Kornimate/medical-research-clean huggingface dataset:
prospective collection of biospecimen in pediatric patients and adult guardians diagnosed with glycogen storage disease type 1b (gsd1b)

Conditions: Glycogen Storage Disease Type IB

Summary: the primary objective of this study is to collect whole blood from patients diagnosed with glycogen storage disease type 1b, which will be used to support the investigation of potential therapies that address the genetic basis of this disease.


Eligibility criteria:
inclusion criteria: * subjects ages 4-85 * subjects that are diagnosed with glycogen storage disease type ib (gsd1b, von gierke disease) * subjects who are g339c homozygous or l348fs homozygous or g339c/ l348fs heterozygous or are the biological parent of subjects diagnosed with glycogen storage disease type ib (gsd1b, von gierke disease) and are a biological parent of a subject in cohort 1 (g339c homozygous) or cohort 2 (l348fs homozygous) * subjects or guardians must be willing and able to provide appropriate photo identification to mobile phlebotomist during at-home visit for verification of identity * subjects and/or guardians must be willing and able to provide appropriate written informed consent and/or assent, as applicable exclusion criteria: * subjects will be excluded if they experienced excess blood loss, including blood donation defined as 250 ml in the last month or 500 ml in the last two months. for subjects in the pediatric population, subjects will be excluded if they experienced excess blood loss, including blood donation defined as 3 ml/kg or up to 50 ml in the last 8-weeks. * for requests that do not include pregnant subjects, subjects will be excluded if they are pregnant or nursing. * for requests that do not include subjects with infectious diseases, subjects will be excluded if they have current, active hiv, hepatitis or other infectious diseases (self-reported or medical history reviewed). * subjects will be excluded if they have taken an investigational product in the last 30 days.
treatment outcome after surgical treatment of osteoid osteoma

Conditions: Osteoid Osteoma

Summary: purpose of the study: to retrospectively determine the clinical results in an unselected group of consecutive patients with osteoid osteoma treated by surgery. materials and methods: in 150 consecutive patients with clinical and/or radiological evidence for osteoid osteoma at any location, the clinical symptoms and imaging findings (radiographs and computed tomography (ct)) were assessed before and after surgery. there were no exclusion criteria for this study. a good response was defined as disappearance of symptoms that were manifested at presentation and were attributed to osteoid osteoma. clinical assessment after the procedure was performed prior to discharge; within 2 weeks after the procedure; and at 3, 6, and 12 months follow-up. after 24 months, a postal questionnaire was used for assessment. radiographic evaluation (radiographs and thin-slice ct) was performed routinely pre-operatively and one year after surgery. in case of persisting or recurring symptoms the follow-up protocol was again performed according to the initial protocol. all patients gave their informed consent both for the surgical intervention as for the use of their patient data in this retrospective study.


Eligibility criteria:
inclusion criteria: * patient with osteoid osteoma at any location. exclusion criteria: * none.

Keywords: osteoma, osteoid surgical procedures, operative bone neoplasms
is there a risk of perioperative stroke due to the retraction used during anterior cervical discectomy?

Conditions: Cervical Disc Disease; Cervical Stenosis; Cervical Disc Herniation

Summary: in anterior cervical disc surgery, head and neck extansion position and, surgical retraction used while reaching the anterior surface of the cervical vertebra can be affect the carotid blood flow and constituting a risk for cerebral ischaemia. in this study, we planned to investigate the relationship between head position and retraction, cerebral oxygenation and postoperative cognitive functions in patients undergoing anterior cervical disc surgery.


Eligibility criteria:
inclusion criteria: * aged 18-70 years * preoperative mini-mental state examination score of 27-30 * who were planned to undergo anterior cervical disc surgery under general anesthesia exclusion criteria: * patients with hypertension, severe pulmonary disease, anaemia, cardiac disease, ischaemic cerebrovascular disease, renal disease, hepatic disease, pregnancy, uncontrolled diabetes mellitus, stricture or obstruction in the carotid or vertebral arteries or * body mass index (bmi) \>30.

