Cronkhite-Canada syndrome — acquired non-Mendelian polyposis- ectodermal syndrome. Rare (...

Детермінований перегляд YAML-сутності з джерельної бази. Клінічний авторитет лишається за вказаними source ID та статусом клінічного sign-off.

ID	`RF-CRONKHITE-CANADA-CONFIRMED`
Тип	Тривожна ознака
Статус	переглянуто 2026-05-20 \| очікує клінічного підпису
Хвороби	`DIS-CRC` `DIS-GASTRIC`
Джерела	`SRC-AGA-IBD-CRC-2024` `SRC-NCCN-GENETIC-FAMILIAL-CRC-2025`

Походження тривожної ознаки

Визначення	Cronkhite-Canada syndrome — acquired non-Mendelian polyposis- ectodermal syndrome. Rare (~1000 reported cases globally; primarily middle-aged + elderly adults). Clinical presentation: gastrointestinal polyposis (juvenile-like, throughout stomach + small bowel + colon diffusely) + ectodermal changes (diffuse alopecia, nail dystrophy with onycholysis, skin hyperpigmentation) + protein-losing enteropathy (hypoalbuminemia, edema, weight loss, electrolyte derangement). Lifetime CRC risk ~12-25%; gastric cancer also elevated per IPCG case-series. Pathogenesis unclear — autoimmune-like; may respond to immunosuppression (prednisone, azathioprine) + nutritional support; untreated mortality high (~50% 5-year if uncontrolled). Diagnosis is clinical + supported by classic GI biopsy + ectodermal pattern. Patient has confirmed clinical diagnosis; no current personal cancer diagnosis. Prevention-perso...
Клінічний напрям	investigate
Категорія	other

Логіка спрацьовування

{
  "any_of": [
    {
      "finding": "cronkhite_canada_clinical_diagnostic_criteria_met",
      "value": true
    },
    {
      "finding": "gastrointestinal_polyposis_with_ectodermal_changes",
      "value": true
    },
    {
      "finding": "protein_losing_enteropathy_with_diffuse_gi_polyposis",
      "value": true
    }
  ],
  "type": "lab_value"
}

Нотатки

Prevention-persona RedFlag for Cronkhite-Canada syndrome. risk_category set to chronic_condition (not genetic) because etiology is acquired autoimmune-like + non-Mendelian. Surveillance: colonoscopy q1-2y from diagnosis; upper endoscopy q1-2y; small-bowel capsule q2y; treat active disease with immunosuppression (prednisone taper + azathioprine maintenance) + nutritional support (protein replacement, electrolyte correction). Mortality reduced markedly with immuno- suppression treatment. STUB pending two-Co-Lead signoff per CHARTER §6.1 dev-mode.

Де використовується

Indications

IND-CRONKHITE-CANADA-OBSERVATION - IND-CRONKHITE-CANADA-OBSERVATION
IND-CRONKHITE-CANADA-SURVEILLANCE - IND-CRONKHITE-CANADA-SURVEILLANCE