Diencephalic syndrome, or Russells syndrome, is a rare neurological disorder seen in infants and children and characterised by failure to thrive and severe emaciation despite normal or slightly decreased caloric intake. Classically there is also locomotor hyperactivity and euphoria. Less commonly diencephalic syndrome may involve skin pallor without anaemia, hypoglycaemia, and hypotension. The syndrome is a rare but potentially fatal cause of failure to thrive in children. Failure to thrive presents on average at seven months of age. Of note the syndrome is not associated with developmental delay. There may be associated hydrocephalus.Diencephalic syndrome was first described by Dr. A. Russell in 1951. It is usually caused by a brain tumor such as a low-grade glioma or astrocytoma located in the hypothalamic-optic chiasmatic region. It is not yet understood how diencephalic syndrome causes the effects on appetite and metabolism which are seen, though inappropriately high growth hormone release has been proposed, as has excessive β-lipotropin secretion and overall increased metabolic demand. It is treated with nutritional optimisation while the underlying lesion is treated with chemotherapy, surgery or radiotherapy. == References ==
Several guidelines, including those from the American College of Chest Physicians, recommend prothrombin complex concentrate for warfarin reversal in people with serious bleeding.For rapid anticoagulation reversal for surgery, four-factor prothrombin complex concentrate reduces international normalized ratio (INR) and decreases bleeding during surgery when compared with administration of fresh frozen plasma. No differences in thromboembolic event was found. Contraindications
The package insert states that prothrombin complex concentrate is contraindicated in patients with disseminated intravascular coagulation, a pathological activation of coagulation, because giving clotting factors would only further fuel this process. However, if the PCC is given because factor levels are low, it can restore normal coagulation. As PCC products contain heparin, they are contraindicated in patients with heparin-induced thrombocytopenia. Chemistry
Prothrombin complex concentrate contains a number of blood clotting factors. Typically this includes factor II, IX, and X. Some versions also contain factor VII, protein C, and protein S. Heparin may be added to stop early activation of the factors. History
The U.S. Food and Drug Administration (FDA) announced its approval in 2013. The FDA approved Kcentras orphan drug status in December 2012. References
Further reading
External links
"Prothrombin complex concentrate". Drug Information Portal. U.S. National Library of Medicine.