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- W2094578085 abstract "Summary Two cases of congenital familial steatorrhea with fibromatosis of the pancreas and bronchiolectasis are presented, one with post-mortem findings. The past and present concepts of the terms “steatorrhea” and the “celiac syndrome” are described. The steatorrheas are classified as the idiopathic variety, including celiac disease, and the pancreatogenous. Into the latter division fall most of the cases reported in the literature, which begin soon after birth, are refractory to treatment, and show characteristic post-mortem changes in the pancreas. This includes one and presumably the other of our cases. Suggestions for the recognition of these cases are offered. The association of respiratory symptoms and pulmonary findings with the steatorrheas is discussed, and the reasons for considering the pathologic phenomena of both pancreas and bronchi to be based on congenital anomalies are given. The appearance of symptoms from birth, the familial incidence of the disease, the characteristic pertussis-like cough, the presence of transitory glycosuria, and the finding of fatty changes in the liver are mentioned." @default.
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- W2094578085 date "1939-04-01" @default.
- W2094578085 modified "2023-09-23" @default.
- W2094578085 title "Congenital familial steatorrhea with fibromatosis of the pancreas and bronchiolectasis" @default.
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- W2094578085 doi "https://doi.org/10.1016/s0022-3476(39)80166-4" @default.
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