Atypical hemolytic uremic syndrome: A monocentric adult Tunisian study and review of literature.

Atypical hemolytic uremic syndrome (aHUS) is characterized by microvascular thrombosis resulting in thrombocytopenia, hemolytic anemia, and multiorgan dysfunction. It is associated with genetic or acquired disorders of regulatory components of the complement system. For our study, we collected data from 16 patients diagnosed with aHUS between January 2010 and January 2014. The mean age was 33.6 years. The female-to-male ratio was 3. The median follow-up duration was 27 ± 3.5 months. The most common clinical presentation was hypertension. Renal involvement was noted in all cases. Ten patients had extrarenal manifestations. Semi-quantitative dysfunction of the alternative pathway of complement was found in all cases. A genetic study was not available for our patients. During the acute stage, all patients received plasma therapy, and among them, seven required dialysis and five were still on dialysis at the time of discharge. One patient underwent renal transplantation. None of our patients received eculizumab perfusion. The renal survival was inversely correlated to young age (<30 years) (P = 0.001), presence of anti-factor H antibodies (P = 0.003) and serum creatinine at diagnosis >5 mg/dL (P = 0.02). Mortality rate was significantly correlated to young age (<30 years old) (P = 0.01). Collecting multicentric data on adult patients with aHUS will enable better characterization of the spectrum of adult aHUS in our country and the evaluation of current treatments and different outcomes.

A case of exorbitism in association with Wegener's granulomatosis with renal involvement.

Wegener's granulomatosis (WG) is a necrotizing granulomatous vasculitis involving the nose, paranasal sinuses, lungs, and kidneys. Ocular involvement can occur in about 50% of cases. There are very few reports of WG with orbital inflammation and exorbitism. We report a case of a female patient who presented with exorbitism related to orbital inflammation secondary to WG, with renal involvement. A 29-year-old woman with a previous history of recurrent pan-sinusitis presented with bilateral exophthalmos and renal failure with rapidly progressive glomerulonephritis. Computed tomography showed extensive bilateral soft tissue in the retro-orbital area. Immunologic tests showed the presence of type-C anti-neutrophil cytoplasmic antibodies and renal biopsy revealed pauci immune crescentic glomerulonephritis. The patient was treated with corticosteroids and pulses of cyclophosphamide followed by azathioprine and trimethoprim-sulfamethoxazole. After a follow-up of 10 months, the renal outcome was favorable with improvement of renal function but there was persistence of exorbitism and loss of visual function. Our case suggests that WG should be considered in the differential diagnosis of persistent bilateral exophthalmos. Prompt recognition of this early manifestation is important for the institution of early treatment.

Impact of dialysis modality on posttransplantation results in kidney transplantation.

Studies looking at the type of pretransplantation renal replacement therapy on graft and patient survivals after kidney transplantation have produced conflicting results. Therefore, we studied the effect of pretransplantation dialysis modality (peritoneal dialysis [PD] or hemodialysis [HD]) on transplant outcomes. We performed a retrospective study of 78 patients (39 PD and 39 HD) who had their first renal transplantation between January 1986 and December 2004. Comparisons between groups were made using chi-square tests for qualitative parameters and nonpaired Student t tests for continuous variables. Comparisons between actuarial curves of patient and technique survivals used log-rank tests. The percentages of recipient males, cadaveric donors, transplant-induced diabetes, mean period of dialysis, mean transplantation follow-up, mean duration of first hospital stay, first infection, acute tubular necrosis, and acute rejection episodes were not significantly different among PD versus HD patients, whereas recipient and donor mean ages were significantly higher in HD and PD patients, respectively. There were no differences in graft and recipient survivals among PD versus HD patients. After kidney transplantation, there was no difference between PD and HD patients concerning percentages of infection, acute tubular necrosis, acute rejection episodes or graft and recipient survivals.

Avascular osteonecrosis after renal transplantation.

Avascular osteonecrosis (AVN) is a serious osseous complication after renal transplantation. Its prevalence clearly decreased from 20% to 4% after introduction of cyclosporine and reduction of steroid doses. The aim of our study was to evaluate the frequency of AVN among kidney transplant recipients and to determine the risk factors by comparing them with a population without AVN. Among 326 kidney transplant recipients between June 1986 and December 2004, 15 patients developed AVN with mean age of 40.86 years, including 11 men and 4 women. Fifteen kidney transplant recipients without AVN were selected to be matched for age, gender, and date of transplantation (control group). Cases of symptomatic AVN were diagnosed by hip X-ray, radioisotope bone scan, or magnetic resonance imaging. AVN was diagnosed at a mean of 3.5 years after transplantation (range, 0.5-13 years). The main localization of AVN was the femoral head in 12 cases and the femoral condyle in 3 cases. We studied the following risk factors: the type of donor (cadaver or living donor), the duration on dialysis before transplantation, the cumulative steroid dose, the acute rejection rate, and the posttransplantation weight gain. Statistical analysis showed that the cumulative steroid dose and the acute rejection rate were higher among the AVN group than the control group (P=.04 and P=.058, respectively). The prevalence of AVN in our population is 4.6%, which is probably an underestimate since these were symptomatic cases. The reduction or early withdrawal of steroids remains the only efficient preventive treatment for AVN.

