Pediatric Bruton Agammaglobulinemia Questions & Answers

Updated: Mar 18, 2019
  • Author: Terry W Chin, MD, PhD; Chief Editor: Harumi Jyonouchi, MD  more...
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Questions & Answers

Overview

What is X-linked agammaglobulinemia (XLA)?

What is the pathophysiology of X-linked agammaglobulinemia (XLA)?

What is the prevalence of X-linked agammaglobulinemia (XLA) in the US?

What is the global prevalence of X-linked agammaglobulinemia (XLA)?

What is the mortality and morbidity associated with X-linked agammaglobulinemia (XLA)?

What are the racial predilections of X-linked agammaglobulinemia (XLA)?

What are the sexual predilections of X-linked agammaglobulinemia (XLA)?

At what age is X-linked agammaglobulinemia (XLA) typically diagnosed?

What is the prevalence of X-linked agammaglobulinemia (XLA)?

Presentation

Which clinical history findings are characteristic of X-linked agammaglobulinemia (XLA)?

Which physical findings are characteristic of X-linked agammaglobulinemia (XLA)?

What causes X-linked agammaglobulinemia (XLA)?

DDX

How is X-linked agammaglobulinemia (XLA) diagnosed?

What are the differential diagnoses for Pediatric Bruton Agammaglobulinemia?

Workup

What is the role of lab testing in the workup of X-linked agammaglobulinemia (XLA)?

What is the role of imaging studies in the workup of X-linked agammaglobulinemia (XLA)?

What is the role of pulmonary function testing (PFT) in the workup of X-linked agammaglobulinemia (XLA)?

What is the role of bronchoscopy in the workup of X-linked agammaglobulinemia (XLA)?

What is the role of endoscopy and colonoscopy in the workup of X-linked agammaglobulinemia (XLA)?

Which histologic findings are characteristics of X-linked agammaglobulinemia (XLA)?

Treatment

How is X-linked agammaglobulinemia (XLA) treated?

What is the role of surgery in the treatment of X-linked agammaglobulinemia (XLA)?

Which specialist consultations are beneficial to patients with X-linked agammaglobulinemia (XLA)?

Which dietary modifications are used in the treatment of X-linked agammaglobulinemia (XLA)?

Which activity modifications are used in the treatment of X-linked agammaglobulinemia (XLA)?

Medications

What is the role of IVIG replacement therapy in the treatment of X-linked agammaglobulinemia (XLA)?

Which medications in the drug class Corticosteroids, Inhaled are used in the treatment of Pediatric Bruton Agammaglobulinemia?

Which medications in the drug class Bronchodilators are used in the treatment of Pediatric Bruton Agammaglobulinemia?

Which medications in the drug class Antibiotics are used in the treatment of Pediatric Bruton Agammaglobulinemia?

Follow-up

What is included in the long-term monitoring of X-linked agammaglobulinemia (XLA)?

When is inpatient care indicated for the treatment of X-linked agammaglobulinemia (XLA)?

Which medications are used in the treatment of X-linked agammaglobulinemia (XLA)?

When is patient transfer needed for the treatment of X-linked agammaglobulinemia (XLA)?

What is the role of genetic testing in the treatment of X-linked agammaglobulinemia (XLA)?

What is the role of flow cytometry in the workup of X-linked agammaglobulinemia (XLA)?

What is the role of gene therapy in the treatment of X-linked agammaglobulinemia (XLA)?

What is the role of stem cell transplantation in the treatment of X-linked agammaglobulinemia (XLA)?

What are the possible complications of X-linked agammaglobulinemia (XLA)?

What is the prognosis of X-linked agammaglobulinemia (XLA)?

What is included in patient education about X-linked agammaglobulinemia (XLA)?