Micronized acellular dermal matrix combined with platelet rich plasma in the treatment of atrophic acne scars: A self-controlled split face study.

BACKGROUND: Micronized acellular dermal matrix (mADM) can induce tissue regeneration and repair, and filling. OBJECTIVES: The efficacy and safety of (mADM) were evaluated in the treatment of atrophic acne scar. METHODS: In this single-blinded, self-controlled, split-face study, 16 patients (48 scar sites) were divided into treatment group (24 scar sites) and control group (24 scar sites). One side of the affected area was treated with mADM combined with platelet rich plasma (PRP) injection as the treatment group; the other side of the affected area was treated with PRP injection as the control group. The efficacy was evaluated by the Acne scar assessment scale (ASAS) and Acne Scar Weight Rating Scale (ECCA) 3 months after treatment. RESULTS: After 3-month treatment in 16 patients, the atrophic acne scars in both groups were all improved. The ASAS score and ECCA weight score in the treatment group was significantly lower than that in the control group (2.50 ± 0.51 vs. 3.62 ± 0.77 and 14.17 ± 10.18 vs. 31.88 ± 13.25; p < 0.001). LIMITATIONS: Short-term 3-month treatment period. Small sample size limits generalizability of results. CONCLUSION: The curative effect of mADM combined with PRP is significantly better than that of PRP alone.

Mutant huntingtin protein induces MLH1 degradation, DNA hyperexcision, and cGAS-STING-dependent apoptosis.

Huntington's disease (HD) is an inherited neurodegenerative disorder caused by an expanded CAG repeat in the huntingtin (HTT) gene. The repeat-expanded HTT encodes a mutated HTT (mHTT), which is known to induce DNA double-strand breaks (DSBs), activation of the cGAS-STING pathway, and apoptosis in HD. However, the mechanism by which mHTT triggers these events is unknown. Here, we show that HTT interacts with both exonuclease 1 (Exo1) and MutLα (MLH1-PMS2), a negative regulator of Exo1. While the HTT-Exo1 interaction suppresses the Exo1-catalyzed DNA end resection during DSB repair, the HTT-MutLα interaction functions to stabilize MLH1. However, mHTT displays a significantly reduced interaction with Exo1 or MutLα, thereby losing the ability to regulate Exo1. Thus, cells expressing mHTT exhibit rapid MLH1 degradation and hyperactive DNA excision, which causes severe DNA damage and cytosolic DNA accumulation. This activates the cGAS-STING pathway to mediate apoptosis. Therefore, we have identified unique functions for both HTT and mHTT in modulating DNA repair and the cGAS-STING pathway-mediated apoptosis by interacting with MLH1. Our work elucidates the mechanism by which mHTT causes HD.

Microbial production of branched chain amino acids: Advances and perspectives.

Branched-chain amino acids (BCAAs) such as L-valine, L-leucine, and L-isoleucine are widely used in food and feed. To comply with sustainable development goals, commercial production of BCAAs has been completely replaced with microbial fermentation. However, the efficient production of BCAAs by microorganisms remains a serious challenge due to their staggered metabolic networks and cell growth. To overcome these difficulties, systemic metabolic engineering has emerged as an effective and feasible strategy for the biosynthesis of BCAA. This review firstly summarizes the research advances in the microbial synthesis of BCAAs and representative engineering strategies. Second, systematic methods, such as high-throughput screening, adaptive laboratory evolution, and omics analysis, can be used to analyses the synthesis of BCAAs at the whole-cell level and further improve the titer of target chemicals. Finally, new tools and engineering strategies that may increase the production output and development direction of the microbial production of BCAAs are discussed.

New nurses' turnover intention and clinical belonging, based on latent class analysis (LCA).

