Fumi Takemoto

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.

Induction of Anti-Carbonic-Anhydrase-II Antibody Causes Renal Tubular Acidosis in a Mouse Model of Sjögren’s Syndrome

Background/Aim: We recently reported that renal tubular acidosis (RTA) in Sjögren’s syndrome (SjS) is associated with high titers of an autoantibody against carbonic anhydrase (CA) II, an important enzyme in renal acid-base regulation. The purpose of this study was to determine whether a CA-II antibody could cause RTA in a mouse model of SjS. Methods: PL/J mice were immunized with human CA II to induce CA II antibody formation, whereas controls were injected with phosphate-buffered saline and adjuvant. After 6 weeks, anti-CA-II antibody titers were measured, then ammonium chloride was administered orally for 1 week to detect any acidification defect. Results: CA-II-immunized mice showed higher anti-CA-II antibody titers than control mice. Pathologically, lymphocytic and plasma cell infiltration was seen in the salivary glands and kidneys of CA-II-immunized mice, but not in controls. On acid loading, blood pH and urine pH decreased in both groups of mice, but the slope of urine pH versus blood pH was less steep in the CA-II-immunized mice, suggesting that these mice had an impaired ability to reduce their urine pH in the face of metabolic acidosis. Conclusion: CA-II-immunized mice had a urinary acidification defect, which may be similar to that seen in patients with SjS.

Infected hepatic and renal cysts: differential impact on outcome in autosomal dominant polycystic kidney disease.

Background: Infected cysts are a frequent and serious complication of autosomal dominant polycystic kidney disease. Such infections are classified into those affecting hepatic cysts and those affecting renal cysts. The purpose of this study was to compare the clinical course of infected hepatic cysts with that of infected renal cysts in patients with autosomal dominant polycystic kidney disease. Methods: We analyzed 43 patients referred to us for additional treatment of severely infected cysts between January 2004 and December 2006. All patients who required further treatment in addition to antibiotic therapy were included. Results: Aspiration was performed in all 28 patients with infected hepatic cysts. As a result, 17 patients were cured, 4 remain under treatment, and 6 died. One patient was cured by partial hepatectomy. Among the 15 patients with renal cysts, aspiration was performed in 4 with identifiable infected cysts, while renal transcatheter arterial embolization after appropriate antibiotic therapy was performed in 11 without identifiable infected cysts. No patient developed recurrence. Conclusion: In patients with infected renal cysts, aspiration or renal transcatheter arterial embolization after appropriate antibiotic therapy was effective. Although aspiration was often effective in patients with infected hepatic cysts, a good outcome was less likely than in those with renal cysts.

Plasma Adiponectin: A Predictor of Coronary Heart Disease in Hemodialysis Patients – A Japanese Prospective Eight-Year Study

Background/Aim: Plasma adiponectin may play a protective role in the pathogenesis of cardiovascular disease in hemodialysis (HD) patients. We examined the effect of plasma adiponectin levels on the prognosis of the HD patients. Methods: 68 HD patients (male:female = 38:30) were subjected to plasma adiponectin measurement in 1998 and followed up over 8 years. Results: Plasma adiponectin concentrations differed between male and female patients (9.3 vs. 15.7 μg/ml). The plasma adiponectin concentration as a whole was positively correlated with serum high-density lipoprotein cholesterol and negatively with serum creatinine and waist circumference. During an 8-year follow-up, the cardiac events occurred in 7 of 38 men and in 10 of 30 women. Cox’s proportional hazard model analysis in a stepwise manner revealed that coronary heart disease (CHD) was associated with intact parathyroid hormone concentration, age, and the presence of diabetes in men whereas plasma adiponectin concentration was the most powerful single predictor in women. The impact of the plasma adiponectin concentration was strengthened by Kaplan-Meier survival analysis. In the group with a lower plasma adiponectin concentration, CHD events were significantly increased in men (p = 0.043) and in women (p = 0.007). Conclusion: Plasma adiponectin concentration may predict CHD outcomes in HD patients.

Renal Cell Carcinoma in Autosomal Dominant Polycystic Kidney Disease

p g E utosomal dominant polycystic kidney disease (ADPKD) is one of the most common ereditary kidney disorders and accounts for 8% o 10% of patients with end-stage renal failure in he United States and Europe. In some patients ith ADPKD, the kidneys continue to enlarge fter the initiation of dialysis therapy, resulting in uch significant complications as cyst bleeding nd abdominal distention. Keith et al previusly reported that renal cell carcinoma (RCC) is serious complication of ADPKD, but the incience of RCC in patients with ADPKD is controersial because the total number of patients with nderlying ADPKD has not been clarified. In the present study, we discuss the role of maging studies and the incidence of RCC in a enter’s experience with dialysis patients with DPKD.

