1.Effects of LEARNS model-based health education on rehabilitation outcomes in patients with stable pneumoconiosis
Meirui LIU ; Baoping LI ; Fuhai SHEN ; Zhiping SUN ; Qianqian LI ; Haifeng CUI ; Chunxi WANG ; Jianxiang WANG ; Qingchun CAO ; Zhongdong YANG ; Xiangyou LI ; Jiayin ZHONG ; Bowen HOU ; Lini GAO ; Chenyue HOU ; Xuemei SUN
Journal of Environmental and Occupational Medicine 2026;43(9):1139-1145
Background Patients with pneumoconiosis often have limited disease-related knowledge, insufficient access to health education, and substantial health management needs. Health education may help improve rehabilitation outcomes and quality of life in this population. Objective To evaluate the effects of health education based on the Listen-Establish-Adopt-Reinforce-Name-Strength (LEARNS) model on rehabilitation outcomes, health-related behaviors, and quality of life among pneumoconiosis patients, and provide effective intervention strategies for the rehabilitation management of patients with pneumoconiosis. Methods A randomized controlled trial was conducted. A total of 120 pneumoconiosis patients treated in the Department of Occupational Diseases, Beijing Emergency General Hospital, from November 2024 to February 2025 were enrolled. Participants were randomly assigned to a control group or an intervention group using a random number table method, with 60 patients in each group. The control group received routine pharmacotherapy, whereas the intervention group received a 12-week LEARNS model-based health-education intervention in addition to routine treatment. The 6-Minute Walk Test (6MWT) was used to assess exercise tolerance, and the modified Medical Research Council dyspnoea scale (mMRC) was used to evaluate dyspnoea. The Self-Rating Anxiety Scale (SAS) and Self-Rating Depression Scale (SDS) were applied for psychological status assessment. Pulmonary function, including forced expiratory volume in one second (FEV1) and forced vital capacity (FVC), was measured using spirometry. A self-designed scale was used to evaluate eight health-related behaviors targeted by the health education program. The 36-Item Short-Form Health Survey (SF-36) and Chronic Obstructive Pulmonary Disease (COPD) Assessment Test (CAT) were used to assess quality of life, and the Pittsburgh Sleep Quality Index (PSQI) was adopted to evaluate sleep quality. Results After the 12-week intervention, the control group showed no statistically significant changes from baseline in the measured outcomes(P>0.05). In the intervention group, exercise tolerance improved significantly, with the 6-minute walking distance increasing from (382.55±19.52) m to (426.84±33.59) m (P<0.001). Dyspnoea was also alleviated, with the mMRC score decreasing from 1.37±0.66 to 0.96±0.54 (P<0.001). The CAT score decreased from 18.95±7.03 to 10.67±3.64 (P<0.001). Significant improvements were observed in anxiety and depression symptoms (P<0.001), with the SAS score decreasing from 38.85±6.24 to 34.27±2.27, and the SDS score decreasing from 49.01±3.25 to 45.49±3.44. Sleep quality improved significantly, with the PSQI score dropping from 7.89±4.35 to 4.05±2.31 (P<0.001). All eight health-related behaviors in the intervention group improved significantly after the intervention (P<0.001), including acquisition of disease-related knowledge (1.87±0.34 vs. 3.13±0.45), participation in health education activities (1.89±0.52 vs. 3.61±0.38), and regular physical exercise (2.37±0.41 vs. 4.17±0.33). All SF-36 domain scores increased significantly (P<0.05), with the most prominent improvements observed in physical functioning, general health, and vitality. At week 12, all SF-36 domain scores were significantly higher in the intervention group than in the control group (P<0.05). No statistically significant between-group differences were detected in pulmonary function parameters (P>0.05). Conclusion LEARNS model-based health education could improve exercise tolerance, dyspnea, psychological status, sleep quality, health-related behaviors, and quality of life among patients with pneumoconiosis, and therefore may be a useful adjunctive strategy for rehabilitation management in patients with pneumoconiosis.
