Neonatal Nurse Practitioner Practice Exam

Which condition typically differs from meconium plug syndrome primarily due to enzymatic deficiency?

1. Meconium ileus

2. Neonatal Hirschsprung disease

3. Intestinal obstruction

4. Pyloric stenosis

The correct answer is: Meconium ileus

Meconium ileus is primarily associated with cystic fibrosis, which is characterized by an enzymatic deficiency due to a mutation in the CFTR gene. This deficiency leads to thick, viscous secretions that contribute to the obstruction of the intestines, particularly in the ileum, resulting in the retention of meconium. In contrast, meconium plug syndrome is mainly caused by the accumulation of meconium in the intestines without the same enzymatic issues seen in meconium ileus. While both conditions involve obstruction related to meconium, meconium ileus is distinctly linked to the pathophysiology of cystic fibrosis and the resultant inability to secrete adequate fluid due to the lack of specific enzymes, thereby altering the consistency of the meconium. Other options, such as neonatal Hirschsprung disease, intestinal obstruction, and pyloric stenosis, do not involve an enzymatic deficiency but rather are related to anatomical or functional blockages that do not stem from the same underlying enzymatic causes as meconium ileus. Thus, the emphasis on the enzymatic deficiency characteristic of cystic fibrosis firmly supports the differentiation of meconium ileus from meconium plug syndrome.