Published in J Pediatr on November 01, 1990
Intestinal obstruction syndromes in cystic fibrosis: meconium ileus, distal intestinal obstruction syndrome, and constipation. Curr Gastroenterol Rep (2011) 1.14
Gastrointestinal complications in cystic fibrosis. J R Soc Med (1992) 0.82
Abdominal pain in cystic fibrosis. J R Soc Med (1995) 0.82
Colonic strictures in cystic fibrosis. J R Soc Med (1995) 0.81
Electrogastrography in children with cystic fibrosis. Dig Dis Sci (2002) 0.75
Pseudomonas cepacia infection in cystic fibrosis: an emerging problem. J Pediatr (1984) 9.61
Replacement of insulin receptor tyrosine residues 1162 and 1163 compromises insulin-stimulated kinase activity and uptake of 2-deoxyglucose. Cell (1986) 8.87
The human insulin receptor cDNA: the structural basis for hormone-activated transmembrane signalling. Cell (1985) 7.45
Crystal structure of the tyrosine kinase domain of the human insulin receptor. Nature (1995) 6.16
Prediction of mortality in patients with cystic fibrosis. N Engl J Med (1992) 5.46
The relation between genotype and phenotype in cystic fibrosis--analysis of the most common mutation (delta F508). N Engl J Med (1990) 4.70
Sequence of protein disulphide isomerase and implications of its relationship to thioredoxin. Nature (1985) 3.75
MMAC1/PTEN mutations in primary tumor specimens and tumor cell lines. Cancer Res (1997) 3.38
The risk of cancer among patients with cystic fibrosis. Cystic Fibrosis and Cancer Study Group. N Engl J Med (1995) 2.49
Genetic determination of exocrine pancreatic function in cystic fibrosis. Am J Hum Genet (1992) 2.48
Pulmonary function and clinical course in patients with cystic fibrosis after pulmonary colonization with Pseudomonas aeruginosa. J Pediatr (1990) 2.28
Association of respiratory viral infections with pulmonary deterioration in patients with cystic fibrosis. N Engl J Med (1984) 2.22
Analysis of the apoptotic and therapeutic activities of histone deacetylase inhibitors by using a mouse model of B cell lymphoma. Proc Natl Acad Sci U S A (2007) 2.10
Socioeconomic inequalities in cancer survival in England after the NHS cancer plan. Br J Cancer (2010) 2.07
Five- to seven-year course of pulmonary function in cystic fibrosis. Am Rev Respir Dis (1976) 2.03
A randomized controlled trial of a 3-year home exercise program in cystic fibrosis. J Pediatr (2000) 1.93
Dose-response relationship and reproducibility of the fall in exhaled nitric oxide after inhaled beclomethasone dipropionate therapy in asthma patients. Chest (2001) 1.92
Binding of Pseudomonas cepacia to normal human intestinal mucin and respiratory mucin from patients with cystic fibrosis. J Clin Invest (1992) 1.80
Improving prevention systems in primary care practices: the Health Education and Research Trial (HEART) J Fam Pract (2000) 1.78
Microvillus inclusion disease: an inherited defect of brush-border assembly and differentiation. N Engl J Med (1989) 1.77
Role of conventional physiotherapy in cystic fibrosis. J Pediatr (1988) 1.75
Impaired chloride secretion, as well as bicarbonate secretion, underlies the fluid secretory defect in the cystic fibrosis pancreas. Gastroenterology (1988) 1.75
Correlation of sweat chloride concentration with classes of the cystic fibrosis transmembrane conductance regulator gene mutations. J Pediatr (1995) 1.70
The HIV information project for transfusion recipients a decade after transfusion. Arch Pediatr Adolesc Med (1995) 1.70
Expression of a functional human insulin receptor from a cloned cDNA in Chinese hamster ovary cells. Proc Natl Acad Sci U S A (1985) 1.66
Aneuploidy: a report of an ECETOC task force. Mutat Res (1998) 1.65
Diabetes mellitus in patients with cystic fibrosis: effect on survival. Pediatrics (1990) 1.65
Sex differences in habitual physical activity and lung function decline in children with cystic fibrosis. J Pediatr (2005) 1.64
