One year old child with profound B and T cell deficiency! - USMLE Forums
USMLE Forums Logo
USMLE Forums         Your Reliable USMLE Online Community     Members     Posts
Home
USMLE Articles
USMLE News
USMLE Polls
USMLE Books
USMLE Apps
Go Back   USMLE Forums > USMLE Step 1 Forum

USMLE Step 1 Forum USMLE Step 1 Discussion Forum: Let's talk about anything related to USMLE Step 1 exam


Reply
 
Thread Tools Search this Thread Display Modes
  #1  
Old 07-05-2011
INCOGNITO's Avatar
USMLE Forums Guru
 
Steps History: Step 1 Only
Posts: 343
Threads: 127
Thanked 522 Times in 192 Posts
Reputation: 532
Immunology One year old child with profound B and T cell deficiency!

A one year old is evaluated for immunodeficiency disorders because she has had multiple episodes of thrush, pneumonia and diarrhea. Her earliest infection was at two months of age. There is profound deficiency of B-cells.
T-cells studies show very low t-cell levels with poor proliferation response to mitogens. Which of following is most likely diagnosis..?

a) ataxia telangiectasia
b) chediak-higashi syndrome
c) adenosine deaminsae deficiency
d) wiskott-aldrich syndrome
Reply With Quote Quick reply to this message
The above post was thanked by:
Abdulaziz (07-05-2011), bebix (07-05-2011), dr_lizard (09-06-2011), dr.muhamad (07-05-2011), drfchakera (07-06-2011), heights (07-05-2011), hiratamkeen (07-05-2011), usluipek (07-05-2011)



  #2  
Old 07-05-2011
dr.Irina's Avatar
USMLE Forums Veteran
 
Steps History: 1 + CK
Posts: 229
Threads: 6
Thanked 68 Times in 61 Posts
Reputation: 78
Default

d)wiskott-aldrich syndrome
Reply With Quote Quick reply to this message
  #3  
Old 07-05-2011
USMLE Forums Master
 
Steps History: Step 1 Only
Posts: 566
Threads: 25
Thanked 522 Times in 263 Posts
Reputation: 532
Default

Quote:
Originally Posted by IKR123 View Post
a one year old is evaluated for immunodeficiency disorders because she has had multiple episodes of thrush,pneumonia and diarrhea.
her earliest infection was at two months of age.
there is profound deficiency of B-cells.
T-cells studies show veery low t-cell levels with poor proliferation response to mitogens.which of following is most likely diagnosis..?

a)ataxia telangiectasia
b)chediak-higashi syndrome
c)adenosine deaminsae deficiency
d)wiskott-aldrich syndrome

Answer is C) ADA def is the most common form of SCID. It is characterized by Decrease of B and T cell lineage. Pte present with Viral, Bacterial, protozoa and Fungal infection.

Others answer doesn't go with decrease with B/T at the same time...
Reply With Quote Quick reply to this message
The above post was thanked by:
heights (07-05-2011), INCOGNITO (07-05-2011), MANIAKOS (07-05-2011), usluipek (07-05-2011)
  #4  
Old 07-05-2011
Ace3's Avatar
USMLE Forums Master
 
Steps History: 1+CK+CS
Posts: 735
Threads: 89
Thanked 285 Times in 191 Posts
Reputation: 297
Default

Quote:
Originally Posted by IKR123 View Post
a one year old is evaluated for immunodeficiency disorders because she has had multiple episodes of thrush,pneumonia and diarrhea.
her earliest infection was at two months of age.
there is profound deficiency of B-cells.
T-cells studies show veery low t-cell levels with poor proliferation response to mitogens.which of following is most likely diagnosis..?

a)ataxia telangiectasia
b)chediak-higashi syndrome
c)adenosine deaminsae deficiency
d)wiskott-aldrich syndrome
Answer is c... It's severe combined immunodeficiency disorder effecting skin,respiratory tract and git.

