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April 1977, Vol 131, No. 4 >
Article | April 1977

Cholangiocarcinoma Associated With Biliary Cirrhosis Due to Congenital Biliary Atresia FREE

Prakash B. Kulkarni, MB BS, DCH; Eugene C. Beatty, MD
[+] Author Affiliations

Reprint requests to Department of Pediatrics, Children's Mercy Hospital, 24th and Gillham Rd, Kansas City, MO 64108 (Dr Kulkarni).


Am J Dis Child. 1977;131(4):442-444. doi:10.1001/archpedi.1977.02120170068013.
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• An 11-year-old girl developed cholangiocellular carcinoma in association with biliary cirrhosis due to congenital biliary atresia. An exploratory laparotomy and an operative cholangiogram at 3 months of age had confirmed the diagnosis of extra-hepatic biliary atresia. A liver biopsy specimen taken at 6 months of age showed biliary cirrhosis. The subsequent clinical course was characterized by persistent moderate jaundice, anemia, malnutrition, rickets, pathologic fractures, and recurrent gastrointestinal bleeding. The presence of cholangiocellular carcinoma of the liver with advanced biliary cirrhosis was established at an exploratory laparotomy a week before her death. We discuss here the pathogenesis of biliary cirrhosis and carcinoma of the liver; there may be a relation between the two in the childhood population.

(Am J Dis Child 131:442-444, 1977)

REFERENCES

1 +
Silverman A: Pediatric Clinical Gastroenterology . St Louis, CV Mosby Co, 1971;, p 293.
2 +
Thaler M, Gellis S:  Studies in neonatal hepatitis and biliary atresia in diagnosis . Am J Dis Child 116:257-261, 1968;.
3 +
Koop CE:  Biliary atresia and Kasai operation: Commentary . Pediatrics 55:9-11, 1975;.
4 +
Lilly JR:  The Japanese operation for biliary atresia . Pediatrics 55:12-19, 1975;.
5 +
Van Wyks J, Halgrimson CG, Giles G, et al:  Liver transplantation in biliary atresia with concomitant hepatoma . S Afr Med J 46:885-889, 1972;.
6 +
Craig JM, Gellis SS, Hsia DY:  Cirrhosis of liver in infants and children . Am J Dis Child 90:229-322, 1955;.
7 +
Gall EA:  Tumors of the liver , in Diseases of the Liver , ed 2. Philadelphia, JB Lippincott Co, 1963;, p 702.
8 +
Mitchell AG, Nelson WE: Textbook of Pediatrics . Philadelphia, WB Saunders Co, 1946;, p 678.
9 +
Packard GB, Palmer HD:  Primary neoplasm of the liver in infants and children . Ann Surg 142:214-227, 1955;.
10 +
Gall EA:  Primary and metastatic carcinoma of the liver: Relationship to hepatic cirrhosis . Arch Pathol 70:226-232, 1960;.
11 +
Cameron R, Hou PC: Biliary Cirrhosis . Pathologic monograph 1, Springfield, Ill, Charles C Thomas Publisher, 1962;, p 121.
12 +
Mori W:  Cirrhosis and primary cancer of the liver . Cancer 20:627-631, 1967;.
13 +
Alcalde VM, Truisman HS, Baffes T:  Primary carcinoma of the liver in infancy and childhood . Am J Dis Child 104:245-251, 1962;.
14 +
Okayama K:  Primary liver cell carcinoma associated with biliary cirrhosis due to congenital bile duct atresia . Pediatrics 67:89-93, 1965;.
15 +
Absolon KB, Rikkers H, Aust JB:  Thoracic duct lymph drainage in congenital biliary atresia . Surg Gynecol Obstet 120:123-127, 1965;.
16 +
Fish JC, McCary RG:  Primary cancer of the liver in childhood . Arch Surg 93:355-359, 1966;.
17 +
Deores MP, Dicus W:  Hepatocarcinoma associated with biliary cirrhosis: A case due to congenital bile duct atresia . Arch Pathol 86:338-341, 1968;.
18 +
Fraumeni JF, Miller RW, Hill JA:  Primary carcinoma of the liver in childhood: An epidemiologic study . J Natl Cancer Inst 40:1087-1099, 1968;.
19 +
Roth D, Duncan A:  Primary carcinoma of the liver after giant cell hepatitis of infancy . Cancer 8:986-991, 1955;.
20 +
Fajers CM, Falkmers FE, Pehrson M:  Primary carcinoma of the liver and giant cell hepatitis in infancy . Acta Paediatr 49:96-110, 1960;.
21 +
Landing BH:  Consideration of the pathogenesis of neonatal hepatitis, biliary atresia and choledochal cysts: The concept of infantile obstructive cholangiopathy . Prog Pediatr Surg 6:113-139, 1974;.
22 +
Mason HH, Anderson DH:  Glycogen disease of the liver (von Gierke's disease) with hepatoma: Case report with metabolic studies . Pediatrics 16:785-799, 1955;.
23 +
Zengeneh F, Limbeck GA, Kelley VC: Type I glycogen storage (GSD) and malignant hepatoma. Read before the annual meeting of the American Pediatric Society, Atlantic City, NJ, April 26 -29, 1952.
24 +
Edmonds AM, Henninger GR, Crooks R: Galactosemia: Report of a case with autopsy. Pediatrics 10:40-47, 1952.
25 +
Ein SH, Stephens CA:  Malignant liver tumors in children . J Pediatr Surg 9:491-494, 1974;.
26 +
Brires A:  Primary tumors of the liver . Am J Clin Pathol 3:221-235, 1933;.
27 +
Hoyne RM, Kernohun JN:  Primary carcinoma of the liver: A study of 31 cases . Arch Intern Med 79:532-551, 1947;.
28 +
Steiner M:  Primary carcinoma of the liver in childhood . Am J Dis Child 55:807-827, 1938;.
29 +
Bigelow N, Wright A:  Primary carcinoma of liver in infancy and childhood . Cancer 6:170-178, 1953;.
30 +
Jones E:  Primary carcinoma of the liver with associated cirrhosis in infants and children . Arch Pathol 70:5-11, 1960;.

