An 11-year-old boy presented with erythematous plaques over the bilateral mandibular and mental regions for 2 years, accompanied by cough and dyspnea for more than 2 months. Chest computed tomography angiography revealed marked stenosis of the right pulmonary artery, irregular aortic caliber, and aortic wall thickening. Histopathological examination of the skin lesion, including immunohistochemistry and special stains, confirmed a chronic suppurative inflammation. Whole-exome sequencing was negative. A final diagnosis of granuloma faciale and Takayasu arteritis was established. Combination therapy with systemic tocilizumab, prednisone, and methotrexate, along with topical 0.1% tacrolimus ointment, resulted in a favorable clinical response. This report summarizes the clinical features of a pediatric case of granuloma faciale and Takayasu arteritis and reviews the etiology, diagnostic approach, and current treatment strategies for the disorders, aiming to enhance clinicians' understanding of these conditions.
Humans ; Male ; Child ; Takayasu Arteritis/diagnosis* ; Facial Dermatoses/diagnosis*

Standardized reporting is a crucial factor affecting the use of patient guidelines （PGs）， particularly in the reporting and presentation of recommendations. This paper introduced the current status of PG reporting， including the research on PG content and presentation formats， and provided comprehensive recommendations for PG reporting from aspects such as overall framework， recommendations， presentation format， and readability. First， the presentation of PG recommendations should include clearly defined clinical questions， recommendations and their rationale， and guidance on how patients should implement the interventions； for specific content in the PG， such as level of evidence， level of recommendation， it is recommended to explain in text the reasons for giving different levels of recommendation， i.e.， to present the logic behind giving the level of recommendation to the patient； additional information needed in the recommendation framework should be supplemented by tracing references or authoritative textbooks and literature that support the recommendations. Subsequently， the PG text should be written based on the Reporting Checklist for Public Versions of Guidelines （RIGHT-PVG） reporting framework. Finally， to enhance readability and comprehension， it is recommended to refer to the Patient Education Materials Assessment Tool （PEMAT） for translating PG content. To enhance the readability of PGs， it is suggested to present the PG content in a persona-lized and layered manner.

At present， the process and methodology of patient guidelines （PGs） development varies greatly and lacks systematic and standardised guidance. In addition to the interviews with PG developers， we have sorted out the relevant methodology for the adaptation and development of existing clinical practice guideline recommendations and facilitated expert deliberations to achieve a consensus， so as to finally put forward a proposal for guidance on the process and methodology for the development of PGs. The development of PGs can be divided into the preparation stage， the construction stage， and the completion stage in general， but the specific steps vary according to the different modes of development of PGs. The development process of Model 1 is basically the same as the patient version of the guideline development process provided by the International Guidelines Network， i.e.， team formation， screening of recommendations， guideline drafing， user testing and feedback， approval and dissemination. The developer should also first determine the need for and scope of translating the clinical practice guideline into a patient version during the preparation phase. Model 2 adds user experience and feedback to the conventional clinical practice guideline development process （forming a team， determining the scope of the PG， searching， evaluating and integrating evidence， forming recommendations， writing the guideline， and expert review）. Based on the different models， we sort out the process and methods of PG development and introduce the specific methods of PG development， including how to identify the clinical problem and how to form recommendations based on the existing clinical practice guidelines， with a view to providing reference for guideline developers and related researchers.

Humans ; Philadelphia Chromosome ; Neoplasms ; Myelodysplastic Syndromes/genetics* ; Disease Progression

Obstructive sleep apnea hypopnea syndrome (OSAHS) is a common sleep respiratory disorder characterized by upper respiratory collapse during sleep, with a high prevalence and potentially fatal complications. Currently, maxillary transverse deficiency are considered to be an important pathogenic factor of OSAHS. For patients with poor compliance with positive airway pressure therapy, rapid maxillary expansion can increase the volume and ventilation of the upper respiratory tract, which is an alternative treatment. This paper reviewed the current research on surgically assisted rapid palatal expansion, miniscrew assisted rapid palatal expansion, and distraction osteogenesis maxillary expansion in the treatment of adult OSAHS. By comparing the indications, contraindications, complications, efficacy and long-term stability of the three treatment methods, it provided reference for treatment of patients with OSAHS.
Adult ; Humans ; Nose ; Palatal Expansion Technique ; Palate ; Sleep Apnea, Obstructive/surgery* ; Syndrome

