Natural language processing and expert follow-up establishes tachycardia association with CDKL5 deficiency disorder.

Purpose: CDKL5 deficiency disorder (CDD) is a developmental and epileptic encephalopathy with multisystemic comorbidities. Cardiovascular involvement in CDD was shown in animal models but is yet poorly described in CDD cohorts.

Methods: We identified 38 individuals with genetically confirmed CDD through the Cleveland Clinic CDD specialty clinic and matched 190 individuals with non-genetic epilepsy to them as a comparison group.

Osteonecrosis: A disabling disease not to be ignored in asthma and atopic conditions.

Osteonecrosis, also referred to as avascular necrosis, is a disease characterized by necrosis or death of a bone secondary to impairment in blood supply. The condition affects the epiphyseal ends of the bones such as the femur and the humerus, but it can also involve the metacarpal and metatarsal bones, the patella, the knee, the vertebrae, and the jaw. A plethora of inflammatory, autoimmune, hematological, thrombotic, and vascular diseases can lead to osteonecrosis.

Assessing the impact of transplant case management on clinical outcomes.

Objectives: Case management is commonly used by health plans to attempt to improve the care received by their members who have complex needs, such as those who undergo transplantation. There are few observational studies evaluating the effects that transplant case management programs have on clinical outcomes following a solid organ transplant. This limits the understanding of the quantitative effectiveness of such programs.

Odynophagia in a young adult: revisiting herpetic esophagitis and eosinophilic esophagitis.

An immunocompetent man in his 20s presented with a 24-hour history of severe odynophagia, nausea, vomiting and throat pain. Esophagogastroduodenoscopy (EGD) revealed severe esophagitis with ulcerated mucosa, exudative debris, haemorrhage and multiple erosions. Biopsy of the oesophageal tissue demonstrated marginated chromatin, multinucleated giant cells and molding of nuclei, consistent with herpes simplex virus esophagitis (HSE).

Asynchronous current-induced switching of rare-earth and transition-metal sublattices in ferrimagnetic alloys.

Ferrimagnetic alloys are model systems for understanding the ultrafast magnetization switching in materials with antiferromagnetically coupled sublattices. Here we investigate the dynamics of the rare-earth and transition-metal sublattices in ferrimagnetic GdFeCo and TbCo dots excited by spin-orbit torques with combined temporal, spatial and elemental resolution. We observe distinct switching regimes in which the magnetizations of the two sublattices either remain synchronized throughout the reversal process or switch following different trajectories in time and space.

Dupilumab-induced ocular surface disease: a primer.

The management of atopic diseases has been revolutionised by precision therapies and biological drugs that target specific immune proteins. This report elucidates a unique complication from the use of the monoclonal antibody, dupilumab, that primary care providers and subspecialists need to be aware of. A patient in her 40s consulted us for severe atopic asthma, food allergy and eczema involving the face and body.

Mastocytosis and Mast Cell Activation Disorders: Clearing the Air.

Mast cells are derived from hematopoietic stem cell precursors and are essential to the genesis and manifestations of the allergic response. Activation of these cells by allergens leads to degranulation and elaboration of inflammatory mediators, responsible for regulating the acute dramatic inflammatory response seen. Mast cells have also been incriminated in such diverse disorders as malignancy, arthritis, coronary artery disease, and osteoporosis.

Isolation, characterization and cytotoxicity studies of bioactive compounds from Nees.

(Lauraceae) is a traditional medicinal plant historically used in Indian Western Ghats to treat various human diseases. From the past few decades it has been traditionally recognized as an effective agent in cancer treatment. The phytochemical investigation of the stem bark and leaves of led to the isolation of bioactive flavonoid compounds Icariin and Baicalein.

Subcutaneous Immunoglobulin Therapy with IgPro20 in Patients with Stiff Person Syndrome and Primary Immunodeficiency Disease.

Stiff Person Syndrome (SPS), a rare autoimmune neurologic disorder characterized by fluctuating muscle spasms and rigidity, is mediated by autoantibodies to glutamic acid decarboxylase (GAD) antibodies. Symptoms of SPS have been shown to improve after administration of intravenous immunoglobulin (IVIG) however, there is a paucity of information regarding use of SCIg in SPS. Four patients with Stiff Person Syndrome were treated with SCIgPro20 for a period between 31 to 101 months.

Critical Care Management of the Patient With Anaphylaxis: A Concise Definitive Review.

Objectives: Anaphylaxis is a rapidly progressive life-threatening syndrome manifesting as pruritus, urticaria, angioedema, bronchospasm and shock. The goal of this synthetic review is to provide a practical, updated approach to the evaluation and management of this disorder and associated complications.

Data Sources: A MEDLINE search was conducted with the MeSH of anaphylaxis, anaphylactic reaction, anaphylactic shock, refractory anaphylaxis and subheadings of diagnosis, classification, epidemiology, complications and pharmacology.

The enigmatic immunoglobulin G4-related disease and its varied cardiovascular manifestations.

Immunoglobulin G4-related disease (IgG4-RD) is a systemic fibroinflammatory disease characterised by multiorgan lymphoplasmacytic infiltration, obliterative phlebitis and storiform fibrosis. It can be associated with cardiovascular pathology. The objective of this narrative review is to summarise the published literature on cardiovascular manifestations of IgG4-RD and to provide a basis for diagnosis and management of the condition by the practising cardiologist.

Spin-orbit torque switching of an antiferromagnetic metallic heterostructure.

The ability to represent information using an antiferromagnetic material is attractive for future antiferromagnetic spintronic devices. Previous studies have focussed on the utilization of antiferromagnetic materials with biaxial magnetic anisotropy for electrical manipulation. A practical realization of these antiferromagnetic devices is limited by the requirement of material-specific constraints.

Common Variable Immune Deficiency and Associated Complications.

Common variable immunodeficiency disorders refer to a relatively common primary immune deficiency group of diseases that present with infectious and inflammatory complications secondary to defects in antibody production and sometimes in cellular immunity. The disorder often presents in middle age or later with recurrent sinopulmonary infections, bronchiectasis, or a plethora of noninfectious complications such as autoimmune disorders, granulomatous interstitial lung disease, GI diseases, malignancies (including lymphoma), and multisystem granulomatous disease resembling sarcoidosis. Infusion of immunoglobulin by IV or subcutaneous is the mainstay of therapy.

Chirally coupled nanomagnets.

Magnetically coupled nanomagnets have multiple applications in nonvolatile memories, logic gates, and sensors. The most effective couplings have been found to occur between the magnetic layers in a vertical stack. We achieved strong coupling of laterally adjacent nanomagnets using the interfacial Dzyaloshinskii-Moriya interaction.

Comparing the 1997 update of the 1982 American College of Rheumatology (ACR-97) and the 2012 Systemic Lupus International Collaborating Clinics (SLICC-12) criteria for systemic lupus erythematosus (SLE) classification: which enables earlier classification of SLE in an urban Asian population?

Objective: We compared the 1997 update of the 1982 American College of Rheumatology (ACR-97) and the 2012 Systemic Lupus International Collaborating Clinics (SLICC-12) criteria, for earlier classification of systemic lupus erythematosus (SLE) in a multiethnic urban Asian SLE population.

Methods: Patients from a retrospective, nested case-control study of the influence of lupus nephritis on mortality in SLE were studied. For each patient, dates of first manifestations of each criteria (both ACR-97 and SLICC-12) were recorded, and the date of disease classification using ACR-97 or SLICC-12 criteria was compared to determine which criteria resulted in earlier classification.