Option b--> Mainly seen in family, light color hair,eye,skin,mostly neural features like tremor etc..so not answer

Option d-->mainly have eczema and prolong bleeding so its not answer

option a-->ataxia and dilatation of vessel whish is not here

so ill go with c
Reply With Quote Quick reply to this message
The above post was thanked by:
heights (07-05-2011), MANIAKOS (07-05-2011), mzee74 (07-05-2011)
  #5  
Old 07-05-2011
qurat21's Avatar
USMLE Forums Master
 
Steps History: 1 + CK
Posts: 623
Threads: 62
Thanked 287 Times in 186 Posts
Reputation: 297
Default

Quote:
Originally Posted by IKR123 View Post
a one year old is evaluated for immunodeficiency disorders because she has had multiple episodes of thrush,pneumonia and diarrhea.
her earliest infection was at two months of age.
there is profound deficiency of B-cells.
T-cells studies show veery low t-cell levels with poor proliferation response to mitogens.which of following is most likely diagnosis..?

a)ataxia telangiectasia
b)chediak-higashi syndrome
c)adenosine deaminsae deficiency
d)wiskott-aldrich syndrome
the ans is D
Reply With Quote Quick reply to this message
  #6  
Old 07-05-2011
bebix's Avatar
USMLE Forums Master
 
Steps History: 1+CK+CS+3
Posts: 1,357
Threads: 194
Thanked 3,259 Times in 881 Posts
Reputation: 3269
Default

I also think the answer is c)adenosine deaminsae deficiency
Reply With Quote Quick reply to this message
  #7  
Old 07-05-2011
USMLE Forums Addict
 
Steps History: 1+CK+CS
Posts: 124
Threads: 5
Thanked 204 Times in 70 Posts
Reputation: 214
Default Ans.

C.Adenosine Deaminase Deficiency. In SCID both B and T cells are affected
Reply With Quote Quick reply to this message
  #8  
Old 07-05-2011
USMLE Forums Guru
 
Steps History: Not yet
Posts: 339
Threads: 94
Thanked 330 Times in 150 Posts
Reputation: 290
Default

I rhink it is C
Reply With Quote Quick reply to this message
  #9  
Old 07-05-2011
INCOGNITO's Avatar
USMLE Forums Guru
 
Steps History: Step 1 Only
Posts: 343
Threads: 127
Thanked 522 Times in 192 Posts
Reputation: 532
Correct Answer Correct answer

(C) adenosine deaminase deficiency
Reply With Quote Quick reply to this message
  #10  
Old 07-05-2011
heights's Avatar
USMLE Forums Master
 
Steps History: Not yet
Posts: 557
Threads: 69
Thanked 1,038 Times in 351 Posts
Reputation: 1058
Default

Quote:
Originally Posted by IKR123 View Post
a one year old is evaluated for immunodeficiency disorders because she has had multiple episodes of thrush,pneumonia and diarrhea. her earliest infection was at two months of age. there is profound deficiency of B-cells. T-cells studies show very low t-cell levels with poor proliferation response to mitogens.which of following is most likely diagnosis..?

a)ataxia telangiectasia
b)chediak-higashi syndrome
c)adenosine deaminsae deficiency
d)wiskott-aldrich syndrome
Ans c) adenosine deaminase deficiency; this is SCID because both B and T cells are affected.
SCID is most commonly X-linked recessive defect in the IL-2 receptor, but it can occasionally be autosomal recessive adenosine deaminase (ADA) deficiency.
(link to biochem: in ADA deficiency, dATP accumulates, which inhibits ribonuclease reductase -->decreased dNTP synthesis --> DNA can't rapidly replicate--> cells like lymphocytes which need constant DNA replication are affected).

How I remember the others:
a) needs to present with ataxia and telangiectasia (spider angiomas) like the name says- this one is simple
b) Chediak-higashi is BALIN- Bleeding & Bruising, Albinism, Leukopenia, Infection, Neuropathy (peripheral)
d) Wiskott-Aldrich's mother makes him wear a TIE. Mother because it's X-linked recessive, Thrombocytopenia, Infection, Eczema. Note that this also has defect with B and T cell, so don't confuse it with SCID! Look for Wiskott's TIE: if there is no Thrombocytopenia, Infection, Eczema in the question stem, it's probably not Wiscott-Aldrich.
Reply With Quote Quick reply to this message
The above post was thanked by:
Abdulaziz (07-05-2011), Ace3 (07-05-2011), bebix (07-06-2011), Claus_CU (07-06-2011), drtthakur (07-10-2011), INCOGNITO (07-05-2011), Mashee (07-06-2011), pass7 (07-05-2011), prataptetali (07-06-2011), qurat21 (07-05-2011), Sana Zahid (07-06-2011), Usmle16Forall (04-30-2016)
  #11  
Old 07-05-2011
heights's Avatar
USMLE Forums Master
 
Steps History: Not yet
Posts: 557
Threads: 69
Thanked 1,038 Times in 351 Posts
Reputation: 1058
Default

Quote:
Originally Posted by rulz View Post
Answer is C) ADA def is the most common form of SCID. It is characterized by Decrease of B and T cell lineage. Pte present with Viral, Bacterial, protozoa and Fungal infection.

