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Liver in Systemic Disease

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Diseases of the Liver in Children

Abstract

As a major component of the body’s filtration system and metabolic machinery, the liver is affected in many systemic diseases. Systemic diseases that manifest primarily in the liver, or with accompanying liver disease as one of the most common manifestations, have been discussed elsewhere in the text. However, hepatitis, cholestasis, or other liver disease may be secondary manifestations of many other systemic diseases. The pediatric hepatologist is often consulted to interpret liver abnormalities in children with other systemic diseases. This chapter provides an overview of the liver involvement characteristic of major systemic diseases, by organ system.

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Abbreviations

6-MP:

6-mercaptopurine

AIH:

Autoimmune hepatitis

ALP:

Alkaline phosphatase

ALT:

Alanine aminotransferase

AST:

Aspartate aminotransferase

CGD:

Chronic granulomatous disease

DIC:

Disseminated intravascular coagulation

GGT:

Gamma-glutamyl transferase

GVHD:

Graft-versus-host disease

HLH:

Hemophagocytic lymphohistiocytosis

IVC:

Inferior vena cava

IVIG:

Intravenous immunoglobulin

JIA:

Juvenile idiopathic arthritis

LDH:

Lactate dehydrogenase

MAS:

Macrophage activation syndrome

NAFLD:

Nonalcoholic fatty liver disease

NSAIDs:

Nonsteroidal anti-inflammatory drugs

PAN:

Polyarteritis nodosa

PBC:

Primary biliary cirrhosis

PCR:

Polymerase chain reaction

PSC:

Primary sclerosing cholangitis

SCT:

Stem cell transplantation

SLE:

Systemic lupus erythematosus

References

  1. Henrion J, Schapira M, Luwaert R, Colin L, Delannoy A, Heller FR. Hypoxic hepatitis: clinical and hemodynamic study in 142 consecutive cases. Medicine (Baltimore). 2003;82(6):392–406.

    Google Scholar 

  2. Jacquemin E, Saliba E, Blond MH, Chantepie A, Laugier J. Liver dysfunction and acute cardiocirculatory failure in children. Eur J Pediatr. 1992;151(10):731–4.

    PubMed  CAS  Google Scholar 

  3. Garland JS, Werlin SL, Rice TB. Ischemic hepatitis in children: diagnosis and clinical course. Crit Care Med. 1988;16(12):1209–12.

    PubMed  CAS  Google Scholar 

  4. Cassidy WM, Reynolds TB. Serum lactic dehydrogenase in the differential diagnosis of acute hepatocellular injury. J Clin Gastroenterol. 1994;19(2):118–21.

    PubMed  CAS  Google Scholar 

  5. Henrion J, Minette P, Colin L, Schapira M, Delannoy A, Heller FR. Hypoxic hepatitis caused by acute exacerbation of chronic respiratory failure: a case-controlled, hemodynamic study of 17 consecutive cases. Hepatology. 1999;29(2):427–33.

    PubMed  CAS  Google Scholar 

  6. Mace S, Borkat G, Liebman J. Hepatic dysfunction and cardiovascular abnormalities. occurrence in infants, children, and young adults. Am J Dis Child. 1985;139(1):60–5.

    PubMed  CAS  Google Scholar 

  7. Arcidi Jr JM, Moore GW, Hutchins GM. Hepatic morphology in cardiac dysfunction: a clinicopathologic study of 1000 subjects at autopsy. Am J Pathol. 1981;104(2):159–66.

    PubMed  PubMed Central  Google Scholar 

  8. Rychik J, Veldtman G, Rand E, et al. The precarious state of the liver after a Fontan operation: summary of a multidisciplinary symposium. Pediatr Cardiol. 2012;33(7):1001–12.

    PubMed  PubMed Central  Google Scholar 

  9. Schwartz MC, Sullivan L, Cohen MS, et al. Hepatic pathology may develop before the Fontan operation in children with functional single ventricle: an autopsy study. J Thorac Cardiovasc Surg. 2012;143(4):904–9.