Galactinol synthase (GolS) is a key enzyme in the biosynthetic pathway of raffinose family oligosaccharides (RFOs) and plays an important role in plant responses to abiotic stresses. However, the molecular characteristics of the GolS family members in soybean was not well-known. In this study, six members of GmGolS gene family were genome-widely identified, and their physicochemical properties, chromosomal localization, evolutionary relationship, gene structure, conserved motifs, secondary structure, tertiary structure, tissue-specific expression patterns and the expression levels under salt and drought stresses were analyzed. The results showed that six soybean GolS genes were unevenly distributed on four chromosomes, the range of the isoelectric points of six GmGolS proteins was 5.45-6.08, the molecular weight range was 37 567.07-38 817.59 Da, and the number of amino acids was 324-339 aa. The results of subcellular localization showed that 4 proteins were located in the chloroplast, and 2 proteins in the cytoplasm. Phylogenetic tree analysis showed that the members of the soybean GolS gene family were closely adjacent to each other, and were evolutionarily conservative. Six gene members contain 3 or 4 exons. Prediction of secondary and tertiary structures showed that the spatial structure of proteins of all family members was mainly composed of α-helix and random coil structure, with less β-turn and extended chain structure. Tissue-specific expression analysis showed that six GmGolS members expressed to variable degrees in seeds, roots, root hairs, flowers, stems, pods, nodules and leaves. Expression analysis based on qRT-PCR showed that all GmGolS genes showed different degrees of up-regulated expression under salt and drought treatment, indicating that these genes may be related to the response of plants to salt-tolerance and drought-resistance. These results may facilitate subsequent functional analysis of soybean GolS genes.
Droughts ; Soybeans/genetics* ; Phylogeny ; Plant Proteins/metabolism* ; Stress, Physiological/genetics* ; Plants/metabolism* ; Gene Expression Regulation, Plant

Abstract: The demand for reliable toxicological data of chemicals runs through every link of occupational health work. The prevention of occupational diseases involves high requirements for the standardization of chemical toxicity assessment in occupational health institutions. Good laboratory practice (GLP) emphasizes the integrity of the test process to trace and supervise the whole process of the test, which is conducive to the standardization of chemical toxicity identification. Therefore, the standardized construction of GLP laboratories is an important starting point for occupational health institutions to carry out chemical toxicity identification. In the construction and management process of GLP laboratories for chemical toxicity identification, occupational health institutions need to build a sound organization and operation system, carry out systematic training and assessment of personnel, establish standard operating norms and emphasize their importance, strengthen the management of facility environment and laboratory, pay attention to quality control and process supervision, and constantly improve their own ability level. To actively adapt to social development and market demand, to provide strong support for occupational health work.

Objective: To investigate the feasibility and safety of auricle reconstruction combined with Bonebridge implantation for bilateral aural atresia patients. Methods: A retrospective analysis was conducted for 36 cases(72 ears) who underwent Bonebridge implantation combined with bilateral auricle reconstruction from February 1, 2017 to January 15, 2020. All cases were bilateral congenital aural atresia and underwent Nagata auricle reconstruction for both sides simultaneously. Bonebridge implantations were performed during the second stage of auricle reconstruction. Results: All 36 patients healed well and had no surgical complications when discharged. The preoperative average bone conduction threshold of the patients was(8.5±5.8) dB HL and postoperative bone conduction threshold was (8.4±5.2) dB HL. There was no significant change after the implantation (P=0.724). The preoperative average air conduction threshold of was(64.9±7.4)dB HL and postoperative air conduction threshold was (24.0±5.3) dB HL, which had a significant change after the implantation (P<0.001). The hearing threshold with Bonebridge significantly decreased by 40.9 dB HL compared with the preoperative air conduction threshold(P<0.001). The speech recognition rate of monosyllable words, disyllabic words and short sentences in quiet environment increased by 62.5%, 63.5% and 72.2% respectively. The differences were statistically significant (P<0.001). The speech recognition rate of monosyllabic words, disyllabic words and short sentences in noise environment were significantly increased by 55.9%, 58.9% and 69.9% respectively (P<0.001). After a follow-up of 18.3 months in average, the hearing results were stable and the aesthetic outcomes were satisfied. One patient had implant rupture and healed after revision surgery. Conclusions: With an integrated surgical procedure, patients with bilateral congenital aural atresia can complete bilateral auricle reconstruction and hearing implantation within six months. This integrated surgical procedure is safe and efficient, with a stable hearing improvement and good appearance.
Bone Conduction ; Ear, External ; Hearing Aids ; Hearing Loss, Conductive ; Humans ; Retrospective Studies ; Treatment Outcome

