dx the case

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dx the case

Post  asif on Thu Dec 09, 2010 6:00 pm






This gentleman presented to us in neurology ward in jan 2007with complaints of generalized body weakness, dysphagia and double vision, more marked by the end of the day. He also gave history of heat intolerance and palpitations. He was diagnosed as a case of Myasthenia Gravis and graves disease and was treated with carbimazole, inderal, steroids and pyridostigmine 30 mg PO bd. His CT chest showed mediastinal mass and he underwent thymectomy after 3 sessions of plasmaphresis. Four months after he again underwent 3 sessions of plasmaphresis followed by thyroid surgery at mayo hospital.
He was doing fine until April 2009 when he was referred to me in HIV department for consultation. Actually he underwent recurrent episodes of Pneumonia resistant to antibiotics for which he was admitted to gulab devi and underwent bronchoscopy. They diagnosed him to be suffering from Pneumocystis Jiroveci. His HIV testing turned negative on several occasions and he denied any risk behaviour. He was sent to me after for HIV diagnosis. He was not on steroids for past one year. His ELISA testing for HIV1 & @ was negative on three different occasions. 2 different rapid tests with ICT technique were also negative............
what happened to him?
How would you proceed???
What is the final Diagnosis


Last edited by asif on Sun Dec 12, 2010 11:56 am; edited 2 times in total

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Re: dx the case

Post  Dr.Hunain(admin) on Fri Dec 10, 2010 1:04 pm

where...???U havent put up the image

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some other facts..........

Post  asif on Sun Dec 12, 2010 11:59 am

This gentleman makes UPS for domestic purpose and i m his regular customer.....lolz

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Re: dx the case

Post  Dr Hira Tariq on Sun Dec 12, 2010 3:16 pm

plasmaphoresis and surgery(transfusions) themselves are risk factors for HIV infection.
exact dates of plasmaphoresis and surgry are not mentioned. may be he was in window period for ELISA
thymectomy can result in a delayed T-cell regeneration due to the lack of functional thymic epithelium. and this immunosuppressed state itself can cause pneumocystis jiroveci infection.
his HIV antigen should be tested by PCR
HIs CD 4 count should be investigated
thats what.....i think.

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Re: dx the case

Post  asif on Sun Dec 12, 2010 3:34 pm

good attempt hira, but i m afraid that few points need revisions....
1st pcp occurs in patients with AIDS, how come a patient in windiw period of hiv seroconversion will become so much immunocompromised? remember hiv is a chronic infection and it takes a long course before manififesting as full blown aids...
2nd, his thymectomy was done 2 yrs bak and he was fine after that. removing thymus in a patients over 18 yrs of age should not cause a prpoblem as it normaly disappears by this age!
try again....

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Re: dx the case

Post  Dr.Hunain(admin) on Sun Dec 12, 2010 5:47 pm

It all looks like auto-immune polyendocrinopathy with grave's disease and myasthenia gravis. Pneumocystis carinii infection can occur in these individuals. So maybe this chap was one of the rare cases with PCP and auto-immune polyendocrinopathy...well thats what i think...

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Re: dx the case

Post  asif on Sun Dec 12, 2010 9:21 pm

well guys, since we have only 2 persons available to answer so its time to disclose....
he is a case of Good's Syndrome" His immunoglobulin levels were very low!
Good’s syndrome (thymoma with immunodeficiency) is a rare cause of combined B and T cell immunodeficiency in adults. The clinical characteristics of Good’s syndrome are increased susceptibility to bacterial infections with encapsulated organisms and opportunistic viral and fungal infections. The most consistent immunological abnormalities are hypogammaglobulinaemia and reduced or absent B cells. This disorder should be treated by resection of the thymoma and immunoglobulin replacement to maintain adequate trough IgG values. The association between the presence of a thymoma and immunodeficiency was first recognised in 1954 by Dr Robert Good, who described a case of thymoma and hypogammaglobulinaemia in an adult. Although there are no formal diagnostic criteria for this disorder it is classified as a distinct entity by the expert committee of the World Health Organisation/International Union of Immunological Societies on primary immunodeficiencies. Good’s syndrome was noted in 7% of adults with primary antibody deficiency attending a chest clinic. In patients with thymoma the incidence of hypogammaglobulinaemia is 6–11%. The cause and pathogenesis of this disorder are unknown, although there is some evidence that the basic defect may be in the bone marrow (pre-B cell arrest, impaired maturation of erythroid and myeloid precursors in some patients).
so he has to get life long immunoglobulins.........!!!!!!!

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Re: dx the case

Post  Dr Hira Tariq on Mon Dec 13, 2010 11:40 am

CONCLUSION= every solution to dr asif's questions has gotta be a SYNDROME...Smile

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Re: dx the case

Post  asif on Mon Dec 13, 2010 11:46 am

haha...well said
actually main purpose of sharing this case was to make u realize that never stick to a single diagnosis. luk in this case he got myasthenia as well as thyrotoxicosis. both r autoimmune and share many overlapping features. so always jeep yur vision broad...

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Re: dx the case

Post  Dr.Hunain(admin) on Mon Dec 13, 2010 8:50 pm

@hira: well..you always expect something unexpected from Dr.Asif..hehe...that is good in the sense that there is always something new to ponder over....and for some....to get confused... lol!

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Re: dx the case

Post  asif on Mon Dec 13, 2010 11:16 pm

haha...hunain u play naughty...
well i would certainly like to know those "some" who get confuse...!!!!

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Re: dx the case

Post  Dr.Hunain(admin) on Wed Dec 15, 2010 7:31 pm

@dr.asif.....well sir....ye tou aap khudi facial expressions se dekh lya karen kon confuse hojata with the wealth of your knowledge...:-)
Wesy ye baat hai....the syndromes which you usually name...are not easy to forget afterwards once you've mentioned them...lolz

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Re: dx the case

Post  asif on Wed Dec 15, 2010 7:38 pm

really hunain! y is this difficult to forget after my mentioning them?

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Re: dx the case

Post  Dr.Hunain(admin) on Wed Dec 15, 2010 8:53 pm

well...because apart from the usual things...we get to hear from you things which we havent heard before..and i guess that results in not forgetting them...lolz
We can then attribute this to the fact that we heard it from you...:-)

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Re: dx the case

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