Objective To analyze the clinical and cardiac magnetic resonance(CMR)characteristics of patients with Fabry disease(FD)and evaluate the application value of CMR in the early diagnosis of cardiac involvement in FD.Methods This retrospective study involved nine patients with FD confirmed by renal biopsy pathology and genetic testing at our hospital between January 2021 and October 2024.Their clinical baseline data,laboratory test reuslts,and CMR images were collected.CMR images were analyzed using CVI42 software to generate functional,morphological,and structural parameters.Results In this study,78％of the patients were male,and a high proportion(67％)had a family history of FD.Electrocardiographic abnormalities were observed in eight patients(89％),while 33％reported acroparesthesia and 22％exhibited cornea verticillata,a characteristic ocular manifestation of FD.Left ventricular hypertrophy was present in four patients,with one case also showing right ventricular hypertrophy.Late gadolinium enhancement(LGE)was positive in one patient,presenting as intermural enhancement.The mean left ventricular ejection fraction(LVEF)was within the normal range.T1 mapping demonstrated that both global and segmental native T1 values in the left ventricular myocardium were below 1 200 ms.Conclusion Multimodal CMR imaging provides crucial imaging evidence for the diagnosis of FD,with native T1 mapping showing significant clinical potential for disease staging.

Objective To analyze the clinical and cardiac magnetic resonance(CMR)characteristics of patients with Fabry disease(FD)and evaluate the application value of CMR in the early diagnosis of cardiac involvement in FD.Methods This retrospective study involved nine patients with FD confirmed by renal biopsy pathology and genetic testing at our hospital between January 2021 and October 2024.Their clinical baseline data,laboratory test reuslts,and CMR images were collected.CMR images were analyzed using CVI42 software to generate functional,morphological,and structural parameters.Results In this study,78％of the patients were male,and a high proportion(67％)had a family history of FD.Electrocardiographic abnormalities were observed in eight patients(89％),while 33％reported acroparesthesia and 22％exhibited cornea verticillata,a characteristic ocular manifestation of FD.Left ventricular hypertrophy was present in four patients,with one case also showing right ventricular hypertrophy.Late gadolinium enhancement(LGE)was positive in one patient,presenting as intermural enhancement.The mean left ventricular ejection fraction(LVEF)was within the normal range.T1 mapping demonstrated that both global and segmental native T1 values in the left ventricular myocardium were below 1 200 ms.Conclusion Multimodal CMR imaging provides crucial imaging evidence for the diagnosis of FD,with native T1 mapping showing significant clinical potential for disease staging.

Objective:To evaluate the intervention effects of aquatic therapeutic exercise on functioning and quality of life for children and youth with cerebral palsy within framework of World Health Organization Family International Classifications using systematic review. Methods:Literatures were retrieved and reviewed from the databases of Cochrane Library, PEDro, PubMed, EMBASE, Web of Knowledge, Web of Science, OVID, EBSCO, CMCI, CNKI, Wangfang and VIP until May, 2020. The randomized controlled trials (RCTs) about aquatic therapeutic exercise for children and youth with cerebral palsy for functioning were systematically reviewed. The data were analyzed with RevMan 5.3. Results:Nine RCTs were selected, including 162 children and youth. The score of Gross Motor Function Measure improved significantly after aquatic exercise (WMD = 6.31, 95 %CI 2.57 to 10.06, P < 0.001), as well as daily activities and quality of life. Conclusion:Aquatic therapeutic exercise can help improve the gross motor function of children and youth with cerebral palsy, and has positive effects on function of activity and quality of life.

