Long-term auditory monitoring in children with Alport syndrome based on different degrees of renal injury / 临床耳鼻咽喉头颈外科杂志

Objective:To investigate long-term auditory changes and characteristics of Alport syndrome（AS） 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 33（25 males, 8 females, aged 3.4-27.8 years） 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 disease（ESRD）, predominantly males （6/7）. The rate of long-term hearing loss was significantly different between ESRD group and non-ESRD group（P=0.013）. There was no correlation between the progression of renal disease and long-term hearing level（P>0.05）. 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 loss（P=0.048）, and there was no correlation with the severity of hearing loss（P>0.05）. Among 11 cases, theCOL4A5mutationwas most common （8 out of 11）, but there was no significant correlation between the mutation type and hearing phenotype（P>0.05）. 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.

Dual-label Time-resolved Fluoroimmunoassay for Pepsinogen Ⅰ and Pepsinogen Ⅱ and Its Preliminary Application / 生物化学与生物物理进展

A dual-label time-resolved fluoroimmunoassay was established for simultaneously detecting pepsinogen Ⅰ (PGⅠ) and pepsinogen Ⅱ (PG Ⅱ) in human serum. Two capture monoclonal antibodies, 8003# of PGⅠ and 8101# of PGⅡ, were co-coated in 96 microtitration wells. The counterpart tracer monoclonal antibodies, 8016# of PGⅠ and 8102# of PGⅡ, were labeled with Eu3+ and sm3+-chelates, respectively. The samples were assayed by one-step sandwich protocol with the time-resolved fluorometry. The measurement ranges of PGⅠ were 0.2～300.0 ?g/L with the within-run and between-run precision was 5.2% and 8.1%, and that of PGⅡ were 0.05～55.0 ?g/L with the within-run and between-run precision was 7.1% and 11.7%, respectively. The average recovery rates of PGⅠ and PGⅡ were 96.9% and 103.7%, respectively. The results obtained by the dual-label assay agreed well with those by enzyme-linked immunosorbent assays of PGⅠ and PGⅡ, whose correlation ratio were 0.9426 of PGⅠ and 0.9396 of PGⅡ, respectively. The means of 300 healthy volunteers were (157.3 ? 51.0) ?g/L for serum PGⅠ,(10.6 ? 5.9) ?g/L for serum PGⅡ, and (14.8 ? 4.3) for the PGⅠ/PGⅡ ratio. The normal ranges of serum PGⅠ levels for healthy volunteers were 55.3～259.3 ?g/L, those of serum PGⅡ levels were less than 23 ?g/L, the PGⅠ/PGⅡ ratio was more than 6. The proposed dual-label TRFIA for simultaneous detection of PGⅠ and PGⅡ is a simple, sensitive, and rapid method. It could provide serology high-screening of the samples for gastric diseases and would allow investigations into the possible diagnostic value of analysis in various clinical condition.