A case of polyglandular autoimmune syndrome type i with unusual presentation | [Journal of College of Physicians and Surgeons--Pakistan : JCPSP • 2011]

Author(s):

1. Ahmad Usaid Qureshi: Department of Paediatric Medicine, The Children’s Hospital and Institute of Child Health, Lahore, Pakistan.

2. Rabia Abbas: Department of Paediatric Medicine, The Children’s Hospital and Institute of Child Health, Lahore, Pakistan.

3. Tahir Masood Ahmad: Department of Paediatric Medicine, The Children’s Hospital and Institute of Child Health, Lahore, Pakistan.

Abstract:

Eight years old girl presented with mucocutaneous candidiasis, nail dystrophy, twitching left half of face, progressively increasing generalized skin hyperpigmentation and hypopigmented patches over both shins. Her investigations revealed low intact PTH level, low serum cortisol, high ACTH, impaired glucose tolerance test and candidal onycomycosis. She was diagnosed as Polyglandular Autoimmune Syndrome (PGA) type I. She also developed idiopathic generalized epileptiform seizures and Alcaligenes faecalis septicemia not previously reported with PGA type I. The patient responded well to alphacalcidol, hydrocortisone, valproate sodium, topical antifungal and systemic antibiotics.

Page(s): 187-189

DOI: DOI not available

Published: Journal: Journal of College of Physicians and Surgeons--Pakistan : JCPSP, Volume: 21, Issue: 3, Year: 2011

Keywords:

Polyglandular Autoimmune Syndrome type I Seizures Autoimmune Hypocalcaemia Alcaligenes faecalis septicemia

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