Objective: To study the effect of the inhibitor of Notch signaling pathway-γ-secretase inhibitor DAPT on the ultrastructures of middle ear in the ovalbumin (OVA)-mediated allergic OME in vivo. Methods: Male Sprague-Dawley (SD) rats, weighing 250-300 g, were completely and randomly divided into three groups (5 rats, 10 ears in each group):(1)Control group(2)OME group(3)OME+DAPT group. Rats in the OME group underwent systemic and local sensitization by intraperitoneal and intratympanic injection of ovalbumin to make the model of OVA-induced OME. Rats in the control group were sensitized with PBS. On the basis of establishing the OME model, OME+DAPT group were intraperitoneal injected with DAPT (10 mg/kg) for seven consecutive days and were administered before intratympanic injection of ovalbumin. After the model was successfully established, endoscopy,H&E staining and scanning electron microscopy were used to study the histology and mucous-ciliary ultrastructures of the non-ciliated and ciliated mucosa in the middle ear of each group. One-way ANOVA and Tukey methods were used for statistical analysis. Results: H&E staining showed that the three groups had statistically significant differences in submucosal thickness both in non-ciliated and ciliated regions (non-ciliated area:(6.83±1.47)μm, (38.58±9.57)μm, (32.17±11.89)μm, respectively. F=107.9;cilia area:(26.69±3.22)μm, (30.41±6.75)μm, (26.76±4.06)μm, respectively. F=5.62,both P<0.01). The thickness of the submucosa in the non-ciliated area and the cilia area of the OME group were significantly thicker than that of control group (F=42.08 and 4.40,both P<0.05); the thickness of the non-ciliated area and the ciliated area in OME+DAPT group were reduced compared to OME group(F=1.55 and 2.77,both P<0.05). Scanning electron microscopy showed that the array of cilia on the middle ear mucosa was disorderly arranged and inversed, this phenomenon was relieved in the OME+DAPT group. The number of goblet cells in the control group, OME group, and OME+DAPT group were 9.87±1.92; 15.67±5.77; 10.33±1.99 respectively and the difference between them was statistically significant (F=11.43, P<0.01). The number of goblet cells in the OME group were significantly higher than those in the control group (F=9.00,P<0.01) and the number of goblet cells in the OME+DAPT group were decreased compared to those of OME group (F=8.41, P<0.01). Conclusions: The study demonstrates the pathological changes of the ultrastructure in middle ear in OVA-induced OME and the effect of the γ-secretase inhibitor on it. In OME group, the cilia are disorderly arranged and inversed, the number of goblet cell is increased and they are swelled which suggest the hypersecretion of the mucus. DAPT can regulate OVA-induced allergic inflammation and relieve pathological changes of ultrastructure in middle ear mucociliary transport system through alleviating submucosal inflammation, reducing the hypersecretion of goblet cell and the morphological damage of cilia through the Notch signaling pathway.
Amyloid Precursor Protein Secretases ; Animals ; Ear, Middle ; Male ; Otitis Media with Effusion/drug therapy* ; Ovalbumin ; Rats ; Rats, Sprague-Dawley

Objective: To evaluate the auditory efficacy and subjective satisfaction of adhesive bone conduction hearing aid in children with unilateral congenital aural atresia (UCAA). Methods: Ten subjects (5 males and 5 females) diagnosed with UCAA with an average age of 8.3 years old (ranged from 5 to 15) were included in Beijing Tongren Hospital, Capital Medical University from January to August 2019. The free sound field hearing threshold, word recognition score in quiet, speech reception threshold in noise and sound localization ability (results were measured by RMS error) tests were performed in unaided and aided situation, respectively. Subjective satisfaction questionnaires were also distributed to subjects. Paired t test and Wilcoxon signed rank test were used as statistical analysis methods. Results: The average hearing threshold in aided condition was improved by (21.9±4.4) dB (t=15.8，P<0.05). Speech recognition abilities were generally improved both under quiet and noise (P<0.05);however, when the binaural summation, squelch and head shadow effects were analyzed respectively, the binaural squelch effect was not statistically improved (P>0.05), while the other effects were improved in aided condition (P<0.05). In sound localization test, there was no significant difference of the RMS error value between the unaided and aided situation (P>0.05). The subjects got high satisfaction rates in three subjective questionnaires. Conclusion: The adhesive bone conduction hearing aid can provide significant audiological benefit for children with UCAA as well as raising the quality of their life.
Adhesives ; Adolescent ; Bone Conduction ; Child ; Child, Preschool ; Female ; Hearing Aids ; Hearing Loss, Conductive ; Humans ; Male ; Speech Perception ; Treatment Outcome