Tuesday, 20 November 2007

Professor Tierney, Dr Aoki and Some Great Cases

Dear Bloggers

Today Professor Tierney visited our institution for just two hours, but it was two hours of brilliance.

The first cases consisted of a patient with pharyngitis, fever and shoulder pain. This was presented by our fourth year resident Dr Fukuda. Professor Tierney lectured on the quality of information about platelets and how you can predict either marrow failure or consumption just by observing the change in absolute numbers. The result of the case was meningococcal septicaemia with infiltration into the CSF without laboratory data indicating meningitis.

Professor Tierney and Dr Aoki were lecturing on how they had seen previous cases of meningitis without cells in the CSF; rare but still possible ! Never say Never.

However, during Dr Fukuda's presentation, he was being called to say his wife had just given birth to their second child--a daughter. Looks like Professor Tierney is a Lucky Charm too.

The second case presented by Dr Ban, was a very interesting case of a young female with a previous diagnosis of Diffuse PanBronchiolisis (DPB) and Immune Thrombocytopenic Purpura (ITP). The patient had been admitted with recurrent respiratory problems and there was a concern that the original diagnosis was in doubt.

The patient also had hypogammaglobulinemia.

Professor Tierney went through this case methodically and came up with differentials such a Histiocytosis X, Immotile Cilia Syndrome, Kartagener's Syndrome and potentially, Cystic Fibrosis.

However, pulling all the elements together, he considered that the initial diagnosis of DPB, made some 10 years before, was wrong and in fact, he considered it to be a congenital condition called Common Variable Hypogammaglobulinemia otherwise known as Common Variable ImmunoDeficiency (CVID).

I had also been consulted on this very same case a week before and my opinion was also CVID and it is good that two minds can think alike.

The disorder is rare and Prof Tierney admitted only ever seeing 2 previous cases in his whole career and this made his third.

UpToDate 15.3 covers CVID in some detail although I am sure any good medical textbook will detail this condition.

The two hours flew by and it was a shame that it had to end. Anyhow, there is always next year ! :)

Lastly, Congratulations to Dr Fukuda and his Wife on the Birth of their Second Child.

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