Kornimate/medical-research-clean huggingface dataset:
evaluation of the diagnostic value of video-oculography in canvas neuronopathies

Conditions: Cerebellar Ataxia

Summary: cerebellar ataxia syndrome with neuropathy and vestibular areflexia (canvas) is a genetic pathology of recent discovery (bi-allelic expansion in intron 1 of the rfc1 gene with aagg repetition). the clinical picture is protean, associating a neuronopathy, a bilateral vestibulopathy evidenced by an alteration of the oculovestibular reflex (vor), an atrophy of the cerebellum and a chronic cough. in the initial stage of the disease the clinical picture is heterogeneous and often incomplete. ataxia at the beginning of the disease may be the consequence of peripheral nervous system involvement (neuronopathy) and the cerebellar syndrome may manifest itself clinically late. eye movement involvement in central nervous system pathologies is common (4). oculomotor abnormalities are often subclinical and sometimes exclusively identifiable by an instrumental study, video-oculography (vog) (5). vog is a non-invasive examination of eye movements, which is increasingly used in the differential diagnosis of neurodegenerative syndromes (6). this examination allows, among other things, to identify oculomotor anomalies, even discrete and asymptomatic, by studying the combined movements of the eyes and the oculocephalic movements. the study of oculomotricity by vog can therefore potentially contribute to the early differential diagnosis of ataxiating neuropathies, including canvas, by revealing infra-clinical oculomotor abnormalities correlated with a cerebellar expectation (knowing the role of the dorsal vermis in the precision of saccades and pursuits).


Eligibility criteria:
inclusion criteria: patients followed at the university hospital of nîmes between 2018-2021 * age \> 18 years * patients with canvas with genetic confirmation (rfc1 gene mutation) * patients with axonal neuropathy, autoimmune neuronopathy and anti-mag neuropathy * healthy controls
evaluation of effectiveness and safety of promus element stent (iris-element )

Conditions: Coronary Artery Disease

Summary: this study is a non-randomized, prospective, open-label registry to compare the efficacy and safety of promus element stents versus first-generation drug-eluting stent (des) in patients with coronary artery disease.

the element stent platform features a proprietary platinum chromium alloy, designed specifically for coronary stents. this alloy, coupled with a new stent architecture, is designed to enable thinner struts, increased flexibility and a lower profile while improving radial strength, recoil and visibility. in addition, the promus element stent system incorporates the new apex™ dilatation catheter technology, designed to enhance deliverability to complex lesions. currently, there have been limited data regarding comparison of promus element stent with first-generation des in routine clinical practice. therefore, we perform large-scale real-world registry of promus element stents.

Eligibility criteria:
inclusion criteria: * patients receiving promus element stents * the patient or guardian agrees to the study protocol and the schedule of clinical follow-up, and provides informed, written consent, as approved by the appropriate institutional review board/ethics committee of the respective clinical site. exclusion criteria: * patients with a mixture of other dess * terminal illness with life expectancy \<1 year * patients with cardiogenic shock
modified thoracoabdominal nerve plane block in laparoscopic sleeve gastrectomy

Conditions: Obesity; Bariatric Surgery; Laparoscopic Sleeve Gastrectomy; Acute Post Operative Pain

Summary: ultrasound-guided modified thoracoabdominal nerve plane block (m-tapa) is performed into the costochondral aspect at the 9th-10th costal level by injecting local anesthetics deep into the chondrium. it provides blockage of both the anterior and lateral cutaneous branches of the thoracoabdominal nerve. studies show that m-tapa block is effective for postoperative analgesia and other abdominal surgeries, but its effect on patients undergoing lsg surgery has not yet been studied. the hypothesis is that the m-tapa block performed in patients undergoing laparoscopic sleeve gastrectomy (lsg) operation would reduce opioid consumption in the first 24 hours period after surgery.

