Download full-text PDF |
Source |
|---|
Publication Analysis
Top Keywords
Similar Publications
Adjunctive effects of eltrombopag on immunosuppressive therapy for childhood aplastic anemia.
Int J Hematol
December 2024
Department of Pediatrics, Nagoya University Graduate School of Medicine, Nagoya, Japan.
Eltrombopag is used with first-line immunosuppressive therapy for adult aplastic anemia, although its practical utility in childhood remains unclear. We retrospectively analyzed the outcomes of pediatric patients who received eltrombopag in Japan. Of the 27 eligible patients, 23 (85%) were previously treated, and 15 (56%) had severe or very-severe disease.
View Article and Find Full Text PDFEpstein-Barr Virus encephalitis associated hemophagocytic lymphohistiocytosis in childhood-onset systemic lupus erythematosus: a case-based review.
Pediatr Rheumatol Online J
October 2024
Division of Rheumatology, Department of Pediatrics, Faculty of Medicine Vajira Hospital, Navamindradhiraj University, Bangkok, 10300, Thailand.
Article Synopsis
- Hemophagocytic lymphohistiocytosis (HLH) is an immune disorder causing severe inflammation, categorized into primary (familial) and secondary (acquired) types; secondary HLH can occur due to various triggers, including infections like Epstein-Barr virus (EBV).
- A 12-year-old girl with childhood-onset systemic lupus erythematosus (SLE) developed fever, seizures, and altered mental status, raising concerns for neuropsychiatric SLE or infections, leading to extensive testing and treatment.
- Diagnosis of HLH was confirmed through blood tests showing abnormal cell counts and bone marrow analysis revealing hemophagocytic activity, along with CSF analysis that identified EBV as the
Early Immunosuppressive Therapy and Ocular Complications in Pediatric and Young Adult Patients with Non-Infectious Uveitis at a Tertiary Referral Center in Japan.
Ocul Immunol Inflamm
December 2024
Department of Ophthalmology and Visual Science, Graduate School of Biomedical Sciences, Hiroshima University, Minami-ku, Hiroshima, Japan.
Purpose: To evaluate differences in the incidence of ocular complications among pediatric and young adult patients with non-infectious uveitis receiving immunosuppressive therapy (IMT), according to the time from uveitis onset to IMT initiation in Japan.
Methods: Patients aged < 20 years exhibiting uveitis treated with IMT (e.g.
Etoposide Therapy of Cytokine Storm Syndromes.
Adv Exp Med Biol
August 2024
Childhood Cancer Research Unit, Department of Women's and Children's Health, Karolinska Institutet, and Astrid Lindgren Children's Hospital, Karolinska University Hospital Solna, Stockholm, Sweden.
Etoposide has revolutionized the treatment of primary as well as secondary hemophagocytic lymphohistiocytosis (HLH), and it is, together with corticosteroids, the most widely used therapy for HLH. In the early 1980s, long-term survival in primary HLH was <5% but with the etoposide-/dexamethasone-based protocols HLH-94 and HLH-2004, in combination with stem cell transplantation, 5-year survival increased dramatically to around 60% in primary HLH, and based on analyses from the HLH-2004 study, there is likely room for further improvement. Biologically, etoposide administration results in potent selective deletion of activated T cells as well as efficient suppression of inflammatory cytokine production.
View Article and Find Full Text PDFCyclosporine A in children with ABCA3 deficiency.
Pediatr Pulmonol
December 2024
Dr. von Haunersches Kinderspital, University of Munich, German Center for Lung Research (DZL), Munich, Germany.
Background: Biallelic ATP-binding cassette subfamily A member 3 (ABCA3) variants can cause interstitial lung disease in children and adults, for which no proven treatments exist. Recent in vitro evidence suggested that cyclosporine A (CsA) could correct some ABCA3 variants, however for other variants this is unknown and no data in patients exist.
Methods: We retrieved the clinical data of two children aged 2 and 4 years carrying homozygous ABCA3 variants (G210C and Q1045R, respectively) and empiric CsA treatment from the Kids Lung Register database.
Want AI Summaries of new PubMed Abstracts delivered to your In-box?
Enter search terms and have AI summaries delivered each week - change queries or unsubscribe any time!