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Kolestaz ve Kaşıntı: Progresif Familyal İntrahepatik Kolestaz?

Yıl 2016, Cilt: 14 Sayı: 2, 88 - 95, 01.09.2016

Gökhan Tümgör

https://doi.org/10.4274/jcp.05706

Öz

Progresif familyal intrahepatik kolestaz PFİK , çocukluk çağı kolestazları içinde önemli bir yer tutmaktadır. Bazı karaciğer nakil merkezlerinde en sık karşılaşılan ikinci karaciğer nakil endikasyonudur. Tüm kolestazlı ve karaciğer nakli yapılan olguların yaklaşık %10-15’ini oluşturmaktadır. PFİK, otozomal resesif geçişlidir ve hepatosellüler kaynaklı kolestaza yol açmaktadır. Süt çocukluğundan erişkin döneme kadar olan dönemde karaciğer yetmezliğine yol açarak ölüme neden olabilmektedir. PFİK’nin üç tipi vardır ve tüm tiplerde en sık gözlenen klinik bulgular kaşıntı, sarılık, büyüme geriliği, hepatomegali, splenomegalidir. PFİK’nin üç tipinin de prognozu farklıdır. PFİK’nin komplikasyonları portal hipertansiyon, karaciğer yetmezliği, siroz, hepatosellüler karsinom ve ekstrahepatik bulgulardır. Olguların hayat kalitesini yükseltecek medikal ve cerrahi tedaviler uygulanmakla birlikte çoğu olguya erken dönemde karaciğer nakli gerekmektedir

