A case of renal cell carcinoma associated with idiopathic thrombocytopenic purpura

A 29-year-old woman was referred to another hospital with complaints of bruising and ecchymosis and thrombocytopenia (12,000/microl) was pointed out. After some examinations, the patient was diagnosed with idiopathic thrombocytopenic purpura and was started on steroid therapy. Then the patient consulted our hospital and computerized tomography revealed a left renal tumor 4 cm in diameter. Under the diagnosis of left renal neoplasm we performed left nephrectomy and splenectomy with preoperative high-dose intravenous gammaglobulin treatment. Pathological examination revealed clear cell carcinoma. After the operation, the platelet count increased gradually. We should consider bleeding tendency by thrombocytopenia and side effect of long-term steroid treatment when we perform operations on patients with idiopathic thrombocytopenic purpura.     

A case of renal pseudotumor associated with chronic pachymeningitis

BACKGROUND: A 56-year-old woman was referred to our hospital with a left renal mass. METHODS/RESULTS: Radiologic studies demonstrated a solitary space-occupying lesion in the left kidney and a malignant tumor was suspected. Left radical nephrectomy was then performed. Pathological examination revealed a sclerotic fibrous lesion with a rather distinct margin and no evidence of malignancy. These pathological findings were consistent with the diagnosis of a renal pseudotumor. CONCLUSIONS: This patient had a history of chronic pachymeningitis that formed a thoracic epidural focus causing spinal cord compression and the histologic appearance of this focus was similar to the renal lesion. It was concluded that this was a rare case of a renal pseudotumor associated with multifocal fibrosclerosis.     

Primary aldosteronism associated with chronic renal failure

An aldosterone-producing adenoma (APA) was associated with chronic renal failure. Following treatment with Trilostane (a 3-hydroxysteroid dehydrogenase inhibitor), furosemide and continuous ambulatory peritoneal dialysis (CAPD), a left adrenal adenoma was successfully removed. This patient, still being treated with CAPD, is a unique example of primary aldosteronism without showing suppressed plasma renin activity.     

Bilateral ureteral tumors associated with chronic renal failure: a case report

A 66-year-old female with bilateral ureteral tumors associated with chronic renal failure is presented. She received pan-hysterectomy due to uterine cancer in 1957. She was first referred to our clinic to make internal shunt under a diagnosis of chronic renal failure. In 1979, the diagnosis of neurogenic bladder and bilateral vesicoureteral reflux (rt; grade 3, lt; grade 1) was made. She was admitted to our clinic with complaints of macroscopic hematuria and a temperature of 39 degrees C on April 28, 1983. Cystoscopically, pyuria from the right ureteral orifice was found. Right retrograde pyelography revealed severe dilatation of the right ureter and renal pelvis with some filling defects. For drainage of pus retaining in the right renal pelvis, right percutaneous nephrostomy was made under the guidance of ultrasonography. After her general condition improved, right nephroureterectomy was performed under the diagnosis of right pyonephrosis on June 8, 1983. Right pyelonephritis and right ureteral tumor, grade 3, were pathologically demonstrated. After the operation, an invasive bladder tumor was detected on cystoscopy and ultrasonography, subsequently a total of 3,900 rad irradiation was given to the bladder tumor. She died of pulmonary edema 7 months later. Autopsy demonstrated a transitional carcinoma, grade 3, of the left ureter. Bilateral urothelial tumors of the upper urinary tract is rare, and to our knowledge only 29 cases have been reported in Japan.     

A case of multiple urothelial tumors with chronic renal failure

We report a case of multiple urothelial tumors (left renal pelvis, ureter and bladder) with chronic renal failure in a 72-year-old man. The patient was admitted because of gross hematuria with increasing volume and intervals on September 14, 1985. Admission evaluation including excretory urography, retrograde pyelography, computed tomography and cystoscopy revealed multiple urothelial tumors in the left renal pelvis, ureter and bladder. Radical surgery, however, was postponed because of pneumothorax induced by an inadvertent insertion of the CVP catheter at operation. Subsequent respiratory disturbance persisted so that he was observed at the outpatient clinic following right ureterocutaneostomy. Gradual increase in anemia and decrease in renal function, however, prompted another admission. Gross hematuria necessitating frequent blood replacement could not be controlled by transurethral resection of bladder tumors. Therefore left nephroureterectomy with resection of bladder cuff was performed after internal arteriovenous shunt had been established, because favorable results regarding tumor resection were obtained from preoperative evaluations. He showed satisfactory recovery and was spared hemodialysis despite eventful postoperative course with transient decrease in renal function. The patient was discharged on 130th postoperative day and is now being followed up at the outpatient clinic. The relevant literature is also reviewed briefly.     

