1/195. 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 = hypertension (Clic here for more details about this article) |
2/195. Successful obstetric outcome after simultaneous pancreas and kidney transplantation.A 34-year-old woman became pregnant two years after having a simultaneous pancreas and kidney (SPK) transplantation, necessitated by type 1 diabetes and end-stage renal disease. The pregnancy was uneventful until 30 weeks' gestation, when she developed pancreatitis and a worsening of mild hypertension. A healthy 1700 g boy was delivered by caesarean section at 34 weeks' gestation. This is the first report of a successful pregnancy after SPK transplantation in australia.- - - - - - - - - - ranking = 1keywords = hypertension (Clic here for more details about this article) |
3/195. Cardiovascular consequences of renal anaemia and erythropoietin therapy.Cardiovascular disease is the leading cause of increased mortality in patients with renal failure and vigorous attention to cardiovascular risk factors is therefore required to improve patient outcome. The availability of recombinant human Epo has focused the interest on the role of chronic anaemia in the pathogenesis of cardiovascular disease. Severalfold evidence indicates that anaemia can contribute to cardiac volume overload and together with overhydration, fistula flow and the pressure overload secondary to arterial hypertension, it may play a significant role in the development of cardiac hypertrophy. As in the general population left ventricular hypertrophy is a severe adverse risk factor in renal patients. In addition, in the presence of ischaemic heart disease anaemia may further worsen cardiac oxygen supply. This dual effect of anaemia probably explains why epidemiological studies have shown that a 1 g/dl decrease in haemoglobin levels is an independent, statistically significant risk factor for the development of cardiac morbidity and mortality. Follow-up examinations have demonstrated that partial correction of anaemia with recombinant Epo can improve cardiac oxygen supply and partially reverse pathological changes in left ventricular geometry. However, although partial anaemia correction regularly reduces left ventricular volume, the effects on wall thickness are far less significant. Moreover, in patients with advanced cardiac disease it has recently not been possible to demonstrate that a normalization of haemoglobin levels provides further benefit. It is not unlikely therefore that the development of severe anaemia has to be prevented by early implementation of Epo therapy in order to achieve the maximum benefit with respect to the cardiovascular system.- - - - - - - - - - ranking = 1keywords = hypertension (Clic here for more details about this article) |
4/195. dialysis-associated renal cystic disease resembling autosomal dominant polycystic kidney disease: a report of two cases.Acquired renal cystic disease is common in patients receiving dialysis. Characteristically, the kidneys are small or, less often, normal in size, and the cysts are usually less than 0.6 cm in diameter. We present here 2 patients who, after 5 and 7 years on hemodialysis, developed marked renal enlargement, with large cysts in the kidneys and, in 1 patient, in the liver as well; the appearance on ultrasonography and computed tomography was indistinguishable from autosomal dominant polycystic kidney disease. Before starting dialysis the first patient was a 19-year-old man who developed renal shutdown from crescentic glomerulonephritis, and the second patient was a 33-year-old man who developed end-stage renal failure from malignant hypertension. Neither patient had renal cysts at the onset of end-stage renal failure.- - - - - - - - - - ranking = 1keywords = hypertension (Clic here for more details about this article) |
5/195. Rapidly resorptive exudative retinal detachment in a patient with renogenic hypertension: case report.We present an 18-year-old woman who developed rapidly resorptive exudative retinal detachment (ERD) due to chronic renal failure and renogenic hypertension. In July 1998, the patient came to our clinic because of a 2-month-history of progressively deteriorating visual acuity. Initially examination of the fundi revealed typical hypertensive retinopathy. Two weeks later, the patient was admitted due to hypertension and consulted our ophthalmic department again. In addition to hypertensive retinopathy, the fundi showed high bullous ERD, involving the temporal retinas in both eyes. Intensive medical therapy was begun, including blood pressure control and maintenance of body fluid and electrolyte balance, resulting in almost complete regression of retinal detachment within two days. The visual acuity improved during the following 2 weeks. The clinical features and treatment response in this rare case indicate that multiple factors, including fluids overload, hypertension, and possibly renal failure, contributed to the development of ERD. blood pressure control and the balance of fluids are important in patients with renal failure, and may help to prevent the occurrence of ERD.- - - - - - - - - - ranking = 7keywords = hypertension (Clic here for more details about this article) |
