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Abstract

Y. Mori, H. Yamashita, Y. Umeda,Y.U. Uchiyama-Tanaka, A. Nose,N. Kishimoto, Y. Kijima, T. Nagata,M. Mori, H. Matsubara, H. Yoshida and T. Iwasaka

¹Department of Medicine II, Kansai Medical University, Osaka, and
²Department of Clinical Laboratory Medicine, Fukui Medical University,
Fukui, Japan
An otherwise healthy 20-year-old woman presented with an erythematous rash on her face as well as arthralgia and anemia. She also had systemic edema, proteinuria and hypertension. Laboratory data on admission showed hypocomplementemia, human parvovirus B19 (HPV) DNA and both immunoglobulin (Ig) M and IgG antibodies to HPV in her serum. Renal biopsy specimens showed features of endocapillary glomerulonephritis under light microscopy. Electron microscopy showed massive subendothelial electron-dense deposits. No cause was probable other than immune complex-mediated glomerulonephritis associated with HPV infection. In a review of this and similar cases reported in the literature, several characteristic features come to light: female dominance, onset in the second or third decade of life, hypocomplementemia, histologic renal endocapillary and/or mesangioproliferative glomerulonephritis with subendothelial deposits and spontaneous recovery.

Abstract

N. Kishimoto, Y. Mori, T. Nishiue, Y. Shibasaki, O. Iba, A. Nose, Y. Uchiyama-Tanaka, H. Masaki, H. Matsubara and T. Iwasaka

¹Renal Division and ²Cardiovascular Center, Department of Medicine II, Kansai Medical University, Osaka, Japan
Background: An accessible non-invasive method for evaluating renal regional blood flow in real time is highly desirable in the clinical setting. Recent progress in ultrasonography with microbubble contrast has allowed quantification of regional blood flow in animal models. Aims: Goal of this study was to establish a convenient contrast-enhanced harmonic ultrasonography (CEHU) method for evaluating renal cortical blood flow in humans. Methods: We carried out intermittent second harmonic imaging in 9 healthy volunteers. Pulse interval was progressively decreased from 4 s ? 0.2 s during continuous venous infusion of the microbubble contrast agent. Results: Pulse interval versus CEHU-derived acoustic intensity plots provided microbubble velocity (MV) and fractional vascular volume (FVV) during renal cortical perfusion in humans. Low-dose dopamine infusion (2 mg/min/kg) resulted in a significant increase in MV which correlated well with the increase in total renal blood flow (RBF) determined by a conventional study of p-aminohippurate clearance (CPAH) (r = 0.956, p < 0.0001). Although FVV was not significantly increased, alterations in CEHU-derived renal cortical blood flow calculated by the products of MV and FVV were also correlated with alterations in total RBF (r = 0.969, p < 0.0001). Thus, low-dose dopamine infusion increases renal cortical blood flow observed in CEHU, mainly by increasing MV. Conclusions: The present study shows that renal cortical blood flow in humans can be measured non-invasively by CEHU and that CEHU can be used for quantitatively evaluating changes induced by a therapeutic agent such as dopamine in flow velocity and in FVV.

Abstract

Y. Uchiyama-Tanaka, Y. Mori, N. Kishimoto, A. Nose, Y. Kijima, T. Nagata, Y. Umeda, H. Masaki, H. Matsubara and T. Iwasaka

Renal Division and ²Cardiovascular Center, Department of Medicine II, Kansai Medical University, Osaka, Japan
We describe the case of a 51-year-old man with hepatitis C virus (HCV) infection and a 3-month history of facial edema. Laboratory tests upon admission for renal biopsy showed normal renal function and normocomplementemia. Serum HCV antibody (Ab) and cryoglobulin were positive. Renal biopsy specimens showed features of membranous glomerulonephritis. The likely cause was immune complex-mediated glomerulonephritis associated with HCV infection. Reports of similar cases in the literature show the normocomplementemia and negative or slightly positive cryoglobulins observed in our case as well as seropositivity for circulating immune complexes containing HCV RNA. In our case, electron microscopic examination of the subepithelial glomerular lesions revealed massive virus-like particles within unusual multilayers of electron-dense deposits (EDDs), suggesting the existence of HCV in the glomeruli. In the addition to the unique histopathological feature the presence of La/SS-B antibody in his serum indicated an abnormal immune response associated with HCV. We advise him to undergo the therapy with new type of IFN such as pegIFN-a2a and/or anti-viral agent like ribavirin to achieve clinical and histopathological improvement.

