Kornimate/medical-research-clean huggingface dataset:
evaluation of heart involvement in patients with fabry disease

Conditions: Rare Diseases; Fabry Disease; Fabry Disease, Cardiac Variant; Hypertrophic Cardiomyopathy

Summary: this study evaluates predictors for the incidence of arrhythmias and sudden cardiac death as well as terminal heart failure in patients with fabry disease.

fabry disease is a rare disease and part of the group of lysosomal storage disorders. natural history of fabry disease has proven poor survival to ages \>50 years outlining the importance to evaluate cardiac symptoms and outcomes of patients with fabry disease. this study is a prospective cohort study and observes patients since 2001. through this long-term experience and the relative high number of patients this study is suggested to help estimating the risk of cardiac arrhythmias and sudden cardiac death (scd) as well as death or heart transplantation due to terminal heart failure. all patients in treatment in the fabry center wuerzburg (fazit) are included in this study if informed consent is provided.

Eligibility criteria:
inclusion criteria: * fabry disease (genetically confirmed) * signed informed consent * 18 years and older exclusion criteria: * no informed consent * withdrawal of informed consent

Keywords: fabry fibrosis dialysis stroke chronic kidney disease
the coronary access after tavi registry - a multicenter prospective registry for the coronary access after tavi

Conditions: Aortic Valve Disease

Summary: the caveat study is a not for profit, international, multicenter study aimed to assess the feasibility of selective coronary angiography (ca) after transcatheter aortic valve implantation (tavi), to investigate predictors of successful selective cannulation (anatomical, technical, etc.) and to investigate if there is a significant difference among different type of transcatheter valves in selective coronary cannulation.


Eligibility criteria:
inclusion criteria: * symptomatic aortic valve disease (stenosis or combined stenosis and regurgitation) candidate to tavi according to local heart team evaluation, including valve-in-valve procedure due to degeneration of a previously implanted surgical bioprosthesis, who undergo coronary access in the same session of tavi, immediately after transcatheter valve deployment (the procedures have to be consecutive); * provision of signed informed consent; * age ≥18 years. exclusion criteria: * chronic kidney disease stage iv-v (without dialytic treatment; otherwise, patients in dialytic treatment could be included); * current participation in an interventional clinical study; * age \< 18 years.
evaluation of outcome metrics in alexander disease

Conditions: Alexander Disease

Summary: the purpose of this study is to define the natural history of alexander disease, a leukodystrophy that causes neurological dysfunction. investigators will obtain clinical outcome assessments to measure how the disease affects a patient's gross motor, fine motor, speech and language function, swallowing, and quality of life. specimens are collected to measure glial fibrillary acidic protein (gfap) levels in cerebrospinal fluid (csf) and blood. the data obtained from this study will be used for the design of future treatment trials.

participants will be asked to complete physical examinations including physical therapy, occupational therapy, speech and language therapy, neurocognitive and swallowing assessments. patients (or caretakers) may be asked to complete questionnaires as well. specimen collection is an optional procedure. the study asks for participants to return at least once yearly to repeat assessments.

Eligibility criteria:
inclusion criteria: * diagnosed with alexander disease exclusion criteria: * other leukodystrophies will not be enrolled

Keywords: alexander disease
can a pulmonologist reliably assess the tumor burden on rose slides obtained from pulmonary nodules and lymphadenopathy

Conditions: Lung Cancer

Summary: the study is aimed at verifying if a pulmonologist can reliably assess the tumor burden for the extractive molecular analysis in rose samples obtained with endoscopic sampling procedures from intrathoracic lymphadenopathy and pulmonary nodules.