Anahtar Kelimeler

Çocukluk çağı kolestaz kaşıntı

Kaynakça

  • 1. Clayton RJ, Iber FL, Ruebner BH, McKusick VA. Byler disease: Fatal familial intrahepatic cholestasis in an Amish kindred. Am J Dis Child 1969;117:112-24.
  • 2. Jankowska I, Socha P. Progressive familial intrahepatic cholestasis and inborn errors of bile acid synthesis. Clin Res Hepatol Gastroenterol 2012;36:271-4.
  • 3. Morotti RA, Suchy FJ, Magid MS. Progressive familial intrahepatic cholestasis (PFIC) type 1, 2, and 3: a review of the liver pathology findings. Semin Liver Dis 2011;31:3-10.
  • 4. Aykut A, Çakır M, Onay H, Nart D, Arıkan Ç, Özgenç F, ve ark. Klinik olarak ilerleyici ailevi intrahepatik kolestaz ile uyumlu hastalarda ATP8B1 mutasyonunun araştırılması: Ön çalışma. J Pediat Res 2014;1:138-41
  • 5. Davit Spraul A, Gonzales E, Baussan C, Jacquemin E. Progressive familial intrahepatic cholestasis. Orphanet J Rare Dis 2009;4:1.
  • 6. Stapelbroek JM, van Erpecum KJ, Klomp LW, Houwen RH. Liver disease associated with canalicular transport defects: current and future therapies. J Hepatol 2010;52:258-71.
  • 7. Davit- Spraul A, Gonzales E, Baussan C, Jacquemin E. The spectrum of liver diseases related to ABCB4 gene mutations: pathophysiology and clinical aspects. Semin Liv Dis 2010;30:134-46.
  • 8. Klomp LW, Vargas JC, Van Mil SW, Pawlikowska L, Strautnieks SS, Van Eijk MJ, et al. Characterization of mutations in ATP8B1 associated with hereditary cholestasis. Hepatology 2004;40:27- 38.
  • 9. Paulusma CC, Groen A, Kunne C, Ho- Mok KS, Spijkerboer AL, Rudi de Waart D, et al. Atp8b1 deficiency in mice reduces resistance of the canalicular membrane to hydrophobic bile salts and impairs bile salt transport. Hepatology 2006;44:195-204.
  • 10. Verhulst PM, van der Velden LM, Oorschot V, van Faassen EE, Klumperman J, Houwen RH, et al. A flippase- independent function of ATP8B1, the protein affected in familial intrahepatic cholestasis type 1, is required for apical protein expression and microvillus formation in polarized epithelial cells. Hepatology 2010;51:2049-60.
  • 11. Groen A, Romero MR, Kunne C, Hoosdally SJ, Dixon PH, Wooding C, et al. Complementary functions of the flippase ATP8B1 and the floppase ABCB4 in maintaining canalicular membrane integrity. Gastroenterology 2011;141:1927-37.
  • 12. Chen F, Ananthanarayanan M, Emre S, Neimark E, Bull LN, Knisely AS, et al. Progressive familial intrahepatic cholestasis, type 1, is associated with decreased farnesoid X receptor activity. Gastroenterology 2004;126:756-64.
  • 13. Alvarez L, Jara P, Sánchez- Sabaté E, Hierro L, Larrauri J, Díaz MC, et al. Reduced hepatic expression of farnesoid X receptor in hereditary cholestasis associated to mutation in ATP8B1. Hum Mol Genet 2004;13:2451-60.
  • 14. Egawa H, Yorifuji T, Sumazaki R, Kimura A, Hasegawa M, Tanaka K. Intractable diarrhea after liver transplantation for Byler’s disease: successful treatment with bile adsorptive resin. Liver Transplant 2002;8:714-6.
  • 15. Jacquemin E, Malan V, Rio M, Davit- Spraul A, Cohen J, Landrieu P, et al. Heterozygous FIC1 deciency: a new genetic predisposition to transient neonatal cholestasis. J Pediatr Gastroenterol Nutr 2010;50:447-9.
  • 16. Van Mil SW, Van den Woerd WL, Van den Brugge G, Sturm E, Jansen PLM, Bull LN, et al. Benign recurrent intrahepatic cholestasis type2 is caused by mutations in ABCB11. Gastroenterology 2004;127:379-84.
  • 17. Pauli- Magnus C, Lang T, Meier Y, Zodan- Marin T, Jung D, Breymann C, et al. Sequence analysis of bile salt export pump (ABCB11) and multidrug resistance p- glycoprotein 3 (ABCB4, MDR3) in patients with intrahepatic cholestasis of pregnancy. Pharmacogenetics 2004;14:91-102.
  • 18. Lang C, Meier Y, Stieger B, Ulrich B, Lang T, Kerb R, et al. Mutations and polymorphisms in the bile salt export pump and the multidrug resistance protein 3 associated with drug-induced liver injury. Pharmacogenet Genomics 2007;17:47-60.
  • 19. Ziol M, Barbu V, Rosmorduc O, Frassati- Biaggi A, Barget N, Hermelin B, et al. ABCB4 heterozygous gene mutations associated with cholestatic liver disease in adults. Gastroenterology 2008;135:131-41.
  • 20. Gonzales E, Gerhardt MF, Fabre M, Setchell KD, Davit- Spraul A, Vincent I, et al. Oral cholic acid for hereditary defects of primary bile acid synthesis: a safe and effective long- term therapy. Gastroenterology 2009;137:1310-20.
  • 21. Houdayer C, Dehainault C, Mattler C, Michaux D, Caux￾Moncoutier V, Pages- Berhouet S, et al. Evaluation of in silico tools for decision making in molecular diagnosis. Human Mut 2008;29:975-82.
