A 7-year-old Chinese boy presented with acute pancreatitis. The characteristic rash of Henoch-Schonlein purpura (HSP) did not develop until nine days later, together with painful scalp swelling and calf pain. Acute pancreatitis has only rarely been reported in association with HSP and never before as the sole presenting feature.

Pancreatitis associated with Henoch-Schonlein purpura.

Journal of Paediatrics and Child Health

The purpose of this manuscript is to review the effects of sodium-glucose cotransport protein 2 inhibitors (SGLT2is) in patients with chronic kidney disease according to basic mechanisms, current recommendations, and future perspectives. Based on growing evidence from randomized, controlled trials, SGLT2is have proven their benefit on cardiac and renal adverse complications, and their indications expanded into the following five categories: glycemic control, reduction in atherosclerotic cardiovascular disease (ASCVD), heart failure, diabetic kidney disease, and nondiabetic kidney disease. Although kidney disease accelerates the progression of atherosclerosis, myocardial disease, and heart failure, so far, no specific drugs were available to protect renal function. Recently, two randomized trials, the DAPA-CKD and EMPA-Kidney, demonstrated the clinical benefit of the SGLT2is dapagliflozin and empagliflozin in improving the outcome in patients with chronic kidney disease. For the consistently positive results in cardiorenal protection, the SGLT2i represents an effective treatment to reduce the progression of kidney disease or death from cardiovascular causes in patients with and without diabetes mellitus.

SGLT2 Inhibitors: A New Therapeutical Strategy to Improve Clinical Outcomes in Patients with Chronic Kidney Diseases.

International Journal of Molecular Sciences

Patients with SLE are at high risk of various infections as evidenced by a number of studies. The main determinants of infection in SLE are disease activity, organ damage, and often inevitable medication. The molecular and cellular mechanisms underlying infection remain unclear. Impaired immunity, immunosuppressants and corticosteroids clearly increase the risk of infection, whereas some medications, such as low-dose IL-2, hydroxychloroquine and IVIG are safe in SLE patients with substantial evidence. It is important to balance the immunosuppression and infection risks in practice. This article focuses on medication-related infections in SLE and discusses the therapeutic options for the disease in clinical practice.

Dilemma of immunosuppression and infection risk in systemic lupus erythematosus.

Rheumatology

New-onset refractory status epilepticus (NORSE) is "a clinical presentation, not a specific diagnosis, in a patient without active epilepsy or other preexisting relevant neurological disorder, with new onset of refractory status epilepticus without a clear acute or active structural, toxic, or metabolic cause." Febrile infection related epilepsy syndrome (FIRES) is "a subcategory of NORSE that requires a prior febrile infection, with fever starting between 2 weeks and 24 h before the onset of refractory status epilepticus, with or without fever at the onset of status epilepticus." These apply to all ages. Extensive testing of blood and CSF for infectious, rheumatologic, and metabolic conditions, neuroimaging, EEG, autoimmune/paraneoplastic antibody evaluations, malignancy screen, genetic testing, and CSF metagenomics may reveal the etiology in some patients, while a significant proportion of patients' disease remains unexplained, known as NORSE of unknown etiology or cryptogenic NORSE. Seizures are refractory and usually super-refractory (i.e., persist despite 24&#x2009;h of anesthesia), requiring a prolonged intensive care unit stay, often (but not always) with fair to poor outcomes. Management of seizures in the initial 24-48&#x2009;h should be like any case of refractory status epilepticus. However, based on the published consensus recommendations, the first-line immunotherapy should begin within 72&#x2009;h using steroids, intravenous immunoglobulins, or plasmapheresis. If there is no improvement, the ketogenic diet and second-line immunotherapy should start within seven days. Rituximab is recommended as the second-line treatment if there is a strong suggestion or proof of an antibody-mediated disease, while anakinra or tocilizumab are recommended for cryptogenic cases. Intensive motor and cognitive rehab are usually necessary after a prolonged hospital stay. Many patients will have pharmacoresistant epilepsy at discharge, and some may need continued immunologic treatments and an epilepsy surgery evaluation. Extensive research is in progress now via multinational consortia relating to the specific type(s) of inflammation involved, whether age and prior febrile illness affect this, and whether measuring and following serum and/or CSF cytokines can help determine the best treatment.

A practical approach to in-hospital management of new-onset refractory status epilepticus/febrile infection related epilepsy syndrome.

Frontiers in Neurology

Antiphospholipid antibodies (APLAs) are&#xa0; primarily directed toward phospholipid-binding proteins and are responsible for thrombotic events. APLAs include anti-&#x3b2;2Glycoprotein I (anti-&#x3b2;2GPI), anticardiolipin (anti-CL) antibodies, and lupus anticoagulant. These antibodies are typical markers of antiphospholipid syndrome (APS) and are a part of its diagnostic criteria. Many data underline the presence of APLAs in other rheumatic diseases (e.g., systemic lupus erythematosus, systemic sclerosis, Sj&#xf6;gren's syndrome, rheumatoid arthritis and Beh&#xe7;et's disease). However, they are also detected in patients with cancer, infection, and neurological disorders. Furthermore, healthy individuals may be carriers of APLAs. Chronic asymptomatic APLAs presence is most common in the elderly and subjects with chronic diseases (including malignancies). Specific kinds of APLAs are considered markers of oncological progression. These antibodies occur in 6% of pregnant women (without diagnosed APS) and are related to many pregnancy complications. Of worth, various types of APLAs are reported to have different prothrombotic properties. The risk of thrombotic events in APLA-positive but clinically na&#xef;ve patients raises many questions in clinical practice. This manuscript analyses various clinical situations and consequences of the APLAs' presence, particularly in patients without diagnosed APS. The prevalence, etiology, molecular background, and prothrombotic properties of numerous APLAs are broadly discussed. The new management approach in different clinical conditions and organ complications is present in the context of recent recommendations. Discussed data underlines that adequate and timely introduced thromboprophylaxis can decrease the risk of thrombus formation and prevent increased morbidity.

Pancreatitis associated with Henoch-Schonlein purpura.

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Pancreatitis associated with Henoch-Schonlein purpura.

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