We analyzed the Clinicopathological features and therapy in 19 patients with kidney disease accompanied by hepatitis C viral infection, including 12 patients with mesangial proliferative glomerulonephritis (including eight with IgA nephropathy), six with membranoproliferative glomerulonephritis (MPGN), and one with membranous nephropathy. Persistent hematuria and/or proteinuria (10 patients) was the most common finding, followed by nephrotic syndrome (8 patients). Cryoglobulinemia was detected in six of 19 patients examined (four of six patients with MPGN). Analysis of hepatitis C virus (HCV)-RNA genotype in 13 patients revealed that nine of them had type II genotype. All four patients with MPGN, who had serum positive for HCV-RNA, had type II genotype. Five patients were treated with interferon-α (IFN-α) without a demonstrable effect on renal impairment, whereas five of 11 patients treated with steroids showed improvement of the renal impairment. During the course of steroid therapy, the serum titer of HCV-RNA decreased in 5 of 7 patients. These observations suggest that HCV infection may be associated with several forms of glomerulonephritis. Type IIHG V-RNA may have a strong association with MPGN in Japan. Steroid therapy is not contraindicated in patients with HCV-associated nephropathy if they are resistant to IFN-α treatment.
(Internal Medicine 35:529-533, 1996)

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Thirty-eight patients with late sequelae of pulmonary tuberculosis (TB seq.) were studied to clarify the characteristics of sleep desaturation in comparison with 40 patients with chronic obstructive pulmonary disease (COPD). While awake, the TB seq. group had a lower % VC and a higher PaCO₂. In both groups, the sleep lowest SaO₂ was positively correlated with the awake SaO₂. The regression line between the sleep lowest SaO₂ and the awake baseline SaO₂ in the TB seq. group was located below that in the COPD group. Awake PaCO₂ was negatively related to the sleep lowest SaO₂ only in the TB seq. group. These results indicate that the sleep lowest SaO₂ values were lower in TB seq. than in COPD patients with the same levels of SaO₂ while awake. Sleep studies are necessary to reveal the indication for nocturnal oxygen therapy in TB seq. patients, especially when they are hypercapnic in spite of their good awake oxygenation.
(Internal Medicine 35: 534-539, 1996)

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During a 24-year period, seven patients with leukemia undergoing cytotoxic or immunosuppressive therapy developed pulmonary mucormycosis in our hematology ward. The autopsy incidence in patients with acute leukemia was 2.1%, with no significant rise during the last decade. Most cases occurred in early autumn. Two patients diagnosed premortem were successfully treated with antifungal drugs, whereas five patients diagnosed postmortem died within three weeks following radiographic identification. Persistent fever despite antibiotic coverage was the first clinical presentation, followed mostly by the radiographic appearance of rounded densities, subsequently progressing to hemorrhagic infarction with hemoptysis and/or pleuritic pain. Crescentic cavitation developed when hematologic remission was achieved. Thus, certain radiographic patterns with or without pulmonary infarct syndrome in the proper clinical setting should arouse suspicion of mucormycosis, providing the opportunity for early diagnosis and adequate treatment.
(Internal Medicine 35: 540-544, 1996)

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A rare case of functioning oxyphil parathyroid adenoma associated with primary hyperparathyroidism and marked hungry bone syndrome was revealed in a 29-year-old man with hypercalcemia and elevated circulating parathyroid hormone (PTH) level. A large parathyroid tumor weighing 8.4 g was resected and proved to be an oxyphil adenoma. Hypocalcemia was sustained after the operation, despite intensive calcium supplementation. During the postoperative 8 months, bone mineral density at the lumbar spine increased dramatically from 0.892 g/cm² to 1.244 g/cm², and whole body bone mineral content increased from 1, 913.4 g to 2, 419.2 g. This case gives insight to the reversibility of bone loss in this disorder.
(Internal Medicine 35: 545-549, 1996)

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We report herein the fifth family of hereditary deficiency of lactate dehydrogenase (LDH) H-subunit with an autosomal recessive inheritance including two cases of complete deficiency. Their LDH activities were low both in the serum and in the red blood cells (RBC). Electrophoretic analysis revealed that the patients with the complete deficiency had only the LDHs isozyme. The complete deficiency was associated with marked elevation of fructose-l, 6-diphosphate (FDP) and dihydroxyacetonephosphate (DHAP) and a less marked rise in glyceraldehyde-3-phosphate (GA3P) among glycolytic intermediates in the RBC. Furthermore, hemolysis was observed in the present cases, but this finding was not included in the other reports.
(Internal Medicine 35: 550-554, 1996)

