Onset Of Chronic Renal Failure Research Articles

The direct cost of dialysis supported by families for patients with chronic renal failure in Ouagadougou (Burkina Faso)

BackgroundChronic renal failure can lead to dialysis and/or a kidney transplant in the final stage. The number of patients under dialysis has increased considerably in the world and particularly in sub-Saharan Africa. Dialysis is a very expensive care. This is the reason why this study on the costs of dialysis management was initiated in Burkina Faso. The objective of the study is to determine the direct medical and non-medical costs of managing chronic renal failure among dialysis patients in Ouagadougou in 2020.MethodsAn analytical cross-sectional study was conducted. Data were collected in the hemodialysis department of three public university hospitals in Ouagadougou, Burkina Faso. All dialysis patients with chronic renal failure were included in the study. Linear regression was used to investigate the determinants of the direct medical and non-medical cost of hemodialysis.ResultsA total of 290 patients participated in this study, including children, adults, and the elderly with extremes of 12 and 82 years. Almost half of the patients (47.5%) had no income. The average monthly total direct cost across all patients was 75842 CFA or US$134.41.The average direct medical cost was 51315 CFA or US$90.94 and the average direct non-medical cost was 24 527 CFA or US$43.47. Most of the patients (45.2%) funded their hemodialysis by their own source.The multivariate analysis showed that the presence of an accompanying person during treatment, residing in a rural area, ambulatory care, use of personal cars, and treatment at the dialysis center of Yalgado Teaching Hospital were associated with higher direct costs.ConclusionThe average cost of dialysis services borne by the patient and his family is very high in Burkina Faso, since it is 2.1 times higher than the country's minimum interprofessional wage (34664 CFA or US$61.4). It appears that the precariousness of the means of subsistence increases strongly with the onset of chronic renal failure requiring dialysis. Thus, to alleviate the expenses borne by dialysis patients, it would be important to extend the government subsidy scheme to the cost of drugs and to promote health insurance to ensure equitable care for these patients.

Effect of Bushen Qudu Decoction on TGF-β1/Smads signal transduction pathway in rats with chronic renal failure

Purpose: To study the impact of Bushen Qudu Decoction on renal function, renal tissue morphology and TGF-β1/Smads signal transduction pathway in adenine-induced chronic renal failure rats.Methods: 76 rats were assigned to normal group, model group, uremic clearance group, benazepil group, Bushen Qudu decoction gunshan A group and Bushen Qudu decoction Guiza group. Renal failure was induced in rats using intragastric administration of adenine for 30 days. The expressions of TGF-β1, Smad2, Smad3 and Smad6 were determined with immunohistochemistry. Real-time quantitative PCR was employed to assay the mRNA expression of TGF-β1 in each group, while protein expressions of Smad2, Smad3 and Smad6 were determined with western blot. The scores of glomerular and tubulointerstitial lesions in each group were obtained by histopathological examination.Results: Bushen Qudu decoction significantly reduced BUN and creatinine in rats with chronic renal failure; furthermore, it also lowered the protein expressions of TGF-β1 and SMAD2/3, but Smad6 protein, relative to control (p < 0.05). The TGF-β1mRNA expression was down-regulated (p < 0.05), relative to control group.Conclusion: Bushen Qudu decoction reduces renal interstitial fibrosis in rats and delays the progression of chronic renal failure by regulating TGF-β1/Smads signaling pathway. These findings provide some insight that should facilitate the development of new drugs that would delay the onset of chronic renal failure.

