Pancreas histology in Shwachman-Diamond syndrome

A pancreatic duct is shown (center).
(A) Pancreatic biopsy in a six-year-old boy with Shwachman-Diamond syndrome shows extensive replacement of the exocrine pancreas by adipose tissue with remaining small islands of parenchyma.
(B) Extensive fat surrounding acini (large arrows) with poorly granulated exocrine cells and naked islets (small arrows).