Kornimate/medical-research-clean huggingface dataset:
use of phenylbutyrate therapy for patients with pyruvate dehydrogenase complex deficiency.

Conditions: Pyruvate Dehydrogenase Complex Deficiency

Summary: in this study phenylbutyrate is used for patients with pyruvate dehydrogenase complex deficiency. the aim of the study is to investigate the safety and efficacy of therapy.

the investigator will evaluate the safety and efficacy of a 4-weeks treatment with sodium phenylbutyrate in patients with pyruvate dehydrogenase complex deficiency. efficacy will be evaluated based on biochemical endpoints (blood lactate and pyruvate).

Eligibility criteria:
inclusion criteria: 1. subject must be older than 3 months old and younger than 18 years old. 2. clinical diagnosis of pdc deficiency confirmed by dna testing showing a missense mutation in the pdha1 gene. 3. lactate concentration ≥ 2.5 mmol/l or ≥ 2 mmol/l, respectively in venous or arterial blood samples. 4. provision of signed and dated informed consent form by the parents/legal guardians of the patient 5. negative pregnancy test for women of childbearing potential, and agree to use effective form of contraception until 6 weeks post treatment. exclusion criteria: 1. frameshift or nonsense mutations of the pdha1 gene. 2. defects affecting any gene encoding pdc subunits other than pdha1 3. secondary forms of lactic acidosis (e.g. impaired oxygenation or circulation). 4. tracheostomy or requirement for artificial ventilation. 5. hyperlactatemia or organic acidosis associated with other metabolic disorders (e.g. biotinidase deficiency, primary disorders of gluconeogenesis, organic acidurias, primary defects of fatty acids oxidation) 6. evidence of hepatic insufficiency, renal insufficiency, edema with sodium retention, cardiac arrhythmia, congenital heart defects, hypertension, blood dyscrasia, symptomatic pancreatitis, or inflammatory bowel disease. 7. any clinical condition or medications known to significantly affect renal clearance. 8. any other condition that, in the opinion of the investigator, may compromise the safety or compliance of the patient or would preclude the patient from successful completion of the study. 9. known allergic reactions to components of the study agent. 10. treatment with another investigational drug or other intervention (including dca) or participation in a clinical study with an investigational drug within 6 months prior to enrolment. 11. pregnancy or lactation.
a study of wx390 combined with toripalimab in patients with advanced gastric-type endocervical adenocarcinoma with stk11 mutations

Conditions: Gastric Type Adenocarcinoma (GAS) With STK11 Mutation

Summary: the goal of this clinical trial is to evaluate the safety and preliminary efficacy of wx390 combined with toripalimab in patients with advanced gastric-type endocervical adenocarcinoma with stk11 mutations. the main questions it aims to answer are: * pharmacokinetic (pk) characteristics of wx390 combined with toripalimab treatment. * safety and preliminary in combined therapy. participants will be treated with wx390 orally and toripalimab intravenously, and follow the efficacy and safety evaluation according to the protocol.

this study will be an open-label, multicenter phase ii clinical trial. after being informed about the study and potential risks, all patients giving written informed consent will undergo a 28-days screening period to determine eligibility for study entry. and then patents will be administered for 8 cycles treatment and 8 weeks safety follow up after the last dose of treatment. the efficacy and safety measures will be conducted and collected every cycle.

Eligibility criteria:
inclusion criteria: * ≥18 years of age * histological or cytological confirmed advanced gastric-type endocervical adenocarcinoma. * eastern cooperative oncology group (ecog) performance status of 0-1 * life expectancy of more than 3 months * at least one measurable lesion according to recist 1.1 * adequate organic function * signed and dated informed consent exclusion criteria: * anti-cancer therapy within 30 days prior to the initiation of investigational treatment * major surgery within 30 days prior to the initiation of study treatment * received corticosteroids treatment or other immunodepressant within 2 weeks before the first dose of study treatment * toxicity from a previous anti-tumor treatment that does not return to grade 0 or 1 (except for alopecia) * patients who are suffering active interstitial lung disease * evidence of ongoing or active serious infection * history of human immunodeficiency virus (hiv) infection or active hepatitis b or c infection * inability to take medication orally * abuse of alcohol or drugs * people with cognitive and psychological abnormality or with low compliance * pregnant or lactating women
comparison of spo2 measurement accuracy of different types of smartwatches

