Objective:To investigate the related risk factors of surgical appendectomy after endoscopic retrograde appendicitis therapy (ERAT) in children.Methods:From September 2019 to November 2023 at the Children's Hospital Affiliated to Shandong University,the data from all related children with appendectomy after ERAT were analyzed. The general situation and main clinical manifestations of the children were collected. According to the effect of ERAT,the patients were divided into transfer to surgical group and appendicitis recurrence group by the reasons and time of transfer to surgical treatment. The indexes such as ERAT and reoperation process and pathological types of appendix after operation were collected,and the clinical experience was summarized.Results:Among the 242 children who underwent ERAT,32 cases underwent appendectomy again,including 19 males and 13 females,with an average age of（9.16±2.77）years,and the reoperation rate was 13.2%. The clinical manifestation was abdominal pain (32 cases,100.0%),mainly right lower abdominal pain (28 cases,87.5%),and the main duration was less than 3 months (30 cases,93.8%). Abdominal ultrasound or CT before ERAT mainly indicated appendicitis (11 cases,34.4%) and appendicitis complicated with appendiceal calculus (11 cases,34.4%). There were 9 children in transferred to surgical group,of which 5 cases were transferred to surgery because of ERAT intubation failure,and 4 cases were transferred to surgery because of appendix perforation or abscess. Postoperative pathology showed acute suppurative appendicitis in 5 cases and acute gangrenous appendicitis in 4 cases. There were 23 children in appendicitis recurrence group,whose abdominal pain was relieved after ERAT,but their symptoms were repeated after discharge. After clinical evaluation,they were diagnosed as recurrent appendicitis and underwent surgical appendectomy. Most of them were reoperated within 6 months after ERAT (21 cases,91.3%).Postoperative pathology was mainly chronic appendicitis (10 cases,43.5%).Conclusion:Acute suppurative appendicitis and acute gangrenous appendicitis are prone to catheterization or endoscopic failure during ERAT,and there is a high risk of surgical transfer. ERAT may relieve the symptoms of chronic appendicitis in children. If the symptoms are repeated,surgery is still needed to remove the appendix.

Objective:To explore the characteristics of natural foci of hemorrhagic fever with renal syndrome (HFRS) in Gansu Province.Methods:The information of HFRS case data and rodent density monitoring data from 2012 to 2022 in Gansu Province were collected and epidemiological methods were used to analyze and investigate the characteristics of the epidemic focus.Results:A total of 869 cases of HFRS were reported, and four patients died from 2012 to 2022. The annual incidence rate is between 0.05 per 100 000 and 1.21 per 100 000. The cases were mainly distributed in the eastern, southeast, southern, and south of the central region of Gansu Province. Most cases were distributed between age 20-60, and the sex ratio was 1.85∶1 (564∶305). Most cases were farmers (61.80%, 537/869), herdsmen (19.79%,172/869) and students (6.33%, 55/869). In a wild rat-type epidemic focus,the incidence peak was from November to January of the following year. The natural rodent hosts of HFRS were Rattus norvegicus, Apodemus agrarius, and Mus musculus. The hantaan virus carriage rates were 2.79% (21/754), 0.42% (5/1 179) and 0.31% (2/643),respectively. Three epidemic foci were defined: two derived from the Pingliang and Gannan prefecture new outbreaks epidemic foci, respectively, while the other was the residue of the Dingxi epidemic focus. Conclusions:The southern, south of the central region and eastern part of Gansu Province are current key HFRS epidemic foci dominated by Rattus norvegicus, Apodemus agrarius, and Mus musculus, respectively. The virus genotype is hantaan virus. Case reporting areas should strengthen epidemic monitoring; the key epidemic areas should strengthen and implement various prevention and control measures to reduce the harm caused by HFRS.

Background and aim:Hepatitis B virus(HBV)-related gestational acute-on-chronic liver failure(ACLF)is a severe condition with limited treatment options.This study aimed to evaluate the efficacy and ideal timing of plasma exchange and continuous renal replacement therapy(CRRT)in managing pregnant women with HBV-related ACLF. Methods:This study retrospectively analyzed 51 eligible patients with HBV-related gestational ACLF between 2009 and 2020.Patients admitted to the study were divided into a conventional treatment group and a new treatment group according to whether they received the new management protocol,which included more aggressive plasma exchange(PE)and CRRT strategies.All 19 pregnant women with hepatic encephalopathy(HE)were divided into an early treatment group and a non-early treatment group according to whether PE therapy was initiated within three days.Our study had two primary objectives.Firstly,we aimed to evaluate the impact of PE and CRRT on puerperal survival.Secondly,we sought to assess the effects of early PE and CRRT regimens on puerperal survival in women with HE. Results:The levels of total bilirubin on the second day postpartum(D3),the third day postpartum(D4),and the fifth day postpartum(D6)were significantly lower in the new treatment group compared to the conventional treatment group(P=0.02,0.01,and 0.02,respectively).The ALT of D3 was significantly elevated in the new treatment group compared to the conventional treatment group(P=0.02).The incidence of HE overall increased from prenatal to postpartum D4,peaked on D4,and then gradually decreased from the fourth day postpartum(D5)(P=0.027).The first week after delivery revealed a significant difference in survival rate between the two groups,the conventional treatment group had statistically higher mortality rates compared to the new treatment group(P=0.002).Similarly,the entire puerperal period mortality rate of the conventional treatment group was statistically higher than the new treatment group(P=0.002).Moreover,among all patients with HE,the non-early treatment group showed significantly higher puerperal mortality rates compared to the early treatment group(P=0.006). Conclusions:Early PE and CRRT conducted within three days post-childbirth,enhance puerperal prog-nosis for HBV-related gestational ACLF.

