Test Post
December 18th, 2007 by Web AdminTest
Test
Diagnosis of Immunodeficiency syndrome. ( Bruton’s agammaglobulinemia ) Child.
History of Present illness
Patient : Tuhin sarkarAge €” 4.5 year DOB €” 25th Jan 2003Sex €” MalePlace -
India
Symptomatic €” Since 7th month of life developed fever with loose stools, diagnosed as UTI with SEPSIS ( Ecoli Sepsis & UTI ) another episode of fever with loose stool 15 days back. Same episode repeated given i/v antibiotics. 1 month later again the same episode got repeated, This time he was diagnosed Immunodeficiency syndrome. ( Brutons Ds).
Since then child started receiving regular IVIG infusions ( 5 gms per month ) along with Uroprophylesis ; now about two years back May 06 child started having symptoms of failure to thrive & persistent cough. Child had multiple visits to hospital treated with oral as well as I/V antibiotics. Along with that for moderate degree of GERD. In view of persisting Symptoms further work up was done which revealed growth hormone deficiency in year 2007 as probable cause for failure to thrive. Growth Harmone Supplement was started - Pfizer Genotropin. (1iu 6/7 )
Now child has been admitted since 1/8/07 with increasing chest symptoms, unlike previous episode in once per month lasting for 3 -4 days and subsiding on oral augmentin & nebulization , this admission had rapid downhill course from significant oxygen requirement to ventilation with respiratory paralysis. Currently he is being ventilated at moderate pressures with FiO2 ~ 50%.
Birth History & Development €” SIB Death at 1.5 year well thriving child died of ? Encephalitis .
Important Investigation €”
| 19/01/2004 | 7.5.2005 | 3/3/2007 | 20.2.2007 | ||
|
IgG |
5.525 g/L |
< 2.9 g/ L |
< 237 mg% |
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|
IgA |
< 0.221 g/L |
< 0.2 g/L |
< 37 mg% |
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|
IgM |
< 0.168 g/L |
< 0.23 g/L |
< 20 mg% |
| 20 €” 27 /2/ 2007 | 13.6.07 | ||||
|
DMSA |
Bilaterally good cortical function and no evidence of cortical scarring |
Bilaterally good cortical function and no evidence of cortical scarring |
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DTPA |
Bilaterally good cortical with unobstructed drainage |
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MCU |
Normal Study |
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GER |
Scintigraphic findings are suggestive of presence of mild GER in the study |
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HRCT |
Normal Study |
| Date | 3.3.07 | |||
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Sweat Chloride |
47 mEq/lt |
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Mutational Analysis |
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Stool for Fats ( Fat Globules) |
> 200 / HPF |
|
Deuodenal Biopsy |
|
T3 / T4 / TSH |
1.92 / 134.1 / 1.72 |
|
USG |
Normal Study |
Please suggest course of medication and medicines
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