During the past 19 years, mortality due to burn injuries has markedly declined for children at the Boston Unit of the Shriners Burns Institute (SBI), dropping from an average of 9% of SBI admissions during 1968-1970 to an average of 1% during 1981-1986. Detailed statistical analysis using logistic regression was necessary for determining whether this decline in mortality was explained by changes in patient characteristics, such as age or burn size, which are known to strongly influence the outcome of burn injuries. This dramatic decline in mortality during the past 19 years was not the result of change in the age of the patients or their burn sizes; rather, it may be attributed to improvements in burn care. Results of this statistical analysis indicated that, for burn injury patients whose ages ranged from 11 days to 19 years, age had no demonstrable effect on survival from a burn injury. Children survived burn injuries at least as well if not better than the young adult (20-29 years of age). Also, infants (less than 1 year old) survived as well as other children (2-19 years old). Dramatic improvement in survival occurred in patients with burns covering more than 50% of the body surface area. Since 1979, mortality has been essentially eliminated for patients with burn sizes less than 70% of the total body surface area (of 296 patients with burns covering 15-69% of the total body surface area, only two patients died). During the period 1979-1986, 29 of 37 patients (78%) survived an 80% or greater total body surface area thermal injury.

Individuals with Prader-Willi syndrome (PWS) have excessive appetite with the ability to consume large quantities of food. Absence of vomiting and a high pain threshold are considered manifestations of the disorder. We present 6 patients with PWS with acute dramatic gastric distention. In 3 young adult women with vomiting and apparent gastroenteritis, clinical course progressed rapidly to massive gastric dilatation with subsequent gastric necrosis. One individual died of overwhelming sepsis and disseminated intravascular coagulation. In 2 children, gastric dilatation resolved spontaneously. Gastrectomy specimens--in 2 cases subtotal and distal, in the other with accompanying partial duodenectomy and pancreatectomy--showed similar changes. All cases demonstrated signs of ischaemic gastroenteritis. All specimens showed diffuse mucosal infarction with multifocal transmural necrosis. Vascular dilatation and small bifrin thrombi were apparent within the infarcted areas. These 6 women with PWS had acute idiopathic gastric dilatation. It is possible that a predisposition to acute gastric dilatation may be related to abnormal gastric homeostasis on a genetic basis. Understanding the mechanisms responsible for this event could increase the understanding of gastrointestinal and appetite regulation in individuals with PWS.

Most lower extremity amputations result from complications of diabetes and arterio-sclerotic occlusive diseases below the inguinal ligament. Improved limb salvage has been achieved by an aggressive approach to distal revascularization in the severely ischemic lower extremity. There remains, however, a high incidence of amputation resulting from progression of the ulceration or gangrene into deeper and less well-vascularized tissues, such as tendon and bone. Even in the nonischemic extremity, such wounds rarely heal without flap coverage. Microvascular free tissue transfers promote healing by providing coverage with healthy, nondiseased, well-vascularized tissue for these difficult defects. Successful free flap transfer requires a high-pressure recipient inflow vessel. In contrast to individuals with nonarteriosclerotic lesions, many individuals with nonhealing ischemic lesions have no acceptable artery demonstrated on high-resolution angiography to serve as a recipient vessel. Limb salvage has been achieved in four candidates for amputation utilizing distal revascularization followed by free tissue transfer coverage of the ischemic lower leg defects.