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7234458
SCID
Description
Mind Map on SCID, created by Khaled Sadek on 11/12/2016.
Mind Map by
Khaled Sadek
, updated more than 1 year ago
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Created by
Khaled Sadek
almost 8 years ago
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Resource summary
SCID
• Types of primary immunodeficiencies
Antibody
Cellular, combined
Phagocyte
Complement
Malfunction of regulation
Syndromes with compromised DNA repair
Inheritance of immunodeficiency
X-linked recessive
common in males more than females
What is SCID
Severe Combined Immunodeficiency
highly susceptible to life-threatening infections
a rare condition
Caused by defects in any of several possible genes
Adenosine deaminase deficiency
ADA converts adenosine and deoxyadenosine to inosine and deoxyinosine
Accumulation of dATP
inhibition of ribonucleotide reductase
B and T cells unable to divide
fatal, usually within the first year of life
Cellular and Humoral Components
Cellular
Thymus not present
T cells will not mature
Thymus present but T cells are not produced
Thymus will shrink
Humoral
B cells affected from
The absence of T cells
cannot produce antibodies or cannot class switch
diagnosis of SCID
CBC + DBC
Leukopenia if below 3000 in children younger than 1 year
Total serum immunoglobulin (Ig) levels
IGM
Lymphocyte function
Mitogenic signals
Chest x-ray
thymus shadow
treatment and management of SCID
Protective management
Isolated bubble
IV Ig
Prophylaxis
Stopping live vaccinations
Hematopoietic cell transplant
First choice of treatment
Gene therapy
Not available for everyone
Very expensive
Limited studies done on it reducing its reliability
Enzyme replacement therapy ERT
Weekly IM
Might require continuous Ig therapy
Role of MHC (MAJOR Histocompatibility Complex) , HLA (Human Leukocyte Antigen)
Histocompatibility
Major Complex (MHC)
Cluster of genes >>> Glycoproteins
Discriminate self/ non-self
Ag presenting structures
Act as antigens in in-compatible recipient
Minor Antigen (miHA)
Cell-surface receptors of donated organs
Polymorphic proteins e.g. H-Y/ HA-2
A.A differences between donor & recipient
Classes of MHC Genes
Class I
Class II
Class III
Features of MHC
Highly Polymorphic
Co-dominant Expression
Low affinity, broad specificity
Low affinity, broad specificity
Tests for Compatibility
Blood Typing
Tissue Typing
Genomic typing: Allele specific PCR/Sequencing
Cross-Matching
Complement-dependent Cytotoxicity
Flow Cytometry
Mixed Lymphocyte Culture
Panel Reactive Antibody
Others
ELISA
Limiting Dilution Analysis
Direct Cytotoxicity Assay
Donor guidelines
Medical suitability
Donor eligibility
Donor evaluation
pathogens that may be transmitted by the graft
Informed consent
Preoperative assessment
GVHD
Allograft
Definition
types
acute GVHD
Chronic GVHD
Post-transplant care recommendations
Long term screening
Weekly visits for one month
Biweekly visits in the next wo months
Monthly visit for six two twelve months
Vital signs, body weight and height in every visit
Vaccination
vaccines should be offered from about 12 months post HSCT
No live vaccines are given until at least 2 years post HCT
Same as defaulters schedule
patient’s surrounding family must be completely immunised
Screening for chronic GVHD
allogeneic hematopoietic stem cell transplant should be monitored for chronic GVHD
Consent in organ donation
Informed consent
Required request
Presumed consent
Organ donation in Islam
living donor
it is allowed to donate unless
donor’s life is put at risk
vital organs like the heart are not allowed to be donated
physically cannot tolerate the operation
Dead donor
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