*Purpose: Children with chronic liver disease exhibit growth hormone resistance which is an additional risk factor for sarcopenia and growth failure in this vulnerable population. Low IGF-1 is associated with multiple negative effects including fatigue in other pro-inflammatory states, such as inflammatory bowel disease. Portal hypertension has not yet been studied as a risk factor for low IGF-1.

*Methods: Children ages 3 months to 18 years with chronic liver disease were enrolled from an ambulatory clinic at a single center. Patients with comorbidities affecting intestinal inflammation or the growth hormone axis were excluded. Clinical data, nutritional assessmen, and serum samples for IGF-1 were obtained at enrollment. Patients were categorized by the presence or absence of portal hypertension using published criteria for CEPH. IGF-1 Z scores were analyzed as both continuous and dichotomous variable with low IGF-1 defined as Z score < -2. Low mid upper arm circumference (MUAC) was defined as Z score <-1. For children >5 years, both child and guardian completed the PedsQL Multidimensional Fatigue Scale (PedsQL MF) and were compared with a published cohort of 157 healthy children.

*Results: 27 patients with median age 12.2 years were enrolled with the most common diagnoses being autoimmune hepatitis/primary sclerosing cholangitis and biliary atresia. 37% (n=10) patients had CEPH. The median IGF-1 Z score was -1.5. Children with CEPH had lower IGF-1 compared to patients without CEPH (p .003). Median height and weight Z scores in our cohort were close to 0 and when tested linearly, had no association with IGF-1. Low MUAC was associated with low IGF-1 (p .036). Median PedsQL MF scores from child and parent proxy were 63.89 and 75.70 respectively. Both were significantly lower compared to healthy children. Low IGF-1 levels were significantly associated with child reported fatigue (p .028).

*Conclusions: Children with CEPH have low IGF-1 even in this population of stable patients with preserved linear growth. Lower IGF-1 Z scores are associated with an increased burden of child reported fatigue. Next steps will include measurement of serum cytokines and evaluation of the relationship between a pro-inflammatory state and IGF-1 Z scores.

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