Analysis Of Clinical Features Of Chronic Granulomatous Disease In 33 Patients

Objective:To study the chronic granulomatous disease (CGD) in children through clinical manifestations and laboratory findingd and understand its clinical features in China.Method:In the years 2003-2014,33 children were diagnosed with CGD at Children’s Hospital of Chongqing Medical University, and their clinical manifestations, laboratory examinations were retrospectively analyzed.Results: 1) This disease in infants had an onset at an average age of 3.4 months, while the diagnosis was made at an average age of 25.04 months. 2) Pneumonia infection emerged as the primary symptom in 15 cases (45.5%), Mycobacterium Tuberculosis infection in 7 cases (23%), Superficial Tissue Purulent infection in 6 cases (18.2%), two cases (6.1%) of Lymphadenitis and one case (3.0%) of Infectious Diarrhea. 3) In 21 cases (73.4%), patients after BCG vaccination developed Kappa Fester, lymphadenitis, or tuberculosis, as well as other disseminated BCG disease. 4) The most common course of recurrent infections in this disaese in children arises in respiratory tract (82%), followed by gastrointestinal tract, skin and lymph nodes infections. 5) The associated pathogens include Candida Albicans, Aspergillus, Staphylococcus Aureus and Klebsiella Pneumoniae etc. 6) Significant reduction in neutrophil respiratory burst; often elevated white blood cell count, mainly neutrophils; immunoglobulin and lymphocyte subsets with no obvious anomalies.Conclusion: 1) most of the patients with CGD, after being vaccinated against BCG resulted in limited or disseminated BCG infection. 2) Generally CGD manifest an early onset in children with infection as the primary symptoms, however unspecific, adverse BCG reactions and a positive family history has a suggestive significance. 3) CGD is characterised as recurrent severe bacterial or fungal infection and the most important manifestation of the affected area involving the lungs, gastrointestinal tract, skin and lymph nodes.