AIM: To identify potential categories of clinical belonging among new nurses and explore the relationship between different categories and turnover intention. DESIGN: A cross sectional study. METHOD: A cross sectional study was conducted among 348 new nurses from tertiary hospitals in Hainan and Guangdong provinces. A general data questionnaire, clinical belonging scale and turnover intention scale were used for examination. Further, the potential categories were used to analyse the categories of clinical belonging, and latent class analysis was utilized to analyse the relationship between different categories of clinical belonging and turnover intention. RESULTS: The clinical sense of belonging of new nurses were divided into three groups namely C1, C2 and C3. The C1: poor clinical sense of belonging (8.7%), C2: moderate clinical sense of belonging (57.9%) and C3: rich clinical sense of belonging (33.4%). The risk of the turnover intention of new nurses with 'poor clinical sense of belonging' was 0.62 times that of new nurses with 'rich clinical sense of belonging' (OR = 0.62, p < 0.01), which was 0.24 times that of 'moderate clinical sense of belonging' (OR = 0.24, p < 0.01), the risk of the turnover intention of new nurses with 'moderate clinical sense of belonging' was 0.13 times that of new nurses with 'rich clinical sense of belonging'(OR = 0. 13, p < 0.01). CONCLUSIONS: The results of our study revealed that in order to enhance the new nurses' sense of belonging, support was most crucial when they were first encountering difficulties. To reduce turnover intention, more structured learning opportunities are also required to maximize learning for newly graduated nurses with various nursing degrees. PATIENT OR PUBLIC CONTRIBUTION: There are no patient or public contributions in this study.

Differentiation and risk stratification of basal cell carcinoma with deep learning on histopathologic images and measuring nuclei and tumor microenvironment features.

BACKGROUND: Nuclear pleomorphism and tumor microenvironment (TME) play a critical role in cancer development and progression. Identifying most predictive nuclei and TME features of basal cell carcinoma (BCC) may provide insights into which characteristics pathologists can use to distinguish and stratify this entity. OBJECTIVES: To develop an automated workflow based on nuclei and TME features from basaloid cell tumor regions to differentiate BCC from trichoepithelioma (TE) and stratify BCC into high-risk (HR) and low-risk (LR) subtypes, and to identify the nuclear and TME characteristics profile of different basaloid cell tumors. METHODS: The deep learning systems were trained on 161 H&E -stained sections which contained 51 sections of HR-BCC, 50 sections of LR-BCC and 60 sections of TE from one institution (D1), and externally and independently validated on D2 (46 sections) and D3 (76 sections), from 2015 to 2022. 60%, 20% and 20% of D1 data were randomly splitted for training, validation and testing, respectively. The framework comprised four stages: tumor regions identification by multi-head self-attention (MSA) U-Net, nuclei segmentation by HoVer-Net, quantitative feature by handcrafted extraction, and differentiation and risk stratification classifier construction. Pixel accuracy, precision, recall, dice score, intersection over union (IoU) and area under the curve (AUC) were used to evaluate the performance of tumor segmentation model and classifiers. RESULTS: MSA-U-Net model detected tumor regions with 0.910 precision, 0.869 recall, 0.889 dice score and 0.800 IoU. The differentiation classifier achieved 0.977 ± 0.0159, 0.955 ± 0.0181, 0.885 ± 0.0237 AUC in D1, D2 and D3, respectively. The most discriminative features between BCC and TE contained Homogeneity, Elongation, T-T_meanEdgeLength, T-T_Nsubgraph, S-T_HarmonicCentrality, S-S_Degrees. The risk stratification model can well predict HR-BCC and LR-BCC with 0.920 ± 0.0579, 0.839 ± 0.0176, 0.825 ± 0.0153 AUC in D1, D2 and D3, respectively. The most discriminative features between HR-BCC and LR-BCC comprised IntensityMin, Solidity, T-T_minEdgeLength, T-T_Coreness, T-T_Degrees, T-T_Betweenness, S-T_Degrees. CONCLUSIONS: This framework hold potential for future use as a second opinion helping inform diagnosis of BCC, and identify nuclei and TME features related with malignancy and tumor risk stratification.

Heat shock protein DNAJA2 regulates transcription-coupled repair by triggering CSB degradation via chaperone-mediated autophagy.

