The utility of guaiac stool testing in the detection of gastrointestinal complications in infants with critical congenital heart disease.

BACKGROUND: Guaiac stool testing has been routinely used as a method to detect gastrointestinal complications in infants with critical congenital heart disease (CHD); however, the sensitivity and specificity have not been established. METHODS: A retrospective chart review was performed investigating the presence of heme-positive stools and subsequent gastrointestinal complications as well as time to goal caloric intake and radiograph exposure. RESULTS: The presence of heme-positive stools was not a statistically significant factor in patients with critical CHD that experienced gastrointestinal complications. Additionally, patients with heme-positive stools did undergo more abdominal X-rays than those with heme-negative stools. CONCLUSIONS: The routine use of guaiac stool testing in infants with critical CHD is not a predictor of possible gastrointestinal complications and leads to more radiograph exposure for the patient. Close clinical monitoring can be used to evaluate feeding tolerance in infants with critical CHD.

Growth hormone as a rescue treatment in maple syrup urine disease with lessons from pediatric burn literature, case report and brief literature review.

Maple Syrup Urine Disease (MSUD) is a rare inherited disorder of branched chain amino acid metabolism characterized by cerebral edema and death in uncorrected metabolic crisis. It is conventionally treated with intensive nutritional therapy to prevent and correct metabolic crisis. This paper reports the use of growth hormone as a pharmacologic rescue agent in the case of an 11-year-old male with MSUD and metabolic crisis refractory to standard interventions. The initiation of short courses of growth hormone correlated with corrected mental status, resolution of metabolic acidosis, and improvement in plasma leucine levels on two occasions during an admission to the pediatric intensive care unit. This is the first known case report of the use of growth hormone in MSUD since contemporary dietary management became available. The discussion includes a literature review of the use of growth hormone in inherited diseases of amino acid metabolism and a brief discussion of protein anabolic pharmacotherapeutic agents shown to improve net protein balance in pediatric burn patients. We propose that growth hormone and other protein anabolic agents may be valuable adjuvants to standard therapy in children with inherited metabolic disease.