[A cross-sectional survey on uncooked corn starch application and gastrointestinal complications in children with hepatic glycogen storage disease].

Objective

To evaluate the clinical characteristics and current utilization of uncooked cornstarch (UCCS) in children with hepatic glycogen storage disease (GSD), so as to investigate the association between UCCS intake and gastrointestinal (GI) complications.

Methods

A cross-sectional study was conducted using the GSD cohort at Guangdong Provincial People's Hospital. Standardized questionnaire surveys were administered in July 2024 and July 2025 among 343 genetically confirmed pediatric patients with hepatic GSD. Data included demographic information, genetic subtypes, clinical features, UCCS intake regimens, GI symptoms and laboratory findings were collected. Participants were stratified by genetic subtype, age group, and median daily UCCS intake. Growth and nutritional status were evaluated according to World Health Organization (WHO) growth standards, using height-for-age Z-scores (HAZ) and BMI-for-age Z-scores (BMIZ) across age groups and genetic subtypes. Clinical manifestations, laboratory parameters, UCCS intake regimens, and the prevalence of GI symptoms were compared across genetic subtypes. The burden of GI symptoms was quantified using a severity score (ranging from 0 to 3), assigned based on the presence and number of reported symptoms. Between-group comparisons were performed using the Mann-Whitney U test. Binary and ordinal Logistic regression models were employed to assess the association between UCCS intake and the risk or severity of GI symptoms.

Results

Among the 343 enrolled patients, the distribution by genetic subtype was as follows: 138 with type Ⅰa, 75 with type Ⅰb, 55 with type Ⅲ, 3 with type Ⅳ, 24 with type Ⅵ, and 48 with type Ⅸ. Distinct inter-subtype differences in clinical presentation were observed. Metabolic disturbances were most pronounced in types Ⅰa and Ⅰb: hyperlactatemia was highly prevalent in type Ⅰa (124/138, 89.9%) and type Ⅰb (71/75, 94.7%), and hypertriglyceridemia was common in type Ⅰa (104/138, 75.4%) and type Ⅰb (47/75, 62.7%). A total of 166(48.4%) children reported at least one GI symptom during the preceding month. Daily UCCS intake was 5.8 (3.6, 8.0) g/kg, with the highest intakes observed in subtype Ⅰa (7.0 (4.6, 8.8) g/kg) and subtype Ⅰb (6.6 (4.3, 9.2) g/kg). The GI symptom score in the high-dose group (168 cases) was higher than that in the low-dose group (175 cases)(1 (0,1) vs.0 (0,1), P<0.001). After adjustment for age, sex, and body mass index (BMI), UCCS intake remained an independent risk factor for GI symptoms (OR=1.12, P=0.006). Daily UCCS intake was also independently associated with anorexia, diarrhea, and nausea/vomiting (all P<0.05). Growth assessment revealed an overall stunting prevalence (HAZ<-2 s) of 20.6% (66/320), with the highest rates observed in subtype Ⅰb (38.8%, 26/67) and 11-18 years group (37.9%, 22/58). The overall prevalence of overweight or obesity (BMIZ>+2 s) was 18.1%, most notably elevated among 0-3 years group (22.7%, 22/97) and in subtype Ⅰa (22.7%, 29/128).

Conclusions

Children with hepatic GSD exhibit marked clinical heterogeneity across subtypes. A clear dose-response relationship demonstrate between UCCS intake and GI symptom burden. Moreover, these patients face a dual nutritional challenge of stunting and overweight or obesity. In clinical practice, careful attention should be paid to individual UCCS tolerance and subtype-specific management strategies, balancing glycemic control, intestinal health, optimal growth and development.