Transcription-coupled nucleotide excision repair (TC-NER) is an important genome maintenance system that preferentially removes DNA lesions on the transcribed strand of actively transcribed genes, including non-coding genes. TC-NER involves lesion recognition by the initiation complex consisting of RNA polymerase II (Pol II) and Cockayne syndrome group B (CSB), followed by NER-catalyzed lesion removal. However, the efficient lesion removal requires the initiation complex to yield the right of way to the excision machinery, and how this occurs in a timely manner is unknown. Here we show that heat shock protein DNAJA2 facilitates the HSC70 chaperone-mediated autophagy (CMA) to degrade CSB during TC-NER. DNAJA2 interacts with and enables HSC70 to recognize sumoylated CSB. This triggers the removal of both CSB and Pol II from the lesion site in a manner dependent on lysosome receptor LAMP2A. Defects in DNAJA2, HSC70 or LAMP2A abolish CSB degradation and block TC-NER. Our findings discover DNAJA2-mediated CMA as a critical regulator of TC-NER, implicating the DNAJA2-HSC70-CMA axis factors in genome maintenance.

Janus kinase inhibitor, tofacitinib, in refractory juvenile dermatomyositis: a retrospective multi-central study in China.

OBJECTIVES: Juvenile dermatomyositis (JDM) is a chronic autoimmune disease. Some patients remain in an active state even though they were administrated with a combination of corticosteroid and methotrexate. Existing research has suggested that interferon and Janus kinase played an important role in pathogenesis. Existing research has suggested the efficacy of JAK inhibitors (JAKi). Our retrospective study aimed to investigate the efficacy of tofacitinib in refractory JDM patients. METHODS: A total of eighty-eight patients in China who had been diagnosed with JDM and subjected to tofacitinib therapy for over 3 months were retrospectively analyzed. Skin and muscle manifestations were assessed using the Cutaneous Assessment Tool-binary method (CAT-BM), Childhood Myositis Assessment Scale (CMAS), and kinase. Pulmonary function was assessed using a high-resolution CT (computerized tomography) scan and pulmonary symptoms. All patients were subjected to regular follow-up, and core measures were assessed every 3 months after initiation. Furthermore, the data were analyzed using the Wilcoxon single test, Mann-Whitney U test, and chi-square test. RESULTS: Compared with the baseline data, skin and muscle manifestations were found significantly improved during the respective follow-up visit. At the most recent follow-up, nearly 50% of patients achieved a clinical complete response and six patients received tofacitinib monotherapy. Sixty percent of patients suffering from interstitial lung disease well recovered on high-resolution CT. Seventy-five percent of patients showed a reduction in the size or number of calcinosis, and 25% of patients showed completely resolved calcinosis. CONCLUSION: In this study, the result suggested that tofacitinib therapy exerted a certain effect on skin manifestations, muscle manifestations, interstitial lung disease (ILD), calcinosis, as well as downgrade of medication. In-depth research should be conducted to focus on the correlation between the pathogenesis of JDM and JAKi.

The crosstalk between DNA repair and immune responses.

In recent years, increasing evidence has highlighted the profound connection between DNA damage repair and the activation of immune responses. We spoke with researchers about their mechanistic interplays and the implications for cancer and other diseases.

Construction of a plasmid-free L-leucine overproducing Escherichia coli strain through reprogramming of the metabolic flux.

BACKGROUND: L-Leucine is a high-value amino acid with promising applications in the medicine and feed industries. However, the complex metabolic network and intracellular redox imbalance in fermentative microbes limit their efficient biosynthesis of L-leucine. RESULTS: In this study, we applied rational metabolic engineering and a dynamic regulation strategy to construct a plasmid-free, non-auxotrophic Escherichia coli strain that overproduces L-leucine. First, the L-leucine biosynthesis pathway was strengthened through multi-step rational metabolic engineering. Then, a cooperative cofactor utilization strategy was designed to ensure redox balance for L-leucine production. Finally, to further improve the L-leucine yield, a toggle switch for dynamically controlling sucAB expression was applied to accurately regulate the tricarboxylic acid cycle and the carbon flux toward L-leucine biosynthesis. Strain LEU27 produced up to 55 g/L of L-leucine, with a yield of 0.23 g/g glucose. CONCLUSIONS: The combination of strategies can be applied to the development of microbial platforms that produce L-leucine and its derivatives.

