Omenn Syndrome Questions & Answers

Updated: Apr 28, 2021
  • Author: Robert A Schwartz, MD, MPH; Chief Editor: Harumi Jyonouchi, MD  more...
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Questions & Answers

Overview

What is Omenn syndrome?

What is the pathophysiology of Omenn syndrome?

What is the prevalence of Omenn syndrome?

What are the racial predilections of Omenn syndrome?

What is the mortality and morbidity associated with Omenn syndrome?

Which patient groups have the highest prevalence of Omenn syndrome?

At what age does Omenn syndrome typically present?

Presentation

Which clinical history findings are characteristic of Omenn syndrome?

Which physical findings are characteristic of Omenn syndrome?

What causes Omenn syndrome?

DDX

Which conditions are included in the differential diagnoses of Omenn syndrome?

What are the differential diagnoses for Omenn Syndrome?

Workup

What is the role of lab tests in the workup of Omenn syndrome?

What is the role of imaging studies in the workup of Omenn syndrome?

What is the role of genetic testing in the workup of Omenn syndrome?

Which findings of serum interleukin 4 and 5 are characteristic of Omenn syndrome?

What is the role of molecular analysis of HLA alleles in the workup of Omenn syndrome?

What is the role of biopsy in the workup of Omenn syndrome?

What is the role of bronchoscopy in the workup of Omenn syndrome?

Which histologic findings are characteristic of Omenn syndrome?

Treatment

How is Omenn syndrome treated?

Which specialist consultations are beneficial to patients with Omenn syndrome?

Which dietary modifications are used in the treatment of Omenn syndrome?

Which activity modifications are used in the treatment of Omenn syndrome?

Medications

What is the role of medications in the treatment of Omenn syndrome?

What is the role of IV replacement immunoglobulin in the treatment of Omenn syndrome?

Which medications in the drug class Interferons are used in the treatment of Omenn Syndrome?

Which medications in the drug class Immunosuppressive Agents are used in the treatment of Omenn Syndrome?

Follow-up

What is the role of isolation in the treatment of Omenn syndrome?

When is inpatient care indicated for Omenn syndrome?

When is patient transfer indicated for the treatment of Omenn syndrome?

How is Omenn syndrome diagnosed prenatally?

What are the possible complications of Omenn syndrome?

What is the prognosis of Omenn syndrome?

What is included in patient education about Omenn syndrome?