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Biomedical subjects

Shigeko Hara

Publications and source records attributed to Shigeko Hara.

At least 19 recordsLinked to original sources
PubMed2006

Successful renal transcatheter arterial embolization in three patients with symptomatic hydronephrosis due to ureteral obstruction.

BACKGROUND: Conventional treatments for symptomatic enlargement of a nonfunctional hydronephrotic kidney caused by obstructive uropathy include surgical treatments. METHODS: Patients included a 67-year-old woman whose obstruction was caused by a lower urinary tract stone complicating spinal tuberculosis (patient 1); a 52-year-old man with compressive complete congenital obstruction crossing the ureteropelvic junction from an aberrant renal artery (patient 2); and a 19-year-old woman with essentially complete idiopathic congenital obstruction at the ureteropelvic junction (patient 3), who required antibiotics for pyelonephritis before embolization. The renal artery was embolized using platinum microcoils. RESULTS: Although the sensation of abdominal fullness diminished within approximately two week after TAE, it took one to two years until the embolized kidney size shrank from 1377+/-634 cm3 (range, 829 to 2072) to 43+/-46 cm3 (42 to 94) by the evaluation of computed tomography. Fever after embolization persisted for 5 days in patient 1, 3 in patient 2, and 9 in patient 3. Flank pain resolved within 5 days in all. Pyelonephritis and complications of treatment have not occurred. CONCLUSION: Embolization for this indication was safe and effective.

Adult↗
PubMed2006

Outcome and treatment of bucillamine-induced nephropathy.

BACKGROUND: Bucillamine (BCL), a treatment for rheumatoid arthritis, occasionally causes proteinuria. Renal specimens are reported to show segmental granular deposition of immunoglobulin G, associated with membranous nephropathy. Long-term course and optimal treatment have remained unknown, and were investigated here. METHODS: We examined clinical records of 400 patients treated with BCL for rheumatoid arthritis, at our hospital from 1998 to 2003, finding 17 with proteinuria and biopsy-proven BCL-induced nephropathy. RESULTS: In all 17 patients, proteinuria resolved without loss of renal function between 3 and 85 months after discontinuing BCL (14.1 +/- 3.4). The only factor influencing time to remission was pathologic stage of membranous nephropathy (stage I vs. stage II or III: 11.5 +/- 4.8 vs. 21.6 +/- 3.3 months; p = 0.02). Maximal proteinuria, total amount of BCL, BCL exposure time, and use of prednisolone or other immunosuppressant agents did not significantly influence time until remission. CONCLUSION: The most important therapeutic step in treating BCL-induced nephropathy is to discontinue BCL. Prednisolone or other immunosuppressant agents might not be effective.

Adult↗
PubMed2006

Systemic AA-amyloidosis related to MPO-ANCA microscopic polyangiitis: a case report.

We report autopsy findings in an 83-year-old woman with myeloperoxidase-type anti-neutrophil cytoplasmic antibody (MPO-ANCA)-positive microscopic polyangiitis and systemic AA amyloidosis. With a diagnosis of MPO-ANCA-related microscopic polyangiitis, the patient was treated with corticosteroids, but she died of intractable enteritis. Autopsy showed inactive vasculitis affecting small arteries in kidney, lung, intestinal tract, and skeletal muscle. Gastrointestinal viscera were thickened, and AA-amyloid was demonstrated in arterioles and surrounding tissues. Amyloidosis also involved heart, kidney, gallbladder, pancreas, salivary gland, and subcutis. ANCA-positive microscopic polyangiitis appears to have been the likely cause of this patient's AA-amyloidosis.

Aged, 80 and over↗
PubMed2006

Mycosis fungoides in a hemodialysis patient with intractable pruritus.

A 69-year-old Japanese man developed pruritus 3 years after beginning hemodialysis. Although eczema was not apparent at first, erythematous patches and plaques developed gradually on the affected skin. Secondary hyperparathyroidism was considered to be a main cause of this patient's pruritus, but skin lesions worsened even after parathyroidectomy had markedly decreased parathyroid hormone concentrations. Two months later, he developed an antibiotic-refractory fever of unknown origin and cervical, axillary, and inguinal lymphadenopathy. Elevations of soluble interleukin-2 receptor with 7410 U/mL and IgE with 24 600 U/mL in serum were noted, as was eosinophilia. The skin showed multiple slightly scaly patches and infiltrative plaques, which were reddish brown and distributed widely over the body surface except for the scalp and face. Mycosis fungoides, a cutaneous T-cell lymphoma, was diagnosed from biopsy specimens findings in skin and lymph node. Mycosis fungoides has not been documented as a cause of pruritus in hemodialysis patients. However, if skin lesions steadily worsen in hemodialysis patients, malignant diseases such as mycosis fungoides should be considered.

Aged↗
PubMed2005

A patient with symptomatic osteomalacia associated with Fanconi syndrome.