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Country-Specific Mortality and Growth Failure in Infancy and Yound Children and Association With Material Stature

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References

1 +
Silverman A: Pediatric Clinical Gastroenterology . St Louis, CV Mosby Co, 1971;, p 293.
2 +
Thaler M, Gellis S:  Studies in neonatal hepatitis and biliary atresia in diagnosis . Am J Dis Child 116:257-261, 1968;.
3 +
Koop CE:  Biliary atresia and Kasai operation: Commentary . Pediatrics 55:9-11, 1975;.
4 +
Lilly JR:  The Japanese operation for biliary atresia . Pediatrics 55:12-19, 1975;.
5 +
Van Wyks J, Halgrimson CG, Giles G, et al:  Liver transplantation in biliary atresia with concomitant hepatoma . S Afr Med J 46:885-889, 1972;.
6 +
Craig JM, Gellis SS, Hsia DY:  Cirrhosis of liver in infants and children . Am J Dis Child 90:229-322, 1955;.
7 +
Gall EA:  Tumors of the liver , in Diseases of the Liver , ed 2. Philadelphia, JB Lippincott Co, 1963;, p 702.
8 +
Mitchell AG, Nelson WE: Textbook of Pediatrics . Philadelphia, WB Saunders Co, 1946;, p 678.
9 +
Packard GB, Palmer HD:  Primary neoplasm of the liver in infants and children . Ann Surg 142:214-227, 1955;.
10 +
Gall EA:  Primary and metastatic carcinoma of the liver: Relationship to hepatic cirrhosis . Arch Pathol 70:226-232, 1960;.
11 +
Cameron R, Hou PC: Biliary Cirrhosis . Pathologic monograph 1, Springfield, Ill, Charles C Thomas Publisher, 1962;, p 121.
12 +
Mori W:  Cirrhosis and primary cancer of the liver . Cancer 20:627-631, 1967;.
13 +
Alcalde VM, Truisman HS, Baffes T:  Primary carcinoma of the liver in infancy and childhood . Am J Dis Child 104:245-251, 1962;.
14 +
Okayama K:  Primary liver cell carcinoma associated with biliary cirrhosis due to congenital bile duct atresia . Pediatrics 67:89-93, 1965;.
15 +
Absolon KB, Rikkers H, Aust JB:  Thoracic duct lymph drainage in congenital biliary atresia . Surg Gynecol Obstet 120:123-127, 1965;.
16 +
Fish JC, McCary RG:  Primary cancer of the liver in childhood . Arch Surg 93:355-359, 1966;.
17 +
Deores MP, Dicus W:  Hepatocarcinoma associated with biliary cirrhosis: A case due to congenital bile duct atresia . Arch Pathol 86:338-341, 1968;.
18 +
Fraumeni JF, Miller RW, Hill JA:  Primary carcinoma of the liver in childhood: An epidemiologic study . J Natl Cancer Inst 40:1087-1099, 1968;.
19 +
Roth D, Duncan A:  Primary carcinoma of the liver after giant cell hepatitis of infancy . Cancer 8:986-991, 1955;.
20 +
Fajers CM, Falkmers FE, Pehrson M:  Primary carcinoma of the liver and giant cell hepatitis in infancy . Acta Paediatr 49:96-110, 1960;.
21 +
Landing BH:  Consideration of the pathogenesis of neonatal hepatitis, biliary atresia and choledochal cysts: The concept of infantile obstructive cholangiopathy . Prog Pediatr Surg 6:113-139, 1974;.
22 +
Mason HH, Anderson DH:  Glycogen disease of the liver (von Gierke's disease) with hepatoma: Case report with metabolic studies . Pediatrics 16:785-799, 1955;.
23 +
Zengeneh F, Limbeck GA, Kelley VC: Type I glycogen storage (GSD) and malignant hepatoma. Read before the annual meeting of the American Pediatric Society, Atlantic City, NJ, April 26 -29, 1952.
24 +
Edmonds AM, Henninger GR, Crooks R: Galactosemia: Report of a case with autopsy. Pediatrics 10:40-47, 1952.
25 +
Ein SH, Stephens CA:  Malignant liver tumors in children . J Pediatr Surg 9:491-494, 1974;.
26 +
Brires A:  Primary tumors of the liver . Am J Clin Pathol 3:221-235, 1933;.
27 +
Hoyne RM, Kernohun JN:  Primary carcinoma of the liver: A study of 31 cases . Arch Intern Med 79:532-551, 1947;.
28 +
Steiner M:  Primary carcinoma of the liver in childhood . Am J Dis Child 55:807-827, 1938;.
29 +
Bigelow N, Wright A:  Primary carcinoma of liver in infancy and childhood . Cancer 6:170-178, 1953;.
30 +
Jones E:  Primary carcinoma of the liver with associated cirrhosis in infants and children . Arch Pathol 70:5-11, 1960;.

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