Keywords: cerebral blood oxygenation near infrared spectroscopy cervical disc disease cervical microsurgery
efficacy and safety of lanreotide autogel (atg) in combination with temozolomide in subjects with thoracic neuroendocrine tumors.

Conditions: Neuroendocrine Tumours

Summary: the purpose of the protocol is to evaluate the efficacy and safety of lanreotide atg 120 mg in combination with temozolomide in subjects with unresectable advanced neuroendocrine tumours of the lung or thymus as disease control rate at 9 months.


Eligibility criteria:
inclusion criteria: * histological documented unresectable advanced (locally or metastatic) well or moderately differentiated neuroendocrine tumors of the lung or thymus (typical and atypical carcinoids according to the world health organisation (who) 2004 criteria); * imaging documented progression within 12 months before screening visit (v1), according to recist criteria v 1.1; * measurable disease, as defined by recist criteria v 1.1, on a ct scan performed at screening visit (v1); * octreoscan or ga68-dota-tate/toc/noc-pet-tc within 12 months before screening visit (v1); * adequate liver, renal and bone marrow function. exclusion criteria: * poorly differentiated neuroendocrine carcinoma and mixed neuroendocrine tumours (net), according to who 2004 criteria * neuroendocrine tumours other than lung or thymus * non-neuroendocrine thymic neoplasm * received a prior therapy with peptide receptor radionuclide therapy (prrt) within 6 months prior to screening visit (v1) * treated with systemic therapies (chemotherapy, interferon-alpha, somatostatin analogues, molecular target therapies) within 1 month prior to screening visit (v1) * treated with a number of systemic therapy lines \> 3 prior to screening visit (v1), and any of the following: 1. for chemotherapy no more than 1 line prior to v1 2. for somatostatin analogue no more than 1 line therapy, considered as treatment lasting more than 6 months, prior to v1 no therapy with temozolomide (tmz) prior to v1 3. received a prior therapy with peptide receptor radionuclide therapy (prrt) within 6 months prior to screening visit (v1) * received external palliative radiotherapy within the last 28 days prior to screening visit (v1) * received locoregional therapies (transarterial embolization, transcatheter arterial chemoembolization, thermo-ablation with radio-frequency) and selective internal radiotherapy within 3 months prior to screening visit (v1) * presence of symptomatic brain metastasis * subjects with symptomatic cholelithiasis at screening visit (v1)
role of body composition in large for gestational age infants (lga) with oral feeding difficulty

Conditions: Body Composition; Feeding; Difficult, Newborn; Nutrition Disorder, Infant

Summary: large for gestational age (lga) infants have excess fat-mass (fm) proportion secondary to prolonged in utero exposure to an energy-rich environment. our preliminary data suggest that excess fm proportion can be associated with oral feeding delay and a potentially modifiable therapeutic target to improve oral feeding outcomes. the objective of this study is to determine the impact of a short-term fat-free mass (ffm)-indexed feeding on the oral intake volumes in lga infants with oral feeding difficulties.

eligible subjects will be randomized to an innovative ffm-indexed feeding or the standard feeding for up to 2 weeks. the essential component of the ffm-indexed feeding will be the difference in milk prescription dosing that we propose to set the feeding volume to index ffm rather than total mass. in ffm-indexed feeding, there will be a permissive feeding volume restriction to 150 ± 10 ml/kg (ffm)/day without increasing the milk calorie density or changing the type of formula milk, whereas the standard feeding will include a feeding volume goal of 150 ± 10 ml/kg (body weight)/day. infants will receive either breast milk or formula feedings per the standard feeding protocols. body composition (peapod system) and appetite-regulating hormones (arh) levels of enrolled infants will be assessed at baseline (test-1) and at the end 2-week study intervention period (test-2). subjects will be followed for clinical and growth outcomes until neonatal intensive care unit (nicu) discharge and through 6 months of age. growth will be followed through 6 months of age by retrieving anthropometric measurement records from pediatricians at well-child visits (2-, 4-, 6- and 6-month visits). parents will be called at these time points to obtain a history of any further feeding difficulties.