    PubMed  Google Scholar 

  10. Matsumoto T, Kobayashi S, Shimizu H, et al. The liver in collagen diseases: pathologic study of 160 cases with particular reference to hepatic arteritis, primary biliary cirrhosis, autoimmune hepatitis and nodular regenerative hyperplasia of the liver. Liver. 2000;20(5):366–73.

    PubMed  CAS  Google Scholar 

  11. Selmi C, De Santis M, Gershwin ME. Liver involvement in subjects with rheumatic disease. Arthritis Res Ther. 2011;13(3):226.

    PubMed  PubMed Central  Google Scholar 

  12. Rachelefsky GS, Kar NC, Coulson A, Sarkissian E, Stiehm ER, Paulus HE. Serum enzyme abnormalities in juvenile rheumatoid arthritis. Pediatrics. 1976;58(5):730–6.

    PubMed  CAS  Google Scholar 

  13. Gurion R, Lehman TJ, Moorthy LN. Systemic arthritis in children: a review of clinical presentation and treatment. Int J Inflamm. 2012;2012:271569.

    CAS  Google Scholar 

  14. Beukelman T, Patkar NM, Saag KG, et al. 2011 American College of Rheumatology recommendations for the treatment of juvenile idiopathic arthritis: initiation and safety monitoring of therapeutic agents for the treatment of arthritis and systemic features. Arthritis Care Res (Hoboken). 2011;63(4):465–82.

    Google Scholar 

  15. Behrens EM, Beukelman T, Gallo L, et al. Evaluation of the presentation of systemic onset juvenile rheumatoid arthritis: data from the Pennsylvania systemic onset juvenile arthritis registry (PASOJAR). J Rheumatol. 2008;35(2):343–8.

    PubMed  Google Scholar 

  16. Falk RH, Comenzo RL, Skinner M. The systemic amyloidoses. N Engl J Med. 1997;337(13):898–909.

    PubMed  CAS  Google Scholar 

  17. Bloom BJ, Smith P, Alario AJ. Felty syndrome complicating juvenile rheumatoid arthritis. J Pediatr Hematol Oncol. 1998;20(5):511–3.

    PubMed  CAS  Google Scholar 

  18. Abraham S, Begum S, Isenberg D. Hepatic manifestations of autoimmune rheumatic diseases. Ann Rheum Dis. 2004;63(2):123–9.

    PubMed  CAS  PubMed Central  Google Scholar 

  19. Stock H, Kadry Z, Smith JP. Surgical management of portal hypertension in Felty’s syndrome: a case report and literature review. J Hepatol. 2009;50(4):831–5.

    PubMed  Google Scholar 

  20. Lee LA, Sokol RJ, Buyon JP. Hepatobiliary disease in neonatal lupus: prevalence and clinical characteristics in cases enrolled in a national registry. Pediatrics. 2002;109(1):E11.

    PubMed  Google Scholar 

  21. Schoenlebe J, Buyon JP, Zitelli BJ, Friedman D, Greco MA, Knisely AS. Neonatal hemochromatosis associated with maternal autoantibodies against ro/SS-A and la/SS-B ribonucleoproteins. Am J Dis Child. 1993;147(10):1072–5.

    PubMed  CAS  Google Scholar 

  22. Eladawy M, Dominguez SR, Anderson MS, Glode MP. Abnormal liver panel in acute Kawasaki disease. Pediatr Infect Dis J. 2011;30(2):141–4.

    PubMed  PubMed Central  Google Scholar 

  23. Tremoulet AH, Jain S, Chandrasekar D, Sun X, Sato Y, Burns JC. Evolution of laboratory values in patients with Kawasaki disease. Pediatr Infect Dis J. 2011;30(12):1022–6.

    PubMed  PubMed Central  Google Scholar 

  24. Ibanez-Alcalde M, Sanchez-Forte M, Gimenez-Sanchez F, Ortega-Montes A, Martinez-Espinosa G. Cholestasis as the initial feature of Kawasaki disease. Pediatr Infect Dis J. 2012;31(7):766–7.

