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Biomedical subjects

Y Ubara

Publications and source records attributed to Y Ubara.

At least 19 recordsLinked to original sources

Severe ectopic calcification of the intestinal wall in a patient on long-term continuous ambulatory peritoneal dialysis therapy.

We report autopsy findings of a 69-year-old man on long-term CAPD therapy for 13 years who showed linear peritoneal calcification. Continuous ambulatory peritoneal dialysis (CAPD) was started in 1982. He has been administered excessive amounts of vitamin D(3) derivatives (VitD) (2.0 to 2.5 microg daily) and calcium carbonate (4 g daily) for secondary hyperparathyroidism since initiation of CAPD. In May 1995, his intact parathyroid hormone (PTH) level increased over 1,000 pg/mL. Immediately after VitD was changed from pill to liquid, the dose was increased to 5 microg daily. Although the serum calcium level remained between 4.5 and 4.9 mEq/L, and serum phosphate level was 5.0 to 7.2 mg/dL, plain abdominal radiography and computed tomography showed continuous calcification along the intestinal wall in October 1995. In spite of the continuation of CAPD therapy, he remained asymptomatic until he died of congestive heart failure in January 1997. He experienced eight episodes of peritonitis during his clinical course. Autopsy showed that numerous calcified plaques were present on the submucosal portion between the thickened serosa and the longitudinal layer of the muscularis externa. The remainder of the subserosa was fibrotic, and the small arteries had markedly thickened intima and severely narrowed lumina.

Abdominal Muscles↗

Splenectomy may improve the glomerulopathy of type II mixed cryoglobulinemia.

Many patients with type II mixed cryoglobulinemia have been shown to be infected with hapatitis C virus (HCV). Therefore, interferon-alfa has become the first choice of treatment for patients with HCV-associated cryoglobulinemia. However, the disease often relapses after the discontinuation of interferon therapy. The long-term effect of interferon therapy is controversial. Therefore, a more effective therapy needs to be developed. A 62-year-old Japanese woman was admitted to our hospital for the examination of abnormal liver function tests, severe edema, and purpura in her lower extremities. Glomerulopathy secondary to HCV-related cryoglobulinemia was suspected. Her serum creatinine was increased to 2.1 mg/dL. Interferon therapy was considered initially. However, because of pancytopenia caused by liver cirrhosis and splenomegaly, splenectomy was performed in February 1997, before the start of interferon therapy. Renal biopsy specimen taken at the time of the splenectomy showed typical cryoglobulinemic glomerulonephritis. Gradually, after surgery, the patient's thrombocytopenia and anemia improved, her proteinuria and hematuria were decreased, her cryocrit dropped from 15% to 5%, the Ccr increased from 21.1 mL/min to 48.8 mL/min, and the purpura in her lower extremities disappeared. A repeat renal biopsy performed in May 1998 showed marked histological improvement. Splenectomy is not widely accepted as a treatment for cryoglobulinemia. Our case suggests the possibility that the monoclonal-IgM component of the type II cryoglobulin may be formed in the spleen. In conclusion, splenectomy may be an effective therapy for cryoglobulinemia in patients with HCV-positive liver cirrhosis and pancytopenia secondary to splenomegaly.

Anemia↗

A case of mixed connective tissue disease (MCTD) complicated with MPO-ANCA-related necrotizing glomerulonephritis.

Renal diseases of mixed connective tissue disease (MCTD) are not unusual. Although most of them are SLE-like renal impairment with immune complex deposits, systemic sclerosis- (SSc) like renal impairments with intimal thickening of interlobular arteries or arterioles are also encountered. Several cases of SSc complicated with MPO-ANCA-related necrotizing glomerulonephritis (nGN) are reported. Here we report a case which developed MPO-ANCA-related nGN 16 years after the diagnosis of MCTD. She exhibited pauci-immune focal nGN and significantly high titer of MPO-ANCA. She was successfully treated with prednisolone and cyclophosphamide. We believe this is the first case in which MPO-ANCA-related nGN was demonstrated in a patient with MCTD.

Antibodies, Antineutrophil Cytoplasmic↗

Transcatheter renal arterial embolization therapy on a patient with polycystic kidney disease on hemodialysis.

We report a patient with autosomal dominant polycystic kidney disease (ADPKD) undergoing long-term hemodialysis who underwent transcatheter arterial embolization (TAE) of the renal arteries to shrink enlarged kidneys. In 1983, the patient started hemodialysis because of chronic renal failure secondary to ADPKD. However, renal size continued to increase. In January 1997, he was admitted to our hospital with abdominal distension and anorexia, in addition to progression of anemia. Upper gastroendoscopy showed an esophageal ulcer and severe external compression of the stomach. Renal angiography using the Seldinger technique showed stretched and deformed segmental renal arteries with massive enlargement of the kidneys. TAE with stainless steel coils was performed on both renal arteries. With a rapid and progressive decrease in kidney size, anorexia and anemia were improved, and the gastrointestinal compression was eliminated. In some patients with ADPKD, renal size continues to increase even after the initiation of dialysis. In about 10 years, patients develop gastrointestinal complications, such as dysphagia, ileus, severe constipation, and intestinal perforation. Surgical procedures such as nephrectomy are not satisfactory. This report shows that TAE is a safe and effective therapy for patients with ADPKD with massively enlarged kidneys.

