Samenvatting module 3 en 4 minor pediatric infectious diseases MED-MIN23 GNK RU
10 views 0 purchase
Course
Minor pediatric infectious diseases (MEDMIN23)
Institution
Radboud Universiteit Nijmegen (RU)
Samenvatting in het engels (English) van de module 3 immune deficiencies en module 4 vaccines van de minor pediatric infectious diseases. Geneeskunde, radboud universiteit.
Week 1: Primary immune deficiencies
Primary immunodeficiencies(PID) are inborn errors in the immune system. It has
a very low prevalence(<10% of all immunodeficiency cases) → it’s an abnormal
immunsystem. For instance: SCID. It can lead to live threatening symptoms. The
incidence is 1:500-1:500.000. Most frequent are the humoral deficiencies.
Absence or altered function of one or more gene products→ can lead to infection
(most of those are in the respiratory tract), auto-immunity, malignancy or other.
Secondary immunodeficiencies (90% of all cases) are acquired, abnormalities not within the
immune system itself and this has a high prevalence. For instance: HIV, use of prednisone
and chemotherapy.
Cells Primary ID Secondary ID
B-cells XLA, CVID Loss of protein by gut or kidney
T-cells DiGeorge-syndrome HIV
B- & T-cells SCID, CID Drugs (chemotherapy) or
malignancies
Opportunistic infection: a pathogen that doesn’t give a problem in healthy individuals, but in
immunocompromised patients it can cause major infection. For instance: pneumocystis
jiroveci, cytomegalovirus, candida spp, aspergillus spp, mycobacterium spp, nocardia spp.
When can you think about an immunodeficiency→ the key to detect is to
consider the possibility.
1. >8 upper respiratory tract infections a year
2. >2 severe sinusitis a year
3. >2 pneumonia a year
4. >2 months on AB with little help
5. Recurrent skin or organ abscesses
6. Therapy resistant trust or candida infections
7. 2 or more deep-seated infections including septicemia
8. Failure to gain weight or grow normally
9. Need for IV AB to clear infection
10. Family-history positive for immunodeficiency
11. Opportunistic infections
,Physical examination Immunodeficiency:
Skin and appendages: abnormal hair or teeth, eczema, neonatal erythroderma, albinism,
extensive warts or mollusca, congenital alopecia, vitiligo, petechiae, cold abscesses,
telangiectasia, absence of sweating, nail dystrophy.
Oral cavity: gingivostomatitis, periodontitis, aphthae, giant oral ulcers, thrush, dental
crowding, conical incisors.
Eyes: retinal lesions, telangiectasia
Lymphoid tissue: absence of lymph nodes and tonsils, lymphadenopathy, asplenia,
organomegaly.
Neurological: ataxia, microcephaly, macrocephaly
Other: dysmorphism, stunted growth or disproportional growth
Diagnostic approach PID
1. Recurrent ENT and airway infections→ T en B cell
2. Failure to thrive form early infancy→ T and B cell
3. Recurrent pyogenic infections→ neutrophil
4. Unusual infections or unusually severe course of infections→ T and B-cell
5. Recurrent infections with the same pathogen
6. Autoimmune or chronic inflammatory disease and/or lymphoproliferation
7. Characteristic combinations of features in eponymous syndromes→ T and
B-cell
8. Angioedema→ complement
Opsonization defects Pathogens
IgA-deficiency, IgG subclass deficiency or H. influenzae, S. pneumoniae
defect in specific Ab-production
Agammaglobulinemie H. influenzae, S. pneumoniae, M. pneumoniae,
U. urealyticum
Deficiency C2, C3, C4, MBL H. influenzae, S. pneumoniae, meningococcus
Cellular defects Pathogens
Phagocyte dysfunction, hyper-IgE-syndrome S. Aureus, candida spp, A. fumigatus
PID relation with clinical presentation:
- recurrent infections of the respiratory tract and the ear-nose-throat (opsonization
defects)
- unusual or opportunistic infections and general malaise, loss of weight and failure to
thrive (t-cell dysfunction)
- Severe recurrent infections of the skin, oral cavity and mucosa and of the internal
organs (lungs and liver) and the skeleton.(granulocyte dysfunction)--> also ENT and
airway.
, - Recurrent meningococcal infections are suggestive for complement deficiencies.
PID what can you investigate:
- Cell differential in peripheral blood
- Lymphocyte subpopulations (T,B,NK), functional tests
- Immunoglobulins
- Phagocytic functional assays
- Complement activation assays
Leukocyte differential and immunoglobulin isotype levels enable detection in most PID
cases. Use age-matched references to avoid misinterpretation of immunological test results.
Treatment:
- Early diagnosis saves lives and improves quality of life.
- Timely recognition of antibody deficiency prevents future organ damage.
- Prevention→ antibiotic prophylaxis, close surveillance.
- Antibiotic treatment of bacterial infections, HSCT
- When PID is suspected in the family, delay live attenuated vaccinations and do not
postpone immunological investigations.
, Week 1: LE immunodiagnostics
Tests: immunofluorescence(light microscopy, antibodies with fluorescence), flow cytometry.
The benefits of buying summaries with Stuvia:
Guaranteed quality through customer reviews
Stuvia customers have reviewed more than 700,000 summaries. This how you know that you are buying the best documents.
Quick and easy check-out
You can quickly pay through credit card or Stuvia-credit for the summaries. There is no membership needed.
Focus on what matters
Your fellow students write the study notes themselves, which is why the documents are always reliable and up-to-date. This ensures you quickly get to the core!
Frequently asked questions
What do I get when I buy this document?
You get a PDF, available immediately after your purchase. The purchased document is accessible anytime, anywhere and indefinitely through your profile.
Satisfaction guarantee: how does it work?
Our satisfaction guarantee ensures that you always find a study document that suits you well. You fill out a form, and our customer service team takes care of the rest.
Who am I buying these notes from?
Stuvia is a marketplace, so you are not buying this document from us, but from seller juliawentink1. Stuvia facilitates payment to the seller.
Will I be stuck with a subscription?
No, you only buy these notes for $7.56. You're not tied to anything after your purchase.
