Junichi Hoshino

University of California, Los Angeles, Los Angeles, California, United States

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Publications (97)230.24 Total impact

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    ABSTRACT: A bone biopsy specimen in a long-term hemodialysis patient with sarcoidosis coexisting with severe hypoparathyroidism has demonstrated that a persistent near physiological level of 1,25-dihydroxyvitamin D3 contributes to the preservation of bone remodeling and has the potential to retard the development of vascular calcification and atherosclerosis. Sarcoidosis-related hypercalcemia and hypoparathyroidism, which is characterized by 1,25-dihydroxyvitamin D3 (1,25(OH)2D3) overproduction, is rarely seen in hemodialysis patients. Herein, we describe a 60-year-old Japanese woman on hemodialysis for 35 years who presented with malaise and hypercalcemia. Severe hypoparathyroidism without parathyroidectomy and a preserved 1,25(OH)2D3 level were detected. Computed tomography showed bilateral axillary lymphadenopathy and minimal aortic and soft tissue calcification. The axillary node biopsy led to a definite diagnosis of sarcoidosis. A bone biopsy specimen obtained from the right iliac crest showed remodeling of normal lamellar bone with scalloped cement lines and clear double labeling by tetracycline on fluorescence microscopy. Histomorphometric analysis revealed that the bone formation rate was preserved (30.0 %/year), together with a decrease of osteoid volume (5.75 %) and fibrous volume (0 %), indicating that the patient did not have adynamic bone disease and only showed mild disease. This is the first documented case of sarcoidosis-related hypercalcemia associated with severe hypoparathyroidism in a long-term hemodialysis patient who underwent bone histomorphometry. Our findings suggest that, in hemodialysis patients with sarcoidosis coexisting with severe hypoparathyroidism, a persistent near physiological level of 1,25(OH)2D3 contributes to the preservation of bone remodeling and has the potential to retard the development of vascular calcification and atherosclerosis.
    Osteoporosis International 12/2014; · 4.04 Impact Factor
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    ABSTRACT: AimsTo investigate the relationship between the progression of anaemia and renal pathological findings in patients with diabetic nephropathy.MethodsA total of 223 patients with diabetes underwent renal biopsy from 1985 to 2010 and were confirmed to have pure diabetic nephropathy according to the recent classification, of whom 113 (baseline haemoglobin ≥ 11 g/dl) were enrolled in the study. Linear regression analysis was used to estimate the changes in haemoglobin levels during the follow-up period.ResultsIn a multivariate model adjusted for clinical and histopathological variables, higher interstitial fibrosis and tubular atrophy scores were more strongly associated with a decrease in haemoglobin levels than were lower scores. Compared with an interstitial fibrosis and tubular atrophy score of 0, the standardized coefficients for interstitial fibrosis and tubular atrophy scores of 1, 2 and 3 were -0.20 (95% CI -0.93 to 0.31), -0.34 (95% CI -1.34 to 0.22) and -0.47 (95% CI -1.96 to -0.07), respectively, whereas a higher glomerular class, a higher vascular lesion score and the presence of exudative lesions were not strongly correlated with the decrease in haemoglobin.Conclusions Tubulointerstitial lesions that are more advanced are significantly associated with the progression of anaemia in patients with diabetic nephropathy after adjustment for numerous covariates. This finding suggests that tubulointerstitial lesions may be a useful prognostic indicator for anaemia in patients with diabetic nephropathy, and that decreased erythropoietin production attributable to the progression of tubulointerstitial lesions is a major cause of anaemia in these patients.This article is protected by copyright. All rights reserved.
