Neonatal hemochromatosis: management,outcome, and prevention

Neonatal hemochromatosis (NH) is a rare disorder but the most common cause of acute liver failure in neonates. NH is characterized by severe hepatic injury and iron overload and is associated with high perinatal mortality and morbidity rates. NH is often preceded by oligohydramnios and intrauterine growth restriction, suggesting an important impact of NH during fetal life. Stillbirth and prematurity are not uncommon. During the last decade, major discoveries on the etiology of NH have radically changed the management and outcome of this disease. NH is now regarded as an alloimmune disease and is, as such, often referred to as gestational alloimmune liver disease. Antenatal treatment with intravenous immunoglobulins starting at 14 weeks' gestation has been shown to prevent the development of NH in subsequent pregnancies. Postnatal treatment, previously based on the use of anti-oxidants and chelation therapy, has now successfully been replaced by exchange transfusions and intravenous immunoglobulins substitution. This review summarizes the latest discoveries on the etiology of NH and the new recommendations concerning its management and prevention. © 2013 John Wiley & Sons, Ltd.     

Thermal strain in fire fighters while wearing task-fitted versus en 469:2005 protective clothing during a prolonged rescue drill.

Fire fighters are normally overprotected during their working hours because of the tendency to keep the personal protection level sufficiently high in case of the worst possible scenarios. This study investigated the effects of task-fitted protective clothing on thermal strain in fire fighters as compared to EN 469:2005 protective clothing during a prolonged (2 1/2 hrs) job-related rescue drill under neutral and hot climates. The subjects were 23 healthy, physically fit professional male fire fighters aged 26-44 years. Measurements included cardiovascular and thermal responses and subjective assessments. Wearing task-fitted clothing during rescue tasks in a neutral climate considerably reduced total thermal and cardiovascular strain in prolonged rescue work. The fire fighters also perceived physical work as significantly harder on average, and reported more intense subjective discomfort while wearing EN 469:2005 as compared to task-fitted clothing.