    PubMed  Google Scholar 

  25. Zulian F, Falcini F, Zancan L, et al. Acute surgical abdomen as presenting manifestation of Kawasaki disease. J Pediatr. 2003;142(6):731–5.

    PubMed  Google Scholar 

  26. Bader-Meunier B, Hadchouel M, Fabre M, Arnoud MD, Dommergues JP. Intrahepatic bile duct damage in children with Kawasaki disease. J Pediatr. 1992;120(5):750–2.

    PubMed  CAS  Google Scholar 

  27. Rostom A, Goldkind L, Laine L. Nonsteroidal anti-inflammatory drugs and hepatic toxicity: a systematic review of randomized controlled trials in arthritis patients. Clin Gastroenterol Hepatol. 2005;3(5):489–98.

    PubMed  CAS  Google Scholar 

  28. Kahn E, Markowitz J, Simpser E, Aiges H, Daum F. Amyloidosis in children with inflammatory bowel disease. J Pediatr Gastroenterol Nutr. 1989;8(4):447–53.

    PubMed  CAS  Google Scholar 

  29. Kocharla L, Taylor J, Weiler T, Ting TV, Luggen M, Brunner HI. Monitoring methotrexate toxicity in juvenile idiopathic arthritis. J Rheumatol. 2009;36(12):2813–8.

    PubMed  CAS  Google Scholar 

  30. Salliot C, van der Heijde D. Long-term safety of methotrexate monotherapy in patients with rheumatoid arthritis: a systematic literature research. Ann Rheum Dis. 2009;68(7):1100–4.

    PubMed  CAS  PubMed Central  Google Scholar 

  31. Hashkes PJ, Balistreri WF, Bove KE, Ballard ET, Passo MH. The long-term effect of methotrexate therapy on the liver in patients with juvenile rheumatoid arthritis. Arthritis Rheum. 1997;40(12):2226–34.

    PubMed  CAS  Google Scholar 

  32. Lahdenne P, Rapola J, Ylijoki H, Haapasaari J. Hepatotoxicity in patients with juvenile idiopathic arthritis receiving longterm methotrexate therapy. J Rheumatol. 2002;29(11):2442–5.

    PubMed  CAS  Google Scholar 

  33. Becker J, Huber WD, Aufricht C. Short- and long-time effects of pediatric liver transplantation on serum cholesterol and triglyceride levels – the Vienna cohort. Pediatr Transplant. 2008;12(8):883–8.

    PubMed  CAS  Google Scholar 

  34. Hashkes PJ, Balistreri WF, Bove KE, Ballard ET, Passo MH. The relationship of hepatotoxic risk factors and liver histology in methotrexate therapy for juvenile rheumatoid arthritis. J Pediatr. 1999;134(1):47–52.

    PubMed  CAS  Google Scholar 

  35. Mahamid M, Paz K, Reuven M, Safadi R. Hepatotoxicity due to tocilizumab and anakinra in rheumatoid arthritis: two case reports. Int J Gen Med. 2011;4:657–60.

    PubMed  CAS  PubMed Central  Google Scholar 

  36. Mimouni A, Magal N, Stoffman N, et al. Familial mediterranean fever: effects of genotype and ethnicity on inflammatory attacks and amyloidosis. Pediatrics. 2000;105(5):E70.

    PubMed  CAS  Google Scholar 

  37. David J, Vouyiouka O, Ansell BM, Hall A, Woo P. Amyloidosis in juvenile chronic arthritis: a morbidity and mortality study. Clin Exp Rheumatol. 1993;11(1):85–90.

    PubMed  CAS  Google Scholar 

  38. Castile R, Shwachman H, Travis W, Hadley CA, Warwick W, Missmahl HP. Amyloidosis as a complication of cystic fibrosis. Am J Dis Child. 1985;139(7):728–32.

