Liver transplantation for progressive familial intrahepatic cholestasis: Clinical and histopathological findings, outcome and impact on growth

: In this study, we analyze the demographic features, clinical and histopathological findings in patients who underwent liver transplantation for progressive familial intrahepatic cholestasis. We also analyze outcome and impact of liver transplantation on growth and bone mineral content. Most of th...

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Vydáno v:	Pediatric transplantation Ročník 11; číslo 6; s. 634 - 640
Hlavní autoři:	Aydogdu, Sema, Cakir, Murat, Arikan, C., Tumgor, Gokhan, Yuksekkaya, Hasan Ali, Yilmaz, Funda, Kilic, Murat
Médium:	Journal Article
Jazyk:	angličtina
Vydáno:	Oxford, UK Blackwell Publishing Ltd 01.09.2007 Blackwell
Témata:	Biological and medical sciences Bone Density Child Child, Preschool Cholestasis, Intrahepatic - complications Cholestasis, Intrahepatic - genetics Cholestasis, Intrahepatic - pathology Cholestasis, Intrahepatic - surgery Disease Progression Female Gastroenterology. Liver. Pancreas. Abdomen General aspects Growth Humans Infant liver transplantation Liver Transplantation - adverse effects Liver. Biliary tract. Portal circulation. Exocrine pancreas Male Medical sciences Other diseases. Semiology progressive familial intrahepatic Surgery (general aspects). Transplantations, organ and tissue grafts. Graft diseases Treatment Outcome Pediatrics Prognosis Growth Hepatic disease Transplantation Biliary tract disease Homotransplantation Intrahepatic cholostasis Histopathology Surgery Evolution Graft Intrahepatic Somatic growth Child Human Digestive system Family study Progressive Symptomatology Anatomic pathology Treatment Digestive diseases progressive familial intrahepatic liver transplantation
ISSN:	1397-3142, 1399-3046
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Abstract	: In this study, we analyze the demographic features, clinical and histopathological findings in patients who underwent liver transplantation for progressive familial intrahepatic cholestasis. We also analyze outcome and impact of liver transplantation on growth and bone mineral content. Most of the patients were presented with jaundice mainly beginning within the first six months. At the time of initial admission; eight patients had short stature (height SD score <2), and four patients had weight SD score <2. Liver transplantation were performed at the age of 43.2 ± 27 months (range 9 to 96 mfonths), 6.5 ± 3.5 months later after the first admission. Infection, surgical complications and osmotic diarrhea associated with severe metabolic acidosis were noted in 41.4%, 16.6% and 33.3%, respectively. One patient developed posttransplant lymphoproliferative disorder. Overall; 1 year graft and patient survival was 69.2% and 75%, respectively. At the end of the 1st year only 2 patients had height SD score <2. Linear regression of height gain against increase in total body BMD measured at the time of transplantation and 1 year after liver transplantation gave a coefficient r = 0.588 (p = 0.074). No correlation was found between the height gain and age and PELD score at time of transplantation, and no difference was noted between the sexes and donor type. Liver transplantation is effective treatment modality with good outcome and little morbidity, and increases the growth acceleration in patients with PFIC associated with cirrhosis.
