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endo.sheehan-syndrome.core.v1

Sheehan syndrome (postpartum hypopituitarism)

endocrinologyacutechronicpregnancyadultacuteinpatientoutpatient
Start encounter →Print handoutPRODUCTION

PLACEHOLDER manifest — points at prisma/seed/manifests/endo.adrenal-crisis.core.v1.ts until a dedicated endo.sheehan-syndrome manifest is authored (tracked in design brief Open gaps). No problem-package folder under src/lib/tier3/problem-package/packages/ for Sheehan syndrome — design brief + atoms not yet authored. RxNav CUIs intentionally deferred — no rxcui fields authored; RegimenDrug entries carry generic_name/class/role/dose only. RxNav validation to be wired before PRODUCTION. Calculator inventory limited to whitelist (calc.qsofa / calc.news2 / calc.corrected_ca); no Sheehan-specific score exists — severity is clinical (refractory shock, severe Na, symptomatic hypoglycemia). Bayesian likelihood ratios for the failure-to-lactate + PPH recognition pivot to be wired in a later pass.

Entry points (5)

  • symptom
    Failure to lactate after delivery complicated by severe PPH (Fleseriu JCEM 2016; Karaca Pituitary 2021)
    failure_to_lactate_postpartum
  • vital_abnormality
    Postpartum hypotension/shock not responding to fluids and pressors (Fleseriu JCEM 2016; Diri Endocrine 2016)
    postpartum_refractory_shock
  • symptom
    Delayed: agalactia + failure to resume menses + fatigue/cold intolerance months–years after obstetric hemorrhage (Karaca Pituitary 2021; Kilicli J Endocrinol Invest 2023)
    persistent_amenorrhea_fatigue_after_pph
  • lab_abnormality
    Low target hormone with inappropriately low/normal trophic hormone — central pattern (Fleseriu JCEM 2016)
    central_hormone_pattern
  • problem_list
    Prior peripartum hemorrhage / hypovolemic shock with nonspecific hypopituitary features (Karaca Pituitary 2021)
    obstetric_hemorrhage_history

Required inputs (15)

  • agerequired
    demographic • used at CONTEXT
    Woman of reproductive age; estrogen/progestin replacement until natural menopause; pregnancy intent affects plan (Fleseriu JCEM 2016)
  • peripartum_hemorrhage_historyrequired
    history • used at CONTEXT
    Severe PPH / hypovolemic shock is the defining etiologic substrate; high index of suspicion driver (Karaca Pituitary 2021; Diri Endocrine 2016)
  • lactation_historyrequired
    history • used at CONTEXT
    Failure to lactate is the cardinal early pivot (low/absent prolactin effect) (Karaca Pituitary 2021)
  • menstrual_history_postpartumrequired
    history • used at CONTEXT
    Failure to resume menses / amenorrhea is a classic delayed feature (gonadotropin deficiency) (Karaca Pituitary 2021)
  • sbprequired
    vital • used at RED_FLAGS
    Refractory hypotension/shock defines the acute secondary adrenal crisis presentation (Fleseriu JCEM 2016)
  • hrrequired
    vital • used at RED_FLAGS
    Tachycardia with shock; relative bradycardia possible with severe central hypothyroidism (Fleseriu JCEM 2016)
  • cortisolrequired
    lab • used at INITIAL_WORKUP
    Morning cortisol — low with inappropriately low/normal ACTH = central adrenal insufficiency; draw before steroid but do NOT delay treatment in crisis (Fleseriu JCEM 2016)
  • acth
    lab • used at INITIAL_WORKUP
    Inappropriately low/normal ACTH localizes the defect to the pituitary (secondary AI) (Fleseriu JCEM 2016)
  • free_t4required
    lab • used at INITIAL_WORKUP
    Low free T4 with low/normal TSH = central hypothyroidism; TSH is unreliable in central disease (Fleseriu JCEM 2016; Persani JCEM 2018)
  • tshrequired
    lab • used at INITIAL_WORKUP
    Interpreted ONLY alongside free T4 — an inappropriately normal TSH with low free T4 is central hypothyroidism (Persani JCEM 2018)
  • prolactin
    lab • used at INITIAL_WORKUP
    Low/absent prolactin correlates with lactation failure and supports the diagnosis (Karaca Pituitary 2021)
  • sodiumrequired
    lab • used at INITIAL_WORKUP
    Hyponatremia from cortisol +/- thyroid deficiency; SIADH-like dilutional pattern; severe Na drives ICU + correction rate ceiling (Fleseriu JCEM 2016)
  • glucoserequired
    lab • used at INITIAL_WORKUP
    Hypoglycemia from cortisol +/- GH deficiency, especially peripartum and in delayed crises (Fleseriu JCEM 2016)
  • igf_1
    lab • used at BRANCHING_WORKUP
    Low IGF-1 supports GH deficiency once other axes are replaced (Fleseriu JCEM 2016)
  • pituitary_mri
    imaging • used at BRANCHING_WORKUP
    Early — pituitary enlargement / non-enhancing gland; late — partial or empty sella (Karaca Pituitary 2021)

