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HEREDITORY NEPHRITIS - A CASE SERIES

SANDEEP UNNIKRISHNAN

Abstract


Alport syndrome is a primary basement
membrane disorder whose most common
and earliest manifestation is gross
or microscopic hematuria. Hematuria is
usually persistent in males and may be
intermittent in females. Bilateral sensorineural
hearing loss is a characteristic
feature observed frequently, but not universally.
Anterior lenticonus is the ocular
manifestation seen associated with 15 -
20 percent of patients.2 pairs of siblings
with Alport syndrome that presented to
our OPD at the same time period are
hereby presented. Two of them presented
with features of renal failure while
two others presented with altered sensorium.
All the four cases had sensory
neural hearing loss and anterior lenticonus.
We present this case series as
they all presented as sporadic cases
without any significant family history.
Keyword :Alport syndrome, hereditary
nephritis


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