**1.3 Clinical picture**

In most CF patients, the course of hepatic complications is symptomless. Pruritus sometimes occurs and jaundice in patients whose condition is advanced. Incidental finding of hepatomegaly is usually the first sign. In newborns, steatosis may be incidentally discovered in a routine abdominal ultrasound.

Clinical changes of the liver in patients with cystic fibrosis:


**83**

*Liver Changes in the Course of Cystic Fibrosis DOI: http://dx.doi.org/10.5772/intechopen.89306*

10.Cholangiocarcinoma (rarely)

*1.3.1 Laboratory tests*

*1.3.1.1 Periodic laboratory tests*

11.Drug-induced, toxic liver damage [1, 3, 12, 24, 37–40]

ases with concurrent normal GGTP index may indicate steatosis.

*1.3.1.3 Liver biopsy with histopathological assessment.*

*1.3.1.4 Noninvasive parameters of liver fibrosis*

diagnostics [27, 41–48].

**1.4 Treatment**

support,

worse liver function.

*1.3.1.2 Abdominal ultrasound with a Doppler option and elastography*

This is not a routine recommended procedure in patients with CFLD.

The levels of AlAT, AspAT, GGTP, bilirubin and bile acids, the APRI index, and FibroTest are recommended. It is believed that elevated levels of at least two hepatic parameters above the norm within at least 3 months is an indication of advancing hepatic lesions. But they have low sensitivity and specificity. Most patients with multifocal cirrhosis have normal test results. Isolated elevation of aminotransfer-

Ultrasound is inexpensive and is a noninvasive test. It allows assessment of the level of steatosis, symptoms of portal hypertension, and cirrhotic transformation of the liver. However, normal imaging of the liver does not exclude the ongoing process of fibrosis. FibroScan is an effective and noninvasive tool to quantify fibrosis and steatosis in the liver diseases including cystic fibrosis. The stiffness of the liver tissue can be assessed based on shear wave velocity (the stiffness increases with the speed).

Liver biopsy is an invasive procedure and is prone to side effects and sampling.

Fibroindex, aminopeptides of type III procollagen, collagen I, collagen IV, laminine, hyaluronic acid, and/or cytokines and chemokines relating to the process of fibrosis. There are studies on their usefulness in minimally invasive clinical

1.**Background therapy of cystic fibrosis** include pancreas enzyme supplementation, vitamin supplementation, extensive physiotherapy, and nutritional

2.**Diet therapy.** In patients with cystic fibrosis, nutrition and survival are intimately related. Growth failure and body mass deficiency are the prognostic factors in patients with CF. Prevention of undernourishment in cystic fibrosis, sometimes feeding tube or PEG nutrition, is recommended. Hypercaloric diet, which is rich in protein, carbohydrates, and fat, is recommended. The energy needs of patients with CF vary widely and have been stated as 120–150% of those required by healthy individuals of the same age, sex, and size. Reduction of protein only in situation of encephalopathy. Correction of fat-soluble vitamin deficiency is essential. Lower serum levels of vitamin A and E were associated with a higher rate of pulmonary exacerbations and

