1/89. linear iga bullous dermatosis in a patient with chronic renal failure: response to intravenous immunoglobulin therapy.linear iga bullous dermatosis is a blistering disease with a heterogeneous clinical manifestation, characterized by deposition of IgA along the basement membrane zone of perilesional skin on direct immunofluorescence. We describe a patient with chronic renal failure who experienced linear iga bullous dermatosis. Long-term administration of intravenous immunoglobulin therapy was associated with clinical remission lasting more than 12 months.- - - - - - - - - - ranking = 1keywords = membrane (Clic here for more details about this article) |
2/89. Heavy chain deposition disease: the disease spectrum.A 45-year-old white woman was found to have microscopic hematuria during her annual physical examination. After a negative urologic workup, she returned 5 months later with nephrotic syndrome, renal insufficiency, and hypocomplementemia. Renal biopsy showed a nodular sclerosing glomerulopathy that could not be further characterized because of inadequate tissue for immunofluorescence. The patient returned 8 months later with chronic renal failure. A repeat renal biopsy showed deposits composed of immunoglobulin g (IgG) heavy chain and complement components C3 and C1 along glomerular, tubular, and vascular basement membranes, with negativity for kappa and lambda light chains, findings consistent with heavy chain deposition disease (HCDD). The heavy chain subclass was exclusively IgG3. Staining with monoclonal antibodies to epitopes of the constant domains of IgG heavy chain showed a CH1 deletion, indicating a truncated heavy chain. On review of the previously reported cases of HCDD, common clinical presentations include nephrotic syndrome, renal insufficiency, hematuria, and, in some cases, hypocomplementemia. In most patients, the hematologic disorder is mild, without overt myeloma. light microscopy shows a nodular sclerosing glomerulopathy, and heavy chain deposits are detectable within basement membranes throughout the kidney by immunofluorescence and electron microscopy. There is no effective treatment for this condition, and virtually all patients progress to chronic renal failure.- - - - - - - - - - ranking = 2keywords = membrane (Clic here for more details about this article) |
3/89. hemodiafiltration for vancomycin overdose in a neonate with end-stage renal failure.We describe continuous venovenous hemodiafiltration (CVVHD) with a high-flux membrane as a novel treatment modality for vancomycin overdose associated with renal insufficiency. CVVHD was used in a 6-day-old male with a solitary hypodysplastic kidney, suspected sepsis, and anuric renal failure who subsequently received an accidental tenfold overdose of vancomycin. We furthermore present evidence for the importance of countercurrent dialysis in addition to continuous hemofiltration for optimal vancomycin removal.- - - - - - - - - - ranking = 1keywords = membrane (Clic here for more details about this article) |
4/89. Renal transplantation for end-stage renal disease following bone marrow transplantation: a report of six cases, with and without immunosuppression.BACKGROUND. Over 12000 bone marrow transplantations (BMT) are performed in the USA each year. This procedure is associated with significant morbidity including acute and chronic renal failure (CRF). CRF after BMT is usually secondary to radiation nephropathy and,or cyclosporine (CsA) toxicity. survival on dialysis therapy for patients with radiation nephropathy is poor and renal transplantation may be a preferable form of renal-replacement therapy. methods: We report our experience with renal transplantation in 6 patients with end-stage renal disease (ESRD) following BMT: 4 as a result of radiation nephropathy; one secondary to hemolytic uremic syndrome; and 1 as a result of antitubular basement membrane nephritis. Ages at the time of BMT ranged from 26 to 40 yr. ESRD developed after a mean period of 94 months (range 42-140 months) after BMT. The kidney source was from a living donor in 5 patients, and a cadaveric donor (CAD) in 1 patient. In 3 recipients, the bone marrow and kidney were from the same donor. They are managed without any immunosuppressive therapy. The other 3 were initiated on triple therapy (prednisone, mycophenolate mofetil/azathioprine and cyclosporine/tacrolimus). RESULTS: These patients have been followed for up to 31 months (range 3-30 months) after kidney transplant, and 5 out of 6 are alive with functioning bone marrow and renal transplants. Their plasma creatinines range from 70 to 160 micromol/L (mean 97 micromol/L). One patient died following metastatic squamous cell cancer of the genital tract. CONCLUSIONS: 1) Renal transplant is a feasible alternative for patients with ESRD following BMT: 2) if bone marrow and kidney are from the same donor, the recipient requires little or no maintenance immunosuppression; 3) short-term results show good survival, but long-term follow-up is needed: 4) infections and malignancy post-renal transplantation were seen in recipients who needed immunosuppression; and 5) reduction in immunosuppression may be needed in such post-BMT patients who undergo kidney transplants.- - - - - - - - - - ranking = 1keywords = membrane (Clic here for more details about this article) |
5/89. Neonatal end-stage renal failure associated with maternal ingestion of cyclo-oxygenase-type-1 selective inhibitor nimesulide as tocolytic.Cyclo-oxygenase-type-2 (COX-2) enzyme is fundamental for nephrogenesis, upregulated on fetal membranes and myometrium at parturition. Fetal COX-2 inhibition, due to maternal nimesulide assumption, can be responsible for neonatal chronic renal failure.- - - - - - - - - - ranking = 1keywords = membrane (Clic here for more details about this article) |