6/195. Endovascular treatment of arteriovenous fistulas complicating percutaneous renal biopsy in three paediatric cases.DESIGN: We evaluated the incidence and history of arteriovenous fistula (AVF) after kidney biopsy and assessed the use of superselective embolization for treatment. OBSERVATIONS: During the last 10 years, 896 kidney biopsies (age range of the patients: 1 month-18.6 years) have been performed in our institution under real-time ultrasonographic guidance with a 14 gauge cutting biopsy needle, and 32 of the patients had renal allografts (3.4%). We observed three cases of AVF (two in allograft kidneys, one in a native kidney) among all biopsies (0.34%), and the incidence of developing AVF after renal allograft biopsy was 6.3%. All three patients with AVF were symptomatic, and intravascular therapy was indicated. INTERVENTIONS: An angiographic study combined with endovascular treatment of the intrarenal AVF and pseudoaneurysm was performed in all three patients. Embolization was performed with bucrylate and lipiodol in two patients and with micro-coils in one. After successful embolization, all three patients became asymptomatic (in two renal bleeding stopped, in one patient with severe uncontrollable hypertension blood pressure returned to normal limits). No complications were observed secondary to the embolization procedure. CONCLUSION: The technique of superselective embolization using a coaxial catheter is an effective and safe method in the treatment of post-biopsy AVFs and pseudoaneurysm.- - - - - - - - - - ranking = 1keywords = hypertension (Clic here for more details about this article) |
7/195. plasmapheresis for crescentic IgA nephropathy: a report of two cases and review of the literature.Idiopathic IgA nephropathy is widely regarded as a slowly progressive disease that not infrequently results in end-stage renal failure. Only a minority of patients present with either a rapidly progressive form of glomerulonephritis, or with end-stage renal failure. Anecdotal reports of improved renal function after treatment with plasmapheresis have been published, but the efficacy of this therapy remains controversial. We describe the course of two young males presenting with uremia, hypertension, nephrotic-range proteinuria, and crescentic glomerulonephritis on renal biopsy. Both patients underwent therapy with steroids, immunosuppressive agents, and plasmapheresis without an appreciable improvement in renal function. A review of the literature does not offer any conclusive data to support the role of plasmapheresis in the treatment of rapidly progressive glomerulonephritis due to IgA nephropathy and points out the need to define criteria that may identify subsets of patients with this disorder who may potentially benefit from plasma exchange therapy. J. Clin. Apheresis 14:185-187, 1999. Published 1999 Wiley-Liss, Inc.- - - - - - - - - - ranking = 1keywords = hypertension (Clic here for more details about this article) |
8/195. Asymptomatic inferior vena cava abnormalities in three children with end-stage renal disease: risk factors and screening guidelines for pretransplant diagnosis.We report two children with end-stage renal disease (ESRD) found to have inferior vena cava (IVC) thrombosis at the time of renal transplantation. The children suffered from renal diseases that included congenital hepatic fibrosis and portal hypertension as part of their pathophysiology. Neither child had evidence of hypercoaguability or clinical symptoms of IVC thrombosis. Prior to transplantation, the renal replacement therapy consisted primarily of peritoneal dialysis. During their hospital courses, these children had central venous catheters placed for temporary hemodialysis, episodes of peritonitis and numerous abdominal surgeries. The medical literature to date has not identified a link between IVC thrombosis and portal