    Diabetic Medicine 11/2014; · 3.24 Impact Factor
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    ABSTRACT: Aims The kidneys of patients with diabetes mellitus usually exhibit a characteristic pattern of linear immunofluorescent staining for immunoglobulin G (IgG) along the glomerular and tubular basement membranes. However, the association between linear IgG staining and the renal prognosis remains unclear. Methods Among 223 patients with diabetes who underwent renal biopsy from 1985 to 2010 and were confirmed to have pure diabetic nephropathy according to the classification of Tervaert et al., 165 patients (glomerular classes I to III) were enrolled in this study. Immunofluorescent staining was classified into three categories according to its intensity (0 = none, 1 = weakly positive, and 2 = positive). Cox proportional hazards regression analysis was used to calculate the hazard ratio (HR) and 95% confidence interval (CI) for death-censored renal death, with each regression analysis employing four levels of multivariate adjustment. Results After adjustment for important clinical factors at the time of renal biopsy, the HR for death-censored renal death in patients with an IgG staining score of 1 or 2 was respectively 3.01 (95% CI: 1.05-8.68) and 4.68 (1.67-13.1) compared with patients who had a staining score of 0. Even after adjustment for clinical variables and pathological findings, the HR for IgG score of 1 or 2 was higher than that for an IgG score of 0, and it was respectively 2.22 (0.71-7.00) and 3.76 (1.27-11.2). Conclusions More intense linear IgG staining is associated with a higher HR for renal death, which suggests that linear immunofluorescent staining for IgG may be a prognostic indicator in patients with diabetic nephropathy.
    Diabetes Research and Clinical Practice. 10/2014;
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    ABSTRACT: Transcatheter arterial embolization (TAE) has become a therapeutic option for symptomatic polycystic kidney disease (PKD) and polycystic liver disease (PLD). However, factors affecting survival with renal TAE remain unknown.
    Journal of nephrology. 09/2014;
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    ABSTRACT: Background/Aims: This study aims to identify current risk factors for developing dialysis-related amyloidosis using carpal tunnel syndrome (CTS) as proxy for general amyloidosis. Methods: The cohort consisted of 166,237 patients on dialysis (mean age 66.1 ± 12.4 years; mean dialysis vintage 7.2 ± 6.4 years) who could be followed for a year between 2010 and 2011. Of these, 2,157 (1.30%) needed first-time CTS surgery during the study period. Odds ratios (ORs) for CTS were calculated at a 95% confidence interval (95% CI) after adjusting for age, gender, primary kidney disease, history of smoking, history of hypertension vintage, dialysis modality, use of high-flux membrane, body mass index, serum albumin, Kt/V, normalized protein catabolic rate, C-reactive protein, pretreatment β2-microglobulin (β2MG), and β2MG clearance. Results: Adjusted ORs of first-time CTS for vintages 10-15, 15-20, 20-25 (referent), 25-30, and >30 years were, respectively, 0.18 (0.12-0.26), 0.43 (0.31-0.62), 1.00, 2.37 (1.64-3.40), and 3.87 (2.52-5.93). Adjusted ORs for ages 40-50, 50-60 (referent), 60-70, 70-80, and >80 were 0.53 (0.30-0.94), 1.00, 1.89 (1.41-2.52), 1.52 (1.08-2.14), and 1.04 (0.60-1.80). Female gender, low serum albumin, and diabetic nephropathy were also associated with CTS. Pretreatment serum β2MG and β2MG clearance <80% were not significant, although β2MG clearance >80% was negatively associated with CTS [OR 0.34 (0.13-0.90)]. Conclusion: ORs of first-time CTS almost doubled with every 5-year increase in dialysis vintage. ORs of CTS were highest for patients aged 60-70. Other factors associated with CTS were gender, serum albumin, and diabetic nephropathy. β2MG clearance >80% may decrease the incidence of CTS. © 2014 S. Karger AG, Basel.
