Aggressive pulmonary calcification developed after living donor kidney transplantation in a patient with primary hyperparathyroidism.

Metastatic pulmonary calcification, defined as calcium deposition in the intact lung, is commonly seen in patients with chronic renal failure, and it is known to be a benign clinical condition when detected by chance in an asymptomatic patient. Here we report the case of a 33-year-old woman who developed rapid and aggressive metastatic pulmonary calcification shortly after a living donor kidney transplantation, which induced acute antibody-mediated rejection. The patient's metastatic pulmonary calcification was successfully improved by extensive treatment for graft rejection, the correction of her accompanying primary hyperparathyroidism, and medical treatment with a bisphosphonate and sodium thiosulfate. Aggressive pulmonary calcification is reported as a rare complication seen in patients who have undergone a failed renal transplantation. A failed renal graft and accompanying secondary hyperparathyroidism seem to accelerate metastatic calcification. Most of the patients who develop aggressive pulmonary calcification suffer from the rapid progression of dyspnea and occasionally fever, and they die of respiratory failure. Pulmonary calcification should be considered in a patient developing dyspnea and unexplained pulmonary infiltrate, especially in the context of renal graft rejection; otherwise the prognosis of the patient will be very poor.

Glomerulocystic kidney disease in an adult with enlarged kidneys: a case report and review of the literature.

We report the case of a 31-year-old male with enlarged kidneys and glomerulocystic kidney disease (GCKD). The patient had no family history of renal disease or other diseases. On initial presentation he complained of poor eyesight, and hypertensive retinopathy and elevated serum creatinine (5.0 mg/dl) were found at that time. Renal biopsy showed cystic dilatation of Bowman's capsule and atrophy of the glomerular tuft. Thus, an adult case of sporadic GCKD was diagnosed. Based on previous reports, kidney size in patients with adult type GCKD varies from small to large. Our patient's kidneys are the largest ever reported (right kidney was 22 cm×10 cm, left kidney was 19 cm×10 cm). A review of the literature dealing with sporadic adult GCKD suggested that it is difficult to diagnose this disease early in its course.

Kidney transplantation from non-heart-beating donors: analysis of organ procurement and outcome.

INTRODUCTION: Most donors in Japan have been non-heart-beating donors (NHBD), so-called "marginal donors." In Western countries kidney transplants from NHBD have also been increasing. We analyzed 120 kidneys harvested from NHBD with regard to organ procurement, renal function, graft survival, and the donor factors that affected graft survival. METHODS: Donors were moved into the operating room after cardiac arrest. A double-balloon catheter was inserted into the abdominal aorta via laparotomy. In situ cooling by Euro-Collins solution was started at 500 mL/min. We did not performed cannulation into the femoral artery or vein prior to cardiac arrest. RESULTS: Warm ischemia time (WIT) was 18.6 minutes. Among 108 kidneys (90%) used for transplantation, 102 kidneys functioned. There were no cases of bilateral nonfunctioning kidneys. The delayed graft function (DGF) rate was 86%; however, the death-censored graft survival was 80.0% at 5 years and 62.9% at 10 years. Kidneys implanted after more than 24 hours of total ischemia time required a significantly longer period of hemodialysis. Donor risk factors that affected graft survival included WIT >/= 20 minutes, donor age >/= 50 years, and serum creatinine level at admission > 1.0 mg/dL. CONCLUSIONS: Organ procurement without cannulation prior to cardiac arrest entailed a long WIT and a high DGF rate. However, the graft survival was good. It has been necessary to use grafts from NHBD despite the inherent risk factors. It is important to reduce kidney damage both at the organ procurement and during the posttransplant management.

Clinicopathological study of vesicoureteral reflux (VUR)-associated pyelonephritis in renal transplantation.

