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 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.     

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 idiopathic plasmacytic lymphadenopathy with polyclonal hyperimmunoglobulinemia associated with chronic renal failure

A rare case of idiopathic plasmacytic lymphadenopathy with polyclonal hyperimmunoglobulinemia (IPL) associated with chronic renal failure was described in this report. A 73-year-old male was admitted and diagnosed as IPL. IPL is proposed by Mori et al. in 1980. Clinical entity of IPL is (1) Polyclonal hyperimmunoglobulinemia (2) Systemic Lymphadenopathy characterized by remarkable mature plasmacytosis without atypism and by no destruction of the structures. (3) All disease with polyclonal hyperimmunoglobulinemia can be excluded. In this patient, physical findings showed enlarged lymph nodes (1-2 cm) in bilateral nuchal, submandibular, axillary and inguinal lesions. Laboratory examinations showed polyclonal hyperimmunoglobulinemia (especially IgG, IgA), anemia and renal dysfunction. Microscopic observation of hematoxylin-eosin staining in the axillary lymph node showed increased mature plasma cells without evidence of malignant growth. Immunoperoxidase staining showed intracytoplasmatic polyclonal immunoglobulins. IPL is known as invading other organs besides lymph node, for example skin, lung or kidney. This patient showed renal dysfunction (Cr clearance 11 ml/min, severe proteinuria). Nine cases of IPL and multicentric plasma cell type Giant Lymphnode Hyperplasia (GLH) concurrent with renal dysfunction were reported. Only in two of them chronic renal failure were reported. Twice a week hemodialysis improved his condition and laboratory findings.     

Felty综合征并发慢性肾衰竭一例

2009年4月本院收治1例Felty综合征并发慢性肾衰竭患者,现报告如下.患者,女,57岁,农民,主诉无力懒动3年,腰痛1年,恶心、厌食2个月入院.40年前因溃疡行胃大部切除术,有10余年反复对称性手足关节肿痛病史,尤以双腕关节明显,间断自服止痛药物.     

A case of gastric antral vascular ectasia (GAVE) with chronic renal failure

A 76-year-old woman was admitted to our hospital complaining of tarry stool, general fatigue and marked anemia(Hb 5.2 g/dl). Gastric endoscopic findings showed longitudinal red stripes and diffuse erythematous spots, indicating dilated vascular vessels. They resembled the stripes of a watermelon at the gastric antrum. The marked anemia was caused by chronic blood loss from the abnormally dilated mucosal and submucosal capillary veins in the gastric antrum. She was diagnosed as having gastric antral vascular ectasia(GAVE) with chronic renal failure(CRF). The association of GAVE and CRF is considered to be rare according to previous reports in Japan. Endoscopic argon plasma coagulation therapy was performed three times. After therapy, capillary dilatation disappeared, and the marked anemia was greatly improved. Argon plasma coagulation therapy was found to be a safe and effective procedure for this disease. Although GAVE is essentially a benign gastric disease, endoscopic therapy should be the treatment of first choice for this disease.     

A case of living-donor renal transplantation for chronic renal failure caused by secondary amyloidosis

In Japan, amyloidosis is a rare cause of renal failure and of renal transplantation. We treated a patient who underwent a renal transplantation because of chronic renal failure caused by secondary amyloidosis with a good result. The patient was a 50-year-old woman who was diagnosed with secondary amyloidosis and an amyloid kidney. She underwent living donor renal transplantation after about 7 years of hemodialysis. During the 3-year posttransplantation period, she maintained good allograft function with a serum creatinine level about 1.2 mg/dL. Because of amyloidosis is a systemic disease, amyloid kidney patients often experience fatal complications, so the indications for renal transplantation in amyloid patients are still controversial. But if the patient's general condition is good, renal transplantation can be an effective therapy for patients with kidney failure caused by amyloidosis.     

A case of chronic renal allograft failure by effects of elderly donor

Abstract: We experienced a case of chronic renal allograft failure from an elderly donor. A 23-year-old man received living-related transplantation in 1998 . The donor was his father, 66 years old and 69 kg body weight. The recipient was 88 kg and the cause of the renal failure was IgA nephropathy. The graft function decreased 2 months after transplantation. One year later, proteinuria and haematuria developed. Six months later, when the creatinine rose to 2.5 mg/dL, a renal transplant biopsy was performed. Characteristic microscopic features were: diffuse segmental to global glomerulosclerosis, hypertrophy of non-sclerotic glomerulus, tubular atrophy, arteriolar hyalinosis, interstitial lymphocytic infiltration with tubulitis and anti-IgA deposition on glomeruli. The pathological diagnoses were recurrent IgA nephropathy, acute rejection and chronic cyclosporin nephrotoxicity. Diffuse glomerulosclerosis as well as glomerular hypertrophy might be the end result of hyperfiltration, which was a consequence of a small number of functioning nephrons. The old age of the donor and the heavy body weight of the recipient could be risk factors for this case.     

