eMedicine Specialties > Pediatrics: General Medicine > Allergy & Immunology

Complement Receptor Deficiency: Follow-up

Author: Alan P Knutsen, MD, Professor of Pediatrics, Director of Pediatric Allergy and Immunology, Director of Pediatric Clinical Immunology Laboratory, Department of Pathology, St Louis University Health Sciences Center
Contributor Information and Disclosures

Updated: May 20, 2009

Follow-up

Further Inpatient Care

  • Inpatient care is required to treat infections in patients with leukocyte adhesion deficiency (LAD), which is caused by complement receptor deficiency.
  • Patients with infections may need surgical drainage, debridement, and intravenous antibiotics.

Further Outpatient Care

  • Outpatient intravenous antibiotics, usually through a percutaneous line or central line, can be administered to treat infections.
  • Surgical treatment has been performed when necessary, and the organism has been identified.

Deterrence/Prevention

  • Education of the parents and patient for early recognition and need for early treatment of infections is essential.
  • Good oral hygiene is important to manage the oral infections (eg, gingivitis, periodontitis).

Prognosis

  • The prognosis of patients with the LAD type 1 severe phenotype is poor; these patients require immune reconstitution.
  • Patients with mild and moderate phenotypes of LAD type 1 have infections of decreased severity. 
  • CD18 expression of 10% seems to confer protection against invasive life-threatening infections.8

Miscellaneous

Medicolegal Pitfalls

  • The severe phenotype of leukocyte adhesion deficiency (LAD) type 1 is a life-threatening immune deficiency, and hematopoietic stem cell transplantation is potentially curative. Therefore, failure to make the appropriate diagnosis and referral might be a cause of legal liability.
  • LAD type 1 can be diagnosed prenatally. Parents with a previously affected child should be counseled that a prenatal diagnosis is feasible. However, if a prenatal diagnosis of LAD does not alter the decision to either continue or terminate the pregnancy, testing can be performed after birth.
  • LAD type 2 is associated with mental retardation in addition to susceptibility to recurrent infections. Appropriate treatment with fucose supplementations corrects both the immune defect and mental development.
 


More on Complement Receptor Deficiency

Overview: Complement Receptor Deficiency
Differential Diagnoses & Workup: Complement Receptor Deficiency
Treatment & Medication: Complement Receptor Deficiency
Follow-up: Complement Receptor Deficiency
Multimedia: Complement Receptor Deficiency
References
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References

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Further Reading

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Keywords

complement receptor deficiency, leukocyte adhesion deficiency, LAD, LAD I (CD18) deficiency, LAD II deficiency, LAD III deficiency, CR1 (CD35) deficiency, systemic lupus erythematosus, hemodialysis, preeclampsia, skin infections, periodontitis, gingivitis, mental retardation, distinctive facies, short stature, bone marrow transplantation, urinary tract infection, ear infection, otitis media, ulcerative stomatitis, necrotic cutaneous abscesses, cellulitis, omphalitis, pneumonia, aseptic meningitis, chronic granulomatous disease, treatment, diagnosis

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Contributor Information and Disclosures

Author

Alan P Knutsen, MD, Professor of Pediatrics, Director of Pediatric Allergy and Immunology, Director of Pediatric Clinical Immunology Laboratory, Department of Pathology, St Louis University Health Sciences Center
Alan P Knutsen, MD is a member of the following medical societies: American Academy of Allergy Asthma and Immunology and Clinical Immunology Society
Disclosure: Nothing to disclose.

Medical Editor

Ann O'Neill Shigeoka, MD †, Former Clinical Associate Professor, Department of Pediatrics, Division of Immunology-Rheumatology, University of Utah School of Medicine
Ann O'Neill Shigeoka, MD † is a member of the following medical societies: American Federation for Medical Research, Clinical Immunology Society, Pediatric Infectious Diseases Society, and Society for Pediatric Research
Disclosure: Nothing to disclose.

Pharmacy Editor

Mary L Windle, PharmD, Adjunct Assistant Professor, University of Nebraska Medical Center College of Pharmacy, Pharmacy Editor, eMedicine
Disclosure: Pfizer Inc Stock Investment from financial planner; Avanir Pharma Stock Investment from financial planner ; WebMD Salary and stock Employment and investment from financial planner

Managing Editor

John Wilson Georgitis, MD, Consulting Staff, Lafayette Allergy Services
John Wilson Georgitis, MD is a member of the following medical societies: American Academy of Allergy Asthma and Immunology, American Academy of Pediatrics, American Association for the Advancement of Science, American College of Chest Physicians, American Lung Association, American Medical Writers Association, and American Thoracic Society
Disclosure: Nothing to disclose.

CME Editor

David Pallares, MD, Clinical Assistant Professor, Department of Pediatrics, Division of Allergy and Immunology, University of Louisville
David Pallares, MD is a member of the following medical societies: American Academy of Allergy Asthma and Immunology
Disclosure: Nothing to disclose.

Chief Editor

Harumi Jyonouchi, MD, Associate Professor, Division of Pulmonary Allergy/Immunology and Infectious Diseases, Department of Pediatrics, UMDNJ-New Jersey Medical School
Harumi Jyonouchi, MD is a member of the following medical societies: American Academy of Allergy Asthma and Immunology, American Academy of Pediatrics, American Association of Immunologists, American Medical Association, Clinical Immunology Society, New York Academy of Sciences, Society for Experimental Biology and Medicine, Society for Mucosal Immunology, and Society for Pediatric Research
Disclosure: Nothing to disclose.

 
 
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