  • 22. Liu C, Aronow BJ, Jegga AG, Wang N, Miethke A, Mourya R, et al. Novel resequencing chip customized to diagnose mutations in patients with inherited syndromes of intrahepatic cholestasis. Gastroenterology 2007;132:119-26.
  • 23. Chong CP, Mills PB, McClean P, Gissen P, Bruce C, Stahlschmidt J, et al. Bile acid- CoA ligase deficiency: a new inborn error of bile acid metabolism. J Inherit Metab Dis 2012;35:521-30.
  • 24. Cullinane AR, Straatman- Iwanowska A, Zaucker A, Wakabayashi Y, Bruce CK, Luo G, et al. Mutations in VIPAR cause an arthrogryposis, renal dysfunction and cholestasis syndrome phenotype with defects in epithelial polarization. Nat Genet 2010;42:303-12.
  • 25. Grosse B, Cassio D, Yousef N, Bernardo C, Jacquemin E, Gonzales E. Claudin-1 involved in neonatal ichthyosis sclerosing cholangitis syndrome regulates hepatic paracellular permeability. Hepatology 2012;55:1249-59.
  • 26. Lazaridis KN, Gores GJ, Lindor KD. Ursodeoxycholic acid mechanisms of action and clinical use in hepatobiliary disorders. J Hepatol 2001;35:134-46.
  • 27. Jacquemin E, Hermans D, Myara A, Habes D, Debray D, Hadchouel M, et al. Ursodeoxycholic acid therapy in pediatric patients with progressive familial intrahepatic cholestasis. Hepatology 1997;25:519-23.
  • 28. Feranchak AP, Ramirez RO, Sokol RJ. Medical and nutritional management of cholestasis. In: Suchy FJ, Sokol RJ, Balistreri WF (eds). Liver Disease in Children, Philadelphia: Lippincott Williams & Wilins; 2001. p.195-238.
  • 29. Pawlikowska L, Strautnieks S, Jankowska I, Czubkowski P, Emerick K, Antoniu A, et al. Differences in presentation and progression between severe FIC1 and BSEP deficiencies. J Hepatol 2010;53:170-8.
  • 30. Davit-Spraul A, Fabre M, Branchereau S, Baussan C, Gonzales E, Stieger B, et al. ATP8B1 and ABCB11 analysis in 62 children with normal gamma-glutamyl transferase progressive familial intrahepatic cholestasis (PFIC): phenotypic differences between PFIC1 and PFIC2 and natural history. Hepatology 2010;51:1645-55.
  • 31. Arnell H, Papadogiannakis N, Zemack H, Knisely AS, Nemeth A, Fischler B. Follow-up in children with progressive familial intrahepatic cholestasis after partial external biliary diversion. J Pediatr Gastroenterol Nutr 2010;51:494-9.
  • 32. Kalicin´ski PJ, Ismail H, Jankowska I, Kaminski A, Pawlowska J, Drewniak T, et al. Surgical treatment of progressive familial intrahepatic cholestasis: comparison of partial external biliary diversion and ileal bypass. Eur J Pediatr Surg 2003;13:307-11.
  • 33. Whitington PF, Whitngton GL. Partial external diversion of bile for the treatment of intractable pruritus associated with intrahepatic cholestasis. Gastroenterology 1988;95:130-6.
  • 34. Bustorff- Silva J, Sbraggia Neto L, Olímpio H, de Alcantara RV, Matsushima E, De Tommaso AM, et al. Partial internal biliary diversion through a cholecystojejunocolonic anastomosis: a novel surgical approach for patients with progressive familial intrahepatic cholestasis: a preliminary report. J Pediatr Surg 2007;42:1337-40.
  • 35. Gün F, Erginel B, Durmaz O, Sökücü S, Salman T, Celik A. An outstanding non-transplant surgical intervention in progressive familial intrahepatic cholestasis: partial internal biliary diversion. Pediatr Surg Int 2010;26:831-4.
  • 36. Jankowska I, Czubkowski P, Kalicinski P, Ismail H, Kowalski A, Pawlowska J, et al. Ileal exclusion in children with progressive familial intrahepatic cholestasis. J Pediatr Gastroenterol Nutr 2014;58:92-5.
  • 37. Stapelbroek JM, van Erpecum KJ, Klomp LW, Venneman NG, Schwartz TP, van Berge Henegouwen GP, et al. Nasobiliary drainage induces long- lasting remission in benign recurrent intrahepatic cholestasis. Hepatology 2006;43:51-3.
  • 38. Aydogdu S, Cakir M, Arikan C, Tumgor G, Yuksekkaya HA, Yilmaz F, et al. Liver transplantation for progressive familial intrahepatic cholestasis: clinical and histopathological findings, outcome and impact on growth. Pediatr Transplant 2007;11:634-40.
  • 39. De Vree JM, Ottenhoff R, Bosma PJ, Smith AJ, Aten J, Oude Elferink RP. Correction of liver disease by hepatocyte transplantation in a mouse model of progressive familial intrahepatic cholestasis. Gastroenterology 2000;119:1720-30.
  • 40. Boyer JL. Nuclear receptor ligands: rational and effective therapy for chronic cholestatic liver disease Gastroenterology 2005;129:735-40.
  • 41. Knisely AS, Strautnieks SS, Meier Y, Stieger B, Byrne JA, Portmann BC, et al. Hepatocellular carcinoma in ten children under five years of age with bile salt export pump deficiency. Hepatology 2006;44:478-86.

