acyclovir and Systemic-Inflammatory-Response-Syndrome

The objectives of this study were (i) to describe the clinical presentation, treatment and outcome of paediatric inflammatory multisystem syndrome temporally related to Sars-CoV-2 (PIMS-TS) in children; (ii) to propose a framework to guide multidisciplinary team (MDT) management; and (iii) to highlight the role of the paediatric rheumatologist in this context.. This study involved a retrospective case notes review of patients referred to a single specialist paediatric centre with suspected PIMS-TS, with a focus on clinical presentation, laboratory parameters, treatment, and outcome in the context of an MDT framework.. Nineteen children of median age 9.1 years fulfilled the definition of PIMS-TS and were managed within an MDT framework: 5/19 were female; 14/19 were of Black, Asian or minority ethnicity; 9/19 also fulfilled diagnostic criteria for complete or incomplete Kawasaki disease (KD). Severe systemic inflammation, shock, and abdominal pain were ubiquitous. Treatment was stratified within an MDT framework and included CSs in all; i.v. immunoglobulin in all; anakinra in 4/19; infliximab in 1/19; and antiviral (aciclovir) in 4/19.. We observed significant diagnostic equipoise using a current definition of PIMS-TS, overlapping with KD. Outside of clinical trials, an MDT approach is vital. The role of the paediatric rheumatologist is to consider differential diagnoses of hyperinflammation in the young, to advise on empiric immunomodulatory therapy, to set realistic therapeutic targets, to gauge therapeutic success, to oversee timely step-down of immunomodulation, and to contribute to the longer-term MDT follow-up of any late inflammatory sequelae.

Topics: Abdominal Pain; Acyclovir; Adolescent; Adrenal Cortex Hormones; Antirheumatic Agents; Antiviral Agents; Asian People; Black People; Child; COVID-19; COVID-19 Drug Treatment; Diagnosis, Differential; Female; Humans; Immunoglobulins, Intravenous; Immunologic Factors; Inflammation; Infliximab; Interleukin 1 Receptor Antagonist Protein; Male; Mucocutaneous Lymph Node Syndrome; Patient Care Team; Physician's Role; Retrospective Studies; Rheumatologists; SARS-CoV-2; Severity of Illness Index; Shock; Systemic Inflammatory Response Syndrome; United Kingdom; White People

A young woman presented with a febrile illness in the third trimester of pregnancy. Laboratory investigation revealed severe acute hepatitis with thrombocytopenia and coagulopathy. Liver injury progressed despite emergent caesarian section and delivery of a healthy infant. Therefore, therapeutic plasma exchange (TPE) was performed on three consecutive days post-partum for a presumed diagnosis of acute liver failure (ALF) associated with pregnancy due to hemolysis, elevated liver enzymes, and low platelets (HELLP) or acute fatty liver of pregnancy (AFLP). Treatment with TPE was followed by biochemical and clinical improvement but during her recovery herpes simplex virus type 2 (HSV-2) infection was diagnosed serologically and confirmed histologically. Changes in the immune system during pregnancy make pregnant patients more susceptible to acute HSV hepatitis, HSV-related ALF, and death. The disease is characterized by massive hepatic inflammation with hepatocyte necrosis, mediated by both direct viral cytotoxicity and the innate humoral immune response. TPE may have a therapeutic role in acute inflammatory disorders such as HSV hepatitis by reducing viral load and attenuating systemic inflammation and liver cell injury. Further investigation is needed to clarify this potential effect. The roles of vigilance, clinical suspicion, and currently accepted therapies are emphasized.

Topics: Acute Disease; Acyclovir; Anti-Bacterial Agents; Antiviral Agents; Cesarean Section; Combined Modality Therapy; Dexamethasone; Emergencies; Female; Fetal Organ Maturity; Hepatitis, Viral, Human; Herpes Simplex; Humans; Hydrocortisone; Infant, Newborn; Liver Failure; Male; Plasma Exchange; Pregnancy; Pregnancy Complications; Pregnancy Complications, Infectious; Puerperal Disorders; Systemic Inflammatory Response Syndrome; Young Adult