Objective: To explore the potential clinical risk factors related to abnormal microstructure of brain gray matter in end-stage renal disease (ESRD) patients with cognitive dysfunction. Methods: 3D high-resolution structural imaging was applied in 34 ESRD patients before hemodialysis and 34 health controls. We analyzed the characteristics of changes in gray matter cortical thickness in patients with ESRD before the first dialysis by surface based morphology (SBM) analysis. Further Pearson correlation analysis was used to reveal the relationship of the changed cortical thickness with the laboratory test indicators and clinical scale scores in patient group, with the covariants of age, sex and education removed. Results: Compared with that in control group, the broad cortex thickness decreases were observed in patients with ESRD before the first dialysis (P<0.01, FDR corrected), mainly including the bilateral postcentral gyrus, bilateral precentral gyrus, bilateral inferior parietal lobe (IPL), bilateral precuneus and some frontal and temporal lobes. Pearson correlation analysis showed that the cortical thickness in the IPL and precuneus had significantly negative correlation with uric acid level (r=-0.543, P<0.01) and the decreased thickness of bilateral IPL was positively related to AVL-IR score (right r=0.522, left r=0.649, P<0.01). Conclusion: ESRD patients showed extensive cortical thickness reduction before dialysis initiation, which corrected with overall cognition and immediate memory dysfunction. High uric acid level was the major factor of morphlolgical changes in ESRD patients before dialysis initiation.

Objective: To investigate the changes of insular functional connectivity in patients with uremic restless legs syndrome (uRLS) using seed-based resting-state functional connectivity analysis so as to explore the related risk factors for RLS. Methods: 3D high-resolution structural imaging was applied in 29 patients with uremic restless legs (uRLS group) and 29 healthy volunteers (control group) with maintenance hemodialysis. The greymatter volume of the insular cortex was assessed using a voxel-based morphometry (VBM) analysis and functional connectivity of insular variation was evaluated by a seed-based correlation analysis, while the seed was chosen as the significant difference of grey matter volume. Pearson correlation analysis was used to explore the significant correlation between functional connectivity and RLS score. The relationship between brain alterations and clinical variables were investigated with Logistic stepwise multiple regression analysis. Results: Compared with that in the control group, the area with reduced gray matter volume in the uRLS group was located on the left posterior insula (P<0.05, FWE correction). Taking the left post insular as a seed region, we further found reduced functional connectivity in patients mainly between the insular and primary sensorimotor cortex (S1), supplementary motor areas (SMA), and posterior mid-cingulate gyrus (P<0.05, FDR corrected). In uRLS group, the severity score of RLS was negatively correlated with insular-S1 functional connectivity (P<0.05, FDR corrected), while the hemoglobin level was negetively correlated with functional connectivity degree of insular-S1 (r=-0.449, P=0.015, FDR corrected). Conclusion: The altered morphology of the post insular cortex was revealed in uRLS patients. Abnormal sensorimotor activities in patients with RLS were closely associated with reduced functional connectivity of insular-S1. Low hemoglobin level was the most important risk factor for the abnormal functional connectivity of the brain.

With the development of critical care ultrasound technology, clinics begins to pay attention to the role of critical care ultrasound in nursing of critically ill patients. The increasingly extensive application of ultrasound in the field of critical care provides a visual tool for clinical nursing work, which can qualitatively and quantitatively evaluate nursing indicators, help to solve the existing nursing problems and nursing difficulties and promote the improvement of critical care quality and the development of nursing academics. To this end, experts formulates this consensus based on years of experience and application of intensive care ultrasound practice.

