Hyperreactio luteinalis associated with chronic renal failure.
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Hyperreactio luteinalis is a rare benign condition characterized by bilateral ovarian enlargement associated with pregnancies where high concentrations of maternal serum human chorionic gonadotrophins are present. This condition may mimic the ovarian hyperstimulation syndrome. We report a case of a 34 year old woman with a history of chronic renal failure on haemodialysis who presented at 10 weeks' gestational age with hyperreactio luteinalis which was treated conservatively. Because of chronic renal failure, the presentation and course of the disease was different from that which has been previously reported. (+info)
Diagnosis and treatment of chronic renal failure in children.
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This activity is designed for primary care and specialist physicians. GOAL: To provide an overview of the unique features and treatment of chronic renal failure in children. OBJECTIVES: 1. Describe the diagnosis of chronic renal failure in children. 2. Discuss the medical treatment of chronic renal failure in children. 3. Understand the treatment of end-stage renal disease in children. 4. Discuss the financial impact of caring for a child with chronic renal failure. (+info)
The degrees of plasma cell clonality and marrow infiltration adversely influence the prognosis of AL amyloidosis patients.
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BACKGROUND AND OBJECTIVE: Primary amyloidosis is a lethal form of plasma cell (PC) dyscrasia characterized by deposits of monoclonal immunoglobulin light chains that cause organ dysfunction. In contrast to multiple myeloma, the amyloid clone is typically indolent and of small size, and marrow PC clonality is not always apparent. This is generally investigated by analyzing the light chain isotype ratio in bone marrow PC. We investigated whether the degree of PC infiltration (PC%) and clonality (PC isotype ratio) affected survival in 56 consecutive patients with primary amyloidosis. DESIGN AND METHODS: PC% was determined by morphologic examination. Immunofluorescence microscopy was used to determine the PC light chain isotype ratio. Statistical analysis was carried out using Cox regression models. RESULTS: The degrees of PC clonality and infiltration were inversely correlated with survival (PC isotype ratio, p = 0.001; PC%, p = 0.008). The two variables were weakly correlated (p = 0.02; r = 0.3). Bone marrow PC isotype ratio demonstrated a powerful independent prognostic value at multivariate analysis when analyzed together with congestive heart failure (the major known negative prognostic factor) and PC%. k/l ratio cut-off values of 0.2 (l patients, p = 0.022) and 16 (k patients, p = 0.03) discriminated two groups with a similar number of patients and significantly different survivals. INTERPRETATION AND CONCLUSIONS: PC clonality and marrow infiltration are important parameters that influence prognosis, presumably because they reflect the amount of pathogenic light chain synthesis. (+info)
Long-term follow-up of a family with autosomal dominant polycystic kidney disease type 3.
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BACKGROUND: Autosomal dominant polycystic kidney disease is one of the most common hereditary diseases in man with an estimated prevalence of 1:1000. At least three genetic loci are responsible for the development of the disease. PKD1 localized to 16p13 is the most common gene, contributing to almost 85% of all cases, is associated with the most severe form. PKD2, localized to 4q21-23, responsible for almost all the remaining cases, is associated with a milder form. Up to now, only five families have been reported unlinked to the two most common genetic defects, and therefore little is known about the clinical findings of the non-PKD1/PKD2 families. METHODS: In this report we describe the clinical findings of 18 patients of a non-PKD1/PKD2 family, with a mean follow-up of 52 months (range 3-133 months) in our outpatient clinic. RESULTS: Of the 10 patients older than 40 years, nine were hypertensive; in this age group eight patients exhibited renal failure (two of them were on dialysis) and six had hepatic cysts. In eight patients younger than 40 years, the only clinical finding was hypertension in two. Considerable variation in the rate of progression to renal failure among members of this family was found; on the other hand, some patients did not exhibit any signs of progression. CONCLUSION: This family exhibits a more aggressive phenotype, in contrast with the majority of the described non-PKD1/non-PKD2 families. (+info)
Dialock: a new vascular access device for extracorporeal renal replacement therapy. Preliminary clinical results.
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BACKGROUND: Vascular access, a vital tool for end-stage renal disease patients, remains a weak component of extracorporeal renal replacement therapy (RRT) and the first cause of morbidity. Permanent catheters proposed as an alternative to permanent AV fistulae are associated with a significant risk of infection. A subcutaneously implantable chamber connected to permanent catheter appears highly desirable to reduce such hazards. METHODS: Dialock, a metallic port-like valve device connected to permanent silicone twin catheters has been developed (Biolink Corp, Middleboro, MA, USA). After being implanted subcutaneously below the clavicle, Dialock provides a linear flow passage to two Silastic catheters placed in the right atrium via the right internal jugular vein. The valve is accessed percutaneously each dialysis session with needle cannulae that functionally convert the device into twin catheters. Interdialytic patency of the catheters is ensured by antithrombotic lock (heparin or low-molecular-weight heparin). RESULTS: Dialock was implanted in 10 ESRD patients (64+/-12 years) under general anaesthesia, with almost immediate use for HD. RRT consisted of three HD sessions per week lasting 4 h; 699 HD sessions were performed. Average duration of use was 5.7 patient-months (1.3-9.6 months). Patient satisfaction was evident in all cases. Three episodes of bacteraemia occurring in the early phase of the study were cured by appropriate antibiotics. No device was removed because of infection. Skin condition at the puncture sites has remained satisfactory in all patients. Nurse training for cannulating was brief (2-3 x). Effective blood flow was 307+/-3.3 ml/min, with a venous pressure of 195+/-39 mmHg and a recirculation rate of 6.7+/-0.8%. Effective Kt/V dp delivered was 1.36+/-0.03 with a nPCR of 1.20+/-0.005 g/kg/day. Haematoma and a small amount of bleeding of the skin puncture sites observed in the initial period of the study were effectively prevented by reducing heparin lock volume. CONCLUSIONS: The Dialock device offers a new and interesting vascular access alternative for haemodialysis bridging the 'gap' between permanent catheters and arteriovenous fistulae. Dialock's place in the vascular access strategy for haemodialysis patients deserves further long-term clinical studies. (+info)
Leptin elimination in hyperleptinaemic peritoneal dialysis patients.