Cholestasis and Pruritus: Progressive Familial Intrahepatic Cholestasis?

Yıl 2016, Cilt: 14 Sayı: 2, 88 - 95, 01.09.2016

Gökhan Tümgör

https://doi.org/10.4274/jcp.05706

Öz

Progressive familial intrahepatic cholestasis PFIC has a significant place among the childhood cholestasis. It is the second most frequent liver transplant indication met in some liver transplant institutions. It constitutes approximately 10-15% of all cases of cholestasis and liver transplant. PFIC is an autosomal recessively inherited disorder and causes hepatocellular-originated cholestasis. In the period from infancy to adulthood it may cause death due to liver failure. PFIC has three types and the most frequently observed clinical symptoms of all three types are; pruritus, hepatitis, growth retardation, hepatomegaly, and splenomegaly. The prognosis of these three types of PFIC is different. The complications of PFIC are; portal hypertension, liver failure, cirrhosis, hepatocellular carcinoma, and extrahepatic symptoms. Although medical and surgical treatments are applied in order to improve the patients’ life quality, most of the cases require liver transplantation in the early stage

Anahtar Kelimeler

Childhood cholestasis pruritus

Kaynakça

  • 1. Clayton RJ, Iber FL, Ruebner BH, McKusick VA. Byler disease: Fatal familial intrahepatic cholestasis in an Amish kindred. Am J Dis Child 1969;117:112-24.
  • 2. Jankowska I, Socha P. Progressive familial intrahepatic cholestasis and inborn errors of bile acid synthesis. Clin Res Hepatol Gastroenterol 2012;36:271-4.
  • 3. Morotti RA, Suchy FJ, Magid MS. Progressive familial intrahepatic cholestasis (PFIC) type 1, 2, and 3: a review of the liver pathology findings. Semin Liver Dis 2011;31:3-10.
  • 4. Aykut A, Çakır M, Onay H, Nart D, Arıkan Ç, Özgenç F, ve ark. Klinik olarak ilerleyici ailevi intrahepatik kolestaz ile uyumlu hastalarda ATP8B1 mutasyonunun araştırılması: Ön çalışma. J Pediat Res 2014;1:138-41
  • 5. Davit Spraul A, Gonzales E, Baussan C, Jacquemin E. Progressive familial intrahepatic cholestasis. Orphanet J Rare Dis 2009;4:1.
  • 6. Stapelbroek JM, van Erpecum KJ, Klomp LW, Houwen RH. Liver disease associated with canalicular transport defects: current and future therapies. J Hepatol 2010;52:258-71.
  • 7. Davit- Spraul A, Gonzales E, Baussan C, Jacquemin E. The spectrum of liver diseases related to ABCB4 gene mutations: pathophysiology and clinical aspects. Semin Liv Dis 2010;30:134-46.
  • 8. Klomp LW, Vargas JC, Van Mil SW, Pawlikowska L, Strautnieks SS, Van Eijk MJ, et al. Characterization of mutations in ATP8B1 associated with hereditary cholestasis. Hepatology 2004;40:27- 38.
  • 9. Paulusma CC, Groen A, Kunne C, Ho- Mok KS, Spijkerboer AL, Rudi de Waart D, et al. Atp8b1 deficiency in mice reduces resistance of the canalicular membrane to hydrophobic bile salts and impairs bile salt transport. Hepatology 2006;44:195-204.
  • 10. Verhulst PM, van der Velden LM, Oorschot V, van Faassen EE, Klumperman J, Houwen RH, et al. A flippase- independent function of ATP8B1, the protein affected in familial intrahepatic cholestasis type 1, is required for apical protein expression and microvillus formation in polarized epithelial cells. Hepatology 2010;51:2049-60.
  • 11. Groen A, Romero MR, Kunne C, Hoosdally SJ, Dixon PH, Wooding C, et al. Complementary functions of the flippase ATP8B1 and the floppase ABCB4 in maintaining canalicular membrane integrity. Gastroenterology 2011;141:1927-37.