Objective::To assess the clinical features of fetal growth restriction (FGR) in women with hypertensive disorders of pregnancy in China.Methods::This is a retrospective cohort study. The clinical data of 4 451 women with hypertensive disorders of pregnancy were retrospectively collected from 11 tertiary hospitals across ten provinces in China during January 2015 to December 2015. The mean maternal age was (31.0±5.4) years old. Participants were divided into FGR group ( n= 670) and non-FGR group ( n= 3 781). The incidence and clinical features of FGR, and its correlation with gestational age, previous FGR history, 24-hour urinary protein excretion, and hemolysis, elevated liver enzyme and low platelet count (HELLP) syndrome were analyzed. Student’s t-test and Chi-square test were used when comparing clinical features between FGR and non-FGR groups. Results::The overall incidence of FGR was 15.1% (670/4 451). The FGR incidence was 22.4% (433/1 937) in women with severe preeclampsia and 18.6% (68/365) in women with chronic hypertension with superimposed preeclampsia, respectively. FGR was more prevalent in women who had preterm births than those who had term births (22.8% (432/1 898) vs. 9.3% (238/2 553), P < 0.001). It was also more prevalent in women with early-onset preeclampsia than those with late-onset preeclampsia (18.4% (189/1 025) vs. 14.0% (481/3 426), P= 0.001). Women with a previous FGR history had a significantly higher FGR incidence than those without an FGR history (66.7% (4/6) vs. 15.7% (250/1 596), P= 0.007). The presence of abnormal results of the umbilical artery Doppler (13% (87/670) vs. 2.4% (89/3 781), P < 0.001) and the middle cerebral artery Doppler (3.3% (22/670) vs. 0.4% (15/3 781), P < 0.001) was higher in the FGR group compared with the non-FGR group, while the presence of increased uterine artery resistance was not statistically different (1.5% (10/670) vs. 0.8% (29/3 781), P= 0.072). The FGR group delivered earlier than the non-FGR group ((35.3±3.0) weeks vs. (36.4±4.3) weeks, P < 0.001) with lower birth weight (1 731.0±574.5) g vs. (2 753.9±902.1) g, P < 0.001, higher fetal or neonatal death (9.4% (63/670) vs. 4.2% (157/3 781), P < 0.001), and higher cesarean section rate (82.5% (553/670) vs. 70.2% (2 656/3 781), P < 0.001). In the FGR group, more neonates had 5-minute Apgar score ≤7 (7.9% (53/670) vs. 3.9% (149/3 780), P < 0.001), with higher neonatal intensive care unit admission rate (48.1 % (322/670) vs. 23.3% (881/3 781), P < 0.001). More cases of HELLP syndrome occurred in the FGR group (6.9% (46/670) vs. 3.2% (122/3 781), P < 0.001). Women with FGR had heavier 24-hour urinary protein excretion than those without FGR ((3.9±3.7) g vs. (3.1±4.2) g, P= 0.005). Conclusion::In pregnancies with hypertensive disorders, increased risks of FGR are associated with preterm birth, birth before 34 weeks, and a previous FGR history. FGR is related to higher occurrence of abnormal uterine artery Doppler and umbilical artery Doppler. When hypertensive disorders is complicated by FGR, there appears to be higher maternal morbidity including higher rate of HELLP syndrome, cesarean section, and heavier proteinuria, as well as worse neonatal outcomes.

