Objective:To analyze the clinical characteristics of adult patients with phytosterolemia presenting with thrombocytopenia as the initial manifestation.Methods:A retrospective analysis was conducted on eight adult patients with phytosterolemia who visited Union Hospital, Tongji Medical College, Huazhong University of Science and Technology, from December 2020 to December 2023.Results:① The participants consisted of 2 (25%) male and 6 (75%) female patients, with a median age at diagnosis of 55 years (range: 29-66 years). The median duration from the discovery of thrombocytopenia to diagnosis was 10 years (range: 0.2-50 years). ② Compared with the normal control group (30 healthy adult volunteers) and the immune thrombocytopenia (ITP) control group (20 patients with ITP), patients with phytosterolemia exhibited significantly higher mean platelet volume and large platelet ratio. Peripheral blood smears revealed that the mean platelet diameter and the proportion of large platelets (diameter> 4 μm) were significantly higher in patients with phytosterolemia than those in the normal and ITP control groups ( P<0.01). ③ After a low-plant-sterol diet and ezetimibe treatment, five patients demonstrated decreased serum sitosterol and campesterol levels, increased hemoglobin concentration and platelet counts, and reduced platelet volume. Conclusion:Adult-onset phytosterolemia presenting with thrombocytopenia as the initial manifestation is prone to misdiagnosis. The presence of hemolytic anemia, splenomegaly, increased large platelets and schistocytes on peripheral blood smears, and xanthomas are crucial diagnostic indicators. Restricting dietary plant sterol intake and using ezetimibe to inhibit sterol absorption effectively lowers serum plant sterol levels and improves hematological abnormalities.

Objectives:To explore the relationship between plasma big endothelin-1(Big ET-1)level and left ventricular(LV)remodeling assessed by cardiac magnetic resonance(CMR)in patients with hypertrophic obstructive cardiomyopathy(HOCM).Methods:A total of 180 consecutive HOCM patients were enrolled from November 2013 to July 2015 in Fuwai Hospital,Chinese Academy of Medical Sciences.The baseline characteristics of the patients were collected and the plasma Big ET-1 levels were measured.According to LV remodeling index(LVRI),the patients were divided into severe remodeling group(LVRI>1.3 g/ml,n=119)and mild remodeling group(LVRI≤1.3 g/ml,n=61).The Pearson correlation analysis and multivariate logistic regression analysis were conducted to assess the correlations between plasma Big ET-1 level and indicators of LV remodeling.Results:Compared to the mild remodeling group,the plasma Big ET-1 level([0.84±0.30]pmol/L vs.[0.41±0.19]pmol/L),LVRI([1.7±0.3]g/ml vs.[1.1±0.1]g/ml),the extent of late gadolinium enhancement(LGE)([16.8±8.9]g vs.[7.0±6.6]g)were significantly higher in the severe remodeling group(all P<0.001).Pearson correlation analysis revealed that in patients with HOCM,plasma Big ET-1 level were positively correlated with left atrial anteroposterior diameter,interventricular septal thickness(IVS),LV mass(LVM),LVRI,LGE and LV outflow tract(LVOT)gradient and negatively correlated with LV ejection fraction(all P<0.05).Multivariate logistic regression analysis showed that LVM(OR=1.037,95% CI:1.019-1.056,P<0.001)and plasm Big ET-1 level(OR=11.461,95% CI:2.264-58.014,P=0.003)were the independent determinants for severe LV remodeling in HOCM patients.Conclusions:Among patients with HOCM,the plasma Big ET-1 level are markedly higher in those with severe LV remodeling and are positively associated with left atrial anteroposterior diameter,IVS,LVRI,LVM,LVOT gradient and LGE.Elevated Big ET-1 level and LVM are independent determinants of severe LV remodeling in HOCM patients.

