Tuberc Respir Dis.  2016 Oct;79(4):267-273. 10.4046/trd.2016.79.4.267.

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

• Vahedi L
• Jabarpoor-Bonyadi M
• Ghojazadeh M
• Vahedi A
• Rafeey M

• KMID: 2365318
• DOI: http://doi.org/10.4046/trd.2016.79.4.267

Abstract

BACKGROUND
Cystic 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.
METHODS
The 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.
RESULTS
The 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).
CONCLUSION
These findings indicate gender differences in CF patients. Future studies are needed to establish other differences and evaluate the causes for the gender variations.

Keyword

Cystic Fibrosis; Population; Age at Onset; Iran

MeSH Terms

Age of Onset
Cystic Fibrosis*
Diagnosis
Female
Humans
Infertility
Iran
Logistic Models
Male
Odds Ratio

Figure

• 

  Figure 1 Age distribution is shown among cystic fibrosis (CF) patients alive in males and females.

• 

  Figure 2 Gender difference for age at onset, age at diagnosis, age (living patients), age (deceased patients), and duration of illness in cystic fibrosis patients.

Reference

1. Ernst MM, Johnson MC, Stark LJ. Developmental and psychosocial issues in cystic fibrosis. Child Adolesc Psychiatr Clin N Am. 2010; 19:263–283.

2. Haworth CS, Selby PL, Horrocks AW, Mawer EB, Adams JE, Webb AK. A prospective study of change in bone mineral density over one year in adults with cystic fibrosis. Thorax. 2002; 57:719–723.

3. Sorde R, Pahissa A, Rello J. Management of refractory Pseudomonas aeruginosa infection in cystic fibrosis. Infect Drug Resist. 2011; 4:31–41.

4. Salvatore D, Buzzetti R, Baldo E, Forneris MP, Lucidi V, Manunza D, et al. An overview of international literature from cystic fibrosis registries. Part 3. Disease incidence, genotype/phenotype correlation, microbiology, pregnancy, clinical complications, lung transplantation, and miscellanea. J Cyst Fibros. 2011; 10:71–85.

5. Castellani C, Cuppens H, Macek M Jr, Cassiman JJ, Kerem E, Durie P, et al. Consensus on the use and interpretation of cystic fibrosis mutation analysis in clinical practice. J Cyst Fibros. 2008; 7:179–196.

6. Luciani A, Villella VR, Esposito S, Gavina M, Russo I, Silano M, et al. Targeting autophagy as a novel strategy for facilitating the therapeutic action of potentiators on DeltaF508 cystic fibrosis transmembrane conductance regulator. Autophagy. 2012; 8:1657–1672.

7. Rosenfeld M, Davis R, FitzSimmons S, Pepe M, Ramsey B. Gender gap in cystic fibrosis mortality. Am J Epidemiol. 1997; 145:794–803.

8. Vahedi L, Jabarpoor-Bonyadi M, Ghojazadeh M, Hazrati H, Rafeey M. Association between outcomes and demographic factors in an Azeri Turkish population with cystic fibrosis: a cross-sectional study in Iran from 2001 through 2014. Iran Red Crescent Med J. 2016; 18:e29615.

9. Harris H, Scotcher D, Hartley N, Wallace A, Craufurd D, Harris R. Cystic fibrosis carrier testing in early pregnancy by general practitioners. BMJ. 1993; 306:1580–1583.

10. Verma N, Bush A, Buchdahl R. Is there still a gender gap in cystic fibrosis? Chest. 2005; 128:2824–2834.

11. Abbott J, Morton AM, Musson H, Conway SP, Etherington C, Gee L, et al. Nutritional status, perceived body image and eating behaviours in adults with cystic fibrosis. Clin Nutr. 2007; 26:91–99.

12. Groeneveld IF, Sosa ES, Perez M, Fiuza-Luces C, Gonzalez-Saiz L, Gallardo C, et al. Health-related quality of life of Spanish children with cystic fibrosis. Qual Life Res. 2012; 21:1837–1845.

13. Schmidt A, Wenninger K, Niemann N, Wahn U, Staab D. Health-related quality of life in children with cystic fibrosis: validation of the German CFQ-R. Health Qual Life Outcomes. 2009; 7:97.

