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Pancreatitis in Children

Sometimes you donít think of the pancreas as being a cause of obstructive jaundice, but remember how that pancreatic duct and the common bile duct come together as they enter the second part of the duodenum, and in fact often will share a common orifice. So basically, pancreatitis may cause cholestasis by all the edema and swelling that occurs in the pancreas that then goes on to compress the common bile duct. In some instances, with progressive fibrotic pancreatitis, the fibrotic process may actually encase the bile duct as it comes close to the pancreatic duct.

The clue to pancreatitis is that this is an epigastric pain. Very often made worse by eating and it is almost always a bad pain; associated with vomiting, sometimes with pain radiating straight back into the back and with an elevated amylase and lipase. If you image the abdomen, usually you are going to need a CT scan or an MRI, youíll see that the pancreas is enlarged.

Then of course there are all manner of lumps and bumps that can obstruct the bile duct externally. Of the tumors, the primary liver tumors in children are usually right inside the parenchyma of the liver and donít come close to the porta, so most often they donít present with jaundice. So if you see hepatoblastoma, which is the most common childhood cancer of the liver, or HCC causing jaundice, you know that that tumor mass is impinging on the porta hepatis. Now tumors outside of the liver which can obstruct the porta include some of the rare neuroendocrine tumors and the sarcomas.

Beyond malignant masses youíve got to think about all the abscesses and cysts. This is a pretty unusual cause of jaundice, also. You can have an enormous abscess in the liver and no jaundice at all if it isnít getting close to the porta.

Alpha 1-antitrypsin deficiency. The metabolic causes of chronic liver disease in children, by far and away the most common is alpha 1-antitrypsin deficiency. And if you remember, this is, generally speaking, associated with the ZZ phenotype. Occasionally you see it with some of the other phenotypes like MZ, but for purposes of keeping life simple, itís the ZZ phenotype, generally speaking. As I said before, these children may have presented in infancy with a high bilirubin which completely went away.

Cystic fibrosis. You normally think about this as being a disease involving the lungs. Interestingly, cirrhosis with portal hypertension can occur in cystic fibrosis, generally presenting in mid or older age children. Sometimes the liver disease is much more important than the lung disease in these children, and they may also present with portal hypertension and already developed cirrhosis. If theyíve got portal hypertension, you try to shunt some of these children if they are big enough, because of course we are very worried about transplanting children who may already have chronic lung disease and all the problems of immunosuppression and chronic lung disease together might cause.