OBJECTIVE To document bone mineral density of children and adolescents with cystic fibrosis. DESIGN Cross-sectional study. SETTING Tertiary-care center of Northern India, July 2012 to August 2015. PARTICIPANTS 52 children aged 6-18 years with cystic fibrosis and 62 healthy controls of similar age and sex. METHODS Both patients and controls were stratified into two groups, as pre-puberta...

Cystic fibrosis is an autosomal-recessive disorder. In 1989 the gene mutation that causes cystic fibrosis was localized on the long arm of chromosome 7. Cystic fibrosis occurs in 1/2000 children and the majority now reach adulthood. In view of numerous clinical manifestations of cystic fibrosis, these patients frequently require surgery. Cystic fibrosis is therefore of increasing interest to an...

BACKGROUND The respiratory tract in patients with cystic fibrosis is frequently colonised with Staphylococcus aureus. There is great diversity of clinical practice in this area of cystic fibrosis. A systematic review was conducted to study the evidence relating antistaphylococcal therapy to clinical outcome in patients with cystic fibrosis. METHODS A search strategy already evaluated for the ...

Children with cystic fibrosis have variable degrees of exocrine pancreatic insufficiency which, if untreated, is the main cause of fat malabsorption. The impact of pancreatic enzyme supplementation on fat digestion was measured in 41 children with cystic fibrosis, 11 healthy controls, and five children with mucosal diseases by a non-invasive test of intraluminal lipolysis using 13carbon (13C) l...

Cystic Fibrosis is the most common lethal autosomal recessive disorder in the white population, affecting among other organs, the lung, the pancreas and the liver. Whereas Cystic Fibrosis is a monogenic disease, many studies reveal a very complex relationship between genotype and clinical phenotype. Indeed, the broad phenotypic spectrum observed in Cystic Fibrosis is far from being explained by...

1 Scanlin TF. Cystic fibrosis. In: Fishman AP, ed. Pulmonary diseases and disorders. 2nd ed. New York: McGraw-Hill, 1988:1273-94 2 Rommens JM, lanuzzi MC, Kerem B, Drumm ML, Melmer G, Dean M, et al. Identification of the cystic fibrosis gene: chromosome walking and jumping. Science 1989; 245:1059-65 3 Kerem B, Rommens JM, Buchanan JA, Markiewicz D, Cos TK, Chakvarti A, et al. Identification of ...

In this study a perfusion technique has been used to investigate in vivo jejunal absorption and transmural potential difference evoked by the neutral amino acids phenylalanine (56 or 20 mmol/l) and glycine (20 mmol/l), the dibasic amino acid lysine (56 or 5 mmol/l), and a dipeptide glycyl-l-phenylalanine (20 mmol/l) in 11 children with pancreatic insufficiency due to cystic fibrosis and in thre...

Cystic fibrosis is the most common lethal single-gene mutation in people of European descent, with a carrier frequency upwards of 2%. Based upon molecular research, resistances in the heterozygote to cholera and typhoid fever have been proposed to explain the persistence of the mutation. Using a population genetic model parameterized with historical demographic and epidemiological data, we show...

In the submandibular saliva of 10 cystic fibrosis subjects and 10 controls the turbidity and elevated calcium, protein, and amylase concentrations of the cystic fibrosis secretions, and precipitation of calcium and phosphate in a ratio consistent with hydroxyapatite have been confirmed. By electron microscopy the centrifuged deposits of the cystic fibrosis saliva were seen to be composed predom...