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BACKGROUND: Elevated plasma concentrations of leptin, a hormone thought to regulate body composition by influencing food intake/metabolic rate, are prevalent in renal failure patients. The mechanism for these increases is not known, but evidence suggests that simple accumulation due to decreased elimination is insufficient explanation. METHODS: We studied the incidence of hyperleptinaemia in 28 end-stage renal disease patients treated with continuous ambulatory peritoneal dialysis (CAPD), compared with body-mass-index-and sex-matched controls. Results were separated by gender because women have higher leptin concentrations than men. Excretion of leptin and other substances in dialysis fluid was also studied. RESULTS: Hyperleptinaemia was prevalent in women CAPD subjects, but not in men. Plasma leptin concentrations correlated strongly with the daily excretion of leptin in dialysis fluid. Clearance of leptin in dialysis fluid was greater in men than women CAPD subjects. Single regression analysis found that fasting insulin, glucose content of dialysis fluid, plasma albumin, C-reactive protein, erythropoietin dose, urinary creatinine clearance and plasma beta2-microglobulin were not determinants of plasma leptin concentrations. Stepwise forward multiple regression, examining the dependence of plasma leptin on body mass index, renal creatinine clearance, plasma albumin, daily dialysis fluid glucose load, daily leptin in dialysis fluid, erythropoietin dose and plasma C-reactive protein found only erythropoietin dose as a consistent negative predictor of plasma leptin concentrations. CONCLUSIONS: The results suggest that hyperleptinaemia of CAPD was due to predisposing loss of renal elimination capacity combined with increased production due to obesity (more prevalent in women subjects of this study) and potentially female gender. (+info)
Carotid endarterectomy in patients with significant renal dysfunction.
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PURPOSE: Recent reports suggest that carotid endarterectomy (CEA) should not be performed in patients with end-stage renal disease (ESRD) because of an unacceptable rate of perioperative stroke and other morbidity. Because these conclusions were based on a small number of patients, we reviewed the perioperative and long-term outcome of patients with ESRD and chronic renal insufficiency (CRI) who underwent CEA at our institution. METHODS: The 1081 patients who had a CEA between 1990 and 1997 were cross-referenced with those patients in whom renal insufficiency had been diagnosed. These charts were reviewed for patient demographics and perioperative and long-term outcome. Patients undergoing CEA during a 1-year period (1993) served as controls. RESULTS: Fifty-one CEAs were performed in 44 patients with CRI (32 in 27 patients) and ESRD (19 in 17 patients). In the CRI+ESRD group, 66.7% were symptomatic, and 70.7% of the control group were symptomatic. Six operations (11.8%) in the CRI+ESRD group were redo endarterectomies. There were no perioperative strokes in the CRI+ESRD group, but one patient died 29 days postoperatively because of a myocardial infarction, for a combined stroke-mortality rate of 2.0%. The control group had a 2.6% combined stroke-mortality rate. Long-term survival analysis revealed a 4-year survival rate of 12% for patients with ESRD and 54% for patients with CRI, compared with 72% for controls (P <.05). CONCLUSION: CEA can be performed safely in patients with ESRD or CRI, with perioperative stroke and death rates equivalent to that of patients without renal dysfunction. However, the benefit of long-term stroke prevention in the asymptomatic patient with ESRD is in question because of the high 4-year mortality rate of this patient population. (+info)
Insulin-like growth factor I improves renal function in patients with end-stage chronic renal failure.
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There is no pharmacological treatment to increase the glomerular filtration rate in end-stage renal disease (ESRD). The administration of 100 microgram/kg of insulin-like growth factor (IGF) I twice a day to patients with ESRD increases inulin clearance. However, its effect is short-lived and IGF-I has major side effects when given this way. To assess whether the use of a lower intermittent dose of IGF-I would effect sustained improved function with tolerable side effects we performed 1) a prospective open-labeled 24-day trial in which we enrolled five patients and 2) a 31-day randomized, double-blinded, placebo-controlled trial in which we enrolled 10 patients. Patients with ESRD [creatinine clearance of <15 ml. min-1. (1.73 m2)-1] and scheduled to initiate renal replacement therapy received subcutaneous IGF-I, 50 microgram. kg-1. day-1, or vehicle. Treatment with IGF I resulted in significantly increased glomerular filtration rates (inulin clearances) during the 3rd and 4th wk of therapy in both prospective and double-blinded studies. Vehicle had no effect. No patient required discontinuation of drug secondary to side effects. We conclude that IGF-I effects sustained improvement of renal function (clearances comparable to those generally achieved by dialysis) in patients with ESRD and is well tolerated. (+info)