Kornimate/medical-research-clean huggingface dataset:
reliability and validity of the turkish version of the pedhal

Conditions: Hemophilia; Hemophilia A, Severe; Hemophilia B

Summary: this study aimed to establish the validity and reliability of the turkish version of the pediatric haemophilia activities list (short - pedhalshort) questionnaire.

hemophilia is a blood disease characterized by a bleeding disorder that develops due to the deficiency of clotting factor viii (hamophilia a) or coagulation factor ix (hamophilia b) proteins that provide clotting in the blood. in the hereditary disease showing x-linked recessive inheritance, female individuals are carriers and male individuals show a clinical picture. although the clinical picture changes depending on the duration and frequency of bleeding, the severity of the disease is classified as severe, moderate and mild. musculoskeletal problems are the most common complications in patients with severe and moderate hemophilia. hemarthroses due to bleeding in the joint (80%) and hematomas due to intramuscular bleeding (20%) cause joint degeneration and muscle atrophy. recurrent hemarthroses occur when there is not enough clotting factor in the setting, often before the previous hemarthrosis has resolved. this creates a vicious cycle of bleeding-inflammation-rebleeding known as the "target joint". the resulting vicious circle causes permanent damage to bone and cartilage structures and causes "arthropathy". the knee is the joint region where hemarthrosis and arthropathy are most common, followed by the elbow and ankle joints. since these joints in the musculoskeletal system contain more synovial tissue than the others, they are more exposed to trauma and load and are injured. as a result of all these, pain, joint limitations and muscle strength losses occur. all these clinical findings lead to a decrease in functional independence in activities of daily living with the restriction of physical activity in patients. in the literature, there are questionnaires and measurement methods in which many functional evaluations are made for hemophilia patients. however, most of them have not been validated and reliable in turkish. for this purpose, our study aimed to establish the validity and reliability of the turkish version of the pediatric haemophilia activities list (pedhalshort) questionnaire.

Eligibility criteria:
inclusion criteria: * having been diagnosed with hemophilia by a physician (factor viii-ix) * ages 4-17 years old * receiving regular prophylactic treatment * children who volunteered to participate in the study and whose family consent was given exclusion criteria: - children with neurological signs that limit activities of daily living

Keywords: functional independence activities of daily living hemophilia factor viii factor ix
research and characterization of new genes involved in intellectual disability

Conditions: Intellectual Disability

Summary: intellectual disability (id) occurs in 2 to 3 % of the general population but the cause is identified only in 30 to 60% of cases. the purpose of this study is to indentify genes involved in id with new genetics tools (snp-arrays, next generation sequencing...) and establish genotype-phenotype correlations.


Eligibility criteria:
inclusion criteria: * for the patients: clinical diagnosis of intellectual disbility * for the unaffected sibs: to be aged at least 3 years * informed consent exclusion criteria: * absence of informed consent

Keywords: intellectual disability autism spectrum disorders gene next generation sequencing
the role of low molecular weight heparins in hepatocellular carcinoma

Conditions: Hepatocellular Carcinoma

Summary: hepatocellular carcinoma (hcc) is a major tumor type worldwide, especially in china as the sequence of hepatitis b and liver cirrhosis. activation of the coagulation system occurs commonly in patients with malignancy. several studies have suggested that anticoagulant therapy may improve survival in patients with malignancy. the low molecular weight heparins (lmwhs) lend themselves to such studies because of their effects in experimental models of malignancy and the relative ease of administration compared with unfractionated heparin. the purpose of the present rct was to determine whether addition of lmwh to transarterial chemoembolization (tace) would improve hcc patient outcome compared with tace alone.

100 patients will be randomly assigned to receive either tace alone or tace plus lmwh. a block of every 4 participants and a stratified randomization according to portal vein cancer emboli will be used to restrict randomization. lmwh consisted of nadroparin ca will be given at a dose of 4100 u twice daily during 6 weeks after tace. the time to progression(ttp) and overall survival within two years will be used to evaluate the effect of lmwh on hcc.