lsg is defined as first-line bariatric surgery for patients at high surgical risk with better surgical outcomes. effective postoperative pain management is essential for early mobilization, and reduction of the risk of morbidity and mortality. opioids, due to their potent analgesic effects are used as a component of multimodal analgesia in the postoperative period. despite their advantages, opioids may be related to pulmonary complications such as atelectasis and impaired alveolar gas exchange, apnea; postoperative delayed recovery due to ileus, nausea, and vomiting; and prolonged hospital stay. the use of interfascial plane blocks for pain management has increased recently, as ultrasonography (usg) has become a part of daily routine. interfascial plane blocks provide effective postoperative analgesia. additionally, reducing postoperative opioid consumption helps to provide hemodynamic stability and promote early mobilization. studies are showing m-tapa block to be effective for postoperative analgesia for abdominal surgeries, and some studies continued comparing its effect in laparoscopic sleeve gastrectomy with other plane blocks. there are also case reports showing the effectiveness of the m-tapa block in lsg patients. m-tapa block's effect on lsg patients has not been studied yet. the investigators hypothesize that the m-tapa block performed in patients undergoing laparoscopic sleeve gastrectomy would reduce opioid consumption in the first 24-hour period after surgery.

Eligibility criteria:
inclusion criteria: american society of anesthesiologists (asa) classification ii- iii patients scheduled for lsg under general anesthesia - exclusion criteria: patients * with a history of bleeding diathesis, * receiving anticoagulant treatment, * with allergies or sensitivity to drugs used, * with an infection on the puncture site * with a history of alcohol or drug addiction, * with congestive heart failure * with liver or kidney disease * who do not accept the procedure or participate in the study

Keywords: modifiedthoracoabdominal nerve plane block laparoscopic sleeve gastrectomy bariatric surgery acute postoperative pain
hyzaar asia heaalth (0954a-950)

Conditions: Hypertension

Summary: the objective of the study is to estimate the percentage of patients who reach blood pressure goal after 8 weeks of treatment with losartan/hctz combination.


Eligibility criteria:
inclusion criteria: * patient is male or female and = 18 years of age * patients with essential hypertension previously treated with antihypertensive medications for at least 4 weeks but did not reach the blood pressure goal exclusion criteria: * history of any condition, therapy, lab abnormality or other circumstance that might confound the results of the study, or interfere with the patient's participation for the full duration of the study, such that it is not in the best interest of the patient/subject to participate * pregnant or breastfeeding, or expecting to conceive within the projected duration of the study * previous history of severe essential hypertension * history of stroke or myocardial infarction (heart attack) * evidence of renal or liver disease * uncontrolled diabetes mellitus * any known bleeding disorder * known sensitivity or intolerance to the study medication (losartan or hydrochlorothiazide) * other antihypertensive medications or medications that may affect blood pressure
direct comparison of tg and wfo lasik

Conditions: Keratomileusis, Laser In Situ; Contrast Sensitivity

Summary: to compare the efficacy to different profiles, direct head-to-head studies had been conducted. in one previous review study revealed that tg lasik provided patient with better uncorrected visual acuity (ucva) than wfo lasik and wfg lasik. in recent previous contralateral eye studies also showed that tg lasik induced less postoperative higher-order aberration than wfo lasik. however, these studies included only low-to-moderate myopia patients, for patients with high myopia (spherical equivalent \> 6d), the effect of tg lasik comparing with wfo lasik had not been reported before. the aim of our study is to analyze and compare the visual performance of tg lasik and wfo lasik in high myopia and low-to-moderate myopia patients, , particularly ucva, corrected distance visual acuity (cdva), contrast sensitivity (cs), and wavefront aberration.


Eligibility criteria:
inclusion criteria: * age between 20 and 50 years old * cdva of both eyes could reach 0.1 logarithm of the minimum angle of resolution (logmar) * stable refractive errors of myopia and astigmatism exclusion criteria: * cataract * corneal opacities or irregularities * dry eye (schirmer's test i ≤ 5mm) * amblyopia * coexisting ocular pathologies * glaucoma * non-dilating pupil * history of intraocular surgery, laser therapy, or retinopathy * optic nerve or macular diseases * estimated postoperative cornea residual stromal thickness less than 250 μm * pregnancy or under lactation * uncontrolled diabetic mellitus or systemic immune disease * refusal or unable to maintain follow-up
to evaluate the use of radiomics to classify between idiopathic pulmonary fibrosis and interstitial lung disease

Conditions: Interstitial Lung Disease; Idiopathic Pulmonary Fibrosis; Usual Interstitial Pneumonia

Summary: to investigate the ability of machine learning models based on radiomic features extracted from thin-section ct images to differentiate ipf patients from non-ipf interstitial lung diseases.


