Kornimate/medical-research-clean huggingface dataset:
firazyr® patient registry (icatibant outcome survey - ios)

Conditions: Hereditary Angioedema (HAE)

Summary: the icatibant outcome survey (ios) is a prospective, observational disease registry designed to document the routine clinical outcomes over time in participants with angioedema treated with firazyr® (icatibant) and/or cinryze® (c1 inhibitor \[human\]) in countries where it is currently approved. the data collected will be used to evaluate the safety of firazyr (icatibant) and cinryze (c1 inhibitor \[human\]) in routine clinical practice and as a data source for post-marketing investigations.

the icatibant outcome survey (ios) is a multicenter, prospective, observational study for participants treated with firazyr (icatibant) and/or cinryze (c1 inhibitor \[human\]) in countries where it is currently approved. the entry of participants in the icatibant outcome survey (ios) is at the discretion of the physician and the participant and is not a pre-requisite for prescribing firazyr (icatibant) or cinryze (c1 inhibitor \[human\]).

Eligibility criteria:
inclusion criteria: 1. diagnosis of at least 1 of the following: * hereditary angioedema (hae) type i or ii * hae with normal c1 inhibitor * ace-i-induced angioedema * non-histaminergic idiopathic angioedema * acquired angioedema. 2. signed and dated written informed consent from the participant or, for participants aged less than(\<)18 years (or as per local regulation, such as \<16 years in the united kingdom \[uk\]), parent and/or participants legally authorized representative (lar), and assent of the minor where applicable. 3. at sites only participating in the drug registry, participants must have taken at least 1 dose of firazyr (icatibant) or cinryze (c1 inhibitor \[human\]). 4. enrolled participants in germany taking firazyr (icatibant) or cinryze (c1 inhibitor \[human\]) will only use the respective product in accordance with the product label. exclusion criteria: 1. participants enrolled in clinical trials where the product is blinded or where the product under investigation is for the treatment of hae, ace-i-induced angioedema, non-histaminergic idiopathic angioedema, or acquired angioedema. 2. participants enrolled in another shire-sponsored registry involving products for the treatment of hae, ace-i-induced angioedema, non-histaminergic idiopathic angioedema, or acquired angioedema. an exception applies to participants enrolled in the shire lanadelumab enable study.

Keywords: hereditary angioedema
outcome of cataract surgery with uveitis

Conditions: Cataract; Complicata

Summary: aim of the study is to evaluate outcome of cataract surgery in different types of uveitis as regarding best corrected visual acuity (bcva) and rate of post operative complications.

cataract is the main cause of reversible blindness in patients with uveitis. cataract occurs in up to 50% to 70% of patients with uveitis. preoperative complications, including anterior synechiae, posterior synechiae, and pupillary membrane formation, may increase surgical challenges. in addition, recurrent inflammation increases the incidence of postoperative complications and often affects the visual prognosis. in recent years, phacoemulsification with intra ocular lens (iol)implantation has become the main surgical method for treating uveitis (complicated cataract), and the visual prognosis of patients who undergo this procedure is usually favorable. surgical treatment may be effective but is associated with higher rates of complication than in non uveitic eyes. cystoid macular edema (cme) is the most common complication cataract surgery in the general population. although, in most cases, the macular edema is self-limited, in rare cases it can lead to long-term visual deterioration that is difficult to treat. another common complication after cataract surgery is posterior capsule opacification (pco) , leading to symptoms of glare or blurred vision, reduced visual acuity, or impaired posterior segment exam. factors that are critical in the development of pco include surgical technique, type of implanted intra ocular lens (iol) either foldable hydrophilic acrylic, hydrophobic acrylic or silicone and postoperative control of uveitis

Eligibility criteria:
inclusion criteria: - visually significant cataract ( means opacification of the crystalline lens adequate to interfere with vision)in patients with uveitis controlled for at least 1month. exclusion criteria: * irreversible pathology affecting outcome e.g. macular scar ,optic atrophy, and retinal detachment. * patients with active uveitis (means inflammation inside the eye). * patients less than 16 years old.

