immunodeficiency_diseases.pptx

IMMUNODEFICIENCY
DISEASES
Dr. Hayat AL AKOUM
IMMUNODEFICIENCY DISEASES
 Primary immunodeficiencies, rare disorders with
genetic origins are seen primarily in infants and
young children. To date, more than 95
immunodeficiencies of genetic origin have been
identified(Buckley, 2000).
 Symptoms usually develop early in life after
protection from maternal antibodies decreases.
Without treatment, infants and children with these
disorders seldom survive to adulthood.
 These disorders may involve one or more
components of the immune system. Symptoms of
immune deficiency diseases are related to the role
that the deficient component normally plays
IMMUNODEFICIENCY DISEASES
 Immunodeficiency disorders may be caused by a
defect or deficiency in phagocytic cells, B lymphocytes,
T lymphocytes, or the complement system. The specific
symptoms and their severity, age of onset, and
prognosis depend on the immune system components
affected and their degree of functional impairment.
 Regardless of the underlying cause, the cardinal
symptoms of immunodeficiency include chronic or
recurrent severe infections, infections caused by
unusual organisms or organisms that are normal body
flora, poor response to treatment of infections, and
chronic diarrhea.
 In addition, the patient is susceptible to a variety of
secondary disorders, including cancer.
IMMUNODEFICIENCY DISEASES
Immunodeficiencies may be classified as either
primary or secondary and by the components of the
immune system that are affected.
Primary immunodeficiency: abnormalities in
development of immune mechanisms
Secondary immunodeficiency: Consequences of
disease, drugs, nutritional inadequacies & other
processes interfere with proper functioning of mature
immune system
PRIMARY IMMUNODEFICIENCY DISORDERS
Immune component Disorder Major symptoms Treatment
Phagocytic cells Hyperimmunoglo
bulinemia E
(HIE) syndrome
Bacterial, fungal,
and viral infections;
deep-seated cold
abscesses
Antibiotic therapy and treatment
for viral and fungal infections
Granulocyte-macrophage
colonystimulating
factor (GM-CSF);
granulocyte colony-stimulating
factor (G-CSF)
B lymphocytes Sex-linked
agammaglobuline
mia
(Bruton’s
disease)
Common variable
immunodeficienc
y
(CVID)
Severe infections
soon after birth
Bacterial infections,
infection with
Giardia lamblia
Pernicious anemia
Chronic respiratory
infections
Passive pooled plasma or
gammaglobulin
IV immunoglobulin
Metronidazole (Flagyl)
Quinacrine HCl (Atabrine)
Vitamin B12
Antimicrobial therapy
PRIMARY IMMUNODEFICIENCY DISORDERS
Immune component Disorder Major symptoms Treatment
B lymphocytes Immunoglobulin A
(IgA)
Deficiency
IgC2 deficiency
Predisposition to recurrent
infections,
adverse reactions to blood
transfusions or
immunoglobulin,
autoimmune
diseases,hypothyroidism
Heightened incidence of
infectious
diseases
None
Pooled immunoglobulin
T lymphocytes Thymic hypoplasia
(DiGeorge
syndrome)
Recurrent infections;
hypoparathyroidism;
hypocalcemia, tetany,
convulsions;
congenital heart disease;
possible renal
abnormalities;
abnormal facies
Thymus graft
PRIMARY IMMUNODEFICIENCY DISORDERS
Immune component Disorder Major symptoms Treatment
T lymphocytes Chronic
mucocutaneous
candidiasis
Candida albicans
infections of
mucous membrane, skin,
and
nails; endocrine
