Abstract
Objective: Cholestasis in childhood results from different etiologies according to age groups. The differences between clinical and
laboratory findings in patients with cholestasis during the first 3 months of age and after 3 months are unknown. The aim of this study is
to investigate the clinical, laboratory; and histopathological findings of patients with cholestasis according to age groups.
Material and Methods: Between January 2013-March 2017, files of the patients diagnosed as cholestasis in 0-18 year of age were
retrospectively reviewed. Demographic data, complaints, physical examination, laboratory, ultrasonography and if liver biopsy is done
histopathologic findings of the patients were recorded.
Results: Forty-four patients (29 M, 65%) were diagnosed as cholestasis during the study period. Twenty-five (56%) of the patients were
younger than 3 months (Group 1), 19 (44%) of the patients were older than 3 months (Group 2). The patients were diagnosed as cholestasis
in the first 3 months were idiopathic neonatal hepatitis (n=5, 20%), non-syndromic paucity of intrahepatic bile ducts (n=5, 20%), biliary
atresia (n=3, 12%), progressive familial intrahepatic cholestasis (n=3, 12%), cholestasis owing to prematurity/ sepsis/ parenteral nutrition
(n=3, 12%) respectively. Aetiology of the cholestasis after 3 months were cryptogenic hepatitis (n=5, 26.3%) and autoimmune hepatitis
(n=4, 21%) respectively. Mean duration of follow up was 17.7±15.1 months. Total and direct bilirubin levels were higher in Group 1 patients
(p=0.017 and p= 0.023 respectively) whereas albumin levels were lower (p=0.051). Ultrasonographic evaluation revealed statistically
homogenic liver parenchyma in Group 1 (p=0.000). Liver histopathologic examination showed fibrosis in Group 2 (p=0.000).
Conclusion: Cholestasis in childhood occurs different reasons. Although bilirubin levels were higher in young children, chronic changes such
as liver fibrosis are common in older children.
Keywords
References
- Referans1 Venigalla S, Gourley GR. Neonatal cholestasis. Seminars in perinatology. 2004; 28: 348-55.
- Referans2 Fawaz R, Baumann U, Ekong U, Fischler B, Hadzic N, Mack CL, et al. Guideline for the Evaluation of Cholestatic Jaundice in Infants: Joint Recommendations of the North American Society for Pediatric Gastroenterology, Hepatology, and Nutrition and the European Society for Pediatric Gastroenterology, Hepatology, and Nutrition. Journal of pediatric gastroenterology and nutrition. 2017; 64: 154-68.
- Referans3 Squires JE, McKiernan P. Molecular Mechanisms in Pediatric Cholestasis. Gastroenterology clinics of North America. 2018; 47: 921-37.
- Referans4 Lane E, Murray KF. Neonatal Cholestasis. Pediatric clinics of North America. 2017; 64: 621-39.
- Referans5 Pereira TN, Walsh MJ, Lewindon PJ, Ramm GA. Paediatric cholestatic liver disease: Diagnosis, assessment of disease progression and mechanisms of fibrogenesis. World journal of gastrointestinal pathophysiology. 2010; 1: 69-84.
- Referans6 Takcı Ş, Günbey C, Yurdakök M, Korkmaz A, Yiğit Ş. Çok düşük doğum ağırlıklı bebeklerde neonatal kolestaz. Çocuk Sağlığı ve Hastalıkları Dergisi. 2013; 56: 175-80.
- Referans7 Sokol RJ, Mack C, Narkewicz MR, Karrer FM. Pathogenesis and outcome of biliary atresia: current concepts. Journal of pediatric gastroenterology and nutrition. 2003; 37: 4-21.
- Referans8 Mieli-Vergani G, Howard ER, Mowat AP. Liver disease in infancy: a 20 year perspective. Gut. 1991; Suppl: S123-8.
- Referans9 Dilek Gürlek Gökçebay, Hacer Fulya Gülerman, Nilüfer Arda, Sevil Atavcı. Evaluation of Infants with Neonatal Cholestasis: Experience of a Tertiary Referral Center in Turkey. Turkiye Klinikleri J Med Sci 2015; 35: 218-24.
- Referans10 Çayır H, Altuntaş B, Ertan Ü. Neonatal kolestaz olgularında ekstrahepatik bilier hastalık ve hepatoselüler hastalıkların ayırıcı tanısı: 37 olgunun prospektif analizi. Turkiye Klinikleri Journal of Pediatrics. 1998; 7: 175-81.
