P-484: New onset type 1 DM presenting with severe diabetic ketoacidosis, delayed puberty and possible autoimmune polyglandular syndrome-3 in an 18-year-old female: a case report

Introduction

Diabetic ketoacidosis (DKA) is a common and life-threatening initial presentation of type 1 DM, particularly in undiagnosed adolescents. Autoimmune polyglandular syndrome (APS) refers to the coexistence of two or more autoimmune endocrine disorders. APS type 1 is rare and monogenic, while APS type 2 (more common) includes T1DM with thyroid or adrenal disease; APS type 3 involves autoimmune thyroid disease with other autoimmune conditions excluding adrenal insufficiency. APS is uncommon, with prevalence estimated at 1:20,000-1:100,000 for APS-1 and higher for APS-2, but reports from Nigeria remains scarce.

Case Presentation

18-year-old female who presented with a one-week history of abdominal pain, polyuria, polydipsia, vomiting, weakness, and loss of consciousness.

She was unconscious (GCS 3/15), pale, dehydrated, tachycardic, tachypnoeic, Kussmaul respiration. Her height 152cm (between 5th and 10th percentile), weight 35kg (<5th percentile). Sexual maturity ratings- breast-3, pubic hair-2, axillary hair-2. Had vitiligo, bilateral leg oedema, yet to attain menarche.

Investigations showed severe hyperglycaemia (RBG- unrecordably high), Urinalysis (glycosuria++, ketonuria+++), metabolic acidosis (HCO3-15mmol/L), hypokalaemia (K+-3.0mmol/L), anaemia (PCV 20%), hypoalbuminaemia (Albumin-18g/dl), elevated urea/creatinine. Thyroid Function Test showed: Low FT4 (9.74pmol/L), low TSH (0.3691Uiu/mL), normal FT3 (5.01pmol/L). X-ray of the left wrist shows delayed bone age (bone age-16years). Yet to do serum cortisol due to financial constraints. Patient is an orphan and most of investigations were done by donations from the health care team members.

Objectives

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Methods

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Results

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Conclusions

The combination of central hypothyroidism, autoimmune skin disease and T1DM in this patient is more suggestive of APS-3. This case highlights the importance of long-term surveillance in adolescents with T1DM and extra-pancreatic autoimmune features, especially in resource-limited settings where delayed diagnosis is common.