Metachromatic leukodystrophy (MLD) is an inherited disease caused by a deficiency of the enzyme arylsulfatase A (ARSA). Lentivirus-modified autologous hematopoietic stem cell gene therapy (HSCGT) has recently been approved for clinical use in pre and early symptomatic children with MLD to increase ARSA activity. Unfortunately, this advanced therapy is not available for most patients with MLD who have progressed to more advanced symptomatic stages at diagnosis. Patients with late-onset juvenile MLD typically present with a slower neurological progression of symptoms and represent a significant burden to the economy and healthcare system, whereas those with early onset infantile MLD die within a few years of symptom onset. We conducted a pilot study to determine the safety and benefit of HSCGT in patients with postsymptomatic juvenile MLD and report preliminary results. The safety profile of HSCGT was favorable in this long-term follow-up over 9 years. The most common adverse events (AEs) within 2 months of HSCGT were related to busulfan conditioning, and all AEs resolved. No HSCGT-related AEs and no evidence of distorted hematopoietic differentiation during long-term follow-up for up to 9.6 years. Importantly, to date, patients have maintained remarkably improved ARSA activity with a stable disease state, including increased Functional Independence Measure (FIM) score and decreased magnetic resonance imaging (MRI) lesion score. This long-term follow-up pilot study suggests that HSCGT is safe and provides clinical benefit to patients with postsymptomatic juvenile MLD.
Humans ; Leukodystrophy, Metachromatic/genetics* ; Pilot Projects ; Genetic Therapy/methods* ; Hematopoietic Stem Cell Transplantation ; Male ; Follow-Up Studies ; Female ; Lentivirus/genetics* ; Child ; Child, Preschool ; Hematopoietic Stem Cells/metabolism* ; Cerebroside-Sulfatase/metabolism* ; Adolescent

OBJECTIVES: This study aims to analyze the clinical symptoms and imaging manifestations in patients with temporomandibular disorder syndrome (TMD), who are sensitive to sudden temperature drop. METHODS: One hundred and nineteen patients with TMD who attended the Department of Stomatology of the First Medical Center of Chinese People's Liberation Army General Hospital from December 2022 to December 2023 were included, including 44 males and 75 females, with a mean age of 32.4±13.7 years.The questionnaire was used to determine whether they were sensitive to temperature drop, and the TMD patients were divided into a temperature plunge-sensitive group and a temperature drop insensitive group. The clinical symptoms and imaging manifestations of patients in the two groups were observed. SPSS 25.0 was used for statistical analysis. RESULTS: There was no statistically significant difference between the gender and age of patients in the temperature plunge-sensitive group (50 patients) and the insensitivity group (69 patients) (P>0.05). The percentage of patients with pain was slightly higher in the temperature plunge-sensitive group [86.0% (43/50)] than in the insensitive group [68.1% (47/69)], and the difference was statistically significant (χ²=5.031, P=0.025), while the differences in joint murmur and mouth opening limitation between the two groups were not statistically significant. A total of 238 lateral joints were detected in both groups, the percentage of osteoarthropathic imaging changes was significantly higher in the temperature plunge-sensitive group [82.0% (82/100)] than in the insensitive group [53.6% (74/138)] (χ²=20.675, P<0.001). Magnetic imaging showed that the percentage of joint effusion was higher in patients in the temperature plunge-sensitive group [66.0% (33/50)] than in the insensitive group [42.0% (29/69)], and the difference was statistically significant (χ²=5.602, P=0.018). CONCLUSIONS TMD patients with maxillofacial pain symptoms, joint effusions, and abnormal imaging of osteoarticular structures are more likely to be sensitive to sudden temperature drops.
Humans ; Male ; Female ; Adult ; Temporomandibular Joint Disorders/diagnosis* ; Surveys and Questionnaires ; Middle Aged ; Young Adult ; Temperature ; Adolescent