abnormalities
(hypoparathyroidism,
Addison’s
disease)
Antifungal agents:
Topical: miconazole
Oral: clotrimazole,
ketoconazole
IV: amphotericin B
B and T
lymphocytes
Ataxia-
telangiectasia
Ataxia with progressive
neurologic
deterioration;
telangiectasia
(vascular lesions);
recurrent
infections; malignancies
Antimicrobial therapy;
management
of presenting
symptoms;
fetal thymus
transplant,
IV immunoglobulin
PRIMARY IMMUNODEFICIENCY DISORDERS
Immune component Disorder Major symptoms Treatment
B and T
lymphocytes
Nezelof’s
syndrome
Severe infections;
malignancies
Antimicrobial therapy;
IV immunoglobulin,
bone
marrow
transplantation;
thymus
transplantation;
thymus factors
Wiskott-Aldrich
syndrome
Thrombocytopenia,
resulting
in bleeding; infections;
malignancies
Antimicrobial therapy;
splenectomy
with continuous
antibiotic
prophylaxis;
IV immunoglobulin
and
bone marrow
transplantation
PRIMARY IMMUNODEFICIENCY DISORDERS
Immune component Disorder Major symptoms Treatment
B and T
lymphocytes
Severe combined
immunodeficiency
disease (SCID)
Overwhelming severe
fatal infections
soon after birth (also
includes opportunistic
infections)
Antimicrobial
therapy;
IV immunoglobulin
and bone
marrow
transplantation
Complement
system
Angioneurotic
edema
Paroxysmal
nocturnal
hemoglobinuria
(PNH)
Episodes of edema in
various parts
of the body, including
respiratory
tract and bowels
Lysis of erythrocytes due
to lack of
decay-accelerating factor
(DAF)
on erythrocytes
Pooled plasma,
androgen therapy
None
THE 10 WARNING SIGNS OF PRIMARY
IMMUNE DEFICIENCY
1. Eight or more new ear infections within 1 year.
2. Two or more serious sinus infections within 1 year.
3. Two or more months on antibiotics with little effect.
4. Two or more pneumonias within 1 year.
5. Failure of an infant to gain weight or grow
normally.
6. Recurrent, deep skin or organ abscesses.
7. Persistent thrush in mouth or elsewhere on skin,
after age 1.
THE 10 WARNING SIGNS OF PRIMARY
IMMUNE DEFICIENCY
8. Need for intravenous antibiotics to clear infections.
9. Two or more deep-seated infections such as
meningitis, osteomyelitis, cellulitis, or sepsis.
10. A family history of primary immune deficiency.
ALERT
 Though the primary immune deficiency diseases
can be serious, they are rarely fatal and can
generally be controlled. Primary immune deficiency
should not be confused with AIDS.
 Primary immune deficiency can be diagnosed
through blood tests and should be detected as soon
as possible to prevent avoidable permanent
damage. As with all disease, only direct
examination by a physician should be used to
determine the presence of primary immune
deficiency.
MANAGING AN IV IMMUNOGLOBULIN
INFUSION
 Weigh the patient before treatment.
 Obtain vital signs before, during, and after
treatment.
 Administer the prescribed pretreatment prophylactic
aspirin or IV antihistamine, such as
diphenhydramine (Benadryl).
 Be aware that corticosteroids may be used to
prevent possible severe reactions.
 Administer the IV infusion at a slow rate, not to
exceed 3 mL/minute.
 Assess the patient for adverse reactions, including
the early signs of anaphylactic shock; prepare to
slow the infusion rate if necessary.
MANAGING AN IV IMMUNOGLOBULIN
INFUSION
 Be aware that patients with low gammaglobulin levels
have more severe reactions than those with normal
