Association Between Outcomes and Demographic Factors in an Azeri Turkish Population With Cystic Fibrosis: A Cross-Sectional Study in Iran From 2001 Through 2014

Vahedi, Leila; Jabarpoor-Bonyadi, Morteza; Ghojazadeh, Morteza; Hazrati, Hakimeh; Rafeey, Mandana

doi:10.5812/ircmj.29615

Cited by 10 publications

(8 citation statements)

References 38 publications

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“…The incidence of CF in Asians is rare and unknown; however, it is estimated to be 1 in 10,000 in the Middle East (such as Iran, Turkey, and Lebanon) 4 . Although numerous mutations in the CFTR gene are a primary cause on the clinical manifestations of CF, recent research has established that various gender, modifier genes, environmental factors, and socioeconomic factors can modify the development and severity of CF 3 5 6 7 8 .…”

Section: Introductionmentioning

confidence: 99%

Gender Differences in Clinical Presentations of Cystic Fibrosis Patients in Azeri Turkish Population

et al. 2016

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BackgroundCystic fibrosis (CF) is an autosomal recessive disorder with several clinical presentations. This study was undertaken in the Azeri Turkish population in Iran, to investigate gender differences in the age at onset and diagnosis, age of death, and duration of illness of CF.MethodsThe data of 331 CF patients from 2001 to 2015 was surveyed. Parameters including age, sex, ΔF508 mutation, age at onset, age at diagnosis, age of death and clinical presentations were evaluated for both sexes, using descriptive analysis. The association of gender with these variables was studied using logistic regression, chi-square test and Mann-Whitney U test by SPSS version 18. Odds ratio with a confidence interval of 95% and p≤0.05 was considered statistically significant.ResultsThe study included 191 males (57.7%) and 140 females (42.3%), all showing statistically significant difference (p<0.001). Age duration differed between genders. Male and female patients were further under 9 and 4 years, respectively. The occurrence of ΔF508 mutation was 0.51 times more in females than in males. Age, diagnosis and sex were closely associated: males were diagnosed at a significantly later age than females (p=0.05). While this compression performed based on clinical presentations, males with respiratory disease had a later median age at diagnosis than females at lifespan (p=0.001). The risk of infertility in males was approximately two times greater than in females (p=0.02).ConclusionThese findings indicate gender differences in CF patients. Future studies are needed to establish other differences and evaluate the causes for the gender variations.

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Section: Introductionmentioning

confidence: 99%

Gender Differences in Clinical Presentations of Cystic Fibrosis Patients in Azeri Turkish Population

et al. 2016

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“…Therefore genetic disease observed more commonly in these countries [26][27][28]. In our population, the rate of consanguinity marriage was approximately to 41%.…”

Section: Discussionmentioning

confidence: 56%

Genetic pattern of cystic fibrosis patients in Azeri Turkish population

Jabarpoor-Bonyadi¹,

Rafeey²,

Vahedi³

et al. 2017

Russ. Open Med. J.

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Abstract:Objective -This study was designed to analyze the genetic pattern of cystic fibrosis and effects on age, sex and mortality in the Azeri Turkish population in Iran. Material and Methods -This study was a descriptive study that was conducted for cystic fibrosis patients in Azeri Turkish population in Iran from 2001 to 2014. Of 331 patients, the spectrum of cystic fibrosis transmembrane conductance regulator (CFTR) mutations in 263 patients was reviewed. Demographic and genetic data of patients were summarized by descriptive analysis as frequency, percentage, mean and median. Results -The frequency consanguineous marriages was 196 (59.2%) positive and 135 (40.8%) negative with a significant difference (P=0.001). We identified 32 known mutations and 74 kinds of genotypes. The most common mutation and genotype were ∆F508 138 (26.2%) and ∆F508/ ∆F508 41 (15.5%), respectively. The most mortality rate had observed in ∆F508 genotypes. Conclusion -These findings indicate high frequency of consanguinity marriage in this area. A low frequency of the ∆F508 mutation and detection 32 mutations reflect a heterogeneous spectrum of the mutations in this ethnic group. Further examinations are necessary on CFTR gene and affect these items on on age, sex and mortality.

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“…Significant associations between serum albumin and protein with weight, height, and head circumference have been suggested [36]. In a study by Vahedi et al, it was stated that positive family history, a consanguineous marriage, and residence in a rural area increases the rate of mortality [37]. In addition, pseudomonas infection, number of previous admissions and the severity of pulmonary hypertension were mentioned as major determinants of mortality by Baghaie et al [38].…”

Section: Discussionmentioning

confidence: 99%

Clinical manifestation, laboratory findings, and outcome of children with cystic fibrosis over a 10-year period in South Iran

Ziyaee

Dehghani

Hosseini

et al. 2020

Egypt Pediatric Association Gaz

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Background Cystic fibrosis (CF) is a monogenic hereditary disease with diverse complications, which substantially reduce the quality of life and longevity of patients. With regard to the scarcity of information on epidemiological aspects of this disease in Iran, we aimed to examine the current status of pediatric CF patients in a tertiary referral center in south Iran. Results Medical records of 105 patients (51% female) with the mean age of 16.3 ± 34.4 months were reviewed. Twelve patients (13.3%) had siblings with CF. Failure to thrive and respiratory symptoms were the most common presentations. Elevated liver enzymes and steatorrhea were detected in over half of tested patients, while abnormal serum albumin level, urinalysis, positive urine cultures, and positive acute phase reactants were noticed in less than half of the patients. Mild increase in pulmonary artery blood pressure was the most common finding of echocardiography. Increased liver echogenicity was the top finding of abdominopelvic sonography. Out of 42 available patients, 19 (45.2%) died with the mean age of 54.4 ± 101.8 months. The highest rate of mortality was observed in the 2–6 years old age group (42.9%). Conclusion Increased liver echogenicity was the most common finding in hepatobiliary evaluation. Mild increase in pulmonary artery blood pressure was the most common finding of echocardiography.

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Association Between Outcomes and Demographic Factors in an Azeri Turkish Population With Cystic Fibrosis: A Cross-Sectional Study in Iran From 2001 Through 2014

Cited by 10 publications

References 38 publications

Gender Differences in Clinical Presentations of Cystic Fibrosis Patients in Azeri Turkish Population

Gender Differences in Clinical Presentations of Cystic Fibrosis Patients in Azeri Turkish Population

Genetic pattern of cystic fibrosis patients in Azeri Turkish population

Clinical manifestation, laboratory findings, and outcome of children with cystic fibrosis over a 10-year period in South Iran

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