Acute Fatty Liver of Pregnancy

Acute fatty liver of pregnancy (AFLP) is a rare but life-threatening condition that develops in the third trimester of pregnancy. AFLP shares similar clinical features with other more common pregnancy-associated conditions. However, early correct diagnosis is important for maternal and fetal survival. Once the diagnosis has been established, immediate delivery and maternal intensive support should be undertaken. Both parents and the infant should be tested for deficiencies of the mitochondrial fatty acid oxidation, especially for long-chain 3-hydroxyacyl-CoA dehydrogenase (LCHAD) deficiency, as many cases of AFLP are related to disruption of this physiological enzymatic pathway.

Endogamia i zwyczaje małżeńskie na Kaszubach a kwestia tzw. genu kaszubskiego

One of the genetic disorders is LCHAD deficiency. It occurs relatively often in Kashubian population, which is among others attributed to endogamy. Before the second half of the 20th century the Kashubs majorly married within a single parish or a town, village. Also in this day and age, in Kashubia people most of all wed someone living in the neighbouring area. It stems from powerful family and group (ethnic) ties, the traditional family model, low level of education. The Kashubs’ close adherence to family values is best expressed in robust wedding ceremonies along with commonly celebrated Polterabend. This social gathering exhibiting some analogies with potlatch, serves as a kind of status marker for a particular family’s popularity; it confers prestige and indicates position within local community. Despite some anxiety brought about by the promulgation of knowledge about “Kashubian gene,” there is no visible tendency in Kashubian community to change the model of spouse selection.