Fatty liver

Question

Hello,

Today, my daughter (will be 3 years on July 7, 2011) [question was asked in June 2011] and I went to her annual check-up. She has CF (del508). The gastroenterologist was quite satisfied, the pankreas was inconspicuous, although she has a pancreatic insufficiency and needs kreon. Futhermore, he noticed a slight, point-shaped fatty degeneration of the liver. Is this common for this age? What can be done against it? Do you use ursofalk® (ursodeoxycholic acid) at this point already or only in case of a beginning cirrhosis? Or is there nothing that can be done? Is the fatty degeneration the key enabler for a cirrhosis? Can occasional fatty stools caused by too little kreon accelerate the fatty degeneration?

Many thanks.
Vera M.

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Ms M. asked a follow-up question which is copied below in the answer section.

Answer

Dear Ms M.,

You report that the gastroenterologist noticed a „slight, point-shaped fatty degeneration of the liver” during the annual check-up of your 3-year-old daughter and you ask if this is common for her age.

Changes of the liver happen in CF patients in different forms and ages. Much-feared is the development of a so called liver cirrhosis. This complication nowadays usually develops in adulthood and in literature the occurrence is indicated with 2-5%. It is still unclear why a part of the CF patients is affected by this complication.

A slight fat depositing in the liver tissue is found clearly more often in CF patients, sometimes also in the age of your daughter. Generally speaking this is an indicator showing that the liver is under stress. This can be caused by an insufficient substitution of pancreatic enzymes. CF patients, e.g., that are only diagnosed with CF in the age of 2 or 3 years despite an pancreatic insufficiency often show a fatty degeneration of the liver.

The same happens if frequently insufficient or no enzymes are given with a meal with high fat content. On the other hand, a long-term medication or taking harmful substances (alcohol) can lead to a fatty degeneration of the liver. The latter is surely not applicable for your daughter.

It is very likely that the current fatty degeneration of your daugher’s liver is not a pointer to the development of a liver cirrhosis. However, it should be checked if and in which form the substitution of pancreatic enzymes could be improved. Your daughter’s stool should be collected during three days and the expulsion of stool fat should be checked. At the same time you should note down exactly what your daughter ate and drank during the collection period (3 day weighed food record) and it should be recorded how much kreon your child took with every meal. This is a way to find out if the enzyme dosage is correct.

If it is already necessary for your child to take ursodeoxycholic acid should be decided after considering additional factors. If the liver is enlarged or if its texture is modified or if regular check-ups sometimes show an augmentation of the so called transaminases, then ursodeoxycholic acid should be taken. If these points are not applicable, it would be better to optimise enzyme substitution and then wait and see. However, the ultrasonic testing should be done more frequently (every 6 months) and the testing of the transaminases as well (every 3 months).

I hope we were able to calm you down a bit with this information.
Best regards,
Dr. H.-G. Posselt

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Some days later we received this follow-up question:

"Thank you for giving such an extensive answer. But I still have one question. Can we assume that the signs of a fatty liver will regress if the substitution of enzymes is improved now, or will these signs persist? Is it better to continuously give a bit more kreon in order to improve the situation? Currently, we actually try to calculate the fat content as exactly as possible and then give kreon accordingly."

Dr. Posselt gave the following answer:

"Hello,

A slight point-shaped fatty degeneration of the liver in CF could maybe regress over the years if the causing dysfunction is eliminated and no other negative influences occur. But even if it remains, such a slight form of fatty degeneration would not have a negative impact on your daughter’s future quality of life or expectancy of life.
You should not give more pancreatic enzyme than necessary because this could damage the intestine if the doses are much too high.

As mentioned it would make sense to to do a 3-day weighed food record. Then, you will know exactly which dosage of the enzymes would be correct and you do not need to worry about it any more."

02.08.2011