Objective:To investigate the expression and the significance of inflammatory cytokine IL-6 through regulating Gli1 in acute pancreatitis.Methods: In this study,C57 mice were randomly divided into three groups:control group,model group,inhibitor group.caerulein intraperitoneal injection induce acute pancreatitis model.Use HE staining and amylase to testify the model successfully.Use RT-qPCR,Western blot to detect the expression of Gli1 in the pancreas,liver,lung,kidney and intestine and ELISA method to detect inflammatory cytokines IL-6.Results: Compared with control group,the expression of Gli1 is higher in model group,then the expression of IL-6 increases in inhibitor group which uses Gant61 to suppress Gli1 compared with model group.Conclusion: Gli1 may involved in the process of the distant tissue injury and repair in acute pancreatitis and through regulate its downstream cytokines like IL-6 to play a protective role in acute pancreatitis.

OBJECTIVE: To explore the clinical manifestations in pediatric laryngopharyngeal reflux and to provide more information on diagnosis and treatment. METHOD: Sixty-two cases with recurrent respiratory infections, hoarseness and chronic cough were examined with reflux symptom index (RSI), reflux finding score (RFS), and 24-hour pH monitoring. Those who had at least two positive test for reflux were given PPI for diagnostic therapy. RESULT: All patients completed reflux symptom index (RSI) questionnaire and underwent fiberoptic laryngoscopy, and reflux finding score (RFS) was evaluated. The positive rate of RSI and RFS was 91.94% and 79.03% differently. 24-hours pH monitoring and diagnostic therapy was about 30.76% and 85.48% differently. The symptoms show hoarseness 90.32%, postnasal drip 77.42%, difficulty swallowing 74.19%, abdominal pain and chest pain 72.58%, throat clearing 64.52%, chronic cough 56.45%, dysphagia 51.61%, throat abnormal feeling 48.39%. Laryngoscope examination shows inter-arytenoid erythema 100%, vocal mucosal oedema 75.81%, diffuse laryngeal edema 50.00%, posterior commissure hypertrophy 33.87%, subglottic edema 4.84%, no granuloma case. There was 16 cases showing positive in 24-hours pH monitoring test. The positive rate was 30.76%. All cases accepted diagnostic therapy. Fifty-three cases were effective. The positive rate was 85.48%. CONCLUSION There are no clinical presentations specific to pediatric laryngopharyngeal reflux. Patients often present with a wide range of atypical symptoms and signs. RSI questionnaire and RFS may provide diagnostic datas. Primary treatment includes lifestyle and medical therapy.
Adolescent ; Child ; Child, Preschool ; Female ; Humans ; Infant ; Laryngopharyngeal Reflux ; diagnosis ; pathology ; therapy ; Male

Objective:To investigate the clinicopathological features, molecular genetics, treatment and prognosis of Burkitt-like lymphoma with 11q aberration (BLL-11q).Methods:Six cases of BLL-11q diagnosed at the First Affiliated Hospital of Zhengzhou University, from January 2016 to January 2020 were reviewed and analyzed using hematoxylin-eosin staining, immunohistochemistry, EBER in situ hybridization and fluorescence in situ hybridization. Clinical information including follow-up data was collected and analyzed.Results:The median age of the six immunocompetent patients was 29 years (range 20-38 years) and the male to female ratio was 5∶1. All patients had nodal disease in the head and neck region. Five patients had Ann Arbor stage Ⅰ-Ⅱ disease, while one patient had stage Ⅳ disease. Lymph nodes showed partial or total architectural effacement by a diffuse proliferation of monomorphic lymphocytes. Four cases were morphologically similar to Burkitt lymphoma, and two cases were unclassified with histological features between Burkitt lymphoma and diffuse large B-cell lymphoma. Mitotic figures, apoptosis and necrosis were conspicuous. Five cases exhibited the"starry sky"pattern. CD20, CD10 and bcl-6 were diffusely and strongly positive. The Ki-67 index was more than 95%. The follicular-dendritic-cell meshwork was noted in one case using CD21 stain. C-MYC was expressed variably. CD3, bcl-2, MUM-1, CD30 and TDT were negative in all cases. EBER in situ hybridization was also all negative. FISH analyses using C-MYC, bcl-2 and bcl-6 break-apart probes were all negative. All cases had the 11q23.3 gain/11q24.3 loss pattern, and 11q23.3 amplification was found in one case. IgH and IRF4 break-apart probes analysis was also negative. All patients were alive with no disease after a follow-up of 4 to 19 months.Conclusion:BLL-11q is a rare lymphoma that resembles Burkitt lymphoma morphologically and phenotypically, but lacks C-MYC gene rearrangements. Instead, it has a chromosome-11q alteration characterized by proximal gains and telomeric losses. It′s necessary to improve our understanding of BLL-11q to avoid misdiagnosis and missed diagnosis.