We retrospectively studied the occurrence of vesicoureteral reflux (VUR)-associated pyelonephritis using renal biopsies obtained from the transplanted kidneys, and correlated the histological changes with clinical parameters. Out of a total of 131 renal biopsies performed between 1990 and 2001 on renal transplant patients at the department of Urology of Nagasaki University Graduate School of Biomedical Sciences, 12 patients showed pyuria more than twice in a single year. Seven of these 12 patients were available for determining VUR by voiding cystourethrography (VCUG). Cystoureterography demonstrated VUR in three of seven studied patients with pyuria. A histopathological examination revealed dilatation of both proximal and distal tubules in renal biopsies of transplant patients with VUR, compared to renal biopsies of transplant patients without VUR, or non-transplanted patients with thin membrane disease. One of the patients with VUR showed advanced features of chronic pyelonephritis in four consecutive biopsies at different time points, suggesting a late stage of reflux nephropathy in the transplanted kidney. We conclude from our study that the occurrence of VUR-related pyelonephritis may be one of the important long-term complications in the survival of renal allografts.

Different clinicopathological courses of two recipients of kidneys retrieved from the same non-heart beating donor.

We report the clinicopathological courses of two recipients of kidneys retrieved from the same non-heart beating donor (NHBD). A 52-year-old man received a renal transplant from an NHBD. The donor was a 66-year-old woman who died of subarachnoid haemorrhage. The recipient was immunosuppressed by basiliximab, tacrolimus (TAC), mycophenolate mofetil (MMF), methyl prednisolone (MP), and antilymphocyte globulin (ALG). On post-operative day (POD) 21, haemodialysis therapy was withdrawn, however, their serum creatinine (s-Cr) level failed to improve. Four transplant biopsies were performed (1 h and POD 46, 74, and 114). The biopsy showed tubular degeneration but no evidence of TAC nephrotoxicity. The last biopsy after discontinuation of TAC demonstrated acute rejection of borderline grade. The s-Cr level at discharge was 5.0 mg/dL. The contra-lateral kidney was transplanted into a 31-year-old female and showed early functioning, with an s-Cr level at discharge of 1.8 mg/dL. Biopsy examination on POD 38 showed a recovery of tubular degeneration. The causes of delayed graft function and persistently high level of s-Cr in Case 1 remain unclear. Various factors, including donor-related factors, recipient-related factors, TAC nephrotoxicity, acute rejection, and urinary tract infection could all be associated with this condition.

The diagnostic value of bone scan in patients with renal cell carcinoma.

PURPOSE: Bone scan is performed as part of the evaluation of bone metastasis. We assessed the diagnostic value of bone scan in patients with renal cell carcinoma. MATERIALS AND METHODS: Bone scan was performed at presentation in 205 patients with confirmed renal cell carcinoma. Abnormal hot areas were further evaluated by x-ray, computerized tomography or surgery. RESULTS: Of the 56 patients (27%) with an abnormal bone scan 32 (57%) had osseous metastatic lesions. Overall bone metastasis was present in 34 of the 205 patients (17%). Bone scan had 94% sensitivity and 86% specificity. Of the 124 patients with clinically localized, stages T1-2N0M0 disease exclusive of bone metastasis 6 (5%) had bone metastasis only, whereas 28 of 81 (35%) with locally advanced or metastatic disease had bone metastasis, including 12 (35%) who complained of bone pain and 19 (56%) who presented with other symptoms due to local tumor growth or metastasis at other sites. Three patients (9%) were asymptomatic. There was osseous metastasis without other metastasis, enlarged regional lymph nodes or bone pain in 7 patients, including 1 with stage T1b (2% of all with that stage), 2 with stage T2 (5%), 1 with stage T3a (4%), 1 with stage T3b (6%), 1 with stage T3c (14%) and 1 with stage T4 (6%) disease. CONCLUSIONS: Bone scan may be omitted in patients with stages T1-3aN0M0 tumors and no bone pain because of the low proportion of missed cases with bone metastasis.

OK432-induced killer cell activity: potential method for monitoring immunological complications after renal transplantation.

