Kornimate/medical-research-clean huggingface dataset:
di-pkd: a pilot trial of dietary intervention in patients with autosomal dominant polycystic kidney disease

Conditions: Autosomal Dominant Polycystic Kidney Disease (ADPKD)

Summary: the goal of this interventional study is to evaluate the impact of a specific diet (low salt, limited caffeine, high potassium, low phosphate, limited protein, limited carbohydrate intake, adequate water intake) on changes in serum and urinary biomarkers, total kidney volume (tkv), and cyst progression in subjects with autosomal dominant polycystic kidney disease (adpkd). researchers will compare the results of subjects in the study diet (interventional) arm to the results of subjects in the regular diet (control) arm.

15 subjects will be enrolled in each dietary arm for a total of 30 subjects. all subjects will follow the study diet for one week, after which serum and urinary biomarkers will be measured. a baseline evaluation, including genetic testing, mri, mayo clinic imaging classification and 10-question survey will also be conducted. after week 1 of the study, subjects will be assigned to the intervention or control arm. subjects will have six follow-up visits at months 1, 2, 3, 6, 9 and 12 to assess adherence to diet. serum and urinary biomarkers will be repeated at each visit. mri will be repeated at month 12.

Eligibility criteria:
inclusion criteria: * confirmed diagnosis of adpkd * genetic testing confirmation of pkd1 or pkd2 * egfr \>30ml/1.73m2 * willingness to adhere to a preconditioning diet for 1 week and ability to complete the study * mayo clinic imaging classification (mcic): 1c, 1d, 1e exclusion criteria: * history of dietary non-adherence * subjects on tolvaptan * comorbidities that could interfere with participation (diabetes, heart failure (ef\<30%) advanced chronic kidney disease (ckd) stage 4 and higher)

Keywords: adpkd diet
prevalence of peripheral neuropathy among patients with auditory neuropathy

Conditions: Auditory Neuropathy Spectrum Disorder,, Peripheral Neuropathy

Summary: auditory neuropathy/ dys-synchrony/ peri-synaptic audiopathy (an) are terms used to describe a specific hearing disorder with a disturbed auditory neural response in presence of normal cochlear function. initially reported as early as the 1970s by ( hinchcliffe et al) after observation of the patients with normal hearing thresholds, who had difficulty to detect the sounds especially in presence of background noise . auditory neuropathy is characterized by five integral features that distinguish it from other types of hearing loss. these are: variable audiometric results anywhere from normal hearing to sensorineural hearing loss of any degree up to profound hearing loss showing unusual audiometric pattern , preserved outer hair cells (ohcs) responses such as otoacoustic emissions (oaes) and/or cochlear microphonics (cm) , altered neural processing such abnormal auditory brainstem responses (abrs) with a reduced or absent wave v , poor speech perception with poor speech recognition score that seems out of proportion compared to their pure-tone detection thresholds. , absent stapedial reflexes to the ipsilateral and contralateral tone at a 110-db hearing level . in 2008 it was found that an does not represent a single disease entity but it is a heterogeneous disease category with a wide range of hearing loss types ,etiologies , age and clinical manifestations hence the adoption of the term auditory neuropathy spectrum disorder (ansd) by an international newborn hearing screening conference held in italy following a comprehensive study of newborn hearing test results. the prevalence of ansd is variable in published studies from (0.23 to 15%) among hearing impaired individuals . regarding the underlying etiological factors, auditory neuropathy can be congenital or acquired . an without the involvement of the neurological system has been termed as primary auditory neuropathy (pan). peripheral neuropathy is not a constant finding in all patients with this hearing disorder , an may occur in the afferent pathway of acoustic nerve, probably accompanied by the pathological changes of efferent nerve in the olivocochlear system inside the brainstem our study is conducted to detect the prevalence of peripheral neuropathy among patients with auditory neuropathy spectrum disorder .


Eligibility criteria:
inclusion criteria: * age ranges from 12 to 50 years old. * disproportionate speech recognition score (srs) with the hearing threshold level. * the auditory brainstem response (abr) test with no waveform or, disturbed waves or, detectable wave v at high intense stimulus. * the otoacoustic emission (oae) and/or cochlear microphonic (cm) potential may be present. exclusion criteria: * while the participants who have these criteria will be excluded from the study: * consistent srs and abr response with hearing threshold level. * abnormal finding in brain imaging. * patients with history of diabetes and/or other causes of peripheral neuropathy
study to assess the safety, tolerability, and pharmacodynamic (pd) effects of frm-0334 in subjects with prodromal to moderate frontotemporal dementia with granulin mutation

Conditions: Granulin Mutation

Summary: the purposes of this study are to investigate the safety, tolerability, and pharmacodynamics of frm-0334 in subjects with prodromal to moderate frontotemporal dementia with granulin mutation.


