Objective:To analyze the clinical characteristics and gene variations of children with cystic fibrosis (CF), and to improve the understanding of pediatrician on CF.Methods:The data of 7 children with CF admitted to Xi'an Children 's Hospital from July 2018 to January 2024 were retrospectively analyzed. Results:The 7 patients (2 males and 5 females) were diagnosed at a median age of 3 years and 9 month(ranging from 1 year and 1 month to 10 years).The main clinical manifestations included recurrent respiratory tract infection (7 cases), bronchiectasis (3 cases), malnutrition (5 cases), steatosis (5 cases), false Bartter syndrome(2 cases), cirrhosis (1case),infantile vomiting (1 case),and meconium intestinal obstruction (1 case). A total of 14 mutation sites were found in 7 cases by whole exon sequencing and Sanger sequencing verification, including 7 missense mutations, 4 nonsenses mutations, 2 shear mutations, and 1 frameshift mutation which was a new mutation. After symptomatic treatment including anti-infection, chest physical therapy, hypertonic saline atomization, fat-soluble vitamin supplementation and pancreatic enzyme supplementation, the respiratory symptoms were controlled, the number of hospitalization due to respiratory infection was significantly reduced. However，the nutritional status of the 7 children did not reach the expected improvement.Conclusion:The clinical manifestations of CF are varied, among which recurrent respiratory tract infection, bronchiectasis and malnutrition are common.Timely targeted anti-infection and other symptomatic treatments can improve the prognosis. The compliance of the confirmed children with nutritional support is poor, and it is still necessary to strengthen the intervention of nutritional support.

Objective:To analyze the clinical characteristics and gene variations of children with cystic fibrosis (CF), and to improve the understanding of pediatrician on CF.Methods:The data of 7 children with CF admitted to Xi'an Children 's Hospital from July 2018 to January 2024 were retrospectively analyzed. Results:The 7 patients (2 males and 5 females) were diagnosed at a median age of 3 years and 9 month(ranging from 1 year and 1 month to 10 years).The main clinical manifestations included recurrent respiratory tract infection (7 cases), bronchiectasis (3 cases), malnutrition (5 cases), steatosis (5 cases), false Bartter syndrome(2 cases), cirrhosis (1case),infantile vomiting (1 case),and meconium intestinal obstruction (1 case). A total of 14 mutation sites were found in 7 cases by whole exon sequencing and Sanger sequencing verification, including 7 missense mutations, 4 nonsenses mutations, 2 shear mutations, and 1 frameshift mutation which was a new mutation. After symptomatic treatment including anti-infection, chest physical therapy, hypertonic saline atomization, fat-soluble vitamin supplementation and pancreatic enzyme supplementation, the respiratory symptoms were controlled, the number of hospitalization due to respiratory infection was significantly reduced. However，the nutritional status of the 7 children did not reach the expected improvement.Conclusion:The clinical manifestations of CF are varied, among which recurrent respiratory tract infection, bronchiectasis and malnutrition are common.Timely targeted anti-infection and other symptomatic treatments can improve the prognosis. The compliance of the confirmed children with nutritional support is poor, and it is still necessary to strengthen the intervention of nutritional support.

Objective To investigate the value of the peripheral blood neutrophil to lymphocyte ratio(NLR)before nimotuzumab combined with neoadjuvant chemotherapy in predicting the short-term efficacy of neoadjuvant therapy for advanced oral squamous cell carcinoma(OSCC).Methods With the approval of the Ethics Committee and the in-formed consent of the patients,59 patients with stage Ⅲ and Ⅳ OSCC who were admitted to the Oral and Maxillofacial Surgery Department of the First Hospital of Shanxi Medical University from September 2020 to June 2023 were en-rolled.All the patients had complete clinical data,were pathologically diagnosed with squamous cell carcinoma,and re-ceived preoperative and received preoperative nimotuzumab+TP(docetaxel+cisplatin)neoadjuvant chemotherapy.The clinical data were analyzed,and the neutrophil and lymphocyte counts in peripheral blood were collected before and after nimotuzumab combined with neoadjuvant chemotherapy.The NLR was calculated,and the threshold value was calculated using the receiver operating characteristic(ROC)curve.Patients were divided into a high NLR group and a low NLR group according to the NLR threshold before nimotuzumab combined with neoadjuvant chemotherapy with TP.The clinical efficacy after nimotuzumab combined with neoadjuvant chemotherapy with TP was evaluated according to the evaluation criteria for solid tumor efficacy,and the correlation between the NLR and recent neoadjuvant therapy effi-cacy was analyzed.Immunohistochemical staining was used to detect the expression of epidermal growth factor receptor(EGFR)in OSCC tissues before and after nimotuzumab combined with neoadjuvant chemotherapy with TP and to ana-lyze whether the expression of EGFR differed among the different NLR groups.Results A total of 59 patients with ad-vanced OSCC were included.According to the ROC curve,the NLR threshold was 2.377,and the patients were divided into a＜2.377 group(low NLR group),with 24 patients,and a＞2.377 group(high NLR group),with 35 patients.The short-term neoadjuvant therapy effect was significantly greater in the lower NLR group than in the higher NLR group(P＜0.05);EGFR expression in both the low NLR group and the high NLR group decreased after nimotuzumab com-bined with neoadjuvant chemotherapy with TP,and the decrease in the low NLR group was significantly greater than that in the high NLR group(P＜0.05).Conclusion A low NLR before nimotuzumab combined with neoadjuvant che-motherapy with TP is associated with better neoadjuvant therapy outcomes,and such patients are more likely to benefit from preoperative nimotuzumab combined with neoadjuvant chemotherapy.