BACKGROUND: Various clinical and biochemical parameters are currently in use for monitoring allograft rejection. However, the mechanism of allograft rejection is complex and it is frequently difficult to obtain a prompt and accurate diagnosis. We examined the usefulness of OK432-induced killer cell activity as an immunological monitoring system for acute renal rejection after renal transplantation. METHODS: Twenty-four renal transplant recipients, seven patients on haemodialysis, and 10 normal volunteers were enrolled in our study. The killer cell activity of peripheral blood mononuclear cells was induced by culturing these cells with the immunopotentiator, OK432, a heat and penicillin-treated lyophilized powder of the Su-strain of Streptococcus pyogenes. RESULTS: The OK432-induced killer cell activity of renal transplant recipients without acute rejection (stable recipients) was significantly lower than in normal volunteers. In four renal transplant recipients with acute rejection, the killer cell activity was significantly higher than in stable recipients. In three recipients suffering from opportunistic infections, killer cell activity was significantly suppressed compared with stable recipients. CONCLUSIONS: Our new test utilizing OK432-induced killer cell activity is potentially useful for monitoring the immunological state and complications after renal transplantation.

Predictive values of acute phase reactants, basic fetoprotein, and immunosuppressive acidic protein for staging and survival in renal cell carcinoma.

OBJECTIVES: To determine the clinical significance and predictive value of three acute phase reactants (erythrocyte sedimentation rate, C-reactive protein, and ferritin), as well as basic fetoprotein (BFP) and immunosuppressive acidic protein, in patients with renal cell carcinoma. METHODS: Erythrocyte sedimentation rate, C-reactive protein, ferritin, BFP, and immunosuppressive acidic protein levels were measured in 92 patients with renal cell carcinoma diagnosed in 1989 to 1999. The levels were compared with the clinical stage and nuclear grade, and their predictive values of survival were evaluated statistically. RESULTS: All markers, with the exception of BFP, correlated with each other and with the clinical stage and nuclear grade. BFP did not correlate with the acute phase reactants. The log-rank test revealed that the levels of C-reactive protein, immunosuppressive acidic protein, and ferritin significantly influenced survival. Multivariate stepwise analysis identified ferritin as the only independent and significant prognostic marker (hazard ratio = 5.624, P = 0.001). However, when age, sex, clinical stage, and nuclear grade were entered into the same analysis, only clinical stage was an independent marker of prognosis. CONCLUSIONS: The results of our study demonstrated that serum ferritin is the most useful marker among five tested factors for staging and predicting survival, although the clinical stage is the best parameter that predicts the prognosis of patients with renal cell carcinoma accurately.

[Long-term experience with renal transplantation in systemic amyloidosis: a case report].

Renal transplantation was performed on a 39-year old woman with secondary amyloidosis due to rheumatoid arthritis. She remains alive and renal function has been maintained satistfactorily with the exception of proteinuria ten years after transplantation. Recent renal biopsy showed no amyloid recurrence, but the presence of chronic rejection reaction and mild cyclosporin arteriolopathy. Symptoms related to systemic amyloidosis and rheumatoid arthritis improved after transplantation. Renal transplantation is the recommended therapy for the type AA systemic amyloidosis. This is the second report of long-term experience with renal transplantation in systemic amyloidosis in Japan.

[Evaluation of bone metastases from renal cell carcinoma].

Forty-two cases of bone metastasis from renal cell carcinoma were examined. Thirty of the cases had bone metastases at the time of renal cell carcinoma. Bone metastasis appeared after treatment of the primary site in 12 cases. Fifteen cases had only bone metastasis and another 27 had metastasis in multiple organs. The total cause-specific survival curve of these patients was 10% at 5 years. All patients with bone metastases died of cancer within 5 years after the bone metastases had developed. There was no significant difference in the survival rate between patients with bone metastases and patients with lung metastases. We investigated the prognostic value of laboratory studies in bone metastases of renal cell carcinoma. However, no significant markers were detected for bone metastases. The 6 patients were treated with decompressed laminectomy (2), wide resection (3) and excision of the metastatic lesions (3). The quality of life was improved in all the patients although they died of cancer.

[Clinical evaluation of renal angiomyolipoma].