TO STUDY THE BIOCHEMICAL PARAMETER IN HYPERTENSION AND RENAL FAILURE

Background: In both economically developing and non-developing countries, hypertension is a major public health issue. India has been reported to have lower hypertension prevalence rates. The blood pressure of city dwellers is higher than that of rural dwellers. India is a populous and typical developing country with a vast population. Renal failure is defined as the retention of nitrogenous waste products due to impairment of renal function. Acute renal failure and chronic renal failure are the two types of renal failure. Acute renal failure is described as kidney failure that occurs in a matter of days or weeks. Acute renal failure is most common in older persons and is linked with considerable mortality and morbidity, with death rates ranging from 25 to more than 70% among hospitalized patients.
 Aim: To establish the above biochemical parameters for diagnosis and management of hypertension and renal failure.
 Material and Method: The clinical materials for present study entitled “To Study the Biochemical Parameter in Hypertension and Renal Failure” comprised 52 male hypertensive patients. 39 female hypertensive patients, 50 male chronic renal failure patients, 43 female chronic renal failure patients, 42 male chronic renal failure with hypertension, 40 female chronic renal failure with hypertension, 36 male acute renal failure, 48 female acute renal failure were admitted in Hospital.
 Results: Results revealed that highly significant decreased level of protein (Total), creatinine, and P-MDA were observed respectively. Serum sodium was found to be significant. Significant increased potassium was observed. Highly significant level of glucose, glutathione reductase, glutathione peroxidase, and catalase were observed respectively. Focus on acute renal failure patients were analyzed in both male and female. Highly significant decreased level of protein (Total), superoxide dismutase and P-MDA were observed respectively, glutathione reductase, glutathione peroxidase, and catalase were observed respectively. Significant increased serum glucose was observed, and highly significant increased level of glutathione reductase, glutathione peroxidase, and catalase were observed respectively.
 Conclusion: The study's key finding is that oxidative stress causes hypertension and can signal the onset of chronic renal failure. Oxidative stress in older people became more severe, especially if they had renal difficulties, especially in middle age.
 Keywords: P-MDA, Glutathione reductase, glutathione peroxidase, SOD, DBP

Renal cell carcinoma in patients with end-stage renal disease: a clinicopathological analysis

Objective: To investigate the clinicopathological characteristics and prognosis of renal cell carcinoma (RCC) in patients with end-stage renal disease (ESRD). Methods: The clinicopathological data of patients of renal cell carcinoma arising in end-stage renal disease were collected from the Affiliated Hospital of Qingdao University (ten cases) and 971 Hospital of PLA Navy (five cases) from January 2009 to August 2018. Results: Among 15 patients, 14 were male and 1 was female, and the age ranged from 38 to 78 years (mean 51 years, median 49 years). All patients had history of chronic renal failure (7-192 months), including 9 patients treated with hemodialysis for 6 to 132 months. In 12 cases the tumor border was distinct and the tumor size ranged from 1.8 to 11.0 cm. Two cases were multifocal and one case showed extensive renal hemorrhage with an inconspicuous tumor mass. Microscopically, 9 cases were clear cell reanl cell carcinoma including one with sarcomatoid differentiation, 4 were acquired cystic kidney disease-associated(ACKD-RCC) and two were papillary renal cell carcinoma. All patients had a follow-up of 3 to 120 months. Four patients died during a follow-up of 6 to 60 months (mean 30 months) as a result of extensive distant metastases (two cases) and renal failure (two cases), while other eleven patients were alive without tumor recurrence or metastasis (median 40.8 months of follow-up ranging from 3 to 120 months). Conclusions: ESRD-RCC is more often seen in younger male patients. The time intervals from the onset of chronic renal failure to the diagnosis of renal cell carcinoma differ and tumors are frequently incidental findings. The histological types can be sporadic renal cell carcinoma or unique ACKD-RCC. Tumors are often hemorrhagic and necrotic. Routine physical examination and early detection could benefit ESRD-RCC patients. ESRD-RCC may have a favorable prognosis despite of a large tumor size or the presence of sarcomatoid differentiation.

Morphological evaluation of sympathetic renal innervation in patients with autosomal dominant polycystic kidney disease.