Improved respiratory prognosis in patients with cystic fibrosis with normal fat absorption. J Pediatr (1982) 1.64
Nerve growth cones isolated from fetal rat brain: subcellular fractionation and characterization. Cell (1983) 1.62
Intussusception: barium or air? J Pediatr Surg (1991) 1.52
High-dose pancreatic-enzyme supplements and fibrosing colonopathy in children with cystic fibrosis. N Engl J Med (1997) 1.51
Energy expenditure of patients with cystic fibrosis. J Pediatr (1987) 1.51
Effect of cimetidine and sodium bicarbonate on pancreatic replacement therapy in cystic fibrosis. Gut (1980) 1.50
Arbor 3D: an interactive environment for examining phylogenetic and taxonomic trees in multiple dimensions. Bioinformatics (2000) 1.50
Use of high-fat formula for premature infants with bronchopulmonary dysplasia: metabolic, pulmonary, and nutritional studies. J Pediatr (1994) 1.50
Acute pancreatitis in childhood. J Pediatr (1988) 1.49
Do common in silico tools predict the clinical consequences of amino-acid substitutions in the CFTR gene? Clin Genet (2009) 1.47
CFTR gene variant for patients with congenital absence of vas deferens. Am J Hum Genet (1995) 1.47
Shwachman syndrome: phenotypic manifestations of sibling sets and isolated cases in a large patient cohort are similar. J Pediatr (1999) 1.47
Molecular consequences of cystic fibrosis transmembrane regulator (CFTR) gene mutations in the exocrine pancreas. Gut (2003) 1.45
Hypoalbuminemia at initial examination in patients with cystic fibrosis. J Pediatr (1989) 1.45
A position paper of the North American Society for Pediatric Gastroenterology and Nutrition. Pediatric gastroenterology Workforce Survey and future supply and demand. J Pediatr Gastroenterol Nutr (1998) 1.45
Bile acid secretion in cystic fibrosis: evidence for a defect unrelated to fat malabsorption. Gut (1986) 1.44
Detection of a cystic fibrosis modifier locus for meconium ileus on human chromosome 19q13. Nat Genet (1999) 1.41
Clinical and genetic comparisons of patients with cystic fibrosis, with or without meconium ileus. J Pediatr (1989) 1.37
The regulatory role of known tyrosine autophosphorylation sites of the insulin receptor kinase domain. An assessment by replacement with neutral and negatively charged amino acids. J Biol Chem (1991) 1.35
Age-related alterations of immunoreactive pancreatic cationic trypsinogen in sera from cystic fibrosis patients with and without pancreatic insufficiency. Pediatr Res (1986) 1.34
Pregnancy and cystic fibrosis. Obstet Gynecol (1991) 1.33
Role of infant feeding practices in development of Crohn's disease in childhood. BMJ (1989) 1.32
Long-term effects of inhaled tobramycin in patients with cystic fibrosis colonized with Pseudomonas aeruginosa. Pediatr Pulmonol (1989) 1.31
Effects of long-term nutritional rehabilitation on body composition and clinical status in malnourished children and adolescents with cystic fibrosis. J Pediatr (1985) 1.30
Autophosphorylation activates the soluble cytoplasmic domain of the insulin receptor in an intermolecular reaction. J Biol Chem (1989) 1.30
Arteriopathy and coarctation of the abdominal aorta in children with mucopolysaccharidosis: imaging findings. AJR Am J Roentgenol (1991) 1.29
Recommendations for management of liver and biliary tract disease in cystic fibrosis. Cystic Fibrosis Foundation Hepatobiliary Disease Consensus Group. J Pediatr Gastroenterol Nutr (1999) 1.28
Treatment of distal intestinal obstruction syndrome in cystic fibrosis with a balanced intestinal lavage solution. Lancet (1986) 1.28
Pathology of pancreatic and intestinal disorders in cystic fibrosis. J R Soc Med (1998) 1.28
Skeletal phenotype in patients with Shwachman-Diamond syndrome and mutations in SBDS. Clin Genet (2004) 1.27
Evidence for a primary defect of pancreatic HCO3-secretion in cystic fibrosis. Pediatr Res (1982) 1.26
Uncertainty in the diagnosis of cystic fibrosis: possible role of in vivo nasal potential difference measurements. J Pediatr (1998) 1.26