Critically ill patients are at high risk for hospital acquired infections, which can significantly increase the mortality rate and treatment costs for these patients. Therefore, in the process of treating the primary disease, strict prevention and control of new hospital infections is an essential component of the treatment for critically ill patients. The treatment of critically ill patients involves multiple steps and requires a concerted effort from various aspects such as theory, management, education, standards, and supervision to achieve effective prevention and control of hospital infections. However, there is currently a lack of unified understanding and standards for hospital infection prevention and control. To address this, in March 2024, a group of experts in critical care medicine, infectious diseases, and hospital infection from China discussed the current situation and issues of hospital infection control in the intensive care unit together. Based on a review of the latest evidence-based medical evidence from both domestic and international sources, 2024 Expert Consensus on Hospital Acquired Infection Control Principles in the Department of Critical Care Medicine was formed, aiming to provide a basis for the development of hospital infection prevention and control strategies in the field of critical care medicine.

Objective This study aims to observe the clinical effect of bone plate reduction in combination with a re-sorbable plate on large mandibular cysts.Methods Between October 2017 and September 2022,patients with large mandibular cysts in the presence of labial and buccal cortical bone were involved in the study.Intraoral approach was performed for bone plate reduction.Cone beam computed tomography(CBCT)scan was reviewed at 3,6,and 9 months postoperatively to observe postoperative complications.Osteogenic results were assessed at these times to determine the clinical outcomes of this procedure.Results Eleven cases with large mandibular cysts in the presence of cortical bone were evaluated.The average thickness of the cortical bone on the labial and buccal sides was measured to be about(1.98±0.37)mm before surgery,with a mean value of(0.73±0.17)mm at the thinnest part of the plate and up to 0.51 mm at the thinnest part of the plate.The cystic cavities were well re-vealed during the surgeries,which were completed suc-cessfully.Postoperatively,the wounds healed in one stage without infection.The percentages of cyst shrinkage were 20.01%,41.76%,and 73.41%at 3,6,and 9 months after surgery,respectively.Quantitative measurement of bone mineral density in the jaws by CBCT with MIMICS software.The bone mineral densities of the adult bone were 313.78,555.85,and 657.45 HU at the 3,6,and 9 month time intervals,respectively.No significant change in the patient's maxillofacial appearance were observed from the preoperative period as assessed by the patient's and observer's visual analog scale.Conclusion Bone plate reduction is an effective treatment for large mandibular cysts of the oral and maxillofacial re-gion with the presence of cortical bone.

Bronchopulmonary dysplasia is the most common chronic lung disease in very preterm infants.Some severe bronchopulmonary dysplasia is often combined with pulmonary hypertension，characterized by diffuse alveolar damage and abnormal pulmonary vascular remodeling，followed by right heart failure due to increased pulmonary vascular resistance.It seriously affects the physical and neurological development of preterm infants.Pulmonary vasodilator drugs can reduce pulmonary artery pressure through nitric oxide pathway，endothelin pathway and prostaglandin pathway.This review summarized the pulmonary vasodilator drugs in the treatment of pulmonary hypertension associated with bronchopulmonary dysplasia.