Objectives:To explore the relationship between plasma big endothelin-1(Big ET-1)level and left ventricular(LV)remodeling assessed by cardiac magnetic resonance(CMR)in patients with hypertrophic obstructive cardiomyopathy(HOCM).Methods:A total of 180 consecutive HOCM patients were enrolled from November 2013 to July 2015 in Fuwai Hospital,Chinese Academy of Medical Sciences.The baseline characteristics of the patients were collected and the plasma Big ET-1 levels were measured.According to LV remodeling index(LVRI),the patients were divided into severe remodeling group(LVRI>1.3 g/ml,n=119)and mild remodeling group(LVRI≤1.3 g/ml,n=61).The Pearson correlation analysis and multivariate logistic regression analysis were conducted to assess the correlations between plasma Big ET-1 level and indicators of LV remodeling.Results:Compared to the mild remodeling group,the plasma Big ET-1 level([0.84±0.30]pmol/L vs.[0.41±0.19]pmol/L),LVRI([1.7±0.3]g/ml vs.[1.1±0.1]g/ml),the extent of late gadolinium enhancement(LGE)([16.8±8.9]g vs.[7.0±6.6]g)were significantly higher in the severe remodeling group(all P<0.001).Pearson correlation analysis revealed that in patients with HOCM,plasma Big ET-1 level were positively correlated with left atrial anteroposterior diameter,interventricular septal thickness(IVS),LV mass(LVM),LVRI,LGE and LV outflow tract(LVOT)gradient and negatively correlated with LV ejection fraction(all P<0.05).Multivariate logistic regression analysis showed that LVM(OR=1.037,95% CI:1.019-1.056,P<0.001)and plasm Big ET-1 level(OR=11.461,95% CI:2.264-58.014,P=0.003)were the independent determinants for severe LV remodeling in HOCM patients.Conclusions:Among patients with HOCM,the plasma Big ET-1 level are markedly higher in those with severe LV remodeling and are positively associated with left atrial anteroposterior diameter,IVS,LVRI,LVM,LVOT gradient and LGE.Elevated Big ET-1 level and LVM are independent determinants of severe LV remodeling in HOCM patients.

Objective:To analyze the clinical characteristics of adult patients with phytosterolemia presenting with thrombocytopenia as the initial manifestation.Methods:A retrospective analysis was conducted on eight adult patients with phytosterolemia who visited Union Hospital, Tongji Medical College, Huazhong University of Science and Technology, from December 2020 to December 2023.Results:① The participants consisted of 2 (25%) male and 6 (75%) female patients, with a median age at diagnosis of 55 years (range: 29-66 years). The median duration from the discovery of thrombocytopenia to diagnosis was 10 years (range: 0.2-50 years). ② Compared with the normal control group (30 healthy adult volunteers) and the immune thrombocytopenia (ITP) control group (20 patients with ITP), patients with phytosterolemia exhibited significantly higher mean platelet volume and large platelet ratio. Peripheral blood smears revealed that the mean platelet diameter and the proportion of large platelets (diameter> 4 μm) were significantly higher in patients with phytosterolemia than those in the normal and ITP control groups ( P<0.01). ③ After a low-plant-sterol diet and ezetimibe treatment, five patients demonstrated decreased serum sitosterol and campesterol levels, increased hemoglobin concentration and platelet counts, and reduced platelet volume. Conclusion:Adult-onset phytosterolemia presenting with thrombocytopenia as the initial manifestation is prone to misdiagnosis. The presence of hemolytic anemia, splenomegaly, increased large platelets and schistocytes on peripheral blood smears, and xanthomas are crucial diagnostic indicators. Restricting dietary plant sterol intake and using ezetimibe to inhibit sterol absorption effectively lowers serum plant sterol levels and improves hematological abnormalities.

Premature ovarian insufficiency(POI),also known as"ovarian insufficiency",has an incidence of 1％～5％.The incidence has been on the rise in recent years,seriously affecting women's physical and mental health and quality of life.At present,the cause and mechanisms of POI are still unclear,and the method and applications of model construction are also confusing.Most models have some shortcomings in pertinence and stability.The limitations greatly limit research into the clinical diagnosis and treatment of POI.This paper summarizes and discusses the etiology and pathogenesis of POI and the construction of POI animal models to provide a comprehensive reference for those studying POI.

