Combined immunodeficiencies

Source: Wikipedia, the free encyclopedia.
Combined immunodeficiencies
Other namesCombined immunity deficiency, CID
antimicrobial prophylaxis, and Hematopoietic stem cell transplantation.[1]
Frequency1:100,000 to 1:5000 live births.[2]

Combined immune deficiencies (CIDs) are a diverse group of inherited

sinus infections to opportunistic infections caused by mycobacteria, fungi, and vaccination reactions resulting in localized to systemic symptoms.[1]

Antibiotics and immunoglobulin replacement therapy are typically administered to patients as needed. Without hematopoietic cell or other transplantation aimed at correcting the underlying pathophysiological defect, prognosis is frequently poor due to T cell dysfunction.[4]

Signs and symptoms

Patients with combined immune deficiencies typically exhibit recurrent

microbes, such as mycobacteria, can be difficult to control in CID patients, as can opportunistic or normally non-pathogenic infections caused by Pneumocystis jirovecii or Candida.[4]

Diagnosis

When a clinical diagnosis of combined immunodeficiency is suspected, preliminary laboratory tests should be ordered. The patient's

immunoglobulins, vaccinal response after 6 months of life, and flow cytometry measurement of larger leukocyte subtypes.[1]

Treatment

Treatment for combined immunodeficiencies with defects in antibody production primarily consists of

antimicrobial prophylaxis is frequently used to prevent infections and their complications.[5] For patients with severe and lethal forms of inborn errors of immunity, hematopoietic stem-cell transplantation is currently the curative treatment of choice.[1]

Epidemiology

Globally, the reported incidence of CIDs ranges from 1:100,000 to 1:5000 live births; however, due to patient mortality prior to diagnosis,

misdiagnoses of patients exhibiting unusual clinical manifestations, and incomplete national registries recording CID incidence, this is thought to be an underestimate of the true incidence. There has been reported to be a diagnostic delay of a few days to several years between the age at which the disease first manifests and the diagnosis of CID.[2]

References

External links