Immunodysregulation Polyendocrinopathy Enteropathy X-Linked Syndrome (IPEX) Follow-up

Updated: Aug 06, 2019
  • Author: Taylor Banks, MD; Chief Editor: Harumi Jyonouchi, MD  more...
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Follow-up

Further Outpatient Care

Close medical follow-up and specialty care is required to monitor and manage the infections and autoimmune complications of IPEX syndrome.

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Further Inpatient Care

Patients who have immune dysfunction, polyendocrinopathy, enteropathy, X-linked (IPEX) syndrome and severe infections may require intravenous antimicrobial treatment.

Some immunosuppressive drugs may be only administered in an inpatient setting.

Diabetic patients presenting in diabetic ketoacidosis (DKA) may require inpatient monitoring of glucose in addition to fluid and electrolyte resuscitation.

Severe cytopenias may require treatment with intravenous steroids or blood products.

Severe malnutrition from enteropathy may require total parenteral nutrition.

Hospitalization for conditioning prior to hematopoietic stem cell transplantation (HSCT) may be required.

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Transfer

Patients with IPEX syndrome may require transfer to a center that provides multiple subspecialty care.

Care may be obtained at a center that specializes in the diagnosis and treatment of IPEX syndrome.

Transfer to a center that performs hematopoietic stem cell transplantation (HSCT) may be indicated.

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