dinesh keran.s pre final year - mbbs student, department ...s_syndrome_due_to_cvid-_a_… · dinesh...
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,
Dinesh Keran.SPre final Year- MBBS Student,
Department of Pediatrics,Govt., Royapettah Hospital,
Chennai.
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• 9 year old boy
• Born to a 2 degree consanguineous parents
— Since 1 year of age,
• Recurrent episodes of cough, fever, ear discharge
• Frequent passage of normal stools
• Frequent skin abscesses
• Asthmatic father treated for TB 10 years ago
• febrile fits till 5 years
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On examination — Pale and non icteric
— Sub- mandibular lymph nodes palpable
— Pot belly
— Phimosis +
— Pan digital clubbing and marked wrinkles
— Ear discharge
— Kyphoscoliosis
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Systemic Examination
— Respiratory system:
— Dull note heard over the right mammary and infra mammary region on percussion
— Bilateral crepitations over infra mammary, scapular and sub scapular in both the lungs
— Gastrointestinal system:
— Hepatosplenomegaly
— Other systems were normal
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Differential Diagnosis
— Pulmonary TB
— Cystic Fibrosis
— Chronic granulomatous disease
— Primary humoral immunodeficiency disorder
— Immotile cilia syndrome
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Investigations
— Hematological
— Anemia and neutrophil leukocytosis
— Neutrophils containing huge granules +
— Infectious etiologies:
— Urine, stool, Widal, HIV, BAL: Negative, Mx negative,
— Pus Culture: MRSA
— ECG: Normal
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IMAGING— CXR
— Consolidation of the lower lobe of the right lung.
— Atelectasis of the right middle lobe of the right lung
( Brock’s Syndrome)
— Thymus prominent
— X ray(PNS)- Pan sinusitis
— USG abdomen- Hepatosplenomegaly.
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— Sweat chloride test – Negative
— Immunoglobulin assay :
All immunoglobulin levels are low(IgG, IgE, IgA, IgM)
— Lymphocyte subset panel 7-
— No defect in absolute B cell, T cell and N K cells.
— Relative increase in CD8 cells in relation to CD4 cells
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— Nitrozolium blue test – Negative
Not Chronic Granulomatous disease
— Bone marrow aspiration:
— Hypercellular marrow showing striking granulomatoushyperplasia with increased reticulo-endothelial cells
PROVISIONAL DIAGNOSIS
— Common Variable Immunodeficiency Syndrome
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Literature Review-CVID— Prevalence- 1: 50,000 worldwide
— No sex predominance
— commonly presents between 20 to 40 years
— predisposition to autoimmune disorders
— Predisposition to malignancies
— Diagnosis by exclusion.P, Roelandt R. "Common Variable Immunodeficiency (CVID): Case Report and Review of the Literature.“
Acta Clinica Belgica 64.4 (2009): 355-60.
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Acknowledgement— Department of Pediatrics- Govt. Royapettah Hospital
— Dr. Usha MD, DM- MMC
— Department of Biochemistry (KMC)
— Dr Venugopal - (Sooriya hospital)
— Dr. Srilekha(Rheumatologist)- Sundharam Medical Foundation.
— Dr. Vijayasekar ( Pediatric Pulmonologist)- Child trust hospital for doing Fibro optic Bronchoscopy and BAL
— Dr. Shareth( Pulmonologist)- ICH
— Dr. Tripathi( Pulmonologist)- MMC
— Dr. Rengan( GI Surgeon)- Apollo first med Hospital
— Dr. Gopinath( Radiologist)- GRH
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