AbstractList	In this study, we analyze the demographic features, clinical and histopathological findings in patients who underwent liver transplantation for progressive familial intrahepatic cholestasis. We also analyze outcome and impact of liver transplantation on growth and bone mineral content. Most of the patients were presented with jaundice mainly beginning within the first six months. At the time of initial admission; eight patients had short stature (height SD score<2), and four patients had weight SD score<2. Liver transplantation were performed at the age of 43.2+/-27 months (range 9 to 96 months), 6.5+/-3.5 months later after the first admission. Infection, surgical complications and osmotic diarrhea associated with severe metabolic acidosis were noted in 41.4%, 16.6% and 33.3%, respectively. One patient developed posttransplant lymphoproliferative disorder. Overall; 1 year graft and patient survival was 69.2% and 75%, respectively. At the end of the 1st year only 2 patients had height SD score<2. Linear regression of height gain against increase in total body BMD measured at the time of transplantation and 1 year after liver transplantation gave a coefficient r=0.588 (p=0.074). No correlation was found between the height gain and age and PELD score at time of transplantation, and no difference was noted between the sexes and donor type. Liver transplantation is effective treatment modality with good outcome and little morbidity, and increases the growth acceleration in patients with PFIC associated with cirrhosis. In this study, we analyze the demographic features, clinical and histopathological findings in patients who underwent liver transplantation for progressive familial intrahepatic cholestasis. We also analyze outcome and impact of liver transplantation on growth and bone mineral content. Most of the patients were presented with jaundice mainly beginning within the first six months. At the time of initial admission; eight patients had short stature (height SD score<2), and four patients had weight SD score<2. Liver transplantation were performed at the age of 43.2+/-27 months (range 9 to 96 months), 6.5+/-3.5 months later after the first admission. Infection, surgical complications and osmotic diarrhea associated with severe metabolic acidosis were noted in 41.4%, 16.6% and 33.3%, respectively. One patient developed posttransplant lymphoproliferative disorder. Overall; 1 year graft and patient survival was 69.2% and 75%, respectively. At the end of the 1st year only 2 patients had height SD score<2. Linear regression of height gain against increase in total body BMD measured at the time of transplantation and 1 year after liver transplantation gave a coefficient r=0.588 (p=0.074). No correlation was found between the height gain and age and PELD score at time of transplantation, and no difference was noted between the sexes and donor type. Liver transplantation is effective treatment modality with good outcome and little morbidity, and increases the growth acceleration in patients with PFIC associated with cirrhosis.In this study, we analyze the demographic features, clinical and histopathological findings in patients who underwent liver transplantation for progressive familial intrahepatic cholestasis. We also analyze outcome and impact of liver transplantation on growth and bone mineral content. Most of the patients were presented with jaundice mainly beginning within the first six months. At the time of initial admission; eight patients had short stature (height SD score<2), and four patients had weight SD score<2. Liver transplantation were performed at the age of 43.2+/-27 months (range 9 to 96 months), 6.5+/-3.5 months later after the first admission. Infection, surgical complications and osmotic diarrhea associated with severe metabolic acidosis were noted in 41.4%, 16.6% and 33.3%, respectively. One patient developed posttransplant lymphoproliferative disorder. Overall; 1 year graft and patient survival was 69.2% and 75%, respectively. At the end of the 1st year only 2 patients had height SD score<2. Linear regression of height gain against increase in total body BMD measured at the time of transplantation and 1 year after liver transplantation gave a coefficient r=0.588 (p=0.074). No correlation was found between the height gain and age and PELD score at time of transplantation, and no difference was noted between the sexes and donor type. Liver transplantation is effective treatment modality with good outcome and little morbidity, and increases the growth acceleration in patients with PFIC associated with cirrhosis. : In this study, we analyze the demographic features, clinical and histopathological findings in patients who underwent liver transplantation for progressive familial intrahepatic cholestasis. We also analyze outcome and impact of liver transplantation on growth and bone mineral content. Most of the patients were presented with jaundice mainly beginning within the first six months. At the time of initial admission; eight patients had short stature (height SD score <2), and four patients had weight SD score <2. Liver transplantation were performed at the age of 43.2 ± 27 months (range 9 to 96 mfonths), 6.5 ± 3.5 months later after the first admission. Infection, surgical complications and osmotic diarrhea associated with severe metabolic acidosis were noted in 41.4%, 16.6% and 33.3%, respectively. One patient developed posttransplant lymphoproliferative disorder. Overall; 1 year graft and patient survival was 69.2% and 75%, respectively. At the end of the 1st year only 2 patients had height SD score <2. Linear regression of height gain against increase in total body BMD measured at the time of transplantation and 1 year after liver transplantation gave a coefficient r = 0.588 (p = 0.074). No correlation was found between the height gain and age and PELD score at time of transplantation, and no difference was noted between the sexes and donor type. Liver transplantation is effective treatment modality with good outcome and little morbidity, and increases the growth acceleration in patients with PFIC associated with cirrhosis. In this study, we analyze the demographic features, clinical and histopathological findings in patients who underwent liver transplantation for progressive familial intrahepatic cholestasis. We also analyze outcome and impact of liver transplantation on growth and bone mineral content. Most of the patients were presented with jaundice mainly beginning within the first six months. At the time of initial admission; eight patients had short stature (height SD score <2), and four patients had weight SD score <2. Liver transplantation were performed at the age of 43.2 ± 27 months (range 9 to 96 mfonths), 6.5 ± 3.5 months later after the first admission. Infection, surgical complications and osmotic diarrhea associated with severe metabolic acidosis were noted in 41.4%, 16.6% and 33.3%, respectively. One patient developed posttransplant lymphoproliferative disorder. Overall; 1 year graft and patient survival was 69.2% and 75%, respectively. At the end of the 1 st year only 2 patients had height SD score <2. Linear regression of height gain against increase in total body BMD measured at the time of transplantation and 1 year after liver transplantation gave a coefficient r = 0.588 (p = 0.074). No correlation was found between the height gain and age and PELD score at time of transplantation, and no difference was noted between the sexes and donor type. Liver transplantation is effective treatment modality with good outcome and little morbidity, and increases the growth acceleration in patients with PFIC associated with cirrhosis.
