Value in healthcare must focus on accessibility, quality, and affordability. This article describes how a healthcare team provides value by meeting the needs of a rare disease patient and underscores the importance of a chronic multiorgan rare disease home. A nurse practitioner can ensure that barriers to evaluation are removed and communication is prioritized in order to provide accessible and affordable care to a patient with rare disease without jeopardizing quality of care.
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Mol Inform
January 2025
Institute of Pharmaceutical and Medicinal Chemistry, University of Münster, Corrensstr. 48, 48149, Muenster, Germany.
Primary carnitine deficiency (PCD) is a rare autosomal recessive genetic disorder caused by missense mutations in the SLC22A5 gene encoding the organic carnitine transporter novel type 2 (OCTN2). This study investigates the structural consequences of PCD-causing mutations, focusing on the N32S variant. Using an alpha-fold model, molecular dynamics simulations reveal altered interactions and dynamics suggesting potential mechanistic changes in carnitine transport.
View Article and Find Full Text PDFHistopathology
January 2025
Department of Pathology and Laboratory Medicine, University of British Columbia, Vancouver, British Columbia, Canada.
Aims: Classification and risk stratification of endometrial carcinoma (EC) has transitioned from histopathological features to molecular classification, e.g. the ProMisE classifier, identifying four prognostic subtypes: POLE mutant (POLEmut) with almost no recurrence or disease-specific death events, mismatch repair deficient (MMRd) and no specific molecular profile (NSMP), with intermediate outcome and p53 abnormal (p53abn) with poor outcomes.
View Article and Find Full Text PDFGynecol Oncol Rep
February 2025
Department of Obstetrics and Gynecology, Division of Gynecologic Oncology, Long Island Jewish Medical Center, Zucker School of Medicine at Hofstra/Northwell, Northwell Health, New Hyde Park, NY 11040, United States.
Introduction: Adenoid basal cell carcinoma is a rare cervical malignancy which is indolent in nature but resembles more commonly occurring aggressive malignancies.
Cases: Here we describe three cases of cervical adenoid basal cell carcinoma. All patients had a history of cervical dysplasia with high-risk HPV.
Hepatic ischemia-reperfusion injury (IRI) poses a significant threat to clinical outcomes and graft survival during hemorrhagic shock, hepatic resection, and liver transplantation. Current pharmacological interventions for hepatic IRI are inadequate. In this study, we identified ginsenoside Rk2 (Rk2), a rare dehydroprotopanaxadiol saponin, as a promising agent against hepatic IRI through high-throughput screening.
View Article and Find Full Text PDFLancet Reg Health Eur
January 2025
Department of Neurology, St. Josef-Hospital - Katholisches Klinikum Bochum, Ruhr University Bochum, Gudrunstr. 56, 44791, Bochum, Germany.
Background: In recent decades, relapsing remitting multiple sclerosis (MS) became more treatable through new disease-modifying therapies (DMTs). Identifying safe treatments with minimal fetal risks for family planning is needed.
Methods: In this prospective cohort from the German MS and Pregnancy Registry (DMSKW), we analyzed pregnancy and neonatal outcomes in MS-patients using descriptive statistics and logistic/linear regression models to compare DMT-exposed pregnancies to DMT-unexposed pregnancies.
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