Keywords: uveitis
video consultation in cpap for patients with obstructive sleep apnea

Conditions: Obstructive Sleep Apnea

Summary: the objective of this study is to evaluate the effects of video consultation versus face-to-face consultation for patients with obstructive sleep apnea on patients' cpap use (minutes/per night), self-efficacy, risk outcomes, outcome expectancies, experiences with technology and, patients' and nurses' satisfaction.

the objective of this study is to evaluate the effects of video consultation versus face-to-face consultation for patients with obstructive sleep apnea on patients' cpap use (minutes/per night), self-efficacy, risk outcomes, outcome expectancies, experiences with technology and, patients' and nurses' satisfaction. this study is a non-blinded randomized controlled trial with an intervention group (video consultations) and a usual care group (face-to-face consultations), with assessements after 1, 2, 3, 4, 12 and 24 weeks. patients were recruited from a large teaching hospital (rijnstate, hospital), and included from january 2, 2019 until june 26, 2019.in total, 140 patients were randomized, (1:1 allocation).

Eligibility criteria:
inclusion criteria: * older than 18 years * diagnosed with obstructive sleep apnea * ahi \> 15 * requiring cpap treatment * no history of cpap treatment * having access to a tablet or smartphone * proficiency of the dutch language exclusion criteria: * psychiatric or cognitive disorder

Keywords: video consultation e-health cpap randomized controlled trial
real-time identification of the aberrant left hepatic arterial territory in the liver using near-infrared fluorescence imaging

Conditions: Stomach Neoplasm

Summary: " hepatic artery variants are occasionally seen, especially 20-30% of aberrant left hepatic artery. in radical gastrectomy, decision for aberrant left hepatic artery(alha) ligation should consider the oncologic safety and liver-related complication. theoretically, the alha preservation is the most ideal in the aspect of liver function protection. however, it is technically difficult which consumes much time. not only that, oncologic safety could be threatened as some soft tissues, including lymph nodes, could be remained while in preserving the alha. there has been no standardized method to evaluate the alha, and to decide whether preserve or ligate it. this prospective study has been designed to develop the decision algorithm to define the alha preservation/ligation, using near-infrared fluorescence imaging during surgery. "


Eligibility criteria:
inclusion criteria: 1. patients diagnosed with gastric adenocarcinoma pathologically before surgery 2. patients aged between 20 to 80 3. patients with an ecog 0 or 1 4. patients who were confirmed the presence of aberrant left hepatic artery before or during surgery exclusion criteria: 1. patients with abnormal liver function test befor surgery 2. patients who diagnosed liver cirrhosis or infectious liver disease 3. patients who underwent liver resection, or chemotherapy for gastric cancer 4. patients planned for combined liver resection or cholecystectomy during gastrectomy
comparison of hes 130 in balanced and in unbalanced solution

Conditions: Decreased and Nonspecific Blood Pressure Disorders and Shock

Summary: the purpose of this study is to evaluate the efficacy of hes 130/0.42 in an electrolyte infusion in comparison to hes 130/0.4 in a sodium chloride infusion.


Eligibility criteria:
inclusion: * male or female (non pregnant) patients; between 18 and 70 years * elective non-cardiac surgery (except pancreatic or neurological surgery) with an estimated operation duration of 2 hours or longer * general anesthesia * voluntary consent exclusion: * patients of asa-class iii or iv, cardiac insufficiency nyha classes iii. iv * bmi \ 29 * patients with daily urine output \< 1l * patients on hemodialysis * abnormal liver or renal function (i. e. above upper limit of normal range) * abnormal coagulation function (prothrombin time (pt) 3 seconds higher than normal or activated partial thromboplastin time (aptt) 10 seconds higher than normal) * hgb \< 10g/dl or hct \< 30% * potassium, sodium or chloride above the upper limit of normal of the laboratory * history of diabetes mellitus * known history of a mental or neurological disorder, such as epileptic seizures. * history of hypertension and/or taking a beta-receptor blocker or history of atrial fibrillation * patients with known hypersensitivity and with contraindications to hes or any of the excipients
cardiac amyloidosis : diagnostic using red flag signals

Conditions: Cardiac Amyloidosis

Summary: cardiac amyloidosis is an increasingly contributor of degenerative cardiac diseases. however, its frequency remains underestimated, and diagnosis is often realized at late stages of the disease. a larger use of clinical and echographic red flag signals during routine echocardiographic examination may enhance the identification of early stage of the disease.

the objective is to evaluate the clinical and epidemiological aspects of cardiac amyloidosis using a systematic screening of even moderate left ventricular hypertrophy, taking into account the presence of red flag signals. red flag signals will include history or symptoms of carpal tunnel syndrome, hearing loss, chronic gastrointestinal disorders, heart failure, cervical or lumbar stenosis, or echographic abnormalities such as apical sparring, increased left ventricular filling pressure, atrio-ventricular block.