levels (eg, patients who receive gammaglobulin for
thrombocytopenia or Kawasaki disease).
 Keep in mind that patients who have an immunoglobulin
A (IgA) deficiency have IgE antibodies to IgA, which
requires administration of plasma or immunoglobulin
replacement from IgA-deficient patients. Because all IV
immunoglobulin preparations contain some IgA, they
may cause an anaphylactic reaction in patients with IgE
anti-IgA antibodies.
 Remember that the risk for transmission of hepatitis,
HIV, or other known viruses is extremely low.
ADVERSE EFFECTS
 Complaints of flank pain, shaking chills, and
tightness in the chest, terminating with a slight rise
in body temperature
 Hypotension (possible with severe reactions)
 Anaphylactic reactions
SECONDARY IMMUNODEFICIENCY
DISORDERS
Category Examples
Endocrine Diabetes mellitus
GI Hepatic insufficiency, hepatitis, intestinal
lymphangiectasia, protein-losing
enteropathy
Hematologic Aplatic anemia, cancer, graft-vs-host
disease, sickle cell disease
Iatrogenic Certain drugs: chemotherapeutic drugs,
immunosuppressants, corticosteroids,
radiation therapy; splenectomy
Infectious Cytomegalovirus, Epstein-Barr virus, HIV,
measles virus, varicella-zoster virus
SECONDARY IMMUNODEFICIENCY
DISORDERS
Category Examples
Nutritional Alcoholism, undernutrition
Physiologic Physiologic immunodeficiency in infants
due to immaturity of immune system,
pregnancy
Renal Nephrotic syndrome, renal insufficiency,
uremia
Rheumatologic RA, SLE
Other Burns, chromosomal abnormalities (eg,
Down syndrome), congenital asplenia,
critical and chronic illness, histiocytosis,
sarcoidosis
INFECTION PREVENTION FOR THE PATIENT
WITH IMMUNODEFICIENCY
 Identify signs and symptoms of infection to report to
the health care provider, such as fever, chills; wet or
dry cough; breathing problems; white patches in the
mouth; swollen glands; nausea; vomiting; persistent
abdominal pain; persistent diarrhea; problems with
urination; red, swollen, or draining wounds; sores or
lesions on the body; and persistent vaginal
discharge with or without itching.
 Demonstrate correct handwashing procedure.
INFECTION PREVENTION FOR THE PATIENT
WITH IMMUNODEFICIENCY
 State rationale for thorough hand washing before
eating, after using the bathroom, and before and
after performing health care procedures.
 State rationale for use of cream and emollients to
prevent or manage dry, chaffed, or cracked skin.
 Demonstrate recommended personal hygiene in
bathing and foot care to prevent bacterial and
fungal diseases.
 State rationale for avoiding contact with people who
have known illness or who have recently been
vaccinated.
INFECTION PREVENTION FOR THE PATIENT
WITH IMMUNODEFICIENCY
 Verbalize understanding of ways to maintain a well-
balanced diet and adequate calories.
 State the reason for avoiding the eating of raw fruits and
vegetables, cooking all foods thoroughly, and
immediately refrigerating all leftover food.
 Identify the rationale for frequent cleaning of kitchen and
bathroom surfaces with disinfectant.
 Identify rationale and benefits of avoiding alcohol,
tobacco, and unprescribed medications.
 State rationale for taking prescribed medications as
directed.
 Verbalize ways to cope with stress successfully, plans
for regular exercise, and rationale for obtaining
adequate rest.
1 de 20

