Neurofibromatosis type 1 (NF1) presents with a diverse range of symptoms that can affect the skin, bones, eyes, central nervous system, and other organs. This article reports the diagnosis and treatment process of a patient with NF1 complicated by bilateral severe-to-profound sensorineural hearing loss. Genetic testing revealed a heterozygous variant of NF1 NM_ 000267.3: c.4054_ 4058del(p.Ser1352LeufsTer20, supporting the diagnosis of NF1. After thorough evaluation, a right cochlear implantation was performed, yielding satisfactory postoperative results. This case suggests that NF1 patients may exhibit phenotypic heterogeneity and atypicality, providing a reference for clinicians in the diagnosis and treatment of such patients.

Objective:To explore the relationship between parental rearing style and mental health of community correction offenders, as well as the mediating role of personality and coping styles in it.Methods:A questionnaire survey was conducted on 385 community correction offenders by the Egma Minnen av Bardndosna uppforstran(EMBU), symptom checklist-90 (SCL-90), NEO five-factor inventory(NEO-FFI) and simplified coping style questionnaire(SCSQ) from February to July 2022.SPSS 27.0 software was used for correlation analysis and regression analysis.AMOS 26.0 was used for the construction of structural equation, and Bootstrap was used for mediating effect analysis.Results:In parental rearing styles, the dimension of parents' severe punishment(19.09±5.32, 12.93±4.77), father's preference for subjects(9.29±3.30), father's excessive protection(10.40±2.19), mother's excessive interference and protection(33.81±6.06)and parents' refusal and denial (9.08±3.03, 12.17±4.25) were significantly positively correlated with the total score of SCL-90 (140.63±44.28)( r=0.114, 0.168, 0.121, 0.144, 0.224, 0.187, 0.220 respectively, all P<0.05). Parents' emotional warmth and understanding, parents' severe punishment and parents' refusal and denial were significantly correlated with coping styles ( r=0.420, 0.420, -0.189, -0.190, -0.174, -0.163 respectively, P<0.05). Neuroticism, extraversion, conformity, rigor in personality were significantly correlated with the total score of SCL-90 ( r=0.542, -0.442, -0.204, -0.202 respectively, P<0.05). Coping style was significantly negatively correlated with the total score of SCL-90 ( r=-0.352, P<0.05). The father's refusal and denial, the mother's emotional warmth and understanding, the mother's excessive interference and protection in the parental rearing styles could predict the mental health of community correction offenders ( β=0.191, -0.163, 0.233 respectively, P<0.05). Coping styles had a negative predictive effect on mental health ( β=-0.352, P<0.05). Neuroticism, preciseness and extra-version in personality could predict mental health ( β=0.461, 0.183, -0.281 respectively, P<0.05). The pathway coefficient of parental rearing styles → mental health was 0.261 ( P<0.001), and the direct effect was significant. The confidence intervals of parental rearing styles → personality traits → mental health path, parental rearing styles → coping tendency → mental health path did not include 0, indicating that partial mediating effect of personality traits and coping tendency were significant. Conclusion:Personality traits and coping styles play a mediating role in the influence of parental rearing style on mental health of community correction offenders.

Objective:To provide more options for preoperative localization diagnosis in patients with primary hyperparathyroidism (PHPT), the diagnostic efficacy of parathyroid 4-dimensional computed tomography (4D-CT) in patients with PHPT was evaluated.Methods:This was a single-center retrospective study including 57 patients with surgical proved PHPT. All of the patients underwent 4D-CT, 99Tc m -sestamibi parathyroid imaging (MIBI), and ultrasonography (US) preoperatively. The reference standard for correct localization was based on operation reports and pathology confirmation. The patients were grouped according to the preoperative serum calcium levels, tumor diameter, or ectopic lesions (yes/no), respectively. The sensitivity, specificity, positive predictive value, negative predictive value and area under the curve (AUC) of 4D-CT, MIBI and US, alone or in combination, were analyzed in total and each subgroup patients. Results:Fifty-seven patients (39 women, 18 men; mean age of 56.5 years) were evaluated, including four cases with multi-gland disease and thirteen cases with ectopic parathyroid lesions. In all the patients, similar diagnostic efficacy was found in 4D-CT (AUC: 0.943) and MIBI (AUC: 0.927), both of which were higher than that of US (AUC: 0.847) ( P = 0.01 for 4D-CT vs. US; P = 0.04 for MIBI vs. US). In a subset analysis for ectopic quadrants, the diagnostic efficacy of 4D-CT was significantly higher than that of MIBI ( P = 0.04) or US ( P = 0.01), with the sensitivity of 100%, 69.2%, and 61.5%, and AUC of 0.989, 0.846, and 0.808 for 4D-CT, MIBI and US, respectively. Conclusions:4D-CT has similar diagnostic efficacy for preoperative localization to MIBI in patients with PHPT, and it is superior to MIBI and US in identifying the ectopic parathyroid gland. 4D-CT can be recommended as an alternative preoperative localization method, especially when parathyroid lesions could not be precisely located by US and MIBI.

