1/541. Renal granulomatous sarcoidosis in childhood: a report of 11 cases and a review of the literature.We analysed retrospectively 11 children with renal granulomatous sarcoidosis confirmed by renal histology in order to describe the course and prognosis of the disease. Symptomatic sarcoidosis was diagnosed at a mean age of 10.1 years. Nine children had renal involvement at the time of diagnosis. In the course of the disease, nine patients developed renal failure and mild proteinuria, seven had transient sterile leukocyturia, four showed microscopic haematuria, seven had a urinary concentrating defect, and enlarged kidneys were seen in three patients. One child had hypercalcaemia and hypercalciuria, none had hypertension. light microscopy of the kidney showed interstitial infiltration by mononuclear cells in all children, interstitial fibrosis in nine patients, epithelioid granulomas in seven, tubular involvement in eight, and mild glomerular involvement in seven patients. Renal immunofluorescence was negative. Ten children received prednisone for 1-11 years. After a mean follow up of 5.5 years, three patients had entered end-stage renal failure and one had chronic insufficiency after interruption of medical supervision and prednisone therapy. CONCLUSION: Renal failure, proteinuria, leukocyturia, haematuria, and concentration defect are the prominent features of renal granulomatous sarcoidosis in children. Steroid therapy, adjusted according to disease activity, may prevent end-stage renal failure.- - - - - - - - - - ranking = 1keywords = stage, end (Clic here for more details about this article) |
2/541. Remission of the nephrotic syndrome in a patient with renal amyloidosis due to rheumatoid arthritis treated with prednisolone and methotrexate.A 46-year-old woman developed nephrotic syndrome secondary to rheumatoid arthritis (RA). A renal biopsy showed deposition of amyloid fibrils in the subendothelial space of the glomerular capillary walls. After treatment with prednisolone (PSL, 40 mg/day), the levels of c-reactive protein (CRP) and serum amyloid A decreased to within normal limits for 2 weeks. However, the nephrotic syndrome persisted for 6 months after the therapy. To maintain the suppression of disease activity and to reduce PSL, methotrexate (5 mg/week) was added. The nephrotic syndrome resolved gradually, and the level of serum albumin returned to normal. Although renal prognosis of patients with nephrotic syndrome due to amyloidosis caused by RA has been considered poor, adequate and long-term treatment of RA with antiinflammatory drugs, including PSL and methotrexate, is useful for patients with secondary amyloidosis complicated by RA.- - - - - - - - - - ranking = 0.1561194033906keywords = end (Clic here for more details about this article) |
3/541. The use of fluconazole as a local irrigant for nephrostomy tubes.OBJECTIVES: Few data exist concerning the combined use of fluconazole systemically and as an irrigant for nephrostomy tubes in a patient with renal candidiasis. The patient described here presented with renal fungal balls obstructing the drainage of urine from her nephrostomy tubes. methods: Twelve months after chemoradiation for a stage IIB squamous cell carcinoma of the uterine cervix, a 35-year-old woman presented with renal obstruction necessitating insertion of ureteral stents. After 6 months of chemotherapy, the patient developed uremia. After nephrostomy tubes were placed, renal candidiasis was noted, and fluconazole was begun systemically. When the renal candidiasis failed to clear, nephrostomy tube irrigations were begun. RESULTS: Fourteen days of therapy with fluconazole resulted in the resolution of the uremia. The patient died 6 months later with her nephrostomy tubes in situ and without evidence of candidiasis in her urinary tract. CONCLUSIONS: The patient described was successfully treated without having to remove her nephrostomy tubes. Two other authors have reported the successful use of fluconazole irrigation to treat candidiasis in nephrostomy tubes that was unresponsive to systemic fluconazole. Before the appearance of these reports, the best results were obtained with removal of the catheter in renal candidiasis.- - - - - - - - - - ranking = 0.3438805966094keywords = stage (Clic here for more details about this article) |