Eligibility criteria:
inclusion criteria: * uip with final diagnosis in biopsy * ilds with final diagnosis in biopsy exclusion criteria: * patients with no biopsy confirmation

Keywords: lung ct uip ipf ilds
bevacizumab in combination with metronomic temozolomide for recurrent malignant glioma

Conditions: Glioblastoma Multiforme

Summary: this is a phase ii study of the combination of avastin and metronomic temozolomide in recurrent malignant glioma patients. the primary objective will be to determine the efficacy of avastin (bevacizumab) and metronomic temozolomide in malignant glioma patients. the secondary objective will be to determine the safety of avastin, 10 mg/kg every other week, in combination with metronomic temozolomide in terms of progression-free survival.

this is a phase ii trial of the combination of avastin and metronomic temozolomide in recurrent who grade iv malignant glioma patients. patients will receive up to 12 cycles of avastin and temozolomide and cycles are continuous 28 days. patients will receive daily temozolomide at a dose of 50mg/m2 and will receive avastin every other week at a dose of 10mg/kg. patients will be required to have a baseline mri within 2 weeks of starting treatment and a repeat mri every 8 weeks. a total of 32 patients will be enrolled at duke. patients with recurrent malignant gliomas have a very poor prognosis, so new therapies are needed. given the activity of metronomic temozolomide and the safety and activity of avastin against malignant glioma, it is reasonable to study the combination in recurrent malignant glioma patients.

Eligibility criteria:
inclusion criteria: * patients must have histologically confirmed diagnosis of who grade iv primary malignant glioma * karnofsy performance status (kps) \>/= 60% * evidence of measurable primary cns neoplasm on contrast-enhanced mri. * an interval of at least 4 weeks between prior surgical resection or 1 week from a biopsy and enrollment on this protocol * an interval of at least 4 weeks from the end of prior radiotherapy or one week from the end of a cycle of chemotherapy and enrollment on this protocol. * no evidence of cns hemorrhage on the baseline mri or ct scans exclusion criteria: * life expectancy \< 8 weeks * pregnancy or breast feeding * progression to metronomic temozolomide, defined as tumor progression while taking daily temozolomide or progression within 4 weeks of stopping metronomic temozolomide * inadequately controlled hypertension (defined as systolic blood pressure \>150 and/or diastolic blood pressure \> 100 mmhg on antihypertensive medications)

Keywords: glioblastoma multiforme brain and central nervous system tumors recurrent malignant glioma recurrent glioblastoma multiforme avastin bevacizumab temodar temozolomide
wgs of korean idiopathic bronchiectasis

Conditions: Bronchiectasis Idiopathic; Cystic Fibrosis; Primary Ciliary Dyskinesia

Summary: whole genome sequencing of korean patients with idiopathic bronchiectasis and their family will perform to identify disease-causing variants.

idiopathic bronchiectasis may be a manifestation of genetic diseases such as cystic fibrosis, primary ciliary dyskinesia, etc. diagnosis of these rare genetic diseases is crucial not only because some of the rare diseases developed already effective treatment options, but also the detection of syndrome enables us to detect other organ damages before the deterioration of them. several diagnostic tools have been developed; however, the genetic panel has limited screening efficacy due to the genetic heterogeneity of diseases. even more, especially in korea, the incidence of idiopathic bronchiectasis caused by the genetic disease is rare, most clinics have limited to assess specialized diagnostic tools such as the specialized genetic panels, sweat chloride test, and the electromagnetic detection tools for ciliary movement. whole genome sequencing may be an excellent solution to identify the neglected genetic diseases causing idiopathic bronchiectasis and explore the heterogeneity of disease-causing variants in korean patients.