Eligibility criteria:
inclusion criteria: 1. diagnosis of left ventricular hypertrophy defined by a parietal thickness (interventricular septum or posterior wall) ≥ 12 mm on the echocardiogram 2. age equal or greater than 45 years 3. current residency in martinique, guadeloupe or french guyana 4. ability to receive and understand research information 5. ability to freely deliver informed written consent exclusion criteria: 1. pregnant or breastfeeding woman 2. severe uncontrolled hypertension 3. chronic hemodialysis 4. person under legal protection measures (guardianship, curatorship, safeguard of justice), and person deprived of liberty
high flow nasal cannula vs nppv in moderate chronic obstructive pulmonary disease exacerbation

Conditions: COPD Exacerbation

Summary: for moderate aecopd(ph\<7.35), several guidelines have strongly recommended nppv as standard therapy, which can reduce the rate of intubation and mortality. a few previous studies have shown that hfnc can efficiently improve alveoli ventilation and breathing pattern in some severe aecopd patients. therefore, we hypothesize that hfnc is not inferior to nppv for preventing endotracheal intubation in moderate aecopd patients. to assess this hypothesis, we performed a multicenter, randomized, noninferiority trial of hfnv vs nppv in moderate aecopd patients.

recently, many clinical studies have showed that hfnc can be successfully used in patients with hypoxemic respiratory failure to improve the comfort, to attenuate respiratory failure and to decrease the rate of tracheal intubation and mortality. although theoretically hfnc is a potential and ideal treatment for aecopd patients, the related clinical researches are very few. it still needs to further evaluate the safety and efficacy of hfnc in patients with aecopd. for moderate aecopd(ph\<7.35), several guidelines have strongly recommended nppv as standard therapy, which can reduce the rate of intubation and mortality. a few previous studies have shown that hfnc can efficiently improve alveoli ventilation and breathing pattern in some severe aecopd patients. therefore, we hypothesize that hfnc is not inferior to nppv for preventing endotracheal intubation in moderate aecopd patients. to assess this hypothesis, we performed a multicenter, randomized, noninferiority trial of hfnv vs nppv in moderate aecopd patients.

Eligibility criteria:
inclusion criteria: * aecopd; 7.35\>ph≥7.25，paco2\>50 mmhg exclusion criteria: * contraindications for nppv, such as thick sputum, cough weakness, hemodynamic instability, etc.; need to be intubated immediately; refuse to engage in the study; severe organ dysfunction

Keywords: copd exacerbation high flow oxygen noninvasive positive ventilation moderate
operational research for cryptococcal antigen screening

Conditions: Cryptococcal Meningitis; Cryptococcus Neoformans; Cryptococcosis

Summary: this will be a stepped wedge randomized trial design to evaluate the implementation of cryptococcal antigen (crag) screening and preemptive anti-fungal therapy of hiv-infected persons entering antiretroviral therapy (art) outpatient treatment in uganda. those who are art eligible with a cd4≤100 cells/mcl will have a serum/plasma crag performed by lateral flow assay. those who are crag-positive and asymptomatic will be treated with high dose fluconazole. after 6 months survival with retention-in-care will be compared between those who are crag+ and crag negative


Eligibility criteria:
inclusion criteria: * hiv-1 infection * cd4≤100 cells/mcl * cryptococcal antigen (crag) positive * age \>14 years exclusion criteria: * suspected cryptococcal meningitis * prior known history of cryptococcal meningitis * currently receiving hiv antiretroviral therapy * allergy to any azole antifungal medication * persons with known serious hepatic co-morbidities, transaminitis, or clinical jaundice who should not receive fluconazole in the opinion of the study investigator. * current known pregnancy

Keywords: cryptococcal meningitis hiv aids implementation science stepped wedge design retention-in-care
natural history of types 2 and 3 sma in taiwan