Author	Arikan, C. Cakir, Murat Yuksekkaya, Hasan Ali Tumgor, Gokhan Yilmaz, Funda Kilic, Murat Aydogdu, Sema
Author_xml	– sequence: 1 givenname: Sema surname: Aydogdu fullname: Aydogdu, Sema organization: Department of Pediatric Gastroenterology Hepatology and Nutrition – sequence: 2 givenname: Murat surname: Cakir fullname: Cakir, Murat organization: Department of Pediatric Gastroenterology Hepatology and Nutrition – sequence: 3 givenname: C. surname: Arikan fullname: Arikan, C. organization: Department of Pediatric Gastroenterology Hepatology and Nutrition – sequence: 4 givenname: Gokhan surname: Tumgor fullname: Tumgor, Gokhan organization: Department of Pediatric Gastroenterology Hepatology and Nutrition – sequence: 5 givenname: Hasan Ali surname: Yuksekkaya fullname: Yuksekkaya, Hasan Ali organization: Department of Pediatric Gastroenterology Hepatology and Nutrition – sequence: 6 givenname: Funda surname: Yilmaz fullname: Yilmaz, Funda organization: Department of Pathology – sequence: 7 givenname: Murat surname: Kilic fullname: Kilic, Murat organization: Organ Research and Transplantation Center, Ege University, Faculty of Medicine, Bornova, İzmir, Turkiye
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Keywords	Pediatrics Prognosis Growth Hepatic disease Transplantation Biliary tract disease Homotransplantation Intrahepatic cholostasis Histopathology Surgery Evolution Graft Intrahepatic Somatic growth Child Human Digestive system Family study Progressive Symptomatology Anatomic pathology Treatment Digestive diseases progressive familial intrahepatic liver transplantation
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References	Knısely AS, Strautnıeks SS, Meıer Y, et al. Hepatocellular carcinoma in ten children under five years of age with bile salt export pump deficiency. Hepatology 2006: 44: 478-486. Melter M, Rodeck B, Kardorff R, et al. Progressive familial intrahepatic cholestasis: partial biliary diversion normalizes serum lipids and improves growth in noncirrhotic patients. Am J Gastroenterol 2000: 95: 3522-3528. Nagasaka H, Yorifuji T, Kosugiyama K, et al. Resistance to parathyroid hormone in two patients with familial intrahepatic cholestasis: Possible involvement of the ATP8B1 gene in calcium regulation via parathyroid hormone. J Pediatr Gastroenterol Nutr 2004: 39: 404-409. Kurbegov AC, Setchell KD, Haas JE, et al. Biliary diversion for progressive familial intrahepatic cholestasis: improved liver morphology and bile acid profile. Gastroenterology 2003: 125: 1227-1234. Glasova H, Beuers U. Extrahepatic manifestations of cholestasis. J Gastroenterol Hepatol 2002: 17: 938-948. Jacquemin E, Hermans D, Myara A, et al. Ursodeoxycholic acid therapy in pediatric patients with progressive familial intrahepatic cholestasis. Hepatology 1997: 25: 519-523. Van Mil SW, Klomp LW, Bull LN, Houwen RH. FIC1 disease: A spectrum of intrahepatic cholestatic disorders. Semin Liver Dis 2001: 21: 535-544. Bull LN, Carlton VE, Stricker NL, et al. Genetic and morphological findings in progressive familial intrahepatic cholestasis [Byler disease (PFIC-1) and Byler syndrome]: Evidence for heterogeneity. Hepatology 1997: 26: 155-164. Jansen PL, Sturm E. Genetic cholestasis, causes and consequences for hepatobiliary transport. Liver Int 2003: 23: 315-322. Heubi JE, Hollis BW, Tsang RC. Bone disease in chronic childhood cholestasis. II. Better absorption of 25-OH vitamin D than vitamin D in extrahepatic biliary atresia. Pediatr Res 1990: 27: 26-31. Egawa H, Yorifuji T, Sumazaki R, et al. Intractable diarrhea after liver transplantation for Byler's disease: Successful treatment with bile adsorptive resin. Liver Transpl 2002: 8: 714-716. Heubi JE, Hollis BW, Specker B, et al. Bone