Several evidences support the hypothesis that patients affected by autosomal dominant polycystic kidney disease (ASPKD) show a sympathetic renal hyperactivity. Nevertheless, no morphological evidences are available yet. Therefore, the aim of the study was to demonstrate that an increase in sympathetic renal artery innervation was present in the ADPKD patients by using histological methods. In addition, here we correlated the sympathetic renal artery innervation with the evolutionary state of ADPKD (increase in volume of kidney, onset of chronic renal failure and hypertension). To this end, peri-adventitial innervation of renal arteries was studied using morphological methods from 49 patients in total: 29 underwent surgical nephrectomies for ADPKD and 20 non-dialysis patients (CTRL group) undergoing nephrectomy for other diseases. Nerve density (number of nerves per mm2) was evaluated in the peri-adventitial tissue in a concentric ring that was located within 2mm from the beginning of the adventitia by using immunohistochemistry. The total nerve density was significantly increased in the ADPKD group (1.26 ± 0.82 × mm2) as compared to controls (0.78 ± 0.40 × mm2) (p = 0.02). Hypertensive patients with ADPKD showed a greater nerve density than control hypertensives. However, the increase in renal sympathetic innervation in the ADPKD patients was found to be independent of hypertension, resistance to antihypertensive therapy, age, sex and kidney volume, as demonstrated by the uni and multivariate analysis. In conclusion, our study better clarifies the effect of sympathetic hyperactivity in the progression of polycystic disease.

Aortic Valve Replacement Performed Twice Through Ministernotomy 15 Years After Lung Transplantation

After transplantation, steroids and calcineurin inhibitors together with end-stage renal failure may lead to associated cardiovascular diseases, particularly in long-term survivors. We present a case of aortic valve replacement 15 years after lung transplantation, followed by reoperative valve replacement for late infective endocarditis. Lung compliance and gas exchange were excellent during recovery. Despite adequate prophylaxis, immunosuppression and hemodialysis likely contributed to repeated episodes of sepsis, which caused detachment of the first aortic prosthesis. Despite the high mortality of prosthetic valve endocarditis, the postoperative course was uneventful and the patient is doing well at 24-month follow-up.

A milder variant of Pierson syndrome

Congenital nephrotic syndrome (CNS) comprises a heterogeneous group of conditions having in common the disruption of normal glomerular permselectivity, and it carries a poor prognosis, with most patients progressing to end-stage renal disease. Recently, mutations in the LAMB2 gene encoding laminin beta2 were described as the cause of Pierson syndrome, which is characterized by CNS and a complex ocular maldevelopment with microcoria as the most prominent clinical features. Most affected children exhibit early onset of chronic renal failure, neurodevelopmental deficits, and blindness. We report on a patient with CNS, high-grade myopia, and minor structural eye anomalies, including remnants of pupillary membranes, but no microcoria. The patient had not developed renal failure by the age of 16 months, and he showed no neurodevelopmental deficits. He was identified to be homozygous for a novel LAMB2 missense mutation. This observation, together with two recent reports on milder variants of Pierson syndrome, corroborates the concept that the clinical expression of Pierson syndrome is more variable than initially described, and that milder phenotypes may be related to hypomorphic LAMB2 alleles.

Identification of Nonneurogenic Neurogenic Bladder in Infants

To report the third series of the severe neonatal form of Allen-Hinman syndrome. Hinman and Baumann (1973) and Allen (1977) reported the first two series (14 and 21 patients, respectively) of children with severe functional vesicourethral dysfunction producing changes indistinguishable from an obstructive factor. From January 1995 to April 2006, 7 boys with Allen-Hinman syndrome were observed. They had been symptomatic since early infancy with a poor stream and chronic urinary retention. They had bilateral hydroureteronephrosis on renal ultrasonography and intravenous urography (grade 4 to 5 vesicourethral reflux) during the first year of age. They were initially thought by urologists to have a posterior urethral valve. When cystoscopy excluded anatomic obstruction, the urologists were unable to provide an alternative diagnosis. The patients' age on referral ranged from 5 months to 15 years (mean 6 years). They had been followed up for a period of 2 weeks to 6 years. Four patients had been treated with chronic catheterization and were referred after the age of 5 years with end-stage renal failure. One of them underwent cutaneous vesicotomy after the onset of chronic renal failure and the need for dialysis. I recommended early vesicotomy for the remaining 3 patients. One boy had undergone vesicotomy during the first 6 months of age and had been followed up for 6 years. At 6 years of age, the dilation had disappeared and his renal function had been preserved. The nonneurogenic neurogenic bladder represents a rare, severe form of dysfunctional voiding that can present even in the neonatal period and can lead to renal failure.