A case of Fabry's disease with chronic renal failure]

Fabry's disease is a genetic disorder caused by the absence of alpha-galactosidase (alpha-Gal), the gene of which is carried on the long arm of the X chromosome. This enzymatic defect leads to an accumulation of glycosphingolipids in the plasma and lysosomes of endothelial, perithelial, and smooth muscle cells, especially involving those of the cardiovascular, renal and cerebrovascular systems. We report one male case of Fabry's disease with renal deterioration. A 36-year-old man who was a classic case with acroparesthesia, angiokeratoma, and hypohidrosis from 10 years of age, was diagnosed to be a hemizygote of Fabry's disease at 27 years as a result of severe decreased alpha-Gal activity of his peripheral white blood cells. This patient was found to have a point mutation of a G to A transition in exon 1. In May, 1989, he was reported to have proteinuria with normal renal function and admitted to our hospital due to renal deterioration in September, 1993. Laboratory examinations revealed a serum urea nitrogen of 65 mg/dl and creatinine value of 6.9 mg/dl. Urinary protein excretion was 3.9 g/day and urinary sugar was negative. On the renal biopsy specimens, light microscopic examinations revealed multiple sclerosing and collaptic lesions in glomeruli without severe tubulo-interstitial damage, but with stenotic change of the small arteries and arterioles. Electron microscopic examinations revealed a large number of electron dense deposits in the tubules. We diagnosed this case as Fabry's disease with chronic renal failure, however the pathogenesis of this renal progressive deterioration remained obscure. In this case, degenerative changes in the renal vessels due to Fabry's disease may be associated with rapid deterioration in renal function.     

A case of calciphylaxis in chronic renal failure

Calciphylaxis is characterized by progressive vascular calcification, soft tissue necrosis, and ischemic necrosis of the skin. The condition is usually associated with end-stage renal disease and has a poor prognosis. We present a 76-year-old man on hemodialysis who developed small, painful purpura on the thigh. The purpura rapidly spread to his back and hip and became ulcerated. Histological examination of a skin biopsy revealed arterial calcification in the subcutaneous adipose tissue. We therefore diagnosed calciphylaxis and administered intravenous antibiotics and debrided the necrotic soft tissue. However, the lesions did not heal and the patient died from sepsis related to cellulitis.     

A case of idiopathic chronic interstitial nephritis progressed to renal failure without proteinuria nor hematuria]

A case of idiopathic interstitial nephritis who underwent to chronic renal failure without history of hematuria nor proteinuria is discussed. A 46 years old woman who showed gradually elevation of serum creatinine (1.3-2.5 mg/dl) admitted on our hospital. On occasions of pregnancy, health examination or hospital visit, she has never been pointed out hematuria nor proteinuria. Immunological disorders such as SLE, metabolic diseases, urinary tract obstruction and chronic urinary tract infection were excluded by the examinations after admission. Because of the severe enzymuria (beta 2-microglobulin, N-acetyl glucosaminidase), chronic interstitial nephritis was considered, and renal biopsy was performed. Severe tubulointerstitial changes were observed histologically, however, glomerular damage was comparatively mild. From these results, she was diagnosed idiopathic chronic tubulointerstitial nephritis. In this case, hematuria and proteinuria were absent until severe renal dysfunction. This may be caused by that inflammation was located to the tubulointerstitial area. The observation of enzymuria seemed to be important to diagnosis and follow-up of the interstitial nephritis.     

A case of acute renal failure in a patient with idiopathic hypouricemia]

This report is a 17-year-old man with an acute renal failure who complained of nausea, vomiting, bilateral loin pain and abdominal pain after scuffle. Renal biopsy specimen obtained from the left kidney revealed acute tubular necrosis. After recovering renal function he showed extreme hypouricemia (serum uric acid, 0.6 mg/dl) and elevated uric acid clearance (62-78 ml/min). The fractional excretion of uric acid (CUA/Ccr) could not be influenced by either oral pyrazinamide or probenecid. As no other renal tubular or metabolic abnormalities were detected, it is suggested that presecretory reabsorption defect or subtotal defect in uric acid transportation was responsible for the hypouricemia in this patient.     