  • 12. Chen F, Ananthanarayanan M, Emre S, Neimark E, Bull LN, Knisely AS, et al. Progressive familial intrahepatic cholestasis, type 1, is associated with decreased farnesoid X receptor activity. Gastroenterology 2004;126:756-64.
  • 13. Alvarez L, Jara P, Sánchez- Sabaté E, Hierro L, Larrauri J, Díaz MC, et al. Reduced hepatic expression of farnesoid X receptor in hereditary cholestasis associated to mutation in ATP8B1. Hum Mol Genet 2004;13:2451-60.
  • 14. Egawa H, Yorifuji T, Sumazaki R, Kimura A, Hasegawa M, Tanaka K. Intractable diarrhea after liver transplantation for Byler’s disease: successful treatment with bile adsorptive resin. Liver Transplant 2002;8:714-6.
  • 15. Jacquemin E, Malan V, Rio M, Davit- Spraul A, Cohen J, Landrieu P, et al. Heterozygous FIC1 deciency: a new genetic predisposition to transient neonatal cholestasis. J Pediatr Gastroenterol Nutr 2010;50:447-9.
  • 16. Van Mil SW, Van den Woerd WL, Van den Brugge G, Sturm E, Jansen PLM, Bull LN, et al. Benign recurrent intrahepatic cholestasis type2 is caused by mutations in ABCB11. Gastroenterology 2004;127:379-84.
  • 17. Pauli- Magnus C, Lang T, Meier Y, Zodan- Marin T, Jung D, Breymann C, et al. Sequence analysis of bile salt export pump (ABCB11) and multidrug resistance p- glycoprotein 3 (ABCB4, MDR3) in patients with intrahepatic cholestasis of pregnancy. Pharmacogenetics 2004;14:91-102.
  • 18. Lang C, Meier Y, Stieger B, Ulrich B, Lang T, Kerb R, et al. Mutations and polymorphisms in the bile salt export pump and the multidrug resistance protein 3 associated with drug-induced liver injury. Pharmacogenet Genomics 2007;17:47-60.
  • 19. Ziol M, Barbu V, Rosmorduc O, Frassati- Biaggi A, Barget N, Hermelin B, et al. ABCB4 heterozygous gene mutations associated with cholestatic liver disease in adults. Gastroenterology 2008;135:131-41.
  • 20. Gonzales E, Gerhardt MF, Fabre M, Setchell KD, Davit- Spraul A, Vincent I, et al. Oral cholic acid for hereditary defects of primary bile acid synthesis: a safe and effective long- term therapy. Gastroenterology 2009;137:1310-20.
  • 21. Houdayer C, Dehainault C, Mattler C, Michaux D, Caux￾Moncoutier V, Pages- Berhouet S, et al. Evaluation of in silico tools for decision making in molecular diagnosis. Human Mut 2008;29:975-82.
  • 22. Liu C, Aronow BJ, Jegga AG, Wang N, Miethke A, Mourya R, et al. Novel resequencing chip customized to diagnose mutations in patients with inherited syndromes of intrahepatic cholestasis. Gastroenterology 2007;132:119-26.
  • 23. Chong CP, Mills PB, McClean P, Gissen P, Bruce C, Stahlschmidt J, et al. Bile acid- CoA ligase deficiency: a new inborn error of bile acid metabolism. J Inherit Metab Dis 2012;35:521-30.
  • 24. Cullinane AR, Straatman- Iwanowska A, Zaucker A, Wakabayashi Y, Bruce CK, Luo G, et al. Mutations in VIPAR cause an arthrogryposis, renal dysfunction and cholestasis syndrome phenotype with defects in epithelial polarization. Nat Genet 2010;42:303-12.
  • 25. Grosse B, Cassio D, Yousef N, Bernardo C, Jacquemin E, Gonzales E. Claudin-1 involved in neonatal ichthyosis sclerosing cholangitis syndrome regulates hepatic paracellular permeability. Hepatology 2012;55:1249-59.
  • 26. Lazaridis KN, Gores GJ, Lindor KD. Ursodeoxycholic acid mechanisms of action and clinical use in hepatobiliary disorders. J Hepatol 2001;35:134-46.