hypertension, nor has an association between the patients' primary renal disease and IVC thrombosis been found. We also report the finding of asymptomatic IVC narrowing in a third patient with obstructive uropathy, colonic dysmotility and numerous abdominal surgeries. IVC narrowing was diagnosed by CT scan during his pretransplant evaluation. In this paper, we consider similarities between these three patients that may have predisposed each of them to asymptomatic IVC pathology, including large-bore central venous access as young children and/or recurrent scarring abdominal processes. A discussion regarding appropriate screening of the 'high-risk patient' for IVC pathology prior to kidney transplantation and surgical options for children with this rare complication are presented.- - - - - - - - - - ranking = 2keywords = hypertension (Clic here for more details about this article) |
9/195. A case of late onset cyclosporine-induced hemolytic uremic syndrome resulting in renal graft loss.A case of late onset hemolytic uremic syndrome (HUS) associated with cyclosporine (CYA) is described in this report. A 50-yr-old man with end-stage renal failure due to immunoglobulin a (IgA) nephropathy received a renal transplant from his wife. Human leucocyte antigen was completely unmatched. Immunosuppressant was a combination of prednisolone, azathioprine, and CYA. He was discharged 1 month after transplantation, with no episode of acute rejection. Twenty-one months after transplantation, his platelet count and hematocrit began to decrease and lactate dehydrogenase began to increase. Graft biopsy showed thrombotic microangiopathy and recurrent IgA nephropathy. Graft function was rapidly deteriorated and methylprednisolone pulse therapy was not effective. Twenty-five months after transplantation, he returned to a regular hemodialysis. hemolysis was immediately improved after a reduction of the dose of CYA to 50 mg/d. The trough level of CYA was less than 200 ng/mL in most periods of his clinical course. blood pressure was high throughout the clinical course. Although acute vascular rejection or malignant hypertension could also cause a thrombotic microangiopathy, CYA was most likely a cause of HUS in the present case because of the following reasons: neither anti-acute rejection therapy nor an adequate control of his blood pressure was effective in improving clinical features of HUS; hemolysis and thrombocytopenia disappeared immediately after the reduction of the dose of CYA to 50 mg/d. It has been reported that HUS carried poor prognosis only when occurring shortly after transplantation in cadaver kidney recipients. The present transplant was from a living donor and HUS occurred 21 months after transplantation and was severe enough to result in graft loss. High blood pressure might be one of the predisposing factors of HUS associated with CYA in the present case. CYA should be stopped and other alternative immunosuppressants should be given in cases of acute graft deterioration with hemolysis and thrombocytopenia, irrespective of the interval from transplantation, CYA dose, or CYA trough level.- - - - - - - - - - ranking = 1keywords = hypertension (Clic here for more details about this article) |
10/195. tacrolimus-induced hemolytic uremic syndrome and end-stage renal failure after liver transplantation.BACKGROUND: Hemolytic uremic syndrome (HUS) is a rare complication in solid organ transplantation. It can be associated with severe hypertension. Several risk factors have been identified including immunosuppressive drugs such as cyclosporin A and, more recently, tacrolimus. methods: Here we report a case of tacrolimus-induced HUS in a 61-yr-old woman after liver transplantation. hypertension, microangiopathic anemia and end-stage renal failure occurred 2 yr after liver transplantation. RESULTS: At admission, she had malignant hypertension with a severe hypertensive retinopathy, renal failure (creatininemia: 800 micromol/L) and microangiopathic anemia (Hb: 7.3 g/dL, a low platelet count and elevated lactate dehydrogenase). At renal biopsy, histologic findings were ischemic and sclerotic glomeruli with hyaline thrombi, severe mesangiolysis and interstitial fibrosis. CONCLUSION: Despite steroid treatment, antihypertensive agents and fresh frozen plasma therapy, end-stage renal failure was observed and chronic hemodialysis treatment was required.- - - - - - - - - - ranking = 2keywords = hypertension (Clic here for more details about this article) |
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