6,186,237 results match your criteria patients age = 120

HSC70-JNK-BAG3 complex is critical for cardiomyocyte protection of BAG3 through its PXXP and BAG structural domains.

Front Biosci (Landmark Ed) 2021 May;26(6):102-113

Cardiovascular Laboratory, Department of Pharmacology, School of Pharmacy, Nantong University, 226001 Nantong, China.

Notwithstanding previous studies have proved the anti-apoptotic effect of Bcl-2 associated athanogene3 (BAG3) in myocardium, the structural domains PXXP and BAG responsible for its protection are not reformed. Since BAG3 in cardiomyocytes is a new target for inhibiting apoptosis induced by hypoxia/reoxygenation (H/R) stress, we demonstrated that over-expression of BAG3 reduced the injury induced by H/R in either neonatal or adult rat cardiomyocytes (NRCMs and ARCMs, respectively) and PXXP and BAG domains play an important role in cellular protection in H/R stress. Apoptosis in cardiomyocytes induced by hypoxia-reperfusion was evaluated with propidium iodide (PI) staining, cleaved caspase-3, and terminal deoxynucleotidyl transferase dUTP nick end labelling (TUNEL) staining in cultured NRCMS. Read More

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Biological variation of cardiac myosin-binding protein C in healthy individuals.

Clin Chem Lab Med 2021 Jun 23. Epub 2021 Jun 23.

Department of Medical Biochemistry and Pharmacology, Haukeland University Hospital, Bergen, Norway.

Objectives: Cardiac myosin-binding protein C (cMyC) is a novel biomarker of myocardial injury, with a promising role in the triage and risk stratification of patients presenting with acute cardiac disease. In this study, we assess the weekly biological variation of cMyC, to examine its potential in monitoring chronic myocardial injury, and to suggest analytical quality specification for routine use of the test in clinical practice.

Methods: Thirty healthy volunteers were included. Read More

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Acromesomelic dysplasia-Maroteaux type, nine patients with two novel variants.

J Pediatr Endocrinol Metab 2021 Jun 24. Epub 2021 Jun 24.

Department of Pediatric Metabolism, Sami Ulus Children Hospital, Ankara, Turkey.

Objectives: Acromesomelic dysplasia, type Maroteaux, is an autosomal recessive skeletal dysplasia caused by biallelic loss of function variations of , which encodes a cartilage regulator C-type natriuretic peptide receptor B. variations impair skeletal growth. It is a rare type of dwarfism characterized by shortening of the middle and distal segments of the limbs with spondylar dysplasia. Read More

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Growth hormone treatment and bone mineral density in pediatric patients with Prader-Willi syndrome.

J Pediatr Endocrinol Metab 2021 Jun 23. Epub 2021 Jun 23.

Department of Pediatrics, Dokkyo Medical University Saitama Medical Center, Saitama, Japan.

Objectives: Previous reports indicate that growth hormone (GH) treatment for Prader-Willi syndrome (PWS) improves bone mineral density (BMD) only when initiated at a young age and not when initiated in adulthood. However, there are no data on BMD during long-term GH treatment of Japanese children and adolescents with PWS. Thus, this study aimed to investigate BMD changes among patients with PWS, who were undergoing GH treatment from childhood to adolescence. Read More

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Genome analyses and androgen quantification for an infant with 5α-reductase type 2 deficiency.

J Pediatr Endocrinol Metab 2021 Jun 23. Epub 2021 Jun 23.

Department of Molecular Endocrinology, National Research Institute for Child Health and Development, Tokyo, Japan.

Objectives: 5α-reductase type 2 deficiency due to biallelic variants is a common form of 46,XY disorders of sex development.

Case Presentation: A Chinese neonate presented with ambiguous genitalia. He carried a homozygous likely_pathogenic variant (c. Read More

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An intronic variant disrupts mRNA splicing and causes -related skeletal dysplasia.

J Pediatr Endocrinol Metab 2021 Jun 23. Epub 2021 Jun 23.

Department of Pediatric Endocrinology and Genetics, Xinhua Hospital, Shanghai Jiao Tong University School of Medicine; Shanghai Institute for Pediatric Research, Shanghai, China.