  • 27. Jacquemin E, Hermans D, Myara A, Habes D, Debray D, Hadchouel M, et al. Ursodeoxycholic acid therapy in pediatric patients with progressive familial intrahepatic cholestasis. Hepatology 1997;25:519-23.
  • 28. Feranchak AP, Ramirez RO, Sokol RJ. Medical and nutritional management of cholestasis. In: Suchy FJ, Sokol RJ, Balistreri WF (eds). Liver Disease in Children, Philadelphia: Lippincott Williams & Wilins; 2001. p.195-238.
  • 29. Pawlikowska L, Strautnieks S, Jankowska I, Czubkowski P, Emerick K, Antoniu A, et al. Differences in presentation and progression between severe FIC1 and BSEP deficiencies. J Hepatol 2010;53:170-8.
  • 30. Davit-Spraul A, Fabre M, Branchereau S, Baussan C, Gonzales E, Stieger B, et al. ATP8B1 and ABCB11 analysis in 62 children with normal gamma-glutamyl transferase progressive familial intrahepatic cholestasis (PFIC): phenotypic differences between PFIC1 and PFIC2 and natural history. Hepatology 2010;51:1645-55.
  • 31. Arnell H, Papadogiannakis N, Zemack H, Knisely AS, Nemeth A, Fischler B. Follow-up in children with progressive familial intrahepatic cholestasis after partial external biliary diversion. J Pediatr Gastroenterol Nutr 2010;51:494-9.
  • 32. Kalicin´ski PJ, Ismail H, Jankowska I, Kaminski A, Pawlowska J, Drewniak T, et al. Surgical treatment of progressive familial intrahepatic cholestasis: comparison of partial external biliary diversion and ileal bypass. Eur J Pediatr Surg 2003;13:307-11.
  • 33. Whitington PF, Whitngton GL. Partial external diversion of bile for the treatment of intractable pruritus associated with intrahepatic cholestasis. Gastroenterology 1988;95:130-6.
  • 34. Bustorff- Silva J, Sbraggia Neto L, Olímpio H, de Alcantara RV, Matsushima E, De Tommaso AM, et al. Partial internal biliary diversion through a cholecystojejunocolonic anastomosis: a novel surgical approach for patients with progressive familial intrahepatic cholestasis: a preliminary report. J Pediatr Surg 2007;42:1337-40.
  • 35. Gün F, Erginel B, Durmaz O, Sökücü S, Salman T, Celik A. An outstanding non-transplant surgical intervention in progressive familial intrahepatic cholestasis: partial internal biliary diversion. Pediatr Surg Int 2010;26:831-4.
  • 36. Jankowska I, Czubkowski P, Kalicinski P, Ismail H, Kowalski A, Pawlowska J, et al. Ileal exclusion in children with progressive familial intrahepatic cholestasis. J Pediatr Gastroenterol Nutr 2014;58:92-5.
  • 37. Stapelbroek JM, van Erpecum KJ, Klomp LW, Venneman NG, Schwartz TP, van Berge Henegouwen GP, et al. Nasobiliary drainage induces long- lasting remission in benign recurrent intrahepatic cholestasis. Hepatology 2006;43:51-3.
  • 38. Aydogdu S, Cakir M, Arikan C, Tumgor G, Yuksekkaya HA, Yilmaz F, et al. Liver transplantation for progressive familial intrahepatic cholestasis: clinical and histopathological findings, outcome and impact on growth. Pediatr Transplant 2007;11:634-40.
  • 39. De Vree JM, Ottenhoff R, Bosma PJ, Smith AJ, Aten J, Oude Elferink RP. Correction of liver disease by hepatocyte transplantation in a mouse model of progressive familial intrahepatic cholestasis. Gastroenterology 2000;119:1720-30.
  • 40. Boyer JL. Nuclear receptor ligands: rational and effective therapy for chronic cholestatic liver disease Gastroenterology 2005;129:735-40.
  • 41. Knisely AS, Strautnieks SS, Meier Y, Stieger B, Byrne JA, Portmann BC, et al. Hepatocellular carcinoma in ten children under five years of age with bile salt export pump deficiency. Hepatology 2006;44:478-86.
Toplam 41 adet kaynakça vardır.

