The paper reports the treatment of a maintenance hemodialysis patient with pseudoaneurysm (PSA) caused by accidental injury of brachial artery during the puncture of internal fistula. The main treatment methods of PSA include surgical incision and repair, local pressure therapy, ultrasound-guided intraluminal thrombin injection, implantation of covered stent, coil embolization and so on, but they all have some defects. The patient was admitted to hospital due to poor fistula function, and the formation of brachial artery PSA was confirmed by color ultrasound. PSA was successfully treated with ultrasound-guided and balloon-assisted injection of human fibrin sealant. The fistula had good function 3 months after the operation.

Objective To investigate the effect of NOD-like receptor family pyrin domain containing 3(NLRP3)knockdown on a mouse model of nonalcoholic steatohepatitis(NASH)induced by high-fat high-carbohydrate(HFHC)diet.Methods A total of 44 mice were randomly divided into normal diet group(CON group)with 20 mice and HFHC group with 24 mice.At the end of week 14 of modeling,4 mice were randomly selected from the HFHC group for the pre-experiment of adeno-associated virus(AAV)by tail vein injection,and NLRP3 knockdown was verified after 4 weeks.After NLRP3 knockdown was verified at the end of week 18,the remaining 40 mice were given a single tail vein injection of AAV,and then they were divided into CON+NLRP3 knockdown negative control group(CON+NLRP3-NC group),CON+NLRP3 knockdown group(CON+NLRP3-KD group),HFHC+NLRP3-NC group,and HFHC+NLRP3-KD group,with 10 mice in each group.At the end of week 24,the activation of NLRP3 inflammasome was observed;related indicators were measured,including body weight,liver weight,liver index,and glucose metabolism(fasting blood glucose,fasting insulin,and Homeostasis Model Assessment of Insulin Resistance[HOMA-IR]index);the indicators of liver lipid content(liver triglyceride[TG]and oil red O staining),liver inflammation(serum alanine aminotransferase[ALT]activity,HE staining,and inflammation-related genes),and liver fibrosis(Sirius Red staining and fibrosis-related genes)were measured.A one-way analysis of variance was used for comparison of continuous data between multiple groups,and the least significant difference t-test was used for further comparison between two groups.Results Compared with the CON+NLRP3-NC group based on the results of Western Blot,the HFHC+NLRP3-NC group had significant increases in the protein expression levels of NLRP3,pro-Caspase1,Caspase1,ASC,and IL-1β,while the HFHC+NLRP3-KD group had significant reductions in these levels(all P<0.05).The HFHC+NLRP3-NC group showed varying degrees of increase in body weight,liver weight,liver index,and glucose metabolism indicators,while the HFHC+NLRP3-KD group showed significant improvements in these indicators(all P<0.05).As for hepatic fat deposition,compared with the CON+NLRP3-NC group,the HFHC+NLRP3-NC group had a significant increase in liver TG,with a large number of red lipid droplets shown by oil red O staining,and the HFHC+NLRP3-KD group had significant reductions in liver TG and the number of lipid droplets in the liver(all P<0.01).In terms of liver inflammation,compared with the CON+NLRP3-NC group,the HFHC+NLRP3-NC group had significant increases in serum ALT,NAFLD activity score,and inflammation-related genes,while the HFHC+NLRP3-KD group had significant reductions in these indicators(all P<0.01).As for liver fibrosis,compared with the CON+NLRP3-NC group,the HFHC+NLRP3-NC group had significant increases in collagen fiber area and fibrosis-related genes,and the HFHC+NLRP3-KD group had significant reductions in fibrosis-related genes(all P<0.05)and a tendency of reduction in collagen fiber area(P>0.05).Conclusion NLRP3 knockdown can significantly improve hepatic fat deposition and inflammation in a mouse model of HFHC-induced NASH.

Objective:To explore the characteristics, clinical manifestations and gene mutation types of Cornelia de Lange syndrome (CdLs), and to improve the understanding of the disease.Methods:Clinical data and gene test results of a pediatric case of CdLs diagnosed in the First Affiliated Hospital of Xinxiang Medical University in August 2019 were analyzed retrospectively.Results:A female patient with 2 years and 8 months old presented a special appearance with a low and flat nose, a wide eye distance, audition ears, a downward inclination of the mouth corner, a high arch of the jaw and a small jaw deformity, who had recurrent seizures, speech and mental retardation.The result of gene test showed the mutation of SMC1A gene c. 2923C > T, and thus the patient was diagnosed as type 2 CdLs. Conclusions:CdLs is a rare genetic metabolic disease with special facial features and physical signs.There is only one case of CdLs with gene mutation of SMC1A in China through literature review.The mutation of SMC1A gene c. 2923C>T in CdLs cases has not been reported at home and abroad, which expands the variation spectrum of the SMC1A gene.

