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.

Quality of life improvement and long-term effects of peripheral blood mononuclear cell transplantation for severe arteriosclerosis obliterans in diabetic patients on dialysis.

BACKGROUND: Severe arteriosclerosis obliterans (ASO) can be intractable, especially in diabetic patients on hemodialysis (HD). Recently, the transplantation of autologous peripheral blood mononuclear cells (PBMNCs) has been reported to have beneficial effects, but the long-term effects and impact on quality of life (QOL) have not been studied. METHODS AND RESULTS: Autologous PBMNCs were transplanted into 7 diabetic patients on HD who had severe ASO (5 cases with Fontaine IV and 2 with Fontaine III) after administration of 5 microg/kg granulocyte colony stimulating factor; QOL and degree of ischemia was assessed by measuring skin temperature, skin perfusion pressure (SPP), ankle-brachial index (ABI), and ulcer size, and from angiographic findings. At 4 weeks after the procedure, skin temperature was significantly improved, and SPP and ABI also were increased. These beneficial effects persisted for up to 24 weeks. Angiographic findings and ulcer size improved in 3 of 7 and 3 of 4 patients, respectively. SF-36v2 analysis revealed significant improvements in pain scores. No serious complications were detected. CONCLUSION: Transplantation of PBMNCs resulted in improvement in pain and leg ischemia for over 6 months without serious complications. This therapy is safe and effective for severe ASO in diabetic patients on HD.

Induction of anti-carbonic-anhydrase-II antibody causes renal tubular acidosis in a mouse model of Sjogren's syndrome.

BACKGROUND/AIM: We recently reported that renal tubular acidosis (RTA) in Sjogren'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.

Percutaneous transcatheter hepatic artery embolization for liver cysts in autosomal dominant polycystic kidney disease.

BACKGROUND: We have achieved renal contraction therapy in patients with autosomal dominant polycystic kidney disease (ADPKD) by means of renal transcatheter arterial embolization (TAE) using intravascular coils, decreasing renal size and improving quality of life in almost all patients. We presently perform hepatic TAE in patients with intractable symptomatic polycystic liver. STUDY DESIGN: Uncontrolled trial. SETTING & PARTICIPANTS: 30 patients with ADPKD referred for arteriography to an academic medical center. 22 patients had kidney failure treated by means of dialysis. INTERVENTION: We embolized arteries supplying hepatic segments replaced by cysts that were associated with well-developed hepatic arteries, but obstructed intrahepatic portal veins. OUTCOMES & MEASUREMENTS: Various volumes before and after TAE were compared by using computed tomography and National Institutes of Health Image software in 30 patients with follow-up computed tomography 18 to 37 months after therapy. RESULTS: Total liver volume and total intrahepatic cyst volume decreased from 7,882 +/- 2,916 and 6,677 +/- 2,978 to 6,041 +/- 2,282 and 4,625 +/- 2,299 cm(3), respectively (P < 0.0001 for both). Fractions of remaining (FR) total liver volume and FR of intrahepatic cyst volume were 78.8% +/- 17.6% and 70.4% +/- 20.9%, respectively. Hepatic parenchyma increased from 1,205 +/- 250 to 1,406 +/- 277 cm(3) (P = 0.0004). In 29 of 30 patients, both total liver volume and intrahepatic cyst volume decreased; in 1 patient, total liver volume increased from 5,755 to 7,069 cm(3), whereas cysts enlarged from 4,500 to 5,531 cm(3). No serious complications were experienced. In 24 patients, the post-TAE course was favorable. TAE failed to benefit 6 patients because of unrelated hepatic infection, peritonitis, hepatic failure, acute leukemia, or pelvic fracture. LIMITATIONS: Absence of a control group. CONCLUSIONS: TAE may be an option for patients with ADPKD with symptomatic polycystic liver who are not candidates for surgical treatment.

Tonsillectomy and corticosteroid therapy with concomitant methylprednisolone pulse therapy for IgA nephropathy.

IgA nephropathy (IgAN) is the most common chronic kidney disease in Japan, but the optimum treatment remains controversial. Our objective was to evaluate the effect of tonsillectomy and corticosteroid therapy combined with methylprednisone pulse therapy in patients at our hospital who had IgAN. Tonsillectomy plus pulse therapy was evaluated in 72 patients (33 men and 39 women) with a diagnosis of IgAN based on renal biopsy who were followed up for more than 1 year. The mean age of the patients was 35.2 +/- 10.9 years (range: 20-58 years) and the mean observation period after tonsillectomy was 20.3 +/- 9.7 months (range: 12-36 months). After tonsillectomy, steroid pulse therapy was administered (methylprednisolone at 500 mg daily for 3 days) 1-3 times and was followed by oral prednisolone from an initial dose of 30 mg on alternate days that was tapered gradually over one year. At 2 years after tonsillectomy, serum creatinine was unchanged or improved in the majority of patients, but worsened in 5 patients. Hematuria (erythrocytes/HPF) improved from Grade 3.76 (11-30/HPF) to Grade 1.94 (1-5/HPF) on average (we defined the grade of hematuria). None of the patients experienced exacerbation of hematuria. Proteinuria decreased from 1.32 g/day to 0.86 g/day (65% of the pretreatment value), and only 4 patients showed an increase of proteinuria. Mean protein loss decreased to less than 0.5 g/day in patients with creatinine clearance > or =90 ml/min and/or patients with initial protein excretion < or =1.0 g/day.

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.