title: Primary antiphospholipid antibody syndrome presenting with adrenal insufficiency in a child: case report and review of literature.
abstract: A 7-year-old boy presented with adrenal insufficiency. He subsequently developed venous thrombosis in the limbs and was diagnosed with primary antiphospholipid syndrome (PAPS) based on clinical and laboratory parameters. Both adrenals were normal on imaging. He required thrombolysis and anticoagulation. The progressive course of PAPS was controlled with methylprednisolone. There are few reports of PAPS in pediatric patients, and associated adrenal involvement is rare. The unusual presentation, course and management of the patient and of four other reported children with adrenal insufficiency heralding manifestation of PAPS are discussed.
authors.affiliation: All India Institute of Medical Sciences, New Delhi, India. drbhanu04@yahoo.co.in
authors.forename: B K
authors.initials: BK
authors.lastname: Bhakhri
authors.forename: S
authors.initials: S
authors.lastname: Katewa
authors.forename: R
authors.initials: R
authors.lastname: Sharma
authors.forename: S
authors.initials: S
authors.lastname: Mahajan
mesh_terms: D000305:Adrenal Cortex Hormones / Q000627:therapeutic use; D000309:Adrenal Insufficiency / Q000150:complications* / Q000175:diagnosis* / Q000188:drug therapy; D016736:Antiphospholipid Syndrome / Q000150:complications* / Q000175:diagnosis* / Q000188:drug therapy; D002648:Child; D006801:Humans; D008297:Male; D015912:Thrombolytic Therapy; D014057:Tomography, X-Ray Computed
chemical_list: D000305:Adrenal Cortex Hormones