[Application of speech induced ABR in rehabilitation intervention for hearing impaired children].

Objective:Exploring the electrophysiological changes of auditory rehabilitation in young children with hearing impairment, providing more methods for early assessment and intervention. Methods:Twenty children aged 2-4 were enrolled, with moderate hearing loss and no other abnormalities in the ears. Divide them into group 1 with normal hearing, group 2 with abnormal hearing, group 3 with abnormal hearing receiving hearing aid intervention for one year, and group 4 with abnormal hearing receiving hearing aid and language training rehabilitation for one year. The SmartEP auditory evoked potential instrument was used to detect speech induced ABR and conduct screening for 'Standards and Evaluating Hearing and Language Abilities of Children with Hearing Impairment in 80 enrolled children after rehabilitation training, and the latency、amplitude of speech induced ABR waveform and evaluation scale scores for each group after rehabilitation intervention were compared. Results:Compared with the normal group, the latency of each wave in the other three groups was prolonged. The differences in each wave between Group 2 and Group 3 were statistically significant, while the differences in D and F waves between Group 3 and Group 4 were statistically significant. Compared with the normal group, the maximum amplitude at F0 decreased in the other three groups, and the differences in maximum amplitude between Group 2 and Group 3, Group 2 and Group 4, and Group 3 and Group 4 were statistically significant. Compared with the normal group, the scores of the auditory language assessment scale in the hearing intervention group and the hearing aid plus language training group were significantly higher than those in the abnormal group in terms of recognition rate. The recognition rates of hearing impaired children with language training foundation are similar to those of the normal group of children. Conclusion:Auditory rehabilitation can alter the electrophysiological aspects of hearing and serve as a basis for early assessment and intervention in young children.

[λ approach for the repairment of large skin defects after resection of preauricular fistula with cellulitis in children].

Objective:To explore the feasibility and advantages of λ approach for the repairment of large skin defects after resection of preauricular fistula with cellulitis in children. Methods:The clinical data of patients with preauricular fistula with cellulitis treated by λ approach from January 2016 to January 2021 were analyzed retrospectively. Results:After follow-up for 10-18 months, the primary healing rate of incision was 97.8%, and the survival rate of λ flap was 100%. Conclusion:λ approach is a safe, effective and cosmetic method. It can be used to repair the large skin defects after resection of preauricular fistula with cellulitis in children.

[Hyoid body morphology of thyroglossal duct cyst: a comparative study based on imaging].

Objective:To provide reference for preoperative diagnosis and treatment of thyroglossal duct cyst by studying the morphological changes of hyoid body. Methods:The CT data（midsagittal image） of congenital thyroglossal duct cyst（TGDC group） diagnosed by pathology and the control group（C group） were collected from January 2016 to October 2021. The differences of hyoid body height（HBH）, hyoid body width（HBW）, hyoid bone thickness（HBT）, HBW/HBH, HBT/HBH between the two groups were compared. HBH, HBW and HBT were analyzed by t-test; The HBW/HBH and HBT/HBH were analyzed by Mann-Whitney U test. Results:Twenty-nine cases were included in the TGDC group and 58 in the C group. The HBH in the TGDC group was（8.93 ±0.22） mm and that in the C group was（8.94±0.12） mm, there was no significant difference between the two groups（P>0.05）. The HBW in the TGDC group and the C group were（5.09±0.21） mm and（4.48±0.11） mm, and the HBT were（3.84±0.12） mm and（3.13±0.08） mm, respectively, the difference between the two groups was statistically significant（P<0.05）. The average rank sum of HBW/HBH in the TGDC group and the C group was 53.95 and 39.03, respectively, and the average rank sum of HBT/HBH was 59.90 and 36.05, respectively, the difference between the two groups was statistically significant（P<0.05）. Conclusion:The morphological changes of hyoid body of thyroglossal duct cyst may be helpful for preoperative diagnosis, and it also suggests that hyoid body resection may reduce the possibility of postoperative recurrence.

The pro-tumorigenic activity of p38γ overexpression in nasopharyngeal carcinoma.

It is urgent to identify and validate biomarkers for early diagnosis and efficient treatment of nasopharyngeal carcinoma (NPC). Recent studies have proposed p38 gamma (p38γ) as a cyclin-dependent kinase (CDK)-like kinase that phosphorylates retinoblastoma (Rb) to promote cyclins expression and tumorigenesis. Here the Gene Expression Profiling Interactive Analysis (GEPIA) database and results from the local NPC tissues demonstrate that p38γ is significantly upregulated in NPC tissues, correlating with poor overall survival. Furthermore, p38γ mRNA and protein expression is elevated in established NPC cell lines (CNE-1 HONE-1 and CNE-2) and primary human NPC cells, but low expression detected in human nasal epithelial cells. In established and primary NPC cells, p38γ depletion, using the shRNA strategy or the CRISPR/Cas9 gene-editing method, largely inhibited cell growth, proliferation and migration, and induced significant apoptosis activation. Contrarily, ectopic p38γ overexpression exerted opposite activity and promoted NPC cell proliferation and migration. Retinoblastoma (Rb) phosphorylation and cyclin E1/A expression were decreased in NPC cells with p38γ silencing or knockout, but increased after p38γ overexpression. Moreover, mitochondrial subcellular p38γ localization was detected in NPC cells. Significantly, p38γ depletion disrupted mitochondrial functions, causing mitochondrial depolarization, reactive oxygen species production, oxidative injury and ATP depletion in NPC cells. In vivo, intratumoral injection of adeno-associated virus-packed p38γ shRNA potently inhibited primary human NPC xenograft growth in nude mice. In p38γ shRNA virus-injected NPC xenograft tissues, p38γ expression, Rb phosphorylation, cyclin E1/A expression and ATP levels were dramatically decreased. Taken together, we conclude that p38γ overexpression is required for NPC cell growth, acting as a promising therapeutic target of NPC.