    American Journal of Nephrology 05/2014; 39(5):449-458. · 2.62 Impact Factor
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    ABSTRACT: Antineutrophil cytoplasmic antibody (ANCA)-associated glomerulonephritis is a vasculitis affecting the glomerular capillaries and small renal arteries. Although crescent formation has been reported to be characteristic of this condition, the significance of coexisting vasculitis affecting the small renal arteries has not been investigated. Fifty patients with ANCA-positive rapidly progressive glomerulonephritis whose renal biopsy specimens contained arterioles and/or interlobular arteries were retrospectively evaluated. Cellular crescents and/or necrotizing glomerulonephritis were noted in all 50 patients. Ten patients had vasculitis of the small renal arteries (group A) and 40 patients were without such vasculitis (group B). The clinical features of these 2 groups were compared. Group A comprised 4 patients who had granulomatosis with polyangiitis (GPA) and 6 with microscopic polyangiitis (MPA), while group B included 1 patient with GPA and 39 with MPA. No patient in either group had eosinophilic granulomatosis with polyangiitis. The C-reactive protein (CRP) level was significantly higher in group A compared with group B (11.58 ± 6.19 vs 2.7 ± 3.55 mg/dl, p < 0.05), and pulmonary involvement was more frequent in group A than group B (80% vs 37.5%, p < 0.05). In patients with ANCA-positive glomerulonephritis, vasculitis of small renal arteries may be associated with systemic vasculitis (including pulmonary involvement) because of elevated CRP, a systemic inflammatory marker related to overproduction of interleukin 6.
    The Journal of Rheumatology 04/2014; · 3.26 Impact Factor
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    ABSTRACT: A 14-year-old Japanese girl was admitted to our institution for the evaluation of renal dysfunction. Her serum creatinine was 1.1 mg/dL, proteinuria was 1.5 g/day, the urine sediment contained numerous erythrocytes per high-power field, and she was positive for myeloperoxidase anti-neutrophil cytoplasmic antibody (MPO-ANCA). Proteinuria was first noted at the age of 12 years. Renal biopsy showed crescentic glomerulonephritis with slight immunoglobulin A (IgA) deposition. A diagnosis of ANCA-associated vasculitis was made. Immunosuppressive therapy was initiated, including steroid pulse therapy and intravenous cyclophosphamide pulse therapy, but hemodialysis was required after 6 years. Eight months after the patient became anuric and her MPO-ANCA titer became negative, living-related donor kidney transplantation was done from her mother. ANCA became slightly positive 2 years later, but the patient remains stable without proteinuria or hematuria at 4 years after surgery. This case suggests that kidney transplantation can be performed successfully for a patient with refractory childhood-onset ANCA-associated vasculitis, and that remission of vasculitis associated with ANCA negativity at transplantation may contribute to a better renal prognosis in this patient.
    Modern Rheumatology 03/2014; · 1.72 Impact Factor
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    ABSTRACT: Hepatic transcatheter arterial embolization (TAE) has become an accepted treatment option for patients with symptomatic autosomal dominant polycystic kidney disease (ADPKD) who also have polycystic liver disease and who are not good candidates for surgery. However, indications for TAE and long-term outcome with it are still unclear. Retrospective cohort study. Symptomatic patients with ADPKD with polycystic liver disease who underwent hepatic TAE, June 2001 to December 2012, at Toranomon Hospital and whose liver volume data were available were studied (N=244; 56% on dialysis therapy, none with kidney transplants). Mean age was 55±9 (SD) years, and mean liver volumes were 8,353±2,807 and 6,626±2,485cm(3) in men and women, respectively. Target arteries were embolized from the periphery using platinum microcoils. Sex-specific quartiles (6,433, 8,142, and 9,574cm(3) in men and 4,638, 6,078, and 8,181cm(3) in women) of total liver volume pretreatment. All causes of mortality were obtained from medical records, followed up until July 31, 2013. Laboratory values were measured before TAE and 1, 3, 6, and 12 months after. Organ volumes were measured pretreatment, then 6 and 12 months after, by summing the products of the organ areas traced in each computed tomographic image. Liver/cyst volume decreased to 94.7% (95% CI, 93.5%-95.8%) at 6 months and 90.8% (95% CI, 88.7%-92.9%) at 12 months of pretreatment volumes. Serum protein and hematocrit values improved significantly without liver damage. Survival was significantly better for patients with liver volume≤9,574cm(3) (men) and≤8,181cm(3) (women) than for those with larger livers (5-year survival, 69% and 48%; P=0.02). Infection and liver failure caused most deaths, especially in patients with larger livers. Referral bias and lack of control group. Hepatic TAE appears to be a safe and less invasive option for patients with symptomatic polycystic liver, especially those contraindicated for surgical treatment (eg, with malnutrition or on dialysis therapy), improving both hepatic volume and nutrition.
