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q____________? - sarim
#1
A 2 y/o boy is brought to the office due to recurrent skin and soft tissue infections.When he was two months old he had a perianal furuncle that was incised and drained because it was unresponsive to oral antibiotics.At 7 months of age he had a left inguinal klebsiella pneumoniae lymphadenitis.His other past infections include a left calf cellulitis that grew serratia marcescens and a left inguinal abscess that grew staph epidermidis.On examination he has hepatosplenomegaly and enlarged axillary and inguinal lymph nodes.The screening tests for humoral and cell mediated and complement-mediated immunity are normal.The nitroblue tetrazolium test is abnormal.What is the most likely Dx?

A-Wiskott-Aldrich syndrome
B-Chediak-Higashi syndrome
C-Chronic Granulomatous disease
D-Leukocyte adhesion defect
E-Job's Syndrome
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#2
cc
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#3
CGD-- catalase +ve organisms..
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#4
C-
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#5
very well

Nitroblue Tetrazolium Test is abnormal.....what is that mean here ?
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#6
that means its negative.....
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#7
yes sirig ...explain it please
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#8
THAT IS MEAN NO RADICALS SO NO REACTION WITH TETRAZOLIUM....AND THIS IS MIGHT BE
THE ONLY TEST ...ABNORMAL NEGATIVE
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#9
failure of the cells to generate oxygen radicals due to absence of NADPH oxidase
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#10
great.

yes it's Oxygen radicals that convert "Colorless NTB" ------->Deep Blue Color

-and that's why PMN leukocytes will look Blue under microscope.
-if there is no change in color ...means Respiratory burst is absent as we see in "CGD"
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