Autoantibodies against carbonic anhydrase II are increased in renal tubular acidosis associated with Sjogren syndrome.
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Acidosis, Renal Tubular↗
Biomedical subjects
Fumi Takemoto
Publications and source records attributed to Fumi Takemoto.
Distribution of IgG subclasses in a biopsy specimen showing membranous nephropathy with anti-glomerular basement membrane glomerulonephritis: an uncharacteristically good outcome with corticosteroid therapy.
Few occurrences of anti-glomerular basement membrane glomerulonephritis superimposed on membranous nephropathy have been reported; most reported cases began as membranous nephropathy. Little is known about the etiology, pathogenesis, or immunoglobulin G subclasses in glomerular basement membrane deposits in these combined cases. The authors report the case of an elderly patient with apparently simultaneous onset of both membranous nephropathy and anti-glomerular basement membrane glomerulonephritis who had an excellent outcome with only pulse corticosteroid therapy. Successive renal biopsy specimens showed occurrence and disappearance of immunoglobulin G4 deposits in the glomerular basement membrane. Immunoglobulin G subclass analysis was important in recognizing membranous nephropathy underlying the anti-glomerular basement membrane disease.
Aged↗
Intravascular embolization therapy in a patient with an enlarged polycystic liver.
Because the kidneys in patients with autosomal dominant polycystic kidney disease (ADPKD) are usually supplied by well-developed arteries, the authors attempted renal contraction therapy in such patients with renal transcatheter arterial embolization (TAE) using intravascular coils. In most patients with marked nephromegaly, renal TAE was effective. However, in patients with marked hepatomegaly without significant nephromegaly, renal TAE was not effective, and hepatic treatment was always required. In June 2001, the authors obtained approval for a new treatment,"TAE for enlarged polycystic liver," from an ethics committee discussing new treatment at their hospital. A 56-year-old man undergoing hemodialysis for 2 months was referred to the authors' institute with the complaint of severe abdominal distension and loss of appetite in March 2001. Most of the liver (about 90%) had been replaced by multiple cysts, and near-intact hepatic parenchyma was quite scarce. The kidneys were quite small compared with the liver. Angiography results showed that almost all hepatic arterial branches were well developed, although most of the portal vein was obstructed, and only the left medial portal vein was spared; the former corresponded to the hepatic region replaced by multiple cysts and the latter to the preserved intact hepatic parenchyma. The target of TAE was the hepatic arterial branches of the former. Microcoils were used as embolization material. Two years after TAE, abdominal distension has markedly decreased because of decrease in liver size (to 54% of the previous value), and muscle and fat volume in the thoracic region have increased owing to improvement of appetite. Ascites became obscure. Nutrition and activities of daily living have improved. Hepatic TAE may be an option for treatment of patients in poor condition with symptomatic polycystic liver.
Ascites↗
An abrupt onset of seropositive polyarthritis with prominent distal tenosynovitis concomitant with bronochiolitis obliterans organizing pneumonia (BOOP): consideration of the relationship with RS3PE syndrome.
A 64-year-old Japanese woman with a two-week history of polyarthralgia and persistent cough was diagnosed as seropositive polyarthritis and fulfilled the criteria of early rheumatoid arthritis (RA). In addition, inflammatory pitting edema of the distal extremities was apparent, suggestive of the remitting seronegative symmetrical synovitis with pitting edema (RS3PE) syndrome. A number of investigations including hand MRI, bone scintigraphy and HLA typing supported a diagnosis of RS3PE syndrome rather than RA. Chest computed tomography revealed concomitant evidence of bronchiolitis obliterans organizing pneumonia (BOOP). Treatment with 30 mg of prednisolone daily immediately ameliorated the polyarthritis and the BOOP. Seropositive polyarthritis with distal pitting edema may be categorized as both RA and the RS3PE syndrome.
Arthritis↗
Severe interstitial cystitis associated with Sjögren's syndrome.