Is there a link between diabetic glomerular injury and crescent formation? A case report and literature review

Glomerular crescents are most commonly associated with rapidly progressive crescentic glomerulonephritis; however, they also develop in response to a wide range of primary and secondary glomerular injuries. Since various kind of glomerulopathies occasionally overlay diabetic glomerular injuries, the presence of crescents in renal biopsy specimens of diabetics may have stimulated a search for etiologies other than diabetes. In this report, we describe an unusual case of diabetic glomerulosclerosis with peculiar extracapillary proliferation. Although such a relationship has so far been ignored in most of the literature, the etiological linkage between diabetic glomerulosclerosis and the development of crescents may not be exceptional. We have reviewed the previous literature and herein discuss the pathological implications of the development of crescents in patients with diabetic glomerulosclerosis.Virtual slidesThe virtual slide(s) for this article can be found here: http://www.diagnosticpathology.diagnomx.eu/vs/3950457896920255.

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 patients 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.

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.

Takotsubo cardiomyopathy in a patient undergoing hemodialysis

A 61-year-old woman hospitalized following surgery for cervical spondylosis developed severe chest pain and dyspnea during a hemodialysis session. She had been receiving chronic hemodialysis for 20 years secondary to glomerulonephritis. Electrocardiography (ECG) revealed ST-segment elevation in the precordial leads, and Q waves in the anterior and inferior leads. The creatine kinase MB and troponin I concentrations were slightly elevated. Echocardiography showed an apical wall motion abnormality. These findings were compatible with acute anterior wall myocardial infarction (MI). Emergent coronary angiography demonstrated normal coronary arteries. Left ventricular angiography revealed apical ballooning of the left ventricle in the systolic phase (Figure 1), not typical of the wall motion abnormality seen after acute MI. A diagnosis of Takotsubo cardiomyopathy was made based on the presence of this characteristic finding (Takotsubo is a Japanese octopus trap). The cardiomyopathy resolved spontaneously after 1 month, with normalization of the ECG. Takotsubo cardiomyopathy (transient left ventricular apical ballooning syndrome) is increasingly being recognized worldwide. In the setting of cardiogenic shock, recognition of Takotsubo cardiomyopathy is important to distinguish this cardiomyopathy from acute MI, which is especially relevant in end-stage renal disease patients because of their high cardiovascular risk. Although the etiology of Takotsubo cardiomyopathy remains uncertain in most patients, long-term outcomes appear favorable compared with myocardial infarction.

An Intron 4 Gene Polymorphism in Endothelial Cell Nitric Oxide Synthase Might Modulate Lipid Metabolism in Nondiabetic Patients on Hemodialysis

We investigated the relationship between endothelial constitutive nitric oxide synthase (ecNOS) gene polymorphism and lipid metabolism in patients with nondiabetic chronic renal failure on hemodialysis. Serum from 181 nondiabetic patients on hemodialysis were examined. A genomic DNA fragment was amplified by polymerase chain reaction (PCR) for determining the ecNOS genotype. The PCR products were designated as a and b alleles by electrophoresis. In hemodialysis patients, the frequency of the ecNOS4 for b/b, b/a and a/a genotype was 76.6, 22.8 and 0.6%, respectively. There was not significant difference in the levels of total cholesterol (TC), triglyceride (TG) and calculated low-density lipoprotein cholesterol (LDL-c) in sera between patients (aa and ba) with the a allele and patients (bb) without the a allele. On the other hand, the levels of serum high-density lipoprotein cholesterol (HDL-c) in patients with the a allele (51.9 ± 3.33 mg/dl) were significantly higher than those in patients without the a allele (43.05 ± 1.40 mg/dl) (p = 0.005). The frequency of patients with the a allele and low levels of serum HDL-c among patients with a long duration of dialysis (≧10 years) was significantly lower than that in patients with short duration of dialysis (<10 years) (p = 0.05). It appears that an intron 4 gene polymorphism in ecNOS may modulate lipid metabolism in nondiabetic patients on hemodialysis and the a allele of ecNOS gene polymorphism may affect the prognosis of hemodialysis patients with low levels of serum HDL-c.