Eligibility criteria:
inclusion criteria: * age \> 18 years * indication for endoscopic sampling of suspected malignant lymphadenopathy or pulmonary lesion * written informed consent exclusion criteria: * high risk conditions for the performance of bronchoscopy and/or ebus-tbna * high risk condition for deep sedation (asa 4) * pregnancy
sleep disorders of patients with diseases of the nervous system

Conditions: Hypersomnia; Narcolepsy; Nervous System Disease; Sleep Disorder

Summary: the purpose of this study is to assist training doctors about different diseases of the nervous system affecting sleep. patients selected to participate in this study will have any of a variety of sleep disorders. they will undergo several tests including an overnight recording of brain activity, eye movement, leg movement, breathing, heart rate, and other measures. results of these tests will be used to better understand diseases causing sleep disorders and may be used to develop better treatments for them.

the objective of this protocol is to provide training in a broad range of neurological sleep disorders to clinical associates who are eligible for sitting in the board examination of the added qualification of clinical neurophysiology administered by the american board of psychiatry and neurology. this protocol is to supplement small accrual of research patients who are referred from within the nih.

Eligibility criteria:
* iinclusion criteria: patients 5 years of age and older, with the following referring diagnoses: narcolepsy hypersomnia rem behavior disorder frontal nocturnal dystonia restless legs syndrome insomnia parasomnia nocturnal epileptic seizure nocturnal movement disorder sleep-related diagnostic dilemma sleep apnea associated with neurological disorders patients who are ambulatory and who are able to comply with the study procedures. patients who are able to understand and sign the informed consent. (parent or guardian for minors). exclusion criteria: patients who have been diagnosed with acute and progressive neurological disorders. patients who are unable to comply with the study procedures and require other than standard care.

Keywords: narcolepsy polysomnography multiple sleep latency test sleep disorders hypersomnia periodic leg movements
long term outcome study in patients with pituitary disorders

Conditions: Pituitary Diseases; Pituitary Neoplasms

Summary: to determine the outcomes of patients with different pituitary disorders whether they are managed medically, surgically or expectantly.

a chart audit of around 1500 patients seen in two specific clinics, the medical pituitary clinic and the combined endocrine/neurosurgical pituitary clinic at the university of alberta hospital, will be performed to determine the effectiveness of various treatment strategies on the long term outcome of specific pituitary disorders. the investigator's will collect retrospective demographic (age, sex, ethnicity), clinical (mode of presentation, hormonal parameters) and therapeutic outcome (surgical, medical, expectant and / or radiation treatment; co-morbidities and complications) of patients seen in these two clinics between january 2000 and december 2020.

Eligibility criteria:
inclusion criteria: * patients with confirmed pituitary disease exclusion criteria: * patients who do not have confirmed pituitary disease

Keywords: pituitary tumor acromegaly cushing prolactinoma
dosage determination trial for indigo naturalis extract in oil ointment

Conditions: Psoriasis Vulgaris

Summary: the use of refined indigo naturalis (indigo naturalis extract in oil, ineo)ointment to treat psoriasis has been proven effective in our previous study. this study aims to evaluate the efficacy and safety of ineo ointment, and further determine the optimal concentration of ineo ointment (per gram of ointment containing either 200 μg, 100 μg, 50 μg or 10 μg of indirubin) in treatment of various local skin signs and thickness of psoriasis plaque.

the use of indigo naturalis ointment to treat psoriasis has been proven effective in our previous clinical studies which demonstrated the efficacy and safety of topical indigo naturalis ointment, showing its ability to provide significant improvement of psoriatic skin lesions. we developed a new formulation in which indigo naturalis powder is refined and reduces the blue discoloration of skin and clothes, making the treatment more user-friendly. we had observed an equivalent efficacy of the refined form of indigo naturalis (ineo) ointment on treating psoriasis as the crude form. however, it is necessary to determine an appropriate dosage of the refined form regarding its efficacy and safety. this is a double-blind, 4-arm parallel study. the aim of this study is 1. to determine an appropriate dosage of indirubin in ineo ointment, regarding its efficacy and safety. 2. to evaluate the efficacy of ineo ointment in different local skin signs of plaque lesions. 3. to evaluate the efficacy of ineo in different traditional chinese medicine clinical syndromes of psoriasis vulgaris. 4. to investigate the effects of ineo ointment on the immune system. 5. to evaluate the effects of ineo ointment in improving quality of life after treatment.

