noted infrequently even in primary hyperparathyroidism and it may cause respiratory failure. Tc-99m MDP scintigraphy three months after surgery showed a complete disappearance of Tc-99m uptake in the stomach and an obvious reduction in the kidneys but no significant change in the lungs, indicating metastatic calcification can be reversibly reabsorbed. This case indicates that the adequate excision of parathyroid carcinoma as well as the early detection of metastatic calcification by Tc-99m MDP are obligatory.
(Internal Medicine 35: 392-395, 1996)

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We report here a rare case of 47 XXY/46 XY mosaic Klinefelter's syndrome associated with multiple endocrine disorders. A 35-year-old male admitted for the evaluation of renal dysfunction and recurrent bone fractures was diagnosed as having Klinefelter's syndrome by endocrinological examinations and sex chromosome analysis. He has suffered from diabetes mellitus for more than ten years. The serum FSH and LH levels were high together with low free testosterone and estradiol levels. There was a discrepancy between basal serum GH and somatomedin-C levels. On admission, thyroid function revealed thyrotoxicosis with low radioactive iodine uptake and negative thyroid autoantibodies. During hospitalization, serum FT₃ and FT₄ levels were gradually decreased and serum TSH levels became elevated, leading to the diagnosis of subacute thyroiditis. Serum ACTH levels showed high basal levels with delayed, exaggerated responses to insulin-induced hypoglycemia. Rapid ACTH test (^1-24ACTH 0.25 mg) showed low cortisol responses and many of the adrenocortical steroids in plasma and urine were low or low normal. Furthermore, bone mineral density (BMD) by DEXA showed marked osteoporosis. Possible mechanisms underlying these varied endocrine disorders remain to be elucidated.
(Internal Medicine 35: 396-402, 1996)

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A 64-year-old female with McArdle's disease and non-insulin-dependent diabetes mellitus (NIDDM) is reported. She had none of the characteristic symptoms of McArdle's disease such as muscle cramps but her serum creatine kinase level was elevated. Muscle biopsy with negative muscle phosphorylase staining showed McArdle's disease. Modified forearm ischemic exercise test was done at two conditions; fasting and two hours after a meal. When fasting, the level of lactic acid did not elevate after exercise. After a meal, however, the serum lactic acid level rose with the elevation of plasma glucose and IRI. Thus, we suggested that high plasma glucose and insulin due to NIDDM may induce blood-borne glucose uptake with exercise.
(Internal Medicine 35: 403-406, 1996)

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A 63-year-old male was admitted to our hospital because of a coin lesion in the intrapulmonary bronchus. The patient had no symptoms such as cough or sputum. Imaging studies showed that the tumor was present in the S10b, c of the right lung. There was no metastasis or salivary gland tumor. Transbronchial tumor biopsy revealed a primary adenoid cystic carcinoma. The patient underwent a lobectomy, and has been well since the operation. This is an uncommon case of primary adenoid cystic carcinoma arising from the intrapulmonary bronchus.
(Internal Medicine 35: 407-409, 1996)

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A 69-year-old alcoholic man with pneumonia and sepsis due to Aeromonas hydrophila is presented. He died of suffocation by a copious amount of hemoptysis six hours after his first symptoms of abdominal pain, diarrhea and dyspnea. Aeromonas hydrophila was isolated from blood and bronchial secretion. A fulminant form of pneumonia could develop in patients with predisposing underlying conditions such as alcoholism with chronic hepatitis and diabetes mellitus. Aeromonas hydrophila pneumonia may be characterized by hemoptysis and rapid clinical deterioration with a high mortality rate.
(Internal Medicine 35: 410-412, 1996)

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A female patient with idiopathic interstitial pneumonia (IIP) was admitted again 40 months after the diagnosis due to progression of clinical findings and increased activity of serum lactic dehydrogenase (LDH). Analysis of LDH isoenzyme disclosed a broad band between LDH4 and LDH5. Gel filtration and immunoelectrophoresis showed that immunoglobulin (Ig) G (κ type) bound the LDH. With prednisolone and azathioprine, her symptoms and radiological findings improved concomitant with a decrease in the serum LDH activity. The LDH-IgGκ complex disappeared in the circulation 14 months after initiation of the therapy. We report circulating LDH-Ig complex in a patient with IIP, which may be related to the disease progression of IIP.
(Internal Medicine 35: 413-415, 1996)

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We report a case of sequential occurrence of hepatocellular carcinoma (HCC) following Waldenström's macroglobulinemia (WM) in the course of chronic liver disease. The diagnosis of WM was made in a 55-year-old man with a history of alcohol abuse. He developed HCC in the course of WM, and hepatitis C virus (HC V) infection was detected by enzyme-linked immunosorbent assay. Recent reports suggest that persistent HCV infection is related to the pathogenesis of B cell malignancy. Chronic liver disease may be associated with the pathogenesis of the double malignancies in the present case.
(Internal Medicine 35: 416-418, 1996)

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A 39-year-old male was admitted with fever, systemic lymph node swelling, liver dysfunction and mild splenomegaly. Liver biopsy specimen showed histiocytic aggregation in portal areas. These histiocytes were closely packed with granules, dyed sea-blue with May-Giemsa staining. Further microscopical examination of lymph nodes, gastro-intestinal tract and bone marrow also revealed the accumulation of sea-blue histiocytes. Activities of lipid metabolic enzymes were normal and hematopoietic diseases which are sometimes accompanied by secondary sea-blue histiocytosis were ruled out. We diagnosed this case as syndrome of the sea-blue histiocyte.
(Internal Medicine 35: 419-421, 1996)

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A case of extramedullary plasmacytoma (EMP) of the jejunum, an uncommon neoplasia, is reported. A 56-year-old Japanese woman who experienced intermittent upper abdominal pain and weight loss had a large movable mass in the upper abdomen. The mass was hypervascular in an angiographic study and positive for gallium-67 citrate scintigraphy. Immunoelectrophoresis showed the presence of an M-component of immunoglobulin (Ig) A-λ in the serum. It was identified as an EMP immunohistochemically positive for IgA-λ, . This M-component disappeared after resection and chemotherapy. The clinical features of this rare neoplastic disorder are discussed.
(Internal Medicine 35: 422-426, 1996)

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Hypoxic encephalopathy and osmotic demyelination are independent clinical entities. We describe a rare case with these two complications as demonstrated by magnetic resonance imaging (MRI). A 58-year-old woman had adrenal crises twice a decade due to Sheehan syndrome. At the second crisis, hyponatremia was remarkable with consciousness disturbance which was rapidly corrected by intraveneous administration of glucocorticoid and hypertonic saline. The maneuver improved consciousness disturbance, but resulted in hypokalemic ventricular fibrillation with circulatory failure. After the normalization of the circulation, however, her consciousness level deteriorated again. Repeated brain MRI revealed acute and chronic phases of cortical laminar necrosis and central pontine myelinolysis.
(Internal Medicine 35: 427-431, 1996)

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