12-phase flow (12)

  1. 1FRAME
    Recognize Sheehan pattern: severe PPH/hypovolemic shock substrate + EITHER acute postpartum refractory shock/hypoglycemia/failure to lactate OR delayed central panhypopituitarism unmasked by stress (Fleseriu JCEM 2016; Karaca Pituitary 2021)
    inputs: peripartum_hemorrhage_history, lactation_history, sbp
    advance: obstetric-hemorrhage substrate present AND acute or delayed hypopituitary pattern recognized (Karaca Pituitary 2021)
  2. 2ENTRY
    Capture triggering presentation — failure to lactate, postpartum refractory shock, or delayed amenorrhea/fatigue/cold intolerance — and confirm Sheehan is on the differential (Karaca Pituitary 2021; Kilicli J Endocrinol Invest 2023)
    inputs: age
    advance: entry trigger + demographic documented; acute vs delayed track assigned (Karaca Pituitary 2021)
  3. 3CONTEXT
    Capture obstetric history (PPH severity, transfusion, DIC, hypovolemic shock), small-sella/autoimmune predisposition, lactation and menstrual history, current intercurrent stressor (infection/surgery/labor) that may unmask delayed disease (Karaca Pituitary 2021; Diri Endocrine 2016)
    inputs: peripartum_hemorrhage_history, lactation_history, menstrual_history_postpartum, age
    advance: etiologic substrate + acute/delayed track + intercurrent stressor captured (Karaca Pituitary 2021)
  4. 4RED_FLAGS
    Postpartum refractory hypotension/shock unresponsive to fluids and pressors (secondary adrenal crisis), symptomatic hypoglycemia, severe hyponatremia <125, AMS/seizure, postpartum DI (rare) (Fleseriu JCEM 2016; Diri Endocrine 2016)
    inputs: sbp, hr, sodium, glucose
    actions: calc.qsofa, calc.news2
    advance: red flags screened; empiric stress-dose hydrocortisone ordered FIRST without waiting for cortisol/ACTH (Fleseriu JCEM 2016)
  5. 5INITIAL_WORKUP
    Anterior pituitary panel interpreted by CENTRAL rules: morning cortisol +/- ACTH, free T4 with TSH (TSH unreliable in central disease), prolactin, gonadotropins/estradiol, plus Na, glucose, CMP; postpartum cortisol/ACTH-stim caveats noted (Fleseriu JCEM 2016; Persani JCEM 2018)
    inputs: cortisol, acth, free_t4, tsh, prolactin, sodium, glucose
    actions: workup.hypopituitarism, panel.hormone, panel.thyroid, panel.metabolic
    advance: anterior pituitary panel sent and interpreted by central rules; empiric hydrocortisone given if acute (Fleseriu JCEM 2016)
  6. 6BRANCHING_WORKUP
    Once stabilized: IGF-1 (GH axis), LH/FSH/estradiol (gonadotropin axis), pituitary MRI (early enlargement/non-enhancing; late partial/empty sella), paired serum/urine osmolality if polyuria for central DI, and dynamic testing only after acute glucocorticoid replacement (Fleseriu JCEM 2016; Karaca Pituitary 2021)
    inputs: igf_1, pituitary_mri
    actions: workup.hyponatremia, workup.amenorrhea
    advance: all anterior axes characterized + MRI obtained + DI assessed (Karaca Pituitary 2021)
  7. 7DIFFERENTIAL