We report a patient with renal tubulointerstitial fibrosis and symptomatic osteomalacia associated with Fanconi syndrome. A 55-year-old woman was hospitalized because of an inability to walk. Beginning approximately 2 years previously, she had experienced gradually worsening pain in the hips, shoulders, and trunk, culminating in a bedridden state. Serum urea nitrogen was 38 mg/dl; creatinine, 2.6 mg/dl; uric acid. 3.6 mg/dl; phosphate, 2.3 mg/dl; and alkaline phosphatase, 2111 IU/l. Urinary beta2 microglobulin was 72 331 microg/day. Aminoaciduria, renal glucosuria, and proximal renal tubular acidosis with a normal anion gap were also noted. The patient was diagnosed with Fanconi syndrome. Radiography demonstrated typical Looser zones in the proximal portion of the left and especially the right femoral shaft, and at several other sites. A renal biopsy specimen disclosed severe tubulointerstitial fibrosis with little cellular infiltration. Glomeruli were largely intact. A bone biopsy specimen indicated osteomalacia; no tetracycline labeling could be seen along most trabecular bone surfaces, and the ratio of total osteoid volume to bone volume was increased (71.8%). Bicarbonate administration (9 g/day) gradually lessened most symptoms, permitting ambulation. Calcitriol administration decreased excessive intact-parathyroid hormone emerging after 2 months of acidosis correction. Thus, severe acidosis associated with Fanconi syndrome can induce osteomalacia showing serious skeletal complications, but also responsiveness to bicarbonate therapy.

Journal Article↗
PubMed2005

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↗
PubMed2005

Pelvic insufficiency fracture related to autosomal dominant polycystic kidney disease.

We report the case of a patient with autosomal dominant polycystic kidney disease (ADPKD) and an insufficiency-type fracture of the pelvis. A 60-year-old Japanese woman was admitted because of pain in the right ischium and pubis that began suddenly with no precipitating cause. Computed tomography showed the bony pelvis to be compressed by enlarged dependent kidneys and an enlarged liver. We relieved compression on the pelvic bones by means of transarterial embolization (TAE) to the kidneys and liver after initiation of hemodialysis therapy. The fracture healed gradually after TAE, and the patient could walk 4 months later. In an iliac bone specimen obtained before TAE, cancellous bone was intact, but periosteal and endosteal surfaces of cortical bone showed marked resorption and were irregular. Normally, many ligaments are connected tightly to the periosteal surface, supporting the cortical bone. However, because of extensive surface resorption associated with pressure from enlarged kidneys, connections between ligaments and the periosteal surface presumably became fragile, promoting an insufficiency fracture from unapparent external forces. Thus, ADPKD is a potential cause of insufficiency fracture owing to abnormalities of cortical bone.

Bone Density↗
PubMed2005

Long-term course of post-transplant mesangial IgA deposition: clinicopathologic study of nine cases.

We conducted the present study to elucidate the fate of post-transplant mesangial IgA deposit under the long-term observation. Out of a total of 45 cases with post-transplant mesangial IgA deposition, nine cases with more than 4 yr of follow-up term were enrolled in this study, and clinicopathologic characteristics were described. The study included three men and six women with a mean age of 34.2 yr. The average observation time from the detection of mesangial IgA deposition was 6.1 yr. Three cases were categorized as recurrent IgA nephropathy, while six cases were classified into latent mesangial IgA deposition. One case with hypertension developed end-stage renal disease. The significant improvement in microscopic hematuria was observed in one recurrent IgA nephropathy case. Microscopic findings included mild mesangial stalk thickening in all but one case. IgA deposition demonstrated a significant decrease in three latent mesangial IgA deposition cases. No apparent reduction in dense deposit quantity was observed on electron microscopy. There was no association between clinicopathologic findings and the regimen of anti-immunosuppressive agents. This study showed the improvement of the disease activity did occur in both recurrent IgA nephropathy and latent mesangial IgA deposition. Further investigation of latent mesangial IgA deposition may present the important clue to the pathogenesis of IgA nephropathy.

Adult↗
PubMed2005

Significance of minimodeling in dialysis patients with adynamic bone disease.

