ObjectivePatients with hepatic glycogen storage disease（GSD）have recurrent episodes of hypoglycemia. This study aimed to investigate and analyze blood glucose and biochemical indicators in pediatric patients with hepatic GSD， thus provide data support for hypoglycemia prevention and its clinical management. MethodsA cross-sectional field study was conducted among patients with hepatic GSD treated in the Department of Pediatrics of Guangdong Provincial People's Hospital on July 14， 2024. We collected the peripheral blood samples of the patients and their healthy family controls on site， then analyzed and compared their blood glucose and biochemical indicators. ResultsOf the 44 patients with hepatic GSD， there were 34 males and 10 females， including GSD Ib（n =14）， GSD Ia（n=15）， GSD Ⅲ（n=2）， GSD Ⅵ（n=7）and GSD Ⅸ（n=6）. The average age was 7.60（5.08-11.98）years. All patients were on uncooked cornstarch（UCCS）therapy. Of the patients， 77.3%（34/44）had hepatomegaly， 61.4%（27/44）had recurrent hypoglycemia， 61.4%（27/44）had blood glucose ≤ 3.9 mmol/L， 18.2%（8/44）had blood glucose ≤ 2.8 mmol/L， and none of the 8 cases was GSD Ib. The lowest blood glucose level was 1.19 mmol/L and no episodes of hypoglycemia occurred. Of the family control subjects， 65.9%（29/44）had blood glucose ≤ 3.9 mmol/L. There was no significant difference in hypoglycemia prevalence between hepatic GSD group and control group（P=0.658）. The hepatic GSD patients had hyperlactacemia， hyperuricemia and hypercholesterolemia prevalence rates of 65.9%， 45.5% and 9.1%， respectively， as compared with 18.2%， 43.2% and 15.9%， respectively， for the family control subjects. No significant difference was found in the prevalence rates of hyperuricemia and hypercholesterolemia between the two groups（P=0.830 and P=0.334， respectively）. ConclusionsAsymptomatic hypoglycemia is common in patients with hepatic GSD， especially in non-GSD-Ib patients. It is necessary to optimize the diet management of UCCS， conduct dynamic blood glucose monitoring and follow a light diet， so as to decrease hyperuricemia and hypercholesterolemia， avoid and reduce the serious adverse reactions and complications caused by severe hypoglycemia.

Objective:To evaluate the effect of reactivated ultrasound contrast agent on prevention of ischemia-reperfusion (IR) injury in rat hindlimb ischemia model.Methods:Microbubbles were compressed into nanodroplets (NDs) and the particle size range was determined.In vitro experiments were carried out to observe the acoustic phase transition of nanodroplets in the ultrasound field and the change of dissolved oxygen (DO) concentration in the surrounding solution. Forty-one male Sprague Dawley rats were divided into 5 groups: acoustic nanodroplet vaporization treatment group (NDs+ US group, n=9), saline+ ultrasound treatment group (Saline+ US group, n=8), nanodroplet without ultrasound treatment group (NDs group, n=8), ischemia/reperfusion injury group (IRI group, n=8) and sham operation group (Sham group, n=8). Ultrasound imaging was performed before operation and 12 hours after reperfusion to evaluate the improvement of hemodynamics of criminal artery under different treatments. Tissue injury were evaluated by analyzing immunohistochemistry staining results. Results:The formed NDs ranged in size from approximately 68.0-295.4 nm and the highest concentration in the 100 nm range. In vitro studies, a decrease in DO was measured during the phase transition.In the animal experiment, after ischemia/reperfusion, NDs+ US, Saline+ US, NDs and IRI groups demonstrated a significantly higher resistance index (RI) and pualsatility index (PI) of the right common iliac artery compared with before operation (NDs+ US group: PI 1.79±0.17 vs 1.57±0.23, P=0.014; RI 0.80±0.02 vs 0.75±0.04, P=0.002. Saline+ US group: PI 2.29±0.16 vs 1.57±0.16, P<0.001; RI 0.90±0.06 vs 0.74±0.03, P<0.001. NDs group: PI 2.17±0.14 vs 1.53±0.15, P<0.001; RI 0.91±0.04 vs 0.75±0.04, P<0.001. IRI group: PI 2.12±0.22 vs 1.58±0.20, P<0.001; RI 0.88±0.04 vs 0.75±0.04, P<0.001). The increases of PI and RI (ΔPI, ΔRI) in NDs+ US group were higher than those in sham group (all P<0.05), but significantly lower than those in saline+ US group, NDS group and IRI group (all P<0.05). Immunohistochemical results indicated the percentages malondialdehyde (MDA) positive cells in Saline+ US, NDs and IRI groups were higher than those in NDs+ US and sham groups (all P<0.05). Conclusions:The acoustic phase transition of nanodroplets in the ultrasound field can reduce ischemia/reperfusion injury and improve hemodynamics abnormality after reperfusion.