Eligibility criteria:
inclusion criteria: * lga infants with oral feeding difficulty born at ≥ 35 weeks gestation, with fm z-score \> +1.0 in body composition measurement exclusion criteria: * infants on any respiratory support, infants on enteral feeding duration \> 60 minutes due to hypoglycemia concerns, videofluoroscopic swallow study (vfss) demonstrating unsafe swallowing function, gi surgical conditions, significant neurological morbidities, and major congenital, genetic syndromes/anomalies
ccrc: a project of the treatment of hiv enteropathy with immunolin® supplements

Conditions: HIV

Summary: the purpose of the study is to see if immunolin® will reduce the frequency of bowel movements and gastrointestinal (gi) symptoms in hiv volunteers with persistent gi symptoms. the study will also examine the effect of immunolin® on the bacteria in the gut and the immune system in gut tissue as well as in the blood.


Eligibility criteria:
inclusion criteria: * gi complaints consisting of at least 2 loose or watery stools per day or marked abdominal bloating that adversely effects activities of daily living (adls) or common social functioning for greater than 6 months, even though the symptomatology may wax and wane over that time period. * subjects should have had routine testing to exclude enteric pathogens. * subjects should have made an effort to identify food intolerance, especially lactose intolerance. * symptoms should be believed to be independent of art or other medications known to have the potential to cause similar gi complaints; either because the symptoms predate initiation of the medications or shifts between different options for art has had no appreciable effect on symptoms. * over the previous 6 months, alcohol use should be characterized as occasional with less than 1.5 ounces per day being the maximum estimated use (\<2 beers or glasses of wine or 1 drink of hard liquor). * subjects will be greater than 18 years of age. * plasma hiv load should be suppressed to less than 400 cp/ml for at least four months, but there is no restriction on peripheral cd4+ t-cell count. blips of \<1000 cp/ml are allowed, provided that these are not associated with medication interruptions and that the pvl is undetectable before and after the observed blip. * subjects are willing to maintain a food and gi-symptom diary while receiving the immunolin® supplement. * subjects should be willing to attempt to maintain a stable regimen of art for the duration of the study unless indicated for patient safety. * subjects should be free of antibiotic use for at least 3-weeks prior to study entry except for chronic antibiotics used for prophylaxis according to soc in hiv management. usage of antibiotics during the course of the study will be assessed on a case by case basis by the study team. * subjects must be free of conditions which require chronic therapy that are known to alter the gut flora. steroid use must be limited to intermittent topical preparations only which includes inhaled or dermatologic routes of application. exclusion criteria: * known unrelated causes for gi abnormalities. * abnormal coagulation parameters (pt\>1.2 uln) * thrombocytopenia (platelet count \<50,000 within 6 weeks) * contra-indications to upper endoscopy or conscious sedation * anemia (\> grade 1 \[appendix d\]) * aspirin, ibuprofen, warfarin or other agents that interfere with the coagulation cascade are prohibited within 1 week of endoscopy. * positive pregnancy test

Keywords: hiv diarrhea
efficacy and safety of genoss ses in patients with coronary artery disease

Conditions: Coronary Arterial Disease (CAD)

Summary: efficacy and safety of genoss ses in patients with coronary artery disease

genoss ses

Eligibility criteria:
inclusion criteria: 1. patients of 19 and over 2. patients with coronary artery disease treated with genoss ses 3. participants who voluntarily decide to participate in this clinical trial, agree to the study protocol and clinical follow-up plan, and provide written informed consent as study participants exclusion criteria: 1. patients who are contraindicated in the use of heparin, aspirin, clopidogrel, sirolimus, cobalt chromium and contrast agents 2. patients who are pregnant or planning to become pregnant 3. patients with a planned surgery to discontinue antiplatelet agents within 12 months 4. patients with a life expectancy of less than 1 year 5. patient with cardiogenic shock at the time of hospitalization and had a low chance of survival based on medical judgment. 6. patients who have already received treatment with another des (drug eluting stent) or bms (bare metal stent) at the time of registration 7. patients participating in randomized controlled trials using other medical devices
xeltis hemodialysis access (axess) conduit: axess-e study

Conditions: End Stage Renal Disease

Summary: a prospective, single arm, non-randomized feasibility study to confirm the safety and performance of the xeltis hemodialysis access conduit, axess-e, in subjects older than 18 years with end-stage renal disease, who plan to undergo hemodialysis for at least the first 6 months after study access creation.


