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.
Male ; Child ; Female ; Humans ; Nephritis, Hereditary/pathology* ; Retrospective Studies ; Kidney ; Deafness ; Hearing Loss/genetics* ; Kidney Failure, Chronic/pathology* ; Mutation

Purpose To study the clinical and pathological features of angiomatoid fibrous histocytoma(AFH)and to ex-plore its diagnosis,differential diagnosis and prognosis.Meth-ods The clinicopathological and follow-up data were analyzed in 14 cases of AFH,and the literatures were reviewed.Results There were 11 males and 3 females.The age ranged from 11 months to 12 years and 11 months,with average 5.9 years.3 cases were located in limbs,and 5 cases in trunk,5 cases in head and neck region,and 1 of intracranial tumor.Histological-ly,14 cases were composed of fibrous capsules and lymphocyte sheaths,and cell nucleus were vacuolar,forming fascicles with focal whirling and synteny.Intralesional pseudoangiomatous spaces were noted in 9 cases.Calcification was found in 2 ca-ses.2 cases showed high mitotic acticity(11/10 HPF).Scle-rosing and/or myxoid stroma was seen in 3 cases.Tumors were immunopositive for desmin(10/14),EMA(12/14),CD99(12/14),SMA(9/12),ALK(7/8),and the average of Ki67 index was 16％.7 cases harbored EWSR1 rearrangenent(part-ner gene not identified),2 cases had EWSR1-ATF1 fusion and 2 EWSR1-CREB1 fusion.Clinical follow-up information was a-vailable for 14 cases(average 46 months).All the 14 cases were alive without recurrence and metastasis.Conclusion AFH is a borderline or low-grade malignant tumor,often demon-strates indolent behavior in children,but rarely recurs and me-tastasizes.The diagnosis and differential diagnosis require a comprehensive analysis of clinical features,histopathologic changes,immunohistochemical finding and EWSR1 or FUS gene detection results.

L-theanine has been shown to have a therapeutic effect on depression.However,whether L-theanine has an excellent preventive effect on depression in children and adolescents and what its mechanism is have not been well explained.Given the complexity of the pathogenesis of depression,this study investigated the preventive effect and mechanism of L-theanine on depression in juvenile rats by combining serum and hippocampal metabolomic strategies.Behavioral tests,hippocampal tissue sections,and serum and hippocampal biochemical indexes were studied,and the results confirmed the preventive effect of L-theanine.Untargeted reversed-phase liquid chromatography-quadrupole-time-of-flight mass spec-trometry and targeted hydrophilic interaction liquid chromatography-triple quadrupole mass spec-trometry were developed to analyze the metabolism changes in the serum and hippocampus to screen for potential biomarkers related to L-theanine treatment.The results suggested that 28 abnormal me-tabolites in the serum and hippocampus that were considered as potential biomarkers returned to near-normal levels after L-theanine administration.These biomarkers were involved in various metabolic pathways,mainly including amino acid metabolism and lipid metabolism.The levels of amino acids and neurotransmitters in the phenylalanine,tryptophan,and glutamic acid pathways were significantly reduced after L-theanine administration compared with chronic unpredictable mild stress-induced rats.In summary,L-theanine had a significant preventive effect on depression and achieved its preventive results on depression by regulating various aspects of the body,such as amino acids,lipids,and inflammation.This research systematically analyzed the mechanism of L-theanine in preventing depression and laid the foundation for applying L-theanine to prevent depression in children and adolescents.

Background/Aims: Anti-phospholipase A2 receptor (PLA2R) autoantibody is the main biomarker of idiopathic membranous nephropathy (IMN). We aimed to find a new cutoff value of anti-PLA2R for patients with IMN and to explore the relevance between this antibody and baseline clinical parameters. Methods: A total of 670 subjects including 374 IMN cases and 296 non-IMN controls were included between January 2017 and January 2020. All clinical parameters were collected at the time of renal biopsy. The levels of anti-PLA2R were detected by a commercial enzyme-linked immunosorbent assay (ELISA) kit. The optimal cutoff value was calculated by a receiver operating characteristic curve and compared in diagnostic efficiency. Results: The optimal cutoff value of anti-PLA2R for IMN was 7.45 RU/mL with the highest Youden index, and the corresponding sensitivity, specificity, positive predictive value and negative predictive value were 80.75%, 97.97%, 98.05% and 80.11%, respectively. Anti-PLA2R levels in IMN patients demonstrated a significant positive correlation with serum creatinine and 24-hour urinary protein, while they showed a negative correlation with serum albumin and estimated glomerular filtration rate. Conclusions The recommended cutoff value of anti-PLA2R is 7.45 RU/mL using ELISA detection for distinguishing IMN from non-IMN nephropathy. The level of anti-PLA2R is related to baseline renal function in IMN. This new threshold can improve the diagnostic efficiency and facilitate early diagnosis of IMN.