The accessory parotid gland is a normal salivary gland tissue completely separated from the main parotid gland,which is mostly located in the middle of the face.Any tumor originating from the parotid gland can occur in the accessory parotid gland.Due to the special location of the disease,the diagnosis and treatment of accessory parotid gland tumor is more difficult and more dangerous than that of the parotid gland tumor.This article reviews the anatomy and physiology,clinical diagnosis and treatment of accessory pa-rotid gland tumors,in order to provide guidance for clinical diagnosis and treatment.

In view of the surgical complexity of parapharyngeal space tumors involved, this paper summarized the disease data of patients with parapharyngeal space tumors involved in the Department of Oral and Maxillofacial Surgery, the First Hospital of Shanxi Medical University from January 2015 to January 2021. It also summarized the surgical approach and mandibular management, so as to explore surgical strategies for different characteristics of parapharyngeal space tumors involved. A total of 49 patients, including 28 males and 21 females, median age 52 years (range 24-72 years). They were treated with four surgical approaches for tumor resection, 25 cervical approach, 5 cheek and neck approach, 3 transoral approach, and 16 cervical-maxillary approach. Among the patients treated with cervical-maxillary approach, 3 patients were treated with mandible square resection, and 6 patients were treated with temporary mandible dissection. Seven cases were treated with tumor resection and partial mandibular resection. There are various surgical approaches and mandibular management methods involving tumors in the parapharyngeal space, and clinical decisions should be made based on tumor diameter, location, boundary, blood supply and pathological types.

Objective: To investigate the application value of 3D microscope in vascular anastomosis in oral and maxillofacial surgery, to provide a reference for clinicians. Methods : Eighty-seven cases of free flap reconstruction in oral and maxillofacial surgery were retrospectively included, including 30 cases in the 3D microscope group and 57 cases in the optical microscope group. The differences in intraoperative vascular anastomosis time, postoperative flap survival rate and doctor evaluation scores between the 3D microscope group and the optical microscope group were compared and statistically analyzed, and the feasibility of using three-dimensional microscope in surgery was evaluated. Results : The arterial anastomosis time was (26.53±3.83) min/root in the 3D microscope group and (24.88 ± 2.97) min/root in the optical microscope group, and the difference was statistically significant (P<0.05). The venous anastomosis time was (30.68 ± 3.51) min/root in the three-dimensional microscope group and (28.70 ± 2.91) min/root in the optical microscope group, and the difference was statistically significant (P<0.05). There was no significant difference in the survival rate of flaps between the 3D microscope group (n = 28, 93.33%) and the optical microscope group (n = 53, 92.98%) (P>0.05). The doctor's evaluation scores of visual fatigue, training and learning, operative difficulty index, image sharing in the three-dimensional microscope group were higher than those in the optical microscope group, and the differences were statistically significant (P<0.05). Conclusion 3D microscope has good reliability and safety in surgery, a strong sense of three-dimensionality, and the convenience of teaching and training. It can be well applied to vascular anastomosis in oral and maxillofacial surgery.