Eligibility criteria:
inclusion criteria: * if the patient has bronchiectasis proved by computed tomography (ct). * the clinical features of the patient are suitable for ciliary dysfunction disease (primary ciliary dyskinesia, cystic fibrosis), alpha1-antitrypsin deficiency, and primary immune deficiency (hyper-immunoglobulin e syndrome, hypogammaglobulinemia, activated phosphoinositide 3-kinase (pi3k) delta syndrome, bare lymphocyte syndrome) * the patient has no apparent medical events causing bronchiectasis. exclusion criteria: * if the patient does not agree or withdraw * if the patient has any clear etiology causing bronchiectasis including aids, malignancy, receiving immunosuppressant or chemotherapy.
laparoscopic antireflux surgery versus endoscopic full-thickness gastroplication for gastroesophageal reflux disease (gerd)

Conditions: Gastroesophageal Reflux Disease

Summary: endoscopic full thickness gastroplication (plicator-procedure) has the potential to be a safe and effective alternative to laparoscopic antireflux surgery (lars)to improve symptoms of gerd. this prospective randomized study compares objective and subjective outcome parameters of plicator with that of lars.


Eligibility criteria:
inclusion criteria: * long history of gerd symptoms * persistent or recurrent symptoms despite optimal medical treatment * persistent or recurrent complications of gerd * reduced quality of life owing to increasing esophageal exposure to gastric juice * pathological values in the preoperative evaluated functional parameters. exclusion criteria: * any distinct hiatal hernia detectable by gastroscopy or barium radiography * dysphagia * esophageal strictures * poor physical status (american society of anesthesiologists (asa) scores iii and iv) and pregnancy.
effect of tadalafil (cialis) on the cardiovascular system of spinal cord injury (sci) males

Conditions: Hypotension; Spinal Cord Injury

Summary: to address the effect of tadalafil (cialis) on the cardiovascular system of men with complete spinal cord injury at t-6 and above. the hypothesis is that tadalafil will cause significant hypotension in people with tetraplegia.

tadalafil is a long-acting pde-5 inhibitor, used to treat erectile dysfunction (ed). ed is a common issue for males with spinal cord injury (sci).patients with sci are at risk of developing orthostatic hypotension, especially those with an injury at or above the t-6 level.an earlier study of the shorter-acting pde-5 inhibitor sildenafil (viagra) revealed that it can cause orthostatic hypotension, tachycardia and dizziness after administration in the sci population, and suggested that caution should be used in prescribing sildenafil in this population, as blood pressure can drop significantly. there is potential for the significant hypotension seen with the short-acting medications to be much more problematic with the longer-acting tadalafil. the study will be a prospective, randomized, double-blind, placebo-controlled cross-over design. this will be done by comparing the effects of administering tadalafil 20 mg and placebo on blood pressure (lying and sitting), and heart rate (lying and sitting). these measurements will be taken in the clinic setting prior to dosing, then repeated hourly for the first two hours post-dose, and again at four hours post-dose. the patient will repeat the measurements at home using an automated blood pressure apparatus at 12, 22,29 and 36 hours post-dose.in addition, the patient will record perceived dizziness on a visual analog scale ecah time the bp and hr are recorded.

Eligibility criteria:
inclusion criteria: * males 18 to 70 years old, with complete spinal cord injury (asia-a) between c-4 and t-6 * minimum 6 months post-injury exclusion criteria: * diabetic * taking nitroglycerin * ischemic heart disease or significantly abnormal ekg * lower motor neuron dysfunction * heroin or cocaine use * history of adverse reaction to tadalafil or any other pde-5 inhibitor * any condition that may put the subject at risk, confound the study results, or interfere significantly with subject's ability to participate in the study * have used any other (pde-5) medications within 1 week of study medication administration

Keywords: hypotension in sci males erectile dysfunction
exploring patient reported outcomes in inherited ichthyosis

Conditions: Congenital Ichthyosis

Summary: the goal of this observational study is to learn about the presence of extracutaneous manifestations in patients with congenital ichthyosis. the main question it aims to answer is: - do patients with congenital ichthyosis experience extracutaneous manifestations? participants will fill in questionnaires in which the investigators will explore whether patients experience extracutaneous manifestations, and if so what these manifestations entail. examples of such questions are whether patients experience (joint) pain or whether they experience hindrance due to their complaints.

there is only limited knowledge about extracutaneous manifestations in patients with congenital ichthyosis. with this study, the investigators aim to identify extracutaneous manifestations with questionaires based on patient reported outcomes. secondary research questions include: * what is the prevalence of joint complaints in patients with congenital ichthyosis * what is the influence of ichthyosis on the quality of life patients experience furthermore, the investigators aim to discover knowledge gaps in current patient management and possible areas of improvement in patient care towards healthcare professionals.