- Referans11 Doğancı T, Acun C, Yaşar Z, Mısırlıoğlu E. Neonatal kolestazlı vakaların geç dönemde değerlendirmesi. Çocuk Sağlığı ve Hastalıkları Dergisi. 2002; 45: 216-21.
- Referans12 Kuloğlu Z, Ödek Ç, Kırsaçlıoğlu CT, Kansu A, Erden E, Girgin N, ve ark. Yenidoğan kolestazı olan 50 vakanın değerlendirilmesi. Çocuk Sağlığı ve Hastalıkları Dergisi. 2008; 51: 140-6.
- Referans13 Takagi K, Tanaka H, Nishijima S, Masaoka N, Miyake Y, Sakata H, et al. Fetal blood values by percutaneous umbilical blood sampling. Fetal therapy. 1989; 4: 152-60.
- Referans14 Kaysen GA. Biochemistry and biomarkers of inflamed patients: why look, what to assess. Clinical journal of the American Society of Nephrology : CJASN. 2009; 4 Suppl 1: S56-63.
- Referans15 Rockey DC, Friedman SL. Hepatic Fibrosis and Cirrhosis. In: Zakim and Boyer's Hepatology: A Textbook of Liver Disease. 6th ed. Philadelphia. Elsevier Inc. 2012. 64-85.
- Referans16 Suchy FJ. Approach to the infant with cholestasis. In: Suchy FJ, Sokol RJ, Balistreri WF, eds. Liver Disease in Children. 3rd Ed. New York: Cambridge University Press; 2007. 179-89.
Abstract
Amaç: Çocukluk çağında kolestaz yaş gruplarına göre farklı nedenlerle ortaya çıkabilmektedir. Yaşamın ilk 3 ayında ve 3 aydan sonra kolestaz tanısı alan hastaların klinik ve laboratuar bulguları arasındaki farklılıklar bilinmemektedir. Bu çalışmanın amacı kolestaz tanısıyla izlenen hastaların yaş gruplarına göre klinik, laboratuvar ve histopatolojik bulgularını incelemektir.
Gereç ve Yöntemler: Ocak 2013-Mart 2017 tarihleri arasında kolestaz tanısı alan 0-18 yaş arasındaki hastaların dosyaları geriye dönük olarak incelendi. Hastaların demografik verileri, yakınmaları, fizik muayene, laboratuar, ultrasonografi ve yapılmışsa karaciğer biyopsi bulguları kaydedildi.
Bulgular: Çalışma süresinde 44 hasta (29 E, %65.9) kolestaz tanısı almıştı. Hastaların 25’inin (%56) yaşı 3 aydan küçük (Grup 1), 19’unun (%44) 3 aydan büyüktü (Grup 2). İlk 3 ayda kolestaz tanısı alan hastalarda en sık nedenler sırasıyla idiopatik neonatal hepatit (n=5,%20), nonsendromik intrahepatik safra yolu azlığı (n=5, %20), bilier atrezi (n=3, %12), progresif familyal intrahepatik kolestaz (n=3, %12), prematürite/ sepsis/ total parenteral nutrisyona bağlı kolestaz (n=3, %12) iken, üç aylıktan büyük hastalarda en sık nedenler kriptojenik hepatit (n=5, %26.3) ve otoimmun hepatit (n=4, %21) idi. İzlem süresi ortalama 17.7±15.1 aydı. Grup 1'deki hastalarda total ve direkt bilirubin değerleri daha yüksek (sırasıyla p=0.017 ve p=0.023) iken albümin değeri daha düşüktü (p=0.051). Ultrasonografide karaciğer parankimi Grup 1'deki hastalarda anlamlı olarak normal saptandı (p=0.000). Karaciğer histopatolojik incelemesinde Grup 2'deki hastalarda fibrozis daha yüksekti (p=0.000).
Tartışma: Çocukluk çağında kolestaz yaş gruplarına göre farklı nedenlerle ortaya çıkmaktadır. Küçük çocuklarda bilirubin değerleri daha yüksek olsa da karaciğerde fibrozis gibi kronik değişiklikler büyük yaştaki çocuklarda daha sıktır.
Keywords
References
- Referans1 Venigalla S, Gourley GR. Neonatal cholestasis. Seminars in perinatology. 2004; 28: 348-55.