Is smart carbon emission reduction justified in China? Evidence from national big data comprehensive pilot zones.

The intelligent revolution caused by new digital technologies has provided new impetus to reduce carbon emissions. However, the current research on new digital technologies and carbon emissions is still in its infancy and lacks empirical conclusions between them. Therefore, this paper studies the impact of new digital technologies on carbon emissions, identifies its mechanism, and analyzes the regional heterogeneity of its effects. This research treats the National Big Data Comprehensive Pilot Zones pilot in China as a quasi-natural experiment for the development of new digital technologies along with city-level data covering from 2011 to 2019 to conduct a staggered difference-in-difference (DID) model analysis. We find that new digital technologies significantly reduce carbon emissions. This conclusion is still valid after a series of robustness tests such as heterogeneity treatment effect analysis, ex-ante trend test, spillover effect test, and placebo test. Additionally, new digital technologies can reduce carbon emissions by promoting the transformation of industrial structure, improving the level of green technology innovation, and promoting industrial agglomeration. At the same time, the heterogeneity analysis shows that new digital technologies' carbon emission reduction effect is more evident in non-western regions, southern regions, and large cities. To expand the carbon emission reduction effect of new digital technologies, the government should promote the development and application of new digital technologies, and implement differentiated policies based on regional characteristics.

DNAJA2 deficiency activates cGAS-STING pathway via the induction of aberrant mitosis and chromosome instability.

Molecular chaperone HSP70s are attractive targets for cancer therapy, but their substrate broadness and functional non-specificity have limited their role in therapeutical success. Functioning as HSP70's cochaperones, HSP40s determine the client specificity of HSP70s, and could be better targets for cancer therapy. Here we show that tumors defective in HSP40 member DNAJA2 are benefitted from immune-checkpoint blockade (ICB) therapy. Mechanistically, DNAJA2 maintains centrosome homeostasis by timely degrading key centriolar satellite proteins PCM1 and CEP290 via HSC70 chaperone-mediated autophagy (CMA). Tumor cells depleted of DNAJA2 or CMA factor LAMP2A exhibit elevated levels of centriolar satellite proteins, which causes aberrant mitosis characterized by abnormal spindles, chromosome missegregation and micronuclei formation. This activates the cGAS-STING pathway to enhance ICB therapy response in tumors derived from DNAJA2-deficient cells. Our study reveals a role for DNAJA2 to regulate mitotic division and chromosome stability and suggests DNAJA2 as a potential target to enhance cancer immunotherapy, thereby providing strategies to advance HSPs-based cancer therapy.

Severe gastrointestinal involvement in pediatric IgA vasculitis: a retrospective single-center cohort study in China.

Objectives: The study aimed to describe the characteristics of gastrointestinal (GI) involvement in a cohort of hospitalized children with IgA vasculitis (IgAV) in China. Method: We reviewed the records of hospitalized IgAV patients from January 2014 to December 2020 at one tertiary medical center. The patients were divided into the severe GI group and the non-severe GI group according to the presence of massive GI bleeding and complications. The clinical manifestations, laboratory factors, and treatment were analyzed between the two groups. Results: A total of 1,179 patients were hospitalized due to IgAV. GI involvement was noted in 50% (589) of the patients, of whom 288 (48.9%) had severe GI involvement. GI complications were observed in 34 patients with IgAV with GI involvement. Rare onset age (<3 years or within 13-17 years), purpura above the waist, vomiting, high neutrophil-to-lymphocyte ratio, and decreased serum albumin were factors associated with severe GI involvement. Frequencies of renal involvement and biopsy-proven nephritis were higher in the severe GI group. The most commonly used medications were corticosteroids (100.0%) in the severe GI group. The maximum corticosteroid dose was higher (2.9 vs. 2.0 mg/kg), and more second-line therapies were needed (30.9% vs. 16.94%) in the severe GI group. Conclusions: Severe GI involvement in children is common in our center. Rare onset age, purpura above the waist, vomiting, high neutrophil-to-lymphocyte ratio, and decreased serum albumin are associated with severe GI involvement. Patients with severe GI involvement need higher doses of corticosteroids and second-line therapy.

