Objective:To investigate the clinical characteristics of urea cycle disorder (UCD), the efficacy and safety of glyceryl phenylbutyrate (GPB) therapy in pediatric patients with UCD.Methods:This study was a retrospective, single-arm, multicenter clinical study. The clinical data of 20 pediatric patients with UCD who received GPB treatment at 9 hospitals nationwide between December 2021 and August 2024 were collected. The clinical manifestations, laboratory results, and molecular genetic characteristics were analyzed, ammonia levels and other laboratory results were evaluated pre-post GPB therapy by paired t-tests or Wilcoxon tests. Results:Among the 20 pediatric patients with UCD, there were 8 males and 12 females, and the onset age was 2.8 (1.4, 5.7) years. The ammonia levels were 174 (125, 342) μmol/L at first onset. The symptoms included vomiting in 6 cases, drowsiness in 5 cases, epilepsy in 5 cases, developmental delay in 5 cases, psychiatric and behavioral abnormalities in 3 cases, and lethargy in 1 case, and 18 cases exhibited abnormal liver function. Twenty cases included 6 UCD subtypes, with 11 cases being ornithine transcarbamylase deficiency. A total of 27 variants were identified, 11 (41%) of which were novel. The age of patients who began GPB therapy was 4.0 (1.5, 6.6) years. Ten cases stopped GPB after 4.2 (3.4, 5.3) months, with 4 patients undergoing liver transplantation and 6 discontinuing for financial reasons. The remaining ten patients continued GPB therapy for 11.6 (8.6, 14.0) months. The duration of GPB treatment was 6.0 (4.2, 12.3) months, at the final visit, the levels of ammonia, platelets and aspartate aminotransferase were lower compared to those of pre-treatment (all P<0.05). The serum albumin level was higher than that of pre-treatment ( P=0.016). Two patients suffered only one episode of acute hyperammonaemia, with ammonia levels of 232 and 141 μmol/L, respectively. Nine cases experienced adverse effects potentially related to GPB, decreased appetite in 6 cases, vomiting in 3 cases, abnormal skin oil odor in 2 cases, somnolence, fatigue and diarrhea each in 1 case, with symptoms improved within 6 (3, 10) days. Conclusions:UCD primarily manifests with neurological and gastrointestinal symptoms, and early diagnosis of UCD could be achieved through the analysis of ammonia. GPB may effectively reduce ammonia levels in UCD pediatric patients, with favorable safety and tolerability.

Objective To explore the application of bundled nursing care for recurrent obstruc-tive esophageal cancer treated by photodynamic therapy.Methods Thirty patients with recurrent ob-structive esophageal cancer were administered with photosensitizer hematoporphyrin derivative injec-tion.After 24 hours,an optical fiber was introduced under endoscopic guidance,and 630 nm laser was used to irradiate the tumor locally.The degree of relief in dysphagia,improvement in perform-ance status,changes in body mass index,and treatment-related adverse reactions were recorded.Bundled nursing strategies were implemented,including preoperative assessment,education,prepara-tion,postoperative positioning,observation,prevention of complications,and light protection meas-ures.Results After treatment,the median diameter of the narrowest esophageal lesion was increased[(8.92±0.64)mm versus(4.77±0.60)mm],the Karnofsky Performance Status(KPS)score was improved[(77.69±5.99)versus(84.62±6.60)],BMI was increased[(17.17±1.66)kg/m2 ver-sus(18.08±1.60)kg/m2],and the Stooler dysphagia grade was decreased compared with treatment be-fore.The main treatment-related adverse reactions were retrosternal pain and fever.Conclusion Photo-dynamic therapy for recurrent obstructive esophageal cancer has a rapid onset of action and mild ad-verse reactions,and ensures the smooth implementation of PDT and patients'safety.

