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 construct a regulatory network of competing endogenous RNA(ceRNA)with prognostic value for bladder urothelial carcinoma(BLCA),and analyze the relationship between key messenger RNA(mRNA)and immune function.Methods The UCSC Xena database was used to download mRNA expression data from 404 BLCA patients and 28 normal individuals and key mRNAs were screened by differential analysis.ENCORI database was utilized to search microRNAs(miRNAs)that bind to key mRNAs and all long non-coding RNAs(LncRNAs)that bind to miRNAs.The expression data of miRNA and LncRNA were downloaded from TCGA database,co-expression analysis was performed to identify key mRNA with all miRNAs and miRNA with all LncRNAs,and thus key miRNAs and LncRNAs were screened out.Survival analysis was conducted based on the differences in expression levels of these key mRNAs,miRNAs,and LncRNAs between tumor patients and normal individuals,and finally a ceRNA regulatory network was constructed.The correlation between key mRNAs and immune cells,immune checkpoints(CD274,PDCD1 and CTLA4),and immune cell marker genes(IG)was analyzed using the TIMER 2.0 database.Results A total of 22 key mRNAs were screened,with the most significant difference being proline 3-hydroxylase 4(P3H4).The expression of P3H4 in patients with BLCA was high,and survival time was shorter in patients with high expression.A sum of 33 miRNAs and 14 LncRNAs were screened using the key mRNAs as the central link.Through co-expression analysis and survival analysis,hsa-miR-151a-3p and MIR100 HG were identified as the key miRNA and key LncRNA with prognostic value.The differences in the above analysis results were statistically significant(all P＜0.05).Based on these findings,a ceRNA regulatory network consisting of 1 mRNA,1 miRNA,and 1 LncRNA was constructed.Immunoassay firstly revealed a significant positive correlation between double positive T cells and P3H4 expression in the tumor microenvironment of BLCA.Moreover,there were 3 types of immune cells(tumor-associated neutrophils,and tumor-associated macrophages,dendritic cells),3 immune checkpoints(CD274,PDCD1,CTLA4),and 15 IGs with significant correlation with P3H4.These differences were statistically significant(all P＜0.01).Conclusion This study could help to reveal the progression mechanism of BLCA.The constructed ceRNA network and immune analysis can offer new insights into potential biological targets and immunotherapy directions for the diagnosis,treatment,and prediction of BLCA patients.

Objective To evaluate the efficacy of medroxyprogesterone acetate(MA)plus metformin as the primary fertility-sparing treatment for atypical endometrial hyperplasia(AEH)and early-stage grade 1 endometrial adenocarcinoma(G1 EAC)and the recurrence rate after treatment.Methods Sixty patients(aged 20-42 years)with AEH and/or grade 1 EAC limited to the endometrium were enrolled prospectively and randomized into two groups(n=30)to receive oral MA treatment at the daily dose of 160 mg(control)or MA plus oral metformin(850 mg,twice a day)for at least 6 months.The treatment could extend to 12 months until a complete response(CR)was achieved,and follow-up hysteroscopy and curettage were performed every 3 months.For all the patients who achieved CR,endometrial expressions of IGFBP-rP1,p-Akt and p-AMPK were detected immunohistochemically.Results A total of 58 patients completed the treatment.After 9 months of treatment,23(76.7%)patients in the combined treatment group and 20(71.4%)in the control group achieved CR;two patients in the control group achieved CR after converting to the combined treatment.The recurrence rate did not differ significantly between the control group and combined treatment group(30.0%vs 22.7%,P>0.05).Ten(35.7%)patients in the control group experienced significant weight gain of 5.7±6.1 kg,while none of the patients receiving the combined treatment exhibited significant body weight changes.Compared with the control group,the patients receiving the combined treatment showed enhanced endometrial expressions of IGFBP-rP1 and p-AMPK with lowered p-Akt expression.Conclusion Metformin combined with MA may provide an effective option for fertility-sparing treatment of AEH and grade 1 stage IA EAC,and the clinical benefits of metformin for controlling MA-induced weight gain and promoting endometrial expressions of IGFBP-rP1 and p-AMPK while inhibiting p-Akt expression warrants further study.