A 53-year-old woman presented with oliguria, urinary frequency, abdominal pain and severe edema of the lower extremities. Her serum creatinine was 8.1 mg/dl. Computed tomographic and ultrasonographic studies showed a severely dilated urinary bladder, and bilateral hydroureteronephrosis. Examination of a urinary bladder biopsy specimen showed subepithelial edema and infiltration by lymphocytes and plasmacytes. However, the patient complainted of dry mouth and dry eyes. Ophthalmologically, the Schirmer test was positive. A biopsy of the minor salivary glands in the lip showed chronic sialoadenitis. A diagnosis of Sjögren's syndrome complicated by interstitial cystitis was made. Since she had been anuric, secondary to urinary obstruction, intermittent self-catheterization was started. Combination of corticosteroid and cyclosporin therapy was initiated. Spontaneous urination began, and gradually the patient's symptoms remitted. After 8 months of therapy, bladder capacity increased from 140 ml to 350 ml, and she voided approximately 1,200 ml by herself and 600 ml by catheterization daily. This case suggests that when severe interstitial cystitis is associated with Sjögren's syndrome, a therapeutic trial of corticosteroids and cyclosporin may be beneficial.
Cyclosporine↗
Hemodialysis-related portal-systemic encephalopathy.
The authors encountered a case of portal-systemic venous shunt newly diagnosed after initiation of hemodialysis. A 68-year-old Japanese woman began hemodialysis because of symptoms of uremia including loss of appetite and pulmonary edema. Loss of consciousness occurred suddenly after her ninth session of hemodialysis. No hepatic functional abnormality was found other than hyperammonemia (314 microg/dL [184 micromol/L]). Loss of consciousness subsequently occurred often after hemodialysis. Color Doppler ultrasonography and magnetic resonance angiography depicted a large shunt between the left gastric vein and left renal vein resulting in portal flow entering the systemic circulation via the renal vein. Because the shunt was large, ligation of it was performed surgically. Results of histologic examination of a liver biopsy specimen obtained intraoperatively were normal. The patient became well postoperatively. This patient's encephalopathy appeared to be caused by the flow of ammonia-rich portal venous blood into the systemic circulation via the large shunt owing to a decrease in intravenous pressure after rapid hemodialysis. Portal-systemic shunt encephalopathy should be recognized as a "new" neuropsychiatric disorder characteristic of patients undergoing hemodialysis.
Aged↗
[Serum calcium concentration and the safety of vitamin D therapy].
Vitamin D and its analogues are administered to patients with secondary hyperparathyroidism (SHPT) in chronic renal failure (CRF). Hypercalcemia is one of the complications of this therapy, and is thought to be not rare, though its frequency is not clearly documented. There is a need to monitor serum corrected (ionized) calcium concentration and parathyroid hormone (PTH) in order to administer vitamin D and its analogues safely. Intravenous administration of vitamin D provides advantages over oral administration, though no definite evidence could show which is better. Calcitriol and maxacalcitol are administered intravenously, and the latter is more likely to increase serum calcium concentration than the former. It must be very careful to administer vitamin D to patients with predialysis CRF because hypercalcemia can accelerate progression of renal dysfunction.
Administration, Oral↗
[Management and diagnosis of water electrolyte imbalance due to kidney diseases].
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Acid-Base Imbalance↗
Sarcomatoid collecting duct carcinoma arising in the hemodialysis-associated acquired cystic kidney: an autopsy report.