Objective::To assess the clinical features of fetal growth restriction (FGR) in women with hypertensive disorders of pregnancy in China.Methods::This is a retrospective cohort study. The clinical data of 4 451 women with hypertensive disorders of pregnancy were retrospectively collected from 11 tertiary hospitals across ten provinces in China during January 2015 to December 2015. The mean maternal age was (31.0±5.4) years old. Participants were divided into FGR group ( n= 670) and non-FGR group ( n= 3 781). The incidence and clinical features of FGR, and its correlation with gestational age, previous FGR history, 24-hour urinary protein excretion, and hemolysis, elevated liver enzyme and low platelet count (HELLP) syndrome were analyzed. Student’s t-test and Chi-square test were used when comparing clinical features between FGR and non-FGR groups. Results::The overall incidence of FGR was 15.1% (670/4 451). The FGR incidence was 22.4% (433/1 937) in women with severe preeclampsia and 18.6% (68/365) in women with chronic hypertension with superimposed preeclampsia, respectively. FGR was more prevalent in women who had preterm births than those who had term births (22.8% (432/1 898) vs. 9.3% (238/2 553), P < 0.001). It was also more prevalent in women with early-onset preeclampsia than those with late-onset preeclampsia (18.4% (189/1 025) vs. 14.0% (481/3 426), P= 0.001). Women with a previous FGR history had a significantly higher FGR incidence than those without an FGR history (66.7% (4/6) vs. 15.7% (250/1 596), P= 0.007). The presence of abnormal results of the umbilical artery Doppler (13% (87/670) vs. 2.4% (89/3 781), P < 0.001) and the middle cerebral artery Doppler (3.3% (22/670) vs. 0.4% (15/3 781), P < 0.001) was higher in the FGR group compared with the non-FGR group, while the presence of increased uterine artery resistance was not statistically different (1.5% (10/670) vs. 0.8% (29/3 781), P= 0.072). The FGR group delivered earlier than the non-FGR group ((35.3±3.0) weeks vs. (36.4±4.3) weeks, P < 0.001) with lower birth weight (1 731.0±574.5) g vs. (2 753.9±902.1) g, P < 0.001, higher fetal or neonatal death (9.4% (63/670) vs. 4.2% (157/3 781), P < 0.001), and higher cesarean section rate (82.5% (553/670) vs. 70.2% (2 656/3 781), P < 0.001). In the FGR group, more neonates had 5-minute Apgar score ≤7 (7.9% (53/670) vs. 3.9% (149/3 780), P < 0.001), with higher neonatal intensive care unit admission rate (48.1 % (322/670) vs. 23.3% (881/3 781), P < 0.001). More cases of HELLP syndrome occurred in the FGR group (6.9% (46/670) vs. 3.2% (122/3 781), P < 0.001). Women with FGR had heavier 24-hour urinary protein excretion than those without FGR ((3.9±3.7) g vs. (3.1±4.2) g, P= 0.005). Conclusion::In pregnancies with hypertensive disorders, increased risks of FGR are associated with preterm birth, birth before 34 weeks, and a previous FGR history. FGR is related to higher occurrence of abnormal uterine artery Doppler and umbilical artery Doppler. When hypertensive disorders is complicated by FGR, there appears to be higher maternal morbidity including higher rate of HELLP syndrome, cesarean section, and heavier proteinuria, as well as worse neonatal outcomes.

Objective To study neuromechanism for uremic restless syndrome (URLS),functional MRI (fMRI) analysis and were used to explor main activity.Methods Resting state functional magnetic resonance imaging (MRI) was performed on 29 patients with uremic restless legs syndrome and 25 healthy controls.The values of the regional homogeneity (ReHo) of the two groups were calculated and analyzed statistically.Result As compared with the control group,patients with uremic restless legs syndrome showed emotional and mental abnormality.Increased ReHo values in bilateral supplementary motor area,bilateral thalamus,left insular lobe,left hippocampus (P＜0.05),and decreased ReHo values in anterior cingulate gyrus,bilateral posterior central gyrus,right amygdala were found in patients with uremic restless legs syndrome (P＜0.05).The severity score of RLS was respectively positively correlated with dialysis duration (r =0.57,P =0.002),PTH level (r =0.419,P =0.033)BAI (r =0.528,P=0.006),and BDI (r =0.567,P =0.003).Conclusion Neuronal activity in related brain area in patients with uremic restless legs syndrome were found.This abnormality provides an objective diagnostic basis for the explanation of restless legs syndrome in maintenance hemodialysis patients.

Objective To evaluate the effect of endoscopic management of foreign bodies in the upper digestive tract. Methods Clinical data and endoscopic treatment methods of 41 patients were retrospectively analyzed from October 2014 to May 2016. Patients with incomplete medical records were excluded. Results Foreign bodies in the upper digestive tract occurred high frequency in elderly. 53.6% of the foreign bodies were located in the esophagus. Date stones was the main type of foreign bodies (56.1%). 41 cases with foreign bodies in digestive tract were successfully extracted, while 1 case occurred perforation. Conclusion Endoscopic management of gastrointestinal foreign bodies is safe and effective.