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Purpose: Congenital malformation of the ossicular chain results in challenges with hearing and language development in children. We aimed to analyze the clinical characteristics, prognosis, and surgical treatments of different types of congenital ossicular chain malformations in children. Methods: Eight cases (10 ears) treated between October 2019 and February 2022 were analyzed retrospectively. Patients were divided according to the location of the ossicular chain malformation and whether it was complicated by external ear malformation. Imaging, audiological examination, intraoperative exploration of the middle ear, and postoperative outcomes were recorded. Results: Group 1 incudostapedial joint deformity): 6 ears/60%; Group 2 (simple incus deformity): 2 ears/20%; Group 3 (simple malleus deformity): 2 ears/20%; Group A (with external ear malformations): 4 ears/40%; Group B (without external ear malformations): 6 ears/60%. The average hearing threshold before and after the operation was 51.25 ± 12.88 and 31.94 ± 12.96 dB, respectively. There were differences in the intervention effects of different malformed sites (Group 1: t = 5.139, P = .004; Group 2: t = 13.500, P = .047; Group 3: t = 15.000, P = .042). The effect of the intervention in cases of malformation with mobile stapes footplates was better than that with immobile stapes footplates (t = 4.082, P = .027). The effect of the intervention without external ear malformations was better than that with external ear malformations (t = 7.706, P = .001). Conclusions: The intervention yielded superior results in cases of malformation with mobile stapes footplates compared to those with immobile stapes footplates. Different intervention strategies should be determined through precise deformity assessments, with the degree of stapes mobility serving as a crucial factor in improving the prognosis.
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Objective:To investigate long-term auditory changes and characteristics of Alport syndromeï¼ASï¼ patients with different degrees of renal injury. Methods:Retrospectively analyzing clinical data of patients diagnosed AS from January 2007 to September 2022, including renal pathology, genetic detection and hearing examination. A long-term follow-up focusing on hearing and renal function was conducted. Results:This study included 70 AS patients, of which 33ï¼25 males, 8 females, aged 3.4-27.8 yearsï¼ were followed up, resulting in a loss rate of 52.9%.The follow-up period ranged from 1.1to 15.8 years, with 16 patients followed-up for over 10 years. During the follow-up, 10 patients presenting with hearing abnormalities at the time of diagnosis of AS had progressive hearing loss, and 3 patients with new hearing abnormalities were followed up, which appeared at 5-6 years of disease course. All of which were sensorineural deafness. While only 3 patients with hearing abnormalities among 13 patients received hearing aid intervention. Of these patients,7 developed end-stage renal diseaseï¼ESRDï¼, predominantly males ï¼6/7ï¼. The rate of long-term hearing loss was significantly different between ESRD group and non-ESRD groupï¼P=0.013ï¼. There was no correlation between the progression of renal disease and long-term hearing levelï¼P>0.05ï¼. kidney biopsies from 28 patients revealed varying degrees of podocyte lesion and uneven thickness of basement membrane. The severity of podocyte lesion was correlated with the rate of long-term hearing lossï¼P=0.048ï¼, and there was no correlation with the severity of hearing lossï¼P>0.05ï¼. Among 11 cases, theCOL4A5mutationwas most common ï¼8 out of 11ï¼, but there was no significant correlation between the mutation type and hearing phenotypeï¼P>0.05ï¼. Conclusion:AS patients exhibit progressive hearing loss with significant heterogeneity over the long-term.. THearing loss is more likely to occur 5-6 years into the disease course. Hearing abnormalities are closely related to renal disease status, kidney tissue pathology, and gene mutations, emphasizing the need for vigilant long-term hearing follow-up and early intervention.
Asunto(s)
Sordera , Pérdida Auditiva , Fallo Renal Crónico , Nefritis Hereditaria , Masculino , Niño , Femenino , Humanos , Nefritis Hereditaria/genética , Nefritis Hereditaria/patología , Estudios Retrospectivos , Riñón , Pérdida Auditiva/genética , Fallo Renal Crónico/genética , Fallo Renal Crónico/patología , MutaciónRESUMEN
OBJECTIVE: To investigate the influence of CD4+ CD25+ regulatory T cells(Treg cells) on mouse gastric cancer. METHODS: Treg cell in mouse spleen bearing gastric tumor was tested in different time points. Magic cell sorting(MACS) method was used to purify mouse Treg cells and the Treg cells were injected into mouse bearing gastric tumor with different dosage. After 3 weeks, the tumor size and tumor cell apoptosis rate were measured. RESULTS: Treg existed in normal mouse spleen with a rate of (3.86+/-0.07)%. In tumor model this percentage increased gradually and was (4.12+/-0.13)% after 3 weeks, which was significantly higher than that in control. When Treg cell applied in mouse reached 2.0 x 10(5), the tumor size enlarged significantly(P=0.013) and tumor cell apoptosis rate decreased significantly (P=0.012). CONCLUSIONS: Treg cell is associated with gastric cancer progress in mouse tumor model. Treg cell can promote gastric cancer growth and decrease tumor apoptosis. The anti- Treg GITR can improve anti- tumor effects.