dimps
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In kaplan medicine on pg 57...it is given that C11 hydroxylase deficiency lead to hypertension & hyperkalemia ....is it so
on pg 79...there is a Q
a 72 yr old woman comes to u because she has been bothered by a right sided headache for the past 4 weeks .She has never had migraine headaches & denies blurry vision ,nausea or vomiting. The headache does not get worse any specific time of day .She has noticed a feverish feeling & hip stiffness along with the headache .What should be the first test ordered on this patient
what will u do if the test confirms ur hypothesis.?
why does the patient have hip stiffness.?
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| dnaprimase
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THIS 72 YR OLD WOMAN HAS TEMPORAL ARTERITIS AKA GIANT CELL ARTERITIS . 40% OF PXS PRESENT WITH NON CLASSICAL SYMPTOMS I.E U DO`NT HAVE THE JAW CAUDICATION OR VISUAL ABNORMALITIES.
THIS PATIENT HOWEVER HAS HEADACHE, FEVER AND HIP STIFFNESS WHICH POINTS TO POLYMYALGIA RHEUMATICA.A LOT OF PATIENTS WITH TEMPORAL ARTERITIS ALSO HAVE PLOYMYALGIA RHEUMATICA, COS OF MEDIUM SIZED AND LARGE VESSEL INVOLEMENT .
THE FIRST THING TO DO IS
1. CHECK BLOOD FOR ESR AND WBCS. A MARKEDLY ELEVATED ESR IN THE PRESENCE OF NORMAL WBC COUNT SHOULD STRENGTHEN MY HYPOTHEIS
2. TEMPORAL ARTERY BIOPSY SHOULD BE PROMPTLY OBTAINED THIS WOULD CONFIRM MY HYPOTHESIS
3. START PREEDNISONE 60MG DAILY IMMEDIATELY
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| dimps
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thanx dnaprimase but what about my first Q
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| dr.shar
Forum Elite
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order esr, give immediatly prednesone
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| tuesday
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if the tests confirms PMR (or even not confirmed by tests but you have a high index of suspicion) give IV prednisone immed. bec. of the high risk of visual loss then you'll do temporal artery biopsy.
This is a very high yield question!!!
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| Wyoming Sioux
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so, just to be sure:
if given a case which is obviously temporal arteritis, clinically, (but no tests done yet) and the question asks, what is your next step in mgmt?
Treat with prednisone first? Or ESR first? Biopsy would be last, I think.
For sure, you don't wait for results of biopsy before treating, since consequences are severe (blindness) and can be prevented with early treatment.
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| drvic
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hey no one so far has replied to your first query in the post, i.e. - does 11 beta hydroxylase deficiency cause hypertension?
the answer lies in the pathway of adrenal steroid biosynthesis ( and u thought these pathways were only for Step1, wait... don't give out those books just yet! :lol: ) - i know its a bit difficult to memorise but you have to grasp the concept only!
here it goes: 11 beta hydroxylase deficiency (seen in 5-8% cases of CAH) will not allow synthesis of cortisol and corticosterone, leading to the accumulation of 11-Deoxycortisol and 11-deoxycorticosterone (DOC). hypertension is believed to be caused by elevated DOC in these patients. but the important thing to remember is that hypertension does not occur in the first few years of life and appears in later years (dunno why  )
in addition to increased androgens and their urinary metabolites, there is raised ACTH because of loss of normal feedback via Cortisol, which is no longer being synthesised now. so the result is female pseudohermaphroditism and continued postnatal virilization due to elevated androgens.
another disorder of adrenal steroid biosynthesis which causes hypertension is 17-hydroxylase deficiency. if u look carefully at that pathway in you book, you'll easily be able to deduce why :wink: .
regards
drvic
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