Objective:To analyze the efficacy, safety and prognostic factors of immune checkpoint inhibitors in the treatment of recurrent and metastatic cervical cancer.Methods:A total of 87 patients with recurrent and metastatic cervical cancer admitted to the First Affiliated Hospital of Soochow University from January 2018 to June 2022 were retrospectively analyzed. They were divided into non immunotherapy group ( n=32) and immunotherapy group ( n=55) according to whether immune checkpoint inhibition was applied after recurrence and metastasis. The disease control rate (DCR), progression free survival (PFS), overall survival 1 (OS1, date of pathology diagnosis to the end of follow-up or time of death), overall survival 2 (OS2, time of first immunotherapy/non-immunotherapy to the end of follow-up or time of death), safety and prognostic factors of the two groups were analyzed and compared. Results:In 87 patients with recurrent and metastatic cervical cancer, the DCR of the non immunotherapy group and immunotherapy group were 53.1% (17/32) and 72.7% (40/55) respectively ( χ2=3.44, P=0.064). The median OS1 of the non immunotherapy group was 51.0 months, while the immunotherapy group did not reach the median OS1, with a statistically significant difference ( χ2=7.50, P=0.006). The median OS2 of the non immunotherapy group was 28.0 months, while the immunotherapy group did not reach the median OS2, with a statistically significant difference ( χ2=7.07, P=0.008). The median PFS of the non immunotherapy group and immunotherapy group were 18.0 months and 23.0 months respectively, with no significant difference ( χ2=0.01, P=0.915). In the immunotherapy group, 70.9% (39/55) of patients received immune checkpoint inhibitors as first-line treatment and 29.1% (16/55) received as second-line and above treatment. Both groups of patients did not achieve median OS2, with median PFS of 23.0 and 17.0 months respectively, and there were no statistically significant differences ( χ2=0.94, P=0.333; χ2=2.00, P=0.158) ; 38.2% (21/55) of patients received immune checkpoint inhibitor combined with local radiotherapy, 61.8% (34/55) patients did not receive radiotherapy. And neither group of patients achieved median OS2, with median PFS of 19.0 and 25.0 months respectively, with no statistically significant differences ( χ2=0.62, P=0.432; χ2=0.01, P=0.906). The incidences of grade 1-2 hematuria and hypothyroidism in the non immunotherapy group and immunotherapy group were 53.1% (17/32) vs. 27.3% (15/55, χ2=5.82, P=0.016), 3.1% (1/32) vs. 21.8% (12/55, χ2=4.19, P=0.041) respectively. The incidence of myelosuppression in the non immunotherapy group [grade 1-2: 59.4% (19/32), grade 3-4: 34.4% (11/32) ] was significantly different from that in the immunotherapy group [grade 1-2: 80.0% (44/55), grade 3-4: 3.6% (2/55) ; Z=3.50, P<0.001]. There were no statistically significant differences between creatinine increase, glutamic-oxaloacetic transaminase and glutamic-pyruvic transaminase increase, lymphocyte decrease, hypoproteinemia, proteinuria, rash, fatigue (all P>0.05). Univariate regression analysis showed that the use of immune checkpoint inhibitor was an independent protective factor affecting the prognosis of patients ( HR=0.31, 95% CI: 0.12-0.77, P=0.012) . Conclusion:Whether used as first-line or second-line or above treatment, the use of immune checkpoint inhibitors in patients with recurrent and metastatic cervical cancer prolongs their OS1, OS2, and has good safety. The application of immune checkpoint inhibitors is an independent protective factor affecting the prognosis of patients.

Vasovagal syncope(VVS) is a common clinical reflex syncope, which is easy to occur repeatedly.Although there is no direct life risk, it causes varying degrees of physical injury and psychological disorders to children, affects their daily life and study, and also causes anxiety of parents.At present, the diagnosis of VVS mainly depends on the vertical tilt table test.With the development of translational medicine, identifying VVS and other types of syncope by other methods has achieved important clinical value, which will be more convenient for primary hospital to carry out the related work of syncope diagnosis and treatment.This paper will review the related progress in the differential diagnosis of VVS.

