Objective:To investigate the clinical manifestations, pathological features, and genetic variant characteristics of children with glycogen storage disease type Ⅸa (GSD Ⅸa).Methods:A retrospective case series analysis was conducted to collected and analyzed the medical history, biochemical markers, liver ultrasound results, liver histopathological findings, genotypes, treatment regimens, and follow-up data of 4 pediatric patients diagnosed with GSD IXa in the Department of Infectious Diseases at Xiamen Children′s Hospital from January 2018 to May 2024. All patients were confirmed by genetic testing.Results:All 4 pediatric patients diagnosed with GSD Ⅸa were male. The ages of onset were 8 months, 2 years, 3 years and 3 months, 1 year and 5 months, respectively, with initial presentations including chronic diarrhea (Case 1), incidentally detected transaminase elevation during routine examinations (Cases 2 and 3), and delayed motor development (Case 4). Diagnosis was confirmed at ages 10 months, 3 years, 3 years 4 months and 1 year 6 months, respectively.At diagnosis, anthropometric parameters and biochemical profiles revealed:Height: 68 cm (< P3), 96 cm ( P25-50), 94 cm ( P3-10), and 94 cm ( P3-10).Weight: 7 kg (< P3), 17 kg ( P90-97), 14.4 kg ( P25-50), and 10.5 kg ( P25-50).Alanine aminotransferase: 299, 500, 271, and 313 U/L (reference range 0-40 U/L).Aspartate aminotransferase: 285, 543, 337 and 357 U/L (reference range 0-40 U/L).Fasting glucose: 2.80, 3.67, 2.98, and 3.66 mmol/L (reference range 3.90-6.10 mmol/L).Lactate: 4.3, 2.1, 1.3, and 2.6 mmol/L (reference range 0.5-2.2 mmol/L).Triglycerides: 5.22, 1.38, 1.32, and 1.88 mmol/L (reference range 0.56-1.70 mmol/L).Case 1 exhibited poor adherence to uncooked cornstarch therapy during initial treatment, with no significant improvement in biochemical parameters. Follow-up imaging at age 4 revealed hepatic adenoma. Subsequent improvement in therapeutic compliance led to biochemical normalization, reduced hepatic adenoma size, and growth parameters of 113 cm ( P10-25) and 26 kg ( P90-97) at 6 years 2 months. Cases 2-4 demonstrated biochemical improvement with regular uncooked cornstarch therapy and no evidence of hepatic adenoma.Liver histopathology in Cases 1-3 confirmed glycogen accumulation consistent with GSD, without cirrhotic changes. Genetic analysis identified PHKA2 variations in all cases: 2 missense variants, 1 frameshift variant and 1 nonsense variant. The c.2839dup and c.3267G>A variants represent novel pathogenic mutations. Conclusions:GSD Ⅸa in pediatric patients is predominantly characterized by hepatomegaly, hepatic dysfunction, and hypoglycemia. While uncooked cornstarch therapy typically yields favorable prognoses, a subset of patients may develop hepatic adenomas. Notably, children with hepatic adenoma exhibited younger age of onset, significant growth retardation, and more severe metabolic disturbances, suggesting that hepatic adenoma development may be closely linked to the severity of metabolic dysregulation.

Objective:This study retrospectively analyzed the clinical characteristics of patients newly diagnosed with acute myeloid leukemia (AML) who were admitted to the hematology intensive care unit (HCU) with critical illness. It also examined factors associated with critical illness and early mortality in these patients.Methods:Clinical data were collected from 91 newly diagnosed AML patients admitted to the HCU of the Department of Hematology, First Affiliated Hospital of Soochow University, from October 2020 to 2024. Reasons for HCU admission, major therapeutic interventions, and risk factors for critical illness and early mortality were analyzed.Results:The median time from diagnosis to HCU admission was 3 days ( IQR: 3–9 days), and the median HCU stay was 10 days ( IQR: 3–23 days). Of the 91 patients, 71 were admitted to the HCU before induction chemotherapy, while 20 were transferred to the HCU after its initiation. The leading causes of HCU admission were pulmonary infection (78.0% ), respiratory failure (44.0% ), hepatic insufficiency (28.6% ), renal insufficiency (27.5% ), disseminated intravascular coagulation (DIC; 25.3% ), and sepsis (23.1% ). Median Acute Physiology and Chronic Health Evaluation Ⅱ (APACHE Ⅱ) and SOFA scores at HCU admission were 14 ( IQR: 11–18) and the median Sepsis Related Organ Failure Assessment (SOFA) score was 7 ( IQR: 4, 10). Major HCU interventions included vasoactive drugs, noninvasive and invasive mechanical ventilation, continuous renal replacement therapy, therapeutic leukocyte clearance, and cardiopulmonary resuscitation. Among patients receiving induction chemotherapy, the composite complete remission rate was 65.4%, and the overall remission rate was 88.5%. Thirty-five (38.5% ) patients died within 28 days of HCU admission. Independent risk factors for 28-day mortality were DIC ( OR=9.350, 95% CI 1.999–43.745, P=0.005), sepsis ( OR=6.817, 95% CI 1.571–29.582, P=0.010), and cardiac insufficiency ( OR=12.281, 95% CI 2.385–63.254, P=0.003) . Conclusion:The main reason for HCU admission in newly diagnosed critically ill AML patients was pulmonary infection. Nearly 40% of patients experisenced early death, and DIC, sepsis, and heart failure were factors influencing early mortatlity.

