ECE2016 Eposter Presentations Adrenal cortex (to include Cushing's) (85 abstracts)
1American Hospital, Tirana, Albania; 2Neo-Style Clinic, Tirana, Albania; 3Regional Hospital, Korce, Albania; 4UHC Mother Teresa, Tirana, Albania.
Introduction: Polyglandular autoimmune syndrome type II (PGA-II) or Schmidts syndrome is a very rare autoimmune disorder and difficult to diagnose because the symptoms of this syndrome depends on the gland which gets involved first. Approximately 1420 people per million population are affected by polyglandular autoimmune syndrome type II. It is characterised by the obligatory occurrence of autoimmune Addisons disease in combination with thyroid autoimmune diseases and/or type 1 diabetes mellitus.
Case presentation: A 40-year-old lady presented to the Emergency Department with complaints of progressive weight loss 13 kg in eight months, anorexia, headache and vomiting. Physical examination on admission revealed: Hyperpigmentation was mostly expressed in the face, upper part of thorax, dorsal part of the hand. pulse was 114/mins, BP-80/60 mmHg and respiratory rate-22/mins. leucocyte count-6200 cells/mm3, Platelet count-31 4000 cells/mm3, fasting blood sugar-86 mg/dl, renal function tests, liver function tests, serum potassium 8.1 (3.75.5) sodium 135 (136148) Free T3 2.8 pmol/l, Free T4 10.6 pmol/l, TSH 12.16 IU/ml, random cortisol-2.23 ng/ml anti TPO level, Ac anti Tg 477.5 IU/ml. Hyperpigmentation, low cortisol level and high potassium raised the possibility of Type II polyglandular autoimmune syndrome. cosyntropin test was suggestive of primary adrenal insufficiency. Normal Abdominal CT and Pituitary MRI. Antibody against 21 (OH) were high 23 Norma <10. She was diagnosed as polyglandular autoimmune type 2 (or Schmidts syndrome). She was started on steroid replacement therapy. thyroxin 2 weeks after the steroid therapy. During her follow- up after one year later she developed also Diabetes Mellitus and insulin treatment was started. Our patient was treated and improved with corticosteroid, thyroxine and insulin therapy.
Conclusion: Every patient with endocrine deficiency should be screened for other insufficiency of other endocrine organs.