Objective:To investigate the clinical characteristics of urea cycle disorder (UCD), the efficacy and safety of glyceryl phenylbutyrate (GPB) therapy in pediatric patients with UCD.Methods:This study was a retrospective, single-arm, multicenter clinical study. The clinical data of 20 pediatric patients with UCD who received GPB treatment at 9 hospitals nationwide between December 2021 and August 2024 were collected. The clinical manifestations, laboratory results, and molecular genetic characteristics were analyzed, ammonia levels and other laboratory results were evaluated pre-post GPB therapy by paired t-tests or Wilcoxon tests. Results:Among the 20 pediatric patients with UCD, there were 8 males and 12 females, and the onset age was 2.8 (1.4, 5.7) years. The ammonia levels were 174 (125, 342) μmol/L at first onset. The symptoms included vomiting in 6 cases, drowsiness in 5 cases, epilepsy in 5 cases, developmental delay in 5 cases, psychiatric and behavioral abnormalities in 3 cases, and lethargy in 1 case, and 18 cases exhibited abnormal liver function. Twenty cases included 6 UCD subtypes, with 11 cases being ornithine transcarbamylase deficiency. A total of 27 variants were identified, 11 (41%) of which were novel. The age of patients who began GPB therapy was 4.0 (1.5, 6.6) years. Ten cases stopped GPB after 4.2 (3.4, 5.3) months, with 4 patients undergoing liver transplantation and 6 discontinuing for financial reasons. The remaining ten patients continued GPB therapy for 11.6 (8.6, 14.0) months. The duration of GPB treatment was 6.0 (4.2, 12.3) months, at the final visit, the levels of ammonia, platelets and aspartate aminotransferase were lower compared to those of pre-treatment (all P<0.05). The serum albumin level was higher than that of pre-treatment ( P=0.016). Two patients suffered only one episode of acute hyperammonaemia, with ammonia levels of 232 and 141 μmol/L, respectively. Nine cases experienced adverse effects potentially related to GPB, decreased appetite in 6 cases, vomiting in 3 cases, abnormal skin oil odor in 2 cases, somnolence, fatigue and diarrhea each in 1 case, with symptoms improved within 6 (3, 10) days. Conclusions:UCD primarily manifests with neurological and gastrointestinal symptoms, and early diagnosis of UCD could be achieved through the analysis of ammonia. GPB may effectively reduce ammonia levels in UCD pediatric patients, with favorable safety and tolerability.

Objective:To explore the effects of practice teaching based on Gagne's teaching theory combined with scene teaching method on core competency of nursing students in department of respiratory and critical care medicine.Methods:A total of 42 nursing students from the Respiratory and Critical Care Medicine Department of Beijing Jishuitan Hospital were enrolled and divided into observation group (21 cases) and control group (21 cases) using a random number table. Traditional teaching method was used in the control group, while Gagne's teaching theory combined with scene teaching method was used for teaching the observation group. After teaching, theoretical knowledge and actual operation ability of nursing students were assessed. The core competency of nursing students was evaluated by core competency assessment scale. The independent learning ability of nursing students was evaluated using an assessment tool. The self-evaluation of teaching effect in the two groups was compared. The t test or chi-square test was performed in SPSS 22.0. Results:The scores of theoretical knowledge and actual operation ability in the observation group were (88.10±7.52) and (90.81±4.88), respectively. These scores were significantly higher than those in the control group [(83.62±6.58) and (72.57±8.94), t=2.06, 8.21, P<0.05]. The scores of core competency and independent learning ability were significantly higher in the observation group than in the control group [(185.53±20.19) and (129.09±16.14) vs. (170.52±20.32) and (105.86±12.23), t=2.40, 5.10, P<0.05]. The score of self-evaluation on teaching effect was significantly higher in the observation group than in the control group [28 (25, 29) vs. 21 (20, 22), Z=5.39, P<0.05]. Conclusion:Gagne's teaching theory combined with scene teaching method in practice teaching of nursing students in the department of respiratory and critical care medicine should be promoted because this combined method improves the mastery of theoretical knowledge and actual operation skills, the independent learning ability and core competency of nursing students, and the effect of nursing teaching.