Objective To investigate the effects of paravertebral nerve block(PVNB)under thoracoscopic direct vision on postoperative pain,stress response and T lymphocyte subsets in patients undergoing thoracoscopic lobectomy under general anesthesia.Methods A total of 104 patients who were scheduled to undergo thoracoscopic lobectomy in the hospital from January 2021 to August 2023 were enrolled in this study.They were divided into the control group(52 cases)and the observation group(52 cases)using the random number table.The control group was given general anesthesia,and the observation group was given PVNB on the basis of general anesthesia.Both groups received patient-controlled intravenous analgesia(PCIA)after surgery.Visual analogue pain(VAS)scores were recorded 1 hour(T1),6 hours(T2),12 hours(T3),24 hours(T4)and 48 hours(T5)after surgery in both groups.The drug use for PCIA,postoperative stress response,changes in T lymphocyte subsets,and incidence rates of adverse reactions in the two groups were analyzed.Results From T,to T5,VAS scores of the two groups increased firstly and then decreased.From T,to T4,VAS scores of the observation group were lower than those of the control group(P＜0.05).The first pressing time of PCIA in the observation group[(11.18±3.29)h]was longer than that in the control group[(9.61±2.48)h].The pressing times of PCIA and dosage of sufentanil[(4.63±1.51)times and(46.29±6.24)ml]were lower than those in the control group[(7.22±1.86)times and(55.41±7.88)ml](P＜0.05).At 24 h after surgery,the levels of cortisol,norepinephrine and blood glucose in the observation group[(252.27±19.75)ng/ml,(346.63±25.06)ng/ml and(5.48±0.72)mmol/L]were lower than those in the control group[(275.78±21.46)ng/ml,(381.71±23.71)ng/ml and(6.03±0.64)mmol/L](P＜0.05).The levels of CD3+,CD4+,CD8+T lymphocyte count and CD4+/CD8+T lymphocyte count in the observation group[(0.57±0.05)× 109/L,(0.39±0.04)× 109/L,(0.26±0.02)× 109/L and(1.50±0.35)]were higher than those in the control group[(0.55±0.05)× 109/L,(0.36±0.03)× 109/L,(0.29±0.04)× 109/L and(1.24±0.31)](P＜0.05).The incidence rates of adverse reactions in the two groups were 1.92％and 2.77％,without statistically significant difference between groups(P＞0.05).Conclusion Applying PVTB under thoracoscopic direct vision to patients undergoing thoracoscopic lobectomy under general anesthesia is beneficial for reducing postoperative pain,stress response,and the influence on immune system.

Objective:This study retrospectively analyzed the clinical characteristics of patients newly diagnosed with acute myeloid leukemia (AML) who were admitted to the hematology intensive care unit (HCU) with critical illness. It also examined factors associated with critical illness and early mortality in these patients.Methods:Clinical data were collected from 91 newly diagnosed AML patients admitted to the HCU of the Department of Hematology, First Affiliated Hospital of Soochow University, from October 2020 to 2024. Reasons for HCU admission, major therapeutic interventions, and risk factors for critical illness and early mortality were analyzed.Results:The median time from diagnosis to HCU admission was 3 days ( IQR: 3–9 days), and the median HCU stay was 10 days ( IQR: 3–23 days). Of the 91 patients, 71 were admitted to the HCU before induction chemotherapy, while 20 were transferred to the HCU after its initiation. The leading causes of HCU admission were pulmonary infection (78.0% ), respiratory failure (44.0% ), hepatic insufficiency (28.6% ), renal insufficiency (27.5% ), disseminated intravascular coagulation (DIC; 25.3% ), and sepsis (23.1% ). Median Acute Physiology and Chronic Health Evaluation Ⅱ (APACHE Ⅱ) and SOFA scores at HCU admission were 14 ( IQR: 11–18) and the median Sepsis Related Organ Failure Assessment (SOFA) score was 7 ( IQR: 4, 10). Major HCU interventions included vasoactive drugs, noninvasive and invasive mechanical ventilation, continuous renal replacement therapy, therapeutic leukocyte clearance, and cardiopulmonary resuscitation. Among patients receiving induction chemotherapy, the composite complete remission rate was 65.4%, and the overall remission rate was 88.5%. Thirty-five (38.5% ) patients died within 28 days of HCU admission. Independent risk factors for 28-day mortality were DIC ( OR=9.350, 95% CI 1.999–43.745, P=0.005), sepsis ( OR=6.817, 95% CI 1.571–29.582, P=0.010), and cardiac insufficiency ( OR=12.281, 95% CI 2.385–63.254, P=0.003) . Conclusion:The main reason for HCU admission in newly diagnosed critically ill AML patients was pulmonary infection. Nearly 40% of patients experisenced early death, and DIC, sepsis, and heart failure were factors influencing early mortatlity.