    American Journal of Kidney Diseases 03/2014; · 5.29 Impact Factor
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    Clinical and Experimental Nephrology 01/2014; · 1.25 Impact Factor
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    ABSTRACT: Background Hepatic transcatheter arterial embolization (TAE) has become an accepted treatment option for patients with symptomatic autosomal dominant polycystic kidney disease (ADPKD) who also have polycystic liver disease and who are not good candidates for surgery. However, indications for TAE and long-term outcome with it are still unclear. Study Design Retrospective cohort study. Setting & Participants Symptomatic patients with ADPKD with polycystic liver disease who underwent hepatic TAE, June 2001 to December 2012, at Toranomon Hospital and whose liver volume data were available were studied (N = 244; 56% on dialysis therapy, none with kidney transplants). Mean age was 55 ± 9 (SD) years, and mean liver volumes were 8,353 ± 2,807 and 6,626 ± 2,485 cm3 in men and women, respectively. Target arteries were embolized from the periphery using platinum microcoils. Predictors Sex-specific quartiles (6,433, 8,142, and 9,574 cm3 in men and 4,638, 6,078, and 8,181 cm3 in women) of total liver volume pretreatment. Outcomes All causes of mortality were obtained from medical records, followed up until July 31, 2013. Measurements Laboratory values were measured before TAE and 1, 3, 6, and 12 months after. Organ volumes were measured pretreatment, then 6 and 12 months after, by summing the products of the organ areas traced in each computed tomographic image. Results Liver/cyst volume decreased to 94.7% (95% CI, 93.5%-95.8%) at 6 months and 90.8% (95% CI, 88.7%-92.9%) at 12 months of pretreatment volumes. Serum protein and hematocrit values improved significantly without liver damage. Survival was significantly better for patients with liver volume ≤ 9,574 cm3 (men) and ≤8,181 cm3 (women) than for those with larger livers (5-year survival, 69% and 48%; P = 0.02). Infection and liver failure caused most deaths, especially in patients with larger livers. Limitations Referral bias and lack of control group. Conclusions Hepatic TAE appears to be a safe and less invasive option for patients with symptomatic polycystic liver, especially those contraindicated for surgical treatment (eg, with malnutrition or on dialysis therapy), improving both hepatic volume and nutrition.
    American Journal of Kidney Diseases 01/2014; · 5.29 Impact Factor
  • Kidney International 01/2014; 85(1):217. · 8.52 Impact Factor
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    ABSTRACT: We report a 68-year-old Japanese man with end-stage renal failure requiring hemodialysis and chronic disseminated intravascular coagulation (DIC) related to thrombosis in an aortic aneurysm. He had undergone graft replacement for the dissection of the ascending and descending thoracic aorta in 1990 and 2002, respectively. Computed tomography disclosed an aneurysm with thrombosis in the residual aorta adjacent to the graft anastomosis. DIC was diagnosed based on elevation of serum fibrinogen degradation products while his activated partial thromboplastin time, prothrombin time and fibrinogen level were normal. In 2008, hemodialysis was initiated for end-stage renal failure. Dialysis was performed without administration of an anticoagulant because his activated clotting time (ACT) was prolonged to 150-180 s. Thereafter, stable hemodialysis continued without clotting in the dialysis circuit until 2013. If monitoring of ACT can be done, hemodialysis without anticoagulation may be a therapeutic option in such patients.
    Case reports in nephrology and urology. 01/2014; 4(1):25-30.
