been found, improvements in the course of the disease have been significantly seen after the administration of nutritional and vitamin supplements, especially in children. It is stated that if there is no remission by about 12 to 18 months of childhood, liver transplantation is necessary at a later age. Neonatal cholestasis lasted no more than one year in some patients or lasted until the age of 6/7 years in some cases. In recent decades, cholestatic episodes have lasted a shorter time than before 1970. There has also been a decline in rickets, growth retardation, and neuropathy over time. The reasons for this are improved nutritional habits, vitamin and cholestyramine supplementation. Nevertheless, it is not yet possible to prevent serious liver disease. History The first reported cases of Aagenaes syndrome were described in a study in 1968 from the southwest of Norway. Four out of five children born before 1939 died of bleeding in the first weeks of life. Vitamin K was introduced this year, after the birth of another four children after 1939. When these children received one dose of this vitamin, three of them died at the age of 5–9 months. The confirmed cause of death in one or two children in this group was bleeding. The remaining seven children, born in 1935 and in 1942–1966, who were part of a study published in 1968, were alive at the time. See also Lymphedema-distichiasis syndrome Lymphedema praecox Blueberry muffin baby References External links Vascular-related cutaneous conditions Immune system disorders Congenital disorders Syndromes affecting the hepatobiliary system