Objective:To summarize the clinical features of reversible posterior encephalopathy syndrome(PRES)after allogeneic hematopoietic stem cell transplantation(allo-HSCT)in children.Methods:The clinical data of six children who developed PRES after undergoing allo-HSCT in the Department of Hematology of Wuhan Children's Hospital from June 2016 to December 2022 were retrospectively analyzed,and their clinical characteristics,imaging examination,laboratory examination,and treatment regression were summarized.Results:Among 281 children underwent allo-HSCT,6 cases(2.14％)developed PRES,with a median age of 5.1(1.5-9.7)years old.4 cases underwent related haploidentical donor transplantation,and 2 cases underwent sibling allografting and unrelated donor allografting donor transplantation,respectively.All six children had an acute onset of illness,with clinical manifestations of nausea and vomiting,seizures,psychiatric disorders,visual disturbances.The five cases elevated blood pressure.All children with PRES were treated with oral immunosuppressive drugs during seizures,and 3 cases were combined with different degrees of graft-versus-host disease.Most of the children showed effective improvement in clinical symptoms and imaging after adjusting/discontinuing suspected medications(cyclosporine,etc.)and symptomatic supportive treatments(oral antihypertensive,diazepam for antispasmodic,mannitol to lower cranial blood pressure),and one of them relapsed more than 8 months after the first seizure.Conclusion:PRES is rare after hematopoietic stem cell transplantation in children,and its onset may be related to hypertension,cytotoxic drugs,graft-versus-host disease,etc.Most of them can be recovered after active treatment,but not completely reversible,and the prognosis of those who combined with TMA is poor.

OBJECTIVE: To investigate the efficacy and safety of VDZ (Vedolizumab) in the salvage treatment of glucocorticoid resistance to gastrointestinal graft-versus-host disease (GR-GI GVHD) after allogeneic hematopoietic stem cell transplantation (allo-HSCT) in children. METHODS: The clinical data of 5 patients with refractory GI GVHD who received allo-HSCT in Wuhan Children's Hospital from December 2020 to December 2021 were retrospectively analyzed with VDZ salvage therapy. RESULTS: Among the 5 children with refractory GI GVHD, there were 1 male and 4 female, including 2 cases of extremely severe aplastic anemia, 1 case of acute myeloid leukemia (M2, high-risk), 1 case of fanconi anemia and 1 case of myelodysplastic syndrome. The median age of transplant recipients was 54.4 (12-164) months. The median treatment time from transplantation to VDZ was 1.4 (0.6-6.8) months. On average, 3.5 (2-5) doses of VDZ were received. After receiving treatment, 2 patients achieved a complete response (CR), 2 patients achieved a very good partial response (VGPR), 1 patient was non-responsive (NR) after a short-term partial response (PR). Compared with that before VDZ treatment, the amount of diarrhea, stool color, blood and traits of the children after medication were effectively improved. The median follow-up time was 9.3 (7.23-12.83) months. No disseminated or severe bacterial/fungal infections occurred during VDZ treatment and follow-up, and 2 children died of leukemia recurrence and pulmonary bronchiolitis obliterans. CONCLUSION VDZ salvage treatment of refractory GI GVHD in children has obvious short-term efficacy and good safety.
Child ; Humans ; Female ; Male ; Child, Preschool ; Salvage Therapy ; Glucocorticoids/therapeutic use* ; Retrospective Studies ; Graft vs Host Disease

Objective: The relationship between serum uric acid (SUA) levels and glycemic indices, including plasma glucose (FPG), 2-hour postload glucose (2h-PG), and glycated hemoglobin (HbA1c), remains inconclusive. We aimed to explore the associations between glycemic indices and SUA levels in the general Chinese population. Methods: The current study was a cross-sectional analysis using the first follow-up survey data from The China Cardiometabolic Disease and Cancer Cohort Study. A total of 105,922 community-dwelling adults aged ≥ 40 years underwent the oral glucose tolerance test and uric acid assessment. The nonlinear relationships between glycemic indices and SUA levels were explored using generalized additive models. Results: A total of 30,941 men and 62,361 women were eligible for the current analysis. Generalized additive models verified the inverted U-shaped association between glycemic indices and SUA levels, but with different inflection points in men and women. The thresholds for FPG, 2h-PG, and HbA1c for men and women were 6.5/8.0 mmol/L, 11.0/14.0 mmol/L, and 6.1/6.5, respectively (SUA levels increased with increasing glycemic indices before the inflection points and then eventually decreased with further increases in the glycemic indices). Conclusion An inverted U-shaped association was observed between major glycemic indices and uric acid levels in both sexes, while the inflection points were reached earlier in men than in women.
Aged ; Asian Continental Ancestry Group ; Blood Glucose/analysis* ; China/epidemiology* ; Cohort Studies ; Diabetes Mellitus/blood* ; Female ; Glucose Tolerance Test ; Glycated Hemoglobin A/analysis* ; Glycemic Index ; Humans ; Male ; Middle Aged ; Uric Acid/blood*