Objective:To investigate the clinical manifestations, pathological features, and genetic variant characteristics of children with glycogen storage disease type Ⅸa (GSD Ⅸa).Methods:A retrospective case series analysis was conducted to collected and analyzed the medical history, biochemical markers, liver ultrasound results, liver histopathological findings, genotypes, treatment regimens, and follow-up data of 4 pediatric patients diagnosed with GSD IXa in the Department of Infectious Diseases at Xiamen Children′s Hospital from January 2018 to May 2024. All patients were confirmed by genetic testing.Results:All 4 pediatric patients diagnosed with GSD Ⅸa were male. The ages of onset were 8 months, 2 years, 3 years and 3 months, 1 year and 5 months, respectively, with initial presentations including chronic diarrhea (Case 1), incidentally detected transaminase elevation during routine examinations (Cases 2 and 3), and delayed motor development (Case 4). Diagnosis was confirmed at ages 10 months, 3 years, 3 years 4 months and 1 year 6 months, respectively.At diagnosis, anthropometric parameters and biochemical profiles revealed:Height: 68 cm (< P3), 96 cm ( P25-50), 94 cm ( P3-10), and 94 cm ( P3-10).Weight: 7 kg (< P3), 17 kg ( P90-97), 14.4 kg ( P25-50), and 10.5 kg ( P25-50).Alanine aminotransferase: 299, 500, 271, and 313 U/L (reference range 0-40 U/L).Aspartate aminotransferase: 285, 543, 337 and 357 U/L (reference range 0-40 U/L).Fasting glucose: 2.80, 3.67, 2.98, and 3.66 mmol/L (reference range 3.90-6.10 mmol/L).Lactate: 4.3, 2.1, 1.3, and 2.6 mmol/L (reference range 0.5-2.2 mmol/L).Triglycerides: 5.22, 1.38, 1.32, and 1.88 mmol/L (reference range 0.56-1.70 mmol/L).Case 1 exhibited poor adherence to uncooked cornstarch therapy during initial treatment, with no significant improvement in biochemical parameters. Follow-up imaging at age 4 revealed hepatic adenoma. Subsequent improvement in therapeutic compliance led to biochemical normalization, reduced hepatic adenoma size, and growth parameters of 113 cm ( P10-25) and 26 kg ( P90-97) at 6 years 2 months. Cases 2-4 demonstrated biochemical improvement with regular uncooked cornstarch therapy and no evidence of hepatic adenoma.Liver histopathology in Cases 1-3 confirmed glycogen accumulation consistent with GSD, without cirrhotic changes. Genetic analysis identified PHKA2 variations in all cases: 2 missense variants, 1 frameshift variant and 1 nonsense variant. The c.2839dup and c.3267G>A variants represent novel pathogenic mutations. Conclusions:GSD Ⅸa in pediatric patients is predominantly characterized by hepatomegaly, hepatic dysfunction, and hypoglycemia. While uncooked cornstarch therapy typically yields favorable prognoses, a subset of patients may develop hepatic adenomas. Notably, children with hepatic adenoma exhibited younger age of onset, significant growth retardation, and more severe metabolic disturbances, suggesting that hepatic adenoma development may be closely linked to the severity of metabolic dysregulation.