Eligibility criteria:
inclusion criteria: * male or female ages aged ≥21 and ≤75 years * genotyped positive for a ftd-grn mutation, and aware of it * prodromal to moderate ftd-grn * resides in a stable living situation, living at home, senior residential setting, or an institutional setting without the need for continuous (ie, 24-hour) nursing care * proficiency (oral and written) in the language in which study-related documents, including the icf and standardized tests, will be administered * able to swallow capsules * be in good general health, willing and able to comply with the protocol requirements, and expected to complete the study as designed (in the judgment of the investigator) exclusion criteria: * clinically significant abnormalities on physical examination, medical history, ecg, vital signs, laboratory values, or unstable medical or psychiatric illness * females who are pregnant, breastfeeding, or planning to become pregnant during the study

Keywords: histone deacetylase inhibitor frontotemporal dementia granulin
psychosocial risks in liver transplant recipients

Conditions: End Stage Liver Disease

Summary: a retrospective chart review in which the information in the standard psychosocial evaluations done pre-transplant for liver transplant recipients will be coded, recorded, and correlated with posttransplant outcomes of the same recipients. evaluated outcomes include rejection episodes and adherence to tacrolimus, calculated through the mlvi (medication level variability index). the researchers will evaluate the degree to which both single elements in the evaluation as well as a cumulative score derived by a structured review of the chart using the stanford integrated psychosocial assessment for transplantation (sipat) model can predict posttransplant outcomes.

the research team will perform a chart review and extract data without identifiers from paper charts and electronic charts and data tracking system. this data will be obtained from ottr /epic as well as paper charts if needed. those charts will be accessed to obtain demographic data (age, gender, income, employment status, marital/relationship status, insurance payer); transplant specific data (primary diagnosis, transplant year, tacrolimus blood levels, rejection episodes (yes/ no and number of), graft loss (yes/no), re-transplantation (yes/no); related medical data (creatinine levels, dialysis dependence (yes/no), icd diagnoses; and psychosocial variables (social worker risk assessment before transplant, presence of social and family support, conviction history (yes/no), psychiatric history (diagnoses yes/no). variables will be obtained from the psychosocial pretransplant evaluation write-up (which in different years was attached to ottr or epic datasets, or may be present in paper form, and from electronic datasets (ottr / epic). the data extraction as well as dataset building and data entry will be performed in office spaces at ismms. no patient contact is anticipated for this study. the predictive model will include evaluations of single variables as well as an evaluation of the predictive ability of a pre-defined scoring system (the sipat) which will be applied to existing data.

Eligibility criteria:
inclusion criteria: * received a liver transplant in the year 2012 or earlier * received tacrolimus * had at least 3 tacrolimus blood tests * received their transplant at rmti exclusion criteria: * patients who received more than 1 solid organ transplant * patients who were not prescribed tacrolimus * patients who died within the first year after transplant * patients with cognitive impairment

Keywords: transplant adherence
novel imaging tools in newly-diagnosed patients with cardiac al amyloidosis

Conditions: AL Amyloidosis

Summary: this will be a systematic, combined, prospective assessment of the novel echographic, cmr, and pet imaging tools in newly-diagnosed patients with cardiac al amyloidosis at baseline and after treatment.


Eligibility criteria:
inclusion criteria: * age more than 18; * histological diagnosis of al amyloidosis; * measurable cardiac involvement as per current response criteria (i.e. nt-probnp \>650 ng/l); * measurable hematologic disease (dflc \>20 mg/l); * adequate renal function (egfr \>30 ml/min) in order to be safely administered gadolinium; * absence of atrial fibrillation with uncontrolled heart rate; * absence of implantable cardiac devices; * absence of pulmonary amyloidosis histologically documented; * plan to start anti-plasma cell chemotherapy; * plan to assess response at the pavia center after 6 months; * have given written informed consent to participate. exclusion criteria: * non-al amyloidosis; * nyha class iv; * ps-ecog \>3; * severe allergy to paramagnetic tracer; * severe claustrophobia; * pregnant or nursing women;

Keywords: amyloidosis prognosis [18f]florbetaben
effect of metformin use on mortality in diabetic and non diabetic patients with sepsis and septic shock.

