[HouseWannabe]

" A 6 year old boy is brought to the physician by his parents because of a 3-day history of fever, headache, and cough productive of foul-smelling discharge that also exits from his nose. He has had repeated episodes of similar symptoms during the past 4 years. He appears pale, and lethargic. His height and weight are below 10%. Coarse ronchi are heard bilaterally. An x-ray of the chest shows scattered peripheral capacities, dilated and thickened airways consistent with bronchiectasis and cardiac apex that is directed towards the right. The most likely cause of his recurrent infections is a dysfunction of the following cell type?

a. Alveolar Capillary Endothelial cell
b. Alveolar Macrophages
c. Chondrocyte
d. Ciliated columnar epithelium cell
e. Clara cells
f. Goblet cell
g. Kluchitsky cell
h. Squamous epithelial cell
i. Type I pneumocytes
j. Type II pneumocytes. "

Good luck
taken from NMBE

 

[Seetal]

hmm this is a tough one.

i wanna say D, but why is his heart directed towards the right? whats the answer. the suspense is killing me.

 

[drsue]

its D, kartageners syndrome abnormality in the cilia resulting in right sided heart (dextrocardia), bronchiectasis and sinusitis..........infertility is also common.

 

[Seetal]

its D, kartageners syndrome abnormality in the cilia resulting in right sided heart(dextrocardia), bronchiectasis and sinusitis..........infertility is also common.

oh yes i forgot about the dextrocardia in kartagener's syndrome. :happy: thx!

yes infertility is common because of the dynein protein disorder, the cilia becomes immotile and cannot whoosh the sperm out.

 

[zinah]

Hi, why you think its keratgeners syndrome not cystic fibrosis, the clinical presentation of recurrent lung infection and bronchiectasis also go with cystic fibrosis except the dextrocardia of course !

 

[Seetal]

Hi, why you think its keratgeners syndrome not cystic fibrosis, the clinical presentation of recurrent lung infection and bronchiectasis also go with cystic fibrosis except the dextrocardia of course !

i did think of it initially but none of the choices match. in CF the goblet cell number is increased or there is metaplasia to it. not dysfunction.

 

[zinah]

Well, I agree ..even the clinical presentation in this question is overlapped between cystic fibrosis and keratageners syndrome, we have to think of the keratageners becuase of the Dextrocardia; its the only clue in the question as I see !
I have a question, do females with keratageners syndrome also have infertility if yes why? because I thought infertility in men due to immotile sperms, am I correct or I need to be corrected ?
Thank you in advance

 

[Seetal]

Well, I agree ..even the clinical presentation in this question is overlapped between cystic fibrosis and keratageners syndrom, we have to think of the keratageners becuase of the Dextrocardia; its the only clue in the question as I see !
I have a question, do femals with keratageners syndrome also have infertility if yes why? becuase I thought infertility in men due to immotile sperms, am I correct or I need to be corrected ?
Thank you in advance

yes u are right. immotile sperm because the cilia in sperm (called axonemm) in the tail are immotile. so the sperm cannot make its way into the fallopian tubes.

they always say MALE infertility when it comes to Kartagener's syndrome but yeah this does raise a question. if the syndrome is due to cilia dyskinesia, then the fallopian tubes would have cilia too, and if they cant push the ovum toward the ampulla (where fertilization most often takes place) then doesn't this cause infertility in females too? and if it doesn't and the ovum does get fertilized, doesn't this cause ectopic pregnancy because the cilia can't push the fertilized egg toward the uterus?

good question!

 

[zinah]

Thank you so much, I like it when it makes sence !

 

[HouseWannabe]

Answer: D

Good job guys, the answer is D because like it was mentioned earlier the child has keratageners syndrome which has a clinical triad of:

1. Immotile cilia
2. Bronchiectasis
3. Dextrocardia

 

[tomymajor]

Greaaaaaat discussion

 

[Seetal]

Thank you so much, I like it when it makes sence !

woot!!! ill second that!!:happy:

 

[drsue]

yea, agree with u guys! it was a great discussion............i enjoyed every bit of it. thanks so much guys:happy: