The case reported here was successfully managed through the administration of gammaglobulin and antibiotics. This report describes the management of agammaglobulinemia in a patient with Down syndrome during oral care procedures. Autoimmune diseases are frequently observed in patients with Down syndrome, with prevalence of immune deficiency, mild to moderate T-cell and B-cell lymphopenia with decreased naive lymphocytes, impaired mitogen-induced T-cell proliferation, reduced specific antibody responses to immunizations, and defects in neutrophil chemotaxis [4, 5]. These abnormalities may contribute to increased susceptibility to viral infections, hematologic malignancies, and autoimmune diseases associated with Down syndrome [4, 5]. For invasive dental procedures, such patients are at a high risk of severe infection and septicemia caused by the spread of oral microorganisms and their toxins through circulating blood. Clinical use of IVIG therapy has increased in the treatment of patients with PADS. IVIG therapy in patients with agammaglobulinemia reduces the risk of infection [6, 7]. It involves therapeutic preparations of pooled polyspecific IgG, obtained from the plasma of a large number of healthy individuals. IVIG approach had a significant and positive therapeutic impact in our patient (Fig. 1).
IVIG therapy prevents many, though not all, pulmonary complications. Though they are receiving IVIG therapy, in some patients with relatively more severe antibody deficiencies, may be in high risk of chronic bacterial infections . Then a standard course of antibiotics for acute infections stemming from surgical treatment would not be sufficient in severely immunodeficient patients and may lead to rapid relapse or recurrence of infections and further morbidity, including permanent scarring and loss of function. Experienced clinical immunologists often prescribe a course of antimicrobials that are two to three times longer than standard recommendations [9, 10]. In the case described here, antibiotic prophylaxis besides the induction of IVIG treatment was effective for dental treatment of the immunodeficient patient.
Owing to their susceptibility to infection, immunodeficient patients require precautions during dental treatment. Dental treatment of patients with severe combined immunodeficiency has not been previously reported in dental or medical literature. Based on our experience with this patient, we recommend that IgG, IgA, and IgM should be evaluated in patients with Down syndrome before they undergo dental procedures. Delayed diagnosis of agammaglobulinemia and other PADs might result in frequent hospitalizations owing to bacterial infections, including pneumonia, which could lead to chronic lung diseases.
In the current case, initial oral examination revealed poor oral hygiene. Home care is essential for a patient’s oral hygiene and dental health. However, this is difficult to achieve in patients with Down syndrome owing to the intellectual impairment and decreased manual dexterity . Since patients with Down syndrome frequently experience respiratory infections, regular oral and dental examination should be performed to reduce the risk of aspiration pneumonia; it should be remembered that as they age, people with Down syndrome are more likely to present with immune deficiency syndromes related to early senescence  and innate abnormalities in the immune response. Physicians and dentists should take exceptional precautions to detect oral pathogens in these patients, since the pathogens may result in pneumonia and other severe infection. Early diagnosis and treatment, including IVIG, is essential to improve prognosis and quality of life of patients with PADs . In addition, if possible, newborn mass screening for PADs is recommended [14,15,16,17].