Studies in Cystic Fibrosis of the Pancreas: Protein Pattern in Meconium Ileus

Abstract

Aqueous extracts of meconium obtained from normal children and from patients with meconium ileus were examined by means of chemical precipitation, paper electrophoresis and precipitin tests. All the extracts from patients with meconium ileus were found to contain protein which was readily precipitated by trichloracetic acid. In 59 extracts of meconium from normal infants no protein could be demonstrated by the use of trichloracetic acid. In the abnormal extracts, the major constituent was found to be identical with serum albumin, both by paper, electrophoretic-migration studies at two pH levels and by precipitin tests with anti-albumin horse serum. Other protein components were found on electrophoresis that were similar, but not necessarily identical with, the alpha-, beta-, and gamma-globulins of human serum. The extracts from normal meconium gave only a very faint reaction for protein. The electrophoretic pattern did not in any way resemble that obtained with the abnormal extract. Precipitin tests showed that the albumin content of the normal extract was about 1-2% of that of the abnormal extract. After electrophoretic separation of the components of extracts of meconium from patients with meconium ileus, the stainable carbohydrate was found to be greater in the "alpha" region than in the corresponding area for normal serum. The possible role of plasma proteins in the formation of abnormal meconium in meconium ileus is discussed.

Contemporary management of meconium ileus

“…In 1952 Buchanan and Rapoport demonstrated that the abnormally viscid meconium in babies with meconium ileus contained less carbohydrate and more protein than normal meconium [14]. In 1958 Green et al [15] reported that the major protein in meconium is albumin. This observation has subsequently been confirmed by several other authors, with some noting that the albumin concentration in meconium was rive to ten rimes higher than the levei in the meconium of unaffected neonates [16,17].…”

“…Diez pacientes con necomium ileus no complicado fueron tratados exitosamente con enema de gastrografina. Los 15 pacientes restantes requirieron laparotomfa; en nueve se hizo resecci6n intestinal y enterostom[a y seis casos recientes fueron manejados con resecci6n intestinal y anastomosis (15) o enterostomia (20). La sobrevida a un afio fue de 92% en los pacientes con meconium ileus complicado.…”

Levels and Molecular Forms of Immunoreactive Trypsin and Chymotrypsin in Amniotic Fluids from Normal and Cystic Fibrosis Fetus

“…Such a lack of activation of proteolytic zymogens in CF fetus is in keeping with previous data showing an absence of active proteases in CF meconium, the first example of which being an increased albumin content discovered by Green et at. (22). This observation led to the so called " BM test ," in which a strip impregnated with buffered tetrabromophenolphtalein becomes intensely blue if > 20 mg of albumin per gram (dry weight) of meconium is present.…”