CF patient and hepatic problem

Question

Hello,

I'm the mother of an 11-year-old CF child weighing 37kg and having the d508 mutation. I live in Morocco.

About 1 year ago, we had an ultrasound and we detected hepatomegaly. His liver measured 13.6cm. He was treated with ursolvan (ursodeoxycholic acid) 200mg / d.

This week, we carried out an ultrasound again and his hepatomegaly was accentuated (15 cm), so a scanner revealed an hepatic steatosis.

I looked for publications to discover that steatosis occurs as a result of lipid imbalance and that Ursolvan had no effect on CF hepatic steatosis.

Please, I would like to know:
- If there is a particular diet to overcome this steatosis problem
- If it's true that ursolvan has no effect on steatosis
- If not, which dose should it be taken?
- And if the dose of 200mg per day is an insufficient dose (so steatose is a consequence of this maladaptive treatment)?

Thank you so much

Answer

Hello,

In the follow-up of cystic fibrosis, ultrasound of the liver is recommended annually as well as the liver biological balance (transaminases, gammaGT ..). Ultrasound and liver scan have diagnosed in your child as a hepatic steatosis. It would be interesting to know the result of the blood test.

Hepatic steatosis affects 25-50% of CF patients, and appears to be linked to nutritional causes such as poorly controlled pancreatic insufficiency, essential fatty acid deficiency, which need to be corrected.

The diet must be balanced, hypercaloric, bringing essential fatty acids (rapeseed oil, nuts, fatty fish ...). Similarly, the supply of pancreatic extracts should be optimized. Ursodeoxycholic acid is not necessary in the case of a single steatosis. When prescribed, the usual dosage is 20 mg / kg / day in two doses. In your child, other factors such as the blood test, for example, may have motivated his prescription.

Do not hesitate to talk to the doctor who follows him.

Best regards,
Dr. Michèle Gérardin

16.01.2017