Conditions: Septic Shock

Summary: we aimed to determine if metformin use in both diabetic and non diabetic patients with sepsis and septic shock affects 28 day mortality and its effect on inflammatory markers. plasma rennin, serum lactate concentration and il6 will be measured for predicting 28 days in-hospital mortality in patients with sepsis.

sepsis, is a life-threatening condition arises when the body's response to infection causes injury to its own tissues, followed by suppression of the immune system. signs and symptoms include fever, increased heart rate, increased breathing rate, and confusion.there may be symptoms of a specific infection, such as cough, or painful urination. sepsis is caused by many organisms including bacteria, viruses and fungi. . risk factors include extreme of ages, a weakened immune system. previously, a sepsis diagnosis required the presence of at least two systemic inflammatory response syndrome criteria. sepsis requires immediate treatment with intravenous fluids and antimicrobials in icu. if fluids is not enough to maintain blood pressure, the use of medications that raise blood pressure becomes necessary. mechanical ventilation and dialysis may be needed. a central venous catheter and an arterial catheter may be placed. corticosteroid use is controversial. metformin is a biguanide antihyperglycemic agent. it works by decreasing glucose production in the liver, by increasing the insulin sensitivity of body tissues, and by increasing gdf15 secretion, which reduces appetite. metformin is a first-line therapy for type 2 diabetes mellitus, taken by mouth and well tolerated. common adverse effects include diarrhea, nausea, and abdominal pain. metformin inhibit pathways linked to inflammation, immune reactions, mammalian target of rapamycin (mtor) signaling, and cell senescence. some of the changes were confirmed by western blot. therefore, metformin prevented part of the deleterious actions of pro-inflammatory cytokines in human β-cells, which was accompanied by islet proteome modifications.

Eligibility criteria:
inclusion criteria: * both sexes aged ≥18 years old complaining of sepsis or septic shock with anticipated stay \>24 hours, and signed informed consent by patient or next-of-kin. exclusion criteria: * age less than 18 years old, chronic kidney disease stage iv/v (baseline egfr \<30ml/min/1.73m2), patients with acute coronary syndrome, or do not resuscitate order. patients could be enrolled at any point during their admission.
management of acute pulmonary hypertensive crisis in children with known pulmonary arterial hypertension

Conditions: Pulmonary Arterial Hypertension

Summary: pulmonary arterial hypertension (pah) is a disease where the blood pressure in the pulmonary arteries (pap) is high. pah increases the risk of adverse events, including death, during and or after procedures. the severity of baseline pah correlates with the incidence of major complications, such that those with pap higher than their systemic blood pressure (sbp) had a 8 fold increased risk of complications. these children present for procedures where an acute exacerbation of their chronic illness-termed pulmonary hypertensive (ph)crisis, can occur, often resulting in death if not detected and managed expeditiously. unfortunately there is little data and no consensus in the pediatric literature on how ph crisis should be managed. --------------------------------------------------------------------------------

pulmonary arterial hypertension (pah) is a disease where the blood pressure in the pulmonary arteries (pap) is high. pah increases the risk of adverse events, including death, during and or after procedures. the severity of baseline pah correlates with the incidence of major complications, such that those with pap higher than their systemic blood pressure (sbp) had a 8 fold increased risk of complications. these children present for procedures where an acute exacerbation of their chronic illness-termed ph crisis, can occur, often resulting in death if not detected and managed expeditiously. unfortunately there is little data and no consensus in the pediatric literature on how ph crisis should be managed. over the last 10 years we have developed considerable expertise in managing children with pah and preventing and treating their acute crisis, using a medication called phenylephrine. this medication is routinely used to increase the blood pressure in patients (adults and children) to treat hypotension. our theory has been that by increasing sbp, we can increase the blood flow to the coronary arteries and prevent the right ventricle from failing acutely. the latter results in catastrophic hypotension, heart arrythmias and death. there is no consensus or protocol guiding the management of the acute crisis. this purpose of this study is to close that gap.

Eligibility criteria:
inclusion criteria: * patients presenting for cardiac catheterization procedure with a diagnosis of pah either by previous cardiac catheterization or echocardiography exclusion criteria: * children presenting for cardiac catheterization who do not have pah; * children with pah but with intracardiac shunts
intervention project to increase adherence to colorectal cancer screening in barcelos/esposende

Conditions: Colon and Rectal Cancer

Summary: an experimental study through a randomized clinical trial, without blinding (for participants and the research team), with a control group (not subject to intervention) and an intervention group (subject to two text messages, an automated non-human call or a call made by the administrative assistant, and a phone call made by a physician).