2.Analysis of drug resistance and treatment efficacy of Helicobacter pylori infection in children
Yanfei CHEN ; Xingwei ZHANG ; Li MENG ; Jiao WANG ; Xiaohan ZHU ; Yanling ZHANG ; Xuemei ZHONG
Chinese Journal of Pediatrics 2025;63(10):1110-1114
Objective:To investigate the antibiotic resistance of Helicobacter pylori (Hp) and to evaluate the eradication efficacy of individualized treatment for Hp in children. Methods:A retrospective cohort study was conducted on 227 children who visited the Department of Gastroenterology, Capital Center for Children′s Health, Capital Medical University from June 2022 to December 2023 due to gastrointestinal symptoms. All patients underwent gastroscopy and tested positive on 13C-urea breath testing. They were grouqed according to the Hp culture and drug susceptibility test. Children with positive Hp culture received personalized treatment based on the results of their drug sensitivity tests, while the other children who didn′t undergo Hp culture received empirical treatment. The effects of different treatment groups was compared by chi-square test or Fisher exact probability test. Results:A total of 227 children with Hp infection (121 males and 106 females) were included, with the age of 11.7 (8.9, 13.6) years. Among the 131 samples submitted for testing, 105 cases (80.1%) had positive results. Only 9.5% (10/105) of patients were sensitive to 6 antibiotics. The resistance rates to clarithromycin, metronidazole and levofloxacin were 90.5% (95/105), 86.7% (91/105) and 22.9% (24/105) respectively. The resistance rate to both clarithromycin and metronidazole was 77.1% (81/105). The resistance rate to both levofloxacin and metronidazole was 19.0% (20/105). The resistance rate to both levofloxacin and clarithromycin was 21.9% (23/105). The resistance rate to these three antibiotics was 16.2% (17/105). No strains resistant to furazolidone, amoxicillin or tetracycline hydrochloride were found. Eighty-nine cases were treated with bismuth quadruple therapy based on the drug sensitivity results, and the overall eradication rate was 88.8% (79/89), including 42 treatment-naive cases with a 100% eradication rate (42/42) and 47 retreatment cases with a 78.7% eradication rate (37/47). The eradication rate of empirical treatment was 75.7% (56/74). Among them, 65 patients received amoxicillin, clarithromycin and omeprazole because of negative penicillin skin tests, with a 75.4% (49/65) eradication rate; 9 patients received clarithromycin, metronidazole, omeprazole and bismuth with positive penicillin skin tests, achieving 7/9 eradication rate. The comparison of eradication rates between two treatment groups suggested a statistically significant difference ( P<0.05). No statistically significant difference was found in drug reactions such as nausea, vomiting, and rash between the two groups ( P>0.05). Conclusions:Hp strains had a relatively high dual resistance to clarithromycin and metronidazole, especially clarithromycin. For areas with a high resistance rate to clarithromycin, the bismuth quadruplet of clarithromycin removal combined with bismuth agent can be chosen as empirical treatment. In medical institutions where drug susceptibility test can be conducted, personalized treatment plans are recommended as the first-line treatment.
3.Clinical characteristics of monogenic and non-monogenic early-onset inflammatory bowel disease
Youzhe GONG ; Yanfei CHEN ; Fuping WANG ; Jiao WANG ; Li MENG ; Xi HE ; Xuemei ZHONG
Chinese Journal of Inflammatory Bowel Diseases 2025;09(2):143-148
Objective:To compare the clinical characteristics of monogenic and non-monogenic early-onset inflammatory bowel disease (EO-IBD) in children and to explore the necessity of genetic analysis in EO-IBD research.Methods:A retrospective analysis of clinical data was conducted on 73 children diagnosed with EO-IBD at the Children's Hospital affiliated with Capital Institute of Pediatrics between January 2017 and December 2023. Genetic analysis was performed utilizing next-generation sequencing technology, with patients stratified into monogenic and non-monogenic groups based on the presence or absence of pathogenic mutations. Subsequently, a comparative analysis of clinical characteristics was conducted between these two cohorts of EO-IBD patients.Results:Among the 73 EO-IBD cases, 27 (37%) were diagnosed as monogenic IBD, and 46 (63%) as non-monogenic IBD. Compared to the non-monogenic group, the monogenic group had an earlier age of onset [1 (0.2, 3.0) months vs. 15 (4.1, 51.3) months, P < 0.001], with a higher incidence within the first month of life (70.4% vs. 13.0%, P < 0.001). Monogenic IBD cases were more likely to present with Crohn's disease (CD) phenotypes (88.9% vs. 52.2%, P = 0.003) and colonic involvement (L2) (91.7% vs. 62.5%, P < 0.001), but were less likely to present with non-penetrating, non-stricturing (B1) disease (87.5% vs. 95.8%, P = 0.019). Children in the monogenic group were more prone to severe malnutrition (74.1% vs. 21.3%, P < 0.001), perianal abscesses (40.7% vs. 8.7%, P < 0.001), perianal tags (22.2% vs. 0%, P = 0.004), fever (74.1% vs. 23.9%, P < 0.001), oral ulcers (44.4% vs. 6.5%, P < 0.001), and skin lesions (33.3% vs. 2.2%, P < 0.001). Regarding treatment, the monogenic group had higher usage of thalidomide (88.9% vs. 54.3%, P = 0.002) and hematopoietic stem cell transplantation (HSCT) (37.0% vs. 0, P < 0.001) and a higher mortality rate (22.2% vs. 2.2%, P = 0.017) . Conclusions:For children with IBD presenting at an early age, especially within the first month of life, and showing symptoms like fever, oral ulcers, skin lesions, severe malnutrition, and perianal disease, monogenic IBD should be considered. Genetic testing results can aid in guiding treatment decisions.
