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BACKGROUND: Zhu-Tokita-Takenouchi-Kim syndrome (ZTTK syndrome) is a severe multi-systemic developmental disorder, caused by variants in the SON gene. A patient diagnosed with ZTTK syndrome who carried a de novo SON mutation and exhibited recurrent myocardial injury was described in this case. CASE PRESENTATION: A 7-year-old girl was admitted to the Cardiology Department of Beijing Children's Hospital in November 2019 due to myocardial injury following respiratory infection. She displayed elevated myocardial enzymes and severe T-wave changes on electrocardiogram. Over the past three years, she had experienced myocardial injury on three occasions. Additionally, she exhibited intellectual disability, congenital amblyopia, and dysmorphic facial features. Genetic analysis revealed a de novo heterozygous mutation c.3852_3856delGGTAT in the SON gene, which was confirmed by Sanger sequencing of her parents. She received anti-infection treatment and was administered metoprolol orally. Her condition was stable at the time of discharge. Over a 42-month follow-up period at the outpatient clinic, she complained intermittent fatigue and palpitation. CONCLUSIONS: The identified SON mutation, which plays a crucial role in heart development and mitochondrial function, may be associated with an increased susceptibility to myocardial injury or cardiomyopathy. This case report contributes novel insights into this rare condition and suggests the expansion of the ZTTK syndrome phenotype.
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Anormalidades do Olho , Deficiência Intelectual , Criança , Feminino , Humanos , Deficiência Intelectual/genética , Mutação , Heterozigoto , Fenótipo , Arritmias CardíacasRESUMO
Neonatal arrhythmias are significant contributors to infant mortality. Timely diagnosis and treatment are essential for neonates with non-benign arrhythmias to avoid severe complications, and ongoing treatment and follow-up are sometimes needed. The main objective of this study will be to understand the incidence and demographic characteristics of arrhythmias in hospitalized neonates in China and the related factors of outcomes. A secondary objective will be to establish the first follow-up system for neonatal arrhythmias in China. The medical burdens of neonatal arrhythmias in China will also be investigated. The data from the Futang Research Center of Pediatric Development (FRCPD) database between January 2016 and December 2021 were obtained. Newborns admitted to member hospitals with a discharge diagnosis of "neonatal arrhythmia" (ICD-10 code P29.151) or "arrhythmia" (ICD-10 code I49.904) were included. The medical record information was collected and classified into two groups: heart failure and non-heart failure. The differences between the two groups and independent risk factors for neonatal arrhythmias complicated with heart failure were analyzed. In addition, a follow-up study of patients discharged from Beijing Children's Hospital was conducted to evaluate their outcomes at the age of 3 years old. Factors influencing hospitalization costs were analyzed using rank-sum tests and multiple linear regression. It is anticipated that the study findings will provide new and comprehensive data on the health needs of neonatal arrhythmias in China. The study will establish the first follow-up system for neonatal arrhythmias in China. This study will help reduce the burden of patients and their families as well as the society.
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Insuficiência Cardíaca , Hospitais , Lactente , Humanos , Recém-Nascido , Criança , Pré-Escolar , Seguimentos , Estudos Retrospectivos , China/epidemiologia , Arritmias Cardíacas/epidemiologia , Arritmias Cardíacas/terapiaRESUMO
We aimed to analyse the clinical characteristics of children with different types of anomalous aortic origin of the coronary artery (AAOCA) at different ages, and to discuss the factors related to myocardial ischaemia. In this retrospective study, we included 69 children diagnosed with AAOCA using CT coronary angiography; we classified the participants based on the type of AAOCA, age, and high-risk anatomy. The clinical characteristics of the different AAOCA types and age groups were compared, and the correlation between manifestations and high-risk anatomy was analysed. Anomalous origin of the left coronary artery from the right coronary artery sinus, anomalous origin of the right coronary artery from the left coronary artery sinus, and a coronary artery origin without coronary sinuses was found in 10 (14.5%), 57 (82.6%), 2 (2.9%) patients, respectively. There were no significant differences in sex, clinical manifestations, percentage of positive myocardial injury markers, electrocardiogram, transthoracic echocardiography, or proportion of high-risk anatomy among the groups with different AAOCA types. According to age group, the proportion of asymptomatic infants and pre-schoolers was the highest (p < 0.001). Forty-three patients (62.3%) had high-risk anatomy and were more likely to present with severe symptoms and cardiac syncope (p < 0.05). Conclusion: There were no significant differences in the proportions of high-risk anatomy and clinical characteristics among children with different AAOCA types. We found a relation between the severity of AAOCA clinical symptoms and anatomical risk. What is Known: ⢠Clinical symptoms in children with AAOCA are varied and the results of routine cardiological examinations lack specificity. ⢠High-risk anatomical features, exercise, cardiac symptoms, and ALCA are risk factors for the occurrence of SCD in patients with AAOCA. What is New: ⢠Compared the clinical characteristics of different types of AAOCA and ages. ⢠Analysed the correlation between symptoms and high-risk anatomical features.
