[Pulmonary artery stenosis after single lung transplantation: a case report and literature review].

Objective: To summarize and analyze the clinical features, treatment, and prognosis of pulmonary artery stenosis post-lung transplantation. Methods: A 62-year-old male patient was admitted to the hospital with a cough and chest tightness of over a year's duration, which had worsened in the last two months, leading to the diagnosis of idiopathic pulmonary fibrosis. The clinical data were observed and reviewed post-left allograft single lung transplantation. Literature searches were conducted using the keywords "lung transplantation" "stenosis, pulmonary artery" and "postoperative complications" in CNKI, Wanfang Medical Network, and PubMed databases up to December 2022. Results: On January 26, 2022, a left allograft single lung transplantation was performed under general anesthesia. Postoperatively, extracorporeal membrane oxygenation and mechanical ventilation were successfully weaned off at 22 hours and 2 days, respectively, with transfer from the intensive care unit 12 days after surgery. PaO2 and PaCO2 were 50 mmHg and 40 mmHg after deoxygenation. Both pulmonary CT angiography and ventilatory-perfusion imaging indicated stenosis of the left pulmonary anastomosis. Balloon dilation and pulmonary artery stenting were performed, with PaO2 and PaCO2 improving to 87 mmHg and 42 mmHg, respectively. The patient was discharged 102 days post-surgery, and was followed up for 1 year, with a good prognosis. Additionally, 36 related articles were retrieved, encompassing 69 cases with a median age of 53 years (38.5-59.0 years). Of these, 27.54% (19/69) were diagnosed with idiopathic pulmonary fibrosis, 46.38% (32/69) underwent single lung transplantation, with the primary clinical symptom being hypoxemia in 71.01% (49/69) cases. Left pulmonary artery anastomotic stenosis was observed in 43.48% (30/69), with 65.22% (45/69) being diagnosed in the late postoperative period. Interventional therapy was performed to 44.93% (31/69), with a mortality rate of 21.74% (15/69). Conclusions: The primary clinical manifestation of post-lung transplantation pulmonary artery stenosis is hypoxemia and can be diagnosed by pulmonary artery CT angiography, transesophageal echocardiography, and pulmonary angiography. Early diagnosis can significantly reduce mortality, and interventional therapy is an effective treatment for severe pulmonary artery stenosis post-lung transplantation.

Magnetic resonance Index of Activity (MaRIA) is reliable in assessing response to treatment in patients with Crohn's disease (CD).

AIM: To assess the accuracy of Magnetic Resonance Index of Activity (MaRIA) in evaluating therapeutic efficacy in Crohn's disease (CD) patients with different activity levels using ileocolonoscopy as the reference standard. MATERIALS AND METHODS: Forty-eight patients underwent magnetic resonance enterography (MRE) and ileocolonoscopy at baseline, week 26, and week 52, along with the Simple Endoscopic Score for Crohn's Disease (SES-CD) and MaRIA scores. According to the SES-CD score at baseline, all patients were subdivided into mild, moderate, and severe activity subgroups. The identification of endoscopic mucosal healing (MH) was explored primarily. Moreover, the Crohn's Disease Activity Index (CDAI), C-reactive protein (CRP), erythrocyte sedimentation rate (ESR), procalcitonin (PCT), and interleukin-6 (IL-6) levels were collected and analysed. RESULTS: MaRIA correlated significantly with SES-CD and CRP at baseline, week 26, and week 52. The discrepancies in MaRIA and SES-CD were statistically significant before and after treatment. MaRIA = 24.43 and ΔMaRIA = 12.77 as the cut-off points were found to have high diagnostic accuracy for predicting MH. MaRIA (p<0.001), SES-CD (p<0.001), CRP (p<0.05), ESR (p<0.05), and CDAI score (p<0.05) in patients with MH were considerably decreased compared to those in patients without MH. CONCLUSIONS: MRE has good application value in evaluating the therapeutic response of CD patients treated with biological agents. MaRIA is a reliable indicator in the follow-up of CD patients, which is strongly correlated with SES-CD, and it has high accuracy in predicting endoscopic MH.

