IntroductionThe Dent's Disease (Dd) Is An X-Linked Genetic Kidney Disease. We Undertook A Retrospective Study Of 4 Dd

Hereditary angioedema (HAE) is rare; it is usually a monogenic, genetic disease with autosomal dominant transmission, resulting in C1-inhibitor deficiency (C1InH). Renal involvement associated with HAE has been known for nearly thirty years. We are reporting on two observations of a father and his daughter presenting a glomerulonephritis evolving towards to CKD class V. Renal needle biopsy had evidenced acute vascular lesions associated with segmental and focal hyalinosis. Since these lesions were not described, we discussed the possible causal links with HAE. The accumulation of bradykinin caused by mutation in C1-Inh could have been misleading, considering all the beneficial vascular and renal effects widely described in the literature, if we stick to that fact, in the study of vascular and glomerular lesions. On the other hand, taken as a whole, HAE appears to pave the way for the development of a glomerular and vascular disease: dysimmunity, circulating immune complexes, and vascular hyperpermeability favor deposition of immune complexes, overexpression and activation of B1 receptors.

Background: Studies in Cameroon reported high prevalence of urinary abnormalities in sickle cell anemia (SCA). There is a lack of data in this setting on the prevalence of chronic kidney disease (CKD) in SCA and Sickle cell trait (SCT).Objective: Assess the prevalence and associate factors of CKD in people with sickle cell disease.Patients and method: This was a cross-sectional study of six months durations (April-September 2017) involving SCA (HB SS) and SCT (HBAS) subjects at Douala Laquintinie hospital. CKD was diagnosed and classified according to 2012 Kidney Disease Improving Global Outcomes (KDIGO) criteria. Only patients with persistent urinary anomalies or decreased eGFR at 3 months were considered to have CKD.Results: We included 107 subjects among which 81 SCA (62% males) and 26 SCT (46% males) with a mean age of 19.5 ± 10 and 35.8 ± 7.8 years (p < 0.001) respectively. Compare to SCT, SCA subjects had lower body mass index and systolic blood pressure. Overall, CKD was found in 16 patients (15%): 13 (16%) patients in the SCA group and 3 patients (11%) in the SCT group. CKD frequency was comparable in both groups (p = 0.76). CKD was found in 37% of adult SCA patients. Albuminuria was more common in the SCA group [12 (15%) vs. 1 (4%) patient, p = 0.018]. Age ≤ 25 years was protective factor for both CKD (OR = 0.20 [0.003-0.135], p < 0.001) and albuminuria (OR = 0.23 [0.004-0.124], p < 0.001) in SCD group. Conclusion:CKD in sickle cell disease is highly prevalent young adult in our setting.