1/423. Autosomal dominant polycystic kidney disease associated with familial sensorineural deafness.Autosomal dominant polycystic kidney disease (ADPKD) is characterized by both renal and non-renal disorders. Extrarenal involvement includes noncystic manifestations such as cardiovascular abnormalities, colonic diverticula and intracranial aneurysms. Familial sensorineural hearing loss (SNHL) has been included in the definition of Alport's syndrome. However, other types of nephropathy have been occasionally associated with hereditary deafness. The association of ADPKD with hereditary SNHL has not been previously documented. We report a family with ADPKD associated with bilateral sensorineural deafness in a pedigree of four affected members in four generations.- - - - - - - - - - ranking = 1keywords = family, member (Clic here for more details about this article) |
2/423. Parathyroid carcinoma secondary to prolonged hyperplasia in chronic renal failure and in coeliac disease.The presentation of parathyroid carcinoma in patients with chronic renal failure is rare, although with improvements in life expectancy associated with this condition there have now been 12 reported cases, including the first case we report here. It has been proposed that in these cases there has been a malignant transformation of benign parathyroid hyperplastic tissue. We also report the first case of parathyroid carcinoma associated with coeliac disease and suggest that the same mechanism may be responsible. We review the presentation, diagnosis, treatment and natural history of the disease.- - - - - - - - - - ranking = 0.030857023813947keywords = life (Clic here for more details about this article) |
3/423. propofol infusion for induction and maintenance of anaesthesia in patients with end-stage renal disease.We have investigated the pharmacokinetics and pharmacodynamics of propofol in 11 patients with end-stage renal disease (ESRD) compared with nine healthy patients during and after a manually controlled three-stage infusion of propofol 21, 12 and 6 mg kg-1 h-1 lasting a minimum of 2 h. Mean total body clearance was not reduced significantly in the ESRD group (30.66 (SD 8.47) ml kg-1 min-1) compared with the control group (33.75 (7.8) ml kg-1 min-1). ESRD patients exhibited a greater, but not statistically significant, volume of distribution at steady state compared with patients in the control group (11.25 (8.86) vs 5.79 (2.14) litre kg-1, respectively). Elimination half-life values were unchanged by renal failure. Mean times to induction of anaesthesia were similar in both groups: 177 (SD 57) and 167 (58) s for the ESRD and control groups, respectively. Waking time after cessation of propofol infusion was significantly shorter in the ESRD group (474 (156) s) compared with the control group (714 (240) s) (P < 0.05). Mean plasma concentrations on waking were similar. We conclude that the pharmacokinetic and pharmacodynamic profiles of propofol after infusion were not markedly affected by renal failure.- - - - - - - - - - ranking = 0.030857023813947keywords = life (Clic here for more details about this article) |
4/423. Focal segmental glomerulosclerosis: a need for caution in live-related renal transplantation.Focal segmental glomerulosclerosis (FSGS) has increasingly been recognized to occur in a familial pattern. We have observed the development of biopsy-confirmed FSGS and subsequent end-stage renal disease (ESRD) in one live related kidney donor and ESRD without biopsy in another. Both donors had family members with ESRD secondary to FSGS. Both donors were apparently healthy by routine physical examination, urinalysis, and serum creatinine at the time of evaluation as live related donors. We believe these cases emphasize the need for great caution when evaluating siblings as potential live related donors.- - - - - - - - - - ranking = 1.2456598144217keywords = family, family member, member (Clic here for more details about this article) |
5/423. Post transplant CD8 gammadelta T-cell lymphoma associated with human herpes virus-6 infection.Gammadelta T-cell lymphoma is a rare T-cell lymphoproliferative disorder that has been reported in both immunocompetent and immunocompromised persons. This report describes a forty eight year old patient who developed gammadelta T-cell lymphoma four years after undergoing living-related kidney transplantation. The lymphoma expressed CD2, CD3, CD7, CD8 and CD56, and the gammadelta T-cell receptor and did not express CD5, CD4 and the alphabeta T-cell receptor. In addition, HHV-6 was cultured from the patient's bone marrow, marking the first time that this virus has been associated with gammadelta T-cell lymphoma. Since all patients with gammadelta T-cell lymphoma described to date have responded poorly to standard combination chemotherapies, the patient was treated with the purine analogue 2-chlorodeoxyadenosine. While he responded transiently to treatment, long term remission was not achieved indicating that additional therapeutic approches still need to be developed, for the management of this disorder.- - - - - - - - - - ranking = 0.030857023813947keywords = life (Clic here for more details about this article) |
6/423. Intravascular and diffuse dermal reactive angioendotheliomatosis secondary to iatrogenic arteriovenous fistulas.Reactive angioendotheliomatosis is a rare benign process that has been mainly described in patients with systemic infections, such as subacute bacterial endocarditis or tuberculosis, and in association with intravascular deposition of cryoproteins. Histopathologically, it is characterized by a proliferation of endothelial cells within vascular lumina resulting in the obliteration of the involved vessels. Another rare variant of reactive angioendotheliomatosis has been described in the lower extremities of patients with severe peripheral vascular atherosclerotic disease. It consists of violaceous and purpuric plaques histopathologically characterized by diffuse proliferation of endothelial cells interstitially arranged between collagen bundles of the reticular dermis. This second variant has been named diffuse dermal reactive angioendotheliomatosis. We report two patients with reactive cutaneous angioendotheliomatosis appearing distally to arteriovenous fistulas used for hemodialysis because of chronic renal failure. The first patient showed intravascular reactive angioendotheliomatosis, while the second one had purpuric plaques that were characterized histopathologically by diffuse dermal angioendotheliomatosis. Both patients showed an arteriovenous "steal" syndrome with distal ischemia, and it is possible that a local increase of vascular endothelial growth factor, as is the case in hypoxia situations, induces the endothelial proliferation. To the best of our knowledge, cutaneous reactive angioendotheliomatosis has not been previously described in association with arteriovenous shunts.- - - - - - - - - - ranking = 0.092571071441841keywords = life (Clic here for more details about this article) |