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BACKGROUND: To investigate the clinical manifestations, prognosis, and possibly related genes of anomalous right coronary artery originating from the aorta (ARCA-L) in children. METHODS: This case series study included pediatric patients diagnosed with ARCA-L at the Department of Cardiology in Beijing Children's Hospital affiliated to Capital Medical University, between January 2017 and December 2019. RESULTS: Nine pediatric patients (aged 3 months to 12 years, 4 boys) were included. Two cases presented with cardiac insufficiency as their primary manifestation, while the remaining seven had post-infection or post-exercise symptoms such as chest pain, chest tightness, long exhalation, lack of strength, and dizziness. Six patients displayed varying degrees of ST-T changes on the electrocardiograph, while two patients had a reduced left ventricular ejection fraction (LVEF) of 20-32% according to echocardiography. Multislice computed tomographic angiography confirmed the presence of ARCA-L in all patients. One patient underwent the unroofing technique. The remaining eight received conservative treatment. After a follow-up of 2-64 months, eight children had a good prognosis and survived. One child experienced sudden death due to aggravated heart failure. Whole exome sequencing revealed that one child tested negative, one had mutations in the RYR2 and LDB3 genes, and the remaining four patients had a mutation in the GDF1, LRP6, MEF2A, and KALRN genes, respectively. CONCLUSIONS: ARCA-L in children might have a wide variation in clinical manifestations and a risk of sudden death. The occurrence of the disease might be associated with genetic defects.
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Anomalias dos Vasos Coronários , Insuficiência Cardíaca , Masculino , Criança , Humanos , Volume Sistólico , Anomalias dos Vasos Coronários/diagnóstico por imagem , Função Ventricular Esquerda , Aorta , Estudos Retrospectivos , Morte SúbitaRESUMO
Chiral drugs are of great significance in drug development and life science because one pair of enantiomers has a different combination mode with target biological active sites, leading to a vast difference in physical activity. Metal-organic framework (MOF)-based chiral hybrid materials with specific chiral sites have excellent applications in the highly effective sensing of drug enantiomers. Sitagliptin and clonidine are effective curing drugs for controlling diabetes and hypertension, while insulin and norepinephrine are the biomarkers of these two diseases. Excessive use of sitagliptin and clonidine can cause side effects such as stomach pain, nausea, and headaches. Herein, through post-synthetic strategy, MOF-based chiral hybrid material Eu-BTB@d-carnitine (H3BTB = 1,3,5-benzenetrisbenzoic acid) was synthesized. Eu-BTB@d-carnitine has dual emission peaks at 417 and 616 nm when excited at 330 nm. Eu-BTB@d-carnitine can be applied in luminescent recognition toward sitagliptin and clonidine with high sensitivity and low detection limit (for sitagliptin detection, Ksv is 7.43 × 106 [M-1]; for clonidine detection, Ksv is 9.09 × 106 [M-1]; limit of detection (LOD) for sitagliptin is 10.21 nM, and LOD of clonidine is 8.34 nM). In addition, Eu-BTB@d-carnitine can further realize highly sensitive detection of insulin in human fluids with a high Ksv (2.08 × 106 [M-1]) and a low LOD (15.48 nM). On the other hand, norepinephrine also can be successfully discriminated by the hybrid luminescent platform of Eu-BTB@d-carnitine and clonidine with a high Ksv value of 4.79 × 106 [M-1] and a low LOD of 8.37 nM. As a result, the chiral hybrid material Eu-BTB@d-carnitine can be successfully applied in the highly effective ratiometric sensing of curing drugs and biomarkers for diabetes and hypertension.