Prostate cancer in patients with dwarfism is rarely reported. One case was reported in this article. The patient was admitted to the hospital due to the PSA elevation for more than 4 years. Due to the dwarf disease, the patient could not accommodate the transrectal ultrasound probe, and was highly suspected of prostate cancer.The prostate needle biopsy was not performed. Combined with the medical history, PSA level, preoperative MRI and PSMA-PET/CT examination, the patient was clinically diagnosed with localized prostate cancer, and radical surgical treatment was performed.

HYD-PEP06, an endostatin-modified polypeptide, has been shown to produce effective anti-colorectal carcinoma effects through inhibiting epithelial-mesenchymal transition (EMT). However, whether HYD-PEP06 has similar suppressive effect on hepatocellular carcinoma (HCC) remained unknown. In this study, HYD-PEP06 inhibited metastasis and EMT but not proliferation

Objective:To identify the influence of age factors on biological characteristics of human adipose-derived stem cells (ADSCs), and to compare the differences in morphology, aging characteristics, differentiation potential, surface marker expression, and proliferation ability of human ADSCs with different ages and the effect of human ADSCs of different ages on proliferation and migration of fibroblasts.Methods:Adipose tissues from 27 individuals of healthy beauty treatments, aged 0-59 years, were collected from Department of Burns and Plastic Surgery, the 940th Hospital of Joint Logistics Support Force from January 2020 to December 2020. According to the age of the donor, they were divided into 6 groups. 0-9 years old group (4 cases), 10-19 years old group (4 cases), 20-29 years old group (5 cases), 30- 39 years old group (5 cases), 40-49 years old group (5 cases), and 50-59 years old group (4 cases). We obtained and cultured the ADSCs of each group, subcultured to obtain the ADSCs, and observed the morphological characteristics of each group; the proliferation ability of each group of ADSCs was detected by CCK-8 method; the differentiation potential of each group of ADSCs was detected by in vitro adipogenesis and osteogenic induction. Flow cytometry was used to detect the surface marker expression levels of each group of ADSCs, and qPCR was used to detect the expression levels of HGF, PPAR-γ, type Ⅲ collagen and DbX2 in each group. We also collected the skin tissues of a healthy person from Department of Burns and Plastic Surgery of the 940th Hospital of Joint Logistics Support Force in 2020, obtained and cultured fibroblasts, subcultured to obtain the second generation of fibroblasts, and prepared each group of ADSCs conditioned medium (ADSCs-CM); the proliferation ability of fibroblasts cultured in each group of ADSCs-CM was detected by CCK-8 method, and the migration ability of fibroblasts cultured in each group of ADSCs-CM was detected by cell scratch method. SPSS 21.0 software was used for data analysis and P<0.05 was considered statistically significant. Results:The cells of all age groups were in a typical spindle shape. The cells of the younger age group showed small nuclei and the cell morphology were uniform and similar; the cells of the older age group had larger nuclei and uneven morphology. With aging, the proliferation ability of ADSCs gradually decreased. In vitro adipogenic differentiation induction results showed that with the increase in age, the ability of ADSCs to differentiate into adipocytes gradually decreased. In vitro osteogenic differentiation induction results also demonstrated that with the increase in age, the ability of ADSCs to differentiate into osteoblasts gradually decreased. The expression rates of positive surface markers of ADSCs in each group of ADSCs were all above 93%. With aging, the expression levels of PPAR-γ, HGF, and type Ⅲ collagen decreased, and the expression levels of DbX2 showed an upward trend with age. The number of β-galactosidase staining positive cells increased with age, while the difference between groups A, B and C was not statistically significant, and the difference between groups A and D, E, and F was statistically significant ( P<0.05). The ADSCs conditioned medium of each group was able to promote the proliferation of fibroblasts, but the difference between the groups was not statistically significant. With aging, the ability of ADSCs conditioned medium to promote the migration of fibroblasts gradually decreased. Conclusions:The proliferation ability and multidirectional differentiation ability of human ADSCs and the ability to secrete a variety of cytokines that promote the proliferation and migration of fibroblasts show a downward trend, but the adipogenic differentiation ability of ADSCs declines significantly after the age of 40 years. ADSCs in all age groups could promote the proliferation and migration of fibroblasts.

