A study on the nutritional status and body composition of children with Hutchinson–Gilford progeria syndrome

Objective: Patients with Hutchinson‒Gilford progeria syndrome (HGPS) often have severe growth retardation, generalized fatty malnutrition, and cardiovascular complications. There are different cardiovascular risks due to the different distributions of fat and muscle tissues in different regions. Therefore, it is necessary to investigate nutritional status and analyze the distribution of muscle and fat tissues in HGPS. Method: A cross-sectional survey was conducted to select HGPS patients aged 3 years and above who received treatment at Children’s Hospital, Zhejiang University School of Medicine. The medical history, physical measurements, laboratory examination data, dietary data, body composition data, and bone density data of the subjects were collected and analyzed. Results: A total of eight HGPS patients were included. The weight and height of all the subjectsat birth were normal but decreased to below the 3rd percentile by 2 months of age. The rates of increase in weight and height were relatively slow. A dietary survey was conducted on all patients, and the results revealed that 87.5% (7 out of 8) of the subjects were exposed to insufficient energy intake. Dual-energy X-ray absorptiometry (DXA) was employed to investigate bone density, and the results revealed a decrease in bone density (-2.82±1.46) in the subjects at the chronological age; the Z score of the subjects at height was -0.72±0.99. The fat mass results revealed that 62.5% of the subjects’ total body fat percentage and 100% of the subjects’ fat mass index (FMI) were within the normal range. However, 100% of the subjects’ android to gynoid A/G fat ratios and 75% of the subjects’ torso-leg (T/L) fat ratios were higher than normal. The muscle mass results revealed a decrease in the total and appendicular muscle masses of all the subjects, with lower limb muscles showing a more severe decline than upper limb muscles did. Conclusions: HGPS patients presented severe growth retardation, with abnormal fat metabolism mainly manifested as abnormal fat distribution, which may increase the risk of cardiovascular events in these patients.