Objective To evaluate the efficacy of medroxyprogesterone acetate(MA)plus metformin as the primary fertility-sparing treatment for atypical endometrial hyperplasia(AEH)and early-stage grade 1 endometrial adenocarcinoma(G1 EAC)and the recurrence rate after treatment.Methods Sixty patients(aged 20-42 years)with AEH and/or grade 1 EAC limited to the endometrium were enrolled prospectively and randomized into two groups(n=30)to receive oral MA treatment at the daily dose of 160 mg(control)or MA plus oral metformin(850 mg,twice a day)for at least 6 months.The treatment could extend to 12 months until a complete response(CR)was achieved,and follow-up hysteroscopy and curettage were performed every 3 months.For all the patients who achieved CR,endometrial expressions of IGFBP-rP1,p-Akt and p-AMPK were detected immunohistochemically.Results A total of 58 patients completed the treatment.After 9 months of treatment,23(76.7%)patients in the combined treatment group and 20(71.4%)in the control group achieved CR;two patients in the control group achieved CR after converting to the combined treatment.The recurrence rate did not differ significantly between the control group and combined treatment group(30.0%vs 22.7%,P>0.05).Ten(35.7%)patients in the control group experienced significant weight gain of 5.7±6.1 kg,while none of the patients receiving the combined treatment exhibited significant body weight changes.Compared with the control group,the patients receiving the combined treatment showed enhanced endometrial expressions of IGFBP-rP1 and p-AMPK with lowered p-Akt expression.Conclusion Metformin combined with MA may provide an effective option for fertility-sparing treatment of AEH and grade 1 stage IA EAC,and the clinical benefits of metformin for controlling MA-induced weight gain and promoting endometrial expressions of IGFBP-rP1 and p-AMPK while inhibiting p-Akt expression warrants further study.

Objective:To investigate the status quo of pain management for cancer patients in hospice care wards of community health service centers.Methods:The electronic medical records of 373 cancer patients admitted in hospice wards of Kangjian Community Health Center of Xuhui District and Jinshanwei Town Community Health Center of Jinshan District from January 2015 to July 2021 were collected. The characteristics of cancer pain, the use of analgesic drugs, the effects of analgesic drugs and its influencing factors were analyzed.Results:The incidence of cancer pain in 373 patients was 93.0% (347/373), and the proportion of moderate to severe cancer pain was 55.6% (193/347). Analgesics were used in 304 patients, among whom 233 (76.6%) patients used oral analgesics, 297 (97.7%) used on time, 97.6%(285/292) used sustained-release opioids, and 94 (30.9%) used combinedly. Breakout pain occurred in 100 cases (32.9%), all of which was controlled with immediate-release morphine. Cancer pain was not relieved in 132 cases (43.42%), and multivariate logistic regression analysis showed that the pain degree on admission (moderate: OR=3.69, 95 %CI:2.09-6.49; severe: OR=5.52, 95 %CI:2.43-12.53), the presence of burst pain ( OR=3.28, 95 %CI:1.77-6.06), the type of analgesics used (non-steroidal+weak opioids: OR=0.39, 95 %CI:0.20-0.76; nonsteroidal+strong opioids: OR=0.20, 95 %CI:0.08-0.51) and the adverse reactions ( OR=1.92, 95 %CI:1.03-3.60) were the influencing factors of pain relief in cancer pain patients (all P<0.05). Conclusion:The pain of cancer patients admitted to community palliative care wards cannot be ignored. Although most cancer pain patients use analgesic drugs in a standard way, there are still a high proportion of patients whose pain is not controlled. Various factors affect the effect of analgesic treatment.