4/541. Delayed methotrexate clearance in a patient with sickle cell anemia and osteosarcoma.A 15-year-old girl with homozygous sickle cell anemia (HbSS) and osteosarcoma is described. Delayed clearance of methotrexate (MTX) after the second course of high-dose MTX (HDMTX) led to the development of renal and hepatic toxicities. Rescue was accomplished with high-dose leucovorin, intravenous carboxypeptidase G2, and thymidine. Although the renal and hepatic abnormalities resolved, focal tonic-clonic seizures developed, accompanied by abnormal brain imaging. Four weeks after this episode, all clinical and biochemical abnormalities resolved. Preexistent end-organ damage associated with HbSS may compromise the ability to deliver high-dose chemotherapy with curative intent in patients with malignant disease.- - - - - - - - - - ranking = 2.6249880110258keywords = liver, end (Clic here for more details about this article) |
5/541. Subcapsular hemorrhage of adult Wilms' tumor.Wilms' tumor is a rare malignancy of kidney in adults. It usually cannot be differentiated from other renal masses preoperatively. This is a presentation of a case of adult Wilms' tumor who developed spontaneous hemorrhage during radiological evaluation. As tumor rupture can change the stage of the tumor and alter the prognosis of the patient, urgent work-up and treatment is advised.- - - - - - - - - - ranking = 0.3438805966094keywords = stage (Clic here for more details about this article) |
6/541. Fine-needle aspiration findings in idiopathic retroperitoneal fibrosis.Idiopathic retroperitoneal fibrosis (IRF) is an inflammatory and fibrosing process that can be complicated by periureteral encasement, ureteral obstruction, and subsequent renal failure if left untreated. Unfortunately, treatment is often delayed due to the nonspecific nature of the presenting signs and symptoms. Clinical, radiologic, and microscopic findings in IRF, if examined independently, are all nonspecific for its diagnosis. Rendering a diagnosis of IRF by fine-needle aspiration (FNA) requires supportive clinical and radiologic data and systematic evaluation of entities in the differential diagnosis. Herein we report 2 cases of IRF diagnosed by FNA with subsequent histologic confirmation. Smears prepared from the aspirates revealed a combination of inflammatory cells and fibrous tissue. The inflammatory component was comprised of a mixture of lymphocytes, plasma cells, and rare eosinophils and mast cells. These 2 cases represent, to our knowledge, only the second report of IRF diagnosed by FNA.- - - - - - - - - - ranking = 0.31223880678121keywords = end (Clic here for more details about this article) |
7/541. serratia marcescens renal abscess with endophthalmitis: a case report.A renal abscess, caused by serratia marcescens with endophthalmitis in a 68-year-old diabetic female, is described. endophthalmitis presented with visual loss, conjunctiva injection and lid edema with eye pain. Right costovertebral knocking pain was also noted. Sonography and computed tomography of abdomen showed a 4 cm hypoechoic lesion in the middle portion of the right kidney with marginal enhancement after contrast media injection. Percutaneous abscess drain was performed. Pus culture from the drain tube revealed S. marcescens, yet, vitreous cultures yielded no growth, which was ascribed to previous antibiotics use. Although vitrectomy, fortified eye drops, intravitreal and systemic intravenous antibiotics were administered, the visual function was still lost. To our knowledge, this is the first reported case of S. marcescens renal abscess complicated with endophthalmitis.- - - - - - - - - - ranking = 0.93671642034363keywords = end (Clic here for more details about this article) |
8/541. Renal involvement of thrombotic thrombocytopenic purpura: special reference to the glomeruloid structures.We report the case of a 9-year-old girl with biopsy-proven renal thrombotic microangiopathy in thrombotic thrombocytopenic purpura (TTP), with particular reference to the glomeruloid structures. The renal biopsy sample from this TTP patient revealed platelet thrombus deposition, a glomeruloid structure and aneurysm with relative sparing of the glomeruli. The glomeruloid structure displayed a proliferation of mainly capillary-sized channels lined by factor viii-related, antigen-positive plump endothelial cells embedded in the edematous connective tissue. These glomeruloid vessels communicated with the aneurysmal segment at the end portion of the arteriolar branch. We believe that the glomeruloid structures in TTP represent not merely organization or recanalization of thrombus but rather active angiogenesis through aneurysmal dilation in the arteriolized vessel, probably initiated by platelet agglutination.- - - - - - - - - - ranking = 0.31223880678121keywords = end (Clic here for more details about this article) |