Eligibility criteria:
inclusion criteria: participants must be registered in the uls of barcelos/esposende and meet the following criteria: 1. age between 50 and 74 years. 2. no symptoms such as weight loss in the last 6 months or gastrointestinal symptoms. 3. no prior diagnosis of crc. 4. no diagnosis of inflammatory bowel disease (ulcerative colitis or crohn's disease). 5. no family history of crc in first- and second-degree relatives diagnosed before age 60. 6. no prior fecal occult blood test (fobt) in the 11 months before receiving the collection kit or no prior sigmoidoscopy or colonoscopy in the last 24 months. exclusion criteria: 1. participants who did not receive the screening kit at home. 2. participants without a phone number. 3. participants who do not answer the phone or refuse to participate in the study. 4. participants unable to consent or perform the screening due to physical, mental, or other conditions that seriously impair comprehension.
trial of dialysate sodium in chronic hospitalized hemodialysis patients

Conditions: Intra-dialytic Hypotension

Summary: intra-dialytic hypotensive (idh) events can be defined as an abrupt decline in blood pressure that cause symptoms and/or require an intervention. they are common, affecting up to one third of maintenance hd sessions. detrimental associations include: development of myocardial stunning, cerebral hypo-perfusion, vascular access thrombosis and greater mortality. rapid solute removal by hd generates temporary osmotic gradients between the intra-vascular and intra-cellular compartments, promoting trans-cellular fluid movement and resultant hypotension. manipulation of osmotic gradients, e.g. using higher dialysate sodium (dna), may ameliorate excess sbp decline during hd. this study aims to assess the effects of higher (142 mmol/l) versus lower (138 mmol/l) dialysate sodium (dna) use in adult chronic hemodialysis patients admitted to hospital on intra-dialytic blood pressure and biomarkers of cardiac ischemia. the investigators will randomly assign subjects to higher versus lower dna during their hospital stay, up to a maximum of six hd sessions.


Eligibility criteria:
inclusion criteria: * chronic hd (\>90 days) * age ≥18y * informed consent * first admission during study period. exclusion criteria: * use of pressors * pre-dialysis serum sodium \ 145 mmol/l * pre-dialysis sbp \>180 mmhg * intensive care stay earlier in admission * expected length of stay \<24 hours (e.g. admission for hd access procedure) * acute coronary syndrome within seven days * acute stroke * institutionalized individuals * pregnancy

Keywords: hemodialysis hypotension hospitalization
the sleepio after cancer study

Conditions: Insomnia; Cancer

Summary: this study will recruit women over the age of 18 with a current or prior cancer diagnosis who have clinical insomnia. this study will examine the efficacy of digital cognitive behavioural therapy for insomnia (dcbt-i) compared to sleep hygiene education.

women who are eligible and provide informed consent will be enrolled into this 2-armed, parallel group open label randomised controlled trial. participants will be randomised 1:1 to receive dcbt-i (intervention arm) or to sleep hygiene education (control arm). the primary outcome will be the mean continuous change in sleep condition indicator (sci) score in the intervention arm compared to the control arm at 6 months. in addition to this, the proportion of women with an sci \> 16 at 6 months will be assessed. secondary outcomes will include fatigue, sleep related quality of life, depression, anxiety, as well as hot flush interference in those experiencing vasomotor symptoms.

Eligibility criteria:
inclusion criteria: * women * aged 18 and over * sleep condition indicator (sci) score of 16 or less * current or past diagnosis of cancer * fluent in written and spoken english * internet access and comfortable with its use exclusion criteria: * acute illness * life expectancy less than 6 months * evidence of another sleep disorder * untreated psychiatric disorder * drug misuse * currently receiving cbt for insomnia from a health professional or taking part in an online treatment programme for insomnia * any condition that may be exacerbated by sleep restriction therapy (obstructive sleep apnoea, bipolar disorder, psychosis, schizophrenia, epilepsy, current suicidal ideation, shift work, dementia, parkinson's disease, lewy body dementia) * planned major surgery * commencement or a change in sleep medication within the last 4 weeks

Keywords: cancer insomnia sleep survivorship quality of life anxiety depression fatigue hot flushes
visual feedback in chronic low back pain of pregnant and nonpregnant women

Conditions: Low Back Pain

Summary: the objectives of this study are to: * evaluate the effect of visual feedback on the intensity of low back pain, range of motion and function of pregnant and non-pregnant women with chronic low back pain. * determine the association between low back pain, catastrophizing, fear of movement and low back perception/image.

it is antecipated that 15 pregnant women with low back pain and 15 non-pregnant women with low back pain will enter the study. each group will receive two different interventions, one consisting of visual feedback when performing 10 repetitions of low back flexion, extension and right and left side-flexion and another intervention consisting of the same low back movements without visual feedback. participants will be assessed for pain intensity, pain location, disability, kinesiophobia, catastrophizing, perception of the low back, flexion range of motion and time to perform the sit to stand test both at baseline and after the intervention.

Eligibility criteria:
inclusion criteria: * the sample will consist of 15 pregnant participants (ideally between the 2nd and 3rd trimester of gestation) with chronic idiopathic low back pain and a group of 15 non-pregnant women with chronic idiopathic low back pain. exclusion criteria: * pregnant women with risk pregnancy will be excluded as well as low back pain with a known cause (e.g. radiculopathy) and all participants with serious pathologies (e.g. history of trauma, surgery for less than 12 months, neurological pathologies, tumors).