Abstract

N. Kishimoto, Y. Mori, H. Yamahara, Y. Kijima, A. Nose, Y. Uchiyama-Tanaka, T. Tokoro, T. Nagata, Y. Umeda, N. Takahashi, H. Yoshida and H. Matsubara

¹Division of Cardiology and Nephrology, Department of Medicine, Kyoto Prefectural University of Medicine, Kyoto, ²Department of Medicine II, Kansai Medical University, Osaka, and ³Division of Nephrology, University of Fukui Hospital, Fukui, Japan
Renal deterioration often occurs in cases of infectious endocarditis (IE), but, IE- associated nephritis with rapidly progressive glomerulonephritis (RPGN) is rare. Patients with severe infection (e.g., IE) sometimes show positivity for cytoplasmic antineutrophil cytoplasmic antibodies (C-ANCA). Therefore, diagnosis and treatment are very difficult in cases of RPGN with IE and positivity for C-ANCA. Such cases are rare, only 12 have been reported in the English literature. Herein, we describe the case of a 50-year-old man who presented with RPGN with IE and tested positively for C-ANCA. He was referred to our hospital because of leg edema, purpura and renal dysfunction. Laboratory tests revealed serum creatinine elevation and positivity for C-ANCA and proteinase 3-specific (PR3)-ANCA. RPGN and acute renal failure were diagnosed. Hemodialysis and steroid therapy were started. Streptococcus oralis was isolated by blood culture. Transthoracic echocardiography revealed grade III mitral valve insufficiency with two vegetations. Therefore, IE was diagnosed. The steroid therapy was stopped, and antibiotic therapy was begun. Because there was no improvement, surgical therapy was performed. The operation was successful, but the patient died of brain hemorrhage. Our experience in this case indicates C/PR3-ANCA positive RPGN must be ruled out in patients with infectious disease, particularly IE, together with renal symptoms, and renal biopsy should be performed. Correspondence to:
Y. Mori, MD Division of Cardiology and Nephrology Department of Medicine Kyoto Prefectural University of Medicine 465 Kajii-cho Kawaramachi-Hirokoji, Kamigyo-ku, Kyoto, 602-8566, Japan
Email: [email protected]

Abstract

T. Kusaba, T. Hatta, K. Sonomura, Y. Mori, T. Tokoro, T. Nagata, Y. Umeda, K. Nagata, T. Yasuda, T. Sato, K. Kimura and H. Matsubara

¹Division of Hypertension and Nephrology, Department of Medicine, Kyoto Prefectural University of Medicine, Kyoto, ²Division of Internal Medicine II, Kansai Medical University, Osaka, ³Division of Nephrology and Hypertension, Department of Internal Medicine, St. Marianna University School of Medicine, Kanagawa and ⁴Division of Cardiology and Vascular Regenerative Medicine, Department of Medicine, Kyoto Prefectural University of Medicine, Kyoto, Japan
Idiopathic nodular glomerulosclerosis (ING) is characterized as diffuse nodular glomerulosclerotic lesions, closely resembling Kimmelstiel-Wilson lesions without diabetic mellitus. We report here three Japanese cases of ING and discuss the previous reports. The patients were 75-, 48- and 84-year-old males with a history of long-term hypertension. Laboratory examination revealed moderate proteinuria and mild renal dysfunction. Diabetes mellitus was excluded by repeated clinical and laboratory investigations. Renal histology revealed nodular glomerulosclerosis, and both afferent and efferent arteriolosclerosis in all patients. In electron microscopy, the glomerular basement membrane was markedly thick in all patients. A low-protein diet and potent anti-hypertensive treatment using angiotensin-converting enzyme inhibitors were initiated in all patients and urinary protein excretion significantly reduced without the progression of renal dysfunction. We reviewed 42 previously reported cases and our three cases. The analysis revealed that common clinical features of ING are being male (82.2%) of relatively advanced age (mean age 61.3 years), with hypertension (82.2%), mild renal dysfunction (mean serum creatinine 2.9 mg/dl) and moderate urinary protein excretion (mean 4.05 g/day). Common histopathological findings of ING are nodular glomerulosclerosis (100%), arterio-arteriolosclerosis (91.2 and 89.7%) and glomerular basement membrane thickening (85.7%). In conclusion, ING is one of the phenotypes of arteriosclerotic renal disease without diabetes mellitus. Severe arterio-arteriolosclerosis may contribute to the progression to glomerular nodular formation in ING. The combination of renin-angiotensin system inhibition and a low protein diet can be beneficial for the reduction of urinary protein excretion.Correspondence to:
T. Kusaba, MD Division of Nephrology and Hypertension Department of Internal Medicine Kyoto Prefectural University of Medicine 456 Kajii-cho, Kamigyo-ku, Kyoto-city, 602-8566 Japan
Email: [email protected]

Abstract

Y. Uchiyama-Tanaka, Y. Mori and H. Matsubara

Department of Medicine, Division of Cardiology and Nephrology, Kyoto Prefectural University of Medicine, Kyoto, Japan
Correspondence to:
Y. Uchiyama-Tanaka, MD, PhD Department of Medicine Division of Cardiology and Nephrology Kyoto Prefectural University of Medicine Kamigyoku, Kyoto 606-8507, Japan
Email: [email protected]

Abstract

T. Kusaba, T. Hatta, T. Kimura, K. Sonomura, S. Tanda, N. Kishimoto, H. Kameyama, M. Okigaki, Y. Mori, N. Ishigami, T. Mizuno, M. Nakagawa and H. Matsubara