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Diabetes Mellitus , Hipertensão , Insulinas , Estruturas Metalorgânicas , Biomarcadores , Carnitina , Clonidina , Európio/química , Humanos , Estruturas Metalorgânicas/química , Norepinefrina , Fosfato de SitagliptinaRESUMO
Through powerful solvothermal and facile ultrasonic synthetic strategies, two unique cluster-based lanthanide Lu and Y nanoporous metal organic frameworks (MOFs) have been successfully prepared, namely, {[Lu2(L)2]·2DMF·H2O}n (Lu-MOF) and [Y(L)(DMF)0.75]n (Y-MOF) (H3L = terphenyl-3,4'',5-tricarboxylic acid). In addition, both the morphologies and nanosizes of Lu-MOF and Y-MOF materials also have been deliberately tuned by adjustable ultrasonic conditions including irradiation time (40, 60, and 80 min) and power (70 w, 100 w). Currently, it is noted that the abuse of antibiotics such as ornidazole and ronidazole leads to great damage to human health, and therefore the development of highly effective and facile detection methods for ornidazole and ronidazole is quite important. Herein, to improve the fluorescent sensing sensitivity of antibiotics, Eu3+ and Tb3+ have been introduced into Lu-MOF (under a solvothermal preparation method) to fabricate a dual-emission hybrid material Eu3+/Tb3+@Lu-MOF through a postsynthesis strategy, which can be successfully applied as a self-calibrated ratiometric fluorescent sensor for ornidazole and ronidazole with high selectivity and sensitivity (the Ksv value for ornidazole is 1.0854 × 106 [M-1], and the Ksv value for ronidazole is 1.0595 × 107 [M-1]) and low detection limit values (2.85 nM for ornidazole and 26.7 nM for ronidazole). On the other hand, amoeba liver abscess (ALA) will easily lead to irregular fever, night sweats, and other tortured symptoms; C-reactive protein autoantibody (CRP Ab) is the important biomarker for the detection of ALA. Given this, Y-MOF (under the solvothermal preparation method) also has been successfully designed to combine FAM-labeled NH-ssDNA to construct the scarcely reported excellent hybrid FAM-labeled NH-ssDNA/Y-MOF sensing platform for the highly effective discrimination of CRP Ab with excellent sensitivity and selectivity in real samples such as human serum solution.
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Técnicas Biossensoriais , Abscesso Hepático Amebiano/diagnóstico por imagem , Estruturas Metalorgânicas/química , Ornidazol/análise , Ronidazole/análise , Temperatura , Biomarcadores/análise , Cristalografia por Raios X , Fluorescência , Humanos , Lutécio/química , Estruturas Metalorgânicas/síntese química , Modelos Moleculares , Estrutura Molecular , Tamanho da Partícula , Solventes/química , Propriedades de Superfície , Ultrassom , Ítrio/químicaRESUMO
OBJECTIVE: The aim of this study is to describe the technique and to evaluate the outcome of single-incision laparoscopic (SILC) approach for linea alba hernia in children. MATERIALS AND METHODS: A 2 cm vertical umbilical incision was made and stretched horizontally. A 5-mm trocar was inserted through middle port for the telescope. Another extra-long 5-mm 30° trocar was inserted through the lateral port, 5 mm beside the middle port. The extraperitoneal fat was removed, and the defect of linea alba was repaired after hernial sac was excised. The peritoneum was reconstructed with interrupted suture. RESULTS: From May 2014 to May 2015, eight children with linea alba hernia underwent SILC. Pre-operative abdominal ultrasound showed the average diameter of hernia ring was 3.2 ± 0.7 cm. Mean operation time was 32.5 min (range = 30-45 min). Oral intake was resumed during anaesthesia recovery period. All could endure pain and discharge on the post-operative 12 h. There was no post-operative wound infection. The follow-up period was 1-12 months, no recurrence and other complications occurred. CONCLUSIONS: SILC approach for linea alba hernia is a safe and effective, minimally invasive new technology. The linea alba hernia could be repaired with a cosmetic outcome.
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BACKGROUND: We compared the effects of whole-body vibration training and proprioceptive neuromuscular facilitation on the biomechanical characteristics of the lower limbs in functional ankle instability patients during cutting movement to ascertain the superior rehabilitation method. METHODS: Twenty-two male College students with unilateral functional ankle instability volunteered for this study and were randomly divided into whole-body vibration training group and proprioceptive neuromuscular facilitation group. Kinematics data and ground reaction forces were collected using infrared motion capture system and 3-D force plates synchronously during cutting. Repeated measures two-way ANOVA was performed to analyze the data. FINDINGS: Both training methods reduced the maximum hip abduction angle (p = 0.010, effect size: proprioceptive neuromuscular facilitation = 0.69; whole-body vibration training = 0.20), maximum knee flexion angle (p = 0.008, effect size: proprioceptive neuromuscular facilitation = 0.39, whole-body vibration training = 1.26) and angular velocity (p = 0.014, effect size: proprioceptive neuromuscular facilitation = 0.62, whole-body vibration training = 0.55), maximum ankle inversion angular velocity (p = 0.020, effect size: proprioceptive neuromuscular facilitation = 0.52, whole-body vibration training = 0.81), and knee flexion angle at the time of maximum vertical ground reaction forces (p = 0.018, effect size: proprioceptive neuromuscular facilitation = 0.27, whole-body vibration training = 0.76), and increased the maximum ankle dorsiflexion moment (p = 0.049, effect size: proprioceptive neuromuscular facilitation = -0.52, whole-body vibration training = -0.22). Whole-body vibration training reduced the maximum ground reaction forces value in the mediolateral directions (p = 0.010, effect size = 0.82) during cutting movement. INTERPRETATION: These findings suggested that the two types of training might increase neuromuscular conduction function around the ankle. After these two types of training, functional ankle instability patients showed a similar risk of injury to the lateral ankle ligaments during cutting.
