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Polyarteritis nodosa with life-threatening intracranial aneurysms in a child, and treatment with infliximab.
Turk J Pediatr
December 2024
Department of Pediatric Rheumatology, Umraniye Training and Research Hospital, University of Health Sciences, İstanbul, Türkiye.
Background: Polyarteritis nodosa (PAN) is a rare and serious form of systemic necrotizing vasculitis that predominantly affects medium and small-sized arteries, with central nervous system involvement being particularly uncommon. Treatment strategies are tailored according to the extent and severity of the disease. While conventional therapy includes glucocorticoids and conventional disease-modifying-rheumatic drugs (cDMARDs), biologic agents may be critical for severe and refractory cases.
View Article and Find Full Text PDFFeasibility and resource utilization of nurse-administered subcutaneous immunoglobulin therapy in antibody deficiency: A cross-sectional study.
PLoS One
January 2025
Department of Medicine, Division of Pulmonary Medicine, University of Alberta, Edmonton, AB, Canada.
Primary and secondary antibody deficiencies (PAD and SAD) are amongst the most prevalent immunodeficiency syndromes, often necessitating long-term immune globulin replacement therapy (IRT). Both intravenous immunoglobulin (IVIG) and subcutaneous immunoglobulin (SCIG) have demonstrated efficacy in antibody deficiency. Comparative analyses of these two routes of administration are limited to nurse-administered IVIG and home therapy with self-administered SCIG.
View Article and Find Full Text PDFVacuolar myopathy with monoclonal gammopathy and stiffness (VAMMGAS).
Eur J Neurol
January 2025
Groupe Hospitalier Pitié-Salpêtrière, Institut de Myologie, AP-HP, Sorbonne Université, Paris, France.
Background: Monoclonal gammopathy (MG) has been reported in association with numerous neurological disorders but the spectrum of MG-associated myopathies remains poorly described.
Objective: To report a newly acquired myopathy associated with MG.
Methods: Three adult patients with the same phenotype from two French referral centers were prospectively analyzed.
An Atypical Case of Neonatal Alloimmune Thrombocytopenia.
J Pediatr Hematol Oncol
January 2025
Departments of Laboratory Medicine.
Fetal and neonatal alloimmune thrombocytopenia (FNAIT) results from maternal antibodies targeting fetal platelets during pregnancy, often causing hemorrhagic manifestations detectable antenatally or shortly after birth. We report an atypical form of FNAIT with delayed onset in a healthy, breastfed male infant who developed diffuse petechiae 2 weeks after birth due to severe thrombocytopenia. The mother was shown to be negative for the human platelet antigen-1a (HPA-1a) allele but had anti-HPA-1a IgG antibodies, while the father and newborn were HPA-1a positive, confirming the diagnosis.
View Article and Find Full Text PDFThyroid Autoimmunity: The Treatment of Hashimoto's Disease, or the Presence of Antithyroid Antibodies Alone, in Pregnancy.
Am J Reprod Immunol
January 2025
Placental Analytics, LLC, New Rochelle, New York, USA.
Problem: Hashimoto's disease is the commonest autoimmune disease of pregnancy. The presence of Anti-Thyroid antibodies (ATAs) alone [subclinical hypothyroidism] has also been shown to have adverse pregnancy effects. These can result in failure to conceive, recurrent miscarriages, anemia, preeclampsia, and abruption.
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