disease in chronic childhood cholestasis. I. Vitamin D absorption and metabolism. Hepatology 1989: 9: 258-264. Shneider BL. Progressive intrahepatic cholestasis: Mechanisms, diagnosis and therapy. Pediatr Transplant 2004: 8: 609-612. Clayton RJ, Iber FL, Ruebner BH, et al. Byler disease. Fatal familial intrahepatic cholestasis in an Amish kindred. Am J Dis Child 1969: 117: 112-124. Thompson R, Strautnieks S. BSEP: Function and role in progressive familial intrahepatic cholestasis. Semin Liver Dis 2001: 21: 545-550. Lykavieris P, Van Mil S, Cresteil D, et al. Progressive familial intrahepatic cholestasis type 1 and extrahepatic features: No catch-up of stature growth, exacerbation of diarrhea, and appearance of liver steatosis after liver transplantation. J Hepatol 2003: 39: 447-452. Naveh Y, Bassan L, Rosenthal E, et al. Progressive familial intrahepatic cholestasis among the Arab population in Israel. J Pediatr Gastroenterol Nutr 1997: 24: 548-554. Kagalwalla AF, Al Amir AR, Khalifa A, et al. Progressive familial intrahepatic cholestasis (Byler's disease) in Arab children. Ann Trop Paediatr 1995: 15: 321-327. Wanty C, Joomye R, Van Hoorebeek N, et al. Fifteen years single center experience in the management of progressive familial intrahepatic cholestasis of infancy. Acta Gastroenterol Belg 2004: 67: 313-319. Harris MJ, Le Couteur DG, Arias IM. Progressive familial intrahepatic cholestasis: Genetic disorders of biliary transporters. J Gastroenterol Hepatol 2005: 20: 807-817. Ismail H, Kalicinski P, Markiewicz M, et al. Treatment of progressive familial intrahepatic cholestasis: Liver transplantation or partial external biliary diversion. Pediatr Transplant 1999: 3: 219-224. Alonso EM, Snover DC, Montag A, et al. Histologic pathology of the liver in progressive familial intrahepatic cholestasis. J Pediatr Gastroenterol Nutr 1994: 18: 128-133. Hollands CM, Rivera-Pedrogo FJ, Gonzalez-Vallina R, Loret-de-Mola O, Nahmad M, Burnweit CA. Ileal exclusion for Byler's disease: An alternative surgical approach with promising early results for pruritus. J Pediatr Surg 1998: 33: 220-224. 2002; 17 1990; 27 1995; 15 2004; 39 2006; 44 1997; 26 2004; 8 1997; 25 2004; 67 1989; 9 1997; 24 2002; 8 2000; 95 2005; 20 2003; 39 1999; 3 1994; 18 1969; 117 2003; 125 1998; 33 2003; 23 2001; 21 Melter M (e_1_2_6_21_2) 2000; 95 Kurbegov AC (e_1_2_6_20_2) 2003; 125 e_1_2_6_8_2 e_1_2_6_7_2 e_1_2_6_18_2 Wanty C (e_1_2_6_9_2) 2004; 67 e_1_2_6_19_2 e_1_2_6_4_2 e_1_2_6_3_2 e_1_2_6_6_2 e_1_2_6_5_2 e_1_2_6_12_2 e_1_2_6_24_2 e_1_2_6_13_2 e_1_2_6_23_2 e_1_2_6_2_2 e_1_2_6_10_2 e_1_2_6_22_2 e_1_2_6_11_2 e_1_2_6_16_2 e_1_2_6_17_2 e_1_2_6_14_2 e_1_2_6_15_2
References_xml	– reference: Glasova H, Beuers U. Extrahepatic manifestations of cholestasis. J Gastroenterol Hepatol 2002: 17: 938-948. – reference: Heubi JE, Hollis BW, Specker B, et al. Bone disease in chronic childhood cholestasis. I. Vitamin D absorption and metabolism. Hepatology 1989: 9: 258-264. – reference: Nagasaka H, Yorifuji T, Kosugiyama K, et al. Resistance to parathyroid hormone in two patients with familial intrahepatic cholestasis: Possible involvement of the ATP8B1 gene in calcium regulation via parathyroid hormone. J Pediatr Gastroenterol Nutr 2004: 39: 404-409. – reference: Kagalwalla AF, Al Amir AR, Khalifa A, et al. Progressive familial intrahepatic cholestasis (Byler's disease) in Arab children. Ann Trop Paediatr 1995: 15: 321-327. – reference: Kurbegov AC, Setchell KD, Haas JE, et al. Biliary diversion for progressive familial intrahepatic cholestasis: improved liver morphology and bile acid profile. Gastroenterology 2003: 125: 1227-1234. – reference: Egawa H, Yorifuji T, Sumazaki R, et al. Intractable diarrhea after liver transplantation for