Objectives: Achondroplasia and hypochondroplasia are the most common forms of disproportionate short stature, of which the vast majority of cases can be attributed to the hotspot missense mutations in the gene . Here we presented cases with a novel cryptic splicing variant of gene and aimed to interrogate the variant pathogenicity.

Case Presentaiton: In whole exome sequencing of two patients with hypochondroplasia-like features, a intronic variant c. Read More

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Retrospective evaluation of patients with X-linked adrenoleukodystrophy with a wide range of clinical presentations: a single center experience.

J Pediatr Endocrinol Metab 2021 Jun 23. Epub 2021 Jun 23.

Department of Pediatric Metabolism, Dr. Sami Ulus Maternity and Child Health Training and Research Hospital, Ankara, Turkey.

Objectives: X-linked adrenoleukodystrophy (X-ALD), is a peroxisomal inborn error of metabolism caused due to the loss of function variants of gene that leads to accumulation of very long chain fatty acids (VLCFAs) in several tissues including the neurological system. Childhood cerebral X-ALD (CCALD) is the most common and severe form of X-ALD, if left untreated. Allogenic hematopoietic stem cell transplantation (HSCT) is the only available therapy that halts neurological deterioration in CCALD. Read More

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Molecular and clinical findings of Turkish patients with hereditary fructose intolerance.

J Pediatr Endocrinol Metab 2021 Jun 23. Epub 2021 Jun 23.

Ankara Yıldırım Beyazıt University, Ankara City Hospital, Ankara, Turkey.

Objectives: Hereditary fructose intolerance (HFI) is an autosomal recessive disorder caused by a deficiency in aldolase B that can result in hypoglycemia, nausea, vomiting, abdominal pain, liver and kidney dysfunction, coma, and even death. This study aims to represent the clinical features and molecular genetic analysis data of the patients diagnosed with HFI in our study population.

Methods: The medical records of the 26 patients with HFI were evaluated retrospectively. Read More

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Evaluation of children with type 1 diabetes mellitus in terms of overweight/obesity in tertiary care hospital.

J Pediatr Endocrinol Metab 2021 Jun 23. Epub 2021 Jun 23.

Department of Pediatrics, Ondokuz Mayis University, Faculty of Medicine, Division of Pediatric Endocrinology, Samsun, Turkey.

Objectives: Obesity is a growing problem in type 1 diabetes mellitus (T1DM) today. The aim of our study is to determine the frequency of overweight/obesity at the time of diagnosis and during follow-up in children with T1DM as well as review the conditions that may accompany.

Methods: A total of 315 patients with T1DM were retrospectively analyzed. Read More

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MAN1B1-CDG: novel patients and novel variant.

J Pediatr Endocrinol Metab 2021 Jun 23. Epub 2021 Jun 23.

Department of Pediatrics, Center for Metabolic Diseases, KU Leuven, Leuven, Belgium.

Objectives: Congenital disorders of glycosylation (CDGs) are a group of genetic disorders due to hypoglycosylation of proteins and lipids. A type I pattern is associated with defects in glycan assembly and transfer (CDG-I; cytosol; and endoplasmic reticulum defects), a type II pattern is seen in processing defects of the Golgi apparatus. MAN1B1-CDG is an autosomal recessive CDG-II due to mutations in the 1,2-mannosidase gene (MAN1B1), mainly characterized by psychomotor disability, facial dysmorphism, truncal obesity, and hypotonia. Read More

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A non-inferiority randomized controlled clinical trial comparing Unani formulations & PUVAsol in non-segmental vitiligo.

J Complement Integr Med 2021 Jun 23. Epub 2021 Jun 23.

Department of Moalajat, Luqman Unani Medical College Hospital & Research Center, Bijapur, Karnataka, India.

Objectives: Greco-Arab medicine is an ancient system of medicine with greater treasure on therapeutics of vitiligo. The trial Unani formulations have not been scientifically explored for their safety and efficacy, but have been repeatedly prescribed by the great Unani physicians in the management of (vitiligo). Hence, these interventions were selected for the trial. Read More

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Validity and reliability of the Thai version of the pediatric obstructive sleep apnea screening tool.

Pediatr Pulmonol 2021 Jun 23. Epub 2021 Jun 23.

