Evolution of filiform polyposis from classical pseudopolyposis in a pediatric ulcerative colitis patient.

Filiform polyposis (FP) is a morphologic variant of pseudopolyposis associated with inflammatory conditions of the gastrointestinal tract, namely, inflammatory bowel disease. Pediatric cases are uncommon in the literature. Here, we present a pediatric patient with FP arising from ulcerative colitis (UC).

Clinical characteristics and management of acute generalized exanthematous pustulosis with haemodynamic instability.

Background: Acute generalized exanthematous pustulosis (AGEP) is a severe pustular drug eruption with rare reports of haemodynamic instability.

Objective: To describe the clinical characteristics, management, and outcomes of patients with AGEP-associated haemodynamic instability.

Methods: This retrospective case series identified adult patients diagnosed with AGEP who had haemodynamic instability from November 2012 to February 2020 that were seen at two academic teaching hospitals with roles as a burn centre and tertiary referral centre at the University of Texas Southwestern Medical Center in Dallas, TX USA.

Risk Factors for Mortality and Circulatory Outcome Among Neonates Prenatally Diagnosed With Ebstein Anomaly or Tricuspid Valve Dysplasia: A Multicenter Study.

Background In a recent multicenter study of perinatal outcome in fetuses with Ebstein anomaly or tricuspid valve dysplasia, we found that one third of live-born patients died before hospital discharge. We sought to further describe postnatal management strategies and to define risk factors for neonatal mortality and circulatory outcome at discharge. Methods and Results This 23-center, retrospective study from 2005 to 2011 included 243 fetuses with Ebstein anomaly or tricuspid valve dysplasia.

Outcomes of Multi-Organ Transplant in Adult Patients With Congenital Heart Disease.

Background The prevalence of adult congenital heart disease (ACHD) is increasing in the United States because of improved survival into adulthood. The unique physiology of ACHD commonly leads to multiorgan dysfunction, prompting interest in outcomes after multiorgan (heart+X) transplantation. Methods and Results We queried the SRTR (Scientific Registry of Transplant Recipients) database to examine 5-year outcomes in ACHD patients (aged ≥18 years) who underwent dual organ (heart+kidney/liver/lung) transplantation between 2000 and 2016.

Neuroimaging evolution of ischemia in men and women: an observational study.

Objective: We present an exploratory study for identification of sex differences in imaging biomarkers that could further refine selection of patients for acute reperfusion therapy and trials based on sex and imaging targets.

Methods: The Lesion Evolution in Stroke and Ischemia On Neuroimaging (LESION) study included consecutive acute stroke patients who underwent MRI within 24 h of time from last known well and prior to therapy. Those demonstrating a potential therapeutic target on imaging were identified by presence of: (1) arterial occlusion on angiography, (2) focal ischemic region on perfusion maps, or (3) a mismatch of perfusion versus diffusion imaging lesion size.

Current Practices and the Provider Perspectives on Inconclusive Genetic Test Results for Osteogenesis Imperfecta in Children with Unexplained Fractures: ELSI Implications.

Genetic testing can be used to determine if unexplained fractures in children could have resulted from a predisposition to bone fractures, e.g., osteogenesis imperfecta.

Hereditary angioedema: a case of near fatal laryngeal swelling in a 41-year-old woman.

Hereditary angioedema (HAE) is characterized by nonpitting, nonpruritic subcutaneous, or submucosal edema that may be accompanied by a nonpruritic serpentine erythematous rash. The swelling in this autosomal dominantly inherited disorder is mediated by uncontrolled localized bradykinin production. The extremities, face, gastrointestinal tract, genitalia, larynx, and trunk are commonly involved.

Blood pressure outcomes in patients receiving angiotensin II receptor blockers in primary care: a comparative effectiveness analysis from electronic medical record data.

The authors examined the comparative effectiveness of 4 angiotensin receptor blockers (ARBs) in patients with hypertension using a large electronic medical record database. Analysis of covariance and logistic multivariate regression models were used to estimate the blood pressure (BP) outcomes of 73,012 patients during 13 months of treatment with olmesartan, losartan, valsartan, and irbesartan. Results were adjusted by baseline BP, starting dose, year, age, sex, race, body mass index, comorbid conditions, and concomitant medications of patients.

Demonstrated and inferred metabolism associated with cytosolic lipid droplets.

Cytosolic lipid droplets were considered until recently to be rather inert particles of stored neutral lipid. Largely through proteomics is it now known that droplets are dynamic organelles and that they participate in several important metabolic reactions as well as trafficking and interorganellar communication. In this review, the role of droplets in metabolism in the yeast Saccharomyces cerevisiae, the fly Drosophila melanogaster, and several mammalian sources are discussed, particularly focusing on those reactions shared by these organisms.

Autoantibody Testing in Neuromuscular Disorders, Part II: Neuromuscular Junction, Hyperexcitability, and Muscle Disorders.

In myasthenia gravis and Lambert-Eaton myasthenic syndrome, antibodies against ion channels are clearly related to pathogenesis; however, in the inflammatory myopathies, a link to disease causation is not as evident, except as a marker of autoimmunity. Autoantibody testing in patients with neuromuscular junction and skeletal muscle disorders is useful in diagnosis and, in some diseases, prognosis. This review briefly summarizes methods of autoantibody testing and then proceeds to a clinician-friendly guide to autoantibody testing of neuromuscular junction disorders, hyperexcitability syndromes, and inflammatory muscle diseases.

Interlocking calvarial bone grafts: a solution for the short, depressed nose.

Objectives/hypothesis: In patients severely affected with collapse of the nose, deprojection and upward rotation of the nasal tip are commonly seen. Traditional maneuvers to derotate and project the tip may be insufficient, because of the natural tendency of the nasal skin/soft tissue envelope to pull the tip in a cephalic and posterior direction. If the forces of scar contracture can be resisted, the tip and dorsum should remain adequately positioned.