Conditions: Spinal Muscular Atrophy

Summary: the primary objective of this study is to investigate the natural history of spinal muscular atrophy (sma) types 2 and 3 patients in taiwan. this study will provide further insights into the clinical course sma. several analyses will be conducted regarding overall survival, demographic characteristics, motor function, respiratory and nutritional support, and genotype and phenotype correlation.

as with other rare diseases, individual groups of sma have therefore opted to share patient information in the form of clinical sites to increase the overall patient cohorts on which clinical outcomes and new assisted-healthcare technologies can be assessed. using the collaborative and retrospective study of types 2 and 3 sma patients in taiwan, the investigators aim to 1) characterize the correlation of genotype and phenotype, 2) correlate the onset, progression, management with disease outcome, 3) depict comorbidity and within type 2 and 3 sma patients with different smn2 copy number.

Eligibility criteria:
inclusion criteria: 1. patients are diagnosed with sma types 2 or 3 2. generalized hypotonia and muscle weakness, weakness of the legs is greater than the arms, and the proximal part is weaker than distal part of extremities. 3. smn1 gene deletion or mutation and/or neurogenic changes in electromyogram and/or muscle pathology. exclusion criteria: 1. non-5q sma (no deletion or mutation of smn1 gene) patients. 2. type 1 sma patients.

Keywords: spinal muscular atrophy natural history
physical activity choices everyday

Conditions: Obesity

Summary: this study aims to test two strategies for weight loss maintenance. it involves two phases, a weight loss phase and a maintenance phase. during phase i (the weight loss phase), participants will receive a 16-week, web-based behavioral weight loss program that involves access to weekly weight loss information and weekly personalized feedback on diet, activity, and weight loss goals. individuals who lose at least 5% of their initial body weight during this program, will be invited to participate in phase ii. during phase ii, two 4-month treatments for weight loss maintenance will be tested. at the beginning of phase ii, participants will be randomly assigned to one of the two maintenance programs: (1) a behavioral maintenance program that involves in-person group meetings plus daily exercises in which information about a healthy lifestyle is reviewed or (2) a behavioral maintenance program that involves in-person group meetings plus daily exercises in which descriptions of positive future events are reviewed. throughout the study, participants will complete assessments that examine the effects of the interventions on delay discounting, physical activity, weight, and other important health and psychosocial outcomes.


Eligibility criteria:
inclusion criteria: * age between 18-70 * body mass index between 30-50 * english speaking * have a smartphone that can be used for study activities exclusion criteria: * report being unable to walk 2 blocks without stopping * are currently participating in weight loss treatment, have a history of bariatric surgery, or lost ≥5% in the past 6-months * are pregnant or plan to become pregnant within 1 year * report a heart condition, chest pain during periods of rest or activity, or loss of consciousness on the physical activity readiness questionnaire * report a medical condition that could jeopardize their safety in a weight control program with diet and exercise guidelines * history of or current traumatic brain injury, dementia or alzheimer's disease; a history of multiple sclerosis, or other neurological disorders * report conditions that, in the judgment of the pi, would render them unlikely to follow the protocol (e.g., relocation, dementia, unable to read and write in english) * have no internet access or unwilling to use personal smartphone for study.

Keywords: weight loss maintenance
molecular aspects of preimplantation genetic diagnosis for nf1

Conditions: Neurofibromatosis Type 1

Summary: neurofibromatosis 1 (nf1) is a multisystem disorders characterized by skin abnormalities, such as café-au-lait spots and neurofibromas, learning disabilities, skeletal anomalies and vascular complications. experience learns that this disorder is a great burden for patients. nf1 is an autosomal dominant disorder with 50% risk of transmission. the penetrance is nearly 100%, but the expression varies greatly even within families, which makes it nearly impossible to predict severity in offspring. preimplantation genetic diagnosis (pgd) is a reproductive option for couples at risk of transmitting nf1 to their offspring. we perform a retrospective and observational multicentric study in the maastricht university medical center, the university hospital of brussel and strasbourg university hospital. our specific (and first) goal is to evaluate the molecular aspects of pgd for nf1 in an international cohort of couples requesting pgd for nf1. about 50% of the patients with nf1 have a de novo mutation that can complicate development of a pgd test. earlier studies from 1990 and 1992 have shown that de novo nf1 mutations usually occur on the paternal allele. we want to confirm these findings with collected data from our cohort. the high incidence of de novo mutations results in a higher chance of finding mosaicism in patients or their parents. as a result of this, it can become apparent during pgd test preparation that pgd treatment is no longer possible or indicated. the investigators will evaluate these aspects of pgd for nf1 in our cohort. they are also interested, as a second goal, in other aspects of pgd treatment for nf1, such as the success rate in thier cohort. they expect the success rate to be the same as for other autosomal dominant disorders


