A Case Of Chronic Granulomatous Disease Secondary To Pulmonary Aspergillus Fumigatus Infection And Literature Review

Objective:To analyze the clinical characteristics of children with chronic granulomatous disease?CGD?secondary to pulmonary Aspergillus fumigatus?PAF?infection and to improve the understanding and clinical importance of chronic granulomatous disease and invasive fungal infection.Methods:In PICU child with chronic granulomatous disease secondary to pulmonary Aspergillus fumigatussymptoms,signs,auxiliary examination,diagnosis and treatment were analyzed in detail,and relevant literatures review.Results:Child male 2 month old,chief complaint fever more than 15days,cough 10 days.Auxiliary examination considering severe pneumonia,type II respiratory failure and anemia.After admission in PICU,we given ECG monitoring,CPAP breath support,antibiotics such as meropenem,tecoplanin IV human immunoglobulin.Ambroxol,budesonideand,terbutaline sulfate nebulization was give to reduce cough and airway mucosal inflammation lung infection still progressive.Auxiliary examination:chest CT showed:1,Low transparency and multiple high-density in both lung.2,Heart edge unclear and heart shadow increased.Sputum smear:Leukocytes greater than25/LP,no Mycelium spores observed;Sputum culture indicated filamentous fungus growth,suspected of aspergillus fumigata;G test:242.8pg/ml;GM test:negative;BNP:1110 pg/ml;Immunoglobulin:Ig G12.4g/l,Ig A0.63g/l,Ig M0.92g/l;CD series:CD₃cells 70.39%,CD₄/CD₈ 3.73,CD₁₉ 27.09%,CD₁₆CD₅₆ 2.59%.No abnormalities were found in blood coagulation routine,myocardial enzymes,liver and kidney function.Mycoplasma pneumoniae antibody negative;Negative antibody against respiratory pathogen spectrum;Negative influenza A and B virus antigen;Negative T cell test for tuberculosis infection;The neutrophil respiratory burst test showed a lack of stimulation.Following medical history,The patient's aunt's son had a history of aspergillus fumigata infection and a genetic test showed chronic x-linked granulomatosis or immunodeficiency type 34.Additional diagnosis:fungalinfection,x-linked chronic granulomatosis?Fluconazole and voriconazole was given combined with inhalation of amphotericin B as antifungal treatment.After treatment,body temperature was normal,cough symptoms were reduced,and the child was discharged automatically.After discharged,Oral administration continued:voriconazole 2 m L.Venous blood was taken for genetic test,Gene test result showing CYBB gene,chrx-37651277 nucleotide change,c.302A>c.Test result showed:hemizygous mutation and the revised diagnosis was x-linked granulomatosis.Conclusion:1.Chronic granulomatosis is a rare disease of primary phagocyte function deficiency,which is rare in the clinic.We should raise our awareness.2.Children's immune deficiency diseases are common in clinical practice.When severe infection or repeated infection occurs and possibility of immune deficiency should be considered.3.Severe infection exists and antibiotic treatment is not effective.The possibility of fungal infection should be considered,and the G test,GM test,and fungal culture diagnosis should be performed in time,and appropriate antifungal drugs should be selected according to the type of fungus and drug sensitivity.