Anti-glomerular basement membrane glomerulonephritis and thrombotic microangiopathy in first degree relatives: a case report.

BACKGROUND: Anti-glomerular basement membrane glomerulonephritis and thrombotic microangiopathy are rare diseases with no known coherence. CASE PRESENTATION: A daughter and her biological mother were diagnosed with pregnancy-induced thrombotic microangiopathy and anti-glomerular basement membrane glomerulonephritis, respectively. Both developed end-stage renal disease. Exploration of a common aetiology included analyses of HLA genotypes, functional and genetic aspects of the complement system, ADAMTS13 activity and screening for autoantibodies.The daughter was heterozygous carrier of the complement factor I G261D mutation, previously described in patients with membranoproliferative glomerulonephritis and atypical haemolytic uremic syndrome. The mother was non-carrier of this mutation. They shared the disease associated complement factor H silent polymorphism Q672Q (79602A>G). CONCLUSION: An unequivocal functional or molecular association between these two family cases was not found suggesting that the patients probably share another, so far undiagnosed and unknown, predisposing factor. It seems highly unlikely that two infrequent immunologic diseases would occur by unrelated pathophysiological mechanisms within first degree relatives.

Couple Formation is Prolonged not Postponed. New Paths to Union Formation in Contemporary France.

In France, as in other European countries, the age at first cohabiting union has risen over the past decades, as a result of longer school enrolment, structural economic changes, and new family norms. While the median age at first co-resident couple was 23.8 for men born in France in the beginning of the 1950s, it was 26.0 among the generation born in the beginning of the 1970s. This tendency is often referred to as a postponement of couple formation and as a part of a broader delay in the transition to adulthood. This article argues, on the contrary, that couple formation has not been postponed but prolonged. In fact, age at first couple formation has remained stable across generations born since the mid-twentieth century in France. Starting from there, we take on a biographical approach to examine the nature, duration, and articulation of the successive stages that make up young people's conjugal trajectories in France. What are the different pathways into couple life, and how have these changed over time? In order to answer these questions, we use optimal matching methods to identify ideal typical trajectories and then logistic regressions in order to see how these relate to generational differences as well as sociodemographic characteristics. We observe three traditional, three timeless and five new paths to couple life. The main historical change is the increasingly gradual nature of union formation, a trend that reflects a dual pattern. First, unions are progressively institutionalized: the time laps between different relationship stages, such as "going out," "settling in," and eventually marrying, have expanded. Second, young people increasingly experience several relationships during youth: the different steps of couple formation are taken with different partners. We conclude that couple formation is not delayed per se; it is rather the material and institutional formalization of unions that is put off for the future. We discuss the scientific and methodological implications of this finding. Supplementary Information: The online version contains supplementary material available at 10.1007/s10680-022-09629-0.