Eligibility criteria:
inclusion criteria: 1. adults patients with a diagnosis of hcc which is not amenable to surgical resection, liver transplantation or local ablative therapy 2. without metastasis out of liver 3. patients must have at least one tumor lesion that meets both of the following criteria: 1. the lesion can be accurately measured in at least one dimension according to recist criteria 2. the lesion has not been previously treated with surgery, radiation therapy, radiofrequency ablation, percutaneous ethanol or acetic acid injection, or cryoablation. 4. ecog performance status (ps) \<2 5. no prior targeted antiangiogenic therapy. metronomic chemotherapies are allowed. at least 4 weeks since prior systemic chemotherapy 6. child-pugh class a or b 7. no significant renal impairment (creatinine clearance \< 30 ml/minute) or patients on dialysis 8. ability to understand the protocol and to agree to and sign a written informed consent document - exclusion criteria: 1. hbsag(-)，afp(-). 2. prothrombin time prolonged more than 4s. 3. blood platelets count less than 50000/l. 4. renal failure requiring dialysis. 5. child-pugh class c hepatic impairment. 6. clinically significant gastrointestinal bleeding within 30 days prior to study entry. 7. history of organ allograft. 8. substance abuse (current), psychological, or social conditions that may interfere with the patient's participation in the study or evaluation of the study results. 9. known or suspected allergy to the investigational agents or any agent given in association with this trial. 10. pregnant or breast-feeding patients.

Keywords: liver neoplasms liver diseases digestive system diseases digestive system neoplasms carcinoma, hepatocellular gastrointestinal neoplasms adenocarcinoma hepatocellular carcinoma neoplasms transarterial chemoembolization nadroparin low-molecular-weight heparin chemoembolization
evaluation of the association between cyp2d6 genetic polymorphisms and the treatment effect of tamoxifen

Conditions: Breast Cancer; Metastatic Disease

Summary: primary objectives of this study is to evaluate the effects of cyp2d6 genotypes on time to progression after tamoxifen treatment in pre- or postmenopausal women with metastatic breast cancer. furthermore, we will evaluate the effects of cyp2d6 genotypes on clinical benefit and response duration to tamoxifen administration in pre- or postmenopausal women with metastatic breast cancer and also evaluate the effects of cyp2d6 genotypes on the steady state plasma concentration of tamoxifen and its metabolites


Eligibility criteria:
inclusion criteria: * histologically or cytologically diagnosed stage iv or recurrent breast cancer patients according to american joint committee on cancer (ajcc) * positive estrogen receptor or positive progesterone receptor. * females at least 18 years of age. * prior radiation therapy is allowed as long as the irradiated area is not the only source of measurable disease * prior hormone therapy less than 2. * no history of megace medication for recent 28 days * performance status of 0, 1 and 2 on the ecog criteria * clinically measurable disease, defined as bidimensionally measurable lesions with clearly defined margins on x-ray, ct scan, mri or physical examination. lesions serving as measurable disease must be at east 1 cm by 1 cm, as defined by x-ray, ct scan, mri, or physical examination * bone only or pleural fluid only disease is included as long as evaluation for clinical benefit is possible * estimated life expectancy of at least 12 weeks * compliant patient who can be followed-up adequately. * adequate hematologic (wbc count 3,000/mm3, platelet count 100,000/mm3), hepatic (bilirubin level 1.8 mg/dl, ast, alt 1.5xuln, albumin 2.5 g/dl), and renal (creatinine concentration 1.5 mg/dl) function. * informed consent from patient or patient's relative * childbearing women should use non-hormonal contraceptive method exclusion criteria: * active or uncontrolled infection. * second primary malignancy (except in situ carcinoma of the cervix or adequately treated basal cell carcinoma of the skin or prior malignancy treated more than 5 years ago without recurrence).
repeat ivermectin mass drug administrations for malaria control ii