Expression of water-soluble nucleocapsid protein of SARS-CoV-2 and analysis of its immunogenicity.

Coronavirus disease 2019 (COVID-19) caused by severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) continues to be a major public health concern. Nucleocapsid (N) protein is the most abundant structural protein on SARS-CoV-2 virions and induces the production of antibodies at the early stage of infection. Large-scale preparation of N protein is essential for the development of immunoassays to detect antibodies to SARS-CoV-2 and the control of virus transmission. In this study, expression of water-soluble N protein was achieved through inducing protein expression at 25°C with 0.5 mM IPTG for 12 h. Western blot and ELISA showed that recombinant N protein could be recognized by sera collected from subjects immunized with Sinovac inactivated SARS-CoV-2 vaccine. Four monoclonal antibodies namely 2B1B1, 4D3A3, 5G1F8, and 7C6F5 were produced using hybridoma technology. Titers of all four monoclonal antibodies in ELISA reached more than 1.28×10 6.0. Moreover, all monoclonal antibodies could react specifically with N protein expressed by transfection of pcDNA3.1-N into BHK-21 cells in IPMA and IFA. These results indicated that water-soluble N protein retained high immunogenicity and possessed the same epitopes as that of native N protein on virions. In addition, the preparation of water-soluble N protein and its monoclonal antibodies laid the basis for the development of immunoassays for COVID-19 detection.

Study on self-management of real-time and individualized support in stroke patients based on resilience: a protocol for a randomized controlled trial.

BACKGROUND: The transitional period from hospital to home is vital for stroke patients, but it poses serious challenges. Good self-management ability can optimize disease outcomes. However, stroke patients in China have a low level of self-management ability during the transitional period, and a lack of effective support may be the reason. With the rapid development of technology, using wearable monitors to achieve real-time and individualized support may be the key to solving this problem. This study uses a randomized controlled trial design to assess the efficacy of using wearable technology to realize real-time and individualized self-management support in stroke patients' self-management behavior during the transitional period following discharge from hospital. METHODS: This parallel-group randomized controlled trial will be conducted in two hospitals and patients' homes. A total of 183 adult stroke patients will be enrolled in the study and randomly assigned to three groups in a 1:1:1 ratio. The smartwatch intervention group (n = 61) will receive Real-time and Individualized Self-management Support (RISS) program + routine care, the wristband group (n = 61) will wear a fitness tracker (self-monitoring) + routine care, and the control group (n = 61) will receive routine stroke care. The intervention will last for 6 months. The primary outcomes are neurological function status, self-management behavior, quality of life, biochemical indicators, recurrence rate, and unplanned readmission rate. Secondary outcomes are resilience, patient activation, psychological status, and caregiver assessments. The analysis is intention-to-treat. The intervention effect will be evaluated at baseline (T0), 2 months after discharge (T1), 3 months after discharge (T2), and 6 months after discharge (T3). DISCUSSION: The cloud platform designed in this study not only has the function of real-time recording but also can push timely solutions when patients have abnormal conditions, as well as early warnings or alarms. This study could also potentially help patients develop good self-management habits through resilience theory, wearable devices, and individualized problem-solution library of self-management which can lay the foundation for long-term maintenance and continuous improvement of good self-management behavior in the future. TRIAL REGISTRATION: The ethics approval has been granted by the Ethics Committee of West China Hospital, Sichuan University (2022-941). All patients will be informed of the study details and sign a written informed consent form before enrollment. The research results will be reported in conferences and peer-reviewed publications. The trial registration number is ChiCTR2300070384 . Registered on 11 April 2023.

[Maresin1 inhibits the NF-κB/caspase-3/GSDME signaling pathway to alleviate hepatic ischemia-reperfusion injury].

Objective: To investigate the role of Maresin1 (MaR1) in hepatic ischemia-reperfusion injury (HIRI). Methods: The HIRI model was established and randomly divided into a sham operation group (Sham group), an ischemia-reperfusion group (IR group), and a MaR1 ischemia-reperfusion group (MaR1+IR group). MaR1 80ng was intravenously injected into each mouse's tail veins 0.5h before anesthesia. The left and middle hepatic lobe arteries and portal veins were opened and clamped. The blood supply was restored after 1h of ischemia. After 6h of reperfusion, the mice were sacrificed to collect blood and liver tissue samples. The Sham's group abdominal wall was only opened and closed. RAW267.4 macrophages were administered with MaR1 50ng/ml 0.5h before hypoxia, followed by hypoxia for 8h and reoxygenation for 2h, and were divided into the control group, the hypoxia-reoxygenation group (HR group), the MaR1 hypoxia-reoxygenation group (MaR1 + HR group), the Z-DEVD-FMK hypoxia-reoxygenation group (HR+Z group), the MaR1 + Z-DEVD-FMK hypoxia-reoxygenation group (MaR1 + HR + Z group), and the Con group without any treatment. Cells and the supernatant above them were collected. One-way analysis of variance was used for inter-group comparisons, and the LSD-t test was used for pairwise comparisons. Results: Compared with the Sham group, the levels of alanine aminotransferase (ALT), aspartate aminotransferase (AST), interleukin (IL)-1ß, and IL-18 in the IR group were significantly higher (P < 0.05), with remarkable pathological changes, while the level in the MaR1 + IR group was lower than before (P < 0.05), and the pathological changes were alleviated. Compared with the Con group, the HR group had higher levels of IL-1ß and IL-18 (P < 0.05), while the MaR1 + HR group had lower levels of IL-1ß and IL-18 (P < 0.05). Western blot showed that the expressions of caspase-3, GSDME, and GSDME-N were significantly higher in the HR group and IR group than in the other groups; however, the expression was lower following MaR1 pretreatment. The Z-DEVD-FMK exploration mechanism was inhibited by the expression of caspase-3 in HIRI when using MaR1. Compared with the HR group, the IL-1ß and IL-18 levels and the expressions of caspase-3, GSDME, and GSDME-N in the HR + Z group were decreased (P < 0.05), while the expression of nuclear factor κB was increased, but following MaR1 pretreatment, nuclear factor κB was decreased. There was no significant difference in the results between the MaR1 + H/R group and the MaR1 + H/R + Z group (P > 0.05). Conclusion: MaR1 alleviates HIRI by inhibiting NF-κB activation and caspase-3/GSDME-mediated inflammatory responses.

The radiomic biomarker in non-small cell lung cancer: 18F-FDG PET/CT characterisation of programmed death-ligand 1 status.

AIM: To present an integrated 2-[18F]-fluoro-2-deoxy-d-glucose (18F-FDG) positron-emission tomography (PET)/computed tomography (CT) radiomic characterisation of programmed death-ligand 1 (PD-L1) status in non-small-cell lung cancer (NSCLC). MATERIALS AND METHODS: In this retrospective study, 18F-FDG PET/CT images and clinical data of 394 eligible patients were divided into training (n=275) and test sets (n=119). Next, the corresponding nodule of interest was segmented manually on the axial CT images by radiologists. After which, the spatial position matching method was used to match the image positions of CT and PET, and radiomic features of the CT and PET images were extracted. Radiomic models were built using five different machine-learning classifiers and the performance of the radiomic models were further evaluated. Finally, a radiomic signature was established to predict the PD-L1 status in patients with NSCLC using the features in the best performing radiomic model. RESULTS: The radiomic model based on the PET intranodular region determined using the logistic regression classifier preformed best, yielding an area under the receiver operating characteristics curve (AUC) of 0.813 (95% CI: 0.812, 0.821) on the test set. The clinical features did not improve the test set AUC (0.806, 95% CI: 0.801, 0.810). The final radiomic signature for PD-L1 status was consisted of three PET radiomic features. CONCLUSION: This study showed that an 18F-FDG PET/CT-based radiomic signature could be used as a non-invasive biomarker to discriminate PD-L1-positive from PD-L1-negative in patients with NSCLC.