Department of Child Health and the Child Health Research Institute, University of Missouri School of Medicine, Columbia, Missouri, USA.

Objective: Obstructive sleep apnea (OSA) is highly prevalent in children and requires an expensive and relatively unavailable sleep study for diagnosis. This study was undertaken to translate the previously validated pediatric OSA screening tool (POSAST) to the Thai language and assess its accuracy and test-retest reliability in at-risk symptomatic children.

Study Design: Prospective cross-sectional cohort study. Read More

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Comparative study of pregnancy outcomes in women with inflammatory bowel disease treated with thiopurines and/or anti-TNF: a French nationwide study 2010-2018.

Aliment Pharmacol Ther 2021 Jun 23. Epub 2021 Jun 23.

EPIPHARE, Épidémiologie des produits de santé, ANSM-CNAM, Denis, France.

Background: Data about thiopurines or anti-TNF use during pregnancy in women with inflammatory bowel diseases (IBD) are reassuring. However, many studies are based upon small sample sizes.

Aims: To assess IBD medication safety during pregnancy. Read More

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MRGPRX4 in Cholestatic Pruritus.

Semin Liver Dis 2021 Jun 23. Epub 2021 Jun 23.

Department of Neuroscience, Perelman School of Medicine, University of Pennsylvania, Philadelphia, Pennsylvania.

Pruritus (itch) is a debilitating symptom in liver diseases with cholestasis, which severely affects patients' quality of life. Limited treatment options are available for cholestatic itch, largely due to the incomplete understanding of the underlying molecular mechanisms. Several factors have been proposed as pruritogens for cholestatic itch, such as bile acids, bilirubin, lysophosphatidic acid, and endogenous opioids. Read More

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Application of Risk Scores for Hepatocellular Carcinoma in Patients with Chronic Hepatitis B: Current Status and Future Perspective.

Semin Liver Dis 2021 Jun 23. Epub 2021 Jun 23.

Genomics Research Center, Academia Sinica, Taipei, Taiwan.

Accurate risk prediction for hepatocellular carcinoma (HCC) among patients with chronic hepatitis B (CHB) may guide treatment strategies including initiation of antiviral therapy and also inform implementation of HCC surveillance. There have been 26 risk scores developed to predict HCC in CHB patients with ( = 14) or without ( = 12) receiving antiviral treatment; all of them invariably include age in the scoring formula. Virological biomarkers of replicative activities (i. Read More

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Hepatic Alveolar Echinococcosis.

Semin Liver Dis 2021 Jun 23. Epub 2021 Jun 23.

Division of Tropical and Humanitarian Medicine, Faculty of Medicine, University Hospitals of Geneva, Faculty of Medicine, Switzerland.

Hepatic alveolar echinococcosis (HAE) is a rare but severe zoonosis caused by the pseudotumoral intrahepatic development of the larval stage of the tapeworm HAE is present only in the Northern Hemisphere, predominantly in China. Currently, there is a significant resurgence of cases in historically endemic areas associated with emergence of HAE in countries not previously concerned. Today, in European countries, HAE is often discovered by chance; however, clinicians should be made aware of opportunistic infections that progressively emerged recently as a result of therapeutic or pathological immunosuppression. Read More

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Polycaprolactone Mesh for Asian Rhinoplasty: Outcomes and Complications of Composite Septal Extension Graft Compared to Mesh-Only Graft.

Facial Plast Surg 2021 Jun 23. Epub 2021 Jun 23.

Department of Plastic and Reconstructive Surgery, Seoul National University Bundang Hospital, Seongnam, Korea.

Despite the great demand of aesthetic rhinoplasty in Asian population, it is difficult to obtain the lasting ideal tip projection along with lengthening of the nose due to the small and weak nasal septum. The shortage of available septal cartilage to work with is another major obstacle. A retrospective study was conducted between January 2017 and December 2019 in Seoul, Korea. Read More

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[Bowel resections for Crohn's disease: developments over the last three decades].

Z Gastroenterol 2021 Jun 23. Epub 2021 Jun 23.

Department of Surgery, Universitätsklinikum Regensburg, Regensburg, Germany.