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Tornozelo , Exercícios de Alongamento Muscular , Humanos , Masculino , Vibração/uso terapêutico , Extremidade Inferior , Movimento , Articulação do Tornozelo , Fenômenos BiomecânicosRESUMO
OBJECTIVE: Coronary artery lesions (CALs) are a major complication of Kawasaki disease (KD); however, data on CAL incidence and risk factors in recurrent KD are limited. METHODS: Ninety-seven children with recurrent KD were retrospectively enrolled from 2013 to 2022, and CAL incidence was tracked during admission, discharge, and during follow-up. RESULTS: Initially, 27.8% had CAL at admission and discharge, declining to 7.2% at 12 months post-discharge. Most patients (66 of 97, 68.0%) did not exhibit CAL at any of the time points, 7 cases presented CAL at all time points, indicating a persistent CAL. The remaining 20 cases presented CAL at admission but recovered at discharge or during follow-up. Notably, transient CALs had presented at discharge, or during the follow-up, but finally resolved at 12 months after discharge. Notably, prior IVIG resistance and increased prothrombin time seemed associated with CAL in recurrent KD, suggesting they could help identify patients needing close monitoring. CONCLUSION: The study highlights decreasing CAL incidence over time in recurrent KD but with diverse patterns, emphasizing the importance of monitoring and further investigations to confirm these findings.
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Síndrome de Linfonodos Mucocutâneos , Recidiva , Humanos , Síndrome de Linfonodos Mucocutâneos/epidemiologia , Masculino , Incidência , Feminino , Pré-Escolar , Estudos Retrospectivos , Lactente , Criança , Doença da Artéria Coronariana/epidemiologia , Imunoglobulinas Intravenosas/uso terapêutico , Fatores de Risco , Vasos Coronários/patologia , Vasos Coronários/diagnóstico por imagem , SeguimentosRESUMO
BACKGROUND: Prolonged pneumoperitoneum has cerebral adverse effects that may delay recovery and cause postoperative cognitive changes. The purpose of this study was to investigate the effect of mannitol infusion after pneumoperitoneum initiation on cerebral oxygen balance and quality of postoperative recovery in patients undergoing prolonged retroperitoneal laparoscopy. METHODS: Forty patients scheduled for retroperitoneal laparoscopic radical excision of prostatic carcinoma were randomly divided into two groups (n = 20, each) to receive either 0.5 g/kg of 20% mannitol 150 min after the initiation of pneumoperitoneum or an equal volume of 0.9% normal saline. After surgery, time to extubation and recovery time were recorded. The Observer's Assessment of Alertness/Sedation (OAA/S) scale was used to assess the quality of recovery. The Mini-Mental State Exam (MMSE) was given to test cognitive function preoperatively and at 1, 2, and 3 h after extubation. Blood samples from the jugular bulb and the radial artery were collected for blood gas analysis before CO2 insufflation and at 10, 60, and 180 min after insufflation. RESULTS: In the control group (without mannitol), the difference between arterial and venous oxygen content (CaO2-CvO2) before insufflation (6.21 ± 2.58 mL/dL) was significantly greater than it was 3 h after insufflation (2.63 ± 1.29 mL/dL; p < 0.05). Furthermore, 3 h after insufflation, the CaO2-CvO2 also was higher in the group that had been administered mannitol (5.93 ± 1.98 mL/dL) than it was in the control group at that time (p < 0.05). Lactic acid in both arterial and jugular venous blood of the control group at 3 h postinsufflation (2.39 ± 0.89 and 2.51 ± 0.72 mg/dL, respectively) had increased significantly from the preinsufflation values (1.18 ± 0.82 and 1.1 ± 0.85 mg/dL). In the group that received mannitol, the lactic acid levels 3 h postinsufflation were essentially the same as the preinsufflation values. The recovery and extubation times in those receiving mannitol (12.19 ± 2.12 and 20.14 ± 3.62 min, respectively) were significantly shorter than in the control group (21.25 ± 3.61 and 28.79 ± 4.73 min; p < 0.05). The OAAS scores of the mannitol group at the time of extubation and 10 min afterward was significantly higher than these scores in the control group (p < 0.05). One hour and 2 h after extubation, the cognitive function score of the mannitol group was significantly higher than for the control group (p < 0.05). CONCLUSIONS: After prolonged retroperitoneal laparoscopy, there is an imbalance between oxygen supply and demand. A small dose of mannitol can effectively improve cerebral oxygen metabolism, recovery, and cognitive function after the operation.