Abdomen ; Hernia ; Humans ; Prostheses and Implants ; Prosthesis Implantation ; Treatment Outcome

BACKGROUND: Gastric cancer (GC) is one of the most globally prevalent cancers in the world. The pathogenesis of GC has not been fully elucidated, and there still lacks effective targeted therapeutics. The influence of altered kinesin superfamily protein 22 (KIF22) expression in GC progression is still unclearly. The aim of this study was to investigate the KIF22 effects on GC and related mechanisms. METHODS: Gastric carcinoma tissues and matching non-cancerous tissues were collected from patients with GC who have accepted a radical gastrectomy in Lanzhou University Second Hospital from May 2013 to December 2014. The expression of KIF22 was examined in GC of 67 patients and 20 para-carcinoma tissues by immunochemical staining. The relationship between the expression of KIF22 and clinicopathologic characteristics was next investigated in the remaining 52 patients except for 15 patients who did not complete follow-up for 5 years. Cell viability was performed via 3-(4,5-dimethyl-2-thiazolyl)-2,5-diphenyl-2-H-tetrazolium bromide (MTT) test and colony formation assay in the MGC-803 and BGC-823 GC cells. Cell scratch and trans-well invasion assay was performed to assess migration ability in the MGC-803 and BGC-823 GC cells. Gene set enrichment analysis (GSEA) pathway enrichment analysis was performed to explore the potential functions. Cell cycle was detected by flow cytometry. In addition, the two GC cell lines were used to elucidate the underlying mechanism of KIF22 in GC in vitro via assessing the effects on mitogen-activated protein kinase and extracellular regulated protein kinases (MAPK/ERK) signal transduction pathway-related expressions by Western blotting assays. The differences were compared by t tests, one-way analysis of variance, and Chi-squared tests. RESULTS: The study showed that KIF22 was up-regulated in GC, and KIF22 high expression was significantly related to differentiation degree (χ = 12.842, P = 0.002) and poorly overall survivals. GSEA pathway enrichment analysis showed that KIF22 was correlated with the cell cycle. Silence of KIF22 decreased the ability of the proliferation and migration in gastric cells, induced G1/S phase cell cycle arrest via regulating the MAPK-ERK pathways. CONCLUSIONS KIF22 protein level was negatively correlated with prognosis. KIF22 knockdown might inhibit proliferation and metastasis of GC cells via the MAPK-ERK signaling pathway.

Objective:To explore a method of improving the nursing service ability after total hip arthroplasty (THA) among nurses in villages and towns medical health institutions under the county medical community model and to evaluate its effects.Methods:From June 1 2018 to September 30 2019, we took Department of Orthopedics, Dazhu County People's Hospital of Dazhou of Sichuan Province, as the core, and united with county medical community built by villages and towns medical health institutions with the country in organizing the nursing theoretical knowledge and specialist service ability training after THA for 140 nurses of villages and towns medical health institutions within county medical community. Before and after training, we carried out the theoretical test and practice ability test in trainers, and implemented the consistency analysis in scores of nursing assessment scales for specialist service so as to evaluate the subjective training effect. We also compared the incidence of postoperative complications, functional recovery of hip joint as well as the satisfaction among 30 THA patients before (October 2017 to September 2018) and after (October 2018 to September 2019) training so as to explore the clinical effect.Results:Before and after training, scores of theoretical achievements of nurses of villages and towns medical health institutions were 60 (56, 62) and 82 (79, 85) respectively, and scores of practice ability were 52 (47, 62) and 86 (81, 91) respectively with statistical differences ( P<0.01) . After training, the incidence of postoperative complications was lower than that before training (6.67% vs. 26.67%) ; the postoperative satisfaction, scores of Harris Hip Score (HHS) one month and three months after surgery were higher than those before training [90.00% vs. 66.67%, (73.1±6.00) vs. (57.6±6.67) , (86.6±4.49) vs. (74.5±6.20) ]among 30 THA patients with statistical differences ( P<0.05) . Conclusions:Training with the county medical community model can improve the nursing service ability of nurses in villages and towns medical health institutions among THA patients, postoperative function of hip joint of THA patients as well as patient satisfaction.