Byler's disease: Successful treatment with bile adsorptive resin. Liver Transpl 2002: 8: 714-716. – reference: Wanty C, Joomye R, Van Hoorebeek N, et al. Fifteen years single center experience in the management of progressive familial intrahepatic cholestasis of infancy. Acta Gastroenterol Belg 2004: 67: 313-319. – reference: Heubi JE, Hollis BW, Tsang RC. Bone disease in chronic childhood cholestasis. II. Better absorption of 25-OH vitamin D than vitamin D in extrahepatic biliary atresia. Pediatr Res 1990: 27: 26-31. – reference: Shneider BL. Progressive intrahepatic cholestasis: Mechanisms, diagnosis and therapy. Pediatr Transplant 2004: 8: 609-612. – reference: Thompson R, Strautnieks S. BSEP: Function and role in progressive familial intrahepatic cholestasis. Semin Liver Dis 2001: 21: 545-550. – reference: Harris MJ, Le Couteur DG, Arias IM. Progressive familial intrahepatic cholestasis: Genetic disorders of biliary transporters. J Gastroenterol Hepatol 2005: 20: 807-817. – reference: Clayton RJ, Iber FL, Ruebner BH, et al. Byler disease. Fatal familial intrahepatic cholestasis in an Amish kindred. Am J Dis Child 1969: 117: 112-124. – reference: Hollands CM, Rivera-Pedrogo FJ, Gonzalez-Vallina R, Loret-de-Mola O, Nahmad M, Burnweit CA. Ileal exclusion for Byler's disease: An alternative surgical approach with promising early results for pruritus. J Pediatr Surg 1998: 33: 220-224. – reference: Knısely AS, Strautnıeks SS, Meıer Y, et al. Hepatocellular carcinoma in ten children under five years of age with bile salt export pump deficiency. Hepatology 2006: 44: 478-486. – reference: Bull LN, Carlton VE, Stricker NL, et al. Genetic and morphological findings in progressive familial intrahepatic cholestasis [Byler disease (PFIC-1) and Byler syndrome]: Evidence for heterogeneity. Hepatology 1997: 26: 155-164. – reference: Jacquemin E, Hermans D, Myara A, et al. Ursodeoxycholic acid therapy in pediatric patients with progressive familial intrahepatic cholestasis. Hepatology 1997: 25: 519-523. – reference: Naveh Y, Bassan L, Rosenthal E, et al. Progressive familial intrahepatic cholestasis among the Arab population in Israel. J Pediatr Gastroenterol Nutr 1997: 24: 548-554. – reference: Lykavieris P, Van Mil S, Cresteil D, et al. Progressive familial intrahepatic cholestasis type 1 and extrahepatic features: No catch-up of stature growth, exacerbation of diarrhea, and appearance of liver steatosis after liver transplantation. J Hepatol 2003: 39: 447-452. – reference: Melter M, Rodeck B, Kardorff R, et al. Progressive familial intrahepatic cholestasis: partial biliary diversion normalizes serum lipids and improves growth in noncirrhotic patients. Am J Gastroenterol 2000: 95: 3522-3528. – reference: Ismail H, Kalicinski P, Markiewicz M, et al. Treatment of progressive familial intrahepatic cholestasis: Liver transplantation or partial external biliary diversion. Pediatr Transplant 1999: 3: 219-224. – reference: Alonso EM, Snover DC, Montag A, et al. Histologic pathology of the liver in progressive familial intrahepatic cholestasis. J Pediatr Gastroenterol Nutr 1994: 18: 128-133. – reference: Van Mil SW, Klomp LW, Bull LN, Houwen RH. FIC1 disease: A spectrum of intrahepatic cholestatic disorders. Semin Liver Dis 2001: 21: 535-544. – reference: Jansen PL, Sturm E. Genetic cholestasis, causes and consequences for hepatobiliary transport. Liver Int 2003: 23: 315-322. – volume: 20 start-page: 807 year: 2005 end-page: 817 article-title: Progressive familial intrahepatic cholestasis: Genetic disorders of biliary transporters publication-title: J Gastroenterol Hepatol – volume: 24 start-page: 548 year: 1997 end-page: 554 article-title: Progressive familial intrahepatic cholestasis among the Arab