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Encéfalo/efeitos dos fármacos , Encéfalo/metabolismo , Diuréticos Osmóticos/uso terapêutico , Laparoscopia/métodos , Manitol/uso terapêutico , Duração da Cirurgia , Oxigênio/metabolismo , Prostatectomia/métodos , Neoplasias da Próstata/cirurgia , Idoso , Humanos , Masculino , Pessoa de Meia-Idade , Pneumoperitônio Artificial , Período Pós-Operatório , Recuperação de Função Fisiológica , Espaço RetroperitonealRESUMO
OBJECTIVE: To study the diagnostic value and pitfalls of ultrasound-guided core needle biopsy (CNB) of soft tissue tumors. METHODS: One hundred and six cases of CNB specimens encountered during the period from 2007 to 2012 were enrolled into the study. The pathologic diagnosis using CNB was compared with that using surgical specimens. Diagnostic accuracy was analyzed using Chi-square test, with respect to the histologic pattern (such as spindle cell and myxoid), biologic behavior (benign versus malignant) and immunohistochemical results. The 59 cases of sarcoma were subdivided into three grades according to FNCLCC grading system. RESULTS: Histologic diagnosis could be made in 84.0% (89/106) cases. Thirteen cases were non-diagnostic on CNB. There were 4 cases on CNB showing diagnostic discrepancy with surgical specimens. Four cases of "benign lesions" on CNB found to be myxoid liposarcoma and lipoma-like liposarcoma upon resection. In general, myxoid pattern (9/17) seen on CNB showed less diagnostic correlation with surgical specimens, as compared to spindle cell and other histologic patterns (P < 0.01). The rate of diagnostic correlation was 79.7% (49/59) for the 59 cases of sarcoma studied, with grade 2 and grade 3 sarcoma showing better correlation (in contrast to 7/17 for grade 1 sarcoma) (P < 0.01). Comparative analysis showed no significant difference between benign/borderline tumors and sarcomas. The application of immunohistochemical study did not result in significant improvement in diagnostic accuracy on CNB. CONCLUSIONS: Ultrasound-guided CNB is a reliable tool in pathologic diagnosis of soft tissue tumors and shows a high accuracy rate especially for high-grade sarcoma. Tumors with myxoid pattern, lipomatous tumors and grade 1 sarcomas are associated with lower diagnostic accuracy on CNB. Correlation with clinicoradiologic findings would also be helpful in diagnostic evaluation and surgical planning.
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Biópsia com Agulha de Grande Calibre/métodos , Extremidades , Sarcoma/diagnóstico , Neoplasias de Tecidos Moles/diagnóstico , Ultrassonografia de Intervenção/métodos , Adolescente , Adulto , Idoso , Idoso de 80 Anos ou mais , Erros de Diagnóstico , Feminino , Humanos , Lipossarcoma Mixoide/diagnóstico , Lipossarcoma Mixoide/diagnóstico por imagem , Lipossarcoma Mixoide/patologia , Masculino , Pessoa de Meia-Idade , Gradação de Tumores , Sarcoma/diagnóstico por imagem , Sarcoma/patologia , Neoplasias de Tecidos Moles/diagnóstico por imagem , Neoplasias de Tecidos Moles/patologia , Adulto JovemRESUMO
BACKGROUND: Kinesio taping can effectively strengthen weakened muscles, increase walking speed, and improve dynamic balance in hemiplegic patients, but its effect on lower-limb coordination is not clear. Improving lower-limb coordination in hemiplegic patients can decrease risk of fall during walking. RESEARCH QUESTION: This study utilized continuous relative phase to depict the pattern and variability of lower-limb coordination in hemiplegic patients and healthy subjects during walking, and investigate whether it has the acute effect of Kinesio Taping on lower-limb coordination in hemiplegic patients during walking. METHODS: Gait was measured by a three-dimensional motion capture system for 29 hemiplegic patients (KT group) and 15 healthy subjects (control group). Mean continuous relative phase (MCRP) and mean continuous relative phase variability (MCRPV) were calculated to describe and evaluate lower-limb coordination. RESULTS: KT intervention only changed the coordination between the bilateral ankle joints in hemiplegic patients. Before the intervention, the MCRP of the two ankles (AA-MCRP) in the stance period of the control group was greater than the KT group (P < 0.001), the MCRPV of the two ankles (AA-MCRPV) in the swing period was lower than that in KT group (P < 0.001). After the intervention, the AA-MCRP in the stance period of the KT group increased (P < 0.001), the AA-MRPV in the swing period of KT group significantly decreased (P = 0.001). SIGNIFICANCE: Immediate ankle KT intervention can result in the in-phase or anti-phase coordination between the two ankles developing to out-of-phase coordination during the stance period of the affected limb during walking, and increase the stability of the out-of-phase coordination between the two ankles during the swing period of the affected limb. KT can be used in rehabilitation treatment for hemiplegic patients to improve acute coordination between the patients' ankles.