[Indications, complications and cost of internal jugular catheters in hemodialysed patients: study of 533 cases]. / Indications, complications et cout des catheters jugulaires internes chez les hemodialyses: etude de 533 cas.

INTRODUCTION: Central venous catheters for hemodialysis are very useful as blood accesses when arteriovenous fistulas (AVF) are not available. The aim of this study is to analyse the clinical impact of internal jugular catheters (IJC) in hemodialysed patients and to assess their economic consequences. PATIENTS AND METHODS: It is a prospective study realised from July 1998 to March 2002 including 533 hemodialysed patients without functional AVF: 280 males and 253 females aged between 17 and 87 years (mean age: 54.1 +/- 15 years). Single lumen polyurethane Vygon were used. All catheters were placed using Seldinger procedure and the posterior route of Jernigan which is more comfortable for patient. RESULTS: Indications of IJC placement were new hemodialysed patients without AVF in 73.5% cases and no functional AVF in 26.5% cases. During the period of the study. IJC was placed in 533 patients. We failed to place the IJC at the first attempt in 42 patients but we succeed in placing it on the controlateral side. During IJC placement, we observed 50 (9.4%) cases of accidental puncture of carotid artery. The median duration use of IJC was 41 days (extreme: 1 to 413 days). IJC were removed mainly because of the use of AVF in 469 (88%) cases and the catheter infection in 41 (77%) cases. Bacteriological analysis were made only for 25 patients: staphylococci were identified in 16 cases and catheter cultures were negative in the 9 other cases. All patients were treated with antibiotics. Outcome was favourable for 39 of them and we had 2 deaths by septicemia. The IJC cost was 44,287 Tunisian Dinars (DT) coresponding to 31.633 Euro including 10125 DT (7,232 Euro) for infectious treatment. CONCLUSION: The use of IJC is frequent in our center with a high rate of infectious complications which increased the hemodialysis cost. The realisation of AVF, in patients with chronic renal failure before the beginning of hemodialysis, is the best way to limit the use of catheters.

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.

Renal replacement therapy for diabetic end-stage renal disease. Experience of a Tunisian hospital centre.

OBJECTIVE: To determine the epidemiological and clinical characteristics of 299 uremic diabetics at the initiation of renal replacement therapy (RRT) and to evaluate their morbidity and the factors influencing their mortality during the dialysis period. PATIENTS AND METHODS: All patients starting RRT for diabetic end-stage renal disease (ESRD) at Charles Nicolle Hospital of Tunis during a period of seven years (1990-1996) constituted the study population. During the follow-up period ended on December 31, 2000, all morbid events, hospitalizations and deaths were recorded. Multivariate analysis according to the Cox proportional hazards model was performed to identify the predictors of mortality. RESULTS: There were 238 type 2 (79.6%) and 61 type 1 diabetics (20.4%) representing 20.3% of all patients accepted for RRT. The mean age at the onset of RRT was 53.16 +/- 12.43 years (range: 23 to 80 years). A poor glycemic control was observed in 46.7% of type 2 diabetics and in 67.3% of type 1 diabetics (p=0.0055). A third of patients started RRT in emergency conditions and 91% required a temporary vascular access. At the time of onset of RRT, 86.7% of patients were hypertensive and 52.1% had a nephrotic syndrome. Blindness was observed in 22.9% of type 1 diabetics and in 7.5% of type 2 diabetics (p=0.0014). During the follow-up period, 217 patients died representing a rate of 22% patient-years, 26.7% of these deaths occurred during the first 3 months of RRT and 53% were attributed to infectious or cardiovascular complications. Actuarial survival rates were 68.4%, 59.6% and 45.3% at 1, 2 and 4 years, respectively. Age, co-morbidities and conditions of onset of RRT were independently correlated with survival time. CONCLUSION: Diabetes is a frequent cause of ESRD in Tunisia, the delayed referral to a nephrologist and coexistence of serious co-morbidities at the initiation of RRT resulted in a high rate of early death. Greater efforts should be made in our country in view of improving the quality of medical care of uremic diabetics before and during RRT.