TTP and aHUS in Complicated Pregnancies
Thrombotic Thrombocytopenic PurpuraAtypical Hemolytic Uremic Syndrome6 moreA single site observational study aiming to: (i) Identify cases of previously undiagnosed thrombotic thrombocytopenic purpura (TTP) and atypical haemolytic syndrome (aHUS) in a cohort of women with complicated pregnancies (ii) Characterise the clinical features of these cases and (ii) Identify clinical features or biomarkers which may help distinguish TTP/aHUS from other complications of pregnancy such as preeclampsia
Early Diagnosis for Ocular Ischemia Syndrome Using Arterial Spin Labeling Magnetic Resonance Imaging...
Ocular Ischemia SyndromeTo evaluate ocular blood perfusion status and the possibility of craniocerebral vascular lesions of patients with ocular ischemia syndrome (OIS) using arterial spin labeling (ASL) magnetic resonance imaging technique. To evaluate the specificity, sensitivity and accuracy of arterial spin labeling (ASL) magnetic resonance imaging technique in OIS diagnosis, compared with the traditional routine examination method.
Neuropsychological Assessment of Children and Adolescents With Turner Syndrome
Turner SyndromeIsolated Growth Hormone Deficiency3 moreTurner syndrome (TS) is a rare chromosomal disorder characterized by partial or complete loss of one of the X chromosomes that affects about one in every 2000 female babies born. These young patients described difficulties making friends, understanding others' emotions and intentions, and controlling their own emotions. Difficulties in these domains could led to social withdrawal, to reduced social skills and could have a significant impact on self esteem and mental health as well as on long-term academic and social functioning in affected individuals. The purpose of this project is to identify functional and dysfunctional cognitive and socio-cognitive abilities in these young patients which could account social difficulties described by some of them and their family. To this end, 35 girls with TS and 35 girls with isolated growth hormone deficiency and normal cerebral MRI will be recruited. Subjects will be 7 to 16 years and 11 months of age. Socio-cognitive and cognitive functions will be assessed with neuropsychological and experimental tasks. Questionnaires completed by patient, parents or teacher, will evaluate social and behavioral functioning.
Establishment of a Biomarkers-based Early Warning System of Acute Respiratory Distress Syndrome...
Respiratory Distress SyndromeAdultThe aim of the investigators 'study is to investigate the relationship between the biomarkers (e.g. protein markers, genetic polymorphisms and epigenetic markers) and the onset of ARDS. In this study, the participants were divided into case group (with ARDS) and control group (without ARDS), based on a nested case-control study method. During the diagnosis and treatment, the clinical data of subjects are collected at the given time point. And the clinical data are extracted from plasma, blood and bronchoalveolar lavage fluid of participants. These data will be analyzed based on statistical methods. In the end ,the investigators can build a multi index early warning model based on the biomarkers,which is meaningful for the early diagnosis of the patient with high-risk for ARDS and provide evidence for the early treatment.
Acute Respiratory Distress Syndrome in Taiwan
Acute Respiratory Distress SyndromeThe study enrolls ARDS patients in medical ICUs in 11 Hospitals in Taiwan. The epidemiology and long-term outcomes of the patients will be recorded.
Vasoplegic Syndrome in Cardiac Surgery
Vasoplegic Syndrome of Cardiac SurgeryVasoplasmic syndrome in cardiac surgery is one of the major postoperative complications.This syndrome is characterized by persistent low blood pressure despite an optimization of preload and inotropism.
Investigation of the Prevalence of Hepatopulmonary Syndrome in Cirrhosis Patients Caused by Hepatitis...
Hepatopulmonary Syndrometo investigate the prevalence of hepatopulmonary syndrome in cirrhosis patients caused by Hepatitis B in western China
The Comorbidity of Benign Hypermobility Joint Syndrome and Functional Constipation in Children
Benign Hypermobility SyndromeFunctional ConstipationBenign Hypermobility Joint Syndrome is a group of inherited abnormalities in the structure of connective tissues, manifested by disturbances in the proportion of collagen. The main symptoms of this syndrome include: laxity of joint capsules and ligaments, hypermobility of the joints, as well as numerous disturbances in the functioning of internal organs that contain connective tissue, including the gastrointestinal tract. Hypermobility of joints affects approximately 10% of the population of Western countries, is more common in small children and female. Modified Beighton scale is the basic scale for assessing hypermobility of joints. The scale (as assessed using the goniometer) is a reliable tool for the evaluation of excessive laxity of the connective tissue in children. Functional constipation is a very common condition, affecting approximately 3-5% of children and adolescents, with peak onset between 2 and 4 years of age. The etiology of this disorder is multifactorial, and till day it is still exactly unknown why some children develop constipation, while in others we can observe the correct scheme of defecation. Suspending stool enhances the retention of fecal masses, which subsequently causes painful defecation. Diagnosis is based on history, clinical symptoms and physical examination. Increased susceptibility of the wall of the distal gastrointestinal tract could explain the predisposition of some children to retain fecal masses and the development of constipation. Due to the unclear etiology of functional constipation, it seems reasonable to conduct a study assessing whether excessive laxity of connective tissue (assessed on the basis of the hypermobility of the joints) facilitates the accumulation of stool in the large intestine, and so is the one of the reasons leading to development of functional constipation in children.
The Findings of MR Imaging in Rett Syndrome
Rett SyndromeIntroduction: Rett Syndrome is a neurodevelopmental disease that primarily affects girls. Clinically, patients are normal before six months to one and half years old, and then develop progressive severe problems with communication, learning, co-ordination and neurodevelopment, with loss of motor skills around the age of two. At the same time, stereotyped hand movement typically appears. However, some of them will improve the abilities in speech and eye gaze, and the repetitive hand movements may decrease after few years. Background / hypothesis:Preliminary evidence suggests that there are many differences in brain structure between Rett syndrome and normal people including cerebral volume reduction in both white matter and gray matter, cerebral blood flow in the frontal area, and density of receptors in basal ganglia. In MR imaging studies, decreases in parietal lobe gray matter were found, and several reductions in cortical white matter were observed by DTI. However, the visual related pathway- posterior corona radiata in Rett syndrome girls was normal to be increased in FA values. The similar result was also discovered in the superior longitudinal fasciculus, associated with speech, which equal to control subjects and patients with preserved speech (phrases and sentences).Despite this, few studies mentioned comparison with brain microstructure in the different stage, especially in adult patients. Therefore, our prospective study will be planned to figure out the specific changing pattern of brain structure in different stages of Rett syndrome. And the trajectory of microstructure in brain is going to be in accordance with the functional improvement. The investigators suppose that (1)there are different microstructural changes for patients with/without preserved mobility, verbal ability and communication skill; (2)there are a few specific brain microstructures changes in visual pathway; (3) there is a specific changing pattern of brain microstructure in different aging of Rett syndrome. To acquire more specific results, the investigators aim to apply conventional MRI, diffusion-spectrum imaging, and some clinical assessment tools like Rett syndrome -related questionnaire, SSI, PDMS-2 and six-minute walk test to investigate their correlation.
Acute Renal Insufficiency (ARI) Rate and Predictive Score of ARI in Hospitalized Patients for Acute...
Acute Renal InsufficiencyAcute Coronary SyndromeThe primary objective of the study aims to evaluate frequence of acute renal insufficiency in patients with ST-segment elevation who need urgent coronary angiography in Ambroise Paré hospital. The secondary objectives are: identify factors of risks associated with the occurrence of acute renal insufficiency after coronarography. establish a preprocedure score, predicting of acute renal insufficiency after urgent coronary angiography in patients with ST+ acute coronary syndrome.