[AA amyloidosis complicating sarcoidosis: two cases and literature review]. / Amylose AA compliquant une sarcoïdose : deux observations et revue de la littérature.

INTRODUCTION: Sarcoidosis is a relatively common granulomatosis. Its outcome is usually favourable. AA amyloidosis is a rare complication of sarcoidosis. We report two new cases and review the literature. CASE REPORTS: A 40-year-old woman followed for a splenomegaly was admitted for assessment of hepatic cholestasis. The diagnosis of sarcoidosis involving lungs, liver, spleen and lymph nodes was obtained on clinical, biological and histological evidence. Simultaneously, AA amyloidosis deposits was demonstrated on lymph nodes and spleen. The second patient was a 42-year-old woman who was evaluated for mediastinal lymph nodes and interstitial pneumonia. Lung biopsy showed epithelioid and giant cell granuloma without caseous necrosis, associated to AA amyloidosis deposits. In these two patients, corticosteroids (initially at 1 mg/kg daily) led to a sustained improvement with a 1 and 5 years follow-up, respectively. CONCLUSION: AA amyloidosis is an uncommon complication of sarcoidosis. Corticosteroids associated with colchicine is proposed to treat simultaneously the two disorders.

Nephrolithiasis-induced end stage renal disease.

INTRODUCTION: Nephrolithiasis still remains a too frequent and underappreciated cause of end stage renal disease (ESRD). METHODS AND PATIENTS: Of the entire cohort of 7128 consecutive patients who started maintenance dialysis in our nephrology department between January 1992 and December 2006, a total of 45 patients (26 women, 19 men) had renal stone disease as the cause of ESRD. The type of nephrolithiasis was determined in 45 cases and etiology in 42. The treatment and evolution of stone disease and patient's survival were studied. RESULTS: The overall proportion of nephrolithiasis related ESRD was 0.63%. The mean age was 48.4 years. Infection stones (struvite) accounted for 40%, calcium stones, 26.67% (primary hyperparathyroidism:15.56%; familial hypercalciuria: 4.44%, unknown etiology: 6.66%), primary hyperoxaluria type 1, 17.78% and uric acid lithiasis in 15.56% of cases. The mean delay of the evolution of the stone renal disease to chronic renal failure was 85.8 months. The feminine gender, obesity and elevated alkaline phosphatases >128 IU/L were significantly correlated with fast evolution of ESRD. The median evolution to ESRD was 12 months. The normal body mass index (BMI), medical treatment of stone and primary hyperoxaluria type 1 were correlated with fast evolution to ESRD. All patients were treated by hemodialysis during a mean evolution of 60 months. Sixteen patients died. The patient's survival rate at 1, 3 and 5 years was 97.6, 92.8 and 69% respectively. Hypocalcemia, cardiopathy and normal calcium-phosphate product were significantly correlated with lower survival rate. CONCLUSION: Severe forms of nephrolithiasis remain an underestimated cause of ESRD. These findings highlight the crucial importance of accurate stone analysis and metabolic evaluation to provide early diagnosis and efficient treatment for conditions leading to ESRD.

Impact of initial blood pressure on the mortality of diabetics undergoing renal replacement therapy.

The aim of this study was to determine the blood pressure (BP) profiles and their impact on mortality among a cohort of uremic diabetics treated by hemodialysis. The studied population includes all type II diabetics starting hemodialysis for end-stage renal disease between 1990 and 1996. There were 221 patients (144 men, 77 women) aged from 37 to 78 years, were all followed until death or December 2003 without any censored data. Survival analysis to identify predictors of death was performed using the actuarial method, Cox proportional model, including systolic, diastolic, mean, and pulse blood pressures (SBP, DBP, MBP, PP). One hundred seventy-eight patients (80.5%) were hypertensive at the start of dialysis. Hypertension preceded the diagnosis of diabetes in eight cases (4.5%); 154 patients (86.5%) received antihypertensive drugs and only 23 (14.9%) had well-controlled hypertension. Our population was subdivided into four groups according to their BP levels at the time of beginning of dialysis; G1 (19.5%): normal BP (SBP [90 to 140] and DBP [60 to 90]); G2 (30.3%): Hypertension stage 1 (SBP [140 to 160] and/or DBP [90 to 100]); G3 (32.1%): hypertension stage 2 (SBP [160 to 180] and/or DBP [100 to 110]); G4 (18.1%) hypertension stage 3 (SBP [180 to 220] and/or DBP [110 to 120]). Mean age and comorbidities were similar among the four groups. During a cumulative follow-up period of 872 patient-years, 191 patients died, representing a rate of 21.9 per 100 patient-years; 20.42% of these deaths occurred during the first 3 months of dialysis. Normotensive patients showed lower survival rates without any significant difference in comparison with those of other hypertensive groups. None of the initial BP parameters (SBP, DBP, PP, MBP, hypertension stages) seemed to influence early or global mortalities, which were rather related to the urgent onset of renal replacement therapy, to age, to serum albumin, and to the score of associated morbidities. We conclude that mortality of our hemodialyzed diabetics was not influenced by the blood pressure parameters recorded at the onset of dialysis.