One of the most common causes of small bowel obstruction in newborns is ileal atresia, and one of the most common causes of colonic obstruction in neonates is aganglionic megacolon (Hirschsprung disease). However, atr...One of the most common causes of small bowel obstruction in newborns is ileal atresia, and one of the most common causes of colonic obstruction in neonates is aganglionic megacolon (Hirschsprung disease). However, atresias of the small intestinal tract associated with Hirschsprung disease are extremely rare. We describe an infant born with both ileal atresia and Hirschsprung disease. This is the 19th known report of the case of an infant who had ileal atresia associated with Hirschsprung disease.展开更多
Background: There is a dearth of information regarding the occurrence of neurocutaneous melanocytosis (NCM) in a large cohort of persons with large congenital melanocytic nevi (LMCN) or multiple congenital melanocytic...Background: There is a dearth of information regarding the occurrence of neurocutaneous melanocytosis (NCM) in a large cohort of persons with large congenital melanocytic nevi (LMCN) or multiple congenital melanocytic nevi (MCMN). Objective: The purpose of this article is to report occurrence of NCM and other complications in 1008 persons having LCMN or MCMN. Methods: Evaluation of information obtained from a database of persons with LCMN or MCMN voluntarily submitted by the affected persons to a nevus support group, the Nevus Network. Results: Of those with truncal LCMN, 6.8% developed significant complications, 4.8% developed symptomatic NCM, and 2.3% died from either benign or malignant NCM or cutaneous melanoma. Of the 4.8% of persons with a truncal nevus who developed symptomatic NCM, 34% died. Of those with head or extremity LCMN, 0.8% developed symptomatic NCM, and, to date, none have died from any cause. Of the small number with MCMN without a giant nevus, 71% developed symptomatic NCM, and 41% died of it. Limitations: Attending physician confirmation of submitted information was unavailable. Conclusions: LCMN of the trunk were associated with a relatively low occurrence of medical complications and death in our group, considering the large nevomelanocytic burden present. If symptomatic NCM developed in those with truncal nevi, the occurrence of death rose to a third. LCMN of the head or extremity were associated with minimal medical complications and no deaths. In contrast, most of the rare persons (N = 17) with MCMN developed symptomatic NCM, and more than a third died.展开更多
文摘One of the most common causes of small bowel obstruction in newborns is ileal atresia, and one of the most common causes of colonic obstruction in neonates is aganglionic megacolon (Hirschsprung disease). However, atresias of the small intestinal tract associated with Hirschsprung disease are extremely rare. We describe an infant born with both ileal atresia and Hirschsprung disease. This is the 19th known report of the case of an infant who had ileal atresia associated with Hirschsprung disease.
文摘Background: There is a dearth of information regarding the occurrence of neurocutaneous melanocytosis (NCM) in a large cohort of persons with large congenital melanocytic nevi (LMCN) or multiple congenital melanocytic nevi (MCMN). Objective: The purpose of this article is to report occurrence of NCM and other complications in 1008 persons having LCMN or MCMN. Methods: Evaluation of information obtained from a database of persons with LCMN or MCMN voluntarily submitted by the affected persons to a nevus support group, the Nevus Network. Results: Of those with truncal LCMN, 6.8% developed significant complications, 4.8% developed symptomatic NCM, and 2.3% died from either benign or malignant NCM or cutaneous melanoma. Of the 4.8% of persons with a truncal nevus who developed symptomatic NCM, 34% died. Of those with head or extremity LCMN, 0.8% developed symptomatic NCM, and, to date, none have died from any cause. Of the small number with MCMN without a giant nevus, 71% developed symptomatic NCM, and 41% died of it. Limitations: Attending physician confirmation of submitted information was unavailable. Conclusions: LCMN of the trunk were associated with a relatively low occurrence of medical complications and death in our group, considering the large nevomelanocytic burden present. If symptomatic NCM developed in those with truncal nevi, the occurrence of death rose to a third. LCMN of the head or extremity were associated with minimal medical complications and no deaths. In contrast, most of the rare persons (N = 17) with MCMN developed symptomatic NCM, and more than a third died.