Conditions: Hypoxia

Summary: the aim of the study is to compare the accuracy of peripheral blood oxygen saturation measurements using smartwatches from three manufacturers compared to a standard medical pulse oximeter.

the aim is to create a study that compares multiple smartwatch models simultaneously with a standard medical pulse oximeter using a single measurement methodology. according to the available literature, no such study exists. the methodology of the study and the subsequent statistical evaluation are based on the guidelines of the standard čsn en iso 80601-2-61, which specifies requirements for the basic safety and necessary functionality of pulse oximetry devices. the comparison will be performed non-invasively in healthy volunteers during gradual desaturation in the range of 70-100 % spo2 (normobaric hypoxia). the data obtained could help to increase the awareness of the possibilities of spo2 monitoring using smartwatches in medical conditions that are manifested by a decrease in spo2 level over a longer time period, where measurement with a conventional oximeter is impractical.

Eligibility criteria:
inclusion criteria: * passing the initial examination, which will determine the following data: wrist circumference, heart rate, blood pressure and blood oxygen saturation exclusion criteria: * pregnancy * severe cardiovascular conditions * severe asthma or other severe respiratory conditions * injury to the upper limbs or hands that could affect the peripheral perfusion * diabetes * hypotension or hypertension

Keywords: spo2 pulse oximetry smartwatch
confirmatory phase ii/iii study assessing efficacy, immunogenicity and safety of ic43

Conditions: Pseudomonas Aeruginosa Infection

Summary: this is a confirmatory, randomized, placebo-controlled, multi-center, double-blinded phase ii/iii study. the study population consists of male or female intensive care unit (icu) patients with a need for mechanical ventilation for more than 48 hours, aged between 18 and 80 years.

this is a confirmatory, randomized, placebo-controlled, multi-center, double-blinded phase ii/iii study. the study population consists of male or female intensive care unit (icu) patients with a need for mechanical ventilation for more than 48 hours, aged between 18 and 80 years. eight-hundred patients will be enrolled at approximately 50 study centers. informed consent (i.e., from the patient or from the patient's legally authorized representative) or waiver will be obtained according to regional requirements prior to any study related procedures. patients will be randomized to receive either ic43 100 mcg or placebo and will receive the first vaccination on day 0. the second vaccination will be applied on day 7. in case icu discharge occurs before day 7, immunization will be done at the hospital ward.

Eligibility criteria:
inclusion criteria: * male or female patients admitted to an intensive care unit (icu) with need for mechanical ventilation for at least 48 hours, aged between 18 and 80 years at visit 0 * written informed consent or waiver according to the national regulations * no childbearing potential or negative pregnancy test exclusion criteria: * sequential organ failure assessment (sofa) \< 4 on day 0 * patients \<6 months post organ transplantation * readmission to icu during the current total hospital stay on day 0 * patients admitted to icu within 2 days after surgery * patients admitted to icu due to trauma * elective surgery until day 28 after first vaccination

Keywords: ic43 pseudomonas aeruginosa mechanically ventilated icu patients icu patients
review of pain management after congenital cardiac surgery

Conditions: Postoperative Pain Management

Summary: one of the main problems that faces children undergoing congenital cardiac surgery is posoperative pain management. pcas (patient controlled analgesia) and ncas (nurse controlled analgesia) are the main means of pain management here at nch. in this study, the investigators will review the difference between different pca and nca modalities. also, the investigators aim to find out if there are differences between age groups in terms of type of drug used, total drug used, side effects and length of use of the pca or nca.

all patients that underwent congenital cardiac surgery from 2004 till 2010 and required pca or nca will be included. data will be collected from the pain team database and charts review. data will include; patient's age, weight, sex, procedure, pain scores and sedation score during the times of pca and nca use, side effects (respiratory depression, nausea, vomitting, pruritus, itching and other).in addition we will record the total days of pca and nca use. we will also look at the incidence of changes done in the pca/nca and drug itself. identifying the ideal drug and dose based on the patient's age and procedure will allow us to take better care of our patients. our patient population is peculiar in the fact that a big percentage of our patients are extubated in the operating room. for this reason, sedation and analgesia play an important role for our continued success in taking care of them.