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    ABSTRACT: Background Although anti-human leukocyte antigen (HLA) antibodies (DSA) is associated with graft loss, 3 things remain unclear: whether the duration and strength of DSA affect renal function; what mean fluorescence intensity (MFI) cut-off should be used; and whether the DSA effect is additive in case of multiple DSAs. Methods A study was made of 63 patients who received living donor kidney transplants with clonal deletion protocol and were followed up for 18 months with reduced doses of immunosuppressants. DSA was tested for monthly, using Luminex Mixed and Single Antigen beads (One Lambda, Inc., Canoga Park, CA, USA). Decrease of estimated glomerular filtration rate (eGFR) was obtained at baseline and 18 months after transplantation. Association of renal damage and DSAs was compared using several DSA models with several MFI cut-offs. Results Additive DSA models always showed better association with renal damage than comprehensive models. When calculating the DSA effect in additive models, “proxy-area under the curve” (AUC)—a triangular approximation of the actual AUC—showed better association with renal damage than did DSA duration (R2 = 0.105 vs 0.087). Adjusting for other factors, 27% of the variation of GFR change was explained by proxy-AUC. No significant change of association occurred if the MFI cut-off level changed from 1000 to 3000. Conclusion Our results support the association of DSA with development of longitudinal renal damage. The clinical interpretation may be similar at MFI cut-offs of 1000, 2000, and 3000. An additive DSA effect may be expected in patients with multiple DSAs. Our study suggests the importance of frequently checking for DSA and reducing their MFI value to minimize renal damage by the antibodies.
    Transplantation Proceedings 01/2014; 46(1):75–80. · 0.95 Impact Factor
  • The Journal of Rheumatology 11/2013; 40(11):1923-4. · 3.26 Impact Factor
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    ABSTRACT: A new classification of diabetic nephropathy was reported by Tervaert et al., but the association between pathological findings and the clinical outcomes remains unclear. Among 310 patients with diabetes mellitus who underwent renal biopsy from March 1985 to January 2010 and were confirmed to have diabetic nephropathy according to the Tervaert's classification, 205 patients were enrolled in this study. Cox proportional hazard regression analysis was used to calculate the hazard ratio (HR) and 95% confidence interval (CI) for death-censored renal death. Each regression analysis employed two levels of multivariate adjustment. After adjustment for age, gender, estimated glomerular filtration rate, type of diabetes, urinary protein excretion, systolic blood pressure, body mass index, HbA1c, diabetic retinopathy and red blood cells in urinary sediment at the time of renal biopsy, compared with glomerular class IIA, the HRs for death-censored renal death of glomerular classes I, IIB, III and IV were 0.23 (95% CI: 0.04-1.34), 2.15 (0.91-5.09), 4.35 (1.86-10.18) and 3.41 (1.38-8.45), respectively. Also, compared with an interstitial fibrosis and tubular atrophy score 1 group, HRs for score 0 group, score 2 group and score 3 group were 0.07 (0.008-0.57), 2.07 (0.92-4.67) and 4.95 (2.04-11.97), respectively. The progression of glomerular, tubulointerstitial and vascular lesions was associated with higher HRs for renal death. These results suggest the clinical utility of Tervaert's pathological classification.
    Nephrology Dialysis Transplantation 10/2013; · 3.37 Impact Factor
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    ABSTRACT: Abstract We report a Japanese woman with variegate porphyria accompanied by amyloid A (AA) amyloidosis. Arthropathy involving multiple joints occurred at 35 years old and persisted. C-reactive protein was 4.0 mg/dL, but rheumatoid factor was negative. Radiographs did not reveal any loss or narrowing of the joint spaces. Two years later, blister formation after sun exposure and reddish urine were first noted. At the age of 45 years, she developed abdominal pain, nausea, vomiting and seizures. After administration of phenobarbital, reddish urine was noted and muscular weakness progressed to atonic quadraparesis. Porphyria attack was diagnosed from high urinary levels of ∂ aminolevulinic acid and porphobilinogen. At the age of 47 years, hemodialysis was started. At the age of 49 years, progression of her gastrointestinal event resulted in death. Autopsy showed massive deposits of AA amyloidosis in various organs, including the kidneys and digestive tract. Thus, amyloid deposition may have contributed to both end-stage renal failure and her gastrointestinal symptoms. This is the first report about the coexistence of porphyria and AA amyloidosis. Chronic inflammation related to this patient's seronegative arthropathy, although atypical for porphyria, might have contributed to the development of AA amyloidosis.
