Nephrology MCQs-4


Mature GBM consists of


A. Type I collagen

B. Type II collagen

C. Type III collagen

D. Type IV collagen



Major symptom of ‘Thin Basement Membrane Disease’


A. Hematuria

B. Hypercalciuria

C. Proteinuria

D. Hyperuricosuria



Type IV collagen is the antigenic target in


A. Cystinosis

B. Alport syndrome

C. Goodpasture disease

D. Fabry disease



ALL are TRUE about Alport syndrome EXCEPT


A. Loss of hearing and eye abnormalities

B. Abnormality of a gene that codes for type IV collagen

C. 80% of cases Alport syndrome is inherited as autosomal recessive pattern

D. Gross or microscopic hematuria is the commonest and earliest sign of Alport syndrome



Commonest type of hearing loss in Alport syndrome?


A. Conductive Hearing Loss

B. Sensorineural Hearing Loss

C. Mixed Hearing Loss

D. Auditory Neuropathy Spectrum Disorder



Occular finding characteristic feature of Alport syndrome


A. Posterior polymorphous corneal dystrophy

B. Severe temporal macular thinning

C. Anterior lenticonus

D. Giant macular holes



Which is the commonest ocular manifestation of Alport syndrome in males with XLAS.


A. Purtscher’s retinopathy

B. Dot-and-fleck retinopathy

C. Valsalva retinopathy

D. Macular Photocoagulation



In-shorts


Alport syndrome


Sensorineural hearing loss


Sensorineural hearing loss is a characteristic feature observed commonly, but not universally, in Alport syndrome.

Hearing loss is never congenital.

Bilateral, high-frequency sensorineural deafness usually begins in late childhood or early adolescence, before the onset of kidney failure.


Hematuria


Gross or microscopic hematuria is the commonest and earliest sign of Alport syndrome.


Proteinuria


Proteinuria is generally absent in childhood but ultimately develops in males with XLAS and male and female patients with ARAS


Sensorineural hearing loss is a characteristic feature observed commonly, but not universally, in Alport syndrome. Hearing loss is never congenital.


Anterior lenticonus


Anterior lenticonus, occurring in around 25% of patients with XLAS, is a characteristic feature of Alport syndrome.


Dot-and-fleck retinopathy


Dot-and-fleck retinopathy is the commonest ocular manifestation of Alport syndrome, occurring in around 85% of males with XLAS. 


Diffuse leiomyomatosis


Diffuse leiomyomatosis of the tracheobronchial tree and esophagus has been observed in some families with Alport syndrome


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