14. Callaghan BD, Hoo AF, Dinwiddie R, Balfour-Lynn IM, Carr SB. Growth and lung function in Asian patients with cystic fibrosis. Arch Dis Child. 2005; 90:1029–1032.

15. Olesen HV, Pressler T, Hjelte L, Mared L, Lindblad A, Knudsen PK, et al. Gender differences in the Scandinavian cystic fibrosis population. Pediatr Pulmonol. 2010; 45:959–965.

16. Havermans T, Colpaert K, Vanharen L, Dupont LJ. Health related quality of life in cystic fibrosis: to work or not to work? J Cyst Fibros. 2009; 8:218–223.

17. McCormick J, Mehta G, Olesen HV, Viviani L, Macek M Jr, Mehta A, et al. Comparative demographics of the European cystic fibrosis population: a cross-sectional database analysis. Lancet. 2010; 375:1007–1013.

18. Arias Llorente RP, Bousono Garcia C, Diaz Martin JJ. Treatment compliance in children and adults with cystic fibrosis. J Cyst Fibros. 2008; 7:359–367.

19. Bonyadi M, Omrani O, Rafeey M, Bilan N. Spectrum of CFTR gene mutations in Iranian Azeri Turkish patients with cystic fibrosis. Genet Test Mol Biomarkers. 2011; 15:89–92.

20. Farrell PM, Rosenstein BJ, White TB, Accurso FJ, Castellani C, Cutting GR, et al. Guidelines for diagnosis of cystic fibrosis in newborns through older adults: Cystic Fibrosis Foundation consensus report. J Pediatr. 2008; 153:S4–S14.

21. De Boeck K, Bulteel V, Tiddens H, Wagner T, Fajac I, Conway S, et al. Guideline on the design and conduct of cystic fibrosis clinical trials: the European Cystic Fibrosis Society-Clinical Trials Network (ECFS-CTN). J Cyst Fibros. 2011; 10:Suppl 2. S67–S74.

22. Jackson A, Foley L, Daly L, Fitzpatrick P, Harrington M, Zhou S, et al. Delayed cystic fibrosis presentation in children in the absence of newborn screening. Ir Med J. 2010; 103:113–116.

23. Steinraths M, Vallance HD, Davidson AG. Delays in diagnosing cystic fibrosis: can we find ways to diagnose it earlier? Can Fam Physician. 2008; 54:877–883.

24. Corey M, McLaughlin FJ, Williams M, Levison H. A comparison of survival, growth, and pulmonary function in patients with cystic fibrosis in Boston and Toronto. J Clin Epidemiol. 1988; 41:583–591.

25. Farrell P, Joffe S, Foley L, Canny GJ, Mayne P, Rosenberg M. Diagnosis of cystic fibrosis in the Republic of Ireland: epidemiology and costs. Ir Med J. 2007; 100:557–560.

26. Sweezey NB, Ratjen F. The cystic fibrosis gender gap: potential roles of estrogen. Pediatr Pulmonol. 2014; 49:309–317.

27. Gee L, Abbott J, Hart A, Conway SP, Etherington C, Webb AK. Associations between clinical variables and quality of life in adults with cystic fibrosis. J Cyst Fibros. 2005; 4:59–66.

28. Dodge JA, Lewis PA, Stanton M, Wilsher J. Cystic fibrosis mortality and survival in the UK: 1947-2003. Eur Respir J. 2007; 29:522–526.

29. Davis PB. The gender gap in cystic fibrosis survival. J Gend Specif Med. 1999; 2:47–51.

30. Rodman DM, Polis JM, Heltshe SL, Sontag MK, Chacon C, Rodman RV, et al. Late diagnosis defines a unique population of long-term survivors of cystic fibrosis. Am J Respir Crit Care Med. 2005; 171:621–626.

31. Li Y, Zhang X, Wang C, Hu Y, Niu X, Pei D, et al. Characterization by phenotypic and genotypic methods of metallo-beta-lactamase-producing Pseudomonas aeruginosa isolated from patients with cystic fibrosis. Mol Med Rep. 2015; 11:494–498.