Non-invasive Evaluation of Liver Involvement of Children with Cystic Fibrosis by Shear-Wave Elastography

Abstract

Objectives: Hepatobiliary complications commonly occur in cystic fibrosis with increasing prevalence due to longer life expectancies and widespread screening efforts. Shear-wave elastography is a novel noninvasive method that involves application of local mechanical compression on soft tissue using focused ultrasonography and acquiring strain images that show tissue response. We aimed to compare abdominal ultrasonography and Shear-wave elastography and also clinical and laboratory findings of children with cystic fibrosis prospectively. Methods: This study is a prospective study conducted in 13 cystic fibrosis patients followed between February 2018 and March 2019. The severity of cystic fibrosis-related liver disease was categorized according to international criteria. Elastography measurement was performed in the same session with the evaluation of the liver by abdominal ultrasonography in the patients. The liver stiffness measurements were compared with clinical data, biochemistry parameters and ultrasound findings. Results: Measurements were performed in 13 cystic fibrosis children (3 boys, 10 girls). The mean kiloPascal values of liver stiffness measurements with shear-wave elastography were 8.68 (4.1-20.2). The median liver stiffness measurement in subjects without cystic fibrosis-related liver disease was 7.79 kiloPascals (n=5); The median liver stiffness measurement in subjects with cystic fibrosis-related liver disease was 9.23 kiloPascals. Conclusion: Cystic fibrosis-related liver disease has a significantly varying disease burden, its prevalence is increasing, and its early recognition is crucial for treatment and follow-up. Although there are no clear range values determined for children in tissue stiffness measurements in Shear-wave elastography, clinical and other laboratory and imaging methods and follow-up and evaluation are important.

Keywords

• Cystic fibrosis
• liver
• children
• shear-wave elastography

Kistik Fibrozisli Çocuklarda Karaciğer Tutulumunun Shear-Wave Elastografi ile Non-invaziv Değerlendirilmesi

Abstract

Amaç: Kistik fibrozis dünyada beyaz ırkın en sık görülen genetik hastalığıdır. Kistik fibroziste ortalama yaşam beklentisiarttığından komplikasyonlar ve yönetimi daha önem kazanmıştır. Shear-wave elastografi, odaklanmış ultrasonografikullanılarak yumuşak doku üzerinde lokal mekanik kompresyon uygulanmasını ve doku tepkisini gösteren gerinim elde edilmesini içeren yeni, invaziv olmayan bir yöntemdir. Bu çalışmada kistik fibrozisli çocukların abdominal ultrasonografive Shear-wave elastografi ile klinik ve laboratuvar bulgularını prospektif olarak karşılaştırmayı amaçladık. Gereç ve Yöntemler: Bu çalışma, Şubat 2018 ile Mart 2019 tarihleri arasında takip edilen on üç kistik fibrozis hastasında gerçekleştirilenprospektif bir çalışmadır. Kistik fibrozis ile ilişkili karaciğer hastalığının şiddeti uluslararası kriterlere göre kategorize edildi. Hastalarda karınultrasonografisi ile karaciğerin değerlendirilmesi ile aynı seansta elastografi ölçümü yapıldı. Bulgular: Toplam on üç kistik fibrozisli çocuk değerlendirildi. Shear-wave elastografi ile karaciğer sertliği ölçümlerinin median değeri 6.36(IQR 5.40-10.80) kiloPaskal’dı. Kistik fibrozise bağlı karaciğer hastalığı olmayan deneklerde medyan karaciğer sertliği ölçümü 6.30 (IQR 5.26-16.18) kiloPaskal (n=5)’di; Kistik fibrozis ilişkili karaciğer hastalığı olan kişilerde medyan karaciğer sertliği ölçümü 6.46 (IQR 5.43-10.80) kiloPaskal olmuştur. kiloPascal değerleri ile yaş, cinsiyet, AST, ALT, HbA1c değerleri arasında anlamlı bir ilişki bulunmazken, kistik fibrozis ilişkili karaciğer hastalığı ile hemoglobin A1c ve ALT değerleri arasında güçlü bir pozitif korelasyon bulundu (sırasıyla r=0.702, p=0.007; r=0.761, p=0.003). Sonuç: Kistik fibrozis ile ilişkili karaciğer hastalığı, önemli ölçüde değişen bir hastalık yüküne sahiptir, prevalansı artmaktadır ve erken tanınması tedavi ve takip için çok önemlidir. Shear-wave elastografide doku sertliği ölçümlerinde çocuklar için belirlenmiş net bir aralık değerleri olmamakla birlikte klinik ve diğer laboratuvar ve görüntüleme yöntemleri ile takip ve değerlendirme önemlidir.

Keywords

• Kistik fibrozis
• Çocuklar
• Karaciğer
• Shear-wave elastografi

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