A case of sarcomatoid collecting duct carcinoma (CDC) arising in a long-term hemodialysis-associated acquired cystic kidney was reported. A 71-year-old woman with a 21-year history of hemodialysis showed a peritoneal metastatic carcinoma (carcinomatous peritonitis) with an unknown primary site. An autopsy revealed a sarcomatoid collecting duct carcinoma of the right kidney with multicyst formation. In addition to the carcinomatous peritonitis, the tumor metastasized to the lymph nodes and bilateral lung. The primary tumor was composed of both carcinomatous and sarcomatous components, suggesting a high-grade transformation. Carcinomatous tumor cells were positive for epithelial membranous antigen (EMA), cytokeratin, and reactive to soybean agglutinin and peanut agglutinin, whereas the sarcomatous cells were positive for vimentin as well as EMA. Thus, the immunohistochemical and lectin-histochemical analysis confirmed that the tumor originated in the medullary collecting duct. Although CDC is not common in acquired cystic kidney disease patients, attention should be given to the occurrence of high-grade carcinoma of rare histological variant, as well as conventional renal cell carcinoma.
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Gene expression of vitamin D hydroxylase and megalin in the remnant kidney of nephrectomized rats.
BACKGROUND: Regulation of vitamin D hydroxylase genes in the early stage of chronic renal failure is not fully understood. Using nephrectomized rats, we examined changes in mRNA levels of CYP27B1 (25-hydroxyvitamin D3-1 alpha-hydroxylase), CYP24 (25-hydroxyvitamin D3-24-hydroxylase), and vitamin D receptor in relation to megalin, recently found to participate in renal vitamin D metabolism. METHODS: A rat model of moderate renal failure was induced by 3/4 nephrectomy. Plasma parameters, including vitamin D metabolite concentrations, were measured at weeks 2, 4 and 8, and poly(A)+ RNA extracted from the remnant kidneys was subjected to Northern blot hybridization. RESULTS: Plasma creatinine concentration at week 2 was 0.40 +/- 0.02 mg/dL in the sham-operated and 0.93 +/- 0.15 mg/dL in the nephrectomized rats, and both values remained constant up to week 8. Plasma concentrations of 25(OH)D3, 1 alpha,25(OH)2D3, and 24,25(OH)2D3 were unchanged between nephrectomized and sham-operated rats at week 8. Intact parathyroid hormone (PTH) increased at week 8 in nephrectomized rats. CYP27B1 mRNA in nephrectomized rats did not vary at week 2, but increased approximately two- and four-fold at weeks 4 and 8, respectively, compared to the sham-operated rats. CYP24 and megalin mRNAs, on the other hand, began to decline as early as at week 2 in nephrectomized rats and kept decreasing throughout the experiment. The expression of vitamin D receptor was modestly but significantly decreased only at week 8. CONCLUSION: Coordinated and reciprocal alterations of the increase in CYP27B1 mRNA and the decrease in CYP24 mRNA may play a pivotal role in maintaining the plasma level of 1 alpha,25(OH)2D3 in the face of reduced nephron mass and/or megalin expression.
25-Hydroxyvitamin D3 1-alpha-Hydroxylase↗
Histomorphometric features of bone in patients with primary and secondary hypoparathyroidism.
BACKGROUND: Idiopathic adynamic bone disease (ABD) in dialysis patients is characterized by low serum parathyroid hormone (PTH) concentration. Whether ABD itself causes serious disease is controversial. Fuller understanding of both primary hypoparathyroidism and secondary hypoparathyroidism resulting in a long-standing low-PTH state may shed light on properties of ABD. METHODS: We performed histomorphometric analysis in bone specimens from biopsy in two female patients with primary hypoparathyroidism and in an autopsy specimen of bone from a male patient with secondary hypoparathyroidism related to long-term hemodialysis; respective ages, 45, 58, and 65 years; dialysis duration, 6 years, 2 months, and 30 years; lumbar bone mineral density, 2.88, 2.43, and 4.1 SD above the normal mean; and serum intact PTH, <5, <20, and <84 pg/mL (mean, 30.4). Tetracycline labeling was performed in the first two cases. RESULTS: Histomorphometric analysis in the first two cases indicated a diagnosis of ABD, since no tetracycline labeling could be seen along most of trabecular bone surfaces, total osteoid volume was decreased, and fibrous tissue was minimal. Bone volume was preserved, and the dense bone-trabecular connectivity was noted, with normal lamellar structure. A small number of hump-like structures protruded from the quiescent surface of trabecular bone, a pattern which has been called "minimodeling." Tetracycline label was observed in only a small area within trabecular bone in patient 1, and at a region of trabecular bone surface showing minimodeling in patient 2. The third case was also diagnosed as ABD; cancellous lamellar structure and bone volume were normal, although trabecular connectivity was poor and island bone was relatively prominent. Minimodeling was evident. Minimodeling bone volume/total bone volume in these three cases was 9.0%, 13.1%, and 6.8%, respectively; number of minimodeling sites/total bone volume (N/mm2) was 4.9, 8.6, and 9.0, respectively. CONCLUSION: Bone formation mechanism by minimodeling might contribute to preserving bone volume in dialysis patients with hypoparathyroidism, even in the absence of remodeling stimulated by PTH.