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Marcha , Hemiplegia , Humanos , Hemiplegia/reabilitação , Marcha/fisiologia , Caminhada/fisiologia , Extremidade Inferior , Articulação do TornozeloRESUMO
Purpose: To explore the risk factors and develop predictive models for intravenous immunoglobulin (IVIG) resistance in children with recurrent Kawasaki disease (KD). Patients and Methods: Patients with recurrent KD were retrospectively reviewed. Clinical and laboratory data at recurrence were collected and compared between patients with and without IVIG resistance. The patients were randomly divided into training and validation cohorts for model development and validation. All variables were subjected to standard Lasso and its variant group Lasso analyses, respectively, to construct predictive models. Model performance was evaluated by receiver operating characteristics (ROC) curves, calibration curves, and Hosmer-Lemeshow tests. Results: A total of 90 children with recurrent KD were included. A total of 16 cases were IVIG resistant. The patients with IVIG resistance had higher age and IVIG resistance probability at the first episode, increased CRP levels, neutrophil count, neutrophil percentage, direct bilirubin level, prothrombin time, and international normalized ratio, and decreased lymphocyte count, lymphocyte percentage, and serum sodium levels. Five variables including age and IVIG resistance at the first episode, lymphocytes count, serum sodium levels, and CRP levels were finally selected by standard Lasso (lLasso model) and four variables including age and IVIG resistance at the first episode, neutrophil percentage, and CRP levels were selected by group Lasso (gLasso). ROC curves suggested lLasso and gLasso models had similar excellent discrimination in both the training cohort (0.895 vs 0.906) and the validation cohort (0.855 vs 0.909). Hosmer-Lemeshow tests suggested the two models exerted a good calibration. Two nomograms were also constructed to facilitate the potential application of the two models. Conclusion: Age and IVIG resistance at the first episode and some laboratory variables may be risk factors for IVIG resistance in recurrent KD. Two predictive models for IVIG resistance with excellent performance were established in recurrent KD. External validation should be performed before clinical use.
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In industry, over usage of Cu2+ and Al3+ will lead to toxic wastewater, which further to give serious pollution for the environment. On the other hand, L-lysine can enhance serotonin release in the amygdala, with subsequent changes in psychobehavioral responses to stress. Therefore it is the urgent problem to design a method for detecting the amount of Cu2+, Al3+, and L-lysine. In this work, through the solvothermal synthesis method, two new coordination complexes based on the new bifunctional building block 4'-(1H-1,2,4-triazole-1-yl)- [1,1'-biphenyl]-4-carboxylic acid (HL) have been synthesized, namely, [Zn(L)2·4H2O] (complex 1) and [Cd(L)2·4H2O] (complex 2). X-ray single-crystal diffractometer was used to analyze its structure, powder X-ray diffraction (PXRD) patterns confirmed that 1 and 2 powder's purity and 1 can keep stable during the detection process of Cu2+, Al3+, and L-lysine, respectively. Elemental analysis, thermogravimetric analysis, infrared analysis, ultraviolet analysis and fluorescent spectrum have been used to characterize these complexes. The photo-luminescent test showed that 1 can accurately recognize Al3+ and Cu2+ among various cations. On the other hand, 1 can distinguish L-lysine among amino acid molecules. Therefore, 1 can be utilized as a multifunctional fluorescent probe for Al3+(Ksv = 1.5570 × 104 [M]-1), Cu2+(Ksv = 1.4948 × 104 [M]-1) and L-lysine (Ksv = 4.9118 × 104 [M]-1) with low detection limits (17.5 µM, 18.2 µM, 5.6 µM) respectively.
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Complexos de Coordenação , Cádmio , Cobre , Cristalografia por Raios X , Lisina , ZincoRESUMO
CdTe quantum dots (QDs) were modified with thioglycolic acid (TGA) and synthesized in aqueous medium. The optimum fluorescence intensity was found to be at pH 6.24 with a CdTe QDs concentration of 4.96 x 10(-7) mol L(-1). The quenched fluorescence intensity of CdTe QDs is linearly proportional to V(V) concentration from 10 to 200 ng mL(-1) with correlation coefficient R = 0.9985. The limit of detection for V(V) was 2.07 ng mL(-1). The proposed method was successfully applied to the analysis of trace amounts of V(V) in water samples with recovery of 96.5-101.8%, and the results were in good agreement with those of electrothermal atomic absorption spectrometry.