Effect of peptide Vilon on the content of transforming growth factor-β and permeability of microvessels during experimental chronic renal failure

We studied the effect of Vilon in rats 2, 4, and 6 months after the onset of chronic renal failure. Subcutaneous injection of Vilon significantly decreased serum concentration of transforming growth factor-beta(1) and permeability of mesenteric microvessels in rats 2 months after the onset of chronic renal failure. Our results indicate that the preparation produces a potent homeostatic effect in the early period of chronic renal failure.

Serum TGF-Beta 1 and TNF-Alpha Levels and Cardiac Fibrosis in Experimental Chronic Renal Failure

Objectives To characterize dynamics of changes of serum levels of TGF-β1 and TNF-α in rats with cardiac fibrosis (CF) occurring during chronic renal failure (CRF), and to reveal the character pf correlations of these factors with amounts of cardiac collagen. Design and Methods. CRF was induced by unilateral nephrectomy and by electrocoagulation of 25% of the cortex of remnant kidney. Post-operative checkpoints were 2, 4, and 6 months. Serum TGF-β1 and TNF-α levels were measured by ELISA. Results. CF became pronounced only at 6 months of CRF, while serum TGF-β1 concentrations reached maximum at 4 months, i.e., at the checkpoint preceding the development of CF. Multiple regression showed cardiac collagen to correlate with both serum TGF-β1 levels and time from the onset of CRF. Sensitivity and specificity of TGF-β1 as serum marker of CF were 86% and 75%.

Imaging medullary cystic kidney disease with magnetic resonance

Medullary cystic kidney disease is characterized by multiple renal cysts at the corticomedullary boundary area, by autosomal dominant inheritance, and by onset of chronic renal failure in the third decade of life. Its clinical manifestations are often insignificant and nonspecific. Furthermore, its diagnosis may be difficult in sporadic forms where genetic linkage analysis cannot be performed. The authors report the case of a patient presenting with a sporadic form of medullary cystic kidney disease whose diagnosis was confirmed using computerized tomography with 3-dimensional reconstruction at the nephrography-excretion time and magnetic resonance imaging (MRI) with magnetic resonance angiography and urography after the injection of gadolinium, a nonnephrotoxic compound. Both imaging techniques showed normal-sized, normal-shaped kidneys containing multiple cysts from 1 to 30 mm in diameter in the medulla and at the corticomedullary junction. A characteristic medullary nephrogram appeared after injection of iodinated contrast medium or gadolinium corresponding to contrast-filled dilated collecting ducts. This report shows that MRI with gadolinium injection can substitute for computerized tomography in azotemic patients. MRI seems particularly promising for the diagnosis of cystic diseases of the kidney and must also be considered when investigating a patient with chronic renal failure of unknown origin.