Objective:To investigate the clinicopathological, immunophenotypic and molecular genetic characteristics, and differential diagnosis of NTRK-rearranged spindle cell neoplasms (NTRK-RSCNs) in the gastrointestinal tract.Methods:Two NTRK-RSCNs diagnosed at the Department of Pathology of the First Affiliated Hospital of Zhengzhou University, Zhengzhou, China and one case diagnosed at Zhengzhou Central Hospital, Zhengzhou, China from 2019 to 2022 were collected. The clinical data, histopathology, immunophenotypes and prognosis were analyzed. Fluorescence in situ hybridization (FISH) and next-generation sequencing (NGS) were used to detect NTRK gene rearrangements, while relevant literature was also reviewed and discussed.Results:Two patients were male and one was female, with the age of 17, 47 and 62 years, respectively. The tumors were located in the duodenum, ascending colon and descending colon, respectively. The tumors were protuberant masses with gray and rubbery sections. Their maximum diameter was 2.5, 5.0 and 10.0 cm, respectively. Histologically, the tumors invaded mucosa, intrinsic muscle and serosal adipose tissue. Tumor cells consisted of spindle or oval shaped cells with monotonous morphology and arranged in bundles or stripes pattern. Spindle cells were mildly to moderately atypical, with slightly eosinophilic cytoplasm and inconspicuous nucleoli. Necrosis and mitotic figures were observed in one high-grade tumor. All tumors expressed CD34, S-100 and pan-TRK in varying degrees. FISH analysis showed that NTRK1 gene was break-apart in 1 case and NTRK2 gene break-apart in 2 cases. NGS technologies showed LMNA::NTRK1 fusion in one case, STRN::NTRK2 fusion in another case. All patients recovered well after the surgery without recurrence at the end of the follow-up.Conclusions:NTRK-RSCN is rarely diagnosed in the gastrointestinal tract and has significant variations in morphology. It overlaps with various other mesenchymal tumors which should be considered as differential diagnoses. Be familiar with the features of histological morphology in combination with immunophenotype and molecular genetic characteristics can not only help diagnose NTRK-RSCNs, but provide therapeutic targets for clinical treatment.

Microsurgery techniques have allowed the development of many new therapeutic methods in plastic surgery, but are difficult to master without hard training. It is very important to set up a standardized microsurgery curriculum and training system for broadening surgical skills training and investigating the plastic surgery specialist training strategy. In our experiences, a series of training models are needed, like non-animal models, non- living animal models, live animal models and so on. This paper shows the training strategy for the primary stage of microsurgery training, non-animal model and non-living animal model training.

Microsurgery techniques have allowed the development of many new therapeutic methods in plastic surgery, but are difficult to master without hard training. It is very important to set up a standardized microsurgery curriculum and training system for broadening surgical skills training and investigating the plastic surgery specialist training strategy. In our experiences, a series of training models are needed, like non-animal models, non- living animal models, live animal models and so on. This paper shows the training strategy for the primary stage of microsurgery training, non-animal model and non-living animal model training.

Objective:To explore the predictive value of psoas muscle index(PMI)on early survival and complications after liver transplantation(LT)in children with biliary atresia(BA).Methods:Between January 1, 2016 and September 30, 2020, the relevant clinical data are retrospectively reviewed for 244 BA children undergoing LT at Department of Pediatric Liver Transplantation, Tianjin First Central Hospital.Total psoas muscle area(PMA)at the level of the third lumbar endplate is measured based upon preoperative abdominal computed tomography(CT)and normalized by the square of length for obtaining the value of PMI.According to the survival at Year 1 post-LT, receiver operating characteristic(ROC)curve is plotted and the cut-off value calculated.According to the cut-off value, they are divided into two groups of high PMI(173 cases)and low PMI(71 cases). Then the clinical data of 2 groups are compared.Kaplan-Meier survival curves at Year 1 post-LT are analyzed.And Cox proportional hazard model is utilized for conducting a multivariate analysis of early death.Results:The cut-off value of PMI is 534.6 mm 2/m 2.Two groups are compared in terms of age, gender, weight-for-age Z-score(WAZ), length-for-age Z-score(LAZ)and preoperative laboratory parameters ( P>0.05). Significant inter-group differences existed in the types of donors and surgery( P<0.05), and the median age of recipients in low PMI is higher than high PMI group(7.77months vs 6.57 months, P<0.01). The proportion of children with a history of Kasai surgery in low PMI group(78.87% vs 53.76%, P<0.01)and the median length of stay in hospital(23 d vs 20 d, P=0.03)is higher in low PMI group, but recipients(88.7% vs 97.1%, P<0.01)and grafts(87.3% vs 96%, P=0.01)1-year survival rate are lower.Multivariate analysis indicated that high PMI is a protective factor for early postoperative survival( HR=0.132, 95% CI: 0.028~0.626, P=0.011). Conclusions:PMI is an independent predictor of early survival post-LT and has a certain predictive value for early graft loss in BA children.