6/89. Left atrial calcification in a hemodialysis patient with cor triatriatum.Myocardial calcification is a rare manifestation of abnormal calcium metabolism seen in some patients with chronic renal failure. This report describes the transesophageal echocardiographic and spiral computed tomography (CT) findings in a young hemodialysis female with severe secondary hyperparathyroidism. These findings included calcification of the multiperforated membrane of a cor triatriatum and the wall of the left atrium.- - - - - - - - - - ranking = 1keywords = membrane (Clic here for more details about this article) |
7/89. Familial interstitial nephritis with progressive renal failure.We describe a 53-year-old woman with chronic interstitial nephritis and asymptomatic impairment of renal function. Seven members of her family were suffering from renal failure and underwent hemodialysis. At the time of their hospital admissions, they had shown evidence of end-stage renal failure at 40 to 50 years of age. Lack of proteinuria, hematuria, hypertension, hyperuricemia, hearing loss, and visual impairment were present before the deterioration of the renal function. Renal biopsy of the presented case indicated chronic interstitial nephritis without glomerular basement membrane abnormalities. Progressive decline of renal function and the inheritance pattern of autosomal dominance in this family suggested the diagnosis of familial interstitial nephritis.- - - - - - - - - - ranking = 1keywords = membrane (Clic here for more details about this article) |
8/89. Late-onset renal failure in Senior-Loken syndrome.We report on four patients, from three different families, with Senior-Loken syndrome (SLS). They were unusual in that they reached end-stage renal failure (ESRF) only during the fifth or sixth decade. SLS is an autosomal-recessive disorder defined by the association of nephronophthisis and retinal dystrophy. Affected individuals invariably progress to ESRF, usually before the age of 20 years. The diagnosis was based on typical clinical presentation and characteristic renal histology, that is, a picture of chronic interstitial nephritis with pronounced thickening and multilayering of tubular basement membranes. Deterioration of renal function was slow, leading to ESRF between the ages of 42 and 56 years. Retinal dystrophy, already symptomatic during childhood in two patients, led to severe visual impairment in all. In contrast with four cases of SLS recently reported in very young patients, the NPH1 gene (the main gene responsible for nephronophthisis) was not deleted in our two tested patients. We conclude that SLS should be considered in adults who suffer from both chronic interstitial nephropathy and retinal degeneration. Whether the SLS is a variant of nephronophthisis and whether early- and late-onset renal failure in SLS is accounted for by genetic or allelic heterogeneity remain to be determined.- - - - - - - - - - ranking = 1keywords = membrane (Clic here for more details about this article) |
9/89. Administration and clearance of amphotericin b during high-efficiency or high-efficiency/high-flux dialysis.Administration and clearance of amphotericin b infused during high-efficiency or high-efficiency/high-flux dialysis were studied in two end-stage renal disease patients requiring systemic antimycotic treatment for fungal peritonitis. amphotericin b concentrations were measured in the arterial and venous dialysis ports as well as in the ultrafiltrate. amphotericin b is poorly dialyzable while administered during hemodialysis sessions with high-efficiency (CA 210) or high-efficiency/high-flux (CT 190 G) membranes. amphotericin b infusion during hemodialysis was well tolerated and can be administered conveniently in an outpatient dialysis setting, avoiding prolonged hospitalization for parenteral antifungal therapy.- - - - - - - - - - ranking = 1keywords = membrane (Clic here for more details about this article) |
10/89. Spontaneous cerebrospinal fluid rhinorrhea associated with chronic renal failure--case report.A 39-year-old woman was admitted with complaints of headache and nasal discharge on the left for 3 months which was later on proved to be cerebrospinal fluid (CSF). Neurological examination found no abnormalities except bilateral papilledema. neuroimaging demonstrated enlargement of the lamina cribrosa foramina through which the olfactory nerves pass, as well as empty sella and cerebral cortical atrophy. Bone mineral densitometry showed osteopenia. CSF Ca and blood parathyroid hormone levels were elevated. CSF pressure was 280 mmH2O. Bilateral frontal craniotomy was performed to expose the anterior fossa. Foraminal enlargement at the lamina cribrosa was confirmed, and islands of extra-osseous calcifications on the arachnoid membrane were identified. The base of the anterior fossa was repaired intradurally with fascial graft and fibrin glue on both sides. No CSF leakage was noted at 1-year follow up. Spontaneous CSF leakage probably resulted from enlargement of the foramina at the lamina cribrosa due to Ca mobilization from bones and pseudotumor cerebri not to the extent of hydrocephalus caused by poor CSF absorption at the arachnoid granulations obliterated by extra-osseous calcareous accumulation.- - - - - - - - - - ranking = 1keywords = membrane (Clic here for more details about this article) |
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