Angiography↗

Proliferation of parathyroid cells negatively correlates with expression of parathyroid hormone-related protein in secondary parathyroid hyperplasia.

BACKGROUND: Parathyroid hormone-related protein (PTHrP) is now suspected to act as an autocrine or paracrine regulator of cell growth or differentiation, although it was originally reported as a hypercalcemic substance in malignancies. This study was performed to assess the relationship between PTHrP expression and cell proliferation in human parathyroid glands. METHODS: The localization of PTH and PTHrP was studied in 42 samples of hyperplastic parathyroid from 14 long-term hemodialysis cases with immunohistochemistry and in situ hybridization. Results were compared with proliferative activity (proliferating cell nuclear antigen index: counts of proliferating cell nuclear antigen-positive cells/100 cells). The localization of the PTH/PTHrP receptor was also examined. Ten normal glands were studied as controls. RESULTS: In hyperplasia, cells positive for PTH, PTHrP, or both were observed immunohistochemically. The areas expressing PTHrP mRNA completely coincided with those positive for PTHrP immunohistochemically. Oxyphilic or transitional oxyphilic cells were consistently positive for PTHrP. PTH/PTHrP receptors were located in the cytoplasmic membrane in most parathyroid cells. Proliferating cell nuclear antigen-positive cells were rare in normal glands with an index of 0. 22 +/- 0.09 (mean +/- sem). They were significantly increased in hyperplastic cases but less for PTHrP-positive than for -negative cells (1.25 +/- 0.16 as compared with 7.80 +/- 0.52; P < 0.0001). CONCLUSION: The observed low level of proliferation of PTHrP-positive cells suggests a functional role for PTHrP as a possible growth suppressor in the human parathyroid.

Adult↗

[A case of rheumatoid arthritis with bucillamine-induced myasthenia gravis treated by immunoadsorption therapy].

We report a case of 48-year old female with rheumatoid arthritis (RA) complicated with myasthenia gravis. In 1988, she was diagnosed of having RA, and several therapeutic drugs were administered, but her disease activity was in poor control. In July 1993, bucillamine (BU) was started at a dose of 100 mg/day, and her arthritis subsided. However, in October 1996, she was admitted with a rapidly progressive ptosis and double vision in the left eye, which became prominent in the evening. Because serum concentration of the antibody to acetylcholine receptors (AchR Ab) was elevated at 12.6 nmol/l, and the ptosis was reversed immediately after a tensilon test, ocular type myasthenia gravis (MG) was diagnosed and it was thought to have been induced by BU. Immunoadsorption therapy was started after discontinuation of this drug, and was continued for 6 months, resulting in improvement of neurological symptoms and decrease in AchR Ab level. MG has not recurred since. Although several cases of D-penicillamine (DP) induced MG are reported, only two cases are reported which were induced by BU, sulfhydryl compound which has a structure similar to DP. Since BU has been widely used as one of the disease modifying anti-rheumatic drugs in Japan, MG induced by this drug should be paid attention as one of the adverse effects.

Anti-Inflammatory Agents, Non-Steroidal↗

Bone histomorphometrical analysis in patients on long-term dialysis treatment for more than ten years.

In Japan there is a steady increase in patients who have been on dialysis therapy for more than 10 years. Bone lesions could emerge as a serious problem during this dialysis period. From 1986 to 1993, bone lesions were examined by histomorphometry of the bone tissues (40 biopsis, 17 autopsies) in fifty seven patients who have undergone a long-term hemodialysis treatment (37 males, 20 females) at Toranomon hospital. Mean age of the patients was 56 +/- 11 (SD) years (range; 22 to 74) and mean dialysis period, 14.5 +/- 11 (SD) years (range; 10 to 28). The results were compared with biochemical and endocrinological data. The subjects were classified into osteitis fibrosa group (33%), osteomalacia group (12%), mixed group (7%) and mild group (48%). Intact parathyroid hormone (PTH) and alkaline phosphatase (Al-P) value were significantly higher in osteitis fibrosa and mixed groups, all cases showing intact-PTH values greater than 500 pg/ml (normal value; 20-53), but not in osteomalcia and mild groups. The analysis of 32 cases with intact-PTH higher than 500 pg/ml showed osteitis fibrosa in 59.4% mixed in 12.5%, osteomalacia in 3.1% and mild in 25%. Aluminum was positive in 41% for osteitis fibrosa, 25% for mixed, 60% for osteomalacia and 52% for mild group. In summary, cases having undergone hemodialysis more than 10 years showed higher frequency of osteitis fibrosa when intact-PTH exceeded 500 pg/ml. Al-P values in such cases were high in association with the intact-PTH values. There was no significant correlation between aluminum deposition and respective bone tissue types.

