Pediatric Cholestatic Liver Diseases-2025

.: PREFACE
ÖN SÖZ
PREFACE
Prof. Dr. Özlem DURMAZ
İstanbul Üniversitesi İstanbul Tıp Fakültesi, Çocuk Gastroenterolojisi, Hepatolojisi ve Beslenme BD, İstanbul, Türkiye
Article Language: TR
Kolestaz; karaciğer ve safra yollarını primer ya da sekonder olarak, makroskopik ya da moleküler düzeyde etkileyen hastalıklar sonucu ortaya çıkan bir klinik tablo, sinsi gelişen ve ciddi klinik sonuçları olan bir fizyopatolojik durumdur. Hepatobilyer sistem ve portal venöz sistem patolojileri sonucu ortaya çıkan diğer klinik senaryolar olan akut ya da kronik hepatit, akut karaciğer yetersizliği ve portal hipertansiyondan bağımsız olarak, klinikteki ilk başvuru bulgusu olduğunda primer kolestatik hastalıktan söz edilir. Ekstrahepatik biliyer atrezi, neonatal sklerozan kolanjit, intrahepatik safra yolu azlıkları ve ilerleyici ailevi intrahepatik kolestazlar bu grubun örnekleridir. Sekonder kolestaz ise iki farklı tabloda tanımlanabilir. Erken süt çocukluğunda üriner sistem enfeksiyonu, hipokortizolemi, hipotiroidi ile örneklenebilecek sekonder kolestaz tablosu, diğer sistemleri öncelikli olarak etkileyen bir patolojinin her zaman olmasa da bazı hastalarda diğer sistemlerle ilişkili klinik bulguların varlığından bağımsız olarak kolestaz kliniğiyle ortaya çıktığı durumlar şeklinde tanımlanabilir. Bu sekonder kolestaz grubunda karaciğerin diğer fonksiyonları ya etkilenmemiş ya da minimal etkilenmiştir. Çocukluk çağının tüm dönemlerinde daha sık karşılaşılan sekonder kolestaz tablosu ise doğrudan hepatobilyer hastalıklarla ilişkili diğer iki ana klinik senaryo olan akut hepatit ya da akut karaciğer yetersizliği tablosunda olan bir hastada, biyokimyasal kolestazın da eşlik ediyor olduğu durumları kapsar. Neonatal hemokramatoz, NBAS eksikliği gibi akut karaciğer yetersizliğine yol açan hastalıklarda eşlik eden kolestaz, bu grubun örnekleridir ve aslında bu hastalıkların kolestatik karaciğer hastalıkları başlığı altında değil akut karaciğer yetersizliği nedenleri arasında değerlendirilmeleri daha doğrudur.

Bilimsel ve teknolojik ilerlemelerin ortaya koyduğu çok sayıda yeni hastalık ve farklı tanı yönteminin varlığı, kolestazlı hastaların değerlendirilmesi sırasında kafa karışıklığına yol açarak gecikmelere neden olabilir. Kolestazın erken ve doğru yöntemlerle tanısı, hem gecikilmeden altta yatan etiyolojinin araştırılarak varsa özgün tedavinin uygulanmasıyla biliyer siroza progresyonu engelleyecek, hem de kolestaza bağlı malabsorpsiyon, parenteral K vitaminine yanıtlı koagülopati, tartı alamama gibi istenmeyen sonuçlara yönelik girişimlerle intrakraniyal kanama, büyüme gelişme geriliği gibi ağır komplikasyonları önleme olanağı verecektir. Bu yüzden bu konuda pratik noktalara vurgu yapan bir kaynağın faydalı olacağı düşünülmüştür.