Eligibility criteria:
inclusion criteria: * 16 years and older * clinically and genetically confirmed congenital ichthyosis exclusion criteria: * \<16 years of age * not able to read dutch * not able to fill in questionnaires online or in print
autonomic dysreflexia in spinal cord injuries: uds filling speed impact

Conditions: Spinal Cord Injuries; Autonomic Dysreflexia; UDS Filling Speed

Summary: in patients with spinal cord injury, the investigators want to understand and understand the differences in ad prevalence and characteristics according to bladder injection rate, and to determine which factors have a greater influence between bladder expansion or injection rate.

it has been reported that there is a difference in the degree of ad between the bladder and urinary tract motility tests, suggesting a potential difference according to the injection rate. according to liu et al., the authors noted that stimulation of the urethra, prostate, and internal sphincter appears to induce ad more strongly than the injection rate. in addition, the two main factors contributing to ad among urinary kinetics tests are the patient's age and bladder maximum urination pressure. considering these factors is important in preventing ad during urinary kinetics tests. despite these previous studies, the prevalence and severity of ad according to the injection rate of urinary kinetics tests in sci have not been quantitatively evaluated.

Eligibility criteria:
inclusion criteria: 1. patients with chronic spinal cord injury 6 months after receiving the award 2. patients with confirmed ad due to bladder fullness in previous urodynamic test 3. patients confirmed to have neurological damage level c1-t6, ais a or b exclusion criteria: 1. patients who cannot perform the test because their systolic blood pressure remains at 150 mmhg during the urodynamic test 2. patients who cannot undergo testing due to hemodynamic instability 3. patients who cannot undergo testing due to overt urinary tract infection
a study to assess efficacy and safety of wangbi capsule in patients with rheumatoid arthritis

Conditions: Rheumatoid Arthritis

Summary: the aim of this multi-center, prospective, non-interventional cohort study is to evaluate the efficacy and safety of wangbi capsule for rheumatoid arthritis patients in the real-world setting.


Eligibility criteria:
inclusion criteria: * subject has a diagnosis of ra as defined by the acr/eular 2010 classification criteria * patients older than 18 * syndrome of insufficiency of liver and kidney or syndrome of cold and dampness defined by traditional chinese medicine exclusion criteria: * patients who are pregnant or breast feeding at enrolment * history of malignancy prior to screening * patients with severe or poorly controlled chronic diseases such as hypertension, diabetes and coronary heart disease * patients taking biological agents, \> 10mg prednisone (or equivalent amount of other glucocorticoids) ，more than 2 kinds of dmards or similar tcm drug like wangbi capsule
to assess the efficacy, safety, and tolerability of incb000928 in participants with fibrodysplasia ossificans progressiva

Conditions: Fibrodysplasia Ossificans Progressiva (FOP)

Summary: this phase 2, randomized, double-blind, placebo-controlled study is intended to evaluate the efficacy, safety, and tolerability and pk of incb000928 administered to participants with a clinical diagnosis of fibrodysplasia ossificans progressiva (fop).


Eligibility criteria:
inclusion criteria: * female and male participants: * cohort 1: ≥ 12 years of age. * cohort 2: 6 to \< 12 years of age. * cohort 3: 2 to \< 6 years of age (after edmc review of interim data from cohort 2). * clinical diagnosis of fop. * willingness to avoid pregnancy or fathering children based on the criteria below. * willing and able to undergo low-dose wbct (excluding the head) imaging without requiring intubation. * further inclusion criteria apply. exclusion criteria: * pregnant or breast-feeding. * cajis score ≥ 24. * fop disease severity that in the investigator's opinion precludes participation. * any clinically significant medical condition other than fop that would, in the investigator's judgment, interfere with full participation in the study, pose a significant risk to the participant, or interfere with interpretation of study data. * chronic or current active infectious disease requiring systemic antibiotic, antifungal, or antiviral treatment. * hiv, hbv, or hcv infection. note: * further exclusion criteria apply.

Keywords: fibrodysplasia ossificans progressiva (fop) heterotopic ossification