Guideline or consensus for the vaccination of children with special conditions like immunocompromised children may be more suitable for pediatricians.However, the vaccination of children in China is mainly performed by general practitioners or child health care practitioners in community health service centers.They need to master the screening knowledge of contraindications and precautions for the vaccination of children, and make the decision to referral to specialists.Based on the technical guidelines for Immunization of National Health Commission of the People′s Republic of China, Best Practices Guidance of the Advisory Committee on Immunization Practices and contraindications and precautions proposed by the Immunization Action Coalition, 20 suggestions for primary screening of children with vaccination contraindications and referral recommendations for primary care providers were developed by experts from the Integration of Medicine and Prevention in Children of Health Commission of Shenzhen Municipality.

In recent years, more and more reports have been reported on the treatment of refractory primary membranous nephropathy with rituximab in bed, and its effect has been confirmed.A case of refractory primary membranous nephropathy was treated in the Department of Nephrology, Peking university Third Hospital.After the treatment of hormone combined with cyclophosphamide, cyclosporine and tacrolimus was ineffective, the patients were treated with low-dose rituximab for 2 years according to their condition, and the condition of the patients was finally relieved.

Glaucoma, alias'the thief of light', characterized by visual field defects that correspond to damaged areas of the optic nerve head, is a common ophthalmopathy causing irreversible blindness. Thus early diagnosis is crucial. Structural assessment can detect Glaucoma in early stage and diminish the risk of irreversible visual impairment. There are now several apparatus which can clinically measure ONH parameters and confirm DDLS (disc damage likelihood scale) precisely and reproducibly. Comprehensive estimate considering RNFLD (retina nervefiberlayer defects), offers reliable evidence as well as potent support for preclinical detecting, measurement and follow-up observations.

Idiopathic membranous nephropathy(IMN) is one of the main pathology of nephrotic syndrome,and has a rising trend year by year.In recent years that IMN is organ specific autoimmune disease.Phospholipase A2 receptor(PLA2R) and thrombospondin type-1 domain containing 7A(THSD7A) are the two main antigens of IMN,which could be used to distinguish the IMN and secondary membranous nephropathy.The anti-PLA2R antibody and anti-THSD7A antibody are associated with diagnosis,severity and activity of the disease.The antibody titer and its change could be used to help deciding the beginning of therapy and the therapitive courses.

Objective To investigate the clinicopathological features,treatment and prognosis of leiomyomatosis peritonealis disseminata (LPD).Methods A total of 10 patients suffered from LPD after laparoscopic uterine myomectomy were collected in the First Affiliated Hospital of Zhengzhou University from September 2012 to September 2016,and all clinical database were retrospectively analyzed.Results (1)Clinical features:the age of 10 cases was 25-50 years old,and 8 cases of them were in child-bearing age,while 2 cases were in perimenopausal period.Of 10 cases,2 cases manifested as discontinuous lower abdominal pain,and the other cases were seen the doctor for the examinations found tumors of pelvis or abdomen.All 10 cases had a history of laparoscopic uterine myomectomy under went power morcellation with an average of (4.0±2.2) years (range 1.3 to 8.1 years),2 cases of them had a history of oral hormone treatment after the first myoma morcellation.(2) Treatment methods and postoperative pathologic diagnosis:during intraoperative exploration,LPD nodules were most distributed in Douglas pouch (10 cases),and next in mesentery (7 cases),abdominal peritoneum (6 cases) and omentum majus (4 cases),etc.Seven of the 8 cases of child-bearing age were performed laparoscopic LPD nodules removal,1 case gone combined with laparotomy and resecting LPD nodules;2 cases in perimenopausal period done laparotomy oophorotomy and resected all LPD nodules and omentum.(3) Postoperative relapse and reproductive outcomes:the follow-up time of all cases was 2.8 years,and no recurrence was found during the follow-up period;2 cases had natural conception and term vaginal birth during the follow-up period.Conclusions LPD is mainly related to iatrogenic planting and spreading,which is a benign disease and characterized by multiple smooth muscle nodules throughout abdominopelvic cavity,and the nodules of LPD is commonly located in Douglas pouch,mesenteric and omentaum majus,etc.The preferred method of LPD should be individual operative treatment according to different situations,and in which patients may be have better prognosis.