¹Division of Hypertension and Nephrology, ²Division of Cardiology and Vascular Regenerative Medicine, Department of Medicine, and ³Research Institute for Neurological Diseases and Geriatrics, Department of Neurology and Gerontology, Kyoto Prefectural University of Medicine, Kyoto, Japan
Cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL) is a hereditary systemic arteriopathy presenting with migraines, mood disorders, focal neurologic deficits, recurrent ischemic attacks and dementia in young adults. The genesis of this disease relates to missense mutation of the Notch3 gene. We report here a newly identified CADASIL patient and discuss unique vascular lesions observed in the kidney. A 64-year-old female was admitted to our hospital for the investigation of proteinuria, hematuria and progressive neurological abnormalities. Her mother and brother died of cerebral infarction at a relatively young age despite a lack of apparent risk factors for arteriosclerosis. Over the past 4 months before admission, she had suffered from frequent transient ischemic attacks despite appropriate antiplatelet therapy. Blood examination revealed mild renal insufficiency and urinalysis revealed moderate protein excretion and dysmorphic hematuria. Magnetic resonance imaging of the brain revealed multiple infarcts and leukoencephalopathy. Histopathological analysis of the kidney revealed focal segmental mesangial proliferation, the loss and degeneration of arterial medial smooth muscle cells and arterial intimal thickening. Immunofluorescence analysis of glomeruli revealed IgA deposition in the mesangial area. Electron microscope analysis revealed electron-dense deposition also in the mesangial area. In addition, granular osmophilic material (GOM) was observed in the extraglomerular mesangial area and around the vascular smooth muscle cells. Genetic analysis of Notch3 revealed an R141C missense mutation and she was diagnosed with CADASIL complicated with IgA nephropathy. In immunohistological analysis, Notch3 stains were positive in vascular smooth muscle cells of the interlobular arteries and both afferent and efferent arterioles, and weak in the glomerular mesangial area. Antihypertensive treatment using angiotensin II receptor blocker and a low protein diet were initiated, and her urinary protein excretion decreased to 0.2 g/day. However, due to the progression of her neurological abnormalities, she became socially withdrawn. In CADASIL, GOM, abnormal accumulation of Notch3 ectodomain, is thought to induce the degeneration and loss of vascular smooth muscle cells and subsequent intimal thickening. Analysis of our cases provided that these morphological abnormalities were also observed in the CADASIL patient kidney.Correspondence to:
T. Kusaba, MD Division of Nephrology and Hypertensio Department of Internal Medicine Kyoto Prefectural University of Medicine 456 Kajii-cho Kamigyo-ku Kyoto-city, 602-8566, Japan
Email: [email protected]

Abstract

Clinical Nephrology, Vol. 75 – No. 4/2011 (384-388)

A case of Goodpasture syndrome positive for anti-GBM antibody and MPO-ANCA complicated by a variety of serious infections

C. Sakoda¹*, T. Kusaba¹*, T. Adachi², K. Sonomura¹, T. Kimura¹, M. Nakayama¹, N. Kishimoto¹, H. Nakagawa¹, M. Okigaki¹, T. Hatta², H. Matsubara¹ and Y. Mori¹

¹Division of Cardiology and Nephrology, Department of Medicine, Kyoto Prefectural University of Medicine, Kyoto, and ²Division of Nephrology, Omihachiman Community Medical Center, Shiga, Japan
A 62-year-old female was admitted to our hospital for investigation of acute progressive renal insufficiency and a systemic inflammatory reaction, despite treatment with several antibiotics. Laboratory data revealed severe renal insufficiency and positive titers for the myeloperoxidase anti-neutrophil cytoplasmic and anti-glomerular basement membrane antibodies. The deterioration of her general status did not allow us to perform the renal biopsy. Although corticosteroid therapy, hemodialysis, and plasma exchange were concomitantly initiated, pulmonary hemorrhage occurred several days after admission. Mechanical ventilation support was provided and continuous hemodiafiltration was carried out, following which the respiratory failure improved immediately. However, she developed clinical depression and suicidal behavior under the intensive therapy. Therefore, plasma exchange was discontinued and corticosteroid was tapered as quickly as possible. Four months after admission, platelet transfusion and short-term mechanical ventilation support improved the pulmonary hemorrhage; however, her mental status deteriorated despite psychiatric consultation and treatment with a tranquilizer. Thereafter, severe and serious systemic infection due to various pathogens including Staphylococcus aureus, Cytomegalovirus, Pneumocystis jiroveci, Pseudomonas aeruginosa, and Bacteroides recurred, and she died from systemic invasive aspergillosis (IA). We suspected severe immunosuppression caused by various factors, such as predonisolone administration, chronic renal failure on maintenance hemodialysis, depression, and malnutrition due to chronic inflammation and granulocytopenia as a side effect of ganciclovir. When treating rapidly progressive glomerulonephritis, immunosuppressive status should be carefully monitored regarding not only the dosage of therapeutic regimen but also the mental health status and nutrition of the patient.Correspondence to:
C. Sakoda, MD
Division of Cardiology and Nephrology
Department of Medicine
Kyoto Prefectural University of Medicine
465 Kajii-cho, Kawaramachi-Hirokoji
Kamigyo-ku Kyoto 602-8566, Japan
Email: [email protected]

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