Objective To explore the potential value of serum lipoxin A4 (LXA4) in monitoring bacterial load and anti-tuberculosis treatment progression in patients with pulmonary tuberculosis (PTB). Methods From January 2021 to January 2022, forty patients with active PTB, who were admitted to Shaanxi Provincial Tuberculosis Prevention and Control Hospital, were selected as the active PTB group, 38 patients with latent tuberculosis infection (LTBI) were selected as the LTBI group, and 28 healthy volunteers who underwent physical examination in our hospital during the same period were included as the healthy control group. The active PTB patients received a 2-month standard anti-tuberculosis chemotherapy, while the other two groups were untreated. Fasting venous blood was drawn from the three groups at enrollment (baseline), after 2 months of treatment, and upon the completion of 6 months of treatment in the active PTB group to measure serum LXA4 levels using enzyme-linked immunosorbent assay (ELISA). The relationship between serum LXA4 level and clinical manifestations, bacterial load, chest imaging manifestations, and treatment progress was analyzed. Results At baseline, serum LXA4 levels in the active PTB group, LTBI group, and healthy control group were [397.72 (210.68, 573.00)], [178.18 (108.17, 271.87)], and [131.06 (76.24, 166.04)] pg/mL, respectively. The levels in the active PTB and LTBI groups were significantly higher than those in the healthy control group, with statistical significance (P<0.01). According to the grading of acid-fast bacilli (AFB) sputum smears at diagnosis, baseline serum LXA4 level increased in the active PTB group with AFB sputum smear grade (P<0.001), and there was a positive correlation between serum LXA4 level and sputum smear grade (rs=0.209, P=0.003). After 6 months of treatment, the serum LXA4 level in the active PTB group was lower than the baseline value (P=0.002). The serum LXA4 level can predict treatment progress, with a baseline sensitivity of 55.0% (22/40), and after 6 months of treatment, 8 patients (20.0%) still showed positive serum LXA4 levels. Conclusions Serum LXA4 may be a useful biomarker for monitoring the progression of PTB treatment.

Objective To investigate the current status of nurses'knowledge,attitude and practice regarding sleep management of critically ill children in pediatric ICU,and to analyze its impact factors.Methods A self-designed questionnaire on general information and a questionnaire on knowledge and practical behaviors of pediatric ICU nurses on child's sleep management were used.In March 2023,902 pediatric ICU nurses from 24 hospitals in China were surveyed using a convenient sampling method,and the impact factors were analyzed using multiple stepwise linear regression.Results 893 valid questionnaires were collected and the recovery rate of valid questionnaires was 99.00％.Nurses in pediatric ICU scored(33.71±7.76)in knowledge dimension,(37.38±4.86)in attitude dimension and(80.60±16.78)in practice dimension,with a total score of(151.78±24.27).The scores of knowledge and attitude,knowledge and practice,attitude and practice are all positively correlated(r=0.393,P＜0.001;r=0.495,P＜0.001;r=0.320,P＜0.001).The results of multiple stepwise linear regression analysis showed that gender,region,whether they had received sleep management training were the influencing factors of pediatric ICU nurses'total score of knowledge,attitude and practice towards children's sleep management(P＜0.05).Conclusion Nurses in pediatric ICU are positive about sleep management for critically ill children,but their knowledge and practice levels need to improve.Nursing managers should strengthen the theoretical knowledge and practical behavioral training of pediatric ICU nurses on child sleep management,develop scientific sleep management plans,and guide nurses to make reasonable evaluation and interventions to improve children's sleep quality.

