{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,27]],"date-time":"2026-09-27T06:25:39Z","timestamp":1790490339317,"version":"4.1.0"},"reference-count":9,"publisher":"BMJ","issue":"1","content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Archives of Disease in Childhood"],"published-print":{"date-parts":[[1977,1,1]]},"DOI":"10.1136\/adc.52.1.76","type":"journal-article","created":{"date-parts":[[2008,12,22]],"date-time":"2008-12-22T16:14:24Z","timestamp":1229962464000},"page":"76-78","source":"Crossref","is-referenced-by-count":24,"title":["Hepatic dysfunction in association with pancreatic insufficiency and cyclical neutropenia. Shwachman-Diamond syndrome."],"prefix":"10.1136","volume":"52","author":[{"given":"M J","family":"Brueton","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"J","family":"Mavromichalis","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"M C","family":"Goodchild","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"C M","family":"Anderson","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"239","reference":[{"key":"ref_1","doi-asserted-by":"crossref","first-page":"349","DOI":"10.4269\/ajtmh.1970.19.349","article-title":"A new technique for sampling duodenal contents","volume":"19","author":"Beal, C.B.; Viens, P.; Grant, R.G.L.; Hughes, J.M.","year":"1970","unstructured":"Beal, C. B., Viens, P., Grant, R. G. L., and Hughes, J. M. (1970). A new technique for sampling duodenal contents. American Journal of Tropical Medicine and Hygiene, 19, 349-352.","journal-title":"American Journal of Tropical Medicine and Hygiene"},{"key":"ref_2","article-title":"notches have been noted. Dys-y-globulinaemia was next noted by Goldstein","volume-title":"and has been described in 5 further patients (Doe, 1973), in each case involving a reduction in the serum concentrations","year":"1968","unstructured":"notches have been noted. Dys-y-globulinaemia was next noted by Goldstein (1968) and has been described in 5 further patients (Doe, 1973), in each case involving a reduction in the serum concentrations"},{"key":"ref_3","doi-asserted-by":"crossref","first-page":"215","DOI":"10.1111\/j.1445-5994.1972.tb03065.x","article-title":"Small-intestinal microflora in Australian Aboriginal children with chronic diarrhoea","volume":"3","author":"Gracey, M.; Stone, D.E.","year":"1972","unstructured":"Gracey, M., and Stone, D. E. (1972). Small-intestinal microflora in Australian Aboriginal children with chronic diarrhoea. Australian and New Zealand Journal of Medicine, 3, 215-219.","journal-title":"Australian and New Zealand Journal of Medicine"},{"key":"ref_4","doi-asserted-by":"crossref","first-page":"1170","DOI":"10.1093\/ajcn\/26.11.1170","article-title":"Microbial contamination of the gut: another feature of malnutrition","volume":"26","author":"Gracey, M.; Suharjono, Sunoto; Stone, D.E.","year":"1973","unstructured":"Gracey, M., Suharjono, Sunoto, and Stone, D. E. (1973). Microbial contamination of the gut: another feature of malnutrition. 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A report of the Wellcome Trust International Working Party, 2, 302-303.","journal-title":"Party"},{"key":"ref_7","doi-asserted-by":"crossref","first-page":"1118","DOI":"10.1093\/ajcn\/25.10.1118","article-title":"Gastrointestinal flora of children with protein-calorie malnutrition","volume":"25","author":"Mata, L.J.; Jimenez, F.; Cord6n, M.; Rosales, R.; Prera, E.; Schneider, R.E.; Viteri, F.","year":"1972","unstructured":"Mata, L. J., Jimenez, F., Cord6n, M., Rosales, R., Prera, E., Schneider, R. E., and Viteri, F. (1972). Gastrointestinal flora of children with protein-calorie malnutrition. American Journal of Clinical Nutrition, 25, 1118-1126.","journal-title":"American Journal of Clinical Nutrition"},{"key":"ref_8","article-title":"of one or more of the immunoglobulins. As far as we can determine only one living patient with histological but not clinical evidence of liver dysfunction has previously been described (Bodian et al., 1964). In a retrospective review of 18 children whose necropsies had shown exocrine hypoplasia and lipomatosis of the pancreas they noted that 11 had shown a fatty liver or cirrhosis. Cyclical neutropenia had not been shown in life in any of them","year":"1967","unstructured":"of one or more of the immunoglobulins. As far as we can determine only one living patient with histological but not clinical evidence of liver dysfunction has previously been described (Bodian et al., 1964). In a retrospective review of 18 children whose necropsies had shown exocrine hypoplasia and lipomatosis of the pancreas they noted that 11 had shown a fatty liver or cirrhosis. Cyclical neutropenia had not been shown in life in any of them. Higashi et al. (1967) observed inflammatory cell infiltration in the periportal tissue of the liver obtained at necropsy in a 2-year-old child with pancreatic insufficiency and cyclical neutropenia. We presently have 4 children with this syndrome under our care as detailed in the Table. One of them (Case 1) has clinical evidence of hepatic","journal-title":"Higashi et al; observed inflammatory cell infiltration in the periportal tissue of the liver obtained at necropsy in a 2-year-old child with pancreatic insufficiency and cyclical neutropenia. We presently have 4 children with this syndrome under our care as detailed in the Table. One of them (Case 1) has clinical evidence of hepatic"},{"key":"ref_9","doi-asserted-by":"crossref","first-page":"282","DOI":"10.1111\/j.1651-2227.1964.tb07237.x","article-title":"Congenital hypoplasia of the exocrine pancreas","volume":"53","author":"Bodian, M.; Sheldon, W.; Lightwood, R.","year":"1964","unstructured":"Bodian, M., Sheldon, W., and Lightwood, R. (1964). Congenital hypoplasia of the exocrine pancreas. 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