Objective:To investigate the technical points and clinical application of combined infratemporal fossa approaches (CIFA) by analyzing the clinical results with CIFA for lesions involved skull base.Methods:A retrospective study was performed on 11 patients underwent CIFA for skull base lesions dissection from December 2014 to January 2019 in the department of otolaryngology, Peking Union Medical College Hospital. There were 6 male and 5 female patients, with age range of 16-72 years old and median age of 53 years old. Five patients underwent CIFA Type B and D, and the other 6 underwent CIFA A and B. All patients were followed up regularly by CT and MRI to observe possible recurrence.Results:Among the 5 patients with CIFA Type B and D, 3 were giant cell tumor and 2 were giant cell reparative granuloma, and median maximum cross-section size was 42 mm×46 mm (range from 37 mm×18 mm to 56 mm×53 mm). Among the 6 patients with CIFA Type A and B, 4 were paraganglioma of head and neck, 1 was schwannoma of skull base, 1 was petrous cholesteatoma, and median maximum cross-section size was 43 mm×36 mm (range from 24 mm×22 mm to 63 mm×35 mm). Nine patients underwent complete resection of the tumor in the first stage. In 2 patients, the extracranial parts were removed in the first stage, and the intracranial part was removed in the second stage. Tympanum and ossicular reconstruction were done in one of the CIFA Type B and group D, and 1 year′s postoperative hearing was mild conductive hearing loss. There was no cerebrospinal fluid leakage of all patients. All the 5 patients with normal facial nerve function before surgery recovered to H-B grade Ⅰ to Ⅱ within 3 months after surgery. Among the 4 patients whose preoperative facial nerve function were grade Ⅱ, 2 recovered to grade I after surgery and the other 2 were still grade Ⅱ. For the patient whose preoperative facial nerve function was grade Ⅴ, his postoperative recovery was grade Ⅲ. There was 1 patient whose pre-operative FN function was H-B grade Ⅲ, and the post-operative FN function was grade Ⅵ due to FN resection. Except for 2 cases with cochlear involved before surgery, cochleae of the other 9 cases were preserved. The follow-up time was 14 to 58 months. No recurrence was observed in all patients.Conclusions:The CIFA can safely and completely remove the extensive lesions that invade the skull base, and the facial nerve function can be well protected and recovered intro-and post-operation. Appropriate use of combined IFA can not only achieve good exposure and complete resection of lesions, but also create conditions for functional reconstruction.

Objective To evaluate the radiation dose and image quality of the third-generation dual-source CT using automated tube voltage adaptation (CARE kV) in temporal bone scanning in pediatric patients with hearing impairment. Methods Totally 27 children with hearing impairment less than 18 years old were randomly divided into two groups:Group A (n=14),examined with CARE kV (reference with 100 kV and 214 mA)and sinogram-affirmed iterative reconstruction;and Group B (n=13),examined with CARE kV (reference with 100 kV and 171 mA)and advanced modeled iterative reconstruction. The scan range was from eyebrow to the end of the mastoid process. CT values and image noise were measured.The signal to noise ratio (SNR)was calculated. Subjective image quality was assessed by two radiologists and later in a consensus reading. Results There was no significant difference in CT value,image noise,and SNR between these two groups (all P>0.05). Also,the subjective scores of the 10 anatomical structures showed no significant difference (all P>0.05). The CT dose index volume and dose-length product were (11.62+1.92)mGy and (106.92+37.48)mGy·cm,respectively,in group B and (21.28+2.19)mGy (t=12.15,P<0.001)and (229.65+56.26)mGy·cm (t=6.62,P<0.001)in group A,decreased by 45% and 53%. Conclusion Compared with the second-generation dual-source CT,the third-generation dual-source CT for the scanning of temporal bone with CARE kV can ensure image quality and reduce radiation dose.
Child ; Hearing Loss ; diagnosis ; Humans ; Radiation Dosage ; Radiographic Image Interpretation, Computer-Assisted ; Signal-To-Noise Ratio ; Temporal Bone ; diagnostic imaging ; Tomography, X-Ray Computed ; methods