[Eigenvalue analysis of wideband acoustic immittance in secretory otitis media in children of different ages].

Objective:The characteristic values of broadband acoustic immittance of children of different ages during secretory otitis media were collected resonance frequency and absorption rate of middle ear in children of different ages were compared. Methods:Eighty-two children with secretory otitis media were selected for the broadband acoustic immittance test, including 62 cases （83 ears） in the young effusion group（[3.5±0.7]months）, and 20 cases （40 ears） in the older effusion group（[4.0±0.6]years old）. 82 normal children were selected for broadband acoustic immittance test in the same environment, including 62 cases （124 ears） in the younger normal group and 20 cases （40 ears） in the older normal group. The resonance frequency and absorptivity of different age groups under broadband peak pressure were compared, and the frequency absorptivity curve was drawn for analysis. Results:The resonance frequencies of older normal group and younger normal group, older effusion group and older normal group, younger effusion group and younger normal group, older effusion group and younger effusion group were statistically significant（P<0.001）. Analysis of sound energy absorption rate at different ages. There was significant difference in sound energy absorption rate between older normal group and younger normal group at 226-1155 / 4757-8000 Hz（P<0.001）. The difference of full frequency sound energy absorption rate between the older effusion group and the older normal group was statistically significant（P<0.001）, among which the difference of 866-1542 / 2520-4362 Hz absorption rate was the largest. The difference of full frequency sound energy absorption rate between young effusion group and young normal group was statistically significant（P<0.001）, and the difference of 1091-2245 / 3776-5494 Hz absorption rate was the largest. Conclusion:Middle ear effusion in children of different ages has different performance under broadband acoustic immittance peak pressure, and has its own characteristic acoustic energy absorption frequency band. This study provides a reference basis for the analysis of the characteristics of middle ear sound transmission in children with secretory otitis media in the future.

[Common clinical causes and audiological manifestations of unilateral hearing loss in children].

Objective:To analyze the common clinical causes and audiological manifestations of unilateral hearing loss in children, and to provide a basis for early prevention and intervention. Method:A retrospective analysis of 20 cases of children diagnosed with unilateral hearing loss was conducted. The medical history was collected, followed by audiological and imaging examinations. Result:The audiological performance of 20 children with unilateral hearing loss: 1 case of conductive deafness, whose hearing curve was flat and showed moderate hearing loss. There were 19 cases of sensorineural deafness, of which 1 case was moderate deafness, 16 cases were severe deafness, and 2 cases were extremely severe deafness. The imaging results showed that there were 1 case with transverse fracture of the temporal bone, 1 case with congenital cholesteatoma, 9 cases with dysplasia of the cochlear nerve, and 4 cases with malformation of the inner ear. The history of the two children was collected and there were clear congenital cytomegalovirus infection and mumps virus infection. The causes of the remaining 3 cases were unknown. Conclusion:Unilateral hearing loss in children is a common clinical diseasewith complex and diverse causes. Carefully analysis should be made to find out the causes and symptomatic treatment to provide a basis for early prevention and intervention of children's hearing.

ZBTB24 regulates the apoptosis of human T cells via CDCA7/TRAIL-receptor axis.

Mutations in ZBTB24 and CDCA7 cause the Immunodeficiency, Centromeric Instability and Facial Anomalies syndrome type 2 and 3 (ICF2/3), respectively. Most ICF2 patients carry ZBTB24 nonsense mutations and are thus ZBTB24-deficient. Although the immune deficiency in ICF2 patients is primarily regarded as a B-cell defect due to the greatly reduced serum antibodies and circulating memory B cells, the reduced expansions of PBMCs stimulated by mitogens or recall antigens suggest a T-cell defect in these patients as well. However, the molecular mechanisms behind this T-cell dysfunction remain unknown. In the present study, we demonstrated that ZBTB24-deficiency significantly represses the proliferation of human T cells by promoting TRAIL-induced cell death. Downregulation of ZBTB24 in both Jurkat and human primary T cells upregulates the expression of TRAIL and/or its death receptors (TRAIL-R1/2), and induces significant amount of cells to undergo apoptosis. The profound survival defects of ZBTB24-deficient cells are largely reversed by blocking TRAIL/TRAIL-R interactions with exogenous recombinant TRAIL-R2. Moreover, ZBTB24-downregulation reduces the expression of CDCA7, and knockdown of the latter in human T cells results in a phenotype resembling that caused by ZBTB24-depletion. Functionally, overexpression of CDCA7 abrogates the increased apoptosis in ZBTB24-depleted Jurkat T cells. Together, these data indicated that ZBTB24 regulates human T-cell apoptosis via CDCA7/TRAIL-R axis. Our study thus not only provides a molecular explanation for the T-cell defects in ZBTB24-deficient ICF2 patients, but also highlights a convergence between ZBTB24 and CDCA7, the two ICF genes, in modulating the functions of T cells.