4.Clinical characteristics analysis of peptic ulcers caused by eosinophilic gastrointestinal disorders in children
Meng JIN ; Mingran MI ; Wenwen LIU ; Kunyu YAO ; Ran ZHANG ; Xuemei ZHONG
International Journal of Pediatrics 2025;52(4):268-273
Objective:To analyze the clinical characteristics,laboratory tests,endoscopic features,treatment,and follow-up of peptic ulcers caused by eosinophilic gastrointestinal disorders(EGIDs)in children,with the aim of improving the level of understanding,diagnosis and treatment of EGIDs in children with the onset of peptic ulcers.Methods:A retrospective analysis was conducted on children admitted to the Department of Gastroenterology,Capital Center for Children's Health,Capital Medical University from January 1st,2019 to October 31st,2023,who underwent complete endoscopic examination. The first examination showed the presence of peptic ulcers(gastric or duodenal ulcers)under the endoscope,and were ultimately diagnosed with peptic ulcers caused by EGIDs through examination and follow-up. The clinical characteristics,laboratory tests,endoscopic results,and treatment follow-up were analyzed.Results:Thirty-five children were EGIDs,22 males and 13 females.Twenty-two cases(62.9%)had abdominal pain as the main symptom.Laboratory tests:17 cases(48.6%)showed a decrease in hemoglobin,15 cases(42.9%)showed an increase in eosinophil count,20 cases(57.1%)tested gastro positive for food allergen specific IgE,and 17 cases(48.6%)showed thickening of the intestinal wall on gastrointestinal ultrasound. Endoscopic features:8 cases(22.9%)showed gastric antral ulcers,including 7 cases(20.0%)with multiple gastric antral ulcers,and 25 cases(71.4%)showed duodenal bulb ulcers.There were 15 cases(42.9%)showed huge ulcers,and 14 cases(40.0%)were located in the duodenal bulb. Comparison of clinical characteristics between children with EGIDs(EGIDs group)and those with peptic ulcers caused by Helicobacter pylori infection(Hp group):the first clinical symptom in both groups was mainly abdominal pain,but the incidence rate in the EGIDs group was lower(62.9% vs 93.5%),and the weigth for length Z score in the EGIDs group was lower[0(-1.6,0.8)vs 1.1(0,1.9)],with statistical significance(all P<0.05). Comparison of laboratory tests:the EGIDs group showed a statistically significant difference in hemoglobin levels[120(101,124)g/L vs 130(100,138)g/L],eosinophil count[0.28(0.13,0.71)× 10 9/L vs 0.16(0.08,0.22)×10 9/L],a positive rate of food allergen specific IgE detection(57.1% vs 32.3%),and a positive rate of intestinal wall thickening detected by gastrointestinal ultrasound(48.6% vs 16.1%)compared with the Hp group(all P<0.05). Comparison of endoscopic examinations:multiple ulcers in the gastric antrum were more common in the EGIDs group than in the Hp group(20.0% vs 0),and the difference was statistically significant( P<0.05). Conclusion:For children with peptic ulcers with onset of abdominal pain,with anemia or malnutrition,or multiple ulcers in the gastric antrum and huge ulcers in the duodenal bulb detected by endoscopy,it is recommended to perform multi site biopsies to help diagnose EGIDs early.
5.Pediatric inflammatory bowel disease in mother‒child pairs: clinical risk factors and gut microbiota characteristics.