[Diagnostic value of lipoprotein-associated phospholipase A2 in OPBR combined with gallbladder cholesterol deposition].

Objective: To study the diagnostic value of lipoprotein-associated phospholipase A2(LP-PL-A2) in occult pancreaticobiliary reflux(OPBR) combined with gallbladder cholesterol deposition. Methods: This was a case-control study. Forty-six patients with OPBR who underwent gallbladder surgery at Shanghai East Hospital from December 2020 to October 2021, with gallbladder cholesterol deposition as the case group and the remainder as the control group, were included for analysis of their clinical data. Results: There were 21 cases in the case group, with 10 males and 11 females, and aged (57±12) years; 25 cases in the control group, with 11 males and 14 females, and aged (56±10) years. Serum LP-PL-A2 [(551.62±128.69) U/L] was significantly higher in the case group than in the control group [(436.70±135.88) U/L] (t=-2.80,P<0.01).Univariate analysis showed that LP-PL-A2 was a risk factor for OPBR combined with gallbladder cholesterol deposition, OR(95%CI):1.007(1.002-1.012), P=0.011. The area under the receiver operating characteristic curve (ROC) curve was 0.742, P=0.005. Conclusion: LP-PL-A2 is of diagnostic value in OPBR combined with gallbladder cholesterol deposition.

Measurement of the

The ^{18}O(α,γ)^{22}Ne reaction is critical for AGB star nucleosynthesis due to its connection to the abundances of several key isotopes, such as ^{21}Ne and ^{22}Ne. However, the ambiguous resonance energy and spin-parity of the dominant 470 keV resonance leads to substantial uncertainty in the ^{18}O(α,γ)^{22}Ne reaction rate for the temperature of interest. We have measured the resonance energies and strengths of the low-energy resonances in ^{18}O(α,γ)^{22}Ne at the Jinping Underground Nuclear Astrophysics experimental facility (JUNA) with improved precision. The key 470 keV resonance energy has been measured to be E_{α}=474.0±1.1 keV, with such high precision achieved for the first time. The spin-parity of this resonance state is determined to be 1^{-}, removing discrepancies in the resonance strengths in earlier studies. The results significantly improve the precision of the ^{18}O(α,γ)^{22}Ne reaction rates by up to about 10 times compared with the previous data at typical AGB temperatures of 0.1-0.3 GK. We demonstrate that such improvement leads to precise ^{21}Ne abundance predictions, with an impact on probing the origin of meteoritic stardust SiC grains from AGB stars.

Protective effects of protocatechuic acid on growth performance, intestinal barrier and antioxidant capacity in broilers challenged with lipopolysaccharide.

