402.2 📚 國考版醫垫國考 / PGY OSCE

402.2.0.1 📌 Cram Sheet

402.2.0.1.1 🔥 高 yield 12
  1. APS-1 / APECED: AR, AIRE gene, juvenile
  2. APS-1 triad: candidiasis + hypoPTH + Addison’s
  3. APS-2 / Schmidt: polygenic, HLA-DR3/4, adult
  4. APS-2 components: Addison + Hashimoto + T1DM (≥ 2)
  5. IPEX: X-linked, FOXP3, severe infant
  6. AIRE specific Ab: anti-IFN-α/ω
  7. APS-2 Ab: 21-OH (Addison), TPO (thyroid), GAD (T1DM), IF (PA)
  8. ICI-induced (22E): thyroiditis (PD-1) > hypophysitis (ipi) > adrenalitis > T1DM
  9. Replacement: always cortisol first if concurrent AI + hypothyroid
  10. Lifelong surveillance for new components
  11. HSCT cure for IPEX
  12. Type 3 APS (informal) = thyroid AI + other (no Addison’s)
402.2.0.1.2 🔢 必背
項目 æ•žå­—
APS-1 inheritance AR
APS-2 inheritance Polygenic
IPEX inheritance XR
APS-1 gene AIRE
IPEX gene FOXP3
APS-1 triad Candidiasis + hypoPTH + Addison
APS-2 typical Addison + Hashimoto
ICI thyroiditis (PD-1) 10-20%
ICI hypophysitis (ipi) 5-10%
ICI T1DM ~1%

402.2.0.2 ⭐ 高 yield

402.2.0.2.1 APS Comparison
APS-1 APS-2 IPEX
Inheritance AR Polygenic XR
Gene AIRE HLA-DR3/4 + polygenic FOXP3
Onset Juvenile Adult (20-40) Infant
Triad/key Candidiasis + hypoPTH + Addison Addison + Hashimoto + T1DM Severe enteropathy + T1DM
Specific Ab Anti-IFN-α/ω 21-OH + TPO + GAD + IF —
Treatment Replace + antifungal Replace HSCT + replace
402.2.0.2.2 ICI Endocrine irAE
ICI Endocrine irAE
Anti-PD-1 (pembro, nivo) Thyroiditis (most common, biphasic)
Anti-CTLA-4 (ipilimumab) Hypophysitis (most common)
Anti-PD-L1 (atezolizumab, durvalumab) Both, less frequent
Combo (ipi + nivo) Higher rates of all
402.2.0.2.3 Treatment Pearls
  • Cortisol ALWAYS first if AI + hypothyroid suspected
  • Replace each hormone
  • Stress dose education
  • Medical alert bracelet
  • Annual surveillance for additional components
  • Genetic counseling (esp. APS-1)

402.2.0.3 🎯 自我檢枬

  1. APS-1 gene? → AIRE
  2. APS-1 triad? → Candidiasis + hypoPTH + Addison
  3. APS-1 specific Ab? → Anti-IFN-α/ω
  4. APS-2 typical components? → Addison + Hashimoto + T1DM
  5. Schmidt syndrome? → Addison + Hashimoto specifically
  6. IPEX gene? → FOXP3
  7. IPEX treatment? → HSCT
  8. ICI most common endocrine irAE? → Thyroiditis (PD-1)
  9. ICI hypophysitis 倚 in? → Ipilimumab
  10. APS-2 give which first? → Cortisol (HC)
  11. Surveillance? → Annual for new components
  12. Inheritance APS-1 vs APS-2? → AR vs polygenic
  13. Anti-21-hydroxylase? → Addison autoimmune
  14. Anti-GAD? → T1DM
  15. Anti-IF? → Pernicious anemia

⚠ AI 草皿。