smal
Forum Guru
Topics: 140
Posts: 727
|
a 12 yr old girl is brought to pediatritian by he r mother because of fever.the physician ntes that the girl has features of albinism and mother states that her daughter has always lookd the way she does.the physician diagnoses the gal with staph infection and prescribes medicines.3 months latter the child returns with the same infection.the medical rec. indicate she has had repeated bouts of staph and strep infection for her entire life.pt has which immuno def?
1. chronic granulomatous dis
2.chediak-Higashi disease
___________________
remedy for weakness is not brooding over it ,but thinking of strength.
|
| fox
Forum Guru
Topics: 70
Posts: 727
|
2
___________________
Aim High
|
| smal
Forum Guru
Topics: 140
Posts: 727
|
ya thats what i thought,
but i saw this qt from some USMLERX qts a friend gave me,and ans was 1!
however ans didnt explain the unnecessary need to mention albinism is the CGD pt.(that was superconfusing)
___________________
remedy for weakness is not brooding over it ,but thinking of strength.
|
| blueocean
Forum Senior
Topics: 15
Posts: 195
|
It is a strange answer because patients with CGD are susceptible to recurrent infections due to catalase-positive organisms and organisms resistant to nonoxidative killing. Catalase-negative bacteria, such as streptococci and pneumococci that have the capacity to generate hydrogen peroxide, are killed as they usually are.On the other hand in CHD complete syndrome includes oculocutaneous albinism with photophobia, neurologic features, recurrent bleeding, recurrent infections, and enterocolitis. So I am complete confused!
|
| yasmeen
Forum Guru
Topics: 68
Posts: 940
|
IT SHOULD BE CHS BESIDES STREP IS NOT CATALASE POSITIVE,AND ALBINISM?
|
| doc179
Forum Guru
Topics: 67
Posts: 1,217
|
it is CHS, it can not be CGD as she has had infections with both staph and strep. and many patients with CHS were found to have albinism
http://www.emedicine.com/derm/topic704.htm
Chédiak-Higashi syndrome (CHS) was described by Beguez Cesar in 1943, Steinbrinck in 1948, Chédiak in 1952, and Higashi in 1954. CHS is a rare childhood autosomal recessive disorder that affects multiple systems of the body. Patients with CHS exhibit hypopigmentation of the skin, eyes, and hair; prolonged bleeding times; easy bruisability; recurrent infections; abnormal natural killer cell function; and peripheral neuropathy. Morbidity results from patients succumbing to frequent bacterial infections or to an accelerated-phase lymphoproliferation into the major organs of the body. Most patients who do not undergo bone marrow transplantation die of a lymphoproliferative syndrome, although some patients with CHS have a relatively milder clinical course of the disease.
|
| jamdoc
Forum Elite
Topics: 30
Posts: 244
|
its given in kaplan immunology notes as well.CHS is assosiated with albinism
___________________
Dont worry.Nobody knows 100%
|
|
| |
| | | | | | | |
