Rare pancreatic disorders
The relationship between the pancreas and diabetes was established when Minkowski performed a pancreatectomy in the dog in 1889. Early clinicians distinguished between 'pancreatic' diabetes, due to obvious pancreatic disease, and the much more common form of diabetes in which the pancreas appeared normal. Only about 1–2% of human diabetes is considered to be due to overt pancreatic disease, but this may be an underestimate. The pancreas has a considerable reserve of islet beta cells, and investigators need to excise 70–90% from healthy animals before they will develop diabetes. Extensive pancreatic damage is therefore needed to cause human diabetes. Such damage occurs in severe cases of acute pancreatitis, in chronic pancreatitis, in pancreatic fibrosis (due for example to iron overload), or following surgical excision of the pancreas. Pancreatic carcinoma predisposes to diabetes by secreting circulating factors promoting insulin resistance as well as by pancreatic destruction. Pancreatic diabetes results in loss of both insulin and pancreatic glucagon, diabetic ketoacidosis is rare, and patients are sensitive to the action of insulin.
Although some 19th century physicians had noted an association between diabetes and pancreatic disease, proof came when Oskar Minkowski removed the pancreas from a dog in 1889, although diabetes was an unexpected development! The role of the pancreatic islets emerged more slowly, in the absence of specific stains for islet cells and insulin, and early pathologists were baffled by the apparently normal appearance of the pancreas in most cases of diabetes.
The work of Frederick Allen showed that dogs did not develop diabetes until 80–90% of the pancreas had been removed, and that the development of diabetes could be avoided by use of a low-carbohydrate, low-energy diet. This was the origin of the starvation regimen for children with diabetes.
Any form of extensive pancreatic damage may result in diabetes. These range from surgical excision of the pancreas (usually for a pancreatic tumour) to acute and chronic pancreatitis, tropical chronic pancreatitis (previously referred to as fibro-calculous pancreatic disease), pancreatic fibrosis, carcinoma of the pancreas, and inherited disorders affecting the pancreas such as cystic fibrosis.
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