Others answer doesn't go with decrease with B/T at the same time...
Careful - ADA deficiency is the most common form of autosomal recessive SCID, but X-linked recessive defect in IL-2 receptor is way more common than the autosomal recessive forms.
Reply With Quote Quick reply to this message
The above post was thanked by:
donofitaly (07-06-2011), INCOGNITO (07-05-2011), rulz (07-06-2011)
  #12  
Old 07-05-2011
dr.Irina's Avatar
USMLE Forums Veteran
 
Steps History: 1 + CK
Posts: 229
Threads: 6
Thanked 68 Times in 61 Posts
Reputation: 78
Default

thank you heights!Good explanation
Reply With Quote Quick reply to this message
The above post was thanked by:
heights (07-06-2011)
  #13  
Old 07-06-2011
INCOGNITO's Avatar
USMLE Forums Guru
 
Steps History: Step 1 Only
Posts: 343
Threads: 127
Thanked 522 Times in 192 Posts
Reputation: 532
Default

i deeply admire heights way of explanation to help all of us remember that way..
Reply With Quote Quick reply to this message
The above post was thanked by:
heights (07-06-2011)
  #14  
Old 07-06-2011
heights's Avatar
USMLE Forums Master
 
Steps History: Not yet
Posts: 557
Threads: 69
Thanked 1,038 Times in 351 Posts
Reputation: 1058
Default

Quote:
Originally Posted by IKR123 View Post
i deeply admire heights way of explanation to help all of us remember that way..
Thanks IKR123, glad it helps I am not good at memorizing straight facts, so it really helps me to use mnemonics and associations.
Reply With Quote Quick reply to this message
The above post was thanked by:
INCOGNITO (07-06-2011)



Reply

Tags
Immunology-, Pathology-, Step-1-Questions

Quick Reply
Message:
Options

Register Now

In order to be able to post messages on the USMLE Forums forums, you must first register.
Please enter your desired user name, your email address and other required details in the form below.
User Name:
Password
Please enter a password for your user account. Note that passwords are case-sensitive.
Password:
Confirm Password:
Email Address
Please enter a valid email address for yourself.
Email Address:
Medical School
Choose "---" if you don't want to tell. AMG for US & Canadian medical schools. IMG for all other medical schools.
USMLE Steps History
What steps finished! Example: 1+CK+CS+3 = Passed Step 1, Step 2 CK, Step 2 CS, and Step 3.

Choose "---" if you don't want to tell.

Favorite USMLE Books
What USMLE books you really think are useful. Leave blank if you don't want to tell.
Location
Where you live. Leave blank if you don't want to tell.

Log-in

Human Verification

In order to verify that you are a human and not a spam bot, please enter the answer into the following box below based on the instructions contained in the graphic.



Thread Tools Search this Thread
Search this Thread:

Advanced Search
Display Modes


Similar Threads
Thread Thread Starter Forum Replies Last Post
3-year-old child with cystic fibrosis sam01 USMLE Step 1 Forum 4 07-15-2012 04:13 AM
Which immune deficiency in this 9 months old child! kemoo USMLE Step 2 CK Forum 8 06-20-2011 11:22 PM
Lipid Profile in 4 year old child! INCOGNITO USMLE Step 1 Forum 3 05-04-2011 11:26 PM
Renal Biopsy of this 6 year old Child! Prepping123 USMLE Step 1 Forum 6 04-09-2011 10:50 PM
Should we give aspirin to a 16-year old child with RF-related arthritis? Haisook USMLE Step 2 CK Forum 10 03-18-2011 01:21 PM

RSS Feed
Find Us on Facebook
vBulletin Security provided by vBSecurity v2.2.2 (Pro) - vBulletin Mods & Addons Copyright © 2017 DragonByte Technologies Ltd.

USMLE® & other trade marks belong to their respective owners, read full disclaimer
USMLE Forums created under Creative Commons 3.0 License. (2009-2014)