*1.3.1 Laboratory tests*

*Cystic Fibrosis - Heterogeneity and Personalized Treatment*

to the formation of gallstones [33, 34].

estrogens in female.

additional factor.

**1.3 Clinical picture**

3.Significant undernourishment.

5.Severe genotype (delta F508) [1, 12, 24, 35, 36].

4.Pancreatic insufficiency.

1.Focal hepatic fibrosis (72%)

4.Portal hypertension (2–5%)

6.Cholelithiasis (14–24%)

8.Cholestasis in newborns (<10%)

9.Primary sclerosing cholangitis (rarely)

7.Steatosis (25–60%)

2.Focal biliary cirrhosis (20–30%)

3.Multilobular biliary cirrhosis (5–15%)

*1.2.4 Cholelithiasis*

cystic fibrosis than the healthy population. The narrowing of the distal regions of the bile ducts is frequent and may occur in even 90% of CF patients and contribute

Cholelithiasis concerns 14–24% of CF patients. The following play a role in the pathogenesis of the formation of gallstones: abnormal bile content, increased excretion of bile acids with stools, and the formation of lithogenic bile where bile acids are interlocked with glycine. No correlation between the formation of gallstones and supplementation with pancreatic enzymes has been confirmed [1, 3, 22]. Risk factors for the development of liver diseases in cystic fibrosis:

1.Male gender—three fourths of patients with CFLD are male. Protective role of

2.Coexisting meconium ileus (inconsistent data—from a five-time higher risk of developing hepatic lesions to a similar risk). Only 25% of patients with CFLD who had meconium ileus in the medical interview was identified. Meconium ileus is not a prerequisite for CFLD. Probably, parenteral nutrition is an

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:

5.Small atrophic gallbladder and narrowing of bile ducts (15–45%)

**82**