Determination of renal porphyrin handling in rats suffering from different kinds of chronic renal failure (CRF): Uranyl nitrate (UN) induced fibrosis or 5/6-nephrectomy (5/6NX)

The renal handling of porphyrins is reported to be a sensitive marker for chronic renal failure (CRF) for two reasons: heme is synthesised in proximal tubules and porphyrins are reabsorbed in the renal proximal tubule by apical peptide transporter PEPT 2. Two different models of CRF in female Wistar rats have been used for investigation of renal porphyrin handling: (1) single administration of uranyl nitrate (UN; 0.5 mg/100 g b.wt.) and (2) 5/6 nephrectomy (5/6NX). Renal clearance experiments were performed at weeks 2 and 10 after the onset of CRF. The concentrations of porphyrin intermediates (uroporphyrin I and III, coproporphyrin I and II, heptaporphyrin, and pentaporphyrin) were measured by HPLC with fluorescence detection. Both after UN and 5/6NX a significant reduction of body weight occurred. The kidney weight was enhanced 2 weeks after UN compared to controls (+31%). After 5/6NX, the weight of the remnant kidney was 44% (2nd week) and 140% (10th week) higher compared to one control kidney. Urine volumes and GFR were significantly reduced at week 2 and 10 after 5/6NX, but at week 10 after UN values were comparable to controls. Two weeks after UN and 5/6NX the concentrations of heptaporphyrin was moderately decreased in renal tissue whereas after 10 weeks the concentrations of most porphyrins were increased in the kidney. The plasma levels of free porphyrins were only slightly enhanced (week 2). The renal excretion of porphyrins was initially slightly reduced in both models, whereas it increases 10 weeks after UN, but it remained reduced 10 weeks after 5/6NX. UN induces tubulointerstitial fibrosis including atrophic glomeruli, whereas 5/6NX was characterized by distinct proteinuria, dilated tubules containing hyaline casts. A modulation of porphyrin metabolism in the kidney seems first of all to be responsible for UN effect on renal porphyrin handling. Summing up the 5/6NX results, both reduction in intact renal tissue mass and a modification of enzymes involved in heme biosynthesis by uraemic toxins are responsible for accumulation of porphyrins in renal tissue. After 5/6NX reduced excretion of porphyrins into urine and enhanced porphyrin concentrations in the kidney indicate more a damage of renal porphyrin biosynthesis than changes in their reabsorption.

Chronic renal failure in Iranian children.

We investigated chronic renal failure (CRF) in 166 Iranian children (95 boys and 71 girls) from July 1991 to June 1999. The mean age at onset of CRF was 7.9+/-4.5 years. The most common cause of CRF was congenital urological malformations (78 cases). The second most common cause of CRF was hereditary nephropathy (21%). Glomerular diseases accounted for only 10% of children who later went on to develop renal failure. High rates of cystinosis and primary hyperoxaluria were seen, and these elevated rates could be due to a high prevalence of parental consanguinity. Eighty-six patients required renal replacement therapy, of whom the majority underwent hemodialysis. The prevalence of primary reflux as a cause of CRF was high compared with reports from western countries. Earlier diagnosis and management of urinary tract infections in this group could reduce the prevalence of reflux as a cause of CRF in this population.

Dietary treatment of diabetic nephropathy with chronic renal failure.

Thirty-two patients with diabetes mellitus (22 IDDM and 10 NIDDM, 21 males and 11 females, age 44+/-11.8 years) were followed for 5.2+/-3.8 years after the onset of chronic renal failure, with the aim of evaluating the effect of low protein diets on the rate of decline of the residual renal function. During the 1.8+/-1.6 year follow-up period on free or uncontrolled low protein diet the mean rate of decline of creatinine clearance was 0.9+/-0.6 ml/min/month, significantly greater than that observed during 3.7+/-3.1 years on low or very low protein diets. The reduction of protein intake was followed by a significant decrease in daily urinary protein loss. A better glycaemic control was obtained on the low protein diet, and the daily insulin requirement decreased. The anthropometry, as well as the serum concentrations of rapid turnover proteins, did not change, in spite of the low or very low protein dietary supply for a long duration. The values of mean arterial pressure were quite similar during the follow-up period on free or uncontrolled low protein diet and during the study period on the low protein diet. A good compliance with reduced dietary intake (as demonstrated by the measurement of the daily urea excretion) was obtained in a large number of patients. In conclusion, our study confirms the protective effect on the residual renal function of low protein diets in IDDM and NIDDM patients with chronic renal failure due to diabetic nephropathy, in the absence of any sign of protein malnutrition.