Eligibility criteria:
inclusion criteria: 1. subjects with end-stage renal disease (esrd) who require placement of an arteriovenous graft (avg) in the upper extremity to start or maintain hemodialysis therapy 2. at least 18 years of age at screening 3. suitable anatomy (e.g. a target vein with a minimum diameter of 5mm) for the implantation of an axess-e conduit 4. the patient has been informed about the nature of the study, understands and agrees to its provisions, and has personally provided written informed consent 5. the patient has been informed and agrees to pre- and post- procedure follow-up 6. life expectancy of at least 12 months exclusion criteria: 1. history or evidence of severe cardiac disease (new york heart association (nyha) functional class iv and/or ejection fraction (ef) \<25%), myocardial infarction within six months of study enrolment, ventricular tachyarrhythmias requiring continuing treatment, or unstable angina 2. uncontrolled or poorly controlled diabetes 3. abnormal blood values that could influence patient recovery and or/ conduit hemostasis 4. reduced liver function, defined as: \>2x the upper limit of normal for serum bilirubin, international normalized ratio (inr) \>1.5 or prothrombin time (pt) \>18 seconds 5. any active local or systemic infection 6. known heparin-induced thrombocytopenia 7. known active bleeding disorder and/or any coagulopathy or thromboembolic disease 8. allergies to study device (nitinol) or agents/medication, such as contrast agents or aspirin, that can't be controlled medically 9. anticipated renal transplant within 6 months 10. known or suspected central vein obstruction on the side of planned conduit implantation 11. previous dialysis access conduit in the operative limb unless the axess-e conduit can be placed more proximally than the previous failed conduit 12. previous enrolment in this study 13. subject is participating in another study 14. pregnant or breastfeeding woman or woman in fertile period not taking adequate contraceptives 15. any other condition which, in the judgement of the investigator would preclude adequate evaluation for the safety and performance of the study conduit intra-operative exclusion criteria: 1. unsuitable anatomy to implant the axess-e conduit (e.g. target vein and/or artery diameter smaller than anticipated; severe calcification)
evaluation of cardiac arrhythmias in hand surgery using local anesthesia with adrenaline

Conditions: Evaluation of Cardiac Arrhythmias in Hand Surgery Using Local Anesthesia With Adrenaline

Summary: goals: 1. to check for frequency and type of arrhythmia in patients undergoing a medical procedure under local anesthesia 2. to examine whether the addition of adrenaline to local anesthesia affects the incidence of arrhythmias

rationale: determining the frequency and type of arrhythmia will help determine the need for monitoring and the type of monitoring required during procedures under local anesthesia. patients will undergo the prescribed medical procedure using local anesthesia. patients will be randomized into two groups: group 1 - local anesthesia of lidocaine and arterial blockage on the arm; group 2 - local anesthesia with lidocaine and adrenalin. the amount and type of anesthetic used shall be recorded. in addition to the routine monitoring performed today (use of non-invasive blood pressure gauge and oxygen saturation), the patients will be connected to a cardiac holter monitor prior to the start of the surgical procedure and until they are released from the recovery room. upon completion of the monitoring, the results of the holter test will be transmitted to the electrophysiology unit and analyzed to detect arrhythmias. there will be a registration of side effects reported by patients, which can result from arrhythmias (palpitations, dizziness, fainting, cpr). in patients in whom arrhythmias are detected, the type of arrhythmia and if it was accompanied by clinical symptoms will be recorded.

Eligibility criteria:
inclusion criteria: * adult patients who undergo hand surgery using local anesthesia at assuta ashdod hospital exclusion criteria: * patients with a known medical history of cardiac arrhythmia
a randomized controlled trial comparing chondro-gide® to microfracture alone for treatment of knee cartilage defects.

Conditions: Cartilage Injury; Osteochondritis Dissecans

Summary: the purpose of this study is to evaluate the safety and effectiveness of using chondro-gide® collagen membrane either sutured or glued compared to microfracture alone in the treatment of symptomatic cartilage defects of the knee.