Objective To investigate the effects of paravertebral nerve block(PVNB)under thoracoscopic direct vision on postoperative pain,stress response and T lymphocyte subsets in patients undergoing thoracoscopic lobectomy under general anesthesia.Methods A total of 104 patients who were scheduled to undergo thoracoscopic lobectomy in the hospital from January 2021 to August 2023 were enrolled in this study.They were divided into the control group(52 cases)and the observation group(52 cases)using the random number table.The control group was given general anesthesia,and the observation group was given PVNB on the basis of general anesthesia.Both groups received patient-controlled intravenous analgesia(PCIA)after surgery.Visual analogue pain(VAS)scores were recorded 1 hour(T1),6 hours(T2),12 hours(T3),24 hours(T4)and 48 hours(T5)after surgery in both groups.The drug use for PCIA,postoperative stress response,changes in T lymphocyte subsets,and incidence rates of adverse reactions in the two groups were analyzed.Results From T,to T5,VAS scores of the two groups increased firstly and then decreased.From T,to T4,VAS scores of the observation group were lower than those of the control group(P＜0.05).The first pressing time of PCIA in the observation group[(11.18±3.29)h]was longer than that in the control group[(9.61±2.48)h].The pressing times of PCIA and dosage of sufentanil[(4.63±1.51)times and(46.29±6.24)ml]were lower than those in the control group[(7.22±1.86)times and(55.41±7.88)ml](P＜0.05).At 24 h after surgery,the levels of cortisol,norepinephrine and blood glucose in the observation group[(252.27±19.75)ng/ml,(346.63±25.06)ng/ml and(5.48±0.72)mmol/L]were lower than those in the control group[(275.78±21.46)ng/ml,(381.71±23.71)ng/ml and(6.03±0.64)mmol/L](P＜0.05).The levels of CD3+,CD4+,CD8+T lymphocyte count and CD4+/CD8+T lymphocyte count in the observation group[(0.57±0.05)× 109/L,(0.39±0.04)× 109/L,(0.26±0.02)× 109/L and(1.50±0.35)]were higher than those in the control group[(0.55±0.05)× 109/L,(0.36±0.03)× 109/L,(0.29±0.04)× 109/L and(1.24±0.31)](P＜0.05).The incidence rates of adverse reactions in the two groups were 1.92％and 2.77％,without statistically significant difference between groups(P＞0.05).Conclusion Applying PVTB under thoracoscopic direct vision to patients undergoing thoracoscopic lobectomy under general anesthesia is beneficial for reducing postoperative pain,stress response,and the influence on immune system.

Periodontitis is a chronic inflammatory condition of the periodontal tissues triggered by bacterial biofilm, leading to manifestations such as gingival bleeding, tooth mobility, and eventual exfoliation. Neutrophils exhibit a dual role throughout the course of periodontitis, both in defense against pathogens and in potentially detrimental effects on periodontal tissues. This article elucidates the intricate mechanisms underlying the dual functions of neutrophils in periodontitis, including respiratory burst, neutrophil extracellular traps (NETs) formation, degranulation, and phagocytosis. By providing a comprehensive understanding of neutrophils involvement in periodontitis, this study aims to empower clinicians with insights into the pathogenesis of periodontitis, thereby fostering novel strategies for its prevention and treatment.

Objective To observe the effect of different doses of esketamine on early mood in pa-tients with preoperative anxiety after thoracoscopic surgery.Methods Eighty patients with preoperative anx-iety before thoracoscopic surgery,37 males and 43 females,aged 25-64 years,BMI 18-26 kg/m2,ASA physical statusⅠ-Ⅲwere selected.According to random number table,the patients were randomly divided into four groups:control group(group C),esketamine 0.1 mg/kg group(group E1),esketamine 0.2 mg/kg group(group E2),and esketamine 0.3 mg/kg group(group E3),20 patients in each group.In groups E1,E2,and E3,the patients were slowly injected with corresponding doses of esketamine intrave-nously 30 minutes before the end of the operation,and the patients in group C were given 10 ml of normal saline at the same time.The 7-item generalized anxiety disorder scale(GAD-7)score and hospital anxiety and depression scale(HADS)score were evaluated 24 hours before surgery,24,48,72,and 120 hours af-ter surgery,respectively.Peripheral venous blood(5 ml)was collected at the same time points,and serum brain-derived neurotrophic factor(BDNF)concentration was detected by ELISA.NRS scores at rest and ex-ercise were recorded 12 and 24 hours after surgery.The number of remedial analgesia and the number of ef-fective compressions of analgesia pump within 48 hours after surgery were recorded.Results Compared with group C,the scores of GAD-7 and HADS in groups E1,E2,and E3 were significantly decreased 24,48,and 72 hours after surgery(P＜0.05).And compared with groups E1 and E2,the scores of GAD-7 and HADS in group E3 were significantly lower(P＜0.05).The serum BDNF level in groups E1,E2,and E3 were significantly increased compared with group C 24,48,and 72 hours after surgery(P＜0.05).Compared with group E1 and E2,the serum BDNF level in group E3 was significantly higher(P＜0.05).Compared with group C,the NRS scores at rest 12 and 24 hours and the scores at exercise 12 hours after surgery in groups E1,E2,and E3 were significantly decreased(P＜0.05).And compared with groups E1 and E2,the scores in group E3 were significantly lower(P＜0.05).The number of postoperative remedial analgesia and the number of effective compressions of analgesia pump in groups E1,E2,and E3 were signif-icantly decreased compared with group C within 48 hours after surgery(P＜0.05).And compared with groups E1 and E2,the number in group E3 was significantly lower(P＜0.05).Conclusion Single intra-venous injection of esketamine 0.1,0.2,and 0.3 mg/kg can improve early postoperative mood of patients with anxiety before thoracoscopic surgery,and esketamine 0.3 mg/kg has better anti-anxiety effect.