Objective:To summarize the clinical and gene mutation characteristics of a child with Cowden syndrome and review the literature.Methods:The clinical data of a child with Cowden syndrome treated in the First Affiliated Hospital of Xinxiang Medical University in June 2020 were analyzed retrospectively.Taking " Cowden syndrome" , " PTEN gene" , " hamartoma polyps" , "child" , " Cowden syndrome and child" and " PTEN and child" as key words, literature was retrieved from Chinese databases (China National Knowledge Internet database and Wanfang database) and the PubMed database from the establishment of the database to March 2021. Results:A 13-year-old male had intermittent abdominal pain and abdominal distension for 5 months.Electron microscopic gastroenteroscopy showed multiple polyps, and focal lymphocyte aggregation and infiltration were found in tissue biopsy.Whole exon sequencing revealed a hemizygous mutation of c. 475 (exon5) A>T in PTEN gene, which led to the transformation of the 159 th amino acid from arginine to tryptophan.The prediction results of the tertiary structure of the protein indicated that the variation might affect the spatial structure of the protein and damage the protein function.According to the clinical characteristics, Cowden syndrome was diagnosed.The pedigree confirmed that the variation was inherited from the mother, who had a similar phenotype.No qualified Chinese report was retrieved.Among 41 English studies on PTEN gene mutation in children, there were only two reports related to pediatric Cowden syndrome.The hemizygous mutation of PTEN gene was not reported. Conclusions:The missense mutation of c. 475 (exon5) A>T in PTEN gene in this study is a novel cause of Cowden syndrome, and the case is the first case report in China.

A case of primary amoebic meningoencephalitis (PAM) treated in the First Affiliated Hospital of Xinxiang Medical University in March 6, 2022 was reported.The proband was a 14-year-old boy, who was admitted to the hospital because of " fever, headache and vomiting for 2 days" . Metagenomic next-generation sequencing (mNGS) was consequently conducted to examine the pathogens in cerebrospinal fluid.Naegleria infection was detected, so the child was diagnosed with PAM.The disease developed rapidly, and the patient died 29 hours after admission.In the paper, a total of 13 studies were reviewed, and 15 children with PAM were reported.Of the reported cases, only 1 case survived, 14 cases died.PAM had a low incidence, a dangerous condition, and high mortality.Most cases were diagnosed by autopsy or pathogen diagnosis in cerebrospinal fluid.This case is confirmed by mNGS of pathogens, and it is rarely reported at home and abroad.

Objective:To observe the efficacy and adverse reactions of adrenocorticotropic hormone (ACTH) in the treatment of recurrent frequently relapsing nephrotic syndrome (FRNS), and explore the feasibility of treatment of ACTH in children.Methods:From November 2017 to June 2018, in the First Affiliated Hospital of Xinxiang Medical University of a total of 32 cases of FRNS ACTH therapy were all the role of ACTH consecutive 3-8 courses of treatment (when the dosage of prednisone was less than or equal to 0.5 mg/kg, 0.4 U/kg ACTH should be used every day.After 3 days of continuous application, the dosage of Prednisone should be reduced to 1.25-5.00 mg.ACTH was used for 3 days as a course of treatment, and continued to use ACTH for 2 courses until corticosteroid stopped). The number of recurrence, Prednisone maintenance dose, immunosuppressive use, serum cortisol and other relevant experimental indicators and adverse reactions were recorded during the follow-up period after ACTH treatment, and were followed up for 8-15 months.Results:Among the 32 children, the onset age (53.47±25.81) months, the course of disease (35.25±23.87) months, 22 patients (68.75%) had no recurrence after corticosteroid withdrawal, 7 patients (21.87%) had recurrence after corticosteroid withdrawal, and 3 patients (9.37%) had recurrence during corticosteroid withdra-wal.Compared with before ACTH treatment, Prednisone was significantly decreased[(0.08±0.14) mg/(kg·d) vs. (0.23±0.23) mg/(kg·d)], and the difference was statistically significant ( t=3.661, P<0.05), the number of immunosuppressant cases decreased significantly[42.38%(12/32 cases) vs. 58.62%(17/32 cases), χ2= 14.500, P<0.05]. Serum cortisol was measured at 8 Am increased significantly[(11.78±4.64) μg/dL vs. (4.42±3.13) μg/dL, t=7.340, P<0.05]. The results were stable during follow-up, with 2 patients presenting with systemic urticaria once and 1 patient presenting with headache after infusion. Conclusions:ACTH therapy is safe and effective in hormone therapy for child with FRNS, and less adverse reactions.