【Objective】 To explore the clinical features, treatment and prognosis of paraganglioma of the urinary bladder (PUB). 【Methods】 The clinical data of 41 PUB patients treated at our hospital during Sep.2012 and Sep.2022 were collected. The clinical features, surgical records, pathological reports and follow-up records were retrospectively analyzed. Patients’ survival was estimated with Kaplan-Meier estimator. The differences among groups were compared with Log-rank test. 【Results】 Among the 41 patients, 20 were male and 21 were female, with a median age of 52 years. All patients were treated with surgery, including transurethral resection of bladder tumor (TURBT) in 16 cases, partial cystectomy (PC) in 23 cases, and radical cystectomy (RC) in 2 cases. All patients were followed up for 4.0 to 125.0 months, with a median of 59.0 months. Local recurrence occurred in 5 patients, and distant metastasis occurred in 5 patients. Survival analysis showed that the 5-year overall survival (OS) rate and 5-year relapse-free survival (RFS) rate were 95.7% and 84.8%, respectively. Further analysis showed statistically significant differences in OS and RFS among groups with different maximum tumor diameters, growth patterns, and Ki-67 expressions (P<0.05). For patients with a maximum tumor diameter ≤2.8 cm, there was no significant difference in OS and RFS among different surgical groups. 【Conclusion】 PUB is rare, and a definitive diagnosis is based on pathology. In addition, the main treatment is surgery and the prognosis is good.

BACKGROUND: Hypertrophic cardiomyopathy (HCM) is an underdiagnosed genetic heart disease worldwide. The management and prognosis of obstructive HCM (HOCM) and non-obstructive HCM (HNCM) are quite different, but it also remains challenging to discriminate these two subtypes. HCM is characterized by dysmetabolism, and myocardial amino acid (AA) metabolism is robustly changed. The present study aimed to delineate plasma AA and derivatives profiles, and identify potential biomarkers for HCM. METHODS: Plasma samples from 166 participants, including 57 cases of HOCM, 52 cases of HNCM, and 57 normal controls (NCs), who first visited the International Cooperation Center for HCM, Xijing Hospital between December 2019 and September 2020, were collected and analyzed by high-performance liquid chromatography-mass spectrometry based on targeted AA metabolomics. Three separate classification algorithms, including random forest, support vector machine, and logistic regression, were applied for the identification of specific AA and derivatives compositions for HCM and the development of screening models to discriminate HCM from NC as well as HOCM from HNCM. RESULTS: The univariate analysis showed that the serine, glycine, proline, citrulline, glutamine, cystine, creatinine, cysteine, choline, and aminoadipic acid levels in the HCM group were significantly different from those in the NC group. Four AAs and derivatives (Panel A; proline, glycine, cysteine, and choline) were screened out by multiple feature selection algorithms for discriminating HCM patients from NCs. The receiver operating characteristic (ROC) analysis in Panel A yielded an area under the ROC curve (AUC) of 0.83 (0.75-0.91) in the training set and 0.79 (0.65-0.94) in the validation set. Moreover, among 10 AAs and derivatives (arginine, phenylalanine, tyrosine, proline, alanine, asparagine, creatine, tryptophan, ornithine, and choline) with statistical significance between HOCM and HNCM, 3 AAs (Panel B; arginine, proline, and ornithine) were selected to differentiate the two subgroups. The AUC values in the training and validation sets for Panel B were 0.83 (0.74-0.93) and 0.82 (0.66-0.98), respectively. CONCLUSIONS The plasma AA and derivatives profiles were distinct between the HCM and NC groups. Based on the differential profiles, the two established screening models have potential value in assisting HCM screening and identifying whether it is obstructive.
Humans ; Amino Acids ; Cysteine ; Cardiomyopathy, Hypertrophic/diagnosis* ; Biomarkers ; Proline ; Arginine ; Ornithine ; Glycine ; Choline

Medoggreenpit-viper bites are extremely rare in northern China. This article analyzed the clinical data, laboratory test results, treatment methods and the outcome of a case of poisoning from the Medoggreenpit-viper snake bite in the northern area. The main clinical manifestations of the patient were local swelling and bleeding. The wound was debrided immediately after the patient was admitted to the hospital, and vacuum suction was given after the debridement. After been treated with anti-Agkistrodon venom serum, anti-Gyrus serpent, anti-infection, organ protection, fluid replacement and diuresis, the patient recovered and was discharged from the hospital. The patients were generally in good condition after follow-up. This case suggested that regional poisoning treatment centers in the northern region should stock various anti-venom serums. At the same time, it is necessary to popularize knowledge of standard treatment of snake bites.