    PubMed  CAS  Google Scholar 

  39. Michels H, Linke RP. Clinical benefits of diagnosing incipient AA amyloidosis in pediatric rheumatic diseases as estimated from a retrospective study. Amyloid. 1998;5(3):200–7.

    PubMed  CAS  Google Scholar 

  40. Stangou AJ, Hawkins PN. Liver transplantation in transthyretin-related familial amyloid polyneuropathy. Curr Opin Neurol. 2004;17(5):615–20.

    PubMed  Google Scholar 

  41. Llado L, Baliellas C, Casasnovas C, et al. Risk of transmission of systemic transthyretin amyloidosis after domino liver transplantation. Liver Transpl. 2010;16(12):1386–92.

    PubMed  Google Scholar 

  42. Sarnaik S, Slovis TL, Corbett DP, Emami A, Whitten CF. Incidence of cholelithiasis in sickle cell anemia using the ultrasonic gray-scale technique. J Pediatr. 1980;96(6):1005–8.

    PubMed  CAS  Google Scholar 

  43. Schubert TT. Hepatobiliary system in sickle cell disease. Gastroenterology. 1986;90(6):2013–21.

    PubMed  CAS  Google Scholar 

  44. Tripathi A, Jerrell JM, Stallworth JR. Clinical complications in severe pediatric sickle cell disease and the impact of hydroxyurea. Pediatr Blood Cancer. 2011;56(1):90–4.

    PubMed  Google Scholar 

  45. Haberkern CM, Neumayr LD, Orringer EP, et al. Cholecystectomy in sickle cell anemia patients: perioperative outcome of 364 cases from the National Preoperative Transfusion study. Preoperative Transfusion in Sickle Cell Disease Study Group. Blood. 1997;89(5):1533–42.

    PubMed  CAS  Google Scholar 

  46. Ahn H, Li CS, Wang W. Sickle cell hepatopathy: clinical presentation, treatment, and outcome in pediatric and adult patients. Pediatr Blood Cancer. 2005;45(2):184–90.

    PubMed  Google Scholar 

  47. Traina F, Jorge SG, Yamanaka A, de Meirelles LR, Costa FF, Saad ST. Chronic liver abnormalities in sickle cell disease: a clinicopathological study in 70 living patients. Acta Haematol. 2007;118(3):129–35.

    PubMed  Google Scholar 

  48. Hatton CS, Bunch C, Weatherall DJ. Hepatic sequestration in sickle cell anaemia. Br Med J (Clin Res Ed). 1985;290(6470):744–5.

    CAS  Google Scholar 

  49. Baichi MM, Arifuddin RM, Mantry PS, Bozorgzadeh A, Ryan C. Liver transplantation in sickle cell anemia: a case of acute sickle cell intrahepatic cholestasis and a case of sclerosing cholangitis. Transplantation. 2005;80(11):1630–2.

    PubMed  Google Scholar 

  50. Gilli SC, Boin IF, Sergio Leonardi L, Luzo AC, Costa FF, Saad ST. Liver transplantation in a patient with S(beta)o-thalassemia. Transplantation. 2002;74(6):896–8.

    PubMed  Google Scholar 

  51. Hankins JS, Smeltzer MP, McCarville MB, et al. Patterns of liver iron accumulation in patients with sickle cell disease and thalassemia with iron overload. Eur J Haematol. 2010;85(1):51–7.

    PubMed  CAS  PubMed Central  Google Scholar 

  52. Karam LB, Disco D, Jackson SM, et al. Liver biopsy results in patients with sickle cell disease on chronic transfusions: poor correlation with ferritin levels. Pediatr Blood Cancer. 2008;50(1):62–5.

    PubMed  Google Scholar 

  53. Brown K, Subramony C, May W, et al. Hepatic iron overload in children with sickle cell anemia on chronic transfusion therapy. J Pediatr Hematol Oncol. 2009;31(5):309–12.

