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.

[Kaposi sarcoma in a patient with ANCA vasculitis requiring hemodialysis]. / Sarcome de kaposi compliquant une vascularite à ANCA en hémodialyse chronique.

INTRODUCTION: Iatrogenic Kaposi's sarcoma is widely reported after transplantation. Less commonly, it occurs in patients receiving immunosuppressive therapy for ANCA associated vasculitis. We report here the rare association of Kaposi's sarcoma, prurigo nodularis and ANCA associated vasculitis in a hemodialysis patient. CASE REPORT: We describe a 58-year-old woman who presented granulomatosis with polyangeiitis with alveolar hemorrhage and renal failure requiring hemodialysis. She developed cutaneous Kaposi's sarcoma seven weeks after the beginning of immunosuppressive therapy. Biological tests showed negative HHV8 virus infection. Lesions of Kaposi's sarcoma responded to a discontinuation of immunosuppressive drugs and a decreasing dosage of corticosteroids. CONCLUSION: Our case showed that the immunosuppressed state related to multiple factors such as underlying disease, immunosuppressive therapy and hemodialysis may all have contributed to the development of this neoplastic disorder in our patient.

Risk factors of arterial hypertension after renal transplantation.

Arterial hypertension often present after kidney transplantation is of multifactorial origin. The aim of this study was to determine the role of donor and recipient factors in the development of hypertension after renal transplantation. We retrospectively analyzed the data of 280 patients transplanted between 1985 and 2005, who still had functioning grafts at 1 year after transplantation. We recorded donor and recipient parameters. One hundred eighty-seven patients (66.8%) were hypertensive. Upon multivariate analysis of recipient factors, pretransplant hypertension (odds ratio) [OR]: 8.5, 95% confidence interval [CI]: 4.5 to 16.1); serum creatinine level > 130 micromol/L at 6 months (OR: 2.5, 95% CI: 1.3 to 4,7), male gender (OR: 2.02, 95% CI: 1.2 to 3.4), and chronic rejection (OR: 2.4, 95% CI: 1.2 to 4.7) were independent predisposing factors. Among donor factors, age was significantly associated with arterial hypertension upon univariate analysis. In conclusion, recipient factors, especially pretransplant hypertension, contribute to the disorder in renal transplant patients.

[Complications of distal tubular acidosis in primary Sjögren's syndrome]. / Complications de l'acidose tubulaire distale au cours du syndrome de gougerot Sjogren primitif.

Renal involvement in primary Sjögren's syndrome occurs in 10-60% of cases. Tubulointerstitial nephritis with distal renal tubular acidosis (DRTA) is the main type of involvement. It's generally asymptomatic and revealed by complications of DRTA. We report 4 cases of GJS complicated by nephrocalcinosis, tetapresic hypokaliemia and osteomalacia. In 2 cases, nephrocalcinosis was diagnosed simultaneously with the GJS. The 2 other cases were diagnosed 3 and 15 years after primary GJS. All patients were treated by coticosteroids at the dose of 0.5 mg/Kg/jour, alkaline solution, K Cl in 2 cases and vitamin D in 2 cases. After a mean follow up of 8.7 years, the renal function remain stable in the 3 cases of nephrocalcinosis and in a patient with osteomalacia, bone lesions progressed. In conclusion, DRTA must be detected by acidification tests in patients with primary Sjögren's syndrome because of their latency and to prevent severe complications.