Eligibility criteria:
inclusion criteria: * aged 20 - 65 years, men or women. * diagnosed as mild to moderate plaque-type psoriasis by the dermatologist, with psoriasis for a minimum of 1 year. * plaque psoriasis involving \<20% of bsa and with pasi \<20. * female patients of child-bearing age with negative pregnancy test at screening. * female patients of childbearing age who have agreed to continue using birth control measures approved by the investigator and agree not to lactate for the duration of the study. * willingness to comply with study protocol. * with signed informed consent form. exclusion criteria: * with history of topically or systematically hypersensitive to indigo naturalis or its excipient in ointment. * with history of sensitivity to chinese herb. * received systematic treatment for psoriasis within 4 weeks. * received topical treatment for psoriasis within 2 weeks. * with abnormal liver or renal function, clinically significant abnormalities in hematology, severe uncontrolled metabolic syndrome,psychiatric disease, cancer or aids. * patients with pustular or erythrodermic psoriasis.

Keywords: indigo naturalis extract in oil
phase 3 study to evaluate the efficacy and safety of peginterferon lambda for 48 weeks in patients with chronic hdv

Conditions: Hepatitis Delta Virus

Summary: the phase 3 limt-2 study will evaluate the safety and efficacy of peginterferon lambda treatment for 48 weeks with 24 weeks follow-up compared to no treatment for 12 weeks in patients chronically infected with hdv. the primary analysis will compare the proportion of patients with hdv rna \< lloq at the 24-week post-treatment visit in the peginterferon lambda treatment group vs the proportion of patients with hdv rna \< lloq at the week 12 visit in the no-treatment comparator group.

this is a randomized, open-label, parallel arm study that will allocate patients (2:1) with chronic hdv infection to one of two treatment groups: peginterferon lambda 180 mcg qw for 48 weeks with 24 weeks follow-up (arm 1, n=100), or no treatment for 12 weeks followed by peginterferon lambda treatment for 48 weeks with 24 weeks of follow-up (arm 2, n=50). all patients will receive concomitant therapy with a potent 2nd generation anti-hbv nucleos(t)ide analogue (nuc) throughout the study duration. data collected during peginterferon lambda treatment in arm 2 will not be included in the primary analysis. the primary purpose of arm 2 is to provide benchmark data in a parallel reference group for the expected rate of hdv rna suppression among patients with chronic hdv infection who receive 12 weeks of anti-hbv nuc therapy alone. prior to randomization, all patients will enter a run-in phase with 12 weeks of anti-hbv nuc therapy, which will ensure virologic control of hbv (\< 100 iu/ml) prior to randomization and start of peginterferon lambda therapy.

Eligibility criteria:
inclusion criteria: * chronic hdv infection documented by a positive hdv antibody test or a positive hdv rna by rt pcr test for at least 6 months at screening visit 2 * quantifiable hdv rna by rt-pcr test at screening visit 2 * documented confirmed suppression of hbv dna (\< 100 iu/ml) following at least 12 weeks of anti-hbv nuc treatment with etv or a tnf-based nuc (tdf or taf) at screening visit 2 * serum alt \> upper limit of normal (uln) and \< 10 × uln. * patients categorized with child-turcotte-pugh score of ≤ 5 with well compensated liver disease. exclusion criteria: * history or current evidence of decompensated liver disease (episodes of bleeding esophageal or gastric varices, ascites and/or encephalopathy) * treatment with interferons (ifns) or immunomodulators within 6 months before screening visit 2 or refractory to prior ifn treatment.

Keywords: hdv hepatitis d viral hepatitis
prospective evaluation of the covered self-expandable metal stents (csems) for incomplete biliary stone clearance

Conditions: Choledocholithiasis

Summary: the current standard of care for bile duct stone are endoscopic sphincterotomy and stone extraction. placement of plastic stents is an option for incomplete biliary stone clearance. this study will use the csems in patients with complex biliary stones who failed stone extraction as they have the advantage of large stent diameter. csems may offer a temporizing measure that allows more successful subsequent stone clearance. however, their use in benign condition has been limited especially for biliary stone removal. the purpose of this study is to determine the feasibility, safety, easy removability, stent-stone formation rate, and migration rate of csems for complex biliary stones.

same as brief summary

Eligibility criteria:
inclusion criteria: * 18 years and older * bilary stone unable to be extracted with balloon, basket, large balloon dilation of the ampulla or mechanical lithotripsy. * stone can be bridged by the metal stent * subject must be able to give informed consent. exclusion criteria: * any contraindication to ercp * patient refuses consent for the study.