A case of multiple brown tumors with primary hyperparathyroidism

We report a case of large multiple brown tumors in a patient with primary hyperparathyroidism. A 52-year-old woman suffered from pain in the ribs and developed left facial swelling and deformity. CT showed a large destructive osteolytic lesion in the left maxillary sinus. Biopsy showed a lesion with newly formed bone tissue, diffuse giant cells and deposits of hemosiderin. In addition, similar lesions were also observed in the ribs, iliac bones and pelvis. The laboratory data showed hypercalcemia and hyperparathyroidism. Cervical echo and ²⁰¹Tl–^99mTcO^4? scintigraphy demonstrated a right lower swollen parathyroid adenoma. The diagnosis was multiple brown tumors with primary hyperparathyroidism and parathyroidectomy was performed. Follow-up CT showed marked decreases in the size of osteolytic lesions with calcification in the brown tumors compared to pre-treatment findings. These changes were associated with marked improvement in pain and facial deformity. We described a rare case of multiple brown tumors appeared in the maxilla associated with primary hyperparathyroidism.     

A case of allopurinol-induced muscular damage in a chronic renal failure patient

A 73-year-old woman with chronic renal failure developed generalized muscular weakness and pain 6 days after the start of allopurinol treatment(200 mg/day). Routine laboratory tests revealed elevated levels of serum creatine kinase, and the patient was clinically diagnosed as rhabdomyolysis, due probably to severe myositis. A high level of serum oxipurinol, the chief active metabolite of allopurinol, was also revealed. The muscular weakness was relieved in seven weeks with intermittent hemodiafiltration.     

A case of adenocarcinoma in the renal pelvis and urinary bladder in a patient with chronic renal failure

The patient was a 61-year-old male who had undergone right nephrectomy for nephrolithiasis 35 years before. He had been on hemodialysis for 6 years for chronic renal failure caused by left renal staghorn calculus and hydronephrosis. He was admitted to our hospital because of macroscopic hematuria and sense of residual urine which had persisted for 3 months. Cystoscopic examination showed an extensive papillary tumor in the urinary bladder, which histologically was an adenocarcinoma. Giant left hydronephrosis associated with staghorn calculus noted by KUB and CT scan. Total cystectomy, intraoperative radiotherapy, and left nephrostomy were performed under the diagnosis of adenocarcinoma of the urinary bladder and left hydronephrosis. The bladder tumor was found to be a mucinous adenocarcinoma. The patient died from a cerebral hemorrhage on the 17th postoperative day. At autopsy, tumors were found in the pelvis of the left kidney and the middle and lower regions of the ureter, which were adenocarcinoma like the bladder tumor. Adenocarcinoma occurring simultaneously in the renal pelvis and bladder has not been previously reported to our knowledge, in Japan or elsewhere.     

A case of multiple renal hemangioma

A 53-year-old man presented with a right heterogeneous renal tumor detected by ultrasonography (US) on a routine health check. He had no complaint. Computed tomography (CT) revealed a 4 cm-diameter heterogeneous tumor located near a calcification of the right kidney, which was not so enhanced. Subsequent magnetic resonance imaging (MRI) and angiography could not rule out the malignancy of the tumor, so we performed total nephrectomy. Pathological diagnosis was multiple renal hemangioma. Multiple renal hemangioma is a rare disease, being the second reported case in the Japanese literature.     

慢性肾功能衰竭并发转移性钙化一例

患者，女，27岁，6年前曾出现间歇性腰部胀痛。6个月前因反复颜面部、双下肢水肿住院。2个月前出现双下肢疼痛，乏力，不能行走，腹部出现米粒大小红色皮疹，质硬，毛棘状，继而扩散融合成片。余既往史无特殊。     

A case of synchronous multifocal urothelial tumors in a patient with phenacetin abuse]

A 79-year-old male with phenacetin abuse was admitted to our University Hospital for treatment of asymptomatic gross hematuria. Intravenous urograpdy and computed tomography revealed synchronous right renal pelvic carcinoma and bladder carcinoma. Right nephroureterectomy and transurethral resection of bladder tumor (TUR-Bt) were performed. Histologically, right renal pelvic tumor and bladder tumor were both transitional cell carcinomas of grade 2, pT1, and grade 1 = 2, Ta, respectively. Additionally, pathological examination revealed two distal ureteral tumors, which were transitional cell carcinomas of grade 2, pTa. He also had a history of heavy tobacco-smoking (20 cigarettes per day for 50 years). We discuss the relationship between transitional cell carcinoma and phenacetin abuse as well as the influence of tobacco-smoking, and review the literature.     

Acute obstructive renal failure after laparoscopic cryoablation of multiple renal tumors in a solitary kidney

A 67-year-old man with a solitary left kidney and baseline serum creatinine of 3.3 ng/mL (normal range 0.7 to 1.4) underwent laparoscopic cryoablation of two enhancing, solid renal tumors. Postoperatively, the case was complicated by a large blood clot in the renal collecting system causing obstruction and anuria. This complication of cryotherapy, never before reported to our knowledge, was treated successfully with a temporary ureteral stent. Follow-up magnetic resonance imaging at 3 months did not demonstrate any new lesions or enhancement of the treated renal masses. His serum creatinine at 3 months was 3.7 ng/mL.