Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0728731 (prematurity)
 7,134 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

The authors observed six cases of cholestatis jaundice occuring during parenteral alimentation in the newborn and the infant. In 4 cases, local factors (duodenal atresia, necrosing enteritis of prematurity) or general factors (mucoviscidosis) probably played an essential role. In two cases, the parenteral alimentation seemed to be a main factor, but in one case with excessive and imbalanced supplies of amino acids. Three patients survived, with regression of the jaundice in 15 days to three weeks following the interruption of parenteral alimentation.
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PMID:[Cholestatic icterus during parenteral feeding in the newborn ano infants]. 2 84

Cystic fibrosis is the most frequent substantially lethal inherited disease in the United States. Newborn screening for cystic fibrosis has been suggested because early diagnosis permits genetic counselling of parents and improved treatment and prognosis for cystic fibrosis patients. The fact that cystic fibrosis newborns have abnormally high meconium albumin seems to offer the best screening approach. Because we have had equivocal and false positive results with meconium albumin test strips, we have developed a nephelometric automated immunoprecipitation method for the quantitative and specific analysis of meconium albumin. On a total of 3895 meconiums so far examined, eleven albumin elevations of undetermined origin have been detected. Nine of these were associated with prematurity. There have been, so far, no detected true positives and no known false negatives. The normal range for meconium albumin is in agreement with previously published ranges determined using manual immunochemical methods. This method is rapid and economic. The question of sensitivity, specificity and predictive value of this and other detection methods is discussed.
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PMID:Automated immunoprecipitation of meconium albumin for cystic fibrosis screening in the newborn. 62 Apr 57

The Boehringer-Mannheim Corporation (BMC) strip test is extremely reliable in indicating an albumin content above 20 mg/gm of dried meconium. All infants born during one year in 14 Milwaukee area hospitals were tested. Of 16,224 newborns, two were diagnosed correctly as suffering from cystic fibrosis and two were missed. False-positive tests were obtained in 0.9% of infants (prematurity, melena, gastroschisis, and intrauterine infection). The strip test is, at present, the best available but not the perfect screening method for cystic fibrosis.
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PMID:Test strip meconium screening for cystic fibrosis. 83 24

Despite reduced fertility, pregnancy is likely to occur with increasing frequency in cystic fibrosis in proportion to the number of patients reaching childbearing age. Thirty-eight pregnancies in 25 patients with cystic fibrosis are presented. Twelve of the 25 mothers were pancreatic-sufficient. Despite previous reports of the hazards of pregnancy in cystic fibrosis, we found that pregnancy was well tolerated by the vast majority of our patients and had little effect on their pulmonary and nutritional status. Therapeutic abortion for medical indications was required in two pregnancies. The incidence of prematurity and the neonatal mortality rate were low, and no congenital abnormalities occurred. We conclude that pregnancy can be tolerated by the majority of cystic fibrosis patients, particularly those who are pancreatic-sufficient.
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PMID:Pregnancy and cystic fibrosis. 203 Aug 56

The essential effects of Tocopherol are based on its antioxidative capacity. Tocopherol, however, is just one in a group of antioxidants, which are important for the organism. Established indication for therapeutical application of vitamin E in infancy is only vitamin-E-malabsorption in connection with chronic cholestasis, pancreatic insufficiency (cystic fibrosis) and short bowel syndrome. In emergency therapy vitamin E is suggested with high dosage in case of shock lung and haemolytic-uraemic syndrome. Positive effects of daily vitamin E application in connection with prophylaxis of retinopathy prematurity, bronchopulmonary dysplasia and intraventricular encephalorrhagia of premature infants of severe underweight are not established. Very questionable therapeutic or prophylactic efficiency is opposed to the risk of higher incidence of severe complications in caring for premature infants of severe underweight, such as enterocolitis necroticans and neonatal septicaemia.
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PMID:[The use of vitamin E in childhood]. 209 6