Between April 1980 and December 1999, 23 kidneys in 20 patients were diagnosed as having renal angiomyolipoma at our institution. The patients were 6 males and 14 females aged 24 to 79 years, with a mean age of 55.4 years. Two patients had associated tuberous sclerosis and 3 had bilateral disease. Of all patients the main clinical symptoms were pain (45%) and palpable mass (40%); 5 patients (25%) had asymptomatic lesions. The size of the tumor ranged from 1.3 to 24 cm (mean 7.7 cm). Treatment consisted of nephrectomy in 9 patients, partial nephrectomy in 6 and selective embolization in one. Pre-operative diagnosis was renal cell carcinoma in 5 of the 9 patients who underwent nephrectomy. Six patients with 7 diseased kidneys were followed radiologically. One patient underwent percutaneous biopsy to confirm the diagnosis. We suggest that nephron-sparing surgery for patients with renal angiomyolipoma should be the first step if tumor size is 4 cm < or = or increasing rapidly. Selective embolization is also a useful method. However, we need long-term follow-up to evaluate the effectiveness of embolization.

[A case of bilateral synchronous renal cell carcinoma treated with left partial nephrectomy and laparoscopic right nephrectomy].

We describe a case of bilateral synchronous renal cell carcinoma. A 70-year-old female was admitted to our department because of further examination for bilateral renal masses. Computed tomographic scanning and ultrasound examinations revealed bilateral solid enhanced renal masses, and bilateral renal cell carcinomas were suspected. First, partial left nephrectomy was performed. On the 21th day after the first operation, we confirmed the recovery of the left kidney, and performed right nephrectomy laparoscopically. The histopathological diagnosis revealed bilateral renal cell carcinomas. The patient is alive with no metastatic lesions and no recurrence at 16 months after the operations.

Relationship between serum ferritin levels and tumour status in patients with renal cell carcinoma.

OBJECTIVE: To assess the relationship between serum ferritin levels (a useful marker for diagnosing and staging renal cell carcinoma, RCC) and tumour status in RCC of

An evaluation of Bosniak's radiological classification of cystic renal masses.

OBJECTIVE: To determine the clinical usefulness of Bosniak's classification of cystic renal masses, the differentiation of which remains difficult despite significant advances in diagnostic imaging. PATIENTS AND METHODS: The computed tomography (CT) findings of all histopathologically examined cystic renal masses diagnosed at our institution were analysed retrospectively; 35 patients with cystic renal masses were treated between 1986 and 1998. Tissues surgically removed were examined pathologically and the final diagnosis compared with the preoperative CT category of Bosniak's classification. RESULTS: The histopathological examined showed cystic renal cell carcinoma in 21 patients, a benign renal cyst in 12, haemangiosarcoma in one and transitional cell carcinoma in one. Most of the 35 masses (26, 74%) were found incidentally during evaluation for an unrelated disease or a routine health check. All 11 masses of Bosniak category I were benign and one category II mass was malignant. All 10 masses of category III and 12 of category IV were malignant. CONCLUSIONS: Bosniak's classification is useful for differentiating category I, III and IV cystic renal masses. There were too few samples to allow meaningful conclusions to be drawn for category II renal masses. It is critical to differentiate between complicated cysts of category II and III because of the major implications for prognosis and clinical management.

Renal cell carcinoma metastatic to the skin.

BACKGROUND: Cutaneous metastases from renal cell carcinoma are rarely diagnosed during life. We reviewed the cases of our institute and from the Japanese literature and analyzed them to define the clinical features. PATIENTS AND METHODS: Six patients with cutaneous metastases from renal cell carcinoma were treated in our institute. Clinical data and follow-up information of these patients were reviewed. RESULTS: The skin metastatic lesions were found before the renal lesion was discovered in one case and they were found after the diagnosis had been established in 5. Four patients presented with solitary cutaneous metastasis at the time of diagnosis of the skin metastatic lesion. Five patients had other sites of metastases such as the lung, liver and bone at the time of diagnosis of cutaneous metastases. Five patients died of renal cell carcinoma at mean 23.8 months after presenting with cutaneous metastases. CONCLUSION: Our 6 cases bring the total reported in the Japanese literature to 75 cases. The results of these 75 cases revealed that the duration from the time of diagnosis of renal cell carcinoma to detection of the cutaneous metastasis was relatively long but the prognosis of patients with these metastatic skin lesions was poor. Cutaneous metastases from renal cell carcinoma were regarded as a late manifestation of the disease.

Outcome of surgery in cystic renal cell carcinoma.