    PubMed  CAS  Google Scholar 

  54. Porter JB. Pathophysiology of transfusional iron overload: contrasting patterns in thalassemia major and sickle cell disease. Hemoglobin. 2009;33 Suppl 1:S37–45.

    PubMed  CAS  Google Scholar 

  55. Ghugre NR, Gonzalez-Gomez I, Butensky E, et al. Patterns of hepatic iron distribution in patients with chronically transfused thalassemia and sickle cell disease. Am J Hematol. 2009;84(8):480–3.

    PubMed  CAS  PubMed Central  Google Scholar 

  56. Liver transplants in the U.S. by recipient age. 1 Jan 1988–30 Apr 2012. http://optn.transplant.hrsa.gov/latestData/viewDataReports.asp. Updated 2012.

  57. Meerpohl JJ, Antes G, Rucker G, et al. Deferasirox for managing iron overload in people with thalassaemia. Cochrane Database Syst Rev. 2012;(2):CD007476.

    Google Scholar 

  58. Linabery AM, Ross JA. Trends in childhood cancer incidence in the U.S. (1992–2004). Cancer. 2008;112(2):416–32.

    PubMed  Google Scholar 

  59. Salmon JS, Thompson MA, Arildsen RC, Greer JP. Non-Hodgkin’s lymphoma involving the liver: clinical and therapeutic considerations. Clin Lymphoma Myeloma. 2006;6(4):273–80.

    PubMed  Google Scholar 

  60. Miller ST, Wollner N, Meyers PA, Exelby P, Jereb B, Miller DR. Primary hepatic or hepatosplenic non-Hodgkin’s lymphoma in children. Cancer. 1983;52(12):2285–8.

    PubMed  CAS  Google Scholar 

  61. Kotlyar DS, Osterman MT, Diamond RH, et al. A systematic review of factors that contribute to hepatosplenic T-cell lymphoma in patients with inflammatory bowel disease. Clin Gastroenterol Hepatol. 2011;9(1):36–41.e1.

    PubMed  CAS  Google Scholar 

  62. Rawat D, Gillett PM, Devadason D, Wilson DC, McKiernan PJ. Long-term follow-up of children with 6-thioguanine-related chronic hepatotoxicity following treatment for acute lymphoblastic leukaemia. J Pediatr Gastroenterol Nutr. 2011;53(5):478–9.

    PubMed  CAS  Google Scholar 

  63. Escherich G, Richards S, Stork LC, Vora AJ, Childhood Acute Lymphoblastic Leukaemia Collaborative Group (CALLCG). Meta-analysis of randomised trials comparing thiopurines in childhood acute lymphoblastic leukaemia. Leukemia. 2011;25(6):953–9.

    PubMed  CAS  PubMed Central  Google Scholar 

  64. Vora A, Mitchell CD, Lennard L, et al. Toxicity and efficacy of 6-thioguanine versus 6-mercaptopurine in childhood lymphoblastic leukaemia: a randomised trial. Lancet. 2006;368(9544):1339–48.

    PubMed  CAS  Google Scholar 

  65. Ball LM, Egeler RM, EBMT Paediatric Working Party. Acute GvHD: pathogenesis and classification. Bone Marrow Transplant. 2008;41 Suppl 2:S58–64.

    PubMed  CAS  Google Scholar 

  66. Bertheau P, Hadengue A, Cazals-Hatem D, et al. Chronic cholestasis in patients after allogeneic bone marrow transplantation: several diseases are often associated. Bone Marrow Transplant. 1995;16(2):261–5.

    PubMed  CAS  Google Scholar 

  67. Ferrell LD, Kakar S. Liver pathology. In: Elder DE, editor. Consultant pathology series. 4th ed. New York: Demos Medical Publishing; 2011. p. 340–2.

    Google Scholar 

  68. Shulman HM, Sharma P, Amos D, Fenster LF, McDonald GB. A coded histologic study of hepatic graft-versus-host disease after human bone marrow transplantation. Hepatology. 1988;8(3):463–70.