population in Israel publication-title: J Pediatr Gastroenterol Nutr – volume: 39 start-page: 447 year: 2003 end-page: 452 article-title: Progressive familial intrahepatic cholestasis type 1 and extrahepatic features: No catch‐up of stature growth, exacerbation of diarrhea, and appearance of liver steatosis after liver transplantation publication-title: J Hepatol – volume: 15 start-page: 321 year: 1995 end-page: 327 article-title: Progressive familial intrahepatic cholestasis (Byler’s disease) in Arab children publication-title: Ann Trop Paediatr – volume: 27 start-page: 26 year: 1990 end-page: 31 article-title: Bone disease in chronic childhood cholestasis. II. Better absorption of 25‐OH vitamin D than vitamin D in extrahepatic biliary atresia publication-title: Pediatr Res – volume: 17 start-page: 938 year: 2002 end-page: 948 article-title: Extrahepatic manifestations of cholestasis publication-title: J Gastroenterol Hepatol – volume: 26 start-page: 155 year: 1997 end-page: 164 article-title: Genetic and morphological findings in progressive familial intrahepatic cholestasis [Byler disease (PFIC‐1) and Byler syndrome]: Evidence for heterogeneity publication-title: Hepatology – volume: 3 start-page: 219 year: 1999 end-page: 224 article-title: Treatment of progressive familial intrahepatic cholestasis: Liver transplantation or partial external biliary diversion publication-title: Pediatr Transplant – volume: 39 start-page: 404 year: 2004 end-page: 409 article-title: Resistance to parathyroid hormone in two patients with familial intrahepatic cholestasis: Possible involvement of the ATP8B1 gene in calcium regulation via parathyroid hormone publication-title: J Pediatr Gastroenterol Nutr – volume: 8 start-page: 714 year: 2002 end-page: 716 article-title: Intractable diarrhea after liver transplantation for Byler’s disease: Successful treatment with bile adsorptive resin publication-title: Liver Transpl – volume: 44 start-page: 478 year: 2006 end-page: 486 article-title: Hepatocellular carcinoma in ten children under five years of age with bile salt export pump deficiency publication-title: Hepatology – volume: 67 start-page: 313 year: 2004 end-page: 319 article-title: Fifteen years single center experience in the management of progressive familial intrahepatic cholestasis of infancy publication-title: Acta Gastroenterol Belg – volume: 125 start-page: 1227 year: 2003 end-page: 1234 publication-title: Biliary diversion for progressive familial intrahepatic cholestasis: improved liver morphology and bile acid profile – volume: 25 start-page: 519 year: 1997 end-page: 523 article-title: Ursodeoxycholic acid therapy in pediatric patients with progressive familial intrahepatic cholestasis publication-title: Hepatology – volume: 8 start-page: 609 year: 2004 end-page: 612 article-title: Progressive intrahepatic cholestasis: Mechanisms, diagnosis and therapy publication-title: Pediatr Transplant – volume: 18 start-page: 128 year: 1994 end-page: 133 article-title: Histologic pathology of the liver in progressive familial intrahepatic cholestasis publication-title: J Pediatr Gastroenterol Nutr – volume: 21 start-page: 545 year: 2001 end-page: 550 article-title: BSEP: Function and role in progressive familial intrahepatic cholestasis publication-title: Semin Liver Dis – volume: 23 start-page: 315 year: 2003 end-page: 322 article-title: Genetic cholestasis, causes and consequences for hepatobiliary transport publication-title: Liver Int – volume: 117 start-page: 112 year: 1969 end-page: 124 article-title: Byler disease. Fatal familial intrahepatic cholestasis in an Amish kindred publication-title: Am J Dis Child – volume: 33 start-page: 220 year: 1998 end-page: 224 article-title: Ileal exclusion