Background: The present observational study demonstrates developments of surgery in Crohn's disease patients undergoing bowel resection at two tertiary referral centers during the recent 3 decades.

Methods: Consecutive patients undergoing intestinal resections were included. Exclusion criteria were: resection for malignancy, mere stoma formation and closure, bowel resections for other reasons than Crohn's disease, abdomino-perineal resections for anal fistula. Read More

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Optimizing Clinical Monitoring Tools to Enhance Patient Review by Pharmacists.

Appl Clin Inform 2021 May 23;12(3):621-628. Epub 2021 Jun 23.

Department of Pharmacy, Mayo Clinic, Rochester, Minnesota, United States.

Background:  The Clinical Monitoring List (CML) is a real-time scoring system and intervention tool used by Mayo Clinic pharmacists caring for hospitalized patients.

Objective:  The study aimed to describe the iterative development and implementation of pharmacist clinical monitoring tools within the electronic health record at a multicampus health system enterprise.

Methods:  Between October 2018 and January 2019, pharmacists across the enterprise were surveyed to determine opportunities and gaps in CML functionality. Read More

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[Assessing Electronic Prescription: A Cross-sectional Study of Pharmacists in Germany].

Gesundheitswesen 2021 Jun 23. Epub 2021 Jun 23.

Institute of Family Medicine, Universitätsklinikum Schleswig-Holstein - Campus Lübeck, Lübeck, Deutschland.

Background: The GERDA ("Protected e-prescription service for pharmacies") project of the Chamber of Pharmacists (LAK) and Pharmacists Association (LAV) of the federal state of Baden-Wuerttemberg provided the opportunity to prescribe medications within the telemedical portal "docdirekt" that is operated by the Association of Statutory Health Insurance Physicians of Baden-Wuerttemberg. Against this background, the aim of the study was to explore and prioritize barriers and enablers among pharmacists to participate in a medical supply system that includes electronic prescriptions (e-prescriptions). Based on these determinants, recommendations for optimizing the successful implementation of similar care offers were derived. Read More

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Impact of the Global COVID-19 Pandemic on the Incidence, Presentation, and Management of Pediatric Appendicitis: Lessons Learned from the First Wave.

Eur J Pediatr Surg 2021 Jun 23. Epub 2021 Jun 23.

Department of Pediatric Surgery, University Hospital Frankfurt, Goethe University Frankfurt, Frankfurt (Main), Germany.

The fast-evolving nature of the coronavirus disease 2019 (COVID-19) pandemic has led to unprecedented clinical, logistical, and socioeconomical challenges for health-care systems worldwide. While several studies have analyzed the impact on the presentation and management of acute appendicitis (AA) in the adult population, there is a relative paucity of similar research in pediatric patients with AA. To date, there is some evidence that the incidence of simple AA in children may have decreased during the first lockdown period in spring 2020, whereas the number of complicated AA cases remained unchanged or increased slightly. Read More

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Patients' Adaptations After Acute Respiratory Distress Syndrome: A Qualitative Study.

Am J Crit Care 2021 May;30(3):221-229

Theodore J. Iwashyna is the co-director of the research core at the Veterans Affairs Center for Clinical Management Research, Health Services Research & Development Center of Innovation, and the Alpheus W. Tucker, MD, Collegiate Professor of Internal Medicine at the University of Michigan, Ann Arbor.

Background: Many patients confront physical, cognitive, and emotional problems after acute respiratory distress syndrome (ARDS). No proven therapies for these problems exist, and many patients manage new disability and recovery with little formal support. Eliciting patients' adaptations to these problems after hospitalization may identify opportunities to improve recovery. Read More

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Structural Validity of the Hospital Anxiety and Depression Scale Among Family Members of Critically Ill Patients.

Am J Crit Care 2021 May;30(3):212-220

Ronald L. Hickman Jr is an associate professor of nursing and associate dean for research, Frances Payne Bolton School of Nursing, Case Western Reserve University.

Background: The Hospital Anxiety and Depression Scale (HADS) is commonly used clinically and scientifically among surrogate decision makers for critically ill patients. The validity of the HADS has been scrutinized, but its use among surrogate decision makers has not been examined.

Objective: To examine the structural validity of the HADS. Read More

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Bridging the Gap Between the Intensive Care Unit and the Acute Medical Care Unit.