Objective:To investigate the clinical symptoms and cone-beam CT (CBCT) imaging characteristics of temporomandibular joint osteoarthritis (TMJOA) with chewing side preference (CSP).Methods:One hundred patients with TMJOA diagnosed in the Department of Stomatology, General Hospital of the Chinese PLA from January 2018 to December 2020 were enrolled, including 32 males and 68 females, with an median age of 27.5 years (16-71 years). According to the habit of CSP, 100 cases were divided into 71 cases of TMJOA with CSP group and 29 cases of TMJOA without CSP group. The clinical symptoms were observed, including pain, TMJ sounds, limited mouth opening as well as the radiograph imaging changes of condylar bone. When analyzing the radiograph imaging changes of condylar, the cases with bilateral TMJ symptoms were excluded and the remaining cases were divided into symptomatic sides and asymptomatic sides with CSP or without CSP according to the symptoms of the chief complaint. SPSS 25.0 was used to analyze the statistical data. Age data did not conform to normal distribution so that median and quartile spacing were used for description, and Mann-Whitney U test was used for nonparametric test. Qualitative data such as gender, clinical symptoms and condylar lesion types were described by composition ratio and chi-square test was performed. Results:There was no statistical significance in age and gender of TMJOA patients in the group with or without CSP ( P>0.05). The incidence of pain in CSP group [83.1% (59/71)] was marginally higher than that in non-CSP group [65.5% (19/29)] but without statistical difference (χ2 =3.71, P=0.054). There was also no significant difference in TMJ sounds and limitation of mandibular movement between the two groups (χ2 =0.11, P=0.742; χ2 =0.48, P=0.489). Among all of joints, the most common types of TMJOA were articular flattening and shortening and erosion. CBCT showed that erosion [65.0% (130/200)], flattening and shortening [73.0% (146/200)], subcortical sclerosis [42.0% (84/200)], osteophyte [30.5% (61/200)] and subcortical cystic [15.5% (31/200)]. According to the different groups of chief complaint sides, intra-group comparisons show that the proportion of erosion in symptomatic sides of CSP group [80.0% (40/50)] was significantly higher than that in asymptomatic sides of CSP group [50.0% (25/50)] (χ2=9.89, P=0.002). Inter-group comparisons show that the proportion of condyle flattening and shortening in symptomatic sides of CSP group [84.0% (42/50)] was significantly higher than that in bilateral joint of non-CSP group (8/15) (χ2=8.81, P=0.032). There was no significant difference in the proportion of subcortical sclerosis, osteophyte and subcortical cystic between the group with or without CSP ( P>0.05). Conclusions:TMJOA patients with CSP may be more prone to clinical symptoms of pain and CBCT imaging changes of condyle erosion as well as flattening and shortening. CSP may be a promoting factor for the development of TMJOA.

Objective:To analyze the clinical symptoms and types of disc displacement of patients with anterior disc displacement (ADD) of temporomandibular joint (TMJ) and to explore the correlation between chewing side preference (CSP) and the clinical symptoms and types of disc displacement of ADD patients.Methods:This was a cross-sectional study of the ADD patients diagnosed by clinical symptoms and MRI findings of TMJ in the Department of Stomatology, General Hospital of Chinese PLA from January 2018 to December 2019. A total of 111 ADD patients [33 males and 78 females, aged (31.0±10.9) years old] were included. The patients were divided into two groups according to whether they had CSP: non-CSP group ( n=40) and CSP group ( n=71). The clinical symptoms and types of ADD were observed and recorded. According to the specific side of CSP in the CSP group, the patients were further divided into the ipsilateral side and the contralateral side of CSP. The pain, TMJ sounds and the ADD types were also observed. Chi square test was used to compare the pain, TMJ sounds, limited mouth opening and ADD types of the two groups. Binary Logistic regression model was used to analyze the correlation between CSP and clinical symptoms and ADD characteristics. Results:The incidence of pain in the CSP group [89% (63/71)] was significantly higher than that in the non-CSP group [70% (28/40)] ( P<0.05). In CSP patients, the incidences of pain and joint sounds on the ipsilateral side of CSP were significantly higher than that on the contralateral side ( P<0.05). MRI showed that the incidence of bilateral ADD in the CSP group [58%(41/71)] was significantly higher than that in the non-CSP group [37%(15/40)] ( P<0.05).There were significant differences in the incidences of no disc displacement (DD), anterior disk displacement with reduction(ADDwR) and anterior disk displacement without reduction (ADDwoR) between ipsilateral and contralateral joints of the CSP patients ( P<0.05). There were no significant differences in the incidences of no DD, ADDwR and ADDwoR between left and right joints of the non-CSP patients ( P>0.05). The incidence of ADDwoR in ipsilateral side of the CSP patients was significantly higher than that in contralateral side ( P<0.05). Pain ( OR=3.375) and the side of disc displacement ( OR=2.278) in patients with ADD were positively correlated with CSP ( P<0.05). Conclusions:The clinical symptoms and types of disc displacement between patients with and without CSP were significantly different. The incidences of pain and bilateral disc displacement in the CSP group were significantly higher than those in the non-CSP group. In addition, the incidences of pain, TMJ sounds and anterior disk displacement without reduction on the ipsilateral side of patients with CSP were significantly higher than those on the contralateral side. The pain symptoms and side of disc displacement were positively related to CSP.