OBJECTIVES: To review cases of cystic renal cell carcinoma treated surgically at our institution and define their clinical and histopathologic features. METHODS: Between 1986 and 1998, 21 patients with cystic renal cell carcinoma were treated surgically. Cystic renal cell carcinoma was categorized using Hartman's classification. RESULTS: Histopathologic examination demonstrated cystic necrosis in 11 patients, multilocular cystic renal cell carcinoma in 9, and unilocular cystic renal cell carcinoma in 1 patient. Tumors were incidentally found during an evaluation of unrelated disease or a general health checkup in 14 patients (67%). The mean tumor size was 5.6 cm (range 0.5 to 12) for cystic necrosis and 5.4 cm (range 2 to 9) for multilocular cystic renal cell carcinoma. All 9 cases of multilocular cystic renal cell carcinoma were of the clear cell type and tumor grade 1. The mean follow-up period was 65 months (range 9 to 141). The 5-year disease-specific survival rates for multilocular cystic renal cell carcinoma and cystic necrosis were 100% and 80%, respectively. CONCLUSIONS: The prognosis for patients with cystic renal cell carcinoma is better than that for patients with solid tumors. In particular, the prognosis of multilocular cystic renal cell carcinoma is excellent. Multilocular cystic renal cell carcinoma represents a distinct subtype of renal cell carcinoma that can be completely cured by surgery.

Systemic immune response after intravesical instillation of bacille Calmette-Guérin (BCG) for superficial bladder cancer.

The mechanism of anti-tumour activity by BCG is not known clearly. However, many studies suggest that immunological response is related to effectiveness of intravesical instillation of BCG in the therapy for superficial bladder carcinoma. Peripheral blood mononuclear cells (PBMC), urine and serum were obtained from patients with superficial carcinoma at various times during the course of BCG instillation. Urine of patients showed increased levels of IL-1beta, IL-2, IL-6, tumour necrosis factor-alpha (TNF-alpha), interferon-gamma (IFN-gamma) and macrophage colony-stimulating factor (M-CSF) after BCG instillation. Levels of IL-2 and IFN-gamma in the serum also increased after BCG instillation, but IL-1beta, IL-6, TNF-alpha and M-CSF were not detectable. Maximal levels of IL-2 and IFN-gamma in the urine or serum were shown after the fourth instillation. BCG-induced killer cell activity in PBMC increased significantly after the third BCG instillation. These results suggest that BCG instillation involved not only local immunological efforts but also systemic immune responses. Tumour-free patients produced higher BCG-induced killer cell activity than tumour recurrence patients. BCG-induced killer cell activity may be useful for monitoring the effectiveness of intravesical BCG instillation.

Renal transplantation in systemic amyloidosis.

Renal transplantation was performed in a 24-yr-old woman with type AA systemic amyloidosis. Renal biopsy at 11 yr post-transplantation showed no amyloid recurrence, but the presence of mesangial proliferative glomerulonephritis and focal segmental glomerular sclerosis. Renal function was satisfactory with the exception of mild proteinuria. Symptoms related to systemic amyloidosis including goiter and cardiac deposition, improved post-transplantation. Renal transplantation is the recommended therapy for type AA systemic amyloidosis.

Breast cancer metastatic to the kidney.

We report a case of renal tumor secondary to a breast cancer occurring 16 years after radical mastectomy. This is the sixth report case of renal metastasis from breast cancer of a 51-year-old woman. Percutaneous biopsy of the renal tumor confirmed the diagnosis during the follow-up. The patient was treated with chemotherapy and is alive 8 months after diagnosis. Previously, cases like our case showed long interval from mastectomy to diagnosis of metastasis.

The role of surgery in renal cell carcinoma with solitary metachronous metastasis to contralateral adrenal gland.

We report a case of renal cell carcinoma with solitary metachronous metastasis to contralateral adrenal gland occurring 9 months after radical nephrectomy. The patient was treated with a contralateral adrenalectomy and is alive for 87 months. The literature was reviewed and 5-year survival of solitary metachronous metastasis to contralateral adrenal gland was 60%. Follow-up duration of our case was the longest in the literature. It is suggested that the solitary contralateral adrenal gland metastasis of renal cell carcinoma should be resected since there is no effective treatment of metastatic renal cell carcinoma. Good prognosis may be then and the good be expected.