Early age-dependent growth impairment in chronic renal failure

We report early linear growth in 73 children (51 boys, 22 girls) with early onset of chronic renal failure (CRF). The inclusion criteria was onset of CRF before 6 months of age, two or more height measurements during the 1st year of life, follow-up for at least 3 years and continuously impaired renal function with a glomerular filtration rate below 50 ml/min per 1.73 m2 at 1 year or later. Only height measurements taken during conservative treatment or dialysis were included. The data were analysed in terms of the infancy-childhood-puberty growth model. There was an age-dependent growth failure in early life leading to an attained height of −3 standard deviation score (SDS) at 3 years of age. Approximately one-third of the reduction in height occurred during fetal life and one-third during the first postnatal months. Between 0.75 and 1.5 years of age height also decreased by 1 SD as a consequence of a delayed onset of the second, the “childhood’, phase of growth in 36% of the patients and by an ‘offset childhood’ growth pattern — i.e. a return to the infancy phase pattern after onset of the childhood phase — in 60% of the patients. Growth between 0.25–0.75 and 1.5–5 years of age was generally percentile parallel and thus less likely to be affected in CRF with early disease onset. The glomerular filtration rate was not related to the height gain in early life. We speculate that the growth failure during fetal life and the first postnatal months reflects metabolic and/or nutritional influences and the impaired growth at 0.75–1.5 years of age is related to a partial insensitivity to growth hormone.

Etiology of chronic renal failure in Turkish children

The etiology of chronic renal failure (CRF) was studied in 459 Turkish children (205 girls, 254 boys) for the period January 1979-December 1993. Their mean age at onset of CRF was 9.5 +/- 4.2 years (range 1-16 years); CRF was defined as a glomerular filtration rate (GFR) below 50 ml/min per 1.73 m2 for at least 6 months. When a GFR determination was not available, the serum creatinine concentration was used: greater than 1 mg/dl for children aged 1-3 years, greater than 1.5 mg/dl for those 3-10 years and greater than 2 mg/dl for those 10-16 years. Primary renal disorders were as follows: reflux nephropathy 32.4% glomerular diseases 22.2%, hereditary renal disorders 11.4%, amyloidosis 10.6%, urinary stones 8% and other renal disorders 15.4%. Twenty-three cases of reflux nephropathy (15.4%) were associated with neural tube defects (NTD) and 20 (13.4%) were caused by infravesical obstruction. CRF caused vesicoureteral reflux associated with NTD and amyloidosis are more frequent in our series compared with west European and Nordic countries.

PRENATAL DETECTION OF CONGENITAL RENAL DISEASE

Prenatal ultrasonography can identify not only the structural aspects of many abnormalities of the urinary tract but also associated anomalies, some of which may be missed even after birth. Prenatal imaging alerts physicians to some infants with urinary tract lesions that ordinarily show no physical findings at birth, leading to earlier diagnosis. An early diagnosis may prevent the first urinary tract infection in an infant at risk. In addition, there are fewer hospitalizations for infection or electrolyte abnormalities in infants with urinary tract disease. Elective surgery may be planned instead of performed on an urgent basis and thus can be performed with lower morbidity. Although the early diagnosis and treatment of pediatric renal disease have certainly been facilitated by prenatal ultrasonography, longer follow-up is required to determine whether prenatal detection prevents the onset of chronic renal failure as children with congenital renal disease approach adolescence and adult life.

Determinants of intact parathyroid hormone and free 1,25-dihydroxyvitamin D levels in mild and moderate renal failure.