Adult↗

Association of Sjögren's syndrome with hereditary angioneurotic edema: report of a case.

A case of hereditary angioneurotic edema (HANE) associated with Sjögren's syndrome is presented. One of the members of a pedigree of HANE due to deficiency of C1 inhibitor (C1INH) had a positive titer for anti-SS-A and anti-SS-B antibodies in the serum, complaining of symptom of dry eyes and dry mouth. A lip biopsy revealed lymphocytic infiltration of minor salivary glands. The patient had renal tubular acidosis (RTA). Thus the patient was diagnosed as suffering from Sjögren's syndrome with RTA.

Angioedema↗

Renovascular hypertension may cause nephrotic range proteinuria and focal glomerulosclerosis in contralateral kidney.

Little attention has been paid to nephropathies and proteinuria in renovascular hypertension (RVH). Recently there has been a growing interest in the conditions induced by RVH. 10 cases of RVH were diagnosed by angiography and renin sampling from renal veins in the last 6 years in our hospital. The patients were all male and mean age was 64 +/- 8 (SD) years. Data were as follow: protein excretion was 3.8 +/- 2.2 g/day (> or = 3.5 g/day in 8 patients), sBP 202 +/- 24 mmHg, dBP 113 +/- 17 mmHg, serum renin concentration 64 +/- 45 pg/ml, and ipsilateral/contralateral renal vein renin ratio 3.3 +/- 1.0. RVH was treated by nephrectomy in 3 patients, percutaneous transluminal renal angioplasty (PTA) in 2, and angiotensin converting enzyme inhibitors (ACE-I) administration in 8. Biopsies were performed on contralateral kidney in 4 patients. Focal segmental glomerulosclerosis (FGS) was found in 3 patients, and nephrosclerosis in 1, whereas only nephrosclerosis was found in nephrectomized kidneys in all 3 patients. After nephrectomy, PTA and the treatment by ACE-I, not only blood pressure but also proteinuria was markedly reduced. These findings suggest that severe stenosis of the renal artery led to renal ischemia, which activated renin excretion, to cause glomerular hyperfiltration through vasoconstriction of the efferent arterioles in the contralateral kidney. FGS-like lesion thus induced appeared to have caused massive proteinuria.

Aged↗

[Computed tomographical evaluation of diabetic nephropathy].

Diabetic nephropathy can be regarded mainly as a type of microangiopathy, but is a disease that may also include aspects of macroangiopathy. This is especially true of renal disease in non-insulin dependent diabetes mellitus (NIDDM), which is characterized not only by diabetic glomerulosclerosis, but also by atherosclerosis. We performed morphological studies on the kidney, using computed tomography (CT), focusing on such points as: (1) abdominal aortic calcifications at the level of kidney, (2) calcifications in the renal artery, and (3) wedge-shaped defects on the renal surface. We noted that these findings became more prominent in NIDDM patients during end-stage renal failure than during normal renal function, and were significantly more common in those two NIDDM groups than in age-matched nondiabetic patients without hypertension, hyperlipidemia or gout. NIDDM patients exhibited these features more frequently than IDDM patients.

Aged↗

Relation between parathyroid hormone and cardiac function in long-term hemodialysis patients.

Radionuclide ventriculographic and echocardiographic assessments of left ventricular cardiac function were studied in 46 long-term maintenance hemodialysis patients, and comparison of cardiac function pre- and post-parathyroidectomy in 10 hemodialysis patients with secondary hyperparathyroidism was investigated. In long-term hemodialysis patients, impairment of cardiac function was observed in 80.4%. In an overall study of 46 patients, no correlation between intact parathyroid hormone (iPTH) level and left ventricular ejection fraction (LVEF) was observed, but a significant (p < 0.05) negative correlation was observed in patients with an iPTh blood level over 200 pg/ml. A negative correlation between fractional fiber shortening and an iPTh level over 200 pg/ml was observed (p < 0.05). There was neither a correlation between the iPTH level and left ventricular (LV) mass, nor was there a correlation between the iPTH level and wall thickness. After parathyroidectomy, systolic and diastolic blood pressure, cardiothoracic ratio, LVEF, and LV mass decreased significantly (p < 0.05), but no significant difference was detected in cardiac thickness. In summary, the present data suggest that high levels over 200 pg/ml of the iPTH in long-term hemodialysis patients adversely affect the myocardial function, induce cardiac hypertrophy and cause high arterial blood pressure. After parathyroidectomy, the cardiac function improved with a reduction of cardiac mass and an improvement of cardiac contraction.

Adult↗