    Amyloid: the international journal of experimental and clinical investigation: the official journal of the International Society of Amyloidosis 10/2013; · 2.51 Impact Factor
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    ABSTRACT: The quality of life (QOL) of patients with autosomal dominant polycystic kidney disease (ADPKD) has not been investigated well. This study was performed to clarify the QOL of patients with ADPKD and to identify factors that affected their QOL. The present cross-sectional study is part of a prospective observational study on the QOL of ADPKD patients. Patients with ADPKD who were referred to Toranomon Hospital between March 2010 and November 2012 were enrolled. The short form-36 (SF-36) questionnaire and our original 12-item questionnaire were used to evaluate QOL. We analyzed the results of the questionnaire survey and then investigated correlations between QOL and clinical features. A total of 219 patients (93 men and 126 women) were enrolled and their mean age was 55.1+/-10.8 years. There were 108 patients on dialysis. The SF-36 scores (PCS, MCS, and RCS) of all patients were significantly lower than the mean scores for the Japanese population. Stepwise multiple regression analysis demonstrated that Hb, serum Alb, ascites, and cerebrovascular disease all had a significant influence on the PCS, while mental disease had a significant influence on the MCS and serum Alb significantly influenced the RCS. The total liver and kidney volume (TLKV) and the dialysis status were not significantly associated with any of the SF-36 scores by multiple regression analysis, but TLKV was closely correlated with abdominal distention and distention had an important influence on QOL. Pain, sleep disturbance, heartburn, fever, gross hematuria, and anorexia also affected QOL, but these variables were not correlated with TLKV. Several factors influence QOL, so improving symptoms unrelated to TLKV as well as reducing abdominal distention can improve the QOL of ADPKD patients.
    BMC Nephrology 08/2013; 14(1):179. · 1.64 Impact Factor
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    ABSTRACT: Thrombotic thrombocytopenic purpura (TTP) is frequently associated with renal abnormalities, but there have been few reports about renal abnormalities in patients with hereditary TTP. In particular, little is known about the long-term prognosis of patients with childhood-onset congenital TTP. We report a Japanese patient with congenital TTP (Upshaw--Schulman syndrome) who was followed for 19 years after initiation of hemodialysis when he was 22 years old. At the age of 6 years, the first episode of purpura, thrombocytopenia, and proteinuria occurred without any precipitating cause. He underwent living-related donor kidney transplantation from his mother, but the graft failed after 5 months due to recurrence of TTP. Even after resection of the transplanted kidney and resumption of regular hemodialysis, TTP became refractory to infusion of fresh frozen plasma (FFP). Therefore, splenectomy was performed and his disease remained in remission for 10 years. However, TTP recurred at the age of 39 years. Plasma activity of ADAMTS13 (a disintegrin and metalloprotease with thrombospondin type I domain 13) was less than 3%, while ADAMTS13 inhibitor was not detected (< 0.5 Bethesda units/mL). The patient died suddenly after hemodialysis at the age of 41 years. Subsequent genetic analysis of this patient and his parents revealed two different heterozygous mutations of ADAMTS13, including a missense mutation in exon 26 (c.T3650C causing p.I1217T) inherited from his father and a missense mutation in exon 21 (c.G2723A causing p.C908Y) inherited from his mother. The former mutation has not been detected before in Japan, while the latter mutation is common in Japan. A retrospective review showed that serum C3 levels were consistently low while C4 levels were normal during follow-up, and C3 decreased much further during each episode of TTP. Congenital TTP was diagnosed from the clinical, biochemical, and genetic findings. Infusion of FFP controlled each thrombotic episode, but the effect was limited and of short duration. Review of the complement profile in this patient suggested that a persistently low serum C3 level might be associated with refractory TTP and a worse renal prognosis.
