[Tuberous sclerosis associated with renal angiomyolipoma, pulmonary lymphangioleiomyomatosis and subungual fibroma: report of a case].

We report a case of tuberous sclerosis associated with bilateral renal angiomyolipomas (AMLs), pulmonary lymphangioleiomyomatosis (LAM) and subungual fibroma of hands and feet. A 42-year-old woman who was diagnosed as tuberous sclerosis at the age of 18 complained of left flank pain and abdominal fullness. Bilateral renal AMLs were pointed out when complete examinations were performed for hypertension at the age of 32. She suffered from severe left flank pain and abdominal distension due to the left renal tumor. Left nephrectomy and excision of the renal hilar tumor were performed. The left renal tumor weighed 1120 g, the perirenal space was filled with the tumor. histopathological diagnosis of the left renal tumor and renal hilar tumor was AML. In our case, bilateral pneumothorax appeared, and chest CT scan revealed bilateral multiple pulmonary cysts. Histopathological diagnosis of pulmonary cysts was LAM. Other complications of our case are intracranial calcification and adenoma sebaceum.

Antitumor killer lymphocytes in the peripheral blood of a patient with transitional cell carcinoma of the bladder.

BACKGROUND: Peripheral blood lymphocytes (PBL) from patients with bladder cancer also contain cells possessing cytotoxic activity against autologous tumor cells. These cells are phenotypically heterogenous and include natural killer (NK) and cytotoxic T cells. This study investigated the role of cytotoxic lymphocytes directed against autologous bladder cancer cells. METHODS: PBL were obtained at intervals before and after surgery and analyzed for cytotoxic activity against autologous bladder cancer cells in 4-hour 51Cr release assay. PBL stimulated with autologous tumor cells were also transformed with human T-lymphotropic virus type-1, establishing a cell line (KB31) which was analyzed for phenotype and cytotoxic activity against the autologous tumor cells. RESULTS: PBL preoperative cytotoxic activity was low, but increased after surgery. Cytotoxic activity was found not only against autologous bladder cancer cells, but also against heterologous bladder cancer (KK-47) and myeloid leukemia (K562) cells, with the highest activity against the heterologous cell lines. The cytotoxic activity of KB31 was 40% against autologous tumor cells 6 weeks after initiation of the cell line, but decreased to 5% by 6 months. This activity was lower than that against the other cell lines, and was similar to that of PBL in short-term culture. Fluorescence-activated cell sorter (FACS) analysis demonstrated that in KB31 cells at 6 weeks, CD8+ cells were dominant, but CD56+ cells predominated at 6 months. CONCLUSION: These results suggest that the presence of cytotoxic activity in the peripheral blood of the patient was due to both cytotoxic T cells and NK cells. The cytotoxic activity was lowest prior to surgery and increased postoperatively.

Relationship between area under the concentration versus time curve of cyclosporin A, creatinine clearance, hematocrit value, and other clinical factors in Japanese renal transplant patients.

We evaluated the relationship between the area under the concentration versus time curve (AUC) of cyclosporin A (CsA) and several other clinical factors, because the clinical utility of AUC monitoring has been ambiguous. Fifty-four clinical time courses from 14 Japanese renal transplant patients during hospitalization, in the period from April 1990 to March 1997, were examined. In a bivariate regression analysis there was no correlation between the AUC and the daily dose of CsA (mg/kg/day) when the individual data or total series data were analyzed. In a chi-square test, the donor type of kidney (chi(2) = 25.254, df = 1, p = 0.0000) and renal function-related episodes, i.e. acute tubular necrosis, hemodialysis, hypertension, nephrotoxicity, or rejection (chi(2) = 13.982, df = 1, p = 0.0002) directly affected posttransplant renal function assessed by creatinine clearance, while episodes of hepatic function as assessed by the glutamate-pyruvate transaminase (GPT) activity level had no correlation with the posttransplant renal function evaluated according to creatinine clearance. In contrast, the renal function-related episodes significantly affected the AUC after renal transplantation (chi(2) = 4.934, df = 1, p = 0.0263), while hepatic function assessed by GPT did not. In a multivariate analysis, the creatinine clearance and obesity had significant positive correlations with the AUC, whereas the hematocrit had a significant negative correlation with the AUC. From these observations, we concluded that the dosage adjustment of CsA cannot be performed using the linear relationship between the daily oral dose and the AUC, and that renal function, obesity, and the CsA blood distribution properties affect the CsA pharmacokinetics after renal transplantation. Posttransplant renal function as well as obesity and CsA blood distribution properties are important factors to be considered when therapeutic monitoring is performed.