Keywords: choledocholithiasis metal stents biliary duct stent
multifocal contact lenses vs bifocal spectacles for congenital cataracts

Conditions: Multifocal Contact Lenses

Summary: in this study, the investigators provide participants who had congenital cataract the multifocal contact lenses and bifocal spectacles in the purpose of seeking out a better way to make a definite diagnosis with high efficiency and accuracy, and report a prospective, randomized controlled study aiming at comparison of multifocal contact lenses and bifocal spectacles.

children diagnosed as cataract without treatment were enrolled to a prospective, randomized controlled study. patients were assigned to four groups: participants in group a wearing multifocal contact lenses a week and then bifocal spectacles a week;in group b, the participants wearing bifocal spectacles a week and then multifocal contact lenses;in group c, the participants wearing multifocal contact lenses four a year;in group d, the participants wearing bifocal spectacles four a year. investigators compare the acuity, refraction, contrast sensitivity and the results of questionnaire between a and b , c and d groups.

Eligibility criteria:
inclusion criteria: * diagnosed as binocular cataract * after the surgery of binocular intraocular lens implantation more than one month exclusion criteria: * diagnosed as xerophthalmus * diagnosed as other eye diseases or systemic diseases

Keywords: multifocal contact lenses bifocal spectacles congenital cataracts
diagnosis of occult hip fractures with ultrasound

Conditions: Hip Fractures; Occult Fractures

Summary: occult hip fractures present to the emergency room with a history of trauma,a painful hip and normal x-ray. these patients usually undergo other imaging modalities such as ct scans, mri, or bone scans. in this study we check the sensitivity and specificity of a directed ultrasound examination of the hip joint in diagnosing or excluding a hip fracture. we also compare the results of ultrasound examination by the orthopedic surgeon in the emergency room versus the consultant radiologist. the patients undergo bone scans or ct to make the diagnosis.


Eligibility criteria:
inclusion criteria: * patients over age of 50 with clinical suspicion of a hip fracture exclusion criteria: * previous fracture or surgical intervention in the hip * pregnancy * flexion contracture of hip

Keywords: fracture occult hip
colesevelam versus placebo in the treatment of nonalcoholic steatohepatitis

Conditions: Nonalcoholic Steatohepatitis

Summary: the purpose of the study is to see if the drug colesevelam is a potential treatment for nonalcoholic steatohepatitis(nash).

we plan to investigate the role of colesevelam, a bile-acid binding resin, in patients with nash residing in the united states and assess liver fat changes during therapy using mri of the liver. it can be hypothesized that colesevelam would lead to a greater improvement in insulin sensitivity and lipid profile compared with placebo and may lead to greater improvement in liver fat by mri as compared to placebo. in this pilot study, we propose to randomize approximately 55 patients (1:1 ratio) to either colesevelam or placebo and treat them for 24-weeks to evaluate changes in baseline insulin sensitivity, serum biochemistry (alt and ast), and liver fat by mri during therapy. liver histologic changes would also be examined as an exploratory outcome for future studies.

Eligibility criteria:
inclusion criteria: 1. age at entry at least 18 years. 2. serum alanine (alt) or aspartate (ast) aminotransferase activities that are above the upper limits of normal. 19 or more in women and 30 or more in men. 3. evidence of hepatic steatosis or liver fat (\>5%) by mri. 4. evidence of definite or suspected nash 5. written informed consent. exclusion criteria: 1. evidence of another form of liver disease. 2. history of excess alcohol ingestion. 3. contraindications to liver biopsy. 4. decompensated liver disease. 5. history of gastrointestinal bypass surgery or ingestion of drugs known to produce hepatic steatosis. 6. recent initiation or change of anti-diabetic drugs. 7. use of colesevelam or other agents in the same class. 8. significant systemic or major illnesses other than liver disease that, in the opinion of the investigator would preclude treatment with colesevelam and adequate follow up. 9. positive test for anti-hiv. 10. active substance abuse, such as alcohol, inhaled or injection drugs within the previous one year. 11. pregnancy or inability to practice adequate contraception in women of childbearing potential. 12. evidence of hepatocellular carcinoma