Recomendados

Immunodeficiency diseases por
Immunodeficiency diseasesImmunodeficiency diseases
Immunodeficiency diseasesVaishnaPriyaAMicro19
45 vistas56 diapositivas
Cytotoxic reaction por
Cytotoxic reactionCytotoxic reaction
Cytotoxic reactionVISHAL VERMA
15 vistas16 diapositivas
CVID por
CVIDCVID
CVIDGizem Yazıcı
643 vistas24 diapositivas
ranjithakm-180315143057.pdf por
ranjithakm-180315143057.pdfranjithakm-180315143057.pdf
ranjithakm-180315143057.pdfSyedMuhammadZakria
7 vistas27 diapositivas
Immunodeficiency por
ImmunodeficiencyImmunodeficiency
ImmunodeficiencyRanjithaKM1
714 vistas27 diapositivas
Primary immunodeficiency diseases by dr.gobinda por
Primary immunodeficiency diseases by dr.gobindaPrimary immunodeficiency diseases by dr.gobinda
Primary immunodeficiency diseases by dr.gobindaGOBINDA PRASAD PRADHAN
3.2K vistas51 diapositivas

Más contenido relacionado

Similar a immunodeficiency_diseases.pptx

Immunodeficiency diseases por
Immunodeficiency diseasesImmunodeficiency diseases
Immunodeficiency diseasesRAJESH KUMAR
28.2K vistas45 diapositivas
immunodeficiencyppt-170409084703.pptx por
immunodeficiencyppt-170409084703.pptximmunodeficiencyppt-170409084703.pptx
immunodeficiencyppt-170409084703.pptxNadiirMahamoud
28 vistas44 diapositivas
IMMUNE DISORDERS, HIV/AIDS, METABOLIC SYNDROME por
IMMUNE DISORDERS, HIV/AIDS, METABOLIC SYNDROMEIMMUNE DISORDERS, HIV/AIDS, METABOLIC SYNDROME
IMMUNE DISORDERS, HIV/AIDS, METABOLIC SYNDROMERakhi Kripa Prince
376 vistas72 diapositivas
Immunodisorders primary and secondary por
Immunodisorders primary and secondaryImmunodisorders primary and secondary
Immunodisorders primary and secondaryArifa T N
2K vistas36 diapositivas
Immuno disorders por
Immuno disordersImmuno disorders
Immuno disordersArifa T N
222 vistas81 diapositivas
Format 2016: masqueradesyndromes in allergicdiseases. por
Format 2016: masqueradesyndromes in allergicdiseases.Format 2016: masqueradesyndromes in allergicdiseases.
Format 2016: masqueradesyndromes in allergicdiseases.Envicon Medical Srl
4.6K vistas156 diapositivas

Similar a immunodeficiency_diseases.pptx(20)

Immunodeficiency diseases por RAJESH KUMAR
Immunodeficiency diseasesImmunodeficiency diseases
Immunodeficiency diseases
RAJESH KUMAR28.2K vistas
immunodeficiencyppt-170409084703.pptx por NadiirMahamoud
immunodeficiencyppt-170409084703.pptximmunodeficiencyppt-170409084703.pptx
immunodeficiencyppt-170409084703.pptx
NadiirMahamoud28 vistas
IMMUNE DISORDERS, HIV/AIDS, METABOLIC SYNDROME por Rakhi Kripa Prince
IMMUNE DISORDERS, HIV/AIDS, METABOLIC SYNDROMEIMMUNE DISORDERS, HIV/AIDS, METABOLIC SYNDROME
IMMUNE DISORDERS, HIV/AIDS, METABOLIC SYNDROME
Rakhi Kripa Prince376 vistas
Immunodisorders primary and secondary por Arifa T N
Immunodisorders primary and secondaryImmunodisorders primary and secondary
Immunodisorders primary and secondary
Arifa T N2K vistas
Immuno disorders por Arifa T N
Immuno disordersImmuno disorders
Immuno disorders
Arifa T N222 vistas
Format 2016: masqueradesyndromes in allergicdiseases. por Envicon Medical Srl
Format 2016: masqueradesyndromes in allergicdiseases.Format 2016: masqueradesyndromes in allergicdiseases.
Format 2016: masqueradesyndromes in allergicdiseases.
Envicon Medical Srl4.6K vistas
Diagnostic approach to primary immunodefidiency disorder por PrernaChoudhary15
Diagnostic approach to primary immunodefidiency disorderDiagnostic approach to primary immunodefidiency disorder
Diagnostic approach to primary immunodefidiency disorder
PrernaChoudhary15240 vistas
immunodeficeincy.pptx por MohedLipan
immunodeficeincy.pptximmunodeficeincy.pptx
immunodeficeincy.pptx
MohedLipan3 vistas
Approach to a child with suspected immunodefeciency por Nitin Pawar
Approach to a child with suspected immunodefeciencyApproach to a child with suspected immunodefeciency
Approach to a child with suspected immunodefeciency
Nitin Pawar4.3K vistas
Dr sujeet kumar jha por DrSujeet Jha
Dr sujeet kumar jhaDr sujeet kumar jha
Dr sujeet kumar jha
DrSujeet Jha125 vistas
Overlap between allergy and immunedeficiency originallllll por Fawzia Abo-Ali
Overlap between allergy and immunedeficiency originallllllOverlap between allergy and immunedeficiency originallllll
Overlap between allergy and immunedeficiency originallllll
Fawzia Abo-Ali193 vistas
Immunodeficiency group 3 por rabbibaidoo
Immunodeficiency group 3Immunodeficiency group 3
Immunodeficiency group 3
rabbibaidoo578 vistas
Anaphylaxis and septicemia por THANUJA MATHEW
Anaphylaxis and septicemiaAnaphylaxis and septicemia
Anaphylaxis and septicemia
THANUJA MATHEW1.8K vistas
Approach to the child with immune based and allergic disease por Khaled Saad
Approach to the child with immune based and allergic diseaseApproach to the child with immune based and allergic disease
Approach to the child with immune based and allergic disease
Khaled Saad1.1K vistas