Objective:To understand quality improvement (QI) in resuscitation and its impacts on the outcomes in very low birth weight infants (VLBWI).Methods:This was a retrospective study involving 318 cases of VLBWI born in Chongqing Health Center for Women and Children and transferred to the Neonatal Intensive Care Unit (NICU) in the same hospital from January 1, 2017 to December 31, 2019. All recruited VLBWIs were divided into three groups based on chronological order: pre-QI group (born in 2017, n=83), under-QI group (born in 2018, n=102) and post-QI group (born in 2019, n=133). The implementation and effects of different resuscitation strategies, including body temperature management, delayed cord clamping (DCC), respiratory support [continuous positive airway pressure (CPAP) in the delivery room, positive pressure ventilation or tracheal intubation] and oxygen therapy [initial fraction of inspired oxygen (FiO 2), maximum FiO 2 and pure oxygen resuscitation], and the neonatal outcomes in each year were analyzed and compared by trend Chi-square test, Kruskal-Wallis H test, Wilcoxon rank-sum test, analysis of variance and LSD test. Results:(1) The proportion of antenatal steroid exposure increased gradually during the three years [44.6% (37/83), 47.1% (48/102), 72.2% (96/133); χ2trend=18.218, P<0.001]. (2) From 2017 to 2019, the incidence of hypothermia on admission decreased [100.0% (83/83), 90.2% (92/102), 33.8% (45/133); χ2trend=136.042, P<0.001], without increasing the proportion of hyperthermia [0.0% (0/83), 1.0% (1/102), 0.0% (0/133); χ2trend=2.124, P=0.346]. (3) The proportion of VLBWIs receiving DCC increased annually [0.0% (0/83), 29.4% (30/102), 90.2% (120/133); χ2trend=178.659, P<0.001], and the concentration of capillary hemoglobin within 12-24 h after birth also increased [(190.1±34.8), (202.0±29.7), (213.6±32.3) g/L; LSD test, P<0.05]. The incidence of suspected polycythemia (capillary hematocrit >68%) increased annually[7.2%(6/83), 7.8%(8/102), 17.3(23/133); χ2trend=5.825, P=0.016]; While the incidence of confirmed polycythemia (venous hematocrit >65%) was not increased[1.3%(1/80), 2.0%(2/100), 4.8%(6/126); χ2trend=2.333, P=0.127] after excluding those untested cases. (4) The use of CPAP in delivery room increased annually [0.0% (0/83), 28.4% (29/102), 87.2%(116/133); χ2trend=167.721, P<0.001], while that of positive pressure ventilation [44.6% (37/83), 31.9% (32/102), 28.6% (38/133); χ2trend=5.371, P=0.020], and the proportion of intubation within 1 min after birth [100.0% (31/31), 45.0% (9/20), 35.0%(7/20); χ2trend=37.467, P<0.001] and overall intubation [37.3% (31/83), 19.6% (20/102), 15.0% (20/133); χ2trend=13.566, P<0.001] decreased year by year. There was no difference in the percentage of infants receiving chest compression or epinephrine, or with low Apgar scores during the three years (all P>0.05). (5) The initial inhaled FiO 2 [30%(21%-100%), 30%(21%-100%) vs 40(25%-100%)] as well as the highest FiO 2 [40%(21%-100%), 30%(21%-100%) vs 40%(25%-100%)] were significantly lower in 2018 and 2019 than in 2017(all P<0.017). The proportion of infants receiving pure oxygen resuscitation decreased year by year [13.3% (11/83), 1.0% (1/102), 0.8% (1/133); χ2trend=17.719, P<0.001]. There was no statistical difference in radial artery blood gas analysis, the proportion of infants receiving pulmonary surfactant or mechanical ventilation, the highest value of percutaneous bilirubin, the incidence of hyperbilirubinemia, necrotizing enterocolitis, periventricular intraventricular hemorrhage, bronchopulmonary dysplasia, or mortality during hospitalization (all P>0.05). Conclusions:Resuscitation QI program helps reduce the incidence of hypothermia, increased the hemoglobin level after birth, and lessen the use of positive pressure ventilation and tracheal intubation in VLBWIs, without influencing the resuscitation effect or increasing the risk of short-term adverse outcomes.