A rare case of methotrexate and primaquine co-administration in a mantle cell lymphoma patient.

What Is Known And Objective: High-dose methotrexate (HD-MTX) is associated with a plethora of adverse drug reactions and potential drug interactions (DIs). But there is a paucity of information regarding the safety of co-administering primaquine with HD-MTX.

Case Summary: A 65-year-old male patient was diagnosed with mantle cell lymphoma (MCL) with CNS involvement and treated with three cycles of IV HD-MTX.

Motor neurons from ALS patients with mutations in C9ORF72 and SOD1 exhibit distinct transcriptional landscapes.

Amyotrophic lateral sclerosis (ALS) is a progressive motor neuron disease that culminates in paralysis and death. Here, we present our analyses of publicly available multiOMIC data sets generated using motor neurons from ALS patients and control cohorts. Functional annotation of differentially expressed genes in induced pluripotent stem cell (iPSC)-derived motor neurons generated from patients with mutations in C9ORF72 (C9-ALS) suggests elevated expression of genes that pertain to extracellular matrix (ECM) and cell adhesion, inflammation and TGFβ targets.

Topical Diacerein Ointment for Epidermolysis Bullosa Simplex: A Review.

Epidermolysis bullosa (EB) is a group of rare mucocutaneous fragility disorders often presenting in infancy and early childhood with painful blistering of the skin and mucous membranes. The severity of EB blister burden varies by disease subtype. Studies have shown that patients with generalized severe epidermolysis bullosa simplex (EBS), a variant characterized by extreme fragility, develop blisters in the setting of overproduced, mutated K14 protein, a component of the intermediate filament integral in keratinocyte stability, and constitutive activation of interleukin (IL)-1 , a pro-inflammatory cytokine that promotes the hyperproliferation of keratinocytes.

Clinical Pharmacogenetics Implementation Consortium (CPIC) Guideline for CYP2B6 and Efavirenz-Containing Antiretroviral Therapy.

The HIV type-1 nonnucleoside reverse transcriptase inhibitor, efavirenz, is widely used to treat HIV type-1 infection. Efavirenz is predominantly metabolized into inactive metabolites by cytochrome P450 (CYP)2B6, and patients with certain CYP2B6 genetic variants may be at increased risk for adverse effects, particularly central nervous system toxicity and treatment discontinuation. We summarize the evidence from the literature and provide therapeutic recommendations for efavirenz prescribing based on CYP2B6 genotypes.

Glycopyrronium Tosylate (Qbrexza) for Hyperhidrosis.

Hyperhidrosis is a condition characterized by excessive sweat production beyond which is physiologically necessary for thermal regulation. Affecting over 4.8% of the United States population, studies have shown that severe primary hyperhidrosis interferes with daily activities and can be considered intolerable, negatively impacting a patient’s quality of life.

The role of metastatic burden in cytoreductive/consolidative radical cystectomy.

Purpose: To describe our institutional experience with cytoreductive/consolidative radical cystectomy (CCRC) for metastatic urothelial carcinoma (UC) and to investigate clinicopathologic features predicting prolonged cancer specific survival (CSS) following CCRC.

Methods: We performed IRB-approved review of our cystectomy database, and identified 43 patients with metastatic UC who underwent CCRC. Baseline demographics, chemotherapy regimen, clinicopathologic features, and perioperative complications were collected.

Cardiac Early Repolarization Pattern Anomalies Among Children and Adolescents With and Without Attention-Deficit Hyperactivity Disorder: A Community Observational Study.

Our research had 2 aims, first, to determine if electrocardiographic early repolarization pattern anomalies (ERPAs) were more likely present among children and adolescents diagnosed with attention-deficit hyperactivity disorder (ADHD; n = 416) when compared with non-ADHD children (n = 187), and second, to asses if ADHD patients whose parents report severe ADHD psychometric scores were more likely to have ERPA in their surface ECG (electrocardiography) when compared with other ADHD patients with mild to moderate dysfunction or no dysfunction. In our unmatched case-control study, ERPA was recognized when there was an end QRS notch (J wave) or slur on the downslope of a prominent R wave with and without ST-segment elevation and the peak of the notch or J wave (Jp) ≥0.1 mV in ≥2 contiguous leads, excluding V1-V3 anterior lead, and QRS duration (measured in leads in which a notch or slur is absent) <120 ms or ST-segment elevation >0.

HRAS-driven cancer cells are vulnerable to TRPML1 inhibition.

By serving as intermediaries between cellular metabolism and the bioenergetic demands of proliferation, endolysosomes allow cancer cells to thrive under normally detrimental conditions. Here, we show that an endolysosomal TRP channel, TRPML1, is necessary for the proliferation of cancer cells that bear activating mutations in Expression of , which encodes TRPML1, is significantly elevated in -positive tumors and inversely correlated with patient prognosis. Concordantly, knockdown or TRPML1 inhibition selectively reduces the proliferation of cancer cells that express oncogenic, but not wild-type, Mechanistically, TRPML1 maintains oncogenic HRAS in signaling-competent nanoclusters at the plasma membrane by mediating cholesterol de-esterification and transport.

Examining the Influence of a New Light Rail Line on the Health of a Demographically Diverse and Understudied Population within the Washington, D.C. Metropolitan Area: A Protocol for a Natural Experiment Study.

Approximately two-thirds of adults and youth in Prince George's County, Maryland, a suburb of Washington, D.C. are overweight or obese and less than half are achieving daily physical activity recommendations.

National Cooperative Growth Study: 25 Years of Growth Hormone Data, Insights, and Lessons for Future Registries.