[A case of pyelitis cystica diagnosed with utilization of ureterofiberscope].

We herein report a case of pyelitis cystica in 65-year-old woman. She was referred to our hospital in order to have a treatment for a stone in the ureter on left side. Excretory urogram showed hydronephrosis on left, and multiple, small, smooth and round filling defects in the renal pelvis on right side. ESWL was performed to the ureteral stone, and the stone was discharged completely in 4 days. Then further examinations were made for the filling defects of right renal pelvis. Nonopaque calculi were ruled out on retrograde pyelogram and CT scan. Urinary cytology from the renal pelvis was class I. Our impression was pyelitis cystica of right kidney. Under spinal anesthesia, ureterofiberscopy was performed. Multiple small cysts were observed in the pelvis and calyx, as well as cystitis cystica. Cold cup biopsy was also done and histopathological finding ws pyelitis cystica, without malignancy. We compared endoscopic findings with radiographic findings in 18 cases of pyloureteritis cystica from the Japanese literature. The radiographic findings were multiple small, in a uniform size, and round filling defects with regular contour, and the endoscopic findings were multiple white or ocher colored, half sphere or sphere shaped, and small cyst with smooth surface in 15 of 18 cases. We thought these findings were characteristic ones in pyloureteritis cystica. Endoscopy and biopsy are mandatory for diagnosis of pyeloureteritis cystica.

Azathioprine-induced megaloblastic anemia with pancytopenia 22 years after living-related renal transplantation.

Macrocytosis and megaloblastic changes in the bone marrow are frequently seen in renal transplant recipients treated with azathioprine (Az). However, severe anemia is a rare side effect of Az. We recently observed a case of severe megaloblastic anemia with pancytopenia in a renal transplant recipient who had been receiving Az therapy for 22 years. The patient was a 46-year-old woman who had been administered Az and prednisolone at a dose of approximately 1.7 mg/kg and 0.17 mg/kg daily, respectively. A bone marrow aspiration revealed megaloblastic anemia with the depletion of myeloid cells and megakaryocytes. She did not have vitamin B12 or folate deficiency. Therefore, FK506 (tacrolimus), a macrolide produced by Streptomyces tsukubaensis, which acts directly on T cells and is known to have less myelosuppression than Az, was substituted for Az. Although the leukopenia improved, the anemia and thrombocytopenia did not improve in the short term. She developed dyspnea and severe subcutaneous bleeding of the right lower extremity due to knee contusions. Hemodialysis was started to treat her uremic state. Although it was impossible to evaluate the long-term effects of FK506 therapy for the pancytopenia in our case, the conversion from Az to a less myelosuppressive drug, such as FK506, should be considered in renal transplant recipients with severe myelosuppression caused by long-term Az treatment.

[Adenocarcinoma of the bladder 19 years after the augmentation ileocystoplasty: report of a case].

We report a case of adenocarcinoma of the augmented bladder 19 years after ileocystolasty. The patient was a 53-year-old man who underwent right nephrectomy and ileocystoplasty (Pyrah's method) for contracted bladder due to tuberculosis in 1965. In another hospital, transurethral resection (TUR) was performed against a tumor in the anastomotic site between the bladder and the ileal segment in 1996. Histopathological examination of the specimen obtained by TUR revealed poorly-differentiated mucinous adenocarcinoma. In our hospital, partial cystectomy with total resection of ileal segment and ileocystoplasty were performed. The tumors located in the anastomotic site between the bladder and ileal segment as well as in the ileal segment. Histopathological examination revealed poorly-differentiated mucinous adenocarcinoma. The patient has survived 12 months without any evidence of tumor recurrence. To our knowledge, this is the eighth case report in Japan.

Increase of growth-associated protein-43 immunoreactivity following cyclophosphamide-induced cystitis in rats.

