Improving glucocorticoid replacement profiles in adrenal insufficiency

There is an increased mortality associated with adrenal insufficiency despite glucocorticoid replacement therapy with a standardized mortality ratio greater than two. The cause of the increased mortality is yet to be definitively elucidated, but may be due to excess steroid exposure, or replacement regimens that are uncoupled from the normal physiological cortisol profile. Cortisol secretion follows an ultradian pattern which is not possible to reproduce using oral replacement. With the advent of new pumps, it is now possible to mimic the pulsatility of the adrenal glands. While the cognitive and emotional benefits of reproducing the ultradian rhythm are known, the presence of long‐term benefits is not yet clear. There is a dearth of evidence and high‐quality studies to underline our current understanding of the pathophysiology of adrenal insufficiency and replacement therapy. There is a particular lack of research comparing objective outcomes between patients receiving hydrocortisone replacement (either standard therapy or new sustained release preparations), prednisolone replacement and ultradian pumps. Direct comparative studies are now warranted to understand the optimal approach.     

An Unusual Case of Neonatal High-Output Heart Failure: Infantile Hepatic Hemangioma

BackgroundInfantile hepatic hemangioma (IHH) is a rare but life-threatening disorder that must be considered in the newborn presenting with high-output heart failure (HF). IHH is a tumor comprised of large vascular beds, which require a significant increase in blood flow as the lesion grows. This, in turn, creates an undue burden on the cardiovascular system, leading to high-output HF and potentially, respiratory distress. Recent changes have been made in the classification of certain hepatic hemangiomas and their treatments.Case ReportA 10-day-old girl presented to the Emergency Department with increased respiratory effort and an episode of apnea and cyanosis. A chest x-ray study was obtained and showed cardiomegaly and pulmonary edema concerning for HF. The patient was promptly admitted to the pediatric intensive care unit, where advanced imaging was obtained revealing findings consistent with IHH.Why Should an Emergency Physician Be Aware of This?HF in an infant is a critical condition often requiring prompt intervention and rapid diagnosis of the correct etiology to save a life. IHH is an example of an extrathoracic etiology of pediatric HF that has undergone recent changes in terminology and diagnosis. Increased awareness among emergency physicians of this disease process and its treatments can lead to expeditious diagnosis and treatment of this potentially life-threatening illness.     

Pediatric vascular tumors of the liver:Review from the pathologist’s point of view

Differential diagnosis of pediatric vascular liver tumors can be challenging due to inconsistent nomenclature, histologic overlap and the rarity of some entities. Here we give an up-to-date overview of the most important entities. We discuss the clinic, histology and pathophysiology of hepatic congenital and infantile heman-gioma, hepatic epithelioid hemangioendothelioma and hepatic angio-sarcoma.