Objective:To prepare human decellular adipose tissue matrix (DAT) as injectable homogenate by a specially developed high-speed soft tissue homogenizer with controllable temperature, and to investigate its cellular compatibility and filling effect.Methods:From March 2019 to March 2021, DAT was obtained in the 940th Hospital from normal adipose tissue after decellular treatment. The DAT was mixed with normal saline at the rate of 1: 12. The specially developed high-speed soft tissue homogenizer with controllable temperature was used to conduct homogenization at 803×g for 10 min. The produced DAT homogenizer could pass through the 27G needle smoothly. DAT homogenate was observed under scanning electron microscope and its cytocompatibility was detected. Finally, granular fat, DAT homogenate and DAT homogenate + ADSCs were injected into the back of rats respectively, and the filling effect, angiogenesis ability and inflammatory infiltration were compared.Results:After decellular treatment, adipose tissue changed into DAT without cellular residue. The particle size of DAT homogenate was about (749.91±136.79) nm, which was prepared by the specially developed temperature controlled high-speed soft tissue homogenater. The adhesion rate was (92.16±1.00) %, and the A value increased with time. The cell growth was good, and the homogenate had no toxicity to the cells. In vivo experiment, the filling effect of DAT homogenate and DAT homogenate + ADSCs was significantly better than that of granulated fat group ( P<0.01). In the granulated fat group, a large number of adipocytes were necrotic and fused to form oil sacs, while DAT homogenate, DAT homogenate + ADSCs showed no obvious degradation, and some adipocytes were generated. The results of CD31 staining indicated that the number of microvessels in DAT homogenate group and DAT homogenate + ADSCs group was higher than that in granulated fat group ( P<0.01). The results of CD68 staining indicated that the inflammatory infiltration in DAT homogenate group and DAT homogenate + ADSCs group was lower than that in granule fat group ( P<0.01). Conclusions:Using the self-developed temperature controlled high-speed soft tissue homogenate device, DAT could be prepared into DAT homogenate that can pass through 27G needle. It has good biocompatibility and filling advantages, and injective process is simple, and the trauma is small, and so it could be used as filling material.

There has been a large number of related literature reports on sleep disorders, but in pediatrics, especially for children aged 0-5 years old, sleep disorders have not received enough attention.In order to raise pediatricians′ awareness of sleep disorders in children aged 0-5 years old, the relevant studies during the past 10 years have been reviewed, and the clinical manifestations and treatments were summarized.The clinical manifestations of sleep disorders in infants aged 0-5 years old are not typical and the incidence is high.Sleep disorders have profound effects on the cognitive and behavioral development of children aged 0-5 years old.

Oral infectious diseases include caries, periodontal disease, halitosis, candidiasis albicans and so on. Over the past few decades, probiotics have mainly been studied in the field of the gastrointestinal tract. In recent years, probiotics have begun to be used in the prevention and treatment of various oral diseases and have become a new field in the research of oral disease prevention and control technology. This paper reviews the research progress of probiotics applied in the prevention and treatment of various oral infectious diseases. A review of the literature shows that probiotics can prevent and cure dental caries by inhibiting the growth of Streptococcus mutans and competing with them for nutrition and attachment sites. Probiotics not only inhibit periodontal pathogens and reduce the production of sulfide, they also regulate the body’s immune function to alleviate halitosis and periodontal inflammation. Probiotics can inhibit periodontal caries by inhibiting them. Probiotics can inhibit them mycelial growth of Candida albicans and interfere with its adherence, thus playing a role in the prevention and treatment of oral candidiasis. Current studies have shown that probiotics play an auxiliary role in the treatment of caries, periodontitis, halitosis and oral candidiasis. However, the mechanism of probiotics in the prevention and treatment of oral infectious diseases is still unclear, and the safety of probiotics remains to be further studied. In the future, oral probiotics should be studied with reference to intestinal probiotics to better work to prevent and treat oral diseases.