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Characteristics of Patients with Cystic Fibrosis: Experience in a Large Referral Children’s Hospital in Tehran, Iran
BACKGROUND Cystic fibrosis (CF) is an autosomal recessive disease caused by a CF trans-membrane regulator (CFTR) defect. Its prevalence is 1:2500 in Caucasians, 1:15300 among African Americans and is rare in Southeast Asia. The present study aims to review demographic data, clinical manifestations a...
Autores principales: | , , , , |
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Formato: | Online Artículo Texto |
Lenguaje: | English |
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Iranian Association of Gastroerterology and Hepatology
2010
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Materias: | |
Acceso en línea: | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4154902/ https://www.ncbi.nlm.nih.gov/pubmed/25197508 |
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author | Khatami, GR Mir-Nasseri, MM Seyghali, F Allah-Verdi, B Yourdkhani, F |
author_facet | Khatami, GR Mir-Nasseri, MM Seyghali, F Allah-Verdi, B Yourdkhani, F |
author_sort | Khatami, GR |
collection | PubMed |
description | BACKGROUND Cystic fibrosis (CF) is an autosomal recessive disease caused by a CF trans-membrane regulator (CFTR) defect. Its prevalence is 1:2500 in Caucasians, 1:15300 among African Americans and is rare in Southeast Asia. The present study aims to review demographic data, clinical manifestations and laboratory findings of Iranian children diagnosed with CF who referred to a Children’s Hospital Medical Center in Tehran, Iran during a ten-year period. METHODS In a retrospective study from 1991-2000, all hospitalized patients with documented CF were reviewed. Diagnosis was based on clinical findings and sweat chloride levels above 60 mEq/L. RESULTS A total of 233 patients [females: 91 (39.1%), males: 142 (60.9%)] were enrolled. The onset of symptoms was before the first month of life in 12.1%, between 1-6 months of age in 75.1%, and between 6-12 months of age in 6.9% of patients. Consanguinity of parents was present in 42.5% of patients. Respiratory (81.5%) and gastrointestinal (73.4%) symptoms, in addition to growth retardation were the most common presentations Eighty-eight percent of patients weighted below the fifth percentile. Of the 207 chest radiographs performed, the most frequent finding was hyper-aeration associated with pneumonia. Among 138 patients in whom barium swallows were performed, 102 (74%) had gastroesophageal reflux. A total of 27 patients expired, mostly from respiratory failure (96.3%). CONCLUSION CF is not a rare disease in Iran. We suggest early diagnosis and appropriate maintenance therapy for improving morbidity and mortality amongst CF patients. |
format | Online Article Text |
id | pubmed-4154902 |
institution | National Center for Biotechnology Information |
language | English |
publishDate | 2010 |
publisher | Iranian Association of Gastroerterology and Hepatology |
record_format | MEDLINE/PubMed |
spelling | pubmed-41549022014-09-05 Characteristics of Patients with Cystic Fibrosis: Experience in a Large Referral Children’s Hospital in Tehran, Iran Khatami, GR Mir-Nasseri, MM Seyghali, F Allah-Verdi, B Yourdkhani, F Middle East J Dig Dis Original Article BACKGROUND Cystic fibrosis (CF) is an autosomal recessive disease caused by a CF trans-membrane regulator (CFTR) defect. Its prevalence is 1:2500 in Caucasians, 1:15300 among African Americans and is rare in Southeast Asia. The present study aims to review demographic data, clinical manifestations and laboratory findings of Iranian children diagnosed with CF who referred to a Children’s Hospital Medical Center in Tehran, Iran during a ten-year period. METHODS In a retrospective study from 1991-2000, all hospitalized patients with documented CF were reviewed. Diagnosis was based on clinical findings and sweat chloride levels above 60 mEq/L. RESULTS A total of 233 patients [females: 91 (39.1%), males: 142 (60.9%)] were enrolled. The onset of symptoms was before the first month of life in 12.1%, between 1-6 months of age in 75.1%, and between 6-12 months of age in 6.9% of patients. Consanguinity of parents was present in 42.5% of patients. Respiratory (81.5%) and gastrointestinal (73.4%) symptoms, in addition to growth retardation were the most common presentations Eighty-eight percent of patients weighted below the fifth percentile. Of the 207 chest radiographs performed, the most frequent finding was hyper-aeration associated with pneumonia. Among 138 patients in whom barium swallows were performed, 102 (74%) had gastroesophageal reflux. A total of 27 patients expired, mostly from respiratory failure (96.3%). CONCLUSION CF is not a rare disease in Iran. We suggest early diagnosis and appropriate maintenance therapy for improving morbidity and mortality amongst CF patients. Iranian Association of Gastroerterology and Hepatology 2010-01 /pmc/articles/PMC4154902/ /pubmed/25197508 Text en © 2010 by Middle East Journal of Digestive Diseases This work is published by Middle East Journal of Digestive Diseases as an open access article distributed under the terms of the Creative Commons Attribution License (http://creativecommons.org/licenses/by-nc-sa/4.0/). Non-commercial uses of the work are permitted, provided the original work is properly cited. |
spellingShingle | Original Article Khatami, GR Mir-Nasseri, MM Seyghali, F Allah-Verdi, B Yourdkhani, F Characteristics of Patients with Cystic Fibrosis: Experience in a Large Referral Children’s Hospital in Tehran, Iran |
title | Characteristics of Patients with Cystic Fibrosis: Experience in a Large Referral Children’s Hospital in Tehran, Iran |
title_full | Characteristics of Patients with Cystic Fibrosis: Experience in a Large Referral Children’s Hospital in Tehran, Iran |
title_fullStr | Characteristics of Patients with Cystic Fibrosis: Experience in a Large Referral Children’s Hospital in Tehran, Iran |
title_full_unstemmed | Characteristics of Patients with Cystic Fibrosis: Experience in a Large Referral Children’s Hospital in Tehran, Iran |
title_short | Characteristics of Patients with Cystic Fibrosis: Experience in a Large Referral Children’s Hospital in Tehran, Iran |
title_sort | characteristics of patients with cystic fibrosis: experience in a large referral children’s hospital in tehran, iran |
topic | Original Article |
url | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4154902/ https://www.ncbi.nlm.nih.gov/pubmed/25197508 |
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