Parameters of calcium and phosphate metabolism were measured in 27 patients with mild renal failure [glomerular filtration rate (GFR) 40-90 ml/min], 12 patients with moderate renal failure (GFR 20-39 ml/min) and in 12 healthy subjects. GFR was determined by technetium-99m diethylenetriamine penta-acetic acid clearance. Intact parathyroid hormone (PTH) was measured by a sensitive immunochemiluminometric assay and somatomedin-C was determined by radioimmunoassay. Both 1,25-dihydroxyvitamin D [1,25(OH)2D] and vitamin-D-binding protein were measured allowing calculation of the free 1,25(OH)2D index. By linear regression and multivariate analysis, PTH was negatively and independently correlated with GFR, plasma bicarbonate and 25-hydroxyvitamin D [25(OH)D] while free 1,25(OH)2D was positively correlated with GFR. Increased PTH secretion and reductions in 1,25(OH)2D were present in mild renal failure patients before any changes in plasma calcium, phosphate and bicarbonate were noted. Plasma alkaline phosphatase was significantly higher in mild chronic renal failure patients compared to normal subjects, possibly indicating early effects of the secondary hyperparathyroidism on the skeleton. Somatomedin-C did not correlate with the free 1,25(OH)2D index or a measure of 1,25(OH)2D production. It is concluded that the secondary hyperparathyroidism which occurs very early in the onset of chronic renal failure may be due to a reduction in the circulating concentration of 1,25(OH)2D consequent upon the renal failure. Low plasma bicarbonate and 25(OH)D also appear to be determinants of a raised PTH concentration. The compensatory increase in PTH presumably maintains extracellular calcium and phosphate levels constant but with possible deleterious effects on the skeleton.

Chronic Renal Failure in Sickle Cell Disease: Risk Factors, Clinical Course, and Mortality

To determine the incidence, clinical course, and risk factors associated with the onset of chronic renal failure in sickle cell anemia and sickle C disease. A prospective, 25-year longitudinal demographic and clinical cohort study. A matched case-control study was conducted to determine risk factors. A total of 725 patients with sickle cell anemia and 209 patients with sickle C disease who received medical care from the hematology service in a large municipal hospital. Most were observed from birth or early childhood. Thirty-six patients developed sickle renal failure: 4.2% of patients with sickle cell anemia and 2.4% of patients with sickle C disease. The median age of disease onset for these patients was 23.1 and 49.9 years, respectively. Survival time for patients with sickle cell anemia after the diagnosis of sickle renal failure, despite dialysis, was 4 years, and the median age at the time of death was 27 years. Relative risk for mortality was 1.42 (95% Cl, 1.12 to 1.81; P = 0.02) compared with patients who did not develop renal insufficiency. Histopathologic studies showed characteristic lesions of glomerular "drop out" and glomerulosclerosis. Case-control analysis showed that ineffective erythropoiesis with increasingly severe anemia, hypertension, proteinuria, the nephrotic syndrome, and microscopic hematuria were significant pre-azotemic predictors of chronic renal failure. The risk for sickle renal failure was increased in patients who had inherited the Central African Republic beta s-gene cluster haplotype. The pre-azotemic manifestations of hypertension, proteinuria, and increasingly severe anemia predict end-stage renal failure in patients with sickle cell disease. The rate of progression of renal insufficiency is genetically determined. Treatment of the uremic phase has been dismal, underscoring the need for the development of useful pre-azotemic therapeutic modalities.

Chronic Renal Failure for Bilateral Spontaneous Kidney Rupture in a Case of Tuberous Sclerosis

A case of spontaneous, but not contemporary rupture of both kidneys in a 42-year-old man with tuberous sclerosis is described. Despite the high incidence of renal involvement, chronic renal failure due to replacement and compression of renal tissue by hamartomas is rare. We reviewed 25 cases in the literature and found only 1 other case which necessitated maintenance hemodialytic treatment for kidney rupture. It is suggested that early diagnosis and conservative partial nephrectomy could prevent or delay the onset of chronic renal failure, malignant transformation and dramatic rupture of hamartomas.