Objective To evaluate the efficacy of clarithromycin (CAM) treatment in adult Chinese patients suffering from chronic rhinosinusitis with nasal polyps (CRSwNP) or without nasal polyps (CRSsNP).Methods A prospective,open and self-controlled clinical trial on patients with CRS was conducted.Fifty patients met inclusion criteria.Of 50 patients,there were 33 patients with CRSsNP and 17 patients with CRSwNP.CAM was administered at 250 mg/d and the duration of administration was 12 weeks.Outcome measures included assessments of visual analogue scale (VAS),the sino-nasal outcome test-20(SNOT-20),the medical outcomes study short-form 36 items (SF-36),Lunid-Kennedy endoscopy score,and Lund-Mackay computed tomography score.Before starting the treatment,2 months after treatment and at the end of treatment,each patient had to complete all the measures except Lund-Mackay computed tomography score,which was only conducted before and after treatment.In order to evaluate the safety of CAM,liver function and renal function in all patients were detected before and after treatment.SPSS 16.0 software was used to analyze the data.Results Forty-five patients completed 3 months follow-up and 5 patients withdrew due to different reasons.The results were as follows:(1) Thirty-three patients with CRSsNP's VAS scores of four time point were 5.81 ± 1.69,3.76 ± 1.94,2.98 ± 1.95,2.06 ±2.13,respectively,there were statistically significant improvements in turn (t values were 5.910,8.090,8.932,all P ＜0.05).Endoscopy score of four time point were 6.28 ± 1.28,5.O0 ± 1.67,4.12 ± 1.76,3.12 ±2.19,respectively,there were statistically significant improvements in tum compared with before treatment (t values were 6.662,9.161,9.936,all P ＜ 0.05).The CT scores before and after treatment were 10.33 ±4.65 and 4.67 ±4.59,respectively(t =7.226,P =0.000).(2) Seventeen patients with CRSwNP's VAS scores of four time point were 6.07 ± 2.02,4.87 ± 2.61,4.06 ± 2.85,4.08 ± 2.80,respectively,there were statistically significant improvements after 2 or 3 months (t values were 3.285,3.468,both P ＜ 0.05) except after one month (t =1.846,P ＞ 0.05).Endoscopy score of four time point were 10.65 ±1.77,9.35 ± 1.93,8.65 ± 2.76,8.47 ± 2.76,respectively,there were statistically significant improvements in turn(t values were 4.068,4.863,5.156,all P ＜0.05).The CT scores before and after treatment were 13.82 ± 4.94 and 11.41 ± 5.12,respectively (t =3.975,P =0.001).(3) During the period of CAM treatment,1 patient reported a tolerable headache and weakness and 1 patient had abdominal pain after two months treatment,all the symptoms disappeared while they were asked to stop the drug.Liver function and renal function were detected in 40 patients,the differences before and after treatment were not significant statistically.Conclusions Long-term,low-dose CAM treatment is effective in the treatment of CRSsNP and CRSwNP in Chinese patients.Meanwhile,the efficacy of CAM is more significant in polyp-free group compared with polyp group.Low does CAM therapy is safe,and the liver function and renal function does not worsen after 3 months treatment.

