Effect of meconium ileus on the clinical prognosis of patients with cystic fibrosis diagnosed at younger than 12 months Original Article

Abstract

Summary Aim: Meconium ileus MI is an early clue to the diagnosis of cystic fibrosis CF and it occurs in about 15 20 of CF patients To determine the possible prognostic factors in patients with cystic fibrosis nbsp; with and without MI Material and Method: The sample consisted of 214 patients with CF diagnosed at younger than 12 months admitted to the pediatric pulmonology unit over a period of 18 years between January 1987 to December 2005 22 patients presented with MI MI group 192 patients had non MI nonMI group Demographic clinical nutritional and laboratory data were obtained nbsp; Results: There were 11 female 11 male patients in MI group 85 female and 107 male patients in nonMI group A statistically significant difference was not observed between the groups studied regarding the following variables: BMI Staphylococcus aureus and Pseudomonas aeruginosa colonisations mutation types respiratory functions assesed by FEV1 lung findings Pseudo Bartter rsquo;s syndrome and progression to bronchiectasis were detected higher in nonMI group statistically Conclusions: There is no prognostic difference between two groups when these patients are diagnosed early and followed up by a multidisciplinary action and a collective treatment approach The results of the present study suggest that meconium ileus is not an indication of a more severe phenotype of cystic fibrosis Turk Arch Ped 2010; 45: 105 10 Key words: Child cystic fibrosis meconium ileus

Keywords

• -

Mekonyum ileusunun 12 ay altında tanı alan kistik fibrozlu hastalarda klinik seyire etkisi Özgün Araştırma

Öz

Özet Amaç: Mekonyum ileusu kistik fibrozda 10 20 oranında görülür İlk tanı bulgularından biridir On iki ay altında tanı alan kistik fibrozlularda mekonyum ileusu varlığının seyire etkisini mekonyum ileusu olmayan grup ile karşılaştırmak Gereç ve Yöntem: Bu çalışmada Hacettepe Üniversitesi Tıp Fakültesi Çocuk Göğüs Hastalıkları Bölümünde 1987 2005 yılları arasında 12 ay altında mekonyum ileusu ile kistik fibroz tanısı alan 22 11K 11E hasta ile diğer nedenlerle tanı alan 192 85K 107E hastanın demografik klinik özellikleri kolonizasyonları solunum işlevleri hastaneye yatış sayıları mutasyon tipleri ve takip süreleri karşılaştırıldı Bulgular: Hastaların son vücut kitle indeksi yaş zorlu ekspirasyonun 1 saniyesinde atılan volüm FEV1 vücut ağırlığı ve boy persantilleri arasında istatistiksel olarak fark saptanmadı Staphylococcus aureus kolonizasyonu Pseudomonas aeruginosa kolonizasyonu mutasyon analizi arasında istatistiksel olarak fark saptanmazken bronşektazi gelişiminin ve psödo Bartter sendromu sıklığının mekonyum ileusu olmayan grupta istatistiksel olarak anlamlı olarak yüksek olduğu bulundu nbsp; Çıkarımlar: nbsp;Bu hastalar çok merkezli olarak ve ortak tedavi yaklaşımıyla tedavi edilip takip edildiğinde erken tanı alan mekonyum ileusu olan ve olmayan grup arasında önemli fark yaşanmamaktadır Mekonyum ileusu kistik fibrozda kötü seyir işareti değildir Türk Ped Arş 2010; 45: 105 10 Anahtar sözcükler: Çocuk kistik fibroz mekonyum ileusu

Anahtar Kelimeler

• Çocuk, kistik fibroz, mekonyum ileusu

Kaynakça

1. Keckler SJ, St Peter SD, Spilde TL, et al. Current significance of meconium plug syndrome. J Pediatr Surg 2008; 43: 896-8. (Abstract) / (Full Text) / (PDF)
2. Bettinelli ME, Civardi A, Costantini D, et al. Survival in cystic fibrosis: preliminary report from an Italian multicenter study. Acta Univ Carol Med 1990; 36: 215-6. (Abstract) / (PDF)
3. Wood RE, Boat TF, Doershuk CF. Cystic fibrosis. Am Rev Respir Dis 1976; 113: 833-78. (Abstract)
4. Kerem E, Corey M, Kerem B, Durie P, Tsui LC, Levison H. Clinical and genetic comparisons of patients with cystic fibrosis, with or without meconium ileus. J Pediatr 1989; 114: 767-73. (Abstract) / (PDF)
5. Li Z, Lai HJ, Kosorok MR, Laxova A, Rock MJ, Splaingard ML, Farrell PM. Longitudinal pulmonary status of cystic fibrosis chil- dren with meconium ileus. Pediatr Pulmonol 2004; 38: 277-84. (Abstract) / (PDF)
6. Hudson I, Phelan PD. Are sex, age at diagnosis, or mode of presentation prognostic factors for cystic fibrosis? Pediatr Pulmonol 1987; 3: 288-97. (Abstract) / (PDF)
7. The cystic fibrozis genotype-phenotype consortium. Correlation between genotype and phenotype in patients with cystic fibrosis. N Engl J Med 1993; 329: 1308-13. (Abstract) / (Full Text)
8. Feingold J, Guilloud-Bataille M. Genetic comparisons of patients with cystic fibrosis with or without meconium ileus. Clinical Centers of the French CF Registry. Ann Genet 1999; 42: 147-50. (Abstract) / (Full Text)

