Prevalence of Hepatic Steatosis Among Children and Adolescents With Cystic Fibrosis and Its Association With Nutritional Status

Abstract

Objective: To determine the prevalence of hepatic steatosis (HS) in children and adolescents with cystic fibrosis (CF) and associate it with nutritional status.Methods: Cross-sectional study with children and adolescents with CF diagnosis. Weight and height were used to calculate the body mass index (BMI) and subsequent classification of the nutritional status. The midarm circumference (MAC), triceps skinfold thickness (TSF) and midarm muscle circumference (MAMC) were used to evaluate body composition. Abdominal ultrasonography was performed for diagnosis of HS. The statistical tests used were Student’s t test, Mann-Whitney test and chi-square test with significance level of 5%.Results: 50 patients with CF were evaluated, 18 (36%) were diagnosed with HS (Group A) and 32 (64%) without HS (Group B). The mean age of Group A was 13,2±4,9 years old and Group B 11,7±4,9; for BMI, the value for Group A was 18,0±4,1 and Group B was 15,7±3,8; the TSF of Group A was 8,4±3,5 mm and Group B was 7,0±2,5 mm. For these variables, there was no significant difference between the groups. The mean of MAC and MAMC differed significantly between the groups, being higher in the HS group, with p values of 0,047 and 0,043.Conclusions: The frequency of HS in patients with CF is high and it is not related to malnutrition, according to the parameters of BMI, TSF and MAMC. The values of MAC and MAMC indicated a greater reserve of muscle mass in patients with HS.

The effects of nutritional status and intervention on pulmonary functions in pediatric cystic fibrosis patients

“…Cystic fibrosis‐related liver disease occurs in 30–50% of CF patients 27 . The prevalence of CFRLD is 2–37% in children and young adults, but there was no significant correlation between CFRLD and nutritional status in children with CF 28,29 …”

“…27 The prevalence of CFRLD is 2-37% in children and young adults, but there was no significant correlation between CFRLD and nutritional status in children with CF. 28,29 Patients in the present study were followed up regularly and given nutritional interventions when necessary in accordance with ESPEN/ESPGHAN guidelines. According to WHO malnutrition classification, at presentation 19.5% of the present study's CF patients had a WFL/H or BMI z-scores <-2 (acute malnutrition), versus 11.2% following nutritional support.…”

Cystic fibrosis-related liver disease: Clinical presentations, diagnostic and monitoring approaches in the era of CFTR modulator therapies

“…phospholipid metabolism abnormalities [122], malnutrition [119,123], or obesity), pancreatic insufficiency, choline depletion [124][125][126], diabetes and prolonged antibiotic therapy. While malnutrition has long been a subject of debate [127], this cannot be the only explanation, as patients with good nutritional status can present with steatosis [128]. On the other hand, the increasing prevalence of overweight and obese patients in the CF population (31.4% in 2019 versus 12.8% in 1999 [129]) is of concern because of the association of this entity with metabolic dysfunctionassociated fatty liver disease [130].…”

Nutritional status in the era of highly effective CFTR modulators

“…Hepatic steatosis—the accumulation of fat in the liver—is highly common in CF, with more recent single‐center reports of 15%–36% prevalence, depending on the diagnosis definition and method used 84–87 . In a small cross‐sectional study, lumacaftor/ivacaftor therapy was associated with less hepatic steatosis, although the mechanism of action is not clear 84 .…”