Bu kitap hazırlanırken güncel bilgiler ışığında kolestazın ve pediatrik kolestatik karaciğer hastalıklarının temel patogenezinin anlaşılması ve primer kolestatik karaciğer hastalığı ya da sık görülen diğer sistem hastalıklarına eşlik eden sekonder kolestazı olan pediatrik hastaların, ülkemiz koşullarına uygun şekilde ve kanıta dayalı veriler ışığında gözden geçirilmesi amaçlanmıştır. Kavram karmaşasını önlemek ve temel klinik yaklaşımları anlaşılır kılabilmek amacıyla ve akut hepatit, akut karaciğer yetersizliği ya da portal hipertansiyon gibi diğer klinik senaryoların başvuru şekli olduğu hastalıklar ve hastalık gruplarına yer verilmemiştir. Temel bilgiler, genel yaklaşım ve farklı hastalık grupları, ayrı bölümlerde ele alınarak okuyucuya anlaşılır ve uygulamaya yönelik bir rehber sunulması hedeflenmiştir. Bölümlerin düzenlenmesinde henüz deneysel olan, kanıtlanmamış ya da ülkemizde halihazırda uygulama imkânı olmayan tanı ve tedavi yöntemlerinden ziyade, ülkemiz pratiğine yönelik bilgi ve yaklaşımlara öncelik verilmiştir. Güncel bilgileri deneyimleri ile harmanlayarak bölümlerin yazılmasında emek veren yazarlara ve sabırla destek veren Türkiye Klinikleri yayın ekibine teşekkür eder, faydalı bir kaynak olmasını dilerim.

Prof. Dr. Özlem DURMAZ
Editör
İstanbul Üniversitesi İstanbul Tıp Fakültesi, Çocuk Gastroenterolojisi, Hepatolojisi ve Beslenme BD, İstanbul, Türkiye
Cholestasis is a clinical condition resulting from diseases that affect the liver and bile ducts either primarily or secondarily, at the macroscopic or molecular level. It is a subtle and insidiously developing pathophysiological condition with severe clinical consequences. When cholestasis presents as the first clinical finding, independent of other clinical scenarios such as acute or chronic hepatitis, acute liver failure, or portal hypertension associated with hepatobiliary system and portal venous system pathologies, it is referred as primary cholestatic disease. Examples of this group include extrahepatic biliary atresia, neonatal sclerosing cholangitis, intrahepatic bile duct paucity, and progressive familial intrahepatic cholestasis.

Secondary cholestasis can be defined in two distinct patterns. Cholestasis associated with urinary tract infections, hypocortisolemia, or hypothyroidism in early infancy constitute typical examples for secondary cholestasis occuring as the initial clinical manifestation of a disease primarily affecting other body systems, even when no other system-related clinical findings are present. In this group, liver functions other than bile production may remain unaffected or only minimally effected. The other secondary cholestasis scenario more commonly encountered during childhood encompasses conditions where biochemical cholestasis coexists with acute hepatitis or acute liver failure, which are the two other main clinical scenarios associated with hepatobiliary diseases. Examples include neonatal hemochromatosis and NBAS deficiency which are diseases leading to acute liver failure with accompanying cholestasis. In fact, these conditions should be evaluated as causes of acute liver failure rather than classified under cholestatic liver diseases.

The advancement of scientific and technological progress, leading to discovery of various new diseases and development of a wide range of diagnostic methods, can cause confusion during the evaluation of cholestatic patients and result in delays. Early diagnosis of cholestasis, using the accurate diagnostic methods, will not only prevent progression to biliary cirrhosis by facilitating prompt investigation of the underlying etiology and starting disease specific treatments but will also provide an opportunity to avoid severe complications like intracranial bleeding and growth retardation by timely management of pathophyiological outcomes of cholestasis such as malabsorption, parenteral vitamin K-responsive coagulopathy, and failure to thrive. Therefore, a review emphasizing practical points in this field will be useful to guide the clinician.

This book aims to discuss the up to date concepts about the basic pathogenesis of cholestasis and pediatric cholestatic liver diseases and to review current management of pediatric patients with cholestatic liver disease either primary, or secondary to other systemic diseases, in a realistic manner considering the actual opportunities in our country and based on evidence-based data. To avoid confusion of concepts and to make fundamental clinical approaches more understandable, diseases and disease groups that represent other clinical scenarios such as acute hepatitis, acute liver failure, or portal hypertension have not been included. Basic information, general approaches, and different disease groups are addressed in separate sections, with the goal of providing the reader with a clear, practical guide. In organizing the sections, priority has been given to information and approaches relevant to clinical practice in our country, rather than experimental, unproven, or currently unavailable diagnostic and therapeutic methods. I would like to thank the authors for their efforts in writing the sections by blending current knowledge with their experience, and the support of the Türkiye Klinikleri publication team, and I hope this book will be a valuable resource in the management of pediatric cholestatic liver disease.

Prof. Dr. Özlem DURMAZ
Editor
İstanbul Üniversity İstanbul Faculty of Medicine, Department of Pediatric Gastroenterology, Hepatology and Nutrition, İstanbul, Türkiyr

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