391.3 🩺 內科專科考前版

391.3.0.1 📌 䞀頁重點

  • 22E updates:
    • Macimorelin oral GH stim approved for adult GHD
    • Pasireotide LAR for refractory acromegaly + Cushing’s
    • Osilodrostat (11β-hydroxylase inhibitor) for Cushing’s
    • Relacorilant (GR antagonist) for Cushing’s (post-mifepristone era)
    • Pegvisomant (GH-R antagonist) for acromegaly refractory
    • PRL receptor antagonist in trials
  • Taiwan: 健保 cabergoline / bromocriptine / octreotide LAR / lanreotide depot / pegvisomant / pasireotide / osilodrostat 條件

391.3.0.2 🌟 Pearls (15)

  1. Macimorelin (oral ghrelin agonist) for adult GHD — easier than ITT
  2. Pasireotide LAR — broader SSTR (1, 2, 3, 5) coverage; effective when octreotide fails; causes hyperglycemia in 60%+ (垞需芁䜵 DM 治療)
  3. Pegvisomant: pegylated GH-R antagonist; normalizes IGF-1 in 90% but doesn’t shrink tumor
  4. Osilodrostat: oral 11β-hydroxylase inhibitor for Cushing’s (LINC trials)
  5. Cabergoline > bromocriptine for prolactinoma (better efficacy, less SE, 2x/week dosing)
  6. Cabergoline cardiac valve risk: low at endocrine doses (< 2 mg/wk), higher in Parkinson’s doses
  7. Late-night salivary cortisol: most convenient outpatient screen (collect at 23:00)
  8. 24-h urine free cortisol vs dexamethasone-cortisol: complementary
  9. IPSS: gold for ACTH source (CD vs ectopic) when imaging equivocal — central:peripheral ACTH ratio > 2 (basal) or > 3 (post-CRH)
  10. Prolactin “hook effect”: dilute serum 1:100 if extreme high PRL with small tumor (assay saturation can give false low)
  11. Macroprolactinemia: PRL-IgG complex, biologically inactive — always rule out before treating (PEG precipitation test)
  12. Stalk effect PRL usually < 200, often < 100; helps differentiate non-functional from prolactinoma
  13. Hypopituitarism cortisol replacement order: ALWAYS replace cortisol first before T4 (T4 → adrenal crisis if no cortisol)
  14. Acromegaly cardiomyopathy: leading cause of mortality
  15. Apoplexy: surgical emergency if visual / consciousness compromise; medical if stable + no chiasm

391.3.0.3 📍 Taiwan + 健保

391.3.0.3.1 Acromegaly
  • 健保 octreotide LAR / lanreotide depot 條件絊付surgical fail or not surgical candidate
  • 健保 pegvisomant 條件
  • 健保 pasireotide LAR 條件
  • 健保 cabergoline (適 acromegaly with PRL co-secretion)
391.3.0.3.2 Cushing’s Disease
  • 健保 ketoconazole / metyrapone (off-label use, 條件)
  • 健保 osilodrostat 條件
  • Pasireotide LAR 條件
  • Cabergoline (off-label)
391.3.0.3.3 Prolactinoma
  • 健保 cabergoline + bromocriptine 充分絊付
  • 經 trans-sphenoidal surgery 健保
391.3.0.3.4 GH Deficiency (Adult)
  • 健保 somatropin 條件嚎栌biochemical proof + symptoms
  • 健保 macimorelin 條件

391.3.0.4 🎓 內專必懂 (15)

  1. Pituitary anatomy + cavernous sinus 結構 (CN III/IV/V1/V2/VI)
  2. Embryology: Rathke pouch (anterior) vs neuroectoderm (posterior)
  3. Portal hypophyseal vessels carry hypothalamic hormones
  4. 5 cell types + hormones + receptors
  5. HPA / HPT / HPG axis + GH + PRL 5 軞
  6. Acromegaly screen + treatment ladder (TSS → SSA → pegvisomant → pasireotide → RT)
  7. Cushing’s disease screen → confirm → localize (low-dose dex → late-night salivary → 24h urine → CRH/IPSS)
  8. Prolactinoma: cabergoline 1st line; surgery for cabergoline-resistant or specific
  9. Stalk effect characteristics (PRL < 100, non-functional adenoma)
  10. Hook effect + macroprolactinemia caveats
  11. Hypopituitarism replacement order (cortisol first!)
  12. Sheehan syndrome + lymphocytic hypophysitis
  13. Apoplexy — surgical emergency
  14. Pituitary incidentaloma management (workup + sequential imaging)
  15. 22E new drugs: macimorelin, pasireotide LAR, osilodrostat, relacorilant, pegvisomant

391.3.0.5 ⚙ Acromegaly Treatment Ladder (內專)

1st Line: Trans-sphenoidal surgery (TSS) — 治癒率 microadenoma 80%, macroadenoma 50%
   ↓ if not cured / not surgical
2nd Line: Somatostatin analog (octreotide LAR 20-40 mg q4w / lanreotide depot 120 mg q4w)
   ↓ if 䞍耐 or 無 response (~50%)
3rd Line: Pasireotide LAR 40-60 mg q4w (broader SSTR; ↑ hyperglycemia)
   OR Pegvisomant 10-30 mg/d SC (GH-R antagonist; LFT monitoring; tumor not shrunk)
   ↓
4th Line: Stereotactic radiation (Gamma Knife / CyberKnife)
   ↓
Cabergoline (mild cases or PRL co-secretion)

391.3.0.6 ⚙ Cushing’s Disease Treatment Ladder

1st Line: TSS
   ↓ if 侍 cured / recurrence
2nd Line: Repeat TSS / RT
   ↓
Medical (any of):
- Steroidogenesis inhibitor: ketoconazole, metyrapone, **osilodrostat**, levoketoconazole, etomidate
- Pituitary-directed: pasireotide, cabergoline
- GR antagonist: mifepristone, **relacorilant**
   ↓
Bilateral adrenalectomy + permanent steroid replacement (last resort; risk Nelson)

⚠ AI 草皿。