Más de hayatalakoum1

patient safety.pptx por
patient safety.pptxpatient safety.pptx
patient safety.pptxhayatalakoum1
67 vistas26 diapositivas
KPI.pptx por
KPI.pptxKPI.pptx
KPI.pptxhayatalakoum1
9 vistas28 diapositivas
Healthcare and facility.pptx por
Healthcare and facility.pptxHealthcare and facility.pptx
Healthcare and facility.pptxhayatalakoum1
5 vistas48 diapositivas
Team work; problems and incentives.pptx por
Team work; problems and incentives.pptxTeam work; problems and incentives.pptx
Team work; problems and incentives.pptxhayatalakoum1
15 vistas51 diapositivas
Nurse as educator.pptx por
Nurse as educator.pptxNurse as educator.pptx
Nurse as educator.pptxhayatalakoum1
7 vistas31 diapositivas
FACTORS THAT INFLUENCES.pptx por
FACTORS THAT INFLUENCES.pptxFACTORS THAT INFLUENCES.pptx
FACTORS THAT INFLUENCES.pptxhayatalakoum1
2 vistas34 diapositivas

Más de hayatalakoum1(20)

Team work; problems and incentives.pptx por hayatalakoum1
Team work; problems and incentives.pptxTeam work; problems and incentives.pptx
Team work; problems and incentives.pptx
hayatalakoum115 vistas
introduction to medical terminology pptx por hayatalakoum1
introduction to medical terminology pptxintroduction to medical terminology pptx
introduction to medical terminology pptx
hayatalakoum148 vistas
Les 8 principes du management de la qualité.pptx por hayatalakoum1
Les 8 principes du management de la qualité.pptxLes 8 principes du management de la qualité.pptx
Les 8 principes du management de la qualité.pptx
hayatalakoum117 vistas
Therapies for Blood Disorders.pptx por hayatalakoum1
Therapies for Blood Disorders.pptxTherapies for Blood Disorders.pptx
Therapies for Blood Disorders.pptx
hayatalakoum112 vistas
1.Research problem.pptx por hayatalakoum1
1.Research problem.pptx1.Research problem.pptx
1.Research problem.pptx
hayatalakoum1135 vistas
8. contact dermatitis.pptx por hayatalakoum1
8. contact dermatitis.pptx8. contact dermatitis.pptx
8. contact dermatitis.pptx
hayatalakoum163 vistas

Último

Anaemia,jaundice.pptx por
Anaemia,jaundice.pptxAnaemia,jaundice.pptx
Anaemia,jaundice.pptxReena Gollapalli
17 vistas7 diapositivas
Top PCD Pharma Franchise Companies in India | Saphnix Lifesciences por
Top PCD Pharma Franchise Companies in India | Saphnix LifesciencesTop PCD Pharma Franchise Companies in India | Saphnix Lifesciences
Top PCD Pharma Franchise Companies in India | Saphnix LifesciencesSaphnix Lifesciences
25 vistas11 diapositivas
occlusion in implantology.pptx por
occlusion in implantology.pptxocclusion in implantology.pptx
occlusion in implantology.pptxDr vaishali shrivastava
7 vistas99 diapositivas
Pregnancy tips.pptx por
Pregnancy tips.pptxPregnancy tips.pptx
Pregnancy tips.pptxreachout7
40 vistas10 diapositivas
Taking Action to Improve the Patient Journey With Transthyretin Amyloidosis (... por
Taking Action to Improve the Patient Journey With Transthyretin Amyloidosis (...Taking Action to Improve the Patient Journey With Transthyretin Amyloidosis (...
Taking Action to Improve the Patient Journey With Transthyretin Amyloidosis (...PeerVoice
7 vistas23 diapositivas
Cholera Romy W. (3).pptx por
Cholera Romy W. (3).pptxCholera Romy W. (3).pptx
Cholera Romy W. (3).pptxrweth613
38 vistas11 diapositivas

Último(20)