Objective To investigate the image quality of head and neck CT angiography (CTA)using the third-generation dual-source CT combined with 70 kV tube voltage and 20-25 ml contrast medium (CM),and evaluate the effects of venous artifacts arising from the CM on the ipsilateral side of injection. Methods Totally 40 consecutive patients with suspected vascular diseases and body weight lower than 75 kg prospectively underwent head and neck CTA examination using the third-generation dual-source CT. CTA was performed with a third-generation dual-source CT system. Patients were randomly divived into 70 kV group (n=20)and 100 kV group (n=20). The 70 kV group used 20-25 ml CM and advanced modeled iterative reconstruction technique,and the 100 kV group used 40 ml CM and filtered back projection. Venous artifacts and CM residues were evaluated by a 3-point scale (1=excellent,3=poor),respectively. Results The effective dose of 70 kV group decreased 58% compared to 100 kV group (t=-18.14,P<0.001).In the 70 kV group,16 patients (80.0%)presented with venous artifacts and six of them (37.5%,6/16)affected the adjacent arteries. In the 100 kV group,19 patients (95.0%)presented with venous artifacts,and seven of them (36.8%,7/19)affected the adjacent arteries (Z=-0.878,P=0.380). In the 70 kV group,13 patients (65.0%)presented with obvious CM residues and two of them (15.3%,2/13)prolonged into the superior vena cava (SVC). In the 100 kV group,19 patients(95.0%)presented with obvious CM residues,and thirteen of them(68.4%,13/19)prolonged into the SVC (Z=-3.654,P<0.001). Conclusion Compared with the 100 kV,the third-generation dual-source CT for head and neck CTA,combined with 70 kV and 20-25 ml CM,can remarkably decrease the radiation dose,along with reduced CM residues and comparable venous artifacts.
Artifacts ; Body Weight ; Computed Tomography Angiography ; methods ; Contrast Media ; Head ; diagnostic imaging ; Humans ; Neck ; diagnostic imaging ; Radiation Dosage ; Radiographic Image Interpretation, Computer-Assisted

Objective: To explore the safety and validity of endoscopic cricopharyngeal myotomy in patients with cricopharyngeal achalasia. Methods: A total of 19 patients with cricopharyngeal achalasia suffered from sustained dysphagia were enrolled in this study. The patients were divided into transcervical cricopharyngeal myotomy(CPM) group and endoscopic CPM (ECPM) group. Swallowing function and complications were evaluated.SPSS7.0 software was used to analyze the data. Results: The swallowing function improved significantly in seven patients in ECPM group, and 9 patients improved in CPM group.The video fluoroscopic swallowing study(VFSS)-swallowing score, VFSS-aspiration score and drinking test score were (3.1±1.1), (3.4±0.8) and (2.0±0.6)in post-ECPM, (3.4±1.4), (3.0±0.9) and (2.2±0.6)in post-CPM. No statistical difference was found in validity between CPM group and ECPM group(t=-0.435, t=1.086, t=-0.607, P>0.05). No statistical difference was observed on the occurrence of complication between two groups. Only one patient had subcutaneous emphysema after operation in ECPM. Conclusions New surgical instruments and endoscopic surgical technique were safe and effective for cricopharyngeal achalasia. Because these instruments are cheaper, laryngeal endoscopic cricopharyngeal myotomy is easier to be popularized more easily than microscopic laser assistted CPM.

Objective: To study the clinical outcomes of stent-thrombosis (ST) at different times in patients after drug-eluting stent (DES) implantation.
Methods: A total of 131 coronary angiography conifrmed ST patients in our hospital from 2005-01 to 2015-04 were studied. According to the time of ST occurrence, the patients were divided into 2 groups: Early ST group, ST occurred ≤30 days,n=42 and Late ST group, ST occurred >30 days,n=89. The in-hospital and follow-up information was collected; clinical outcomes were compared between 2 groups.
Results: The in-hospital MACE occurrence rate in Early ST group was higher than that in Late ST group (16.7% vs 4.5%),P=0.04. There were 123 patients survived to discharge and they were followed-up for the median of 38.00 (15.00, 62.00) months. Kaplan-Meier analysis estimated that the MACE-free survival was similar between 2 groups (41.9% vs 36.3%), P=0.43.
Conclusion: In-hospital MACE occurrence was higher in early ST patients, while the long term prognosis was similar between the early and late ST patients for whom with DES implantation.