'Prohibition of the antibiotic uses' aggravates the problem of intestinal diseases in poultry, and nutritional regulation has become a research hotspot, such as supplementation with active ingredients derived from plants. This research was conducted to investigate the effects of protocatechuic acid (PCA) on growth, intestinal barrier, and antioxidant capacity of broilers injected with lipopolysaccharide (LPS). Four hundred and eighty 1-day-old yellow feather broilers were randomly allocated to four groups, each with six replicates of 20 broilers. The treatments were basal diet + saline injection (CON) or LPS injection (CON-LPS), and diets with 300 or 600 mg/kg PCA supplementation + LPS injection (P300, P600). Birds were injected intramuscularly on 17th and 19th day of age, then sampled on day 21. The LPS injection significantly decreased BW and average daily gain of broilers, and compared with birds in CON-LPS, PCA supplementation increased (P < 0.05) those variables; moreover, 300 mg/kg PCA also decreased the feed-to-gain ratio. No differences were observed in relative weights of immune organs (P > 0.05). LPS decreased the villus height/crypt depth ratio (V/C) in jejunum of broilers, while PCA (P300 and P600) increased (P < 0.05) the jejunal villus height and V/C compared with birds in CON-LPS. LPS challenge increased jejunal malondialdehyde (MDA) concentration and decreased total superoxide dismutase (T-SOD) and glutathione peroxidase (GSH-Px) activities in plasma (P < 0.05); compared with birds in CON-LPS, jejunal and plasmal GSH-Px activity (P300 and P600) and jejunal T-SOD activity (P300) were decreased (P < 0.05), and hepatic MDA concentration (P600) was increased (P < 0.05). LPS significantly decreased the transcript abundances of OCLN, ZO-1, JAM2, MUC2, SOD1, CAT and GPX in jejunal mucosa of birds, and supplementation with PCA attenuated the decrease in OCLN, JAM2, and MUC2 expression compared with birds in CON-LPS; moreover, 600 mg/kg PCA offset the deduction in SOD1, CAT and GPX expression. In conclusion, dietary supplementation with PCA could improve antioxidant status and attenuate the damage in intestinal barrier and loss in growth performance of LPS-challenged broilers, and 600 mg/kg PCA showed more improved effects on antioxidant capacity.

[Naringenin inhibits thoracic aortic aneurysm formation in mice with Marfan syndrome].

OBJECTIVE: To identify whether naringenin plays a protective role during thoracic aneurysm formation in Marfan syndrome. METHODS: To validate the effect of naringenin, Fbn1C1039G/+ mice, the mouse model of Marfan syndrome, were fed with naringenin, and the disease progress was evaluated. The molecular mechanism of naringenin was further investigated via in vitro studies, such as bioluminescence resonance energy transfer (BRET), atomic force microscope and radioligand receptor binding assay. RESULTS: Six-week-old Fbn1C1039G/+ mice were fed with naringenin for 20 weeks. Compared with the control group, naringenin significantly suppressed the aortic expansion [Fbn1C1039G/+ vs. Fbn1C1039G/++naringenin: (2.49±0.47) mm, n=18 vs. (1.87±0.19) mm, n=22, P < 0.05], the degradation of elastin, and the expression and activity of matrix metalloproteinase 2 (MMP2) and MMP9 in the ascending aorta of Fbn1C1039G/+ mice. Besides, treatment with naringenin for 6 weeks also attenuated the disease progress among the 20-week-old Fbn1C1039G/+ mice with established thoracic aortic aneurysms [Fbn1C1039G/+ vs. Fbn1C1039G/++naringenin: (2.24±0.23) mm, n=8 vs. (1.90±0.17) mm, n=8, P < 0.05]. To understand the underlying molecular mechanisms, we examined the effects of naringenin on angiotensin Ⅱ type 1 receptor (AT1) signaling and transforming growth factor-ß (TGF-ß) signaling respectively, which were the dominant signaling pathways contributing to aortopathy in Marfan syndrome as previously reported. The results showed that naringenin decreased angiotensin Ⅱ (Ang Ⅱ)-induced phosphorylation of protein kinase C (PKC) and extracellular regulating kinase 1/2 (ERK1/2) in HEK293A cell overexpressing AT1 receptor. Moreover, naringenin inhibited Ang Ⅱ-induced calcium mobilization and uclear factor of activated T-cells (NFAT) signaling. The internalization of AT1 receptor and its binding to ß-arrestin-2 with Ang Ⅱ induction were also suppressed by naringenin. As evidenced by atomic force microscope and radioligand receptor binding assay, naringenin inhibited Ang Ⅱ binding to AT1 receptor. In terms of TGF-ß signaling, we found that feeding the mice with naringenin decreased the phosphorylation of Smad2 and ERK1/2 as well as the expression of TGF-ß downstream genes. Besides, the serum level of TGF-ß was also decreased by naringenin in the Fbn1C1039G/+ mice. Furthermore, we detected the effect of naringenin on platelet, a rich source of TGF-ß, both in vivo and in vitro. And we found that naringenin markedly decreased the TGF-ß level by inhibiting the activation of platelet. CONCLUSION: Our study showed that naringenin has a protective effect on thoracic aortic aneurysm formation in Marfan syndrome by suppressing both AT1 and TGF-ß signaling.