9. Wilcken B, Chalmers G. Reduced morbidity in cystic fibrosis. Lancet 1986; 22; 1: 439. (Full Text)
10. Waters DL, Wilcken B, Irwing L, et al. Clinical outcomes of newborn screening for cystic fibrosis. Arch Dis Child Fetal Neonatal Ed 1999; 80: 1-7. (Abstract) / (Full Text)
11. Wood BP. Cystic fibrosis. Radiology 1997; 204: 1-10. (Abstract) / (PDF)
12. Halliburton CS, Mannino DM, Olney RS. Cystic fibrosis deaths in the United States from 1979 through 1991. An analysis using multiple-cause mortality data. Arch Pediatr Adolesc Med 1996; 150: 1181-5. (Abstract) / (PDF)
13. Fuchs JR, Langer JC. Long-term outcome after neonatal meconium obstruction. Pediatrics 1998; 101: E7. (Abstract) / (Full Text) (PDF)
14. Mohon RT, Wagener JS, Abman SH, Seltzer WK, Accurso FJ. Relationship of genotype to early pulmonary function in infants with cystic fibrosis identified through neonatal screening. J Pediatr 1993; 122: 550-5. (Abstract) / (PDF)
15. Oliveira MCLA, Reis FJC, Monteiro APAF, Pena FJ. Effect of meconium ileus on the clinical prognosis of patients with cys- tic fibrosis. Braz J Med Biol Res 2002; 35:31-8. (Full Text)
16. Kerem B, Rommens JM, Buchanan JA, et al. Identification of the cystic fibrosis gene: genetic analysis. Science 1989; 245: 1073-80. (Full Text)
17. Zielenski J, Corey M, Rozmahel R, et al. Detection of a cystic fibrosis modifier locus for meconium ileus on human chromo- some 19q13. Nat Genet 1999; 22: 128-9. (Full Text)
18. Tepper RS, Hict P, Eigen H, Scoot P, Grosfeld J, Cohen M. Infants with cystic fibrosis: pulmonary function at diagnosis. Pediatr Pulmonol 1988; 5: 15-8. (Abstract) / (PDF)
19. Lai HC, Kosorok MR, Laxova A, Davis LA, FitzSimmon SC, Farrell PM. Nutritional status of patients with cystic fibrosis with meconium ileus: a comparison with patients without meconium ileus and diagnosed early through neonatal screen- ing. Pediatrics 2000; 105: 53-61. (Abstract) / (Full Text) / (PDF)
20. Munck A, Gérardin M, Alberti C, et al. Clinical outcome of cys- tic fibrosis presenting with or without meconium ileus: a matched cohort study. J Pediatr Surg 2006; 41: 1556-60. (Abstract) / (Full Text) / (PDF)
21. Evans AK, Fitzgerald DA, McKay KO. The impact of meconium ileus on the clinical course of children with cystic fibrosis. Eur Respir J 2001; 18: 784-9. (Abstract) / (Full Text)
22. McPartlin JF, Dickson JA, Swain VA. Meconium ileus: Immediate and long-term survival. Arch Dis Child 1972; 47: 317. (Full Text)
23. Fustik S, Pop-Jordanova N, Slaveska N, Koceva S, Efremov G. Metabolic alkalosis with hypoelectrolytemia in infants with cystic fibrosis. Pediatr Int 2002; 44: 289-92. (Abstract) / (Full Text) / (PDF)
24. Yalcin E, Kiper N, Dogru D, Ozcelik U, Aslan AT. Clinical fea- tures and treatment approaches in cystic fibrosis with pseudo- Bartter syndrome. Ann Trop Paediatr 2005; 25: 119-24. (Abstract)