The depth of invasion is a new index in the 8th edition of TNM classification and staging of oral cancer. Currently, there is no standardized evaluation method for the diagnosis of bone invasion and depth of invasion in lower gingival squamous cell carcinoma (LGSCC). The evaluation of LGSCC bone invasion depth not only provides a reference for surgical margin, but also determines the choice of surgical method, and is an independent prognostic factor for predicting cervical lymphatic metastasis. At present, the main evaluation methods of LGSCC bone invasion and invasion depth include X-ray, MRI, CT, positron emission tomography(PET)/CT, PET/MRI, singlephoton emission CT(SPECT)/CT and pathological examination. In this paper, the evaluation methods and effects of LGSCC bone invasion and invasion depth are summarized, and its advantages and disadvantages are analyzed in order to provide reference for clinical application.

Objective: To summarize the clinical manifestations of IgG4-related diseases in the head and neck, explore treatment methods. Methods : The clinical data of 21 patients diagnosed with IgG4-related diseases were retrospectively analyzed. The clinical data and the results of glucocorticoid and immunosuppressive therapy were studied retrospectively. Results: All patients had swollen sclerotic masses, and CT showed irregular high-density masses with uniform enhancement in the enlarged glands. Some patients had mucosal thickening and mass-like changes in theoral cavity, nose, sinuses, throat and other tissues, and most of the patients had cervical lymphadenopathy and elevated serum IgG4 levels (≥ 1.35 g/L). Histopathological examination of affected exosine glands and affected mucosa and lymph nodes in all patients showed infiltration of lymphocytes, plasma cells and IgG4+ plasma cells. In 21 patients, the mass in the affected glands and mucosa (including head, neck and other tissues) disappeared, and the clinical symptoms were relieved after the application of glucocorticoids. However, with a reduction in glucocorticoids, the mass recurred or even worsened. Conclusion For patients with a single mass in the submandibular gland, parotid gland and other salivary glands, as well as lymph node enlargement, CT is the first choice to identify the nature of gland neoplasms. Combined with pathological examination, related auxiliary examination and peripheral blood examination are also needed to obtain a definitive diagnosis. Glucocorticoid therapy is used to achieve a good prognosis, and long-term follow-up and timely adjustment of medication regimens are required.

Objective : To analyze the accuracy of the infiltrating depth of tongue squamous cell carcinoma measured by magnetic resonance imaging (MRI) using pathological sections under a light microscope to provide a clinical reference. Methods : Seventy-three patients with tongue squamous cell carcinoma who visited the Department of Stomatology of the First Hospital of Shanxi Medical University and Xiangya Stomatological Hospital from January 2018 to September 2020 were selected. Preoperative MRI was performed to evaluate the infiltration depth of tongue squamous cell carcinoma, and intraoperative frozen pathological sections were used to confirm the infiltration depth of tongue squamous cell carcinoma measurement. Results : The infiltration depth of tongue squamous cell carcinoma measured by T1-weighted imaging was 1.11 mm (95% CI=0.51-1.70; t=3.72; P < 0.001), and the correlation coefficient r was 0.95. The T2-weighted average overestimation was 2.17 mm (95% CI=1.32-3.02; t=5.10; P < 0.001), and the correlation coefficient was 0.92. The Bland-Altman plot showed good consistency between T1- and T2-weighted images and pathologic measurements. Conclusion The infiltration depth of tongue squamous cell carcinoma measured by MRI is more accurate, with an average overestimation of 1-2 mm compared with pathological measurements, and T1-weighted images are better than T2-weighted images.

Objective: To examine the outcome of surgical treatment in patients with stages Ⅱ-Ⅲ bisphosphonate-related osteonecrosis of the jaw. Methods: Twenty-nine patients with bisphosphonate-related osteonecrosis of the jaw were examined. The patients were followed up for more than 6 months, and the treatment outcome was reviewed. Results: After curettage of local lesions, 19 out of the 21 patients were cured, and 2 were relieved of symptoms. Six patients underwent subtotal resection of the maxilla, and the symptoms disappeared completely after the surgery. Two patients underwent partial resection of the mandible and recovered. Conclusion Surgical debridement is an effective measure for the treatment of patients with bisphosphonate-related osteonecrosis of the jaw in stages Ⅱ-Ⅲ. In most cases, curettage of local lesions via the intraoral approach can completely remove sequestrum and inflammatory granulomatous tissue. Subtotal maxillary resection or partial mandible resection is performed when the bone death reaches the level of the maxillary sinus floor or continues to the mandible. By timely surgical intervention, the bone lesion is removed to maintain the sterile, active bone microenvironment locally.