We examined the effect of inflammation on immunoreactivity of growth-associated protein (GAP-43) in the rat urinary bladder in which acute cystitis was induced with cyclophosphamide (CPA). Following CPA injection, the number of GAP-43 labeled nerves was significantly increased in the muscle layer. Immunoreactivity of PGP9.5, which was used as an axonal marker, was not augmented following CPA injection. Double fluorescence immunohistochemistry revealed that substance P immunoreactivity was present in most GAP-43 immunoreactive fibers (90.2%) in the inflamed bladder. Electron microscopic examination showed that GAP-43 immunoreactivity was localized on axons. Some GAP-43 positive axons showed degeneration. Possible significance of the increase of GAP-43 immunoreactive afferent nerve fibers in the muscle layer of acutely inflamed bladder was discussed.

[Adenocarcinoma occurring 37 years after augmentation ileocystoplasty for tuberculous bladder atrophy: report of a case].

A 55-year-old woman was admitted with urinary frequency. She had undergone augmentation ileocystoplasty due to tuberculous bladder atrophy 37 years previously. Cystoscopy revealed a tumor on the posterior wall which had been augmented with the ileum. Partial cystectomy and bladder reconstruction using a segment of ileum and ascending colon were performed. Gross inspection showed a 15 x 10 mm, papillary tumor on the ileal mucosa near the vesico-ileal anastomosis. Histologically, moderately differentiated adenocarcinoma infiltrating into the muscle layer was surrounded by the normal ileal mucosa. She has been free of recurrence for 2 years postoperatively. This is the 8th case of adenocarcinoma following augmentation ileocystoplasty reported in the Japanese literature.

Assessment of the availability of intraoperative autotransfusion in urological operations.

PURPOSE: Intraoperative autotransfusion in urological operations has the risks of reinfusing urine constituents, bacteria and cancer cells. We assessed the efficacy of intraoperative autotransfusion and leukocyte removal filters to separate red blood cells from these constituents. MATERIALS AND METHODS: Concentrated red blood cells were mixed with urine, bacteria (Escherichia coli, Pseudomonas aeruginosa and Klebsiella pneumoniae) and tumor cells (KK47 and ACHN), and processed with the intraoperative autotransfusion device. Biochemical analyses of erythrocytes collected in reinfusion bags (collected erythrocyte solution) from the mixtures of concentrated red blood cells and urine (mixture 1), and cultures of collected erythrocyte solution from the mixtures of concentrated red blood cells and bacteria (mixture 2) were performed. Cytology was done on collected erythrocyte solution from the mixtures of concentrated red blood cells and tumor cells (mixture 3) and filtrates were passed through leukocyte removal filters. RESULTS: Biochemical analyses of collected erythrocyte solution from mixture 1 indicated complete removal of urine constituents, while cultures from mixture 2 yielded bacterial growth. Tumor cells from both cell lines were found in the collected erythrocyte solution from mixture 3 although tumor cells were not found in filtrates through leukocyte removal filters. CONCLUSIONS: Our results suggest that intraoperative autotransfusion is safe for urological operations in which bacteria and tumor cells are not present in the operative field. Our data also indicate that intraoperative autotransfusion may be safe for urological cancer operations if it is combined with leukocyte removal filters to prevent unexpected reinfusion of tumor cells.

Menkes' kinky hair disease associated with a large bladder diverticulum: a case report.

We report a 2 year-old boy with Menkes' kinky hair disease associated with a solitary huge bladder diverticulum. To our knowledge this is the first reported case treated successfully by surgical excision under general anesthesia, which has been previously considered hazardous due to the poor general condition of the patient.

Intra-operative autotransfusion in radical cystectomy.