    PubMed  CAS  Google Scholar 

  69. Baird K, Cooke K, Schultz KR. Chronic graft-versus-host disease (GVHD) in children. Pediatr Clin North Am. 2010;57(1):297–322.

    PubMed  PubMed Central  Google Scholar 

  70. Melin-Aldana H, Thormann K, Duerst R, Kletzel M, Jacobsohn DA. Hepatitic pattern of graft versus host disease in children. Pediatr Blood Cancer. 2007;49(5):727–30.

    PubMed  Google Scholar 

  71. Barshes NR, Myers GD, Lee D, et al. Liver transplantation for severe hepatic graft-versus-host disease: an analysis of aggregate survival data. Liver Transpl. 2005;11(5):525–31.

    PubMed  Google Scholar 

  72. Messina C, Faraci M, de Fazio V, et al. Prevention and treatment of acute GvHD. Bone Marrow Transplant. 2008;41 Suppl 2:S65–70.

    PubMed  CAS  Google Scholar 

  73. Risma K, Jordan MB. Hemophagocytic lymphohistiocytosis: updates and evolving concepts. Curr Opin Pediatr. 2012;24(1):9–15.

    PubMed  CAS  Google Scholar 

  74. Jordan MB, Allen CE, Weitzman S, Filipovich AH, McClain KL. How I treat hemophagocytic lymphohistiocytosis. Blood. 2011;118(15):4041–52.

    PubMed  CAS  PubMed Central  Google Scholar 

  75. Parizhskaya M, Reyes J, Jaffe R. Hemophagocytic syndrome presenting as acute hepatic failure in two infants: clinical overlap with neonatal hemochromatosis. Pediatr Dev Pathol. 1999;2(4):360–6.

    PubMed  CAS  Google Scholar 

  76. Suzuki N, Morimoto A, Ohga S, et al. Characteristics of hemophagocytic lymphohistiocytosis in neonates: a nationwide survey in Japan. J Pediatr. 2009;155(2):235–8.e1.

    PubMed  Google Scholar 

  77. Chen JH, Fleming MD, Pinkus GS, et al. Pathology of the liver in familial hemophagocytic lymphohistiocytosis. Am J Surg Pathol. 2010;34(6):852–67.

    PubMed  Google Scholar 

  78. Wright G, Wilmore S, Makanyanga J, et al. Liver transplant for adult hemophagocytic lymphohistiocytosis: case report and literature review. Exp Clin Transplant. 2012;10(5):508–12.

    PubMed  Google Scholar 

  79. Ravelli A, Grom AA, Behrens EM, Cron RQ. Macrophage activation syndrome as part of systemic juvenile idiopathic arthritis: diagnosis, genetics, pathophysiology and treatment. Genes Immun. 2012;13(4):289–98.

    PubMed  CAS  Google Scholar 

  80. LaFranchi SH, Murphey WH, Foley Jr TP, Larsen PR, Buist NR. Neonatal hypothyroidism detected by the northwest regional screening program. Pediatrics. 1979;63(2):180–91.

    PubMed  CAS  Google Scholar 

  81. Rastogi MV, LaFranchi SH. Congenital hypothyroidism. Orphanet J Rare Dis. 2010;5:17. doi:10.1186/1750-1172-5-17.

    PubMed  PubMed Central  Google Scholar 

  82. Arab DM, Malatjalian DA, Rittmaster RS. Severe cholestatic jaundice in uncomplicated hyperthyroidism treated with methimazole. J Clin Endocrinol Metab. 1995;80(4):1083–5.

    PubMed  CAS  Google Scholar 

  83. Elias RM, Dean DS, Barsness GW. Hepatic dysfunction in hospitalized patients with acute thyrotoxicosis: a decade of experience. ISRN Endocrinol. 2012;2012:325092.

