Sevgi Pekcan, Nural Kiper, Mehmet Köse, Ayşe Tana Aslan, Nazan Çobanoğlu, Ebru Yalçın, Deniz Doğru, Uğur Özçelik

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

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

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

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

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