OBJECTIVE: To assess the feasibility of intra-operative autotransfusion (IAT) as a method of decreasing or avoiding homologous blood transfusion during radical cystectomy. PATIENTS AND METHODS: IAT was performed in 10 patients with bladder cancer who underwent radical cystectomy. The patients were divided into two groups: group 1 consisted of six patients without and group 2 of four patients with pre-deposited blood. Blood shed in the operative field was collected and processed with an IAT device. RESULTS: In group 1, the intra-operative blood loss ranged from 1.08 to 2.67 L (mean 1.82) and homologous blood transfusion ranged from 0 to 1 L (mean 0.47). The amount of autologous blood processed and transfused by the IAT device ranged from 0.38 to 0.98 L (mean 0.64). Two patients did not require homologous blood transfusion. In group 2, the volume of pre-deposited blood ranged from 0.8 to 1.2 L (mean 0.9). The intra-operative blood loss ranged from 1.03 to 3.24 L (mean 1.8). The amount of processed autologous blood transfused ranged from 0.4 to 0.6 L (mean 0.45). None of the patients in group 2 required homologous blood transfusion. CONCLUSION: Although there are theoretical risks with IAT, they were not substantiated by the clinical data. IAT seems to be a feasible method of reducing or avoiding homologous blood transfusion in radical cystectomy. These results suggest that IAT in combination with pre-deposited blood will abolish the need for homologous blood transfusion during radical cystectomy.

[Hemo-lymphangioma of the scrotum: a case report].

A 3-year-old boy was referred because of a painless mass in the left scrotum. On palpation, the mass was discriminated from the testis, epididymis and spermatic cord and showed transillumination. A part of the mass was dark purple in color. Total surgical excision of the mass was performed. The lesion was multicystic (2-15 mm), adherent to the scrotal skin, and easily dissectable from the testicle. Pathological examination revealed lymphangioma as a major component and hemangioma as a minor part. He has been free of recurrent disease for 2 years. This is the 7th case of hemo-lymphangioma of the scrotum in the Japanese literature.

Clinical experience of orthotopic urinary reservoirs in male patients with bladder cancer.

Between 1988 and 1996, 23 male patients with bladder cancer underwent bladder substitution after cystectomy, using either the hemi-Kock, Hautmann, and Reddy procedures. The mean postoperative follow-up period was 36 months, with a range of 3 to 85 months. There were no perioperative deaths, and early postoperative complications occurred in 7 patients (30%); transient urine leak from the pouch in 4, wound infection in 3 and pyelonephritis in 2 patients. Twenty-two of the 23 patients (96%) were continent during the day, while 7 (30%) had nocturnal incontinence. All 3 patients with the Reddy procedure had nocturnal incontinence. Complete continence was preserved in 70% of the patients. Dysuria was seen in 4 patients, including retention in 1 patient. Late complications included urethral stricture in 3, wound hernia in 2, metabolic acidosis in 1, stone in the pouch in 1, and gallbladder stone in 1 patient. However, reoperation was necessary in 1 patient for internal urethrotomy and 1 patient for removal of a stone in the neobladder. Mild degree of hydronephrosis and unilateral reflux were seen in 3 patients each, and followed up conservatively. No urethral recurrence has occurred and only 1 patient died of cancer. The need for reoperation was very low and the high reservoir capacity resulted in continence from the beginning in most patients. We considered the neobladder useful as an alternative form of urinary diversion in selected cases.

[Experimental bladder stone production by human uropathogenic bacteria].

To investigate the formation of infection stones by 5 bacterial species isolated from patients with urinary calculi, such as Proteus mirabilis, Staphylococcus saprophyticus, Pseudomonas aeruginosa, Klebsiella pneumoniae and Escherichia coli, a zinc disc was implanted and 10(6) bacteria were surgically inoculated at the same time into the bladder of male Wistar rats (10 rats per group). Urinary pH was measured on days 1, 3, 5 and 7. The rats were killed on day 7 and the bladder examined for the formation of infection stones. Infection stones were formed in all rats inoculated with P. mirabilis (mean stone weight 29.7 mg), and in 60% of those inoculated with S. saprophyticus (9.7 mg). Small bladder stones were formed in 30% of the rats inoculated with K. pneumoniae and 25% of those inoculated with P. aeruginosa. No bladder stones were formed in the rats inoculated with E. coli. Urinary pH was increased by P. mirabilis and S. saprophyticus but was unaffected by the other species. In conclusion, P. mirabilis and S. saprophyticus play a significant role in the formation of infection stones.

[Immunohistochemical study on endothelin in rat kidney].