    PubMed  PubMed Central  Google Scholar 

  84. Yao JD, Gross Jr JB, Ludwig J, Purnell DC. Cholestatic jaundice in hyperthyroidism. Am J Med. 1989;86(5):619–20.

    PubMed  CAS  Google Scholar 

  85. Karnsakul W, Sawathiparnich P, Nimkarn S, Likitmaskul S, Santiprabhob J, Aanpreung P. Anterior pituitary hormone effects on hepatic functions in infants with congenital hypopituitarism. Ann Hepatol. 2007;6(2):97–103.

    PubMed  CAS  Google Scholar 

  86. Binder G, Martin DD, Kanther I, Schwarze CP, Ranke MB. The course of neonatal cholestasis in congenital combined pituitary hormone deficiency. J Pediatr Endocrinol Metab. 2007;20(6):695–702.

    PubMed  CAS  Google Scholar 

  87. Al-Hussaini A, Almutairi A, Mursi A, Alghofely M, Asery A. Isolated cortisol deficiency: a rare cause of neonatal cholestasis. Saudi J Gastroenterol. 2012;18(5):339–41.

    PubMed  PubMed Central  Google Scholar 

  88. Sheehan AG, Martin SR, Stephure D, Scott RB. Neonatal cholestasis, hypoglycemia, and congenital hypopituitarism. J Pediatr Gastroenterol Nutr. 1992;14(4):426–30.

    PubMed  CAS  Google Scholar 

  89. Hauser GJ, Brotzman HM, Kaufman SS. Hepatoadrenal syndrome in pediatric patients with end-stage liver disease. Pediatr Crit Care Med. 2012;13(3):e145–9.

    PubMed  Google Scholar 

  90. Hogler W, Baumann U, Kelly D. Endocrine and bone metabolic complications in chronic liver disease and after liver transplantation in children. J Pediatr Gastroenterol Nutr. 2012;54(3):313–21.

    PubMed  Google Scholar 

  91. O’Beirne J, Holmes M, Agarwal B, et al. Adrenal insufficiency in liver disease – what is the evidence? J Hepatol. 2007;47(3):418–23.

    PubMed  Google Scholar 

  92. Fede G, Spadaro L, Tomaselli T, et al. Adrenocortical dysfunction in liver disease: a systematic review. Hepatology. 2012;55(4):1282–91.

    PubMed  CAS  Google Scholar 

  93. McNeilly AD, Macfarlane DP, O’Flaherty E, et al. Bile acids modulate glucocorticoid metabolism and the hypothalamic-pituitary-adrenal axis in obstructive jaundice. J Hepatol. 2010;52(5):705–11.

    PubMed  CAS  PubMed Central  Google Scholar 

  94. Swain MG, Patchev V, Vergalla J, Chrousos G, Jones EA. Suppression of hypothalamic-pituitary-adrenal axis responsiveness to stress in a rat model of acute cholestasis. J Clin Invest. 1993;91(5):1903–8.

    PubMed  CAS  PubMed Central  Google Scholar 

  95. Torbenson M, Chen YY, Brunt E, et al. Glycogenic hepatopathy: an underrecognized hepatic complication of diabetes mellitus. Am J Surg Pathol. 2006;30(4):508–13.

    PubMed  Google Scholar 

  96. Lorenz G, Barenwald G. Histologic and electron-microscopic liver changes in diabetic children. Acta Hepatogastroenterol (Stuttg). 1979;26(6):435–8.

    CAS  Google Scholar 

  97. Hudacko RM, Manoukian AV, Schneider SH, Fyfe B. Clinical resolution of glycogenic hepatopathy following improved glycemic control. J Diabetes Complications. 2008;22(5):329–30.

    PubMed  Google Scholar 

  98. Murata F, Horie I, Ando T, et al. A case of glycogenic hepatopathy developed in a patient with new-onset fulminant type 1 diabetes: the role of image modalities in diagnosing hepatic glycogen deposition including gradient-dual-echo MRI. Endocr J. 2012;59(8):669–76.

    PubMed  Google Scholar 

  99. Fridell JA, Saxena R, Chalasani NP, Goggins WC, Powelson JA, Cummings OW. Complete reversal of glycogen hepatopathy with pancreas transplantation: two cases. Transplantation. 2007;83(1):84–6.