Cunzheng ZHANG ; Ruqiao DUAN ; Nini DAI ; Yuzhu CHEN ; Gaonan LI ; Xiao'ang LI ; Xiaolin JI ; Xuemei ZHONG ; Zailing LI ; Liping DUAN
Journal of Zhejiang University. Science. B 2025;26(10):995-1014
OBJECTIVES:
The risk factors and role of mother‒child gut microbiota in pediatric inflammatory bowel disease (PIBD) remain unclear. We aimed to explore the clinical risk factors associated with PIBD, analyze the characteristics of gut microbiota of children and their mothers, and examine the correlation of the microbial composition in mother‒child pairs.
METHODS:
We conducted a case-control study including children with PIBD and their mothers as the case group, as well as healthy children and their mothers as the control group. Questionnaires were used to collect information such as family illness history and maternal and early-life events. Fecal samples were collected from the children and mothers for microbiota 16S ribosomal RNA (rRNA) sequencing to analyze the composition and its potential association with PIBD.
RESULTS:
A total of 54 pairs of cases and 122 pairs of controls were recruited. A family history of autoimmune disease and antibiotic use during pregnancy were associated with an increased risk of PIBD, and a higher education level of the father was associated with a decreased risk of PIBD. Children with PIBD and mothers exhibited different gut microbiota compared to healthy children and mothers. Similarities were observed in the gut microbiota of mothers and children in the same groups. Some bacterial biomarkers of mothers discovered in this study had the power to predict PIBD in their offspring.
CONCLUSIONS
PIBD is influenced by maternal risk factors and has unique gut microbiota characteristics. The mother‒child gut microbiota is closely related, suggesting the transmission and influence of the gut microbiota between mothers and children. This study highlights the potential pathogenesis of PIBD and provides a basis for developing targeted interventions.
Humans
;
Gastrointestinal Microbiome
;
Female
;
Risk Factors
;
Case-Control Studies
;
Male
;
Child
;
Inflammatory Bowel Diseases/etiology*
;
Adult
;
RNA, Ribosomal, 16S/genetics*
;
Feces/microbiology*
;
Mothers
;
Pregnancy
;
Child, Preschool
6.Clinical characteristics of monogenic and non-monogenic early-onset inflammatory bowel disease
Youzhe GONG ; Yanfei CHEN ; Fuping WANG ; Jiao WANG ; Li MENG ; Xi HE ; Xuemei ZHONG
Chinese Journal of Inflammatory Bowel Diseases 2025;09(2):143-148
Objective:To compare the clinical characteristics of monogenic and non-monogenic early-onset inflammatory bowel disease (EO-IBD) in children and to explore the necessity of genetic analysis in EO-IBD research.Methods:A retrospective analysis of clinical data was conducted on 73 children diagnosed with EO-IBD at the Children's Hospital affiliated with Capital Institute of Pediatrics between January 2017 and December 2023. Genetic analysis was performed utilizing next-generation sequencing technology, with patients stratified into monogenic and non-monogenic groups based on the presence or absence of pathogenic mutations. Subsequently, a comparative analysis of clinical characteristics was conducted between these two cohorts of EO-IBD patients.Results:Among the 73 EO-IBD cases, 27 (37%) were diagnosed as monogenic IBD, and 46 (63%) as non-monogenic IBD. Compared to the non-monogenic group, the monogenic group had an earlier age of onset [1 (0.2, 3.0) months vs. 15 (4.1, 51.3) months, P < 0.001], with a higher incidence within the first month of life (70.4% vs. 13.0%, P < 0.001). Monogenic IBD cases were more likely to present with Crohn's disease (CD) phenotypes (88.9% vs. 52.2%, P = 0.003) and colonic involvement (L2) (91.7% vs. 62.5%, P < 0.001), but were less likely to present with non-penetrating, non-stricturing (B1) disease (87.5% vs. 95.8%, P = 0.019). Children in the monogenic group were more prone to severe malnutrition (74.1% vs. 21.3%, P < 0.001), perianal abscesses (40.7% vs. 8.7%, P < 0.001), perianal tags (22.2% vs. 0%, P = 0.004), fever (74.1% vs. 23.9%, P < 0.001), oral ulcers (44.4% vs. 6.5%, P < 0.001), and skin lesions (33.3% vs. 2.2%, P < 0.001). Regarding treatment, the monogenic group had higher usage of thalidomide (88.9% vs. 54.3%, P = 0.002) and hematopoietic stem cell transplantation (HSCT) (37.0% vs. 0, P < 0.001) and a higher mortality rate (22.2% vs. 2.2%, P = 0.017) . Conclusions:For children with IBD presenting at an early age, especially within the first month of life, and showing symptoms like fever, oral ulcers, skin lesions, severe malnutrition, and perianal disease, monogenic IBD should be considered. Genetic testing results can aid in guiding treatment decisions.