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A 40-year-old man with Addison's disease due to adrenal tuberculosis retained high levels of adrenocorticotropic hormone (ACTH) after conventional hydrocortisone replacement. Plasma ACTH levels were completely suppressed by usual replacement with hydrocortisone (20 mg at 8:00 and 10 mg at 21:00) but rebounded to abnormally high levels the following morning. Administration of 2 mg or 8 mg of dexamethasone suppressed ACTH and cortisol. Magnetic resonance imaging of the brain showed a low-intensity lesion of the pituitary gland. Pituitary hyperplasia or microadenoma with preserved regulation of ACTH was considered to be the cause of the high plasma ACTH levels. The combination of hydrocortisone and dexamethasone reduced plasma ACTH levels.
(Internal Medicine 35: 555-559, 1996)

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A 22-year-old woman with anorexia nervosa showed recurrent hypoglycemic coma and cerebral hemorrhage (Weber's syndrome). She also showed marked liver dysfunction. However, the liver damage was unlikely the main cause of these complications, because liver function tests were improved, and platelet count and prothrombin time were normal when cerebral bleeding occurred. These complications could be induced by her severe malnutrition. Although she rejected any nutritional support before cerebral hemorrhage, she began to accept glucose infusion after the episode, and psychiatric and nutritional states were apparently improved. Cerebral hemorrhage was a turning point in her disease.
(Internal Medicine 35: 560-563, 1996)

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We encountered a case of familial juvenile gouty nephropathy (FJGN) with an autosomal dominant transmission pattern. Hyperuricemia in the propositus was caused by renal underexcretion of urate although his erythrocyte purine enzyme was normal. A renal biopsy specimen from the propositus showed interstitial fibrosis with tubular atrophy. On pyrazinamide and probenecid tests, the tubular secretion of urate selectively decreased without changes in either presecretory or postsecretory reabsorption of urate when his renal function was normal. Probenecid increased the urinary urate excretion and Cur/Ccr. The serum urate concentration was poorly controlled by allopurinol. When his renal function deteriorated, the uricosuric effects of both probenecid and benzbromarone were attenuated. However, the combined administration of probenecid with allopurinol decreased the serum urate concentration. These data suggest that the tubular secretion of urate is selectively impaired in FJGN and at the stage of renal failure, the combination of an uricosuric agent with allopurinol might be effective in treating hyperuricemia in FJGN.
(Internal Medicine 35: 564-568, 1996)

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A 65-year-old woman with renovascular hypertension and massive proteinuria is reported. Her urinary protein ranged from 3 to 5 g/day. Serum potassium was 2.9 mEq/ι. Plasma renin activity and plasma aldosterone concentration were very high. A CT scan disclosed atrophy of the left kidney. Renogram showed a non-functioning pattern of the left kidney. An abdominal aortogram showed complete occlusion of the left renal artery at its ostium. After left nephrectomy, her high blood pressure and urinary protein were normalized. It seemed that the massive proteinuria was mainly due to some endocrinological effect of the left kidney.
(Internal Medicine 35: 569-573, 1996)

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Both ulcerative colitis and Takayasu's arteritis are thought to be organ-specific immunemediated inflammatory diseases. We present the rare case of a 23-year-old woman with a 4-year history of ulcerative colitis who developed Takayasu's arteritis one month after giving birth. She was found to carry the human leukocyte antigens (HLA)-B52 and DR2, which were previously noted to be associated with these inflammatory conditions in the Japanese population. The pathogenic relevance of this haplotype to the concomitant development of these two conditions is discussed.
(Internal Medicine 35: 574-578, 1996)

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We report an unusual case of sarcoidosis associated with bilateral hydronephrosis. The patient was a 53-year-old Japanese woman who presented with dysuria and urinary incontinence. Computed tomography of the abdomen showed bilateral hydronephrosis caused by a retroperitoneal mass, surrounded by enlarged retroperitoneal lymph nodes. Histological examination of the mass demonstrated noncaseating epithelioid cell granulomas involving the retroperitoneal lymph nodes. Corticosteroid therapy led to complete resolution of the retroperitoneal mass and hydronephrosis. This case emphasizes that sarcoidosis should be included in the differential diagnosis of a retroperitoneal mass.
(Internal Medicine 35: 579-582, 1996)

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We describe a patient with progressive systemic sclerosis who developed ischemic colitis and scleroderma renal crisis following steroid pulse therapy. The possible pathogenic mechanisms of ischemic colitis and scleroderma renal crisis development are discussed. We conclude that the administration of steroids in high doses, especially via steroid pulse therapy, should be undertaken with caution for progressive systemic sclerosis patients.
(Internal Medicine 35: 583-586, 1996)

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A 20-year-old man developed fever and urinary abnormalities with positive antinuclear antibody (ANA) two months after the start of ethosuximide. A renal biopsy showed mild mesangial cell proliferation and cellular crescents predominantly occupying a vascular pole in 7 out of 80 (8.8%) glomeruli. In the arteriole, mainly in close proximity to the glomerulus, proliferation of smooth muscle cells and luminal narrowing were observed. Discontinuation of ethosuximide led to the disappearance of fever, ANA and urinary abnormalities. These findings are strongly suggestive of a causal relationship between ethosuximide and a lupus-like syndrome with peculiar renal involvement.
(Internal Medicine 35: 587-591, 1996)

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