Multiple apical plasma membrane constituents are associated with susceptibility to meconium ileus in individuals with cystic fibrosis
Lei SunJohanna M. RommensHarriet CorvolWeili LiXin LiTheodore ChiangLin FanRuslan DorfmanPierre BussonRashmi V ParekhDiana ZélénikaScott M. BlackmanMary CoreyVishal DoshiLindsay B. HendersonKathleen NaughtonWanda K. O’NealRhonda G. PaceJaclyn R. StonebrakerSally WoodFred A. WrightJulian ZielenskiAnnick ClémentMitchell L. DrummPierre‐Yves BoëlleGarry R. CuttingMichael R. KnowlesPeter DurieLisa J. Strug
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This paper describes some changes in the physical properties of meconium apart from the exceptionably viscid state found in meconium ileus due to cystic fibrosis of the pancreas. The alterations in meconium described include: ano-rectal plugs, meconium plugs elsewhere in the intestinal tract, hard yellow meconium bodies contained within the green fluid mass of meconium, and pale yellow concretions associated with ulceration of the colon. The possible relation of disturbances in the motility and physiology within the intestine in the fetus to the formation of these abnormalities in the meconium discussed.
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Objective. It is unclear whether children with cystic fibrosis (CF) who present with neonatal meconium ileus have a different long-term outcome from those presenting later in childhood with pulmonary complications or failure to thrive. We examined a cohort of patients with meconium ileus, and compared their long-term outcome with children who had CF without meconium ileus and neonates who had meconium obstruction without CF (meconium plug syndrome). Study Design. Comparative study using retrospective and follow-up interview data. Patients. Group 1 consisted of 35 surviving CF patients who presented with meconium ileus between 1966 and 1992. Two control groups were also studied: 35 age- and sex-matched CF patients without meconium ileus (group 2), and 12 infants presenting with meconium plug syndrome during the same time period (group 3). Outcome Measures. Pulmonary, gastrointestinal, nutritional, and functional status were reviewed, and surgical complications were recorded. Results. Mean follow-up was 12.6 ± 6, 12.6 ± 6, and 9.3 ± 4 years in groups 1, 2, and 3, respectively. Patients without CF (group 3) demonstrated better growth and functional status, and had a lower incidence of pulmonary and gastrointestinal problems. Although the presence of meconium ileus among CF patients was associated with an earlier diagnosis, there were no significant differences between groups 1 and 2 with respect to hepatobiliary, nutritional, functional, or respiratory status. Meconium ileus was associated with a higher risk of meconium ileus equivalent (20% vs 6%), although this difference was not statistically significant. Long-term surgical complications (adhesive small bowel obstruction and blind loop syndrome) were seen in 27% of children with meconium ileus; there were no long-term surgical complications in groups 2 or 3, because these infants did not have any neonatal surgical procedures. Children presenting with complicated meconium ileus had a higher rate of long-term surgical complications than those with uncomplicated meconium ileus (36% vs 17%), and those managed with resection or enterostomy had more complications than those treated by enterotomy and lavage (33% vs 0%). Conclusions. Long-term outcome is similar in CF patients who present with meconium ileus and those who do not, except for a slightly higher incidence of meconium ileus equivalent, and a significantly higher rate of surgical complications. The risk of surgical complications is highest in those presenting with complicated meconium ileus and those undergoing resection or enterostomy. Patients with meconium obstruction who do not have CF have an excellent long-term prognosis. This information will be useful in counseling the families of infants presenting with neonatal meconium obstruction.
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A quantitative microcomplement fixation assay and quantitative protein determinations were used to determine albumin as a percentage of total soluble proteins in meconium. Significant differences (p < 0.001 ) were found between the meconium from infants who develop the symptoms of cystic fibrosis (CF) and between healthy siblings of known patients with CF or normal controls. No statistical differences were seen between newborn infants with CF requiring surgical intervention due to meconium ileus and those not requiring surgery.
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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.
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Meconium samples were collected from 5 newborn infants, who had a known family history of cystic fibrosis of the pancreas but who did not present with meconium ileus, and 11 normal newborn infants. Extracts of the meconium samples were examined for the presence of serum proteins by paper and immunoelectrophoresis. Three of the infants who had a family history of cystic fibrosis of the pancreas showed protein in their meconium, and this was identified by immunoelectrophoresis as consisting mainly of albumin; each of these babies subsequently developed classic symptoms of cystic fibrosis of the pancreas. The two remaining infants had no albumin in the meconium and did not develop signs of cystic fibrosis. None of the meconium samples of the control group of infants contained detectable amounts of albumin. Possible sources of the abnormal protein content of meconium are discussed, and the suggestion that the finding of albumin in meconium of newborn infants may prove to constitute a valuable diagnostic procedure for screening newborn infants for cystic fibrosis of the pancreas is advanced.
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The meconium blockage syndrome, also known as the meconium plug syndrome (1) or temporary meconium ileus (2), was described over a decade ago. The modern definition of the term was made by Clatworthy and his co-workers in 1956 when they noted that “newborn infants with intestinal obstruction due to the inability of the colon to rid itself of the meconium residue of nine months of fetal life are not uncommon.” The meconium blockage produces a clinical picture of mechanical obstruction in the newborn which is indistinguishable from other causes of low intestinal obstruction. It is not the same as meconium ileus, which occurs in 5 to 20 per cent of patients with cystic fibrosis and is associated with a deficiency of intestinal enzymes, producing thick, tenacious meconium in the jejunum and ileum (3–5). All prior reports have considered the meconium in meconium blockage as normal (6, 7). Clinical Features Forty-one cases of meconium blockage were reviewed. The maternal ages, parity, and complications of pregnancy or delivery were not different from the obstetric population as a whole. The 41 cases of meconium blockage occurred between January 1963 and January 1966. During this time there were approximately 10,000 deliveries a year at the Los Angeles County Hospital and 1,600 admissions to the premature center a year. The incidence of meconium blockage was approximately 1 in 1,000 admissions. The ratio of males to females was 2∶3, and 22 of the 41 infants were Negroes in a hospital where 50 per cent of the deliveries are in Negro women. Twenty-three per cent of the 34 infants born at the Los Angeles County Hospital were premature by birth weight, while the expected hospital premature rate is 13 per cent. Six other affected premature infants were admitted for care at the Premature Center, following birth elsewhere. There was no history of intestinal obstruction among siblings. Typically, the initial symptoms were green-stained vomitus and abdominal distention appearing between nine and eighty hours after birth in an otherwise normal infant. In one an early diagnosis was made at three hours of age after obstruction to passage of a rectal thermometer. In another infant, who passed his first meconium at the age of forty hours, the symptoms were not noticed until he was seven days old; these cleared at twelve days of age, following a barium enema. In 18 (48 per cent) of the infants one or more greenish-black meconium stools had been passed before the onset of symptoms. Fifteen (36 per cent) of the infants passed meconium in the first twenty-four hours. Abnormal physical findings were limited to early abdominal distention. As the distention progressed, lethargy, irritability, and respiratory distress developed in some of the infants, due to elevation of the diaphragm by the abdominal distention. Aspiration of gastric contents revealed a small volume of bile-stained gastric juices.
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