Defects in oral hard tissue caused by various factors have a negative impact on the functional and aesthetic results of prosthetic treatment. In recent years, the usage of bone tissue engineering for bone reconstruction has drawn widespread attention. Bone tissue engineering exhibits significant advantages, including the abundance of building materials and few side effects. In this paper, the composition and structure of dentin and its application in bone tissue engineering are reviewed, providing a new way to further optimize its performance. The results of a literature review show that the structure of dentin is very similar to that of autogenous bone. The inorganic component is mainly hydroxyapatite (HA), while the organic component is mainly collagen I, noncollagenous proteins (NCPs) and growth factors. Because of its unique composition, dentin can act as a scaffold and/or growth factor source through different processing methods. The deproteinization process removes most of the organic substances and creates a HA-based scaffold material with high porosity, which allows for vascularization and cellular infiltration. Demineralization increases dentin porosity by reducing the crystallinity of the mineralized components, so that part of HA, collagen fibers and growth factors are preserved. Demineralized dentin possesses various regulation functions ranging from differentiation, adhesion and proliferation of primitive cells and bone forming cell lineage. Extracted NCPs, as bioactive molecules, have been proved to play important roles that control cell differentiation, crystal nucleation and mineralization in bone formation. NCPs could be combined with variety of scaffold materials and modify their properties.

ABSTRACT OBJECTIVE：To study the metabolic transformation of total glycosides of Cistanche deserticola in artificial gastric and intestinal juice，and to speculate its metabolic transformation pathway in vivo. METHODS：UPLC/Q-TOF-MS was adopted. The determination was performed on ACQUITY UPLC BEH column with mobile phase consisted of 0.2％ formic acid water-acetonitrile（gradient elution）at the flow rate of 0.2 mL/min. The detection wavelength was set at 330 nm，and column temperature was 25 ℃. The ion source was electrospray ion source，and mass to charge ratio（m/z）was 50→1 000. In the positive and negative ion mode，the metabolic components of the total glycosides of C. deserticola in artificial gastric and intestinal juice were identified analysis，and combined with the literature，the metabolic pathway of total glycosides of C. deserticola in artificial gastric and intestinal juice was speculated. RESULTS：After the total glycosides of C. deserticola were metabolized by artificial gastric juice，and a total of 69 components were estimated，including 14 prototype components （such as Mustard aldehyde glucoside，daucosstorol） and 55 metabolic components （such as Methyl-O-Kankanoside J，Methyl-O-Kankanoside E），it is speculated that its metabolic pathways were methylation，demethylation，hydroxylation，methoxylation，acetylation，sulfation，and glucuronidation. After the total glycosides of C. deserticola were metabolized by artificial intestinal juice，a total of 90 components were estimated，including 4 prototype components（such as Kankanoside M，Kankanoside L）and 86 metabolic components（such as Methyl-O-Kankanoside， Methyl-O-Kankanoside E）. It was speculated that its metabolic pathways were methylation， demethylation，hydroxylation，dehydroxylation，methoxylation，acetylation，sulfation and glucuronidation. CONCLUSIONS：This study preliminarily speculates that the total glycosides of C. deserticola may be metabolized by methylation，demethylation， hydroxylation and other metabolic pathway in artificial gastrointestinal juice，which may provide reference for the in vivo metabolic transformation of total glycosides of C. deserticola.

Autogenous dentin is a promising biological material that can be used as a substitute for autologous bone. It has been used in postextraction site preservation, maxillary sinus floor elevation, and alveolar ridge augmentation. The clinical application methods of autologous dentin have showed great diversity without uniform standard. The present article reviewed the clinical application of autogenous dentin to provide new ideas for its future development. The literature review results show that dentin materials require several preparations before transplantation, among which the demineralization is a common chemical processing method. Demineralization can enhance the osteoconductive and osteoinductive properties of dentin, but the complex and time-consuming operation process has limited its application to a certain extent. Partial demineralization may be a more appropriate choice. During transplantation, the morphology of dentin depends on the condition of the bone defect and the surgical method. Granular materials with different diameters are convenient for filling irregular defects. Block materials are conducive to maintaining the space of the reconstruction site. Hollow frame materials are slightly more complicated to process but can combine the advantages of granular and block grafts. In addition to being used alone, dentin can also be transplanted in combination with multiple biological materials. Platelet-rich plasma combined with dentin materials has shown ideal results in clinical studies. Plaster of paris and calcium phosphate ceramics have also been combined with dentin materials in animal experiments. But since they have not been applied in humans, their clinical effects require further research.