Statins and Clarithromycin : a dangerous combination. Case report and review of the literature.

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Defects in complement and "secondary" hemolytic uremic syndrome

The thrombotic microangiopathy syndromes are extraordinarily diverse. Most thrombotic microangiopathy syndromes, ∼90%, occur in the setting of coexisting diseases and have been termed secondary hemolytic uremic syndrome (HUS), while a subset of thrombotic microangiopathy syndromes occur on the background of complement gene variants and are termed primary atypical HUS. 1 Le Clech et al.2 recently reported the low prevalence of such variants in patients with secondary HUS and poor clinical outcomes. [...

The natural course of pregnancies in women with primary atypical haemolytic uraemic syndrome and asymptomatic relatives

Pregnancy has been linked to various microangiopathies, including primary atypical haemolytic uraemic syndrome (aHUS). Complement dysregulation, often linked to rare variants in complement genes, is key for primary aHUS to manifest and may play a role in pregnancy complications of the mother and fetus. The burden of such complications is unknown, making counselling of women with primary aHUS and asymptomatic relatives difficult. We analyzed the maternal and fetal outcomes of 39 pregnancies from 17 women with primary aHUS and two asymptomatic relatives. Seven out of 39 pregnancies were complicated by pregnancy-associated aHUS. Five out of 32 pregnancies not linked to pregnancy-associated aHUS were complicated by pre-eclampsia or HELLP. Rare genetic variants were identified in 10 women (asymptomatic relatives, n = 2) who had a total of 14 pregnancies, including 10 uncomplicated pregnancies. Thirty-five out of 39 pregnancies resulted in live birth. Eight out of 19 women had progressed to end-stage kidney disease, with an incidence of 2·95 (95% confidence interval, 1·37-5·61) per 100 person-years after the first pregnancy. Thus, we emphasized the frequency of successful pregnancies in women with primary aHUS and asymptomatic relatives. Pregnancies should be monitored closely. Rare genetic variants cannot predict the risk of a given pregnancy