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Loss of dystrophin is associated with increased myocardial stiffness in a model of left ventricular hypertrophy.
(Springer, 2017-03-18)
Transition from compensated to decompensated left ventricular hypertrophy (LVH) is accompanied by functional and structural changes. Here, the aim was to evaluate dystrophin expression in murine models and human subjects ...
Biomarker-driven phenotyping in Parkinson's disease: A translational missing link in disease-modifying clinical trials.
(Wiley, 2017-02-24)
Past clinical trials of putative neuroprotective therapies have targeted PD as a single pathogenic disease entity. From an Oslerian clinicopathological perspective, the wide complexity of PD converges into Lewy bodies and ...
International variations in application of the best-interest standard across the age spectrum.
(Springer Nature [academic journals on nature.com], 2017-02-01)
OBJECTIVE:
Ethically and legally, assertions that resuscitation is in a patient's best interest should be inversely correlated with willingness to forego intensive care (and accept comfort care) at the surrogate's request. ...
Hematopoietic Stem-Cell Gene Therapy for Cerebral Adrenoleukodystrophy.
(Massachusetts Medical Society, 2017-10-26)
BACKGROUND:
In X-linked adrenoleukodystrophy, mutations in ABCD1 lead to loss of function of the ALD protein. Cerebral adrenoleukodystrophy is characterized by demyelination and neurodegeneration. Disease progression, ...
Isolated Intrapulmonary Vascular Dilatations and the Risk of Developing Hepatopulmonary Syndrome in Liver Transplant Candidates.
(Elsevier, 2017-08-01)
BACKGROUND:
The natural history of intrapulmonary vascular dilations (IPVD) and their impact on patient outcomes in the setting of portal hypertension has only been described in small series.
AIMS:
To assess the ...
Ombitasvir/paritaprevir/ritonavir/dasabuvir ± ribavirin is safe and effective in HCV-infected patients in a real-life cohort from Latin America.
(Wiley, 2017-09-01)
Information about the use of ombitasvir/paritaprevir/ritonavir/dasabuvir ± ribavirin (OBV/PTV/r/DSV ± RBV) in real-clinical practice in Latin America is scarce. We aimed to confirm safety and effectiveness of OBV/PTV/r/DSV ± RBV ...
Liver disease and dyslipidemia as a manifestation of lysosomal acid lipase deficiency (LAL-D). Clinical and diagnostic aspects, and a new treatment. An update.
(Sociedad Argentina de Pediatría., 2017-06-01)
Lysosomal acid lipase deficiency (LAL-D) is still a little recognized genetic disease with significant morbidity and mortality in children and adults. This document provides guidance on when to suspect LAL-D and how to ...
Hematopoietic Cell Transplantation-Specific Comorbidity Index Predicts Morbidity and Mortality in Autologous Stem Cell Transplantation.
(Elsevier, 2017-10-01)
The hematopoietic cell transplantation-specific comorbidity index (HCT-CI) score is a useful tool to assess the risk for nonrelapse mortality (NRM) after allogeneic hematopoietic stem cell transplantation. Although the ...
Risk factors for advanced resuscitation in term and near-term infants: a case-control study.
(BMJ Publishing Group Limited., 2017-01-01)
OBJECTIVE:
(1) To determine which antepartum and/or intrapartum factors are associated with the need for advanced neonatal resuscitation (ANR) at birth in infants with gestational age (GA) ≥34 weeks. (2) To develop a risk ...
American Confederation of Urology (CAU) experience in minimally invasive partial nephrectomy.
(Springer, 2017-01-01)
PURPOSE:
To describe the perioperative and oncology outcomes in a series of laparoscopic or robotic partial nephrectomies (PN) for renal tumors treated in diverse institutions of Hispanic America from the beginning of ...