Pediatric Complement Receptor Deficiency Questions & Answers

Updated: Aug 06, 2019
  • Author: Alan P Knutsen, MD; Chief Editor: Harumi Jyonouchi, MD  more...
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Questions & Answers

Overview

What are complement receptors?

What is pediatric complement receptor 1 (CR1) deficiency?

What is pediatric complement receptor 2 (CR2) deficiency?

What is pediatric complement receptor C1qRP deficiency?

What are pediatric complement receptors C3a, C4a and C5a deficiencies?

What is pediatric complement receptor 3 (CR3) and 4 (CR4) deficiency?

What are leukocyte adhesion deficiency (LAD) syndromes?

What is leukocyte adhesion deficiency (LAD) type 1?

What is leukocyte adhesion deficiency (LAD) type 2?

What is leukocyte adhesion deficiency (LAD) type 3?

What is leukocyte adhesion deficiency (LAD) type 1/variant syndrome?

What is the role RAC2 in the etiology of leukocyte adhesion deficiency (LAD)?

What is the role of E-selectin in the etiology of leukocyte adhesion deficiency (LAD)?

What is the pathophysiology of leukocyte adhesion deficiency (LAD)?

What is the prevalence of leukocyte adhesion deficiency (LAD)syndrome?

At what age does leukocyte adhesion deficiency (LAD) syndrome typically present?

What is the mortality and morbidity associated with leukocyte adhesion deficiency (LAD) type 1?

What is the mortality and morbidity associated with leukocyte adhesion deficiency (LAD) type 2?

What is the prognosis of leukocyte adhesion deficiency (LAD) syndrome?

Presentation

Which clinical history findings are characteristic of leukocyte adhesion deficiency (LAD) syndrome?

Which physical findings are characteristic of leukocyte adhesion deficiency (LAD) syndrome?

What causes leukocyte adhesion deficiency (LAD) syndrome?

DDX

Which conditions are included in the differential diagnoses of leukocyte adhesion deficiency (LAD) syndrome?

What are the differential diagnoses for Pediatric Complement Receptor Deficiency?

Workup

What is the role of lab tests in the workup of leukocyte adhesion deficiency (LAD) syndrome?

How is a diagnosis of leukocyte adhesion deficiency (LAD) type 1 confirmed?

Which lab findings are characteristic of leukocyte adhesion deficiency (LAD) type 2?

Which tests are performed in the workup of leukocyte adhesion deficiency (LAD) type 1?

What is the role of imaging studies in the workup of leukocyte adhesion deficiency (LAD) syndrome?

Which histologic findings are characteristic of leukocyte adhesion deficiency (LAD) syndrome?

Treatment

How is leukocyte adhesion deficiency (LAD) type 2 treated?

How is leukocyte adhesion deficiency (LAD) type 3 treated?

How is leukocyte adhesion deficiency (LAD) type 1 treated?

What is the role of surgery in the treatment of leukocyte adhesion deficiency (LAD) syndrome?

Which specialist consultations are beneficial to patients with leukocyte adhesion deficiency (LAD) syndrome?

Which dietary modifications are used in the treatment of leukocyte adhesion deficiency (LAD) syndrome?

What is included in patient education about leukocyte adhesion deficiency (LAD) syndrome?

Medications

What is the role of medications in the treatment of leukocyte adhesion deficiency (LAD) syndrome?

Follow-up

What is included in the outpatient management of infections in leukocyte adhesion deficiency (LAD) syndrome?

When is surgery indicated in the treatment of leukocyte adhesion deficiency (LAD) syndrome?

When is inpatient care indicated for the treatment of leukocyte adhesion deficiency (LAD) syndrome?