    PubMed  Google Scholar 

  100. Hussain N, Feld JJ, Kleiner DE, et al. Hepatic abnormalities in patients with chronic granulomatous disease. Hepatology. 2007;45(3):675–83.

    PubMed  Google Scholar 

  101. Winkelstein JA, Marino MC, Johnston Jr RB, et al. Chronic granulomatous disease. Report on a national registry of 368 patients. Medicine (Baltimore). 2000;79(3):155–69.

    CAS  Google Scholar 

  102. Marchetti F, Taddio A, L’Erario I, Ventura A. A 7-month-old boy with liver abscesses. J Pediatr Gastroenterol Nutr. 2010;50(2):117.

    PubMed  Google Scholar 

  103. Chen LE, Minkes RK, Shackelford PG, Strasberg SM, Kuo EY, Langer JC. Cut it out: managing hepatic abscesses in patients with chronic granulomatous disease. J Pediatr Surg. 2003;38(5):709–13.

    PubMed  Google Scholar 

  104. Feld JJ, Hussain N, Wright EC, et al. Hepatic involvement and portal hypertension predict mortality in chronic granulomatous disease. Gastroenterology. 2008;134(7):1917–26.

    PubMed  PubMed Central  Google Scholar 

  105. Bianchi G, Marchesini G, Marzocchi R, Pinna AD, Zoli M. Metabolic syndrome in liver transplantation: relation to etiology and immunosuppression. Liver Transpl. 2008;14(11):1648–54.

    PubMed  Google Scholar 

  106. Ward C, Lucas M, Piris J, Collier J, Chapel H. Abnormal liver function in common variable immunodeficiency disorders due to nodular regenerative hyperplasia. Clin Exp Immunol. 2008;153(3):331–7.

    PubMed  CAS  PubMed Central  Google Scholar 

  107. Malamut G, Ziol M, Suarez F, et al. Nodular regenerative hyperplasia: the main liver disease in patients with primary hypogammaglobulinemia and hepatic abnormalities. J Hepatol. 2008;48(1):74–82.

    PubMed  Google Scholar 

  108. Cunningham-Rundles C. How I, treat common variable immune deficiency. Blood. 2010;116(1):7–15.

    PubMed  CAS  PubMed Central  Google Scholar 

  109. Debray D, Pariente D, Urvoas E, Hadchouel M, Bernard O. Sclerosing cholangitis in children. J Pediatr. 1994;124(1):49–56.

    PubMed  CAS  Google Scholar 

  110. Daniels JA, Torbenson M, Vivekanandan P, Anders RA, Boitnott JK. Hepatitis in common variable immunodeficiency. Hum Pathol. 2009;40(4):484–8.

    PubMed  Google Scholar 

  111. Levy J, Espanol-Boren T, Thomas C, et al. Clinical spectrum of X-linked hyper-IgM syndrome. J Pediatr. 1997;131(1 Pt 1):47–54.

    PubMed  CAS  Google Scholar 

  112. Hayward AR, Levy J, Facchetti F, et al. Cholangiopathy and tumors of the pancreas, liver, and biliary tree in boys with X-linked immunodeficiency with hyper-IgM. J Immunol. 1997;158(2):977–83.

    PubMed  CAS  Google Scholar 

  113. Jesus AA, Duarte AJ, Oliveira JB. Autoimmunity in hyper-IgM syndrome. J Clin Immunol. 2008;28 Suppl 1:S62–6.

    PubMed  CAS  Google Scholar 

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Correspondence to Philip Rosenthal MD .

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© 2014 Springer Science+Business Media New York

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Perito, E.R., Rosenthal, P. (2014). Liver in Systemic Disease. In: Murray, K., Horslen, S. (eds) Diseases of the Liver in Children. Springer, New York, NY. https://doi.org/10.1007/978-1-4614-9005-0_29

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  • DOI: https://doi.org/10.1007/978-1-4614-9005-0_29

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