Eligibility criteria:
inclusion criteria: * major subjects (≥18 years old) * couples who have been treated at the hus for a preimplantation diagnosis request for type 1 neurofibromatosis (confirmed by a molecular molecular analysis) and who have benefited from preliminary analyzes, between 01/01/2004 and 30/06 / 2021 * couple who, after receiving information, did not express their opposition to the reuse of their data for the purposes of this research exclusion criteria: * couples who expressed their opposition to participating in the study * subject under guardianship, curatorship or safeguard of justice

Keywords: neurofibromatosis type 1 nf1 preimplantation genetic diagnosis de novo mutation mosaicism molecular test
short and prolonged conservative treatment in patients with adhesive intestinal obstruction

Conditions: Small Bowel Obstruction; Small Bowel Adhesion

Summary: this study will compare the results of 24 h conservative treatment and n h conservative treatment of adhesive small bowel obstruction (where n = 72 h minus duration of intestinal obstruction).

patients with acute adhesive intestinal obstruction, who dont need an emergency operation, will be given water insoluble contrast per os and randomized into two groups - early and delayed surgery. the first group will receive conservative treatment within no more than 24 h. duration of non-operative treatment in second group will be 72 h minus duration of intestinal obstruction, based on the onset of symptoms. in both groups surgery will be performed if contrast will not reach colon in mentioned terms or clinical deterioration signs will appear. the results of 24 h conservative treatment and n h conservative treatment (where n = 72 h minus duration of intestinal obstruction) will be compared.

Eligibility criteria:
inclusion criteria: * patients with acute intestinal obstruction exclusion criteria: * pregnancy; * peritonitis or strangulation, revealed during primary examination; * early acute small bowel obstruction (presence of anamnestic surgical intervention within 6 month)

Keywords: acute adhesive intestinal obstruction, conservative treatment
immunomodulatory effects of nb-uvb and steroids in vitiligo: a study on cxcl11 and temra biomarkers

Conditions: Vitiligo; NB-UVB

Summary: this is a prospective, randomized controlled trial (rct) investigating the immunological effects of narrowband uvb (nb-uvb) phototherapy-both as monotherapy and in combination with oral mini-pulse (omp) steroids-in non-segmental vitiligo patients. the study evaluates changes in cxcl11 (a chemokine involved in t-cell recruitment) and temra (terminally differentiated effector memory t cells re-expressing cd45ra) levels in serum before and after treatment.

design: randomized controlled trial (rct) with two parallel arms. duration: six months (including baseline and follow-up assessments). setting: outpatient dermatology clinic at assiut university hospital, egypt. objectives: compare the immunomodulatory effects of nb-uvb alone vs. nb-uvb + omp steroids in vitiligo patients. assess cxcl11 and temra levels as potential biomarkers for disease activity and treatment response. evaluate clinical repigmentation using vitiligo extent score (ves/vesplus). methodology: 40 patients with stable non-segmental vitiligo will be enrolled. 20 patients will undergo serum analysis for cxcl11 and temra via elisa. treatment arms: nb-uvb monotherapy (twice weekly). nb-uvb + omp steroids (dexamethasone 2.5-3 mg/week). follow-up: clinical and immunological assessments at baseline and 6 months.

Eligibility criteria:
inclusion criteria: clinically diagnosed non-segmental vitiligo (stable for ≥3 months). fitzpatrick skin types iii-v (common in the egyptian population). no recent systemic therapy for vitiligo (within the last 3 months). exclusion criteria: photosensitivity disorders (e.g., lupus, porphyria). pregnancy or breastfeeding. active infections, malignancies, or immunosuppressive conditions. history of hypertrophic scarring or melanoma.

Keywords: vitiligo nb-uvb cxcl11 temra