Renal involvement in familial Mediterranean fever in an Algerian population.

The objectives of this study were to investigate the clinical biological and histological renal involvement secondary to familial Mediterranean fever (FMF), the epidemiological data, genetics of our patients and their evolution under treatment. We prospectively studied 58 Algerian patients admitted in our nephrology department from January 2012 to January 2021. The diagnosis of nephropathy was suspected clinically and biologically and confirmed histologically. All our patients were tested for MEFV mutations. Results: 58 patients, 30 males and 28 females, mean age 31.68 ± 12.71; 3 (5.17%) chronic dialysis patients and 55 (94.82%) referred to the nephrology department for renal biopsy with renal symptomatology consisting of nephrotic syndrome in 50 (94. 73%), associated with renal failure 27 (47.36%), mainly primary in 23 (34.5%), secondary to seronegative lupus 13 (22.4%), Crohn's disease 9 (14.5%), sarcoidosis 3 (5.26%), and lymphoma 1 (1.7%); 29 (50%) were from consangineous marriages, the histological study found AA amyloidosis in 52 (89.6%); the genetic study confirmed the diagnosis of FMF in 58 (100%). The evolution of the patients: 20 (34.48%) followed in consultation, 25 (43.10%) in hemodialysis and 13 (22.41%) deceased. Conclusion: Renal involvement was the revealing complication in the diagnosis of FMF which exists in our country, and is still underdiagnosed.

Rapidly progressive renal failure to reveal LCAT deficiency in an Algerian family.

Lecithin-cholesterol acyltransferase (LCAT) deficiency is an autosomal recessive disorder that can reveal two different diseases: a very interesting nephrological picture of complete enzyme deficiency characterized by the association of dyslipidemia, corneal opacities, anemia, and progressive nephropathy; and a partial form (fish-eye disease) with dyslipidemia and progressive corneal opacities only. We report herein the case of a 35-year-old man who presented hypertension, renal symptomatology of rapidly progressive glomerulonephritis associates: nephrotic proteinuria, severe renal failure, in combination with annular corneal opacities, anemia, and dyslipidemia. The diagnosis of familial LCAT deficiency was confirmed by clinical examination, characteristic dyslipidemia, undetectable LCAT levels in plasma, and positive family history.

AA renal amyloidosis: Clinical observations over 20 years.

OBJECTIVE: AA renal amyloidosis is present in Algeria, often secondary to chronic infections, the most frequent being tuberculosis. We studied the evolution of the epidemiology of AA amyloidosis over a period of 20 years. MATERIALS AND METHODS: We conducted a retrospective study of all adult and pediatric patients diagnosed with renal symptomatology of AA amyloidosis from 1994 to 2014 inclusive. The diagnosis was made by renal biopsy performed on native kidneys in the majority of patients, and the biopsy was read by the same pathologist. 378 patients were studied in two groups: G1: 1994 through 2004; G2: 2005 through 2014. RESULTS: The mean age at presentation increased from 42.07 ± 15.82 in G1 to 44.90 ± 14.4 years in G2 (p < 0.00008). Male gender was predominant in both groups. For the comparison of underlying diseases between G1 and G2 we found an increase of inflammatory diseases from 42.84% to 54.6% (p < 0.0011) with a decrease of idiopathic causes from 29.7 to 19.7% (p < 0.042). CONCLUSION: This work shows a decrease in infectious causes and an increase in inflammatory causes of AA amyloidosis, reflecting the progress of antibiotic therapy as well as the protocol put in place by our country to fight tuberculosis. AA amyloidosis of uncertain etiologies has been seen to be decreasing due to better identification of certain inflammatory causes, in particular familial Mediterranean fever.