- Referans2 Fawaz R, Baumann U, Ekong U, Fischler B, Hadzic N, Mack CL, et al. Guideline for the Evaluation of Cholestatic Jaundice in Infants: Joint Recommendations of the North American Society for Pediatric Gastroenterology, Hepatology, and Nutrition and the European Society for Pediatric Gastroenterology, Hepatology, and Nutrition. Journal of pediatric gastroenterology and nutrition. 2017; 64: 154-68.
- Referans3 Squires JE, McKiernan P. Molecular Mechanisms in Pediatric Cholestasis. Gastroenterology clinics of North America. 2018; 47: 921-37.
- Referans4 Lane E, Murray KF. Neonatal Cholestasis. Pediatric clinics of North America. 2017; 64: 621-39.
- Referans5 Pereira TN, Walsh MJ, Lewindon PJ, Ramm GA. Paediatric cholestatic liver disease: Diagnosis, assessment of disease progression and mechanisms of fibrogenesis. World journal of gastrointestinal pathophysiology. 2010; 1: 69-84.
- Referans6 Takcı Ş, Günbey C, Yurdakök M, Korkmaz A, Yiğit Ş. Çok düşük doğum ağırlıklı bebeklerde neonatal kolestaz. Çocuk Sağlığı ve Hastalıkları Dergisi. 2013; 56: 175-80.
- Referans7 Sokol RJ, Mack C, Narkewicz MR, Karrer FM. Pathogenesis and outcome of biliary atresia: current concepts. Journal of pediatric gastroenterology and nutrition. 2003; 37: 4-21.
- Referans8 Mieli-Vergani G, Howard ER, Mowat AP. Liver disease in infancy: a 20 year perspective. Gut. 1991; Suppl: S123-8.
- Referans9 Dilek Gürlek Gökçebay, Hacer Fulya Gülerman, Nilüfer Arda, Sevil Atavcı. Evaluation of Infants with Neonatal Cholestasis: Experience of a Tertiary Referral Center in Turkey. Turkiye Klinikleri J Med Sci 2015; 35: 218-24.
- Referans10 Çayır H, Altuntaş B, Ertan Ü. Neonatal kolestaz olgularında ekstrahepatik bilier hastalık ve hepatoselüler hastalıkların ayırıcı tanısı: 37 olgunun prospektif analizi. Turkiye Klinikleri Journal of Pediatrics. 1998; 7: 175-81.
- Referans11 Doğancı T, Acun C, Yaşar Z, Mısırlıoğlu E. Neonatal kolestazlı vakaların geç dönemde değerlendirmesi. Çocuk Sağlığı ve Hastalıkları Dergisi. 2002; 45: 216-21.
- Referans12 Kuloğlu Z, Ödek Ç, Kırsaçlıoğlu CT, Kansu A, Erden E, Girgin N, ve ark. Yenidoğan kolestazı olan 50 vakanın değerlendirilmesi. Çocuk Sağlığı ve Hastalıkları Dergisi. 2008; 51: 140-6.
- Referans13 Takagi K, Tanaka H, Nishijima S, Masaoka N, Miyake Y, Sakata H, et al. Fetal blood values by percutaneous umbilical blood sampling. Fetal therapy. 1989; 4: 152-60.
- Referans14 Kaysen GA. Biochemistry and biomarkers of inflamed patients: why look, what to assess. Clinical journal of the American Society of Nephrology : CJASN. 2009; 4 Suppl 1: S56-63.
- Referans15 Rockey DC, Friedman SL. Hepatic Fibrosis and Cirrhosis. In: Zakim and Boyer's Hepatology: A Textbook of Liver Disease. 6th ed. Philadelphia. Elsevier Inc. 2012. 64-85.
- Referans16 Suchy FJ. Approach to the infant with cholestasis. In: Suchy FJ, Sokol RJ, Balistreri WF, eds. Liver Disease in Children. 3rd Ed. New York: Cambridge University Press; 2007. 179-89.
Details
| Primary Language | Turkish |
|---|---|
| Subjects | Internal Diseases |
| Journal Section | ORIGINAL ARTICLES |
| Authors | |
| Publication Date | September 29, 2020 |
| Submission Date | January 21, 2020 |
| Published in Issue | Year 2020 Volume: 14 Issue: 5 |
Cited By
Role of Liver Biopsy in the Diagnosis of Liver Diseases in Children
Journal of Ankara University Faculty of Medicine
https://doi.org/10.4274/atfm.galenos.2021.71676
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