prevalence of pancreatic insufficiency & ftt in cystic fibrosis

نویسندگان

مهری نجفی

m najafi

چکیده

cystic fibrosis (cf) is an inherited disease that affects multiple organ systems. it is the most common cause of severe progressive lung disease and exocrine panceratic insufficiency. in our investigation 67 patients had cf. of these, 79% had panceratic insufficiency and 92% had lung disease under 2 years age. also 67% of patients were < 2 percentile and 28% between 3-10 percentile weight for age. unfortunately 27% of them died because of respiratory problems

برای دانلود باید عضویت طلایی داشته باشید

برای دانلود متن کامل این مقاله و بیش از 32 میلیون مقاله دیگر ابتدا ثبت نام کنید

اگر عضو سایت هستید لطفا وارد حساب کاربری خود شوید

منابع مشابه

Prevalence of Cystic Fibrosis Trans-membrane Conductance Regulator Gene common mutations in children with cystic fibrosis in Isfahan, Iran

Background: Cystic fibrosis (CF) is the most common lethal genetic disorder of Cystic Fibrosis Trans-membrane Conductance (CFTR) Regulator gene mutations. We aimed to investigate common mutations in CF patients and to assess its possible relationship with clinical presentations. Materials and Methods: This cross sectional study was conducted on 36 CF patients who were referred to a tertiary ped...

متن کامل

Immunoreactive pancreatic Reg protein in sera from cystic fibrosis patients with and without pancreatic insufficiency.

BACKGROUND The biological function of the Reg protein, a non-enzymic protein produced in fairly large amounts by pancreatic acinar cells, remains elusive. Its susceptibility to proteolysis leading to precipitation of the proteolysis product at neutral pH suggests that it could contribute to the protein plugging observed in cystic fibrosis (CF). AIMS To study its behaviour in the serum of CF p...

متن کامل

Pancreatic cystosis in cystic fibrosis.

To cite: Burt H, Andronikou S, LangtonHewer S. BMJ Case Rep Published online: [please include Day Month Year] doi:10.1136/bcr-2015214288 DESCRIPTION A 17-year-old boy was diagnosed with cystic fibrosis at age 6 years. Genetic analysis revealed homozygous ΔF508 mutations. His first abdominal ultrasound was performed in March 2012 as part of the annual cystic fibrosis review. This demonstrated an...

متن کامل

Diagnosis of exocrine pancreatic insufficiency in cystic fibrosis by use of fluorescein dilaurate test.

In the fluorescein dilaurate test fluorescein dilaurate is cleaved by the pancreas specific cholesterol ester hydrolase activity and the liberated fluorescein is absorbed and excreted in the urine. Fluorescein recovery is a reflection of exocrine pancreatic function. The test was evaluated in 14 patients with cystic fibrosis and 16 healthy volunteers. The test was well tolerated by patients, wa...

متن کامل

Diagnosis of exocrine pancreatic insufficiency in cystic fibrosis by use of fluorescein dilaurate test

Professor Cuschieri and colleagues suggest that use of fluorescein dilaurate in an oral pancreatic function test may, by detecting different levels of pancreatic function, differentiate between patients with cystic fibrosis who do not require exogenous enzyme supplementation and those who do.' The authors also propose the fluorescein dilaurate test as a screening test for pancreatic insufficien...

متن کامل

Diagnosis of exocrine pancreatic insufficiency in cystic fibrosis by use of fluorescein dilaurate test

Professor Cuschieri and colleagues suggest that use of fluorescein dilaurate in an oral pancreatic function test may, by detecting different levels of pancreatic function, differentiate between patients with cystic fibrosis who do not require exogenous enzyme supplementation and those who do.' The authors also propose the fluorescein dilaurate test as a screening test for pancreatic insufficien...

متن کامل

منابع من

با ذخیره ی این منبع در منابع من، دسترسی به آن را برای استفاده های بعدی آسان تر کنید


عنوان ژورنال:
مجله دانشکده پزشکی دانشگاه علوم پزشکی تهران

جلد ۵۶، شماره ۱، صفحات ۵۸-۶۱

کلمات کلیدی

میزبانی شده توسط پلتفرم ابری doprax.com

copyright © 2015-2023