Liver transplantation(LT)is the only effective treatment for hepatopulmonary syndrome(HPS).Moreover,perioperative refractory hypoxemia(pRH)is a prevalent life-threatening condition and has extremely limited treatment options.Here,we report three patients with HPS who experienced pRH after LT and were consecutively treated with different salvage therapies,ephedrine inhalation,intravenous use of methylene blue with nitric oxide(NO)inhalation,and NO inhalation alone.The results showed that unresolved severe hypoxia may induce fatal morbidity such as early biliary leakage and acute kidney injury.Early initiation of NO inhalation,rather than ephedrine,can significantly improve oxygenation in patients with pRH and may help prevent hypoxia-related complications.Therefore,based on the response to these exploratory salvage treatments,we further demonstrate the unique ventilation-perfusion mismatch pathophysiology in specific lung regions during pRH in HPS.We propose that early inhalation of NO is an important treatment option to rescue severe hypoxia in patients with HPS during the perioperative period of LT.

Objective To explore the mechanism of Gusong Yigu Decoction(inchuding Astragali Radix,Codonpsis Radix,Angelicae Sinensis Radix,etc.)in the treatment of postmenopausal osteoporosis(PMOP)based on network pharmacology and animal experiment.Methods The effective ingredients and corresponding targets of Gusong Yigu Decoction were collected by using TCMSP database.GeneCards,TTD,and other databases were used to collect PMOP target proteins.R language was used to obtain the intersection targets and draw Wayne diagram.STRING database was used for the establishment of protein-protein interaction network.At last,GO function enrichment and KEGG pathway enrichment were performed on all common targets.The ovariectomized SD rats were used in the animal experiment.Gusong Yigu Decoction was administered by gavage for 12 weeks.The changes of bone histomorphology were detected by HE staining,the mRNA and protein levels of phosphatidylinositol 3-kinase(PI3K)and protein kinase B(Akt)in bone tissue of proximal tibial were tested by qRT-PCR and Western Blot,respectively.Results A total of 91 effective ingredients of Gusong Yigu Decoction in the treatment of PMOP,70 common targets of drugs-diseases were obtained.GO enrichment analysis mainly included DNA-binding transcription activator activity,RNA polymerase II-specific,ubiquitin protein ligase binding.KEGG pathway enrichment analysis included PI3K/Akt signaling pathway,TNF signaling pathway,and apoptosis.The animal experiment showed that bone histomorphology was significantly improved,meanwhile the mRNA and protein expressions of PI3K and Akt were significantly increased in Gusong Yigu Decoction group(P＜0.01).Conclusion Gusong Yigu Decoction may improve bone microstructure through multiple channels and targets.Gusong Yigu Decoction can increase the number and thickness of bone trabeculae and reduce the separation of bone trabeculae by activating PI3K/Akt signaling pathway,and thus play an anti-osteoporosis role in postmenopausal osteoporosis.

OBJECTIVE: To analyze the clinical and genetic characteristics of a boy featuring unexplained developmental delay, malnutrition and distinct facial appearance. METHODS: Physical examination was carried out for the child. Peripheral blood samples were collected from the child and his parents for the extraction of genomic DNA and trio-whole exome sequencing. Candidate variants were verified by Sanger sequencing. RESULTS: The patient had facial dysmorphism including nasal alae aplasia, scalp defect and teeth deformities, in addition with recurrent diarrhea due to pancreatic exocrine insufficiency. DNA sequencing revealed that he has harbored compound heterozygous variants of the UBR1 gene, namely c.3167C>G (p.S1056X) and c.1911+14C>G, which were inherited from his father and mother, respectively. Database search has suggested the c.3167C>G to be a novel nonsense variant and c.1911+14C>G a known splicing variant. Based on the guidelines of the American College of Medical Genetics and Genomics, the two variants were predicted to be pathogenic and likely pathogenic, respectively. CONCLUSION The child was diagnosed with Johanson-Blizzard syndrome due to the compound heterozygous variants of the UBR1 gene. Above finding has enriched the mutational spectrum of the UBR1 gene and provided a basis for genetic counseling for this family.
Child ; Humans ; Male ; Ectodermal Dysplasia/genetics* ; Pancreatic Diseases/genetics* ; Ubiquitin-Protein Ligases/genetics*