Am J Crit Care 2021 May;30(3):193-200

Liron Sinvani is an associate professor, Division of Hospital Medicine, Department of Medicine, Zucker School of Medicine at Hofstra/Northwell, and an assistant professor, Feinstein Institutes for Medical Research, Northwell Health.

Background: Despite a growing cohort of intensive care unit (ICU) survivors, little is known about the early ICU aftercare period.

Objective: To identify gaps in early ICU aftercare and factors associated with poor hospital outcomes.

Methods: A multisite, retrospective study (January 1 to December 31, 2017) was conducted among randomly selected patients admitted to the medical ICU and subsequently transferred to acute medical care units. Read More

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Vasoplegic Syndrome in Patients Undergoing Cardiac Surgery: A Literature Review.

Colleen M Nash

AACN Adv Crit Care 2021 Jun;32(2):137-145

Colleen M. Nash is an Adult-Gerontology Acute Care Nurse Practitioner, Northwestern Memorial Hospital, 251 E Huron St, Chicago, IL 60611

Vasoplegic syndrome is a rising problem affecting morbidity and mortality in patients undergoing cardiac surgery. Vasoplegia is a vasodilatory, shocklike syndrome characterized by decreased systemic vascular resistance, normal to high cardiac index, and hypotension refractory to fluid resuscitation and vasopressors. This review describes the presentation, physiology, risk factors, treatments, and implications of vasoplegia after cardiac surgery. Read More

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Impact of a Nurse-Driven Heparin Monitoring Protocol for Ventricular Assist Devices.

AACN Adv Crit Care 2021 Jun;32(2):146-151

Pamela B. Simone is Cardiology Clinical Pharmacist Specialist, Department of Pharmacy, UChicago Medicine, 5841 S Maryland Ave, Chicago, IL 60637

Background: Ventricular assist devices require anticoagulation to reduce thrombosis risk. Nurse-driven unfractionated heparin monitoring protocols have been validated for various indications, although data in patients with ventricular assist devices are lacking.

Objective: To evaluate a nurse-driven protocol for managing unfractionated heparin therapy in stable patients with ventricular assist devices. Read More

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Cemented Revision Hip Arthroplasty With Femoral Impaction Bone Grafting.

J Am Acad Orthop Surg 2021 Jun 23. Epub 2021 Jun 23.

From the Orthopaedic and Trauma Service, Royal Adelaide Hospital, and Discipline of Orthopaedics and Trauma University of Adelaide, Adelaide, Australia.

Long-stemmed uncemented implants are commonly used during revision hip arthroplasty but may be difficult to re-revise. Impaction bone grafting allows for the use of a shorter cemented stem during revision hip arthroplasty and may restore bone stock in patients with substantial femoral defects. Femoral impaction bone grafting is particularly beneficial in younger patients, who are more likely to require additional revision procedures in the future. Read More

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Metabolism as Disease Tolerance: Implications for Sepsis-Associated Acute Kidney Injury.

Sarah C Huen

Nephron 2021 Jun 23:1-4. Epub 2021 Jun 23.

Departments of Internal Medicine-Nephrology and Pharmacology, The University of Texas Southwestern Medical Center, Dallas, Texas, USA.

Sepsis is a significant cause for mortality among critically ill patients. Metabolic derangements that develop in sepsis are often considered to be pathologic, contributing to sepsis morbidity and mortality. However, alterations in metabolism during sepsis are multifaceted and are incompletely understood. Read More

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Effectiveness of Acupuncture for Poststroke Aphasia: A Systematic Review and Meta-Analysis of Randomized Controlled Trials.

Complement Med Res 2021 Jun 23:1-11. Epub 2021 Jun 23.

South China Research Center for Acupuncture and Moxibustion, Medical College of Acu-Moxi and Rehabilitation, Guangzhou University of Chinese Medicine, Guangzhou, China.

Background: Aphasia is one of the common complications of stroke, and it considerably influences the quality of life of patients. Acupuncture, a therapy used in traditional Chinese medicine for aphasia after stroke, has potential therapeutic effects. We aimed to investigate the therapeutic effect of acupuncture on individuals with poststroke aphasia. Read More

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