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Journals / Türk Pediatri Arşivi / 2010 / Cilt: 45 - Sayı: 2

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

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

Sevgi PEKCAN
Nural KİPER
Uğur ÖZÇELİK
Deniz DOĞRU
Ayşe Tana ASLAN
Ebru YALÇIN
Nazan ÇOBANOĞLU
Mehmet KÖSE
Pages
105–110
Publication Date
2022-11-05
DOI
—
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AI Summary & Key Takeaways

AI Insight
⚡ TL;DR(Too Long; Didn't Read)

The results of the present study suggest that meconium ileus is not an indication of a more severe phenotype of cystic fibrosis.

📌 3 Key Takeaways

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Objective & Hypothesis: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.
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Key Findings & Data: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).
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Conclusion & Impact:The results of the present study suggest that meconium ileus is not an indication of a more severe phenotype of cystic fibrosis.
🔬Design:Research Article
📍Scope:2005 Verileri
⚙️Variables:Domain Parameters

Abstract

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.

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.Çıkarımlar: 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

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Cite This Article

PEKCAN, S., KİPER, N., ÖZÇELİK, U., DOĞRU, D., ASLAN, A. T., YALÇIN, E., ÇOBANOĞLU, N., KÖSE, M. (2022). Effect of meconium ileus on the clinical prognosis of patients with cystic fibrosis diagnosed at younger than 12 months. Türk Pediatri Arşivi, Cilt: 45 - Sayı: 2, 105–110. https://ajindex.com/articles/641546

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