Shwachman syndrome: exocrine pancreatic dysfunction and variable phenotypic expression. Gastroenterology (1996) 1.25
Mechanisms of receptor-mediated transmembrane communication. Cold Spring Harb Symp Quant Biol (1986) 1.22
Pseudomonas aeruginosa and Burkholderia cepacia infection in cystic fibrosis patients treated in Toronto and Copenhagen. Pediatr Pulmonol (1998) 1.22
Supplementary enteral nutrition maintains remission in paediatric Crohn's disease. Gut (1996) 1.22
Cystic fibrosis patients bearing both the common missense mutation Gly----Asp at codon 551 and the delta F508 mutation are clinically indistinguishable from delta F508 homozygotes, except for decreased risk of meconium ileus. Am J Hum Genet (1992) 1.22
STUDIES ON THE ETIOLOGY OF PRIMARY ATYPICAL PNEUMONIA : II. PROPERTIES OF THE VIRUS ISOLATED AND PROPAGATED IN CHICK EMBRYOS. J Exp Med (1945) 1.21
Shwachman-Diamond syndrome with exocrine pancreatic dysfunction and bone marrow failure maps to the centromeric region of chromosome 7. Am J Hum Genet (2001) 1.21
Evidence for insulin-dependent activation of S6 and microtubule-associated protein-2 kinases via a human insulin receptor/v-ros hybrid. J Biol Chem (1990) 1.19
Immunolocalisation of Burkholderia cepacia in the lungs of cystic fibrosis patients. J Med Microbiol (2001) 1.19
Colipase and lipase secretion in childhood-onset pancreatic insufficiency. Delineation of patients with steatorrhea secondary to relative colipase deficiency. Gastroenterology (1984) 1.17
Assessing activity of pediatric Crohn's disease: which index to use? Gastroenterology (1999) 1.17
Expression, characterization, and crystallization of the catalytic core of the human insulin receptor protein-tyrosine kinase domain. J Biol Chem (1995) 1.17
Bronchial hyperreactivity in cystic fibrosis and asthma. J Pediatr (1978) 1.16
Use of pancreatic enzyme supplements for patients with cystic fibrosis in the context of fibrosing colonopathy. Consensus Committee. J Pediatr (1995) 1.16
Dietary intakes of young children with cystic fibrosis: is there a difference? J Pediatr Gastroenterol Nutr (1996) 1.12
Coagulase-negative staphylococci as true pathogens in newborn infants: a cohort study. Pediatr Infect Dis J (1987) 1.12
Complement factor H or a related protein is a marker for transitional cell cancer of the bladder. Clin Cancer Res (1998) 1.11
Late gastrointestinal bleeding and protein loss after distal small-bowel resection in infancy. J Pediatr Gastroenterol Nutr (1989) 1.11
Antibiotic prophylaxis in cystic fibrosis: inhaled cephaloridine as an adjunct to oral cloxacillin. J Pediatr (1982) 1.09
Simple renal cysts in children: diagnosis and follow-up with US. Radiology (1991) 1.09
Nerve growth cones isolated from fetal rat brain. IV. Preparation of a membrane subfraction and identification of a membrane glycoprotein expressed on sprouting neurons. J Cell Biol (1985) 1.08
Angiotensin converting enzyme inhibitor therapy to decrease microalbuminuria in normotensive children with insulin-dependent diabetes mellitus. J Pediatr (1990) 1.07
Conjugation with (111)In-DTPA-poly(ethylene glycol) improves imaging of anti-EGF receptor antibody C225. J Nucl Med (2001) 1.06
Characterization of insulin-stimulated protein serine/threonine kinases in CHO cells expressing human insulin receptors with point and deletion mutations. Biochem J (1992) 1.06
Phase II trial of cisplatin, interferon alpha-2b, doxorubicin, and 5-fluorouracil for biliary tract cancer. Clin Cancer Res (2001) 1.06
Nerve growth cones isolated from fetal rat brain. III. Calcium-dependent protein phosphorylation. J Neurosci (1985) 1.05
Heterologous transmembrane signaling by a human insulin receptor-v-ros hybrid in Chinese hamster ovary cells. Proc Natl Acad Sci U S A (1987) 1.05
Iatrogenic hyperuricemia in children with cystic fibrosis. J Pediatr (1978) 1.04