BACKGROUND: Clinical genetics and molecular biology studies have shown that the occurrence and development of the keloid is closely related to the inheritance. However, it remians unclear if the same is ture to the hypertrophic scar. OBJECTIVE: To investigate similadties and differences of genetic alteration between the hyperplastic scar and the keloid, DESIGN, TIME AND SETTING: A contrast observational experiment was performed in Guangdong Medical College between March 2007 and December 2008.MATERIALS: Scar samples were taken from 16 patients (in-patient and out-patient) in the Department of Plastic Surgery, the Affiliated Hospital of Guangdong Medical College, with10 patients with hypertrophic scars (3 males and 7 females, 20-50 years old) and 6 patients with keloids (1 males and 5 females, 19-46 years old). METHODS: The DNA of both hyperplastic scar and keloid tissues was extracted to investigate, using comparative genomic hybridization technique, the genomic imbalance (the lose or amplification of genetic material), so as to make a comparative study on differences of the DNA copy number changes between the two. RESULTS: Neither altofrequent loss nor amplification of DNA copy number was found in any specific DNA region of hyperplastic scar tissues; as for the keloid, special DNA altofrequent loss regions were also not found, but altofrequent DNA copy number loss regions presented in 1, 16, 20 and 22 chromosomes. Comparatively, the keloid presented much higher loss rate of the DNA copy number in 1,16,20 and 22 chromosomes than the hyperplastic scar (P < 0.05).CONCLUSION: The hyperplastic scar has no conspicuous DNA copy number lose or amplification compared with the keloid, which indicates that the occurrence and development of the hyperplastic scar may not have any direct relation with the inheritance.

BACKGROUNDHypertrophic obstructive cardiomyopathy (HOCM) carries an increased risk for sudden cardiac death. No data regarding the percutaneous transseptal myocardial ablation (PTSMA) and epicardial left ventricular pacing (LVP) were reported.

METHODSSeven patients with recurrent symptoms and increased resting left ventricular outflow tract pressure gradient (LVOTG) after PTSMA and another 14 patients with HOCM without history of PTSMA were studied. Both resting and dobutamine stress echocardiography, PTSMA and LVP were routinely performed.

RESULTSIn patients without previous PTSMA procedure, mild reduction of resting LVOTG was detected at 5 minutes after left ventricular pacing, and this reduction became significant at 10 minutes. All patients were divided into successful and unsuccessful groups according to their response to LVP. In contrary to patients in unsuccessful group, resting and R-S2 stimuli-induced LVOTG during PTSMA procedure were decreased dramatically ((9 +/- 5) mmHg vs (58 +/- 12) mmHg, (12 +/- 2) mmHg vs (113 +/- 27) mmHg, P < 0.001). Analysis of Logistic regression demonstrated that only LVOTG level during left ventricular pacing was an independent factor predicting the reduction of LVOTG immediately after PTSMA (odds ratio (OR), 0.59; 95% CI 2.67 to 5.82; P = 0.0002).

CONCLUSIONLeft ventricular endocardial temporary pacing plays a critical role in predicting acute effect on the reduction of LVOTG immediately after PTSMA procedure.

Adult ; Cardiac Pacing, Artificial ; Cardiomyopathy, Hypertrophic ; diagnostic imaging ; physiopathology ; therapy ; Catheter Ablation ; Echocardiography ; Female ; Humans ; Logistic Models ; Male ; Middle Aged ; Pressure ; Ventricular Function, Left

OBJECTIVETo investigate the relationship between the polymorphic (AT)n repeats in 3ountranslated region of exon 4 of CTLA4 gene [CTLA4(AT)n] and Graveso disease (GD) in Zhuang nationality population of Guangxi province.

METHODSThe studied groups comprised 48 patients with GD and 44 normal controls. Amplification of target DNA was carried out by polymerase chain reaction (PCR). The amplified products were run by 8% polyacrylamide gel electrophoresis, and then followed by 0.1% silver staining. Some of amplified products were sequenced directly.

RESULTSNineteen alleles of CTLA4 gene microsatellite polymorphism were found in Guangxi Zhuang nationality individuals. The 106 bp long allele was apparently increased in patients with GD of Zhuang nationality but not in healthy controls (P< 0.05).

CONCLUSIONCTLA4 gene microsatellite polymorphism is strongly associated with Graveso disease in Zhuang nationality population of Guangxi province. CTLA4(AT)n 106 bp may be the susceptible gene in GD patients of Zhuang nationality in Guangxi; 19 alleles of CTLA4 gene microsatellite polymorphism were found in Guangxi Zhuang nationality individuals.

Adult ; Antigens, CD ; genetics ; Base Sequence ; CTLA-4 Antigen ; China ; Dinucleotide Repeats ; genetics ; Female ; Genetic Predisposition to Disease ; genetics ; Graves Disease ; genetics ; Humans ; Male ; Middle Aged ; Molecular Sequence Data ; Polymerase Chain Reaction ; Polymorphism, Genetic ; genetics ; Sequence Analysis, DNA