Conditions: Malaria

Summary: rimdamal ii is a double-blind, cluster randomized trial in burkina faso designed to test whether repeated ivermectin mass drug administrations, integrated into a monthly delivery platform with standard malaria control measures of seasonal malaria chemoprevention and insecticide-treated bed net distribution in the sahel, will reduce childhood malaria incidence.

the rimdamal ii trial is designed to determine the efficacy of adding seasonal ivermectin mass drug administrations to the standard-policy malaria control measures in the sahel (seasonal malaria chemoprevention in children, maximum long-lasting insecticidal net coverage, intermittent preventive treatment in pregnancy), for reducing the incidence of uncomplicated malaria episodes in enrolled village children (≤ 10 years of age) assessed by active case surveillance. the investigators will also examine the safety of the intervention, as well as entomological and parasitological endpoints. this is a double-blind, cluster randomized trial in that will occur in villages in southwestern burkina faso over two consecutive rainy seasons. for the intervention, mass administration of ivermectin or placebo will be given monthly over 4 months of each rainy season to the eligible village population, each as 3-day course of 300 µg/kg/day. these mass drug administrations will occur simultaneously with the distribution of seasonal malaria chemoprevention drugs on the same monthly schedule to eligible children aged 3-59 months.

Eligibility criteria:
inclusion criteria (for being enrolled in the study): * residence in selected study village * able to understand the information and willing to give consent or assent (age 12-18) and parent/guardian consent if study participant age is \< 18 years of age. exclusion criteria (for participating in the intervention \[ivermectin or placebo mda\]): * residence outside of the study village * height \< 90 cm (\*note: if subject becomes ≥90cm over course the trial, this exclusion criteria will no longer be valid in subsequent mda) * current treatment with sp+aq as part of smc (restricted to children 3-59 months old) (\*note: if subject discontinuous sp+aq treatment because they become older than 59 months over course the trial, this exclusion criteria will no longer be valid in subsequent mda) * permanent disability or serious medical illness that prevents or impedes study participation and/or comprehension * pregnancy (screened for in women of child-bearing age \[ages 15-45\] using a pregnancy urine rapid test \[e.g. sd bioline hcg\] the week prior to each mda) * breast feeding if infant is within 1 week of birth * known allergy to ivermectin * possibility of loa loa infection as assessed by travel history to angola, cameroon, chad, central african republic, congo, democratic republic of congo, equatorial guinea, ethiopia, gabon, nigeria, and sudan. * enrolled in any other active clinical trials

Keywords: mosquito ivermectin anopheles plasmodium
efavirenz-based versus nevirapine-based antiretroviral therapy among hiv-infected patients receiving rifampin

Conditions: HIV Infections; Tuberculosis

Summary: the randomized controlled trial is conducted among antiretroviral naive co-infected hiv and tuberculosis patients who receiving rifampicin-based antituberculous regimen fro at least 4 weeks butt not exceed 16 weeks before enrolment. all patients receive the same backbone regimen of stavudine (30 mg/40 mg twice daily)+ lamivudie 150 mg twice daily. they are randomized to receive nevirapine 400 mg/day twice daily vs efavirenz 600 mg/day at bed time. all patients are followed through 144 weeks after initiation of antiviral therapy. the primary objective are to compare the proportion of patient who achieve undetectable plasma hiv-1rna\<50 copies/ml at week 48. the previous reports demonstrated that the standard doses of both nevirapine and efavirenz coulde be used among co-infected hiv and tuberculosis patients who receiving rifampicin even though plasma levels are somewhat reduced by rifampicin. however, there have been not been a randomized control trial to compare these two regimens. thus, this trial will provide the efficacy data between these two regimens.