Ayrıntılar

Birincil Dil Türkçe
Bölüm Collection
Yazarlar

Gökhan Tümgör

Yayımlanma Tarihi 1 Eylül 2016
Yayımlandığı Sayı Yıl 2016 Cilt: 14 Sayı: 2

Kaynak Göster

APA Tümgör, G. (2016). Kolestaz ve Kaşıntı: Progresif Familyal İntrahepatik Kolestaz?. Güncel Pediatri, 14(2), 88-95. https://doi.org/10.4274/jcp.05706
AMA Tümgör G. Kolestaz ve Kaşıntı: Progresif Familyal İntrahepatik Kolestaz?. Güncel Pediatri. Eylül 2016;14(2):88-95. doi:10.4274/jcp.05706
Chicago Tümgör, Gökhan. “Kolestaz Ve Kaşıntı: Progresif Familyal İntrahepatik Kolestaz?”. Güncel Pediatri 14, sy. 2 (Eylül 2016): 88-95. https://doi.org/10.4274/jcp.05706.
EndNote Tümgör G (01 Eylül 2016) Kolestaz ve Kaşıntı: Progresif Familyal İntrahepatik Kolestaz?. Güncel Pediatri 14 2 88–95.
IEEE G. Tümgör, “Kolestaz ve Kaşıntı: Progresif Familyal İntrahepatik Kolestaz?”, Güncel Pediatri, c. 14, sy. 2, ss. 88–95, 2016, doi: 10.4274/jcp.05706.
ISNAD Tümgör, Gökhan. “Kolestaz Ve Kaşıntı: Progresif Familyal İntrahepatik Kolestaz?”. Güncel Pediatri 14/2 (Eylül 2016), 88-95. https://doi.org/10.4274/jcp.05706.
JAMA Tümgör G. Kolestaz ve Kaşıntı: Progresif Familyal İntrahepatik Kolestaz?. Güncel Pediatri. 2016;14:88–95.
MLA Tümgör, Gökhan. “Kolestaz Ve Kaşıntı: Progresif Familyal İntrahepatik Kolestaz?”. Güncel Pediatri, c. 14, sy. 2, 2016, ss. 88-95, doi:10.4274/jcp.05706.
Vancouver Tümgör G. Kolestaz ve Kaşıntı: Progresif Familyal İntrahepatik Kolestaz?. Güncel Pediatri. 2016;14(2):88-95.