7.Biejiajian Pill Regulates Ferroptosis in Hepatocellular Carcinoma Cells via p62/Keap1/NRF2 Signaling Pathway:A Mechanism Study
Weiguang CHEN ; Chunyu HE ; Bin WEN ; Haitao SUN ; Xuemei YANG ; Weicong CHEN ; Yang LIU ; Binglian ZHONG ; Songqi HE
Journal of Sichuan University (Medical Sciences) 2025;56(1):51-58
Objective To investigate the mechanism by which Biejiajian Pill(BJJP)regulates ferroptosis in hepatocellular carcinoma(HCC)cells through the p62/Keap1/NRF2 pathway and to provide an experimental basis for its application in the prevention and treatment of HCC.Methods Huh7 HCC cells were divided into a normal control group,a BJJP drug serum group,an erastin(a ferroptosis inducer)group,a BJJP drug serum+erastin group,and BJJP drug serum+ferrostatin-1(Fer-1)(a ferroptosis inhibitor)group.BJJP drug serum was prepared with animals treated with BJJP and CCK-8 assay was performed to determine the optimal concentration and duration of BJJP intervention.The levels of intracellular iron(Fe),reduced glutathione(GSH),lipid peroxides(MDA),and reactive oxygen species(ROS)were measured.Western blot was performed to determine the expression levels of FTH1,GPX4,xCT,SLC40A1,Keapl,p62,and NRF2.JC-1 staining was performed to measure mitochondrial membrane potential,and cell immunofluorescence was performed to determine the expression of p62 and Keap1.Results According to the CCK-8 assay results,the cell inhibition rate was highest when BJJP was administered at a high dose of 2.2 g/kg(P<0.001).Furthermore,the inhibition rate of Huh7 cells was highest when Huh7 cells were treated with high-dose BJJP drug serum for 48 h.Therefore,the serum concentration of high-dose BJJP and 48 h were selected as the treatment dose and duration for the subsequent experiment.Compared with the control group,the BJJP drug serum group,the erastin group,and the BJJP drug serum+erastin group showed increased iron content,decreased GSH content,increased MDA levels,increased ROS aggregation,decreased FTH1,GPX4,xCT,SLC40A1,p62,and NRF2 contents,increased Keap1 content,and decreased mitochondrial membrane potential(P<0.05).Conclusion BJJP regulates ferroptosis in Huh7 HCC cells by inhibiting the p62/Keap1/NRF2 pathway,demonstrating potentials as a therapeutic agent for HCC.
9.Clinical observation of metformin in the treatment of diabetes mellitus type 2 complicated with sarcopenia in elderly patients
Xuemei ZHONG ; Min CHEN ; Yayun LING ; Bingqian ZHANG
China Pharmacy 2025;36(6):732-736
OBJECTIVE To investigate the efficacy and safety of metformin in the treatment of diabetes mellitus type 2 (T2DM) complicated with sarcopenia in elderly patients. METHODS From January 2022 to January 2024, clinical data from eligible patients with T2DM complicated with sarcopenia treated at the First Affiliated Hospital of Chongqing Medical and Pharmaceutical College were collected. Patients were randomly assigned into control group (70 cases) and observation group (70 cases) using a random number table. Both groups received routine interventions; control group additionally received subcutaneous injections of Insulin glargine injection before bedtime and Human insulin injection 30 minutes before breakfast, lunch and dinner every day. In addition to the same treatments as the control group, the observation group was administered 0.5 g of Metformin hydrochloride sustained-release tablets orally once daily. Both groups were treated continuously for 24 weeks. Comparisons were made between the two groups in terms of glucose metabolism indexes [fasting blood glucose (FBG), 2 h postprandial blood glucose (2 hBG), and glycosylated hemoglobin (HbA1c)], homeostasis model assessment of insulin resistance (HOMA-IR), appendicular skeletal mass muscle index (ASMI), grip strength, walking speed, lipid metabolism indexes [serum total triglyceride (TG), total cholesterol (TC), low-density lipoprotein cholesterol (LDL-C), and high-density lipoprotein cholesterol (HDL-C)], serological markers [high-sensitivity C reactive protein (hs-CRP), interleukin-6 (IL-6), and ferritin levels] and quality of life. The occurrence of ADR was recorded in both groups. RESULTS 65 patients in the control group and 63 patients in the observation group completed this study, respectively. After treatment, the levels of FBG, 2 hBG, HbA1c, HOMA-IR,TG and TC in both groups, and the levels of hs-CRP, IL-6 and ferritin in observation group were all significantly reduced compared to those before treatment (P<0.05), and the HOMA-IR in observation group was significantly lower than control group (P<0.05); additionally, the grip strength, walking speed, and scores for daily living and activity abilities of observation group were increased than those before treatment and the control group (P<0.05). The incidence of adverse drug reactions in both groups was 2.86%. CONCLUSIONS Metformin can reduce inflammatory factors and ferritin levels, promote the recovery of muscle mass and strength, improve insulin resistance, and quality of life in elderly patients with T2DM complicated with sarcopenia, and does not increase the occurrence of adverse drug reactions.