Objective: To analyze the causes and management plan of pediatric spontaneous tonsillar haemorrhage(STH). Methods: According to the criteria of STH difined by Griffies, patients with STH from December 2013 to January 2016 were included in this retrospective study. Results: A total of 11 patients were reviewed. The etiological diagnosis included 3 pediatric Epstein-Barr virus associated infectious mononucleosis(EBV-IM), 3 suspected pediatric EBV-IM to 3 acute suppurative tonsillitis, 1 acute viral tonsillitis and 1 hemophilia A. The management strategies included antiviral, antibacteria, transfusion, surgical examination followed with bipolar coagulation hemostasis under general anesthesia. No patient treated with tosillectomy. Conclusions STH is now a rare condition, the causes of which in child are more or less different from adult.This emergency can be treated in time if a detailed management plan for pediatric STH is formulated.

Objectives:To report the sexual functional outcomes of vaginal dilation therapy in Mayer-Rokitansky-Küster-Hauser (MRKH) syndrome patients.Methods:From March 2020 to February 2023, 97 MRKH syndrome patients performed vaginal dilation therapy with guidance from Peking Union Medical College Hospital, and 45 of them engaged in penetrative intercourse and were included in this prospective cohort study. The Chinese version of female sexual function index (FSFI) was used to assess sexual function. Functional success was defined as FSFI>23.45. Forty age-matched healthy women were selected as controls. Kaplan-Meier survival analysis was used to calculate the median time to success. Pearson correlation analysis was used to explore the relationship between neovagina length and sexual function. Complications were collected using follow-up questionnaires.Results:The functional success rate of vaginal dilation therapy was 89% (40/45) with a median time to success of 4.3 months (95% CI: 3.0-6.1 months). Compared to controls, MRKH syndrome patients had significantly lower scores in the orgasm domain (4.72±1.01 vs 4.09±1.20; P=0.013) and pain domain (5.03±0.96 vs 4.26±0.83; P<0.001). However, there were no significant differences in the FSFI total score (26.77±2.70 vs 26.70±2.33; P=0.912), arousal domain (4.43±0.77 vs 4.56±0.63; P=0.422) and satisfaction domain (4.88±0.98 vs 4.65±0.86; P=0.269) between MRKH syndrome patients and controls. MRKH syndrome patients had significantly higher scores in the desire domain (3.33±0.85 vs 3.95±0.73; P<0.001) and lubrication domain (4.37±0.56 vs 5.20±0.67; P<0.001). The prevalence of sexual dysfunction in MRKH patients was non-inferior to controls: low desire [3% (1/40) vs 23% (9/40); P=0.007], arousal disorder [3% (1/40) vs 3% (1/40); P>0.999], lubrication disorder [5% (2/40) vs 25% (10/40); P=0.012], orgasm disorder [40% (16/40) vs 20% (8/40); P=0.051], sexual pain [30% (12/40) vs 15% (6/40); P=0.108]. Conclusions:MRKH syndrome patients undergoing non-invasive vaginal dilation therapy could achieve satisfactory sexual life. Given its high functional success rate and slight complication, vaginal dilation therapy should be recommended as the first-line option, reducing the need for unnecessary surgeries.

Objective: To investigate the diagnosis and management of laryngeal cleft. Method: The clinical data of 13 cases of laryngeal cleft treated between 2007 and 2015 was analyzed retrospectively. Results: The children with laryngeal cleft were classified according to the classification of Benjamin-Inglis, as type Ⅰ(11 cases), typeⅡ(1 case) and type Ⅲ(1 case). All patients were confirmed by microlaryngobronchoscopy under general anaesthetic. Eleven typeⅠ and 1 type Ⅱ clefts were managed conservatively, with which all type Ⅰ patients were successfully managed, while the type Ⅱ patient was resolved by surgical endoscopy. The type Ⅲ patient was treated by open repair but the results was poor. Conclusions Patients who suffered with choking on feeding or recurrent aspiration pneumonia, especially coexisted with other congenital malformation, needed detailed evaluation for laryngeal cleft, although which was a rare congenital abnormality. Electronic laryngoscope could be the first step to screen the cleft, while microlaryngobronchoscopy is the gold standard for diagnosis of laryngeal cleft. The majority of children with lower type clefts can be managed conservatively. Surgical endoscopy has high success rate when strictly following the indication. Type Ⅲ and Ⅳ clefts have high mortality for usually combining with severe complications and abnormalities.