Molten-Salt Electrochemical Deoxidation Synthesis of Platinum-Neodymium Nanoalloy Catalysts for Oxygen Reduction Reaction.

Platinum-rare earth metal (Pt-RE) nanoalloys are regarded as a potential high performance oxygen reduction reaction (ORR) catalyst. However, wet chemical synthesis of the nanoalloys is a crucial challenge because of the extremely high oxygen affinity of RE elements and the significantly different standard reduction potentials between Pt and RE. Here, this paper presents a molten-salt electrochemical synthetic strategy for the compositional-controlled preparation of platinum-neodymium (Pt-Nd) nanoalloy catalysts. Carbon-supported platinum-neodymium (Ptx Nd/C) nanoalloys, with distinct compositions of Pt5 Nd and Pt2 Nd, are obtained through molten-salt electrochemical deoxidation of platinum and neodymium oxide (Pt-Nd2 O3 ) precursors supported on carbon. The Ptx Nd/C nanoalloys, especially the Pt5 Nd/C exhibit a mass activity of 0.40 A mg-1 Pt and a specific activity of 1.41 mA cm-2 Pt at 0.9 V versus RHE, which are 3.1 and 7.1 times higher, respectively, than that of commercial Pt/C catalyst. More significantly, the Pt5 Nd/C catalyst is remarkably stable after undergoing 20 000 accelerated durability cycles. Furthermore, the density-functional-theory (DFT) calculations confirm that the ORR catalytic performance of Ptx Nd/C nanoalloys is enhanced by compressive strain effect of Pt overlayer, causing a suitable weakened binding energies of O* Δ E O ∗ $\Delta {E}_{{{\rm{O}}}^*}$ and Δ E OH ∗ $\Delta {E}_{{\rm{OH}}^*}$ .

DNA mismatch repair in cancer immunotherapy.

Tumors defective in DNA mismatch repair (dMMR) exhibit microsatellite instability (MSI). Currently, patients with dMMR tumors are benefitted from anti-PD-1/PDL1-based immune checkpoint inhibitor (ICI) therapy. Over the past several years, great progress has been made in understanding the mechanisms by which dMMR tumors respond to ICI, including the identification of mutator phenotype-generated neoantigens, cytosolic DNA-mediated activation of the cGAS-STING pathway, type-I interferon signaling and high tumor-infiltration of lymphocytes in dMMR tumors. Although ICI therapy shows great clinical benefits, ∼50% of dMMR tumors are eventually not responsive. Here we review the discovery, development and molecular basis of dMMR-mediated immunotherapy, as well as tumor resistant problems and potential therapeutic interventions to overcome the resistance.

A Chinese girl of Blau syndrome with renal arteritis and a literature review.