To elucidate the role of endothelin (ET) in the kidney, we immunohistochemically examined the precise distribution of various forms of ET-peptides in the rat kidney, using specific polyclonal antibodies to each precursor (Big ET-1, Big ET-2 and Big ET-3), and anti-mature ET antibodies. Immunoreactivity of Big ET-1 was localized mainly in some vascular endothelial cells, glomerular mesangial cells and epithelial cells of proximal tubules in the renal cortex and inner-medullary collecting ducts. The distribution of immunoreactivity for mature ETs was similar to those of Big ET-1. These observations suggest that mature ET-1 after conversion from Big ET-1 is secreted from these cells. Since similar findings were obtained using anti-Big ET-2 and anti-Big ET-3 antibodies, these two isopeptides are also considered to have certain physiological actions in the kidney. Immunohistochemical studies for ET-A receptor were also performed. The distribution of this receptor was similar to those of Big ETs and mature ETs. This suggests that secreted ET acts on adjacent cells in an autocrine/paracrine manner in the kidney.

[An experimental study of infection stone formation by Corynebacterium species].

The relationship between infection stone and Corynebacterium species was investigated in vitro and in vivo. Urease activity of urease-splitting Corynebacterium species was evaluated by 2 methods; an increase in pH of human urine after inoculation of Corynebacterium species and direct measurement of urease activity of 10(7) CFU organisms from amounts of ammonia by indophenol method. Formation of infection bladder stone was induced in male Wistar rats by implanting a zinc disc and inoculating 10(6) CFU organisms surgically into the bladder. Urine was alkalinized by the inoculation of Corynebacterium renale, C. pilosum and group D2 Corynebacterium. C. renale and C. pilosum had strong urease activity, and group D2 Corynebacterium had moderate activity. C. pseudodiphtheriticum did not produce the elevation of urinary pH and had little urease activity. Infection stones were formed in 100% of rats by inoculation of C. renale and C. pilosum and 88% of rats by group D2. Urinary pH was elevated in all inoculated rats. In conclusion, C. renale, C. pilosum and group D2 Corynebacterium may play a role in formation of infection stones.

Assessment of the effects of low dialysate flow rates on removal rates and clearance using high flux membranes.

The effects of the low dialysate flow rates on the removal rate and clearance of urea nitrogen, creatinine, uric acid, beta 2-microglobulin and myoglobin, using high flux membranes were studied. The removal rates for all substances were not significantly decreased. Although clearance of urea nitrogen, creatinine and uric acid was significantly decreased (p < 0.05), clearance of inorganic phosphate, beta 2-microglobulin and myoglobin was not significantly decreased. These results suggest that hemodialysis at low dialysate flow rates for a short term during water shortages due to natural disasters and drier climates can be performed with an insignificant reduction in removal rates and a minimum reduction in clearance.

Quality of life survey of urinary diversion patients: comparison of continent urinary diversion versus ileal conduit.

BACKGROUND: Continent urinary reservoirs (CUR) have become one of the major options for patients requiring urinary diversion to improve their quality of life (QOL). To assess whether CUR enhanced postoperative QOL, we surveyed patients with CUR and ileal conduit (IC) using a questionnaire sent by mail. PATIENTS AND METHODS: The questionnaire consisted of 133 questions that covered physical and mental status, social life, sexual habits and symptoms related to urinary diversions. A total of 172 questionnaires were sent out, and 137 (80%) patients (74 CUR and 63 IC patients) responded. RESULTS: Basic physical conditions were similar in the 2 groups, except for sleeping habits. Regarding social life, however, the CUR group showed better scores in bathing habits and frequency of overnight travel. Parastomal dermatitis was more frequent in the IC group and the patients were more hesitant to show their stoma to others. On the other hand, about half of the patients in the CUR group complained of troublesomeness in self-catheterization, especially at night. Overall, 74% and 41% of the patients in the CUR and IC group were satisfied with their urinary diversion. When the Kock pouch and Indiana pouch were compared, no statistically significant differences were found in average capacity, maximum capacity, or frequency of self-catheterization. CONCLUSIONS: CUR recipients have enhanced QOL regarding the stoma, travel and sleeping habits as compared to ileal conduit. However, troublesomeness of night time self-catheterization was noted in the CUR group. Individualized selection of the type of urinary diversion with informed consent is essential.