Medoggreenpit-viper bites are extremely rare in northern China. This article analyzed the clinical data, laboratory test results, treatment methods and the outcome of a case of poisoning from the Medoggreenpit-viper snake bite in the northern area. The main clinical manifestations of the patient were local swelling and bleeding. The wound was debrided immediately after the patient was admitted to the hospital, and vacuum suction was given after the debridement. After been treated with anti-Agkistrodon venom serum, anti-Gyrus serpent, anti-infection, organ protection, fluid replacement and diuresis, the patient recovered and was discharged from the hospital. The patients were generally in good condition after follow-up. This case suggested that regional poisoning treatment centers in the northern region should stock various anti-venom serums. At the same time, it is necessary to popularize knowledge of standard treatment of snake bites.

To investigate the detection threshold of Treponema pallidum specific antibody method by chemiluminescent immunoassay (CLIA) in Siemens ADVIA Centaur XP for Syphilis serological test, and compare with the results derived from CMIA, TP-WB and TPPA method. The result can serve as reference for the application of CLIA. In total 30 887 samples screened by Treponema pallidum specific antibody method were collected by Abbott architect i2000 CMIA from July 2018 to July 2019 in Yanda Hospital of Hebei Province. We selected 153 patients with the ratio of sample absorbance to critical value (S/CO) of 1-9 by CMIA screening of Treponema pallidum specific antibody as the research objects. The reverse sequence of syphilis serological detection was adopted, and TP-WB and TPPA were used as the confirmation methods respectively. MedCalc software was used to analyze the results of ROC curve, and the cut-off value was obtained. Chi square test was used to test the difference significance of counting data. The detection results of Treponema pallidum specific antibody in the same batch of serum samples were unequal by different methods. There was no significant difference between CLIA method and TPPA method, but significant difference between CLIA method with TP-WB method and CMIA method was found. TPPA test results and TP-WB test results were taken as gold standards, ROC curve analysis showed that the best diagnostic cutoff value of CLIA method was 4.01 and 16.06, respectively, and the area under the curve was 0.961 and 0.838. The suggested cutoff value of CLIA method is quite different when using different syphilis serological test methods as the gold standard, Therefore, when the S/CO value determined by CLIA is between 1.00 to 16.06, TP-WB method should be recommended as the first choice in laboratory serological test for recheck and confirmation to avoid clinical misdiagnosis.

Critical ultrasonography(CUS) is different from the traditional diagnostic ultrasound,the examiner and interpreter of the image are critical care medicine physicians.The core content of CUS is to evaluate the pathophysiological changes of organs and systems and etiology changes.With the idea of critical care medicine as the soul,it can integrate the above information and clinical information,bedside real-time diagnosis and titration treatment,and evaluate the therapeutic effect so as to improve the outcome.CUS is a traditional technique which is applied as a new application method.The consensus of experts on critical ultrasonography in China released in 2016 put forward consensus suggestions on the concept,implementation and application of CUS.It should be further emphasized that the accurate and objective assessment and implementation of CUS requires the standardization of ultrasound image acquisition and the need to establish a CUS procedure.At the same time,the standardized training for CUS accepted by critical care medicine physicians requires the application of technical specifications,and the establishment of technical specifications is the basis for the quality control and continuous improvement of CUS.Chinese Critical Ultrasound Study Group and Critical Hemodynamic Therapy Collabration Group,based on the rich experience of clinical practice in critical care and research,combined with the essence of CUS,to learn the traditional ultrasonic essence,established the clinical application technical specifications of CUS,including in five parts:basic view and relevant indicators to obtain in CUS;basic norms for viscera organ assessment and special assessment;standardized processes and systematic inspection programs;examples of CUS applications;CUS training and the application of qualification certification.The establishment of applied technology standard is helpful for standardized training and clinical correct implementation.It is helpful for clinical evaluation and correct guidance treatment,and is also helpful for quality control and continuous improvement of CUS application.