Drtweetie
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If suspect Alport's syndrome, which is the most appropriate confirmatory test?
a. Kidney biopsy
b. audiologic assessment
c. head CT scan
e. Eye exam
f. skin biopsy
g. Upper GI endoscopy
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| cirus
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B
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| kermina
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why did you choose the audiologic assessment , alport is characterized also with visual defect beside the renal
this is a very confusing question i think A
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| Justice
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Hematuria is usually discovered during the first years of life in males with AS. If individuals do not have hematuria during the first decade of life, they are unlikely to have AS.
Hearing loss and ocular abnormalities are never present at birth and usually become apparent by late childhood or early adolescence, generally before the onset of renal failure.
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Bottomline: Ultrastructural findings are diagnostic and consist of profound glomerular basement membrane (GBM) abnormalities
(A)
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| kochs
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A and or F
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| elitoki
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Answer is B
Alport's syndrome has key word that is "hearing loss" with renal and visual abnormalities.
The hearing loss is high-frequency sensorineural loss
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| Justice
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elitoki wrote:
Alport's syndrome has key word that is "hearing loss" with renal and visual abnormalities.
The hearing loss is high-frequency sensorineural loss
Hearing loss comes much later after hematuria and proteinutia onset... It will be late to refer to audiology test in a guy who dies from chrinic kidney failure
(A)
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| elitoki
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kidney biopsy just will tell u that there is renal failure. It will not help u to differenciated from other causes of renal failure.
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| Justice
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elitoki wrote:
kidney biopsy just will tell u that there is renal failure. It will not help u to differenciated from other causes of renal failure.
How many causes of familial renal failure in males do we know? And, if there is no preexisting history of other dieseases in a one-two year boy that would lead to CRF, the kidney biopsy is the best choice...
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| cirus
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If kidney biopsy is +ve it will show collagen inconsistency, but that is a too invasive procedure to be used for screening.
on the other hand, most pts with alport have sensory neural hearing loss, even before kidney affection, so auditory screen is better
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| Justice
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cirus wrote:
on the other hand, most pts with alport have sensory neural hearing loss, even before kidney affection, so auditory screen is better
I disagree with this statement... Hearing loss comes much later than kidney function deterioration...
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| Justice
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cirus wrote:
If kidney biopsy is +ve it will show collagen inconsistency, but that is a too invasive procedure to be used for screening.
on the other hand, most pts with alport have sensory neural hearing loss, even before kidney affection, so auditory screen is better
the best screening test will be deep-stick for blood and protein in urine...
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| Justice
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And finnaly, we have to decide on confirmatory test not on screening test, and pathologic evaluation is superior to any imagings or anything else... We are not given an option of genetic analysis, which is the best here...
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| elitoki
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The only thing that you can see in renal biopsy is thickening and splitting of the glomerular basement membrane detected by electron microscopy.
How you will be sure that this finding is due to Alport's syndrome? When you find some young pt with renal failure you r going to treat him/her in base of their symptoms. Now if you are thinking about Alport's syndrome(X-linked) is mostly like be in theirs 30 yrs.
In this Q said to conform dx., so I will go for B.
And I found on emedicine that they also said audiometry is conform test. http://www.emedicine.com/ped/topic74.htm#section~workup
I remember that in Kaplan they also said about that.
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| elitoki
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Let me know if I'm wrong, but I'm pretty sure about that~ 
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| liliaeliz
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the most APROPIATE CONFIRMATORY test is A the others are not confirmatory
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| kermina
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believe it or not
i found this question today in qbank and the answer was!!!!
skin biopsy
they say pt with alport syndrome have a defect in the genes encoding the chains responsible for formation of type IV collagen.most pts have a defect in the alpha 5 chain. others have a defect in alfa 3 or alpha 4 cahins
patients with strong clinical suspicion of alport should first undergo a skin biopsy. immunohistochemical analysis for skin is done using a monoclonal antibody against alpha 5 chain, if there is no expression of this protein on a skin biopsy so the diagnosis of alport is confirmed
then kidney biopsy is done only if the skin biopsy is equivocal
this is what kaplan q bank says
skin biopsy is the answer
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| liliaeliz
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the most frec is alt renal,then 60% auditory,15 30% ocular
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| liliaeliz
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thanks kermina
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| Ivonne
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I read that link elitoki
presence of 3 of the following 4 proposed diagnostic criteria establishes the diagnosis of Alport syndrome:
1.Family history of hematuria, progressing mostly in males to end-stage renal disease (ESRD)
2.Thickening and splitting of the glomerular basement membrane detected by electron microscopy
3.Progressive, high-frequency, sensorineural deafness
4.Anterior lenticonus and perimacular flecks
Plus(same revision)
That means that If 3 is absent It is still Alport?
Children with Alport syndrome may initially present with only persistent hematuria and a family history of hematuria. Auditory or ocular manifestations may appear later in life.
I will go with A
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