[First report of invasive Pomacea snails in Shandong Province].

OBJECTIVE: To characterize the species of invasive Pomacea snails that were discovered for the first time in Shandong Province. METHODS: Pomacea snails samples were collected in the field of Jining City, Shandong Province on October 2021 for morphological identification. Pomacea snails were randomly sampled and genomic DNA was extracted from foot muscle tissues of Pomacea snails for multiplex PCR amplification. The PCR amplification product was sequenced. Then, the sequence was aligned and a phylogenetic tree was created using the software MegAlign 7.1.0. In addition, Angiostongylus cantonensis infection was detected in Pomacea snails with the lung microscopy. RESULTS: A total of 104 living Pomacea snails were collected, and all were characterized as Pomacea spp. based on morphological features. Of 12 randomly selected adult Pomacea snails, multiplex PCR assay and sequencing identified eleven snails as P. canaliculata and one as P. maculata. No A. cantonensis infection was detected in 104 Pomacea snails. CONCLUSIONS: This is the first report of invasive Pomacea snails in Shandong Province, where P. canaliculata and P. maculata are found.

[Lung transplantation for pulmonary alveolar proteinosis: a case report and literature review].

Objective: To observe the efficacy of lung transplantation for pulmonary alveolar proteinosis (PAP) patients and to improve the understanding of the therapy. Methods: The clinical data of a patient with autoimmune PAP treated with sequential homogenous bilateral lung transplantation were described and the literatures were reviewed. Results: This 55-year-old female patient was diagnosed with autoimmune PAP and had been treated with whole lung lavage for 19 times, but only achieved short-term symptomatic relief after each operation. Inhalation of granulocyte macrophage colony stimulating factor occurred allergic reactions. Lung transplantation was performed on February 15, 2022, and a significant improvement in oxygenation and clinical symptoms were observed. The patient remained stable during follow-up. Conclusion: Treatment with lung transplantation is safe and effective for end-stage patients with PAP in the early phase, but the long-term effect remains to be observed.

[Research advances on the techniques for diagnosing burn wound depth].

The accurate diagnosis of burn wound depth is particularly important for evaluating the disease prognosis of burn patients. In the past, the diagnosis of burn wound depth often relied on the subjective judgment of doctors. With the continuous development of diagnostic technology, the methods for judging the depth of burn wound have also been updated. This paper mainly summarizes the research progress in the applications of indocyanine green angiography, laser Doppler imaging, laser speckle contrast imaging, and artificial intelligence in the diagnosis of burn wound depth, and compares the advantages and disadvantages of these techniques, so as to provide ideas for accurate diagnosis of burn wound depth.

Rapid screening of monoclonal antibodies against porcine circovirus type 2 using colloidal gold-based paper test.

A proof of concept for using paper test as a suitable method in the production of monoclonal antibodies (MAbs) is reported. The paper test which detects antibodies against porcine circovirus type 2 (PCV2) using colloidal gold-labelled capsid protein as the antigen probe was applied exclusively in the screening of anti-PCV2 MAbs. It allowed the detection of 118 single cell clones within 30 min using naked eyes. MAbs with specific binding to authentic epitopes on the virus were selected using a blocking strategy in which the antibody was pre-incubated with PCV2 viral sample before applying to the test paper. Five hybridomas secreting MAbs against the capsid protein were obtained, with only three of them capable of binding to PCV2. The results were validated and confirmed using enzyme-linked immunosorbent assay and immunofluorescence assay. The paper test is simple, rapid, and independent on professional technicians and proves to be an excellent approach for the screening of MAbs against specific targets.