Objective:To evaluate the clinical value of microprobe endoscopic ultrasonography in the diagnosis and treatment of children with protuberant lesions in the upper gastrointestinal tract.Methods:A retrospective study was performed to analyze the clinical data of children who underwent microprobe endoscopic ultrasonography for treating protuberant lesions in the upper gastrointestinal tract in the Department of Gastroenterology, Jinan Children′s Hospital from January 2018 to June 2021.The endoscopic ultrasound characteristics of children with protuberant lesions in the upper gastrointestinal tract were summarized and compared with the pathological results.Results:Microprobe endoscopic ultrasonography was performed in 29 children.Ectopic pancreas was found in 12 cases (41.4%), Brunner gland hyperplasia in 4 cases (13.8%), cysts in 3 cases (10.3%), duodenum accessory nipple in 3 cases (10.3%), extragastric compression in 2 cases (6.9%), lymphoma in 2 cases (6.9%), gastric duplication malformation in 1 case (3.4%), stromal tumor in 1 case (3.4%) and leiomyoma in 1 case (3.4%). According to the results of microprobe endoscopic ultrasonography, 15 cases with protuberant lesions were treated by deep biopsy and handled under endoscope.The tissue was checked by pathological examination.The microprobe endoscopic ultrasonography diagnosis of 14 cases were in accordance with their pathological diagnosis[93.3% (14/15)].Conclusions:Microprobe endoscopic ultrasonography can effectively diagnose and differentiate protuberant lesions in the upper gastroi-ntestinal tract of children, so it can be used to guide the clinical treatment under endoscope.Microprobe endoscopic ultrasonography is a safe and reliable treatment for children.

Objective:To compare oxytocin combined with ergometrine with oxytocin alone in terms of primary prophylaxis for postpartum hemorrhage (PPH) at the time of cesarean section (CS).Methods:This was a multicenter double-blind randomized controlled interventional study comparing ergometrine combined with oxytocin and oxytocin alone administered at CS. From December 2018 to November 2019, a total of 298 parturients were enrolled in 16 hospitals nationwide. They were randomly divided into experimental group (ergometrine intra-myometrial injection following oxytocin intravenously; 148 cases) and control group (oxytocin intra-myometrial injection following oxytocin intravenously; 150 cases) according to 1∶1 random allocation. The following indexes were compared between the two groups: (1) main index: blood loss 2 hours (h) after delivery; (2) secondary indicators: postpartum blood loss at 6 h and 24 h, placental retention time, incidence of PPH, the proportion of additional use of uterine contraction drugs, hemostatic drugs or other hemostatic measures at 2 h and 24 h after delivery, the proportion requiring blood transfusion, and the proportion of prolonged hospital stay due to poor uterine involution; (3) safety indicators: nausea, vomiting, dizziness and other adverse reactions, and blood pressure at each time point of administration.Results:(1) The blood loss at 2 h after delivery in the experimental group [(402±18) ml] was less than that in the control group [(505±18) ml], and the difference was statistically significant ( P<0.05). (2) The blood loss at 6 h and 24 h after delivery in the experimental group were less than those in the control group, and the differences were statistically significant (all P<0.05). There were no significant differences between the two groups in the incidence of PPH, the proportion of additional use of uterine contraction drugs, hemostatic drugs or other hemostatic measures at 2 h and 24 h after delivery, the proportion requiring blood transfusion, and the proportion of prolonged hospital stay due to poor uterine involution (all P>0.05). (3) Adverse reactions occurred in 2 cases (1.4%, 2/148) in the experimental group and 1 case (0.7%, 1/150) in the control group. There was no significant difference between the two groups ( P>0.05). The systolic blood pressure within 2.0 h and diastolic blood pressure within 1.5 h of drug administration in the experimental group were higher than those in the control group, and the differences were statistically significant ( P<0.05), but the blood pressure of the two groups were in the normal range. Conclusion:The use of ergometrine injection in CS could reduce the amount of PPH, which is safe and feasible.

Organ transplantation is the primary effective treatment for end-stage organ failure. Donor-derived infection (DDI) is significantly associated with the incidence of infection and mortality of the recipients after organ transplantation. Improvement of donor screening technology and early prevention and treatment can improve the safety of transplantation. In this article, the pathogenic characteristics of DDI bacterial infection, fungal infection, viral infection and parasitic infection were summarized, and the research progress upon the prevention and treatment were briefly introduced, aiming to provide reference for reducing DDI.

Children′s esophageal stenosis is mainly benign stenosis. Esophageal foreign bodies and corrosion are common causes of esophageal stenosis in children requiring emergency management. Endoscop￣ic treatment of esophageal stricture includes endoscopic balloon dilatation,endoscopic radial incision,medical treatement,endoscopic stenting and so on. According to the cause of the stenosis,the nature of the lesion and the morphological structure of the lesion,formulating appropriate endoscopic treatment strategy are important for therapeutic effect and reducing the incidence of complications.