Deficiency of vitamins E and A in cystic fibrosis is independent of pancreatic function and current enzyme and vitamin supplementation

1996 ◽  
Vol 155 (4) ◽  
pp. 281-285 ◽  
Author(s):  
L. Lancellotti ◽  
C. D'Orazio ◽  
G. Mastella ◽  
G. Mazzi ◽  
U. Lippi
Keyword(s):  
Cystic Fibrosis ◽  
Pancreatic Function ◽  
Vitamin Supplementation ◽  
Vitamins E And A

Serum Immunoreactive Pancreatic Lipase and Cationic Trypsinogen for the Assessment of Exocrine Pancreatic Function in Older Patients With Cystic Fibrosis

PEDIATRICS ◽  
1986 ◽  
Vol 77 (3) ◽  
pp. 301-306
Author(s):  
Geoffrey Cleghorn ◽  
Lynne Benjamin ◽  
Mary Corey ◽  
Gordon Forstner ◽  
Francesco Dati ◽  
...  
Keyword(s):  
Cystic Fibrosis ◽  
Pancreatic Lipase ◽  
Elevated Serum ◽  
Serum Levels ◽  
Exocrine Pancreatic Function ◽  
Pancreatic Function ◽  
Exocrine Function ◽  
Serum Lipase ◽  
Significant Difference ◽  
Cationic Trypsinogen

Indirect and qualitative tests of pancreatic function are commonly used to screen patients with cystic fibrosis for pancreatic insufficiency. In an attempt to develop a more quantitative assessment, we compared the usefulness of measuring serum pancreatic lipase using a newly developed enzyme-linked immunosorbent immunoassay with that of cationic trypsinogen using a radioimmunoassay in the assessment of exocrine pancreatic function in patients with cystic fibrosis. Previously, we have shown neither lipase nor trypsinogen to be of use in assessing pancreatic function prior to 5 years of age because the majority of patients with cystic fibrosis in early infancy have elevated serum levels regardless of pancreatic function. Therefore, we studied 77 patients with cystic fibrosis older than 5 years of age, 41 with steatorrhea and 36 without steatorrhea. In addition, 28 of 77 patients consented to undergo a quantitative pancreatic stimulation test. There was a significant difference between the steatorrheic and nonsteatorrheic patients with the steatorrheic group having lower lipase and trypsinogen values than the nonsteatorrheic group (P <.001). Sensitivities and specificities in detecting steatorrhea were 95% and 86%, respectively, for lipase and 93% and 92%, respectively, for trypsinogen. No correlations were found between the serum levels of lipase and trypsinogen and their respective duodenal concentrations because of abnormally high serum levels of both enzymes found in some nonsteatorrheic patients. We conclude from this study that both serum lipase and trypsinogen levels accurately detect steatorrhea in patients with cystic fibrosis who are older than 5 years but are imprecise indicators of specific pancreatic exocrine function above the level needed for normal fat absorption.

Management of Pulmonary Disease in Patients with Cystic Fibrosis

2001 ◽  
Vol 14 (3) ◽  
pp. 207-227 ◽  
Author(s):  
Christine A. Robinson ◽  
Robert J. Kuhn
Keyword(s):  
Cystic Fibrosis ◽  
Pulmonary Disease ◽  
Pancreatic Enzyme ◽  
The United States ◽  
Vitamin Supplementation ◽  
Resistant Bacteria ◽  
New Agents ◽  
Pharmacological Management ◽  
Essentially Normal ◽  
Pulmonary Manifestations

Cystic fibrosis is the most common, life-shortening autosomal recessive disease, affecting approximately 1 in 3400 live births in the United States. Gastrointestinal and pulmonary manifestations are most common. With the introduction of pancreatic enzyme and vitamin supplementation, lung disease accounts for the vast majority of morbidity and mortality in patients with cystic fibrosis. The lungs of cystic fibrosis patients are essentially normal at birth but demonstrate evidence of airway inflammation and infection in early infancy. A vicious cycle of inflammation, infection and obstruction ultimately leads to destruction of airways, impairment of gas exchange and death. Current pharmacological management of pulmonary disease targets reducing airway obstruction, controlling infection and more recently, controlling inflammation. An increased recovery of unusual and highly resistant bacteria from patients with more advanced disease has been observed. Aggressive treatment of acute pulmonary exacerbations with combination antibiotic therapy for two to three weeks has shown pronounced beneficial effects. The routine use of prophylactic antistaphylococcal antibiotics is still controversial. Although current pharmacologic treatment is symptomatic, new agents are being developed and studied that target the underlying defect in the CFTR protein. This review focuses on current pharmacologic management of pulmonary disease in patients with cystic fibrosis and the role of new agents emerging for the treatment of this disease.

Development of Pancreatic Function and Cystic Fibrosis

1965 ◽  
Vol 12 (3) ◽  
pp. 655-662
Author(s):  
Margaret E.D. O’Flynn ◽  
William F. Rowley
Keyword(s):  
Cystic Fibrosis ◽  
Pancreatic Function

Exocrine pancreatic function in Japanese patients with cystic fibrosis

Pancreatology ◽  
2016 ◽  
Vol 16 (4) ◽  
pp. S95
Author(s):  
Satoru Naruse ◽  
Shiho Kondo ◽  
Kotoyo Fujiki ◽  
Akiko Yamamoto ◽  
Miyuki Nakakuki ◽  
...  
Keyword(s):  
Cystic Fibrosis ◽  
Japanese Patients ◽  
Exocrine Pancreatic Function ◽  
Pancreatic Function

Pancreatic function in infants identified as having cystic fibrosis in a neonatal screening program

1990 ◽  
Vol 33 (1) ◽  
pp. 83-84
Author(s):  
D.L. Waters ◽  
S.F.A. Dorney ◽  
K.J. Gaskin ◽  
M.A. Gruca ◽  
M O'Halloran ◽  
...  
Keyword(s):  
Cystic Fibrosis ◽  
Neonatal Screening ◽  
Screening Program ◽  
Pancreatic Function ◽  
Neonatal Screening Program

Genetic analysis and pancreatic function in cystic fibrosis

The Lancet ◽  
1990 ◽  
Vol 335 (8705) ◽  
pp. 1601 ◽  
Author(s):  
Hinda Kopelman ◽  
Rima Rozen ◽  
Graziella Borgo ◽  
Gianni Mastella ◽  
Paolo Gasparini ◽  
...  
Keyword(s):  
Cystic Fibrosis ◽  
Genetic Analysis ◽  
Pancreatic Function

scholarly journals HAIR DEPIGMENTATION AND DERMATITIS – AN UNEXPECTED PRESENTATION OF CYSTIC FIBROSIS

2013 ◽  
Vol 16 (2) ◽  
pp. 81-83
Author(s):  
O. Milankov ◽  
R. Savic ◽  
J. Tosic
Keyword(s):  
Cystic Fibrosis ◽  
Female Patient ◽  
Pancreatic Enzyme ◽  
Vitamin Supplementation ◽  
Rare Presentation ◽  
Skin Changes ◽  
Presenting Symptoms ◽  
Brown Colour ◽  
Hair Depigmentation

ABSTRACT Hair depigmentation is very rare presentation of cystic fibrosis (CF). We present 3.5-month-old female patient who had dermatitis and depigmentation of hair and eye lashes, malnutrition, edema and anemia as presenting symptoms of CF. After pancreatic enzyme and vitamin supplementation therapy, skin changes gradually disappeared, and her hair regained its normal brown colour

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