Keywords: nash nafld steatohepatitis nonalcoholic fatty liver disease
a trial on laparoscopic appendectomy versus single port appendectomy

Conditions: Acute Appendicitis

Summary: the aim of this study is to compare patients who undergone single-port access laparoscopic appendectomy to those who underwent conventional three-port laparoscopic appendectomy (tpla) in a prospective randomized trial

* primary objective 1. complication rate * secondary objectives 1. satisfaction rate 2. pain scale difference

Eligibility criteria:
inclusion criteria: * age 5\~85 years * any patient diagnosed with acute appendicitis on appendix sonography or abdominopelvic ct * patient performance status(ps) with greater than 80 or more on karnofsky ps * give written informed consent prior to study specific screening procedures, with the understanding that the patient has the right to withdraw from the study at any time, without prejudice. exclusion criteria: * pregnant or lactating * serious uncontrolled intercurrent infections, or other serious uncontrolled concomitant disease * history of previous cytotoxic chemotherapy, radiotherapy or immunotherapy, for the currently treated cancer * clinically significant (i.e. active) cardiac disease e.g. symptomatic coronary artery disease, new york heart association (nyha) grade ii or greater congestive heart failure or serious cardiac arrhythmia requiring medication or myocardial infarction within the last 12 months.

Keywords: appendectomy appendicitis laparoscopic surgery
adjuvant rezvilutamide in combination with androgen deprivation therapy in androgen receptor-positive, high-risk salivary duct carcinoma

Conditions: Salivary Gland Neoplasm Duct

Summary: the purpose of the study is to evaluate the efficacy and safety of rezvilutamide in combination with androgen deprivation therapy (adt) in participants with androgen receptor (ar) positive, high-risk salivary duct carcinoma (sdc). the procedures include screening, treatment and follow-up period. the treatment includes rezvilutamide plus luteinizing hormone releasing hormone agonist (lhrha) for up to 2 years.


Eligibility criteria:
inclusion criteria: * provision of signed and dated informed consent form * stated willingness to comply with all study procedures and availability for the duration of the study * histologically confirmed salivary duct carcinoma and stage iii or iva or ivb according to ajcc cancer staging manual eighth edition * completed sdc surgery, and adjuvant radiotherapy is planned or ongoing or has completed * androgen receptor (ar) positive * white blood cell (wbc) ≥ 3.0 x 10\^9/l, neutrophil count≥ 1.5 x 10\^9/l, platelet count (plt) ≥ 75 x 10\^9/l, haemoglobin (hb) ≥ 90 g/l * serum creatinine (cr) \< 1.5 x upper limit of normal(uln) or creatinine clearance ≥ 50 ml/min. * total bilirubin (tbil) \< 1.5 x uln, ast\< 2.5 x uln, and alt\< 2.5 x uln * eastern cooperative oncology group (ecog) performance status 0, 1 or 2, and ability to take oral medication * for females of reproductive potential: use of highly effective contraception for at least 1 month prior to screening and agreement to use such a method during study participation and for an additional 1 year after the end of study intervention administration * for males of reproductive potential: use of condoms or other methods to ensure effective contraception with partner exclusion criteria: * received prior anti-ar pathway therapy * treatment with another investigational drug or chemotherapy within 6 months * history of hypothalamus or pituitary dysfunction * history of seizure * clinically uncontrolled diseases, such as septic shock, uncontrolled hypertension, unstable angina, new york heart association (nyha) class iii or iv heart disease, clinically unstable arrhythmia, myocardial infarction (in the past 6 months) * previous cancer except skin cancer rather than malignant melanoma or any cancer curatively treated \> 5 years prior to study entry * pregnancy or lactation