    Phenotype: acute postpartum Sheehan crisis vs classic delayed Sheehan panhypopituitarism; distinguish from pituitary apoplexy (acute hemorrhagic into adenoma, headache/visual), lymphocytic hypophysitis (postpartum, autoimmune, often isolated ACTH/central DI, stalk thickening), primary adrenal/thyroid disease, and postpartum thyroiditis (Karaca Pituitary 2021; Diri Endocrine 2016)
    advance: phenotype assigned and apoplexy / hypophysitis / primary endocrine mimics excluded (Karaca Pituitary 2021)
  8. 8RISK_STRATIFICATION
    qSOFA / NEWS2 for ICU triage of the acute crisis; corrected calcium for symptom interpretation; severity classified by refractory shock, severe hyponatremia, symptomatic hypoglycemia, AMS (Fleseriu JCEM 2016)
    inputs: sbp, hr, sodium, glucose
    actions: calc.qsofa, calc.news2, calc.corrected_ca
    advance: severity documented; ICU disposition decided if shock/AMS/severe Na (Fleseriu JCEM 2016)
  9. 9TREATMENT
    ACUTE crisis: stress-dose hydrocortisone + IV fluids + glucose + correct hyponatremia FIRST. CARDINAL RULE: glucocorticoid BEFORE levothyroxine (levothyroxine first precipitates adrenal crisis). Then levothyroxine after GC, estrogen/progestin until menopause, GH where indicated, desmopressin if central DI; chronic individualized replacement + sick-day/stress dosing + steroid card (Fleseriu JCEM 2016; Karaca Pituitary 2021)
    inputs: sbp, sodium, glucose, cortisol, free_t4
    advance: hydrocortisone + fluids + glucose + Na correction in flight; levothyroxine deferred until glucocorticoid established (Fleseriu JCEM 2016)
  10. 10DISPOSITION
    ICU for refractory postpartum shock/AMS/severe Na; obstetric source control if ongoing hemorrhage; endocrine consult day 1; outpatient endocrine track for delayed presentation (Fleseriu JCEM 2016; Karaca Pituitary 2021)
    advance: ICU vs ward vs outpatient decided; endocrine + OB/MFM consults made as indicated (Fleseriu JCEM 2016)
  11. 11MONITORING
    Acute: hourly vitals, q4–6h Na + glucose, mental status, Na correction <8 mEq/L/24h ceiling; chronic: clinical replacement adequacy, free T4 (NOT TSH) for levothyroxine titration, IGF-1 for GH, BP/weight/electrolytes on hydrocortisone/fludrocortisone (Fleseriu JCEM 2016; Persani JCEM 2018)
    inputs: sodium, glucose, free_t4
    actions: panel.metabolic, panel.thyroid
    advance: acute targets met with safe Na correction OR chronic replacement adequacy documented (Fleseriu JCEM 2016)
  12. 12FOLLOWUP
    Lifelong individualized replacement, sick-day rules + emergency hydrocortisone IM kit + steroid card + medical alert ID, preconception counseling for future pregnancies, transition to chronic hypopituitarism management, family/obstetric documentation (Fleseriu JCEM 2016; Karaca Pituitary 2021)
    advance: sick-day rules taught; steroid card + emergency kit issued; preconception counseling delivered; chronic endocrine follow-up scheduled (Fleseriu JCEM 2016)