Background: The National Cooperative Growth Study (NCGS) data are reviewed from 1985-2010 to report on final demographic, efficacy, and safety findings, and to illustrate the value of long-term, real-world follow-up to physicians and patients.

Methods: The NCGS was a multicenter, open-label, observational, postmarketing surveillance study of Genentech growth hormone (GH) products for the treatment of children with growth failure in North America.

Findings: Data from 65,205 patients representing 240,951 patient-years of experience were collected.

Assessing Burden of Care in the Patient With Cleft Lip and Palate: Factors Influencing Completion and Noncompletion of Nasoalveolar Molding.

Objective: Evaluate the factors that influence caregiver-reported completion of nasoalveolar molding (NAM) therapy for patients with cleft lip and palate.

Design: An IRB-approved 30-question survey.

Setting: Outpatient clinic for patients with cleft lip.

Radiation-induced lung injury: impact on macrophage dysregulation and lipid alteration - a review.

Lung cancer continues to be the leading cause of cancer deaths and more than one million lung cancer patients will die every year worldwide. Radiotherapy (RT) plays an important role in lung cancer treatment, but the side effects of RT are pneumonitis and pulmonary fibrosis. RT-induced lung injury causes damage to alveolar-epithelial cells and vascular endothelial cells.

Risk Factors in Pediatric Blunt Cervical Vascular Injury and Significance of Seatbelt Sign.

Introduction: Computed tomography angiography (CTA) is used to screen patients for cerebrovascular injury after blunt trauma, but risk factors are not clearly defined in children. This modality has inherent radiation exposure. We set out to better delineate the risk factors associated with blunt cervical vascular injury (BCVI) in children with attention to the predictive value of seatbelt sign of the neck.

Development of a framework to describe patient and family harm from disrespect and promote improvements in quality and safety: a scoping review.

Purpose: Patients and families may experience 'non-physical' harm from interactions with the healthcare system, including emotional, psychological, socio-behavioral or financial harm, some of which may be related to experiences of disrespect. We sought to use the current literature to develop a practical, improvement-oriented framework to recognize, describe and help prevent such events.

Data Sources: Searches were performed in PubMed, Embase, PsychINFO, CINAHL, Health Business Elite and ProQuest Dissertations & Theses: Global: Health & Medicine, from their inception through July 2017.

Congenital aneurysm of the palmar digital artery: A case report and literature review.

Spontaneous arterial aneurysms of the hand are uncommon but are well-described in the adult population. In the pediatric population, however, congenital or true aneurysms of the hand are exceptionally rare. A case report and a literature review were performed for published cases of arterial aneurysms of the hand in the pediatric population.

Safety and tolerability of lurbinectedin (PM01183) in patients with acute myeloid leukemia and myelodysplastic syndrome.

Trabectedin is an FDA-approved DNA minor groove binder that has activity against translocation-associated sarcomas. Lurbinectedin is a next-generation minor groove binder with preclinical activity against myeloid leukemia cells. A dose-finding phase 1 clinical trial was performed in patients with acute myeloid leukemia (AML) and myelodysplastic syndrome (MDS) with further assessment of safety and tolerability.

Psychosis and synthetic cannabinoids.

Synthetic cannabinoid (SC) products have gained popularity as abused drugs over the past decade in many countries. The SCs broadly impact psychological state (e.g.

Increased complications in pediatric surgery are associated with comorbidities and not with Down syndrome itself.

Background: Down syndrome (DS) is a genetic condition associated with multiple comorbidities. While physicians may perceive that DS patients have more postoperative complications, the literature remains unclear. This study compared postoperative complications for children with and without DS who underwent abdominal and thoracic procedures.

Planned Gamma Knife Boost After Chemoradiotherapy for Selected Sinonasal and Nasopharyngeal Cancers.

Objective: To determine the feasibility of a Gamma Knife boost after intensity-modulated radiation therapy in combination with multimodal therapy in patients with nasopharyngeal carcinoma and sinonasal malignancies with skull base or cavernous sinus involvement.

Methods: Nine patients were treated with intensity-modulated radiation therapy followed by a Gamma Knife boost. In one case Gamma Knife was given as salvage treatment after resection.

A comparison of resuscitation intensity and critical administration threshold in predicting early mortality among bleeding patients: A multicenter validation in 680 major transfusion patients.

Background: To address deficiencies associated with the classic definition of massive transfusion (MT), critical administration threshold (CAT) and resuscitation intensity (RI) were developed to better quantify the overall severity of illness and predict the need for transfusions and early mortality. We sought to evaluate these as more appropriate replacements for MT in defining mortality risk in patients undergoing major transfusions.

Methods: Patients predicted to receive MT at 12 Level I trauma centers were randomized in the Pragmatic, Randomized Optimal Platelet and Plasma Ratios (PROPPR) trial.

A Review of Traumatic Brain Injury and the Gut Microbiome: Insights into Novel Mechanisms of Secondary Brain Injury and Promising Targets for Neuroprotection.

The gut microbiome and its role in health and disease have recently been major focus areas of research. In this review, we summarize the different ways in which the gut microbiome interacts with the rest of the body, with focus areas on its relationships with immunity, the brain, and injury. The gut⁻brain axis, a communication network linking together the central and enteric nervous systems, represents a key bidirectional pathway with feed-forward and feedback mechanisms.

Unique Contribution of Haptoglobin and Haptoglobin Genotype in Aneurysmal Subarachnoid Hemorrhage.

Survivors of cerebral aneurysm rupture are at risk for significant morbidity and neurological deficits. Much of this is related to the effects of blood in the subarachnoid space which induces an inflammatory cascade with numerous downstream consequences. Recent clinical trials have not been able to reduce the toxic effects of free hemoglobin or improve clinical outcome.