Eligibility criteria:
inclusion criteria: * all patients that underwent congenital cardiac surgery from 2004 till 2010 and required pca or nca. exclusion criteria: * all patients that underwent congenital cardiac surgery from 2004 till 2010 and did not receive pca or nca for postoperative pain management.

Keywords: congenital cardiac surgery postoperative pain management
exacerbation of erythema following pdt in patients with probable capillary malformation-arteriovenous malformation syndrome: retrospective analysis of ultrasound and clinical characteristics.

Conditions: Probable Capillary Malformation-Arteriovenous Malformation Syndrome

Summary: this study aims to reduce the misdiagnosis rate of pwss and improve the diagnosis of cm-avm using ultrasound. furthermore, we seek to provide more evidence-based recommendations for the treatment of patients with probable cm-avm syndrome, particularly those presenting with erythema.

background: exacerbation of erythema following photodynamic therapy (pdt) in patients with probable capillary malformation-arteriovenous malformation (cm-avm) syndrome, may be attributed to misdiagnosis as port-wine stains (pwss). objective: to identify the ultrasound features of patients with exacerbation of erythema following pdt for a definite diagnosis. methods: a retrospective single-center study was conducted on pws patients with exacerbation of erythema following pdt. the clinical and ultrasonic features were reviewed and analysed. one low-frequency and two high-frequency ultrasound (hfus) devices were used to examine the lesions and the contralateral normal skin. results: the clinical characteristics of 16 patients were consistent with those of high-flow vascular stains (hfvs). ultrasound findings revealed skin and hypodermis thickening, increased blood flow signals with an arteriovenous malformation (avm) waveform and deeper, larger blood vessels in the dermis and hypodermis of pink macules (pms) compared to normal skin. in the absence of genetic diagnosis, pws patients with exacerbation of erythema following pdt were diagnosed as having probable cm-avm syndrome. limitations: retrospective single-center design and small sample size. conclusion: patients with pwss are advised to undergo low-frequency ultrasound examinations to avoid misdiagnosis. hfus can serve as a complementary method to promptly identify underlying avms beneath the pms. we do not recommend pdt treatment for patients with probable cm-avm syndrome.

Eligibility criteria:
inclusion criteria: * patients who were greater than or equal to 1 year old at the time of their first hmme-pdt treatment. * patients who experiencing exacerbation of clinical symptoms, such as darker coloration than before treatment. * patients who have undergone one or more hmme-pdt treatments. ④ patients with complete basic information and treatment-related information.

Keywords: exacerbation of erythema following pdt probable cm-avm syndrome ultrasound retrospective study
nasal budesonide efficacy on nasal feno in children with allergic rhinitis

Conditions: Allergic Rhinitis

Summary: in clinical trials for treatment of allergic rhinitis a significant reduction of the total symptom score compared to baseline has been demonstrated by using nasal budesonide.previous results in adults show that the assessment and monitoring del nasal fractional exhaled nitric oxide (nfeno) is useful in controlling inflammation of nasal allergic rhinitis. primary objective of this study is to evaluate efficacy of nasal budesonide (aqueous solution) on the nasal inflammation marker (nfeno). secondary outcomes are the evaluation of: changes in total nasal symptom score (total symptom score, t5ss), changes in cell counts in nasal lavage (ln) and the changes reported sleep quality (pittsburgh sleep quality index, psqi).