for Byler’s disease: An alternative surgical approach with promising early results for pruritus publication-title: J Pediatr Surg – volume: 21 start-page: 535 year: 2001 end-page: 544 article-title: FIC1 disease: A spectrum of intrahepatic cholestatic disorders publication-title: Semin Liver Dis – volume: 9 start-page: 258 year: 1989 end-page: 264 article-title: Bone disease in chronic childhood cholestasis. I. Vitamin D absorption and metabolism publication-title: Hepatology – volume: 95 start-page: 3522 year: 2000 end-page: 3528 publication-title: Progressive familial intrahepatic cholestasis: partial biliary diversion normalizes serum lipids and improves growth in noncirrhotic patients – ident: e_1_2_6_16_2 doi: 10.1053/jlts.2002.34384 – ident: e_1_2_6_23_2 doi: 10.1055/s-2001-19038 – ident: e_1_2_6_15_2 doi: 10.1097/00005176-200410000-00018 – ident: e_1_2_6_8_2 doi: 10.1002/hep.510250303 – volume: 67 start-page: 313 year: 2004 ident: e_1_2_6_9_2 article-title: Fifteen years single center experience in the management of progressive familial intrahepatic cholestasis of infancy publication-title: Acta Gastroenterol Belg – ident: e_1_2_6_2_2 doi: 10.1111/j.1440-1746.2005.03743.x – volume: 95 start-page: 3522 year: 2000 ident: e_1_2_6_21_2 publication-title: Progressive familial intrahepatic cholestasis: partial biliary diversion normalizes serum lipids and improves growth in noncirrhotic patients – volume: 125 start-page: 1227 year: 2003 ident: e_1_2_6_20_2 publication-title: Biliary diversion for progressive familial intrahepatic cholestasis: improved liver morphology and bile acid profile – ident: e_1_2_6_14_2 doi: 10.1097/00005176-199705000-00011 – ident: e_1_2_6_18_2 doi: 10.1111/j.1399-3046.2004.00240.x – ident: e_1_2_6_24_2 doi: 10.1002/hep.21287 – ident: e_1_2_6_10_2 doi: 10.1080/02724936.1995.11747792 – ident: e_1_2_6_3_2 doi: 10.1002/hep.510260121 – ident: e_1_2_6_4_2 doi: 10.1001/archpedi.1969.02100030114014 – ident: e_1_2_6_19_2 doi: 10.1016/S0022-3468(98)90435-3 – ident: e_1_2_6_13_2 doi: 10.1203/00006450-199001000-00006 – ident: e_1_2_6_17_2 doi: 10.1016/S0168-8278(03)00286-1 – ident: e_1_2_6_22_2 doi: 10.1055/s-2001-19034 – ident: e_1_2_6_11_2 doi: 10.1097/00005176-199402000-00002 – ident: e_1_2_6_6_2 doi: 10.1046/j.1440-1746.2002.02717.x – ident: e_1_2_6_5_2 doi: 10.1034/j.1478-3231.2003.00856.x – ident: e_1_2_6_7_2 doi: 10.1034/j.1399-3046.1999.00046.x – ident: e_1_2_6_12_2 doi: 10.1002/hep.1840090216
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Snippet	: In this study, we analyze the demographic features, clinical and histopathological findings in patients who underwent liver transplantation for progressive... In this study, we analyze the demographic features, clinical and histopathological findings in patients who underwent liver transplantation for progressive...
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SubjectTerms	Biological and medical sciences Bone Density Child Child, Preschool Cholestasis, Intrahepatic - complications Cholestasis, Intrahepatic - genetics Cholestasis, Intrahepatic - pathology Cholestasis, Intrahepatic - surgery Disease Progression Female Gastroenterology. Liver. Pancreas. Abdomen General aspects Growth Humans Infant liver transplantation Liver Transplantation - adverse effects Liver. Biliary tract. Portal circulation. Exocrine pancreas Male Medical sciences Other diseases. Semiology progressive familial intrahepatic Surgery (general aspects). Transplantations, organ and tissue grafts. Graft diseases Treatment Outcome
Title	Liver transplantation for progressive familial intrahepatic cholestasis: Clinical and histopathological findings, outcome and impact on growth
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