The role of granulocyte macrophage colony stimulating factor in hospitalized children with Mycoplasma pneumoniae pneumonia.

BACKGROUND: Inappropriate inflammatory response in children with M. pneumoniae infection might be associated with disease severity. The role of Granulocyte macrophage colony stimulating factor (GM-CSF) in hospitalized children with Mycoplasma pneumoniae pneumonia (MPP) has not been fully discussed. METHODS: Clinical and laboratory data of a total 40 children with MPP were collected. GM-CSF and myeloperoxidase (MPO) were detected by ELISAs. Meanwhile, normal human bronchial epithelium was infected by M. pneumoniae and neutrophils were stimulated by GM-CSF to explore GM-CSF and MPO release in supernatant, respectively. RESULTS: Compared to control group, a significant increased percentage of neutrophils and decreased percentage of macrophages in bronchoalveolar lavage fluid of children with MPP was observed (P < 0.05). Children with MPP had significantly higher levels of GM-CSF (P = 0.0047) and MPO (P = 0.0002) in BALF compared to the controls. Level of GM-CSF in BALF was associated with duration of fever (r = 0.42, P = 0.007) and strongly correlated with level of MPO (r = 0.075, P = 0.0005). Levels of GM-CSF and MPO significantly decreased (both P < 0.05) after treatment. In vitro, M. pneumoniae induced GM-CSF expression in a time-dependent manner during a 72-h period (P < 0.05) and MPO secretion significantly increased by recombinant human GM-CSF stimulation at 24h (P < 0.05). CONCLUSION: GM-CSF could be induced by M. pneumoniae infection in vivo and vitro. Childen with high level GM-CSF had longer duration of fever. GM-CSF probably plays a vital role in neutrophil inflammation in M. pneumoniae infection.

Role of the Mycoplasma pneumoniae/Interleukin-8/Neutrophil Axis in the Pathogenesis of Pneumonia.

Neutrophil infiltration is the characteristic pathological feature of M. pneumoniae pneumonia (MPP). This study aimed to explore the associations among neutrophil activity, clinical presentation, and role of the M. pneumoniae/interleukin-8 (IL-8)/neutrophil axis in the pathogenesis of MPP. A total of 42 patients with MPP were prospectively enrolled in the study. Neutrophil activity, including matrix metalloproteinase-9 (MMP-9), myeloperoxidase (MPO), and neutrophil elastase (NE), were measured. Clinical information was collected for all patients and control group. In vitro, IL-8 production was measured at different time points after M. pneumoniae infection of bronchial epithelial cells, and neutrophil activity was analyzed after IL-8 stimulation. The percentage of neutrophil in the bronchoalveolar lavage fluid was higher in the group of patients with high levels of M. pneumoniae DNA than in those with low levels of M. pneumoniae DNA (P < 0.05). IL-8, MMP-9, and NE in patients with MPP significantly increased compared with controls and decreased after treatment (P < 0.05). MPO and MMP-9 were associated with duration of fever (r = 0.332, P < 0.05) and length of stay (r = 0.342, P < 0.05), respectively. In vitro, M. pneumoniae induced IL-8 production by bronchial epithelial cells in a time dependent manner. MPO, MMP-9 and NE production by neutrophils significantly increased compared with medium controls after IL-8 stimulation. In summary, the M. pneumoniae/IL-8/neutrophil axis likely plays a vital role in the pathogenesis of MPP.

[Three cases of eosinophilichyperplastic lymphogranuloma in children's parotid area].

From March 2009 to October 2009, three pediatric patients with parotid tumor were cured. Preoperative physical examination showed regional swelling in parotid area, the surface skin was in moderate reddish purple, the border was vague, and the swelling was inactive. The patients' IgE were significantly increased. B ultrasound examination demonstrated the focus was an isoecho with ringlike dark band around, which was concluded as bull's-eye sign. Magnetic resonance imaging (MRI) examination indicated a cystic mass between the skin and parotid. Preoperative diagnosis was eosinophilichyperplastic lymphogranuloma (Kimura's disease) and the granuloma was excised by operation. Pathological examination revealed the capillary vessel hyperplasia in local tissue with a plenty of eosinophils and lymphocytes infiltrating. The disease was confirmed. Although the disease is rare, the diagnosis still could be made by preoperative physical examination, laboratory and imaging examinations.