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Prep4USMLE » USMLE Step 1 Forum » Histology & Molecular Biology » Comments on Q3


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krsma

Forum Elite

Topics: 40
Posts: 227
Mar 12, 2004 - 3:44 AM       #1

What is chediak-Higashi syndrome and what happens in it?


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gr_calif

Forum Junior

Topics: 3
Posts: 51
Mar 12, 2004 - 11:34 AM       #2

CHEDIAK HIGASHI SYNDROME is an autosomal recessive immunodeficiency disorder
The CHS gene affects the synthesis and/or maintenance of storage/secretory granules in various types of cells like lysosomes of leukocytes, melanosomes of melanocytes etc
the affected individual has INCREASED SUSCEPTIBILITY TO PYOGENIC INFECTIONS the organism is phagocytosed normally but they do not fuse with the lysosome and intracelular killing is defective.
other features are ;
OCULOCUTANEOUS ALBINISM
NYSTAGMUS
ATAXIA
PHERIPHERAL NEUROPATHY
LYPHOMAS


usmleasr

Forum Guru

Topics: 105
Posts: 970
Mar 12, 2004 - 12:12 PM       #3

thanks


asmi

Forum Hero

Topics: 1049
Posts: 4620
Mar 12, 2004 - 4:46 PM       #4

from what i read :

:arrow: DEFECT IN MICROTUBULE POLYMERIZATION.

:arrow: delayed fusion of phagosome with lysosomes in leukocytes...thus no phagocytosis of bacteria...recurrent infections common.
:arrow: increased fusion of melanosomes in melanocytes....albinism
:arrow: granular defects in NK cells and platelets

so to tell about it in one sentence...there will be chemotactic ,degranulation defects , no NK cell activity ,partial albinism.


mohammem

Forum Newbie

Topics: 2
Posts: 198
Dec 15, 2010 - 10:08 PM       #5

KEEP IN MIND ,,,,ALBINISM ,,IT IS YOUR GAID TO ANSWER SUCH IMMUNE Q


aniraiqbal

Forum Newbie

Topics: 2
Posts: 15
Oct 04, 2012 - 3:36 AM       #6

In chediak higashi syndrome,

mutation in LYST gene -> Lysosomal trafficking defect -> phagosomes not targeted to lysosomes -> phagolysosome not formed - immunity compromised -> decreased immunity, neutropenia, partial albinism, peripheral neuropathy, defect in microtubule polymerization.





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