Immunodysregulation Polyendocrinopathy Enteropathy X-Linked Syndrome (IPEX) Questions & Answers

Updated: Aug 06, 2019
  • Author: Taylor Banks, MD; Chief Editor: Harumi Jyonouchi, MD  more...
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Questions & Answers

Overview

What is immune dysfunction, polyendocrinopathy, enteropathy, X-linked (IPEX) syndrome?

What is the pathophysiology of immune dysfunction, polyendocrinopathy, enteropathy, X-linked (IPEX) syndrome?

What is the prevalence of immune dysfunction, polyendocrinopathy, enteropathy, X-linked (IPEX) syndrome?

What is the mortality and morbidity associated with immune dysfunction, polyendocrinopathy, enteropathy, X-linked (IPEX) syndrome?

At what age does immune dysfunction, polyendocrinopathy, enteropathy, X-linked (IPEX) syndrome typically present?

What are the sexual predilections of immune dysfunction, polyendocrinopathy, enteropathy, X-linked (IPEX) syndrome?

What are the racial predilections of immune dysfunction, polyendocrinopathy, enteropathy, X-linked (IPEX) syndrome?

What is the prognosis of immune dysfunction, polyendocrinopathy, enteropathy, X-linked (IPEX) syndrome?

What is included in patient education about immune dysfunction, polyendocrinopathy, enteropathy, X-linked (IPEX) syndrome?

Presentation

Which clinical history findings are characteristic of immune dysfunction, polyendocrinopathy, enteropathy, X-linked (IPEX) syndrome?

Which physical findings are characteristic of immune dysfunction, polyendocrinopathy, enteropathy, X-linked (IPEX) syndrome?

What causes immune dysfunction, polyendocrinopathy, enteropathy, X-linked (IPEX) syndrome?

What are the possible complications of immune dysfunction, polyendocrinopathy, enteropathy, X-linked (IPEX) syndrome?

DDX

What are the differential diagnoses for Immunodysregulation Polyendocrinopathy Enteropathy X-Linked Syndrome (IPEX)?

Workup

What is the role of lab tests in the workup of immune dysfunction, polyendocrinopathy, enteropathy, X-linked (IPEX) syndrome?

What is the role of imaging studies in the workup of immune dysfunction, polyendocrinopathy, enteropathy, X-linked (IPEX) syndrome?

What is the role of antibody testing in the workup of immune dysfunction, polyendocrinopathy, enteropathy, X-linked (IPEX) syndrome?

What is the role of endoscopy in the workup of immune dysfunction, polyendocrinopathy, enteropathy, X-linked (IPEX) syndrome?

Which histologic findings are characteristic of immune dysfunction, polyendocrinopathy, enteropathy, X-linked (IPEX) syndrome?

How is immune dysfunction, polyendocrinopathy, enteropathy, X-linked (IPEX) syndrome staged?

Treatment

How is immune dysfunction, polyendocrinopathy, enteropathy, X-linked (IPEX) syndrome treated?

Which specialist consultations are beneficial to patients with immune dysfunction, polyendocrinopathy, enteropathy, X-linked (IPEX) syndrome?

Which dietary modifications are used in the treatment of immune dysfunction, polyendocrinopathy, enteropathy, X-linked (IPEX) syndrome?

Which activity modifications are used in the treatment of immune dysfunction, polyendocrinopathy, enteropathy, X-linked (IPEX) syndrome?

How is immune dysfunction, polyendocrinopathy, enteropathy, X-linked (IPEX) syndrome prevented?

What is the role of hematopoietic stem cell transplantation (HSCT) in the treatment of immune dysfunction, polyendocrinopathy, enteropathy, X-linked (IPEX) syndrome?

Medications

What is the role of medications in the treatment of immune dysfunction, polyendocrinopathy, enteropathy, X-linked (IPEX) syndrome?

Which medications in the drug class Immunosuppressant Agents are used in the treatment of Immunodysregulation Polyendocrinopathy Enteropathy X-Linked Syndrome (IPEX)?

Which medications in the drug class Immunomodulators are used in the treatment of Immunodysregulation Polyendocrinopathy Enteropathy X-Linked Syndrome (IPEX)?

Follow-up

What is included in the long-term monitoring of immune dysfunction, polyendocrinopathy, enteropathy, X-linked (IPEX) syndrome?

When is inpatient care indicated for the treatment of immune dysfunction, polyendocrinopathy, enteropathy, X-linked (IPEX) syndrome?

When is patient transfer indicated for the treatment of immune dysfunction, polyendocrinopathy, enteropathy, X-linked (IPEX) syndrome?