Clostridium difficile colitis associated with chronic renal failure

Background: Clostridium difficile-associated diarrhoea (CDAD) is a potentially life-threatening illness which has been shown to be more common and more severe in patients with chronic renal failure (CRF) than in other groups. A review of CDAD in our nephrology unit was carried out. Methods: A review of microbiology and histology records identified 32 cases of CDAD in the nephrology unit over a 14-month period. Patient notes were reviewed to identify risk factors, clinical features and outcome. Available isolates of C. difficile underwent 16S ribosomal RNA typing. Results: The incidence of CDAD in the nephrology unit was 10.7 per 1000 admissions, compared to 2.7 per 1000 in other areas of the hospital (P<0.0001). CDAD was considered the sole or principal cause of death in six (19%) and was considered a contributing factor in a further seven (22%). Mortality was significantly higher among patients with established CRF (P=0.04). Seven cases occurred as a cluster, over a 1-month period. Isolates from this cluster, along with comparative strains from other areas of the hospital, were found to be PCR type 1. Diarrhoea occurred in 28 (89%) of cases, pyrexia in 17 (53%) and ileus or abdominal pain in 14 (44%). Six patients responded to discontinuation of antibiotics alone and 22 required metronidazole and/or vancomycin. Three patients had colectomy and one caecostomy because of toxic megacolon. Four patients died before specific therapy could be given and in two of these cases the diagnosis was made at autopsy. Twenty-six patients had a record of recent antibiotic therapy. Of these, 15 had at least one agent considered to be inappropriate (excessively broad spectrum agent in 11, excessive duration of therapy in four). Nine patients had only received antibiotics prior to admission. Conclusions: CDAD carries a high mortality in nephrology patients especially those with established CRF. The diagnosis may be missed if a careful antibiotic history is not taken, including agents received prior to admission. Rational antibiotic prescribing and adherence to infection control measures are vital to reduce the incidence of this serious condition.     

渴感减退性高钠血症并发慢性肾功能衰竭一例

患者，女性，27岁，因渴感减退、发现血钠升高3年余，Scr升高3年于2006年9月12日入院。患者2003年出现渴感减退．头颅核磁共振成像检查发现鞍区占位病变，血Na波动于167-176mmol／L，血K2．9-3．9mmol／L，Scr75μmol／L。2003年10月21日至2003年11月行全脑放疗12次，放疗后肿瘤至今未见复发。放疗后渴感消失，血Na最高197mmol／L，Scr升高至506μmol／L，BUN28．47mmol／L。当地医院给予不规则补液等治疗，血Na波动150-202mmol／L，Scr143-547μmol／L。2005年12月出现纳差、乏力、间断嗜睡，尿量2000ml／d。查Scr761μmol／L，Hb47g／L。B超示双肾萎缩，诊为慢性肾功能衰竭，尿毒症期。予规律血液透析（2次／周）及纠正贫血等治疗。患者既往无龙胆泻肝丸等服用史。     

A case of recanalization of LV-RA communication associated with tricuspid regurgitation complicating active endocarditis in chronic renal failure

Open heart surgery (patch closure of the defect and tricuspid valvuloplasty with DeVega's annuloplasty) has been successfully performed on 35-year-old woman who has had a history of VSD closure and was undergoing hemodialysis. The patient has also been under treatment for active endocarditis due to streptococcal infection. Hemofiltration method was used during cardiopulmonary bypass. We describe our experience and discuss the management of the problems in this case.     

A girl with Peters plus syndrome associated with myelomeningocele and chronic renal failure

A girl was born with sclerocornea of the right eye, corneal staphyloma of the left eye and lumbar myelomeningocele. The myelomeningocele was repaired soon after birth. The corneal staphyloma was perforated during infancy. She received keratoplasty and achieved light perception. Her right kidney revealed multicystic dysplasia and was non-functioning at birth. She had neurogenic bladder, and her renal function deteriorated gradually. Peters plus syndrome was diagnosed based on anterior ocular segment anomalies, short stature, developmental delay and characteristic face. Anterior ocular segment anomalies are rare findings, but seem to be occasionally associated with spina bifida and renal anomalies. Myelomeningocele and chronic renal failure in patients with Peters plus syndrome have not been reported to our knowledge.     

A case of chronic subdural hematoma associated with idiopathic thrombocytopenic purpura (ITP)

A 41-year-old male was admitted to our service with right occipital pain as his chief complaint. CT and MRI examinations revealed bilateral chronic subdural hematomas. The patient had also been affected with ITP since the age of 28. Since emergency operation was thought to be dangerous, he was transferred to Tokushima University Hospital, and treated preoperatively by administration of steroids and a large dose of immunoglobulin. When his platelet count had returned to 146,000/mm3, evacuation of the hematoma through burr holes was performed successfully under local anesthesia. The postoperative course was uneventful. So far as we have been able to find in the literature, only 3 cases of ITP complicated by chronic subdural hematoma have been reported. The characteristic clinical feature of these 4 cases including our own case was noted as the absence of a history of trauma. However, the etiological relationship between ITP and chronic subdural hematoma was controversial. Occurrence of chronic subdural hematoma in patients with ITP and in patients under hemodialyzer treatment is very rare. However, intracerebral hemorrhages are rather common among such patients. So it was suggested that the tendency to bleeding among patients with ITP, and among hemodialyzer patients may contribute little as an etiological factor in the evolution of chronic subdural hematoma.