Eligibility criteria:
inclusion criteria: * history of allergic rhinitis for at least 1 year * children aged 6 - 14 years * total symptom score (t5ss) more than 6 in the last 4 days before the screening visit exclusion criteria: * signs of acute respiratory infection * systemic immunological and metabolic disease * major malformations of the upper airways * topical or systemic therapy with antibiotics, antihistamines and corticosteroids in the 30 days prior to the study * patient active smoker
the role of il-23 in chronic inflammatory disease: exploring the cellular and molecular targets of il-23 signaling in peripheral and axial spondyloarthritis

Conditions: Spondyloarthritis

Summary: this is a research study involving humans, of the interventional type with minimal risks and constraints (riph2). it is a multicentric, non randomized prospective study aiming to better understand the mechanisms of the response to anti-il-23 biologics in spondyloarthritis patients attending the rheumatology department of cochin, saint-antoine, henri-mondor hospitals (aphp) and maison-blanche hospital (reims).

the aim of this project is to improve our understanding of the role of il-23 in the pathophysiology of axial spa and peripheral spa. this objective is detailed in three specific aims: 1. define the effects of il-23 on gene expression and cytokine production in innate and adaptive t lymphocytes from patients with spa, and correlate them with the patient's genotype; 2. phenotypically characterize immune cell populations in peripheral blood and in synovial fluid from peripheral spa patients and identify at the single cell level the cells expressing the il-23 receptor and/or producing il-17. the study population to be included are patients affected by spa, attended to in the rheumatology departments of cochin hospital, saint-antoine hospital henri-mondor hospitals in paris (aphp) and maison-blanche hospital in reims. participants will be divided into two groups: group 1 comprises patients diagnosed with axial spa, group 2 spa patients with peripheral spa or psoriatic arthritis.

Eligibility criteria:
inclusion criteria: * age : adults (\>18 years) * satisfying asas diagnostic criteria for spa * patient has active disease, defined by the presence of active synovitis, tendinitis, or dactylitis or significant inflammatory pain of the spine, judged by the examining clinician to be due to spa. * informed consent signed * beneficiary of health insurance, except for the ame only for patients of group 1 • patient is naïve to biological therapies only for patients of group 2 * patient is affected by peripheral spa (asas criteria) or psoriatic arthritis, with inflammation of peripheral joints * patient requires aspiration, as part of standard care non inclusion criteria: * patient is minor * patient is pregnant or breastfeeding * patient is immunocompromised * patient has received biological therapy with 2 or more biologics * patient is receiving corticosteroid treatment \> 10 mg per day * patient is under legal protection, curators, guardianship * patient refuses consent * previous history of alcoholism, drug addiction, psychological problems, severe concomitant conditions that could invalidate the patient's consent or limit the patient's compliance to the treatment protocol. * beneficiary of the ame only for group 1 • patient has received biological therapy

Keywords: interleukin-23 anti-il-23 therapy immune response spondyloarthritis
prospective study on primary aldosteronism in resistant hypertension

Conditions: Resistant Hypertension; Primary Aldosteronism; Refractory Hypertension; Hypertensive End-Organ Damage; Atrial Fibrillation; Aortic Ectasia

Summary: prevalence of primary aldosteronism (pa) in resistant hypertension is not clear. in addition, emerging evidence supports the role of elevated serum aldosterone in promoting cardiovascular disease, independently from high blood pressure (bp) levels, but current data on this issue are heterogeneous.

pa is the most frequent form of secondary hypertension, with a prevalence that increases with the severity of hypertension. the wide variation of the reported pa prevalence is due to different study design and population. very few data derive from well designed prospective study. additional problems in the interpretation of study results are the different diagnostic cut-off used in various centers and the low diffusion of the adrenal vein sampling, that has a central role in the pa diagnosis. resistant hypertension (rh) is a condition of insufficient bp control, despite appropriate lifestyle measures and treatment with at least 3 drugs at full dose, including a diuretic, in patients whose adherence to therapy has been confirmed. the primary aim of our study is define prospectively the prevalence of pa in rh. moreover, emerging evidence supports the crucial role of elevated serum aldosterone in promoting cardiovascular disease, independently from high bp levels. aldosterone improves oxidative stress, inflammation, impairs insulin metabolic signaling, reduced endothelial-mediated vasorelaxation and is associated to cardiovascular and renal abnormalities. however, current data on the contribution of pa on cardiometabolic complications have heterogeneous results. the secondary outcome of our study is to investigate prospectively the association of pa with cardiometabolic complications in a cohort of patients with rh.