Objective Tubulointerstitial fibrosis(TIF) is the most important marker reflecting the degree of renal function decline and prognosis and hydrogen sulfide ( H2 S) is crucial in maintaining normal renal function and many diseases of renal injury. The aim of the article was to investigate the effects of exogenous H2 S on the expressions of angiotensinⅡ ( AngⅡ) , proliferating cell nuclear antigen (PCNA) and transforming growth factor beta-1 (TGF-β1) in rats with unilateral ureteral obstruction (UUO). Methods TIF rat model was built with UUO. Ninety-six SD rats were randomly divided into four groups:sham operation group, modelgroup, UUO+low-dose NaHS treatment group ( low dose group) and UUO+high-dose NaHS treatment group ( high dose group) ( n=24, respectively) . Rats in model group were treated with left-side ureteral obstruction and ureteral separation without obstruction was done in sham operation group. UUO rats in two treatment groups were injected intraperitoneally with two different doses of sodium hydrosulfide (NaHS, donor of endogenous H2 S), respectively. HE and Massonstaining and immunohistochemical staining were performed at the 7 d, 14 d and 21 d, respectively. Results In sham operation group, the expressions of AngⅡ, PCNA, and TGF-β1 were found in microamount in tubulointerstitium at each time points. Compared with sham operation group, the expressions of AngⅡ, PCNA and TGF-β1 in model group increased( P<0.01) . While in comparison to model group, the expressions of AngⅡ, PCNA and TGF-β1 decreased in low dose group and high dose group, but no significant differ-ence was found between low dose group and high dose group. Conclusion Exogenous H2 S supplementation can attenuate TIF partly via downregulating the expressions of AngⅡ, PCNA and TGF-β1 .

Objective:To study the effect of quality control circle(QCC)on the reasonable ratio of clinical prescriptions. Methods:The dispensed prescriptions in orthopedic emergency department were reviewed in our hospital,and the reasons of unreasonable prescriptions were analyzed. According to the QCC technique,the activities were implemented,the standardized work process was made out and the results were studied. Results:After the six-month QCC activities,the unreasonable ratio of emergency orthopedics prescriptions was reduced from 70% to 21% ,and the target yield rate was 140% and the improvement rate was 70% . Conclusion:The QCC has obvious effect on the improvement of reasonable ratio of emergency orthopedics prescriptions.

OBJECTIVE: To analysis the characteristics of sudden sensorineural hearing loss (SSHL) patients with tinnitus, and explore the relationship of characteristics of tinnitus and audiology. METHOD: Patients diagnosed as SSHL with tinnitus were studied in the research. All patients' clinical features were analyzed, such as tinnitus frequency, pure tone audiometry, tinnitus, hearing loss degree, results of residual inhibition test. RESULT: Thirty cases were identified as mild degree hearing loss, 13 cases as moderate degree, 28 cases as severe degree and 34 cases as profound degree. And hearing impaired frequency of 13 cases was ascertained at low-frequency, 39 cases at middle-high-frequency, and 53 cases at full-range-frequency. The incidence of patients with low-frequency was about 41. 9% (44/105), and it was about 21. 9% (23/105) in those with middle-frequency. And it was 36. 2% (38/105) in cases of high-frequency tinnitus. The chi-square test show statistically significant differences between patients with the low-frequency, middle-frequency and high-frequency of the hearing loss (P<0. 05). In tinnitus residual inhibition test, positive rate of convergence type masking curve was about 72.0%, tinnitus separated type masking curve 20.0%, overlapping type was 57.9%, and the spacing type was 43.5%. There was a statistically significant difference among cases with different type masking curve (P<0. 05)with the spacing residual inhibition test positive rate. CONCLUSION There are individual differences of clinical characteristics among SSHL patients with tinnitus. Tinnitus frequency is consistent with the frequency of hearing loss. Patients had the more serious the degree of hearing loss, who had more serious tinnitus grading. Cases with the converged type curve will be fit for tinnitus masking. Therefore, combining the tinnitus detection with the audiological tests, we could obtain the clinical characteristics of SSHL patients with tinnitus.
Audiometry, Pure-Tone ; Deafness ; Hearing Loss ; Hearing Loss, Sensorineural ; complications ; pathology ; Humans ; Tinnitus ; complications ; pathology