9/541. Hereditary renal amyloidosis associated with variant lysozyme in a large English family.BACKGROUND: Two kindreds with hereditary systemic amyloidosis caused by the first two mutations to be described in the human lysozyme gene were discovered recently and study of the variant lysozyme has been powerfully informative about mechanisms of amyloid fibrillogenesis. However, the clinical manifestations in these families, additional members of which have lately been identified, have not previously been reported in detail. methods: The proband presented with proteinuria aged 50 and a family history of amyloidosis, and underwent renal biopsy, whole-body serum amyloid P component (SAP) scintigraphy, and sequencing of the lysozyme gene. Her family history and the phenotype of hereditary lysozyme amyloidosis were thoroughly documented and compared with the presentation and natural history of all other known patients with this condition. RESULTS: The proband belonged to an extended English family other members of which were known to have hereditary lysozyme amyloidosis. Those with amyloid in previous generations presented with renal involvement, frequently developed complications due to gastrointestinal amyloid, and died before age 60. All amyloid deposits were composed of lysozyme and complete concordance was established between amyloid and heterozygosity for a point mutation in the lysozyme gene, encoding the previously reported Asp67His substitution in the mature protein. CONCLUSION: The phenotype, reported for the first time in this extended kindred, contrasts with that of an apparently unrelated family carrying the same mutation who presented with spontaneous hepatic haemorrhage and rupture, and with the manifestations in a family with the lysozyme Ile56Thr variant who presented with dermal petechiae before proceeding to fatal visceral amyloidosis. A remarkably wide spectrum of disease can be caused by the same amyloid fibril protein, although renal involvement predominates in all cases except those dying of hepatic rupture.- - - - - - - - - - ranking = 0.31223880678121keywords = end (Clic here for more details about this article) |
10/541. Retroperitoneoscopic nephrolympholysis and ureterolysis for management of intractable filarial chyluria.PURPOSE: To evaluate the feasibility of retroperitoneoscopic lymphatic disconnection in patients with intractable filarial chyluria with the aim of reducing the morbidity of the surgery. patients AND methods: Two patients presented to us with intractable chyluria. They did not respond to conservative measures and endoscopic sclerotherapy. In view of the severe lipid and protein loss associated with recurrent bouts of chyluria, they merited surgical correction by lymphatic disconnection. Because this operation requires extensive mobilization within the retroperitoneum over a large area, it necessitates a large flank or midline incision. In order to reduce the incision-related morbidity, it was decided to undertake the procedure laparoscopically. The retroperitoneal route was chosen for its obvious advantages. RESULTS: Surgery was carried out uneventfully in both patients, with a mean operating time of 2 hours. The patients stayed in the hospital for an average of 2.5 days. Chyluria resolved in the immediate postoperative period, and they have been asymptomatic over a follow-up of 30 and 18 months. One patient had drainage of lymph for 5 days, which resolved spontaneously. CONCLUSIONS: The objectives of open surgery can be achieved by the minimally invasive approach of retroperitoneoscopy. The new approach significantly reduces the incision-related morbidity without compromising the principles of open surgery. We propose five important steps necessary to avoid recurrence and reduce postoperative morbidity.- - - - - - - - - - ranking = 0.1561194033906keywords = end (Clic here for more details about this article) |
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