Eligibility criteria:
inclusion criteria: * age over 18 and under 80 years old; * diagnosis of resistant hypertension defined as: uncontrolled blood pressure at ambulatory blood pressure measurement (abpm), despite the use of at least 3 antihypertensive drugs at full dose, including a diuretic. exclusion criteria: * age under 18 or over 80 years old; * pseudo-resistant hypertension (poor medication adherence, high salt intake); * previous cardiovascular disease; * insulin treated diabetes mellitus; * other than primary aldosteronism cause of secondary hypertension (obstructive sleep apnea, renal artery stenosis, pheochromocytoma/paraganglioma, primary hyperparathyroidism, autonomous cortisol secretion or over hypercortisolism); * liver cirrhosis; * chronic heart failure; * known malignant neoplasm; * chronic disease with major organ involvement; * excessive alcohol ingestion; * current steroids assumption; * use of sympathomimetic drugs; * use of contraceptives.

Keywords: resistant hypertension primary aldosteronism renin-angiotensin system blood pressure cardiometabolic risk early atherosclerotic damage oxidative stress
the effect of plyometric training on balance and proprioception in different branches athletes

Conditions: Athlete Foot

Summary: the aim of this study is to investigate the effect of plyometric training on balance and proprioception in athletes in different branches. 54 athletes participated in the study. two groups were included as 27 athletics and 27 karate groups. plyometric training was applied to the athletes participating in our study, 2 days a week for 6 weeks, after 20 minutes of warming up and stretching movements before each training. sociodemographic characteristics of the athletes were recorded. dynamic balance y balance test and static balance stroke balance test; sense of proprioception was evaluated with angle reproduction test.


Eligibility criteria:
inclusion criteria: * being a licensed athlete in the province of tokat * being an athlete who trains regularly * to be healthy, between the ages of 12 and 18 * volunteering to work. exclusion criteria: * presence of any disturbance that may affect balance and balance reactions, * musculoskeletal injury in the last 6 months, * presence of previous musculoskeletal surgery, * pregnancy, * having an acute or chronic illness or injury that will limit performance in training or a match, * calcification or other bone and joint problems, * using cigarettes, alcohol, and drugs that affect balance.

Keywords: plyometrics balance proprioception
efficacy and safety study of budesonide to treat oral chronic graft vs host disease (cgvhd)

Conditions: Oral Chronic Graft vs Host Disease

Summary: the purpose of the study is to compare the efficacy and tolerability of budesonide 3 mg effervescent tablet (9 mg/day) compared to placebo for the treatment of patients with resistant oral cgvhd.


Eligibility criteria:
inclusion criteria: * karnofsky \>= 70 * oral chronic gvhd after allogeneic haematopoietic stem cell transplantation * oral cgvhd of erosive and/or ulcerative type * nih scale \>= 3 * resistant oral cgvhd with no oral response to conventional primary treatment exclusion criteria: * uncertain diagnosis of resistant oral cgvhd * symptomatic oral cgvhd of hyperkeratotic type solely * current active oral bacterial, viral, or fungal infection * unwilling to forego concurrent treatment for mucosal lesions and/or related oral pain * requiring addition of new systemic therapy including steroids, or radiation therapy * local intestinal infection * abnormal hepatic function or liver cirrhosis * if careful medical monitoring is not ensured: tuberculosis, cardiovascular disease, diabetes mellitus, osteoporosis, active peptic ulcer disease, glaucoma, cataract, infection * second line treatment of oral cgvhd with topical steroids