Year: 2010 Month: 6 Volume: 45 Issue 2
Original Article
Year: 2010
Month: 6
Valume: 45
Issue 2
Viewed 400 times
Received
Accepted
Effect of meconium ileus on the clinical prognosis of patients with cystic fibrosis diagnosed at younger than 12 months - Original Article
Doi: 10.4274/tpa.45.105
Sevgi Pekcan;
Selçuk Üniversitesi Meram Tıp Fakültesi, Çocuk Göğüs Hastalıkları Bilim Dalı, Konya, Türkiye
Nural Kiper;
Hacettepe Üniversitesi Tıp Fakültesi, Çocuk Göğüs Hastalıkları Ünitesi, Ankara, Türkiye
Mehmet Köse;
Erciyes Üniversitesi Tıp Fakültesi, Çocuk Sağlığı ve Hastalıkları Anabilim Dalı, Kayseri, Türkiye
Ayşe Tana Aslan;
Gazi Üniversitesi Tıp Fakültesi, Çocuk Göğüs Hastalıkları Bilim Dalı, Ankara, Türkiye
Nazan Çobanoğlu;
Hacettepe Üniversitesi Tıp Fakültesi, Çocuk Göğüs Hastalıkları Ünitesi, Ankara, Türkiye
Ebru Yalçın;
Hacettepe Üniversitesi Tıp Fakültesi, Çocuk Göğüs Hastalıkları Ünitesi, Ankara, Türkiye
Deniz Doğru;
Hacettepe Üniversitesi Tıp Fakültesi, Çocuk Göğüs Hastalıkları Ünitesi, Ankara, Türkiye
Uğur Özçelik;
Hacettepe Üniversitesi Tıp Fakültesi, Çocuk Göğüs Hastalıkları Ünitesi, Ankara, Türkiye
Mailing Address
Sevgi Pekcan;
Selçuk Üniversitesi Meram Tıp Fakültesi, Çocuk Göğüs Hastalıkları Bilim Dalı, Konya, Türkiye
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  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. 
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’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

 

Full Text

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  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. 
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’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

 


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