Objective To explore the safety and feasibility of guiding catheter passing through spasmodic vessels in patients undergoing percutaneous coronary intervention (PCI) via radial artery access by the aid of PCI guiding wire and balloon .Methods The clinical data of 33 coronary artery disease (CAD) patients undergoing PCI via radial artery access with radial artery or (and) brachial artery spasm ( group A ) were retrospectively analyzed .Among all these patients , guiding catheters were delivered through the spasmodic vessels successfully by the aid of PCI guiding wires and balloons .The clinical data of other 38 CAD patients having PCI during the same period performed by other operators via radial artery or ( and ) brachial artery approach and experienced vessel spasm were anlysed as the control ( group B ) .All patients in group B received conventional anti-spasm management during PCI .All vessel spasm was identified by angiography.For patients in group A , a diameter of 0.014 inch guiding wire was chosen to pass through the spasmodic vessel segment carefully and gently .The diameter of balloon should be chosen according to the diameter of guiding catheter .A balloon diameter of 2.0 mm and 2.5 mm was corresponded to 6F and 7F guiding catheter respectively .The balloon was advanced to the tip of guiding catheter , keeping a half in catheter and a half in vessel followed by inflating the balloon with a pressure of 8 atm.The balloon was kept inflated the guiding catheter was pushed in vitro carefully and slowly until the catheter passed through the spasmodic vessel segment .Then the balloon was deflated and pulled out together with PCI guiding wire . Exchanged a diameter of 0.035 inch wire and completed the positioning of guiding catheter .After finishing the PCI, radial or ( and) brachial angiography was performed again to observe if spasm disappeared and to determine if there any contrast medium exudation .For patients in group B , routine approach was applied including administration of nitroglycerine , diltiazem or nitroprusside etc . to relieve vessel spasm. Results The location of vessel spasm was similar in group A and group B ( P=0.150 ) , and the incidence rate of spasm in brachial artery was higher than that in radial artery in both groups .The chance of guiding catheter crossing the spasmodic vessel segment was significantly higher in group A than in group B ( 100%vs.39.5%, P=0.00).In patients whose guiding catheter could pass through the spasmodic vessel segment successfully , time spent in group A was shorter than in group B ( P=0.000 ) .The patient number which time spent was less than five minutes , five to 15 minutes and more than 15 minutes was 30 and 2 ( 90.1%vs.13.3%) , 3 and 7 ( 9.9% vs.46.7%) and 0 and 6 ( 0% vs.40.0%) in group A and in group B respectively.The incidence of forearm hematoma was lower in group A than in group B without statistical difference [6.1%(2/33) vs.18.4%(7/38), P =0.113].Conclusions It is safe and feasible for passing guiding catheter through spasmodic vessels during PCI via radial artery access by the aid of PCI guiding wire and balloon .

Objective To explore the effects of Chinese herbal compound Tangbikang on the expressions of p38 MAPK of sciatic nerve and plasma TNF-α in diabetic rats. Methods Ten of the sixty male SD rats were selected randomly as normal group, and the rest were fed with high-fat diet and low-dosage STZ was used to induce type Ⅱdiabetic rat models. Model rats were randomly divided into model group, mecobalamine group and Tangbikang low-, medium-, and high-dosage groups, 10 rats in each group. Each medication group was intervened with relevant medicine. Rat unilaterals sciatic nerves were taken after 16 weeks. The content of TNF-α in plasma was determined by radioimmunoassay. Western blot method was used to detect the expressions of p38 and p-p38 MAPK protein of sciatic nerve. Results Compared with normal group, the expressions of p38 and p-p38 protein and content of TNF-αin model group significantly increase (P<0.05, P<0.01). Compared with the model group, the expressions of p-p38 protein and the content of TNF-α significantly decreased after medicine intervention in different doses Tangbikang groups and mecobalamin group (P<0.05, P<0.01). The expression of p38 protein in Tangbikang high-dose group significantly decreased (P<0.05), with statistical significance (P<0.05). Conclusion Tangbikang can reduce the expression of p38 and p-p38 MAPK protein of the rat sciatic nerve, and reduce the content of TNF-α protein in rat plasma, which may be one of the effective targets of neuroprotection and abirritation of diabetic peripheral neuropathy.