    BMC Nephrology 07/2013; 14(1):156. · 1.64 Impact Factor
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    ABSTRACT: BACKGROUND: Previous studies on membranoproliferative glomerulonephritis (MPGN) and cryoglobulinemic glomerulopathy (CG) were based upon case series that were performed before hepatitis C virus (HCV) infection was routinely investigated. Therefore, it remains unknown how far HCV contributes to MPGN or CG, and there have only been a few reports about HCV-negative idiopathic MPGN. PATIENTS AND METHODS: Thirty-five patients with MPGN diagnosed by renal biopsy who underwent examination for HCV infection at our institute between 1990 and 2008 were recruited for this study. Patients with HCV infection at presentation were included, but patients with complications such as underlying lymphoproliferative disorders, autoimmune diseases like lupus nephritis, infection, and liver disease due to hepatitis B virus or alcohol abuse were excluded. A total of 35 patients were enrolled and they were divided into two groups according to the presence/absence of circulating cryoglobulins (cryo). The 23 patients who had cryo-negative and HCV-negative idiopathic MPGN were divided into subgroups with type 1 and type 3 disease. RESULTS: In the cryo-positive group (n = 9), 7 patients were positive for HCV infection, while 2 patients were negative. In the cryo-negative group (n = 26), 3 patients were positive for HCV infection, while 23 patients were negative (idiopathic MPGN). Compared with the cryo-negative group, the cryo-positive group had several characteristics such as more severe thrombocytopenia, higher serum immunoglobulin (Ig)G and IgM levels, lower levels of hemolytic complement (CH50) and complement component (C)4, predominant IgM staining, and type 1 histology. Patients with cryo-negative and HCV-negative 'idiopathic' MPGN showed predominant staining for IgG in both type 1 and type 3 cases, unlike the predominant staining for IgM in the cryo-positive group. Compared with type 3 cases, type 1 cases had a younger age, lower levels of CH50, C3 and C4, and less proteinuria. In the cryo-positive group, 4 patients (44.4 %) died, with death from B cell lymphoma and liver failure in 2 patients each, while 1 patient (8 %) developed end-stage renal failure requiring dialysis. In contrast, all patients in the cryo-negative group remained alive during follow-up, although 4 patients (2 type 1 cases and 2 type 3 cases) required dialysis. CONCLUSION: Cryo-positive MPGN shows a close relationship with HCV infection and IgM, resulting in a poor prognosis. Cryo-negative and HCV-negative idiopathic MPGN has a close relationship with IgG staining, and type 1 cases feature characteristics such as a younger age, more severe hypocomplementemia, and less proteinuria than in type 3 cases.
    Clinical and Experimental Nephrology 05/2013; · 1.25 Impact Factor
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    ABSTRACT: A 34-year-old Japanese woman with anorexia nervosa (AN) of a body mass index (BMI) of 11.0kg/cm(2) was admitted to our hospital for assessment of renal dysfunction with a serum creatinine of 1.8mg/dL and hypokalemia (3.0mEq/L). Renal biopsy showed chronic interstitial fibrosis with hypertrophy of the juxtaglomerular apparatus. Iliac crest biopsy was performed because of a severe decrease in bone mineral density. It showed active resorption at the periosteal and endosteal surfaces of cortical bone by numerous osteoclasts, as well as bone island formation in cancellous bone due to marked decrease of trabecular connections. A dynamic study using double labeling showed that mineralization of cancellous bone adjacent to cortical bone occurred between the first and second labelings, but did not occur between the second labeling and osteoid formation during the 28-day period before biopsy, which implied that the mineralization was related to promotion of food intake after hospitalization, while the lack of mineralization was due to poor food intake outside hospital. Empty lacunae that indicated the death of osteocytes were seen. Because her bone mass and kidney injury improved after weight gain and normokalemia were achieved by a highly nutritious diet, malnutrition with hypokalemia may have a negative influence on bone formation due to impaired mineralization and may activate bone resorption by osteoclasts secondary to the formation of empty lacunae. This is the first report about the histological features of premenopausal osteoporosis in a patient with AN and kidney injury.
    Bone 05/2013; · 4.46 Impact Factor

Publication Stats

296 Citations
230.24 Total Impact Points

Institutions

  • 2014
    • University of California, Los Angeles
      Los Angeles, California, United States
  • 2005–2014
    • Toranomon Hospital
      Edo, Tōkyō, Japan
  • 2011–2013
    • Los Angeles Biomedical Research Institute at Harbor-UCLA Medical Center
      • Department of Medicine
      Torrance, California, United States
  • 2012
    • Harbor-UCLA Medical Center
      Torrance, California, United States