10.Clinical analysis and follow-up outcomes of 25 pediatric cases with hepatic glycogen storage disease
Wenwen LIU ; Meijuan WANG ; Meng JIN ; Ran ZHANG ; Mingran MI ; Xuemei ZHONG
Chinese Journal of Hepatology 2025;33(1):63-69
Objective:To explore the clinical and genetic characteristics and follow-up status of pediatric patients with hepatic glycogen storage disease in order to further improve the prognosis.Methods:The clinical data of hospitalized children diagnosed with hepatic glycogen storage disease in the Department of Gastroenterology at the Children's Hospital of Capital Institute of Pediatrics from January 2010 to April 2023 were collected and retrospectively analyzed. The results of laboratory examination and gene sequencing were analyzed, and the number of cases that exceeded three (n) were grouped according to the genetic results: Group 1 was type Ⅰ ( n=8), Group 2 was type Ⅲ ( n=5), and Group 3 was type Ⅸa ( n=8).The growth, development and prognosis of the children were followed up. The related clinical characteristics of pediatric hepatic glycogen storage disease were summarized. Results:Twenty-five pediatric patients with hepatic glycogen storage disease were enrolled in this study, with fifteen males and ten females. The mean age of diagnosis was (29.1±13.5) months. There were twelve cases (48%) accompanied with varying degrees of hypoglycemia, and two cases (8%) with severe hypoglycemia.There were nineteen cases with stature retardation (76%), four cases with anemia (16%), three cases with proteinuria (12%), and one case with cholestasis (4%).The genetic results showed that there were four cases of type Ⅰa (16%), four cases of type Ⅰb (16%), one case of type Ⅱ (4%), five cases of type Ⅲ (20%), two cases of type Ⅳ (8%), one case of type Ⅵ (4%), and eight cases of type Ⅸ (32%).The three subgroups analysis showed that there were significant statistical differences in uric acid and triglycerides among the three groups ( P<0.05), while there were no statistical significant differences in transaminase levels, fasting blood glucose, lactate, cholesterol, and low-density lipoprotein levels ( P>0.05). The height-for-age Z scores of the three groups were -2.86±1.62, -1.46±1.06, and -1.83±0.98, respectively. The growth and development of groups 2 and 3 were significantly improved compared with group 1 ( P<0.05), with Z scores of -2.28±1.07, 0.20±1.54, and 0.10±1.44 after at least one year of follow-up. All pediatric patients with type Ⅸa had discontinued using raw corn starch after more than one year of follow-up and their transaminases had returned to normal. Four pediatric patients with type Ia were orally administered raw corn starch on a regular basis, and the aminotransferases, uric acid, and lactate were normal, with hypoglycemia being monitored. Among the four cases with type Ⅰb, one had recurrent respiratory tract and intestinal infections, two were combined with Crohn's disease, and one was monitored for hypoglycemia. In four cases of type Ⅲ, raw corn starch was discontinued, and a high-protein, low-carbohydrate diet was adopted, with the exception of the presence of high creatine kinase and normal aminotransferase. Liver failure resulted in the death of one type Ⅵ case, while two were type Ⅳ cases; one died, and one case recently had slightly elevated aminotransferase. Conclusion:When pediatric patients exhibit manifestations such as hepatomegaly, elevated transaminases, fasting hypoglycemia, and delayed growth and development, it is necessary to be alert to hepatic glycogen storage disease. Clinical manifestations and biochemical indicators combined with genetic testing are helpful for the diagnosis of hepatic glycogen storage disease. Simultaneously, targeted nutritional management should be carried out according to the metabolic characteristics of different subtypes, with attention on growth and development status.

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