Pediatric Wiskott-Aldrich Syndrome Questions & Answers

Updated: Jun 12, 2020
  • Author: Robert A Schwartz, MD, MPH; Chief Editor: Harumi Jyonouchi, MD  more...
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Questions & Answers

Overview

What is Wiskott-Aldrich syndrome?

What are the signs and symptoms of Wiskott-Aldrich syndrome?

How is Wiskott-Aldrich syndrome diagnosed?

What are the treatment options for Wiskott-Aldrich syndrome?

Which medications are used in the treatment of Wiskott-Aldrich syndrome?

Which surgical interventions are used to treat bleeding complications in Wiskott-Aldrich syndrome?

What is included in supportive care for Wiskott-Aldrich syndrome?

How is Wiskott-Aldrich syndrome characterized?

What is the role of genetics in the etiology of Wiskott-Aldrich syndrome?

What is the pathophysiology of Wiskott-Aldrich syndrome?

What is the prevalence of Wiskott-Aldrich syndrome in the US?

What is the global prevalence of Wiskott-Aldrich syndrome?

What are the racial predilections of Wiskott-Aldrich syndrome?

What are the sexual predilections of Wiskott-Aldrich syndrome?

At what age does Wiskott-Aldrich syndrome typically present?

What is the mortality and morbidity associated with Wiskott-Aldrich syndrome?

Presentation

Which clinical history findings are characteristic of Wiskott-Aldrich syndrome?

Which physical findings are characteristic of Wiskott-Aldrich syndrome?

What causes Wiskott-Aldrich syndrome?

DDX

How is Wiskott-Aldrich syndrome differentiated from neonatal alloimmune thrombocytopenia?

How is the Wiskott-Aldrich syndrome phenotype X-linked thrombocytopenia diagnosed?

Which conditions are included in the differential diagnoses of Wiskott-Aldrich syndrome and generalized eczema?

How is ataxia-telangiectasia (AT) differentiated from Wiskott-Aldrich syndrome?

How is X-linked agammaglobulinemia (XLA) differentiated from Wiskott-Aldrich syndrome?

How is X-linked hyperimmunoglobulin M (XHIM) syndrome differentiated from Wiskott-Aldrich syndrome?

How is Wiskott-Aldrich syndrome differentiated from other T-cell disorders?

What are the differential diagnoses for Pediatric Wiskott-Aldrich Syndrome?

Workup

What is the role of lab tests in the workup of Wiskott-Aldrich syndrome?

What is the role of imaging studies in the workup of Wiskott-Aldrich syndrome?

Which test are performed in the long-term monitoring of Wiskott-Aldrich syndrome?

What is included in the workup to determine feasibility for stem cell transplantation in Wiskott-Aldrich syndrome?

How is food hypersensitivity assessed in Wiskott-Aldrich syndrome?

Which procedures are performed in the workup of pediatric Wiskott-Aldrich syndrome?

Which histologic findings are characteristic of Wiskott-Aldrich syndrome?

Treatment

How is Wiskott-Aldrich syndrome treated?

What is the role of gene therapy in the treatment of Wiskott-Aldrich syndrome?

How are infections managed in Wiskott-Aldrich syndrome?

How is acute bleeding managed in Wiskott-Aldrich syndrome?

How is eczema treated in Wiskott-Aldrich syndrome?

How are autoimmune disorders managed in Wiskott-Aldrich syndrome?

What is the role of surgery in the treatment of Wiskott-Aldrich syndrome?

Which specialist consultations are beneficial to patients with Wiskott-Aldrich syndrome?

Which dietary modifications are used in the treatment of Wiskott-Aldrich syndrome?

Which activity modifications are used in the treatment of Wiskott-Aldrich syndrome?

Medications

What is the role of medications in the treatment of Wiskott-Aldrich syndrome?

What is the role of IV immunoglobulin replacement therapy in the treatment of Wiskott-Aldrich syndrome?

Which medications in the drug class Immunizations are used in the treatment of Pediatric Wiskott-Aldrich Syndrome?

Which medications in the drug class Hyperimmune Globulins are used in the treatment of Pediatric Wiskott-Aldrich Syndrome?

Which medications in the drug class Bronchodilators, Inhaled are used in the treatment of Pediatric Wiskott-Aldrich Syndrome?

Which medications in the drug class Antibiotics are used in the treatment of Pediatric Wiskott-Aldrich Syndrome?

Follow-up

When is inpatient care indicated for the treatment of Wiskott-Aldrich syndrome?

When is patient transfer indicated for the treatment of Wiskott-Aldrich syndrome?

How is Wiskott-Aldrich syndrome diagnosed prenatally?

What are the possible complications of Wiskott-Aldrich syndrome?

What is the prognosis of Wiskott-Aldrich syndrome?

What is included in patient education about Wiskott-Aldrich syndrome?