Eligibility criteria:
inclusion criteria: * age 18-60 years * positive serology for hiv-1 * naïve to antiretroviral therapy * baseline cd4 cell counts \<250 cells/mm3 * diagnosed active tuberculosis by clinical features and/or positive acid fast stain and/or positive culture * received rifampicin at least 4 weeks but not exceed 16 weeks prior to enrollment * willing to participate and sign inform consent exclusion criteria: * aspartate transferase enzymes (ast) or alanine transminase enzyme (alt) \>5 times of upper limit * total bilirubin \>3 times of upper limit * serum creatinine) \>2 times of upper limit * pregnancy or lactation * receiving immunosuppressive drugs * receiving any drugs that may have drug-drug interaction with nevirapine and rifampicin * chronic alcoholic drunken and intravenous drug users * previously received single dose of nevirapine to prevent mother to child transmission * positive for serum cryptococcal antigen

Keywords: hiv tuberculosis efavirenz nevirapine rifampicin co-infected hiv and tuberculosis patients
long term results of amniotic membrane transplant in bullous keratopathy patients

Conditions: Bullous Keratopathy

Summary: a retrospective study including 22 patients who have undergone amniotic membrane transplant in our unit up till 2016. confocal microscopy and anterior segment optical coherence tomography (asoct) were performed to assess the retention of amniotic membrane and to detect any corneal structural changes. comparison was made with 5 controls who had bullous keratopathy awaiting endothelial keratoplasty.

this retrospective cohort study (crec ref no.: cre-2013.687) was approved by the joint chinese university of hong kong-new territories east cluster clinical research ethics committee. all patients who had amt done due to symptomatic bullous keratopathy at the prince of wales hospital and alice ho miu ling nethersole hospital (from 1998 onwards till june 2016) were invited back for confocal microscopy and anterior segment optical coherence tomography (asoct) at the clinic by the principal investigator (gs) from october to november 2016.

Eligibility criteria:
inclusion criteria: * all patients who have undergone amniotic membrane transplant in our cluster up till 2016. * patients who have bullous keratopathy on conservative treatment such as lubricants, bandage contact lens exclusion criteria: * patients who refuse to participate in the study, have passed away or cannot return for the clinical assessment will be excluded.
effect of broccoli sprout extract in patients with chronic kidney disease with diabetes type 2

Conditions: Chronic Kidney Disease Stage 3B; Chronic Kidney Disease stage4; Diabetes Mellitus, Type 2

Summary: this research project aims to test if sulforaphane, administered as broccoli sprout extract (bse) can ameliorate glucose control in adult patients with chronic kidney disease (ckd) and dm 2 with gfr \> 15 \ 18 years old. patients will be randomized into bse group or placebo group. both groups will be followed for 20 weeks: the first 12 weeks patients will receive the bse or placebo and, the next 8 weeks, both groups will be followed with no intervention to observe the changes in the primary and secondary outcomes. patients randomized to bse group will receive 50 µmmol/day of sulforaphane administered as bse (lantmännen®) from week 0 to week 4. if no side-effects are reported, the sulforaphane dose will increase to 100 µmmol/day from week 5 to week 8 and in the absence of side-effects, the dose will increase to 150 µmmol/day from week 9 to week 12. blood and urine samples and ogtt (in non-insulin dependent patients) will be performed at week 0, 12 and 20. on week 4 and 8 blood drawn for partial exam will be performed. the bse and the placebo (maltodextrin sprayed with copper-chlorophyllin) will be administered as powder provided in a double-blind manner as dry mixtures in sealed portion size bags of similar shape and size. randomization will be done using a computer-based block randomization algorithm. comparisons between the primary and secondary studied variables will be done with two-way analysis of variance (anova) with repeated measures for normally distributed variables. variables that can interfere with the glycemic control, such as changes in the dosage of hypoglicemiants agents and insulin during the intervention will be controlled in the analysis. those non-normally distributed will be log transformed aiming to normalize the distribution. all test will consider a p\<0.05 for statistical significance. the software stata will be used for the statistical analysis.


Eligibility criteria:
inclusion criteria: * patients with a gfr 15-45 ml/min/1.73 m2, dm type 2, age \>18 years old, able to read and understand swedish. exclusion criteria: * use of metformin, use of warfarin, levels of asat, alat more than three times the upper limit at screening or at any subsequent visit, kidney transplantation, inflammatory bowel disease, celiac disease, malignant diseases (except skin basalioma) in the previous 3 years, and any other condition that the treating doctor believes is contraindicated; allergy to broccoli; participation in another clinical trial which may affect the outcome of the present study; not to understand the study information.

