“…Patients require immunoglobulin replacement therapy, which can be administered intravenously (IVIG) or subcutaneously (SCIG) [7][8][9][10][11][12][13][14]. Subcutaneous administration produces stable serum IgG levels and is associated with fewer systemic adverse events (AEs).…”
“…Patients require immunoglobulin replacement therapy, which can be administered intravenously (IVIG) or subcutaneously (SCIG) [7][8][9][10][11][12][13][14]. Subcutaneous administration produces stable serum IgG levels and is associated with fewer systemic adverse events (AEs).…”
“…CVID patients have a tendency to form granulomas, which conforms it to sarcoidosis [39]. Granulomas can occur in any organ, the most frequently in the respiratory tract, liver, and spleen, rarely in the kidney, brain, conjunctiva, and skin [35].…”
Section: Clinical Picturementioning
confidence: 89%
“…Such a sequence of events in a number of cohort studies is estimated at 17-76% [24][25][26][27][28][29][30][31][32][33]. Early diagnosis usually protects the patient against this complication, however, development of bronchiectasis occurs even in 30% of patients, despite optimal treatment [34,35].…”
Section: Clinical Picturementioning
confidence: 99%
“…Infections of the nervous system: meningitis, encephalities and brain abscesses may also be rare but life-threatening manifestation of CVID. They are usually caused by S. pneumoniae, H. influenzae, S. aureus [25,38], but also by viral infections, especially enteroviruses [35]. CVID patients have a tendency to form granulomas, which conforms it to sarcoidosis [39].…”
Section: Clinical Picturementioning
confidence: 99%
“…and Shigella spp. play a statistically significant role in the etiology [33,35,38,40]. Giardiasis may manifest as diarrhea (watery or steatorrhea), spasmodic abdominal pain, bloating, and impaired nutritional status.…”
Key Clinical Message
Recurrent severe respiratory infections in Jacobsen syndrome (JS) are unusual and should prompt evaluation of the immune system. A variety of immune defects have been reported in JS and intravenous immune globulin (IVIG) treatment reduces severe infections.
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