BACKGROUND: Blau syndrome is a rare autoinflammatory disease caused by autosomal dominant mutations in the CARD15/NOD2 gene. Vascular involvement is a rare phenotype in Blau syndrome patients. In this study, we aimed to describe a 20-year- old Chinese girl with Blau syndrome complicated by renal arteritis. In addition, we summarized a literature review of published cases of vascular involvement in patients with Blau syndrome. CASE PRESENTATION: We describe a 20-year-old girl who was initially misdiagnosed with juvenile idiopathic arthritis (JIA) almost 15 years prior. In October 2019, she developed renal arteritis at the age of 17 years and was eventually diagnosed with Blau syndrome. A de-novo M513T mutation was found in her gene testing. A review of the literature on patients with Blau syndrome and vasculitis showed that a total of 18 cases were reported in the past 40 years. The vast majority of them were predominantly involved medium and large vessel arteritis. Of the 18 patients included in our literature review, 14 patients had aorto-arteritis, and 4 of them had renal artery involvement. Two patients presented with renal artery stenosis, 1with a sinus of Valsalva aneurysm, and 1 with retinal vasculitis. CONCLUSION: A detailed medical history inquiry and a careful physical examination are helpful for the early identification of Blau syndrome, especially for infant onset refractory JIA. Medium-and large-vessel arteritis is a rare clinical manifestation in Blau syndrome patients. Careful examination of the peripheral pulse and measurement of blood pressure at every regular visit may be helpful in the early identification of Blau syndrome-arteritis. Early diagnosis and appropriate treatment may prevent or delay the occurrence of severe symptoms in patients to improve the patient's quality of life.

A Cohort Study on Deficiency of ADA2 from China.

PURPOSE: Deficiency of adenosine deaminase 2 (DADA2), an autosomal recessive autoinflammatory disorder caused by biallelic loss-of-function variants in adenosine deaminase 2 (ADA2), has not been systemically investigated in Chinese population yet. We aim to further characterize DADA2 cases in China. METHODS: A retrospective analysis of patients with DADA2 identified through whole exome sequencing (WES) at seventeen rheumatology centers across China was conducted. Clinical characteristics, laboratory findings, genotype, and treatment response were analyzed. RESULTS: Thirty patients with DADA2 were enrolled between January 2015 and December 2021. Adenosine deaminase 2 enzymatic activity was low in all tested cases to confirm pathogenicity. Median age of disease presentation was 4.3 years and the median age at diagnosis was 7.8 years. All but one patient presented during childhood and two subjects died from complications of their disease. The patients most commonly presented with systemic inflammation (92.9%), vasculitis (86.7%), and hypogammaglobinemia (73.3%) while one patient presented with bone marrow failure (BMF) with variable cytopenia. Twenty-three (76.7%) patients were treated with TNF inhibitors (TNFi), while two (6.7%) underwent hematopoietic stem cell transplantation (HSCT). They all achieved clinical remission. A total of thirty-nine ADA2 causative variants were identified, six of which were novel. CONCLUSION: To establish early diagnosis and improve clinical outcomes, genetic screening and/or testing of ADA2 enzymatic activity should be performed in patients with suspected clinical features. TNFi is considered as first line treatment for those with vascular phenotypes. HSCT may be beneficial for those with hematological disease or in those who are refractory to TNFi.

Predicting a Favorable (mRS 0-2) or Unfavorable (mRS 3-6) Stroke Outcome by Arterial Spin Labeling and Amide Proton Transfer Imaging in Post-Thrombolysis Stroke Patients.

(1) Background: The objective of this study was to determine whether arterial spin labeling (ASL), amide proton transfer (APT), or their combination could distinguish between patients with a low and high modified Rankin Scale (mRS) and forecast the effectiveness of the therapy; (2) Methods: Fifty-eight patients with subacute phase ischemic stroke were included in this study. Based on cerebral blood flow (CBF) and asymmetry magnetic transfer ratio (MTRasym) images, histogram analysis was performed on the ischemic area to acquire imaging biomarkers, and the contralateral area was used as a control. Imaging biomarkers were compared between the low (mRS 0-2) and high (mRS 3-6) mRS score groups using the Mann-Whitney U test. Receiver operating characteristic (ROC) curve analysis was used to evaluate the performance of the potential biomarkers in differentiating between the two groups; (3) Results: The rAPT 50th had an area under the ROC curve (AUC) of 0.728, with a sensitivity of 91.67% and a specificity of 61.76% for differentiating between patients with low and high mRS scores. Moreover, the AUC, sensitivity, and specificity of the rASL max were 0.926, 100%, and 82.4%, respectively. Combining the parameters with logistic regression could further improve the performance in predicting prognosis, leading to an AUC of 0.968, a sensitivity of 100%, and a specificity of 91.2%; (4) Conclusions: The combination of APT and ASL may be a potential imaging biomarker to reflect the effectiveness of thrombolytic therapy for stroke patients, assisting in guiding treatment approaches and identifying high-risk patients such as those with severe disability, paralysis, and cognitive impairment.