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Chinese herbs and bone disease.
We treated a patient with an unusual bone disease at least partly associated with Chinese herbs. Seven years after 65-year-old man had begun to consume Chinese herbs, multifocal osteoarthralgias were noted, and the patient was hospitalized for renal dysfunction (serum creatinine, 2.8 mg/dl; urea nitrogen, 19 mg/dl). Fanconi syndrome also was apparent. A renal biopsy specimen showed tubulo-interstitial fibrosis. Chinese herbs were discontinued and prednisolone was started, but bone and joint pain as well as renal function gradually worsened. Four years later, creatinine was 9.0 mg/dl and alkaline phosphatase was 571 IU/l. As bone scintigraphy revealed localized asymmetric lesions, Paget's disease of bone was suspected at first. However, neither osteosclerosis nor hypertrophy was seen in radiographs. Based on a bone specimen histology we diagnosed as mixed-type renal osteodystrophy including osteomalacia and osteitis fibrosa. Mosaic pattern of cement lines was not present. This case was not compatible with either Paget's disease or typical renal osteodystrophy as seen in dialysis patients. Etidronate disodium was effective in alleviating bone symptoms. The patient's bone disorder may be a new disease at least partly related to Chinese herbs independently of nephropathy.
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Renal contraction therapy for enlarged polycystic kidneys by transcatheter arterial embolization in hemodialysis patients.
Kidneys of patients with autosomal dominant polycystic kidney disease (ADPKD) usually continue to increase in size, even after patients begin dialysis therapy, and the mass effects may lead to severe complications. Such external conventional therapies as surgical and laparoscopic procedures have not yielded satisfactory results. Because kidneys in patients with ADPKD usually are supplied by well-developed arteries, we attempted renal contraction therapy in patients with ADPKD by renal transcatheter arterial embolization (TAE) using intravascular coils. After obtaining informed consent, we selected anuric patients on dialysis therapy with markedly distended abdomens or macroscopic hematuria. Between October 1996 and December 2000, a total of 64 patients were treated. Renal size, abdominal circumference, dry weight, hematocrit, and insulin-like growth factor-I were measured before TAE and 3, 6, and 12 months after TAE. Renal sizes decreased to 73.8% +/- 12.0%, 61.7% +/- 14.7%, and 53.4% +/- 11.6% of preinterventional values at 3, 6, and 12 months after therapy, respectively (P < 0.0001). Abdominal circumference and dry weight were significantly decreased at 3, 6, and 12 months (P < 0.0001) compared with baseline values before therapy. Hematocrits increased sequentially after 3, 6, and 12 months (P < 0.0001). Levels of insulin-like growth factor-I an index of nutritional status, significantly increased at 3, 6, and 12 months compared with the baseline value (P < 0.001). This therapy was effective for all patients. Serious complications were not seen after this treatment, although such minor complications as fever and flank pain were observed within the first week after the procedure. Our internal treatment with TAE is a safe and effective procedure that has resulted in improvement in the quality of life and nutritional status of patients with ADPKD.
Aged↗