[Cardiometabolic disease patterns among elderly patients with colorectal cancer in China].

Objective: To explore the distribution patterns of cardiometabolic diseases (CMD) in elderly patients with colorectal cancer, and provide a reference for the prevention and treatment of cardiovascular metabolic diseases in these patients. Methods: Clinical data of 3 894 elderly patients with colorectal cancer from January 2008 to March 2018 admitted in the Chinese PLA General Hospital were recruited and the incidence rate of CMD was retrospectively analyzed. The influence factors of elderly patients with colorectal cancer combined with CMD were analyzed by multivariate Logistic regression model. Results: The morbidity rate of CMD in elderly patients with colorectal cancer is 33.4% (1 301/3 894), among them, the morbidity rate of the male was 31.9% (768/2 409), and that of the female was 35.9% (533/1 485). There was not significant difference between these two sex (P=0.074). The morbidity rates of CMD in patients of 65-74 years, 75-84 years and ≥85 years were 30.6% (754/2 462), 37.0% (479/1 294) and 49.3% (68/138), respectively, with significant differences (P<0.001). Multiple Logistic regression analysis revealed that female (OR=1.213, 95%CI: 1.056-1.394), age (75-84 years group: OR=1.344, 95%CI: 1.164-1.552; ≥85 years group: OR=2.345, 95%CI: 1.651-3.331) and body mass index (BMI 18.5-24.9 kg/m(2) group: OR=1.319, 95%CI: 1.065-1.638; ≥25 kg/m(2) group: OR=2.041, 95%CI: 1.627-2.561) were independent risk factors for elderly colorectal cancer patients with CMD. Conclusion: The morbidity rate of CMD in elderly patients with colorectal cancer increases with age and it is urgent to strengthen multidisciplinary cooperation and develop reasonable treatment plans to extend the survival and life quality of these patients.

[Clinical characteristics and molecular mechanisms of hypoparathyroidism related to GATA3 gene mutation].

Objective: To analyze the clinical characteristics and molecular mechanisms of 5 cases of hypoparathyroidism caused by GATA3 gene mutation. Methods: A total of 5 childhood-onset hypoparathyroidism patients with GATA3 mutation were identified from 198 hypoparathyroidism (aged ≤18 years) from 1975 to 2021 in Peking Union Medical College Hospital. Clinical data and biochemical indices of the 5 patients were collected and analyzed retrospectively. Genetic screening was conducted by targeted next-generation sequencing (T-NGS), and bioinformatics analysis was performed to analyze the underline mechanisms. Results: The medium onset age of hypoparathyroidism of the 5 patients was 0.5 (0.1, 1.3) years old, and the time duration from onset to confirmed diagnosis of hypoparathyroidism and hypoparathyroidism- deafness-renal dysplasia syndrome was (7.0±5.2) years and (15.0±5.4) years, respectively. The clinical manifestations included carpopedal spasm accompanied by seizures (5 cases), basal ganglia calcification (5 cases), cataract (1 case), deafness (4 cases), and renal malformations or absence (2 cases). The blood calcium and blood parathormone(PTH) before treatment was (1.65±0.31) mmol/L and (4.64±2.63) ng/L, respectively. The 5 patients carried different heterozygous mutations in GATA3 gene, which caused nonsense mutations, frameshift mutations and splice site mutations, respectively. All the GATA3 gene mutations of the 5 patients are classified as pathogenic or likely pathogenic by the Clin Var database and American College of Medical Genetics and Genomics(ACMG). Conclusions: Attention should be paid to genetic diseases in patients with childhood-onset hypoparathyroidism. The possibility of hypoparathyroidism-deafness-renal dysplasia syndrome should be considered in hypoparathyroidism patients with hearing loss or renal dysplasia. GATA3 gene screening is highly recommended for the confirmation of the diagnosis.