The outlook for people varies greatly depending on their level of lung and other organ damage prior to diagnosis and treatment. Epidemiology
CVID has an estimated prevalence of about 1:50,000 in Caucasians. The disease seems to be less prevalent amongst Asians and African-Americans. Males and females are equally affected; however, among children, boys predominate. A recent study of people in Europe with primary immunodeficiencies found that 30% had CVID, as opposed to a different immunodeficiency. 10–25% of people inherited the disease, typically through autosomal-dominant inheritance. Given the rarity of the disease, it is not yet possible to generalize on disease prevalence among ethnic and racial groups. CVID shortens the life-span; but no study currently has a median age recorded. One study suggests the median age of death for men and women is 42 and 44 years old, respectively, but most patients involved in the study are still alive. Those people with accompanying disorders had the worst prognosis (50% survival 33 years after diagnosis) and those people with only CVID-caused frequent infections had the longest survival rates, with another study stating a life expectancy almost equalling that of the general UK population. Additionally, people with CVID with one or more noninfectious complications have an 11 times higher risk of death as compared to people with only infections. History
Immunodeficiencies comprise many diseases and are genetic defects affecting the immune system. There are roughly 150 immunodeficiencies spanning over 120 genetic defects.Charles Janeway Sr. is generally credited with the first description of a case of CVID in 1953.
When a patient presents with symptoms of BLS, the treating physician basically has two recognized options for management:

Test-and-treat
Treat empirically

Test-and-treat method
Although it would seem to be the better way to go in terms of management, there has been recent criticism on the need for such testing because of reliability issues. However, it must be stated that there are options such as the glucose breath test and jejunal aspiration the explanations of which are beyond the scope of this current article. Treating empirically
The "treat empirically" route also has its difficulties, which have all come under wide debate and study. Recommendations are varied but seem to find some common ground around the notion that treatment should be individualized to the specific circumstances under which a patient has developed BLS since these circumstances affect the complex microbial make up of the affected bowel.Tetracyclines have been the mainstay of treatment for BLS, but recent studies have concluded Rifaximin to be very effective in the treatment of BLS. One study by Di Stefano et al., however, concluded Metronidazole to be more effective than Rifaximin in the treatment of BLS. Surgical management
Surgical management is reserved for fixing anatomical causes of bowel obstruction that interfere with normal function once they are amenable to such intervention. These conditions include:
Strictures
Fistulae
Diverticula

References


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