Clinically inapparent persistent chronic liver disease in a 15-month-old male patient with Shwachman-Diamond syndrome is presented. Cryptic hepatic involvement may be an unrecognized feature of the syndrome and should be evaluated in all cases.
Get full access to this article
View all access options for this article.
References
1.
Shwachman H. , Diamond LK, Oski FA, Khaw KT: The syndrome of pancreatic insufficiency and bone marrow dynsfunction. J Pediatr65:645, 1964
2.
Bodian M., Sheldon W., Lightwood R.: Congenital hypoplasia of the exocrine pancreas. Acta Paediatr Uppsala53:282, 1964
3.
Goldstein R. : Congenital lipomatosis of the pancreas. Malabsorption, dwarfism, leukopenia with relative granulocytopenia and thrombocytopenia. Clin Pediatr7:419, 1968
4.
Colebatch JH , Anderson CM, Simons MJ, et al: Neutropenia and pancreatic disorder . Lancet2: 496, 1965
5.
Burke V., Colebatch JH, Anderson CM, Simons MJ: Association of pancreatic insufficiency and chronic neutropenia in childhood . Arch Dis Child42: 147, 1967
6.
Giedion A., Prader A., Hadorn B., et al: Metaphysare dystose und angeborene pankreasinsuffizien. Forstarchiv Röntgenstr108:51, 1968
7.
Shmerling DH , Prader A., Hitzig WH, et al: The syndrome of exocrine pancreatic insufficiency, neutropenia, metaphysealdysostosis and dwarfism. Helv Paediatr Acta24:547, 1969
8.
Brueton MJ, Mavromichalis J., Goodchild MC, Anderson CM: Hepatic dysfunction in association with pancreatic insufficiency and cyclical neutropenia. Shwachman-Diamond syndrome. Arch Dis Child52:76, 1977
9.
Pringle EM, Young WF, Haworth EM: Syndrome of pancreatic insufficiency, blood dyscrasia and metaphyseal dysostosis . Proc Roy Soc Med61: 776, 1968
10.
Havlíková D. , Vychytil O., Jelínek J.: The syndrome of congenital pancreatic insufficiency, chronic respiratory disease and chronic liver damage. Acta Paeditr Scand56:676, 1967