* prospective, randomized, active treatment-controlled, open-label multicenter study (up to 40 sites in the us and eu). * all subjects that meet preoperative screening eligibility criteria will be randomized to treatment with chondro-gide® either sutured or glued or microfracture alone. * eligible and consenting subjects are randomized in a 1:1:1 ratio using centralized randomization list to receive one of the following treatments: group i: microfracture alone; group ii: microfracture covered with a chondro-gide® membrane glued with fibrin glue (amic glued); group iii: microfracture covered with a chondro-gide® membrane sutured (amic sutured) * all subjects will be assessed at intervals post-procedure (6 weeks, 3 months, and 1,2 and 5 years). * subjects will be required to follow a strict pre-specified post-surgery rehabilitation protocol specific to the defect location. * measures to assess effectiveness and safety will be conducted at all follow-ups. * safety will be assessed by the collection of adverse events at all timepoints.

Eligibility criteria:
inclusion criteria: * male or female between 18 and 50 * one or two cartilage defects grade iii or iv according to outerbridge classification * defect size between 2 and 10 cm2 (inclusive) * intact normal surrounding cartilage to suture the chondro-gide® membrane * informed consent * patient willing to fulfill a strict postoperative physiotherapy scheme exclusion criteria: * more than 2 defects * defects on both knees * x-ray signs of osteoarthritis * bone lesion \> 0.7 cm in the defect * knee instability, varus or valgus deformation, status after complete meniscus resection, status after mosaicplasty, patella dysplasia * rheumatoid, infectious disease * skin lesion on the operated knee * treatment with cartilage building medication * drug and alcohol abuse * chronic heart disease, endocrine or metabolic disease, haemophilia a/b * pregnancy or lactation * collagen allergy * participation in other trials

Keywords: microfracture enhanced marrow stimulation articular cartilage autologous matrix-induced chondrogenesis chondro-gide® knee surgery amic
a prospective analysis of the efficacy of allosync expand and autograft bone graft in open lumbar spinal fusion

Conditions: Spondylolisthesis, Grade 1

Summary: the purpose of this study is to assess the radiographic and clinical outcomes of spinal fusion following use of allosync expand and autograft bone graft.

this is a single site, prospective study with patients selected as study subjects from the investigator's standard patient population who fulfill the inclusion/exclusion criteria and agree to participate in the study. the study surgery is standard of care, with patients identified as requiring surgery prior to enrollment. the study surgery is lumbar posterior fixation with supplemental posterolateral fusion at from 1 to 3 levels. one side of the posterolateral fusion will be allosync expand (utilizing the bma angel kit to hydrate) and the other side to autograft bone (control).

Eligibility criteria:
inclusion criteria: 1. at least 22years of age at the time of consent 2. subject must have a documented diagnosis of spondylolisthesis up to grade i. and have confirmed back and/or radicular pain with associated spinal stenosis as documented by conditions such as: 1. instability as defined by \>3mm translation or \>5 degree angulation 2. osteophyte formation of facet joints or vertebral endplates 3. subject presents with one or more of the following: 1. radiculopathy 2. sensory deficit 3. motor weakness 4. reflex changes 4. subject requires lumbar posterolateral arthrodesis at 1-3 contiguous levels (l1-s1). 5. the number of levels decompressed must equal the number of levels fused. 6. subject must have been unresponsive to conservative care for at least 3 months prior to fusion surgery. 7. subject must be willing and able to sign an informed consent document. 8. subject must be willing and able to return for all follow-up visits, agree to participate in postoperative clinical and radiographic evaluations and comply with the required study regimen. exclusion criteria: 1. subject has had prior lumbar spine fusion surgery at any level. 2. subject has greater than grade 1 spondylolisthesis of the lumbar spine. 3. subject is pregnant or nursing or planning to become pregnant during the two years (24 months) following arthrodesis 4. subject has an active local or systemic infection. 5. subject is a prisoner 6. patient has any condition (including malignancy), that in the opinion of the investigator, would prohibit the patient from complying with and/or completing the protocol