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Chronic Granulomatous Disease Complicated With Dissemination of BCG Vaccine FREE TO VIEW

Gabriela Manonelles, DM; Vanina Martin, DM
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AAMR, Asociación argentina de Medicina Respiratoria, Río Gallegos, Santa Cruz, Argentina

Chest. 2015;148(4_MeetingAbstracts):169A. doi:10.1378/chest.2278572
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SESSION TITLE: Tuberculosis Global Case Reports

SESSION TYPE: Global Case Report Poster

PRESENTED ON: Tuesday, October 27, 2015 at 01:30 PM - 02:30 PM

INTRODUCTION: Chronic granulomatous disease is caused by a deficiency of phagocyte microbicidal function, due to the inability to produce hydrogen peroxide and superoxide anion. The most common form is X-linked and presented with recurrent pulmonary infections.

CASE PRESENTATION: A male patient a one year and nine months old, presented with febriles syndrome treated with multiple antibiotics such as clabulanic amoxicillin due to reoccurrent infection in the whole respiratory system; anemia, axillary adenopathy, without symptoms of recovery. For this reason he was referred to our hospital for diagnosis and treatment. At admission, his general condition was bad, with the following BM16.5 kg/m2, the patient is between 50-25 percentile growth. Presented: asthenia, decreased appetite, fever syndrome with records of 38 °, oxygen desaturation, with a 92 % to 21 % FiO2, respiratory rate of 36 per minute. Physical examination presented: right axillary lymphadenopathy, mobile, painless, hard elastic 1cm diameter, crepitant widespread rales and rhonchi in both lung fields and Other symptoms such as, increased rate of breathing, breathlessness and wheezing. Treatment was initiated with Clarithromycin and Amoxicillin clavulanic. TB empirical first-line treatment is added. He continued, febrile with records of 38-39 ° C, with antipyretics and physical means. Added access nonproductive cough and increased respiratory compromise adding retractions, and tachypnea. Background: Fully vaccinated. From month of life was treated for recurrent respiratory tract infections. Five months olds requiring hospitalization for acute pneumonia record. Laboratory results: anemia, increased erythrocyte sedimentation rate, hyperglycemia, and mild oxygen desaturation. Serological tests, toxoplasmosis, HIV, EBV, parvovirus, CMV, Mycoplasma, Bartonella, Brucella, Leptosipirosis, all negative. Dosage IgA and IgG slightly above its normal value, IgE 541.6 Ul/ml. Elevation of gamma globulin. DNA- antibody, ANCAs, and Rheumatoid Factor negative. Thorax CT: multiple images of lymph nodes in the mediastinum Observed in lung parenchyma patchy areas of airspace occupation distributed in both lungs and cavitated images in the lower lobes. Axillary lymphadenopathy. Upper abdomen no abnormalities. Normal Echocardiogram. Ocular Fundus, right eye net edges whitish lesions without macular involvement Faced with suspected diagnosis of TB specific studies are requested: Gastric Lavage, Lymph Node and Cerebrospinal Fluid: Negative smear and culture. Blood cultures for mycobacteria: Negatives. Tuberculin Skin Test: Negative.

DISCUSSION: Were raised various differential diagnosis: Infectious diseases such as tuberculosis, toxoplasmosis, aspergillosis, staphylococci sepsis, tuberculosis being the entity that most compatible with the characteristics presented by the patient, although the bacillus was not found by the methods referred. Immunological diseases: Wiskott Aldrich immunodeficiency. Severe combined immunodeficiency disease is a group with deficiency of humoral and cellular immunity caused by various genetic mutations and associated enzyme deficiencies. The most common form is X-linked early onset in the first months of life, and is presented as severe mucocutaneous candidiasis, dissemination of BCG vaccine, persistent diarrhea, lung infections, meningitis, septicemia. Complement deficiency can start at different ages. C3 deficiencies present with sino-pulmonary infections by encapsulated bacteria, pneumococcus, Neisseria meningitidis, Haemophilus influenza. Autoimmune disease Systemic lupus erythematosus, polyangiitis, Wegener's granulomatosis.

CONCLUSIONS: The patient remained febrile despite therapy instituted and showed no improvement in respiratory symptoms so it is referred to a specialist center for studies to detect diseases of the immune system. Resulting poor response in nitroblue tetrazolium (NBT) test, it was assumed as a chronic granulomatous disease complicated with dissemination of BCG (Bacille Calmette-Guerin) vaccine , given that the child was vaccinated at birth. Treatment was started with interferon gamma, TB drugs, Trimethoprim-sulfamethoxazole and Itraconazol, and showed significant improvement 40 days after starting treatment.

Reference #1: Chronic granulomatous disease, Current Opinion in Immunology, Volume 15, October 2003, Pages 578-584, P G Heyworth, and col.

DISCLOSURE: The following authors have nothing to disclose: Gabriela Manonelles, Vanina Martin

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