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Compostos de Cádmio/química , Corantes Fluorescentes/química , Nanotecnologia/métodos , Pontos Quânticos , Telúrio/química , Vanádio/análise , Limite de Detecção , Nanopartículas/química , Poluentes Químicos da Água/análiseRESUMO
OBJECTIVE: To study the histologic features and immunohistochemical findings of interfollicular stromal cells in hyaline-vascular Castleman's disease (HVCD), and to explore the role of these stromal cells in the pathogenesis of this disease. METHODS: The clinical findings and microscopic features of 23 cases of HVCD cases were reviewed. Immunohistochemical study for CCL21, MSA, CD21, CD35, S-100 and CD34 was carried out. RESULTS: According to the criteria proposed by Danon et al., stroma-rich variant of HVCD contained prominent interfollicular zone which occupied at least 50% of the lymph node area. In the current study, there were 14 cases of stroma-rich HVCD and 9 cases of conventional HVCD. Eleven of the stroma-rich HVCD had paraneoplastic pemphigus and contrastly, no pemphigus lesion obtained in all the 9 cases of conventional HVCD. The association between stromal cell hyperplasia and paraneoplastic pemphigus was statistically significant (P < 0.01).In all the conventional HVCD cases studied, CCL21 and MSA were positive in the stromal cells.The stromal cells in 13 of the 14 cases of the stroma-rich HVCD were also positive for CCL21 and MSA, however, staining for CD21, CD35, S-100 and CD34 was negative in both groups. There was no statistical significance obtained (P > 0.05) between the differences of the staining results. CONCLUSIONS: Stroma-rich HVCD and conventional HVCD represent two distinctive histologic variants and have a different association with paraneoplastic pemphigus. Most of the stromal cells locating in the interfollicular areas are fibroblastic reticular cells in origin, with the immunophenotype as CCL21(+)/MSA(+)/CD34â»/CD21â»/S-100â». The stromal cells proliferation correlate with the occurrence of paraneoplastic pemphigus, nevertheless, more cases are expected for a further study of the underlying pathogenesis.
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Hiperplasia do Linfonodo Gigante/patologia , Quimiocina CCL21/metabolismo , Linfonodos/patologia , Pênfigo/patologia , Células Estromais/patologia , Actinas/metabolismo , Adolescente , Adulto , Vasos Sanguíneos/patologia , Hiperplasia do Linfonodo Gigante/complicações , Hiperplasia do Linfonodo Gigante/metabolismo , Feminino , Seguimentos , Humanos , Hialina/citologia , Linfonodos/irrigação sanguínea , Masculino , Pessoa de Meia-Idade , Pênfigo/complicações , Pênfigo/metabolismo , Adulto JovemRESUMO
OBJECTIVE: To explore the relationship between the polymorphism of angiotensinogen gene (AGT) M235T and susceptibility to hypertrophic cardiomyopathy (HCM) in Asian population by meta-analysis. METHODS: PubMed, Embase, Web of Science, Cochrane library, CNKI, Wan Fang, and other databases were searched to collect the literature about AGT M235T polymorphism and HCM from the inception to March 1, 2020. The Newcastle-Ottawa Scale (NOS) checklist was uesd to perform independent literature review and study quality assessment. Data was analyzed by Stata 15.0 software. RESULTS: The results showed that, except for the recessive genetic model (TT vs MT+MM: OR = 1.27, 95%CI: 1.05-1.53), in the other four genetic models, the M235T polymorphism had no significant correlation with the risk of HCM (T vs M: OR = 1.17, 95%CI: 0.88-1.57; TT+MT vs MM: OR = 1.13, 95%CI: 0.55-2.33; TT vs MM: OR = 1.25, 95%CI: 0.60-2.59; TM vs MM: OR = 0.95, 95%CI0.5-1.82). The results of subgroup analysis showed that, except for the heterozygous genetic model, in the other four genetic models, M235T polymorphism was significantly associated with sporadic hypertrophic cardiomyopathy (SHCM), but not with familial hypertrophic cardiomyopathy (FHCM) (p > 0.05). CONCLUSION: M235T polymorphism in Asians is associated with HCM, especially SHCM. Heterozygotes increase the risk of patients with SHCM.