BACKGROUND: We previously concluded from histomorphometric analysis that minimodeling contributes to bone formation in adynamic bone disease in patients with primary hypoparathyroidism. Presently we investigated whether this mechanism might be peculiar to adynamic bone disease. METHODS: We histomorphometrically analyzed bone specimens obtained at biopsy or autopsy from 26 maintenance hemodialysis patients with hyperparathyroidism necessitating parathyroidectomy (group A) and from 27 dialysis patients with hypoparathyroidism (group B); respective mean ages were 60 +/- 7 years vs. 64 +/- 8 years; dialysis duration 14 +/- 6 years vs. 11 +/- 9 years; and serum intact parathyroid hormone (PTH) 1205 +/- 439 pg/mL vs. 41 +/- 27 pg/mL. Group B was divided further into outpatient and inpatient subgroups. RESULTS: By histomorphometry, group A patients were diagnosed with osteitis fibrosa, and those in group B with adynamic bone disease. Minimodeling bone volume and minimodeling bone number were significantly greater in group B than group A (P= 0.0028 and P= 0.0008, respectively). Minimodeling bone volume correlated significantly and positively correlated with total bone volume in group B (P= 0.0016), but not in group A. In group B, minimodeling bone volume and total bone voluem were greater in outpatients than inpatients (P < 0.0001 and P= 0. 025, respectively). Minimodeling bone volume and total bone volume showed significant negative correlation with age in group B (P < 0.001 and P= 0.005, respectively). CONCLUSION: Minimodeling might contribute to bone formation in dialysis patients with adynamic bone disease, in the absence of remodeling stimulated by parathyroid hormone (PTH), especially in relatively young patients with good activities of daily living.

Aged↗
PubMed2005

Long-term erythropoietin therapy improves response in myelodysplastic syndrome.

We report on a 53-year-old Japanese female on hemodialysis with myelodysplastic syndrome whose condition improved with recombinant human erythropoietin (epoetin) therapy. In 1992, based on a diagnosis of folic acid deficiency anemia, folate derivatives were administered. However, the anemia did not improve, and red blood cells had to be transfused subsequently. The transfusion volume was gradually increased afterward, as renal failure progressed, probably due to nephropathy by phenacetin. In 1998, when hemodialysis started, epoetin therapy was started with a dose of 3000 units three times per week. In July 2001, myelodysplastic syndrome (MDS) of a refractory anemia type was diagnosed through bone marrow aspiration. Myelodysplastic syndrome might cause an epoetin-resistant renal anemia. Afterwards the transfusion volume was gradually reduced, and transfusions were not performed after March 2002. Improvements of histological findings of MDS as well as anemia were confirmed by bone marrow aspiration in July 2003. This is an unusual case of a patient with a previously existing MDS, who subsequently develops end stage renal disease, and has an amelioration of her underlying MDS with the administration of epoetin over a long-term period, while being treated with chronic hemodialysis, even when not effective for a short-term.

Erythropoietin↗
PubMed2005

Autosomal dominant polycystic kidney disease showing rupture of a lateral ventral hernia following paralytic ileus.

We report an 83-year-old Japanese male with autosomal dominant polycystic kidney disease (ADPKD), which was marked by unusually enlarged kidneys, and in whom ileus occurred after administration of procainamide. The bowels became swollen and ruptured the skin and appeared on the outside of the skin. Even after the ileus state was resolved, the projected intestinal tract was not restored due to a large defect of the skin, and ostomy was performed. Abdominal hernia including lateral ventral hernia due to enlarged kidneys may result in perforation of the abdominal wall as well as intestinal wall.

Aged↗
PubMed2005

Membranous glomerulopathy induced by myeloperoxidase-anti-neutrophil cytoplasmic antibody-related crescentic glomerulonephritis.

A 68-year-old woman was referred for evaluation of nephrotic-range proteinuria and a course suggesting rapidly progressive glomerulonephritis. Serum anti-neutrophil cytoplasmic antibody against myeloperoxidase (MPO) was 204 U/ml. A renal biopsy specimen revealed necrotizing glomerulonephritis with crescent formation. However, immunofluorescence showed staining with IgG and C3 along capillary walls. IgG positivity included both IgG1 and IgG4. Electron microscopic examination disclosed both paramesangial and subepithelial deposits. This case suggests that rarely, MPO-ANCA-related crescentic glomerulonephritis may present nephrotic-range proteinuria and show immune deposits along capillary walls.

Aged↗
PubMed2004

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↗
PubMed2004

Arresting dialysis-related amyloidosis: a prospective multicenter controlled trial of direct hemoperfusion with a beta2-microglobulin adsorption column.

We investigated the clinical efficacy of direct hemoperfusion with a beta2-microglobulin (beta2-m) adsorption column for the treatment of patients with dialysis-related amyloidosis. A 2-year prospective controlled study was performed to compare the effects of passaging blood through a (beta2-m) adsorption column (Lixelle) before it is passaged through the dialysis polysulfone membrane on the severity of amyloidosis in these individuals. Patients (n = 22) whose blood went through the Lixelle column prior to dialysis had a higher beta2-m removal rate compared to an equal number of controls, and they showed earlier improvement in their symptoms which included impaired daily activities, joint stiffness, and pain. The appearance of additional bone cysts was prevented in pre-adsorbed patients but not in the controls. Thus, the Lixelle column is useful in preventing the progression of dialysis-related amyloidosis and in ameliorating or arresting the progression of the symptoms of this disorder.

Activities of Daily Living↗
PubMed2004

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↗