Keywords: sulforaphane nutrition glucose control
comparative study of the efficacy of sarna sensitive lotion for treatment of uremic itch in adult hemodialysis patients

Conditions: Pruritis

Summary: the purpose of this research study is to evaluate the efficacy of sarna sensitive lotion in the treatment of uremic pruritus in adult hemodialysis patients in a double-blind controlled comparative trial.

this was a 4-week, randomized, double-blind, controlled study. fourteen subjects received treatment lotion (1% pramoxine hcl) and the remaining 14 received a bland emollient (cetaphil lotion). a target lesion limited to one anatomic site, excluding face and genitals, was selected at baseline. each subject was instructed to apply lotion twice daily to all affected areas of pruritus for four weeks. the use of any other topical or systemic medication to treat uremic pruritus was not permitted while participating in the study. subjects were clinically evaluated for erythema, xerosis, lichenification and overall severity at baseline, week 1, and week 4 (end of study)

Eligibility criteria:
inclusion criteria: * male or female patients 18 years of age to 70 years of age. * a diagnosis of moderate to severe pruritus. * at least 3 episodes of itch over a period of 2 weeks, each lasting for 2 minutes or more. * symptoms of itch in regular pattern over 6 months. * itch visual analog scale (vas) of 3cm or more out of 10 cm. * all subjects will be end stage renal failure patients undergoing hemodialysis for at least 3 months, who have substantial pruritus for more than 6 months. substantial pruritus is defined as persistent pruritus impairing sleep or daytime activity. * ability and willingness to follow all study procedures, attend all scheduled visits, and successfully complete the study * the ability to understand and sign a written informed consent form, which must be obtained prior to treatment. exclusion criteria: * presence of infection (as defined by the investigator) on the area to be treated. * subjects with history of pruritus predating renal failure and subjects with skin disease unrelated to uremia, such as atopic dermatitis, will be excluded. * use of systemic medications for treatment of pruritus including corticosteroids within the past 4 weeks and during the study. * use of topical medications for treatment of pruritus, including corticosteroids, within the past week. * pregnant women, women who are breast feeding, or women of child bearing potential who are not practicing an acceptable method of birth control (abstinence, birth control pill/patch, barrier with spermicidal jelly, iud, etc.), as determined by the investigator. acceptable contraception must be used during the entire study.

Keywords: uremic pruritis
a multicenter, open-label, randomized crossover trial to assess subject preference for alprazolam orally disintegrating tablets compared to conventional alprazolam tablets in subjects with anxiety

Conditions: ANXIETY

Summary: this was a multicenter, open-label, randomized crossover trial comparing two treatments, alprazolam odt versus conventional alprazolam tablets, in subjects who were already taking conventional immediate-release alprazolam tablets for anxiety. the trial included five study visits (four periods of 7 3 -day duration): screening (visit 1), treatment period 1 (visit 2), treatment period 2 (visit 3), final visit (visit 4), and follow-up (visit 5 \[telephone visit\]). at visit 1, following screening, eligible subjects continued to take their own conventional alprazolam as prescribed by their physician. at visit 2, eligible subjects were randomized in approximately equal numbers to one of two treatment sequences: alprazolam odt/ conventional alprazolam or conventional alprazolam/ alprazolam odt. subjects continued taking alprazolam in accordance with the treatment sequence to which they were assigned, at the same dose regimen as their own prescribed alprazolam. at visit 3, subjects crossed over to the alternate treatment until visit 4. at visit 4, subjects completed the subject preference questionnaire, and a physical examination and clinical laboratory tests were performed. at the end of visit 4, subjects resumed taking their own conventional alprazolam. the site made a follow-up telephone call 7 - 3 days after visit 4 to assess the subject's health status.

see approved package insert for adverse event information.

Eligibility criteria:
inclusion criteria: * currently being treated with conventional alprazolam exclusion criteria: * dose of greater than 10 mg