Objective:Combined retrograde tracing with optogenetic methods,we are investigating the functional role of dorsal raphe nucleus(DRN)GABAergic neurons projecting to lateral habenula(LHb)terminals in anxiety-like behaviors.Methods:The specific retrograde tracing virus AAVretro-Ef1α-DIO-mCherry was injected into the LHb of Vgat-Cre mice.After the viral expression,Multi-brain slides scanning microscope imaging scan and observe the up-stream distribution of GABAergic neural projection in LHb.By retrograde tracing,opto-activated virus AAV2/9-Ef1a-DIO-ChR2-mCherry(ChR2 group)and control virus AAV2/9-Ef1a-DIO-mCherry(mCherry group)were injected into the DRN of Vgat-cre mice respectively.Three weeks after virus expression,DRNGABA neurons and the DRNCABA-LHb neural terminals were activated by optogenetic to observe their role in anxiety-like behaviors.Results:According to the results of retrograde tracing,the midbrain DRN is one of the major GABAergic neural projection brain areas in the LHb nucleus.Optogenetic stimulation of DRNGABA neurons,compared with the mCherry group,the ChR2 group showed sig-nificantly longer total moving distance,central area moving time and distance in the open field test(OFT);In the ele-vated plus maze(EPM),the open arm moving time and distance was significantly increased.When DRN GABA-LHb neural terminals were stimulated,compared with the mCherry group,the ChR2 group showed a significantly longer cen-tral zone moving time,distance and total moving distance in the OFT.During the elevated plus maze(EPM),the open arm moving time was significantly increased.Conclusion:The specific activation of the DRNGABA neuron as well as the DRNGABA-LHb neural projections showed that both significantly improved anxiety-like behaviors in mice.This provides new ideas and evidence for the treatment of anxiety,depression and other psychiatric disorders.

Background::Transplant renal artery stenosis (TRAS) is a vascular complication after kidney transplantation associated with poor outcomes. This study aimed to analyze the efficacy and safety of low-dose aspirin for preventing TRAS.Methods::After kidney transplantation, patients were enrolled from January 2018 to December 2020 in Henan Provincial People’s Hospital. A total of 351 enrolled recipients were randomized to an aspirin group with low-dose intake of aspirin in addition to standard treatment ( n = 178), or a control group with only standard treatment ( n = 173). The patients was initially diagnosed as TRAS (id-TRAS) by Doppler ultrasound, and confirmed cases were diagnosed by DSA (c-TRAS). Results::In the aspirin and control groups, 15.7% (28/178) and 22.0% (38/173) of the recipients developed id-TRAS, respectively, with no statistical difference. However, for c-TRAS, the difference of incidence and cumulative incidence was statistically significant. The incidence of c-TRAS was lower in the aspirin group compared with the control group (2.8% [5/178] vs. 11.6% [20/173], P = 0.001). Kaplan–Meier estimates and Cox regression model identified the cumulative incidence and hazard ratio (HR) of TRAS over time in two groups, showing that recipients treated with aspirin had a significantly lower risk of c-TRAS than those who were not treated (log-rank P = 0.001, HR = 0.23, 95% confidence interval [CI]: 0.09–0.62). The levels of platelet aggregation rate ( P < 0.001), cholesterol ( P = 0.028), and low-density lipoprotein cholesterol ( P = 0.003) in the aspirin group were decreased compared with the control group in the third-month post-transplantation. For the incidence of adverse events, there was no statistical difference. Conclusion::Clinical application of low-dose aspirin after renal transplant could prevent the development of TRAS with no significant increase in adverse effects.Trial Registration::Clinicaltrials.gov, NCT04260828.