7/423. Chronic renal failure in infancy. Two dietetic case reports.Dietetic care of infants with end stage renal disease (ESRD) involving intensive nutritional support and frequent monitoring in attempts to optimise growth has not been previously quantified. We describe the progress of two male infants born with ESRD due to renal dysplasia. child A and child B were commenced on continuous cyclic peritoneal dialysis at mean age of 3 months and required nutritional support via a gastrostomy button. Energy intakes pre-dialysis (147 kcal/kg) and energy and protein intakes during the first year of life on CCPD (137 kcal/kg, 2.6 g/kg actual body weight) were greater than recommended for the healthy population. Over the 2 year period without growth hormone, height SDS increased from -1.66 to -0.17 and 0.67 to 0.78 and weight SDS increased from -1.26 to -0.43 and 0.31 to 1.75 for child A and child B respectively. Mean dietetic contacts (in/out patient and telephone) over the 2 years were 11.8 contacts/mth pre-dialysis, 8.4 contacts during the first year on CCPD and 4.3 contacts during the second year. We conclude that infants with end stage renal disease require frequent dietetic contact in combination with early dialysis and tube feeding to achieve nutritional goals and optimise growth. In addition, dietetic advice provides valuable family support.- - - - - - - - - - ranking = 1.0023954422912keywords = family, life (Clic here for more details about this article) |
8/423. Successful intradialytic parenteral nutrition after abdominal "Catastrophes" in chronically hemodialysed patients.OBJECTIVE: To assess the therapeutic contribution of intradialytic parenteral nutrition (IDPN) in four acutely ill, hypercatabolic, hemodialysed patients. All underwent major surgery, complicated by infection and malnutrition. DESIGN: A retrospective clinical study. SETTING: An in-center hemodialysis unit, at a tertiary referral hospital. patients: Patient 1: a young woman, with a good renal transplant. Developed gastric lymphoma, which required gastrectomy. After cessation of immunosuppression, "lost" her kidney and returned to hemodialysis. Received IDPN for 4 months and recovered well from severe malnourishment. Patient 2: an elderly, malnourished man, on continuous ambulatory peritoneal dialysis (CAPD). Developed biliary peritonitis and bacteremia. In a 3-month period, the patient had four operations. Maintained on IDPN for 4 months. Patient 3: a young and obese man, who suffered from life-threatening staphylococcal aureus peritonitis, resulting in widespread bowel adhesions. Underwent repeated aspirations of purulent ascites, laparoscopy, and explorative laparotomy. IDPN was administered for 4 months and stopped on the patient's request. Patient 4: a young man, who after cadaveric renal transplantation remained hospitalized for 6 months because of acute rejection and peritoneal and retroperitoneal abscesses. Had major surgery performed seven times. Received IDPN for 6 months, and is now well. RESULTS: All four patients benefited from 4 to 6 months of IDPN, as an integral part of intensive supportive and nutritional treatment. weight loss was halted, as patient appetite returned and oral nutrition became adequate. Estimated daily protein intake reached 1.2 g/kg, while caloric intake rose to nearly 30 kcal/kg/d (Table 3). Mean serum albumin levels increased from 25.5 g/L /- 0.9 g/L to 38.0 g/L /- 1.5 g/L. No adverse side effects were seen from IDPN. CONCLUSION: IDPN is a worthwhile part of treatments used in the catabolic, postoperative hemodialysed patient. It is safe and efficient when used over a 6-month period in trying to attenuate existing, or worsening malnutrition in these patients. It should be commenced at an early stage in these patients, after attempts at oral nutritional support have been deemed inadequate.- - - - - - - - - - ranking = 0.030857023813947keywords = life (Clic here for more details about this article) |
9/423. Idiopathic myelofibrosis with extramedullary hematopoiesis in the kidneys.Extramedullary hematopoiesis is a common finding in idiopathic myelofibrosis and is usually found in liver and spleen. We report on a patient with biopsy-proven myeloid metaplasia and fibrosis of the renal parenchyma as a rare cause of chronic renal failure. The renal biopsy specimen showed numerous infiltrates of hematopoietic cells expressing growth factors like M-CSF, GM-CSF, IL-1beta and PDGF while TGF-beta was not elevated. These findings suggest that hematopoietic growth factors play a key role in the pathogenesis of this condition causing proliferating fibrosis and enlargement of the kidneys.- - - - - - - - - - ranking = 0.030857023813947keywords = life (Clic here for more details about this article) |
10/423. Improvement of patient care through a collaborative approach to patient education and triage.Educating and triaging new patients into the End-Stage Renal disease care system are a challenge for all renal providers. This review describes the role of a renal patient educator, the goals of education, and ways in which an educator collaborates with multiple renal team members to assure optional treatment decision-making by patients and a smooth transition to treatments and care facilities. A case study is provided to show the collaborative role and benefits to both patients and facilities. Examples of team collaboration for delivery of education and care and requirements for successful teamwork and communication are provided. Results of this approach are improved continuity of care and coordination of patients, enhanced satisfaction of both patients and providers, and cost savings when appropriate financial and treatment decisions are in place.- - - - - - - - - - ranking = 0.02846158152277keywords = member (Clic here for more details about this article) |
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