An integrated method with adaptive decomposition and machine learning for renewable energy power generation forecasting.

In recent years, traditional energy sources have caused a variety of negative impacts on the environment, and reducing carbon emissions is a top priority. The development of renewable energy technology is the key to transform the energy structure. Renewable energy represented by wind energy and photovoltaics has abundant reserves so they are connected to the grid system on a large scale. However, because of natural energy's randomness, renewable energy power generation poses potential risks to energy production and grid security. By making short-term forecasts of renewable energy generation power, the uncertainty of energy generation can be reduced, and it is crucial to study renewable energy forecasting techniques. This paper proposes an integrated forecasting system for renewable energy sources. Firstly, ensemble empirical mode decomposition is used for data preprocessing, and stationarity analysis is used for modal identification; then, support vector regression optimized by sparrow search algorithm and statistical methods are combined to make forecast according to different characteristics of the series respectively; finally, the feasibility of this method in renewable energy time series prediction is verified by experiments. The experiments prove that the proposed model effectively improves the accuracy and prediction performance on ultra-short-term renewable energy forecasting; and it has good applicability and competitiveness with different forecasting scenarios and characteristics, which satisfy the actual forecasting requirements in terms of operational efficiency and accuracy, thus providing a technical basis for the effective utilization of renewable energy.

Childhood-onset primary Sjögren's syndrome in a tertiary center in China: clinical features and outcome.

OBJECTIVES: To characterize the clinical features and outcomes of childhood-onset primary Sjögren's syndrome (pSS). METHODS: Patients less than 18 years old who were diagnosed with pSS by paediatric rheumatologists were included, and all patients were applied the 2002 American-European Consensus Group (ACEG) criteria, the 2016 American College of Rheumatology/European League Against Rheumatism (ACR/EULAR) criteria for pSS, or the 1999 proposed juvenile pSS criteria. The electronic medical records of patients with pSS from 2013 to 2020 were collected and analysed. RESULTS: Thirty-nine patients were included. Of them, 27 (69.2%), 38 (97.4%) and 35 (89.7%) patients fulfilled the AECG criteria, ACR/EULAR criteria and proposed juvenile pSS criteria, respectively. The female:male ratio was 3.9:1. The median ages at first signs or symptoms and at diagnosis were 9.2 (4.7, 14.5) years and 10.9 (6.3, 15.0) years, respectively. The main clinical manifestations were rash or purpura (20, 51.3%), followed by fever (12, 30.8%), glandular enlargement/recurrent parotitis (10, 25.6%), and dry mouth and/or dry eyes (9, 23.1%). Twenty-eight (56.4%) patients had systemic damage, the most common of which was haematological involvement (14, 35.9%), followed by hepatic (13, 33.3%) and renal involvement (8, 20.5%). Thirty-eight (97.4%) patients underwent labial minor salivary gland biopsy, and all exhibited focal lymphocytic sialadenitis. All patients had a global ESSDAI score ≥ 1 at diagnosis, and the median total score at diagnosis was 8 (2, 31). Thirty-six (92.3%) patients were followed up for a median time of 23.6 (7.9, 79.5) months, and three patients developed systemic lupus erythematosus (SLE) at follow-up times of 13.3, 38.8 and 63.8 months. CONCLUSIONS: The presentation of childhood-onset pSS is atypical, and extraglandular manifestations and systemic involvement are more common than in adult-onset pSS. Labial salivary gland biopsy is vital for patients with probable pSS. Some patients may develop SLE over time.