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Inborn Errors of Immunity

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35 Intervention/management Strength of recommendation Certainty of evidence Immunomodulatory therapy, first-line (e.g., enteral or systemic steroids) Strong Moderate Do not avoid gluten unless patients have celiac- associated HLA markers Strong Moderate Antimicrobials (e.g., nitazoxanide, ribavirin) and/ or diverse drugs (e.g., enteral steroids, enteral immunoglobulin) Conditional Very low 5-Aminosalicylates, corticosteroids, ustekinumab Conditional Low Use tumor necrosis factor (TNF)-α inhibitors with caution of risk of severe infection Conditional Moderate HSCT Conditional Moderate Initial treatment with culture and biopsy-directed antimicrobials and systemic corticosteroids Strong High Surgical management with debridement or resection if medical management is inadequate Strong Moderate Medical and surgical management of non-cirrhotic portal hypertension to prevent variceal bleeding Conditional Low Liver transplantation is discouraged Conditional Low Corticosteroids, immunomodulators Conditional Low Precision therapy based on underlying molecular defect (e.g., abatacept, jakinibs) Conditional Low

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