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Angiotensinogênio/genética , Povo Asiático/genética , Cardiomiopatia Hipertrófica/genética , Predisposição Genética para Doença , Polimorfismo de Nucleotídeo Único/genética , Alelos , Heterozigoto , Homozigoto , Humanos , Modelos Genéticos , Viés de PublicaçãoRESUMO
BACKGROUND: Therapeutic hypothermia has been recommended for eligible patients after cardiac arrest (CA) in order to improve outcomes. Up to now, several comparative observational studies have evaluated the combined use of extracorporeal cardiopulmonary resuscitation (ECPR) and therapeutic hypothermia in adult patients with CA. However, the effects of therapeutic hypothermia in adult CA patients receiving ECPR are inconsistent. METHODS: Relevant studies in English databases (PubMed, ISI web of science, OVID, and Embase) were systematically searched up to September 2019. Odds ratios (ORs) from eligible studies were extracted and pooled to summarize the associations of therapeutic hypothermia with favorable neurological outcomes and survival in adult CA patients receiving ECPR. RESULTS: 13 articles were included in the present meta-analysis study. There were nine studies with a total of 806 cases reporting the association of therapeutic hypothermia with neurological outcomes in CA patients receiving ECPR. Pooling analysis suggested that therapeutic hypothermia was significantly associated with favorable neurological outcomes in overall (N = 9, OR = 3.507, 95%CI = 2.194-5.607, P < 0.001, fixed-effects model) and in all subgroups according to control type, regions, sample size, CA location, ORs obtained methods, follow-up period, and modified Newcastle Ottawa Scale (mNOS) scores. There were nine studies with a total of 806 cases assessing the association of therapeutic hypothermia with survival in CA patients receiving ECPR. After pooling the ORs, therapeutic hypothermia was found to be significantly associated with survival in overall (N = 9, OR = 2.540, 95%CI = 1.245-5.180, P = 0.010, random-effects model) and in some subgroups. Publication bias was found when evaluating the association of therapeutic hypothermia with neurological outcomes in CA patients receiving ECPR. Additional trim-and-fill analysis estimated four "missing" studies, which adjusted the effect size to 2.800 (95%CI = 1.842-4.526, P < 0.001, fixed-effects model) for neurological outcomes. CONCLUSIONS: Therapeutic hypothermia may be associated with favorable neurological outcomes and survival in adult CA patients undergoing ECPR. However, the result should be treated carefully because it is a synthesis of low-level evidence and other limitations exist in present study. It is necessary to perform randomized controlled trials to validate our result before considering the result in clinical practices.
Assuntos
Reanimação Cardiopulmonar/métodos , Oxigenação por Membrana Extracorpórea/métodos , Parada Cardíaca/terapia , Hipotermia Induzida/métodos , Saúde Global , Parada Cardíaca/mortalidade , Mortalidade Hospitalar/tendências , Humanos , Taxa de Sobrevida/tendênciasRESUMO
l-histidine acts as a semi-essential amino acid, which is medically used in the treatment of gastric ulcer, anemia, allergies. However, the overuse of l-histidine will result in terrible damage to heart disease, slow growth of animals and water pollution in the environment. In addition, Cu2+ pollution is common environmental pollution in the industry. It has the characteristics of high accumulation, migration, and persistence. Given this, through the post-synthesis strategy, CdTe quantum dots (QDs) were the first time to introduce into zeolitic imidazolate framework-ZIF-365 to synthesis dual-emission hybrid material CdTe@ZIF-365 with high quantum yield. TEM mappings and N2 absorption tests are applied to confirm the combination mode between CdTe quantum dots and ZIF-365. It should be noted that CdTe@ZIF-365 can be successfully utilized as a bi-functional ratiometric sensor for highly sensitive discrimination of l-histidine and Cu2+. Firstly, CdTe@ZIF-365 is applied to a fluorescent ratiometric sensor for Cu2+ with high sensitivity (the Ksv value is 2.7417â107 [M-1]) and selectivity in the mixed cation ions' solution. On the other hand, CdTe@ZIF-365 also behaved as the first example for an excellent ratiometric fluorescent senor for l-histidine with high sensitivity (the Ksv value is 6.0507â108 [M-1]) and selectivity in the mixed amino acids' solutions.
RESUMO
OBJECT: The aim of this study was to use diffusion tensor tractography (DTT) to define the 3D relationships of the uncinate fasciculus, anterior commissure, inferior occipitofrontal fasciculus, inferior thalamic peduncle, and optic radiation and to determine the positioning landmarks of these white matter tracts. METHODS: The anatomy was studied in 10 adult human brain specimens. Brain DTT was performed in 10 healthy volunteers. Diffusion tensor tractography images of the white matter tracts in the temporal stem were obtained using the simple single region of interest (ROI) and multi-ROIs based on the anatomical knowledge. RESULTS: The posteroinferior insular point is the anterior extremity of intersection of the Heschl gyrus and the inferior limiting sulcus. On the inferior limiting sulcus, this point is the posterior limit of the optic radiation, and the temporal stem begins at the limen insulae and ends at the posteroinferior insular point. The distance from the limen insulae to the tip of the temporal horn is just one third the length of the temporal stem. The uncinate fasciculus comprises the core of the anterior temporal stem, behind which the anterior commissure and the inferior thalamic peduncle are located, and they occupy the anterior third of the temporal stem. The inferior occipitofrontal fasciculus passes through the entire temporal stem. The most anterior extent of the Meyer loop is located between the anterior tip of the temporal horn and the limen insulae. Most of the optic radiation crosses the postmedian two thirds of the temporal stem. CONCLUSIONS: On the inferior limiting sulcus, the posteroinferior insular point is a reliable landmark of the posterior limit of the optic radiations. The limen insulae, anterior tip of the temporal horn, and posteroinferior insular point may be used to localize the white matter fibers of the temporal stem in analyzing magnetic resonance imaging or during surgery.