The use of elevated dosages of vitamin E in humans has led to the discovery of vitamin E deficiency syndromes in neurological areas. This evidence comes from careful clinical studies in which elevated vitamin E dosages were applied. In long-term studies it has now been established that retinal and neurological abnormalities are due to vitamin E deficiency and can be ameliorated by therapy with a large amount of the vitamin enterally or parenterally, which can possibly completely prevent the development of clinical manifestations if adequate treatment is given from an early age. It has also become clear that similar neurological and ocular lesions occur in other chronic fat malabsorptive states such as cholestatic liver diseases, cystic fibrosis, and extensive resection of the gut, with respect to an elevated dosage of vitamin E therapy. More recently, several patients with spinocerebellar degeneration from vitamin E deficiency without other evidence of malabsorption have been reported on in whom the progression of the diseases is cessated by the vitamin E therapy. Whether or not the use of elevated dosages of vitamin E should be recommended for certain diseases in premature infants is controversial. Previously, it has been thought that newborn infants, especially premature infants, suffer from vitamin E deficiency, because of their low plasma vitamin E concentrations and high susceptibility of erythrocytes to hydrogen peroxide hemolysis test. Furthermore, tocopherol deficiency has been implicated in four neonatal conditions: anemia of prematurity, retrolental fibroplasia (RLF), bronchopulmonary dysplasia (BPD), and intraventricular hemorrhage (IVH). A hemolytic anemia, associated with thrombocytosis and edema, which is responsive to vitamin E therapy, is not well recognized and occurs in a minority of preterm infants, who were given high amounts of polyunsaturated fatty acids in their formula. However, prophylactic use of an elevated dosage of vitamin E to prevent anemia in the majority of premature infants is controversial. There is no evidence for beneficial effects in BPD. In addition, the prophylactic use of pharmacological dosages of vitamin E for prevention of RLF and IVH has also had conflicting results. In the course of therapy with elevated dosages of vitamin E, administered either orally, intramuscularly, or intravenously, many problems arose in the infants, such as unexpected death, increased frequency of necrotizing enterocolitis (NEC) and sepsis, and the development of unusual symptoms including hepatic injuries.(ABSTRACT TRUNCATED AT 400 WORDS)
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PMID:Use and safety of elevated dosages of vitamin E in infants and children. 250 8

Meconium ileus and pancreatic changes, as described in cystic fibrosis, were found, at autopsy, in a series of six infants who received prolonged neonatal intensive care for prematurity. Cystic fibrosis had not been suspected clinically. These pathological findings are so frequent in sick premature infants, amounting to 12% of all neonatal autopsies conducted over a period of 2 yr in our unit, that we question their specificity for cystic fibrosis and suggest they may be a manifestation of disordered physiology in the severely ill neonate.
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PMID:Diagnosis of cystic fibrosis in premature infants. 371 63

The two most important pathologic conditions leading to mycotic opportunistic infections in children are impairment of mechanisms of defense due to immunosuppressive drugs and congenital defects of immunity. Other circumstances belong to pediatrics such as prematurity or cystic fibrosis. A few examples are chosen to illustrate these situations: congenital candidiasis, chronic mucocutaneous candidiasis, chronic dermatophytic disease, neonatal candidemia, mycotic infections in chronic granulomatous disease and aspergillosis in cystic fibrosis.
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PMID:[Opportunistic mycoses in pediatrics]. 383 6

The authors point out the problems that are raised when a patient with cystic fibrosis becomes pregnant. Pregnancy in a patient with cystic fibrosis is rare but these patients do not seem to have diminished fertility. So it is likely that in the future this combination will increase in frequency. The genetic risk is raised. Heterozygotic subjects are about 3 to 5% of the population and there is a risk of 1 in 4 that heterozygotic parents who already have one child with cystic fibrosis will have a second. The BM test and the sweat test are used to screen for the disease at birth. The pregnancy does not always progress well. The level of prematurity is more than 26% and perinatal mortality is 11%. Furthermore, the birthweight of these children is at the lower limits of normal. Finally, the pregnancy makes the mother's state worse with an increase in her lung signs and change in her respiratory function. On the other hand, diabetes seems to be easily controlled. In conclusion, pregnancy seems to be detrimental for these patients.
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PMID:[Cystic fibrosis and pregnancy]. 711 86

Disturbances of the excretion and reabsorption of bile acids are discussed. Disturbances in bile acid metabolism can be found after terminal ileum resections, in contaminated small bowel syndrome, Crohn's disease localized in terminal ileum, ileostoma, non-specific diarrhoea of infancy, bulky small bowel contents, coeliac disease, cystic fibrosis, prematurity, intra- and extrabiliary obstruction. They can cause or increase malabsorption. Total bile acid and cholylglycine in serum and duodenal fluid can be used in diagnosing malabsorption. Estimation of serum bile acids and triglycerides after an oral fat loading test may provide information about the localization of small intestinal dysfunction.
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PMID:[Bile acids and malabsorption; physiology, pathology and diagnosis]. 726 54


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