Top PCD Pharma Franchise Companies in India | Saphnix Lifesciences por Saphnix Lifesciences
Top PCD Pharma Franchise Companies in India | Saphnix LifesciencesTop PCD Pharma Franchise Companies in India | Saphnix Lifesciences
Top PCD Pharma Franchise Companies in India | Saphnix Lifesciences
Pregnancy tips.pptx por reachout7
Pregnancy tips.pptxPregnancy tips.pptx
Pregnancy tips.pptx
reachout740 vistas
Taking Action to Improve the Patient Journey With Transthyretin Amyloidosis (... por PeerVoice
Taking Action to Improve the Patient Journey With Transthyretin Amyloidosis (...Taking Action to Improve the Patient Journey With Transthyretin Amyloidosis (...
Taking Action to Improve the Patient Journey With Transthyretin Amyloidosis (...
PeerVoice7 vistas
Cholera Romy W. (3).pptx por rweth613
Cholera Romy W. (3).pptxCholera Romy W. (3).pptx
Cholera Romy W. (3).pptx
rweth61338 vistas
Structural Racism and Public Health: How to Talk to Policymakers and Communit... por katiequigley33
Structural Racism and Public Health: How to Talk to Policymakers and Communit...Structural Racism and Public Health: How to Talk to Policymakers and Communit...
Structural Racism and Public Health: How to Talk to Policymakers and Communit...
katiequigley33627 vistas
Complications & Solutions in Laparoscopic Hernia Surgery.pptx por Varunraju9
Complications & Solutions in Laparoscopic Hernia Surgery.pptxComplications & Solutions in Laparoscopic Hernia Surgery.pptx
Complications & Solutions in Laparoscopic Hernia Surgery.pptx
Varunraju9106 vistas
Preparation and Evaluation Ointment.pptx por Sudhanshu Sagar
Preparation and Evaluation Ointment.pptxPreparation and Evaluation Ointment.pptx
Preparation and Evaluation Ointment.pptx
Sudhanshu Sagar54 vistas
DEBATE IN CA BLADDER TMT VS CYSTECTOMY por Kanhu Charan
DEBATE IN CA BLADDER TMT VS CYSTECTOMYDEBATE IN CA BLADDER TMT VS CYSTECTOMY
DEBATE IN CA BLADDER TMT VS CYSTECTOMY
Kanhu Charan36 vistas
PCD Pharma Franchise In Chandigarh | Saphnix Lifesciences por Saphnix Lifesciences
PCD Pharma Franchise In Chandigarh | Saphnix LifesciencesPCD Pharma Franchise In Chandigarh | Saphnix Lifesciences
PCD Pharma Franchise In Chandigarh | Saphnix Lifesciences
eTEP -RS Dr.TVR.pptx por Varunraju9
eTEP -RS Dr.TVR.pptxeTEP -RS Dr.TVR.pptx
eTEP -RS Dr.TVR.pptx
Varunraju9131 vistas
CMC(CHEMISTRY,MANUFACTURING AND CONTROL).pptx por JubinNath2
CMC(CHEMISTRY,MANUFACTURING AND CONTROL).pptxCMC(CHEMISTRY,MANUFACTURING AND CONTROL).pptx
CMC(CHEMISTRY,MANUFACTURING AND CONTROL).pptx
JubinNath27 vistas
standards-of-care-2022-ADA.pdf por phambang8
standards-of-care-2022-ADA.pdfstandards-of-care-2022-ADA.pdf
standards-of-care-2022-ADA.pdf
phambang87 vistas
PATIENTCOUNSELLING in.pptx por skShashi1
PATIENTCOUNSELLING  in.pptxPATIENTCOUNSELLING  in.pptx
PATIENTCOUNSELLING in.pptx
skShashi116 vistas
BUKTI SOSIALISASI KODE ETIK DAN PERATURAN INTERNAL.docx 4,2,C.docx por InkhaRina
BUKTI SOSIALISASI KODE ETIK DAN PERATURAN INTERNAL.docx 4,2,C.docxBUKTI SOSIALISASI KODE ETIK DAN PERATURAN INTERNAL.docx 4,2,C.docx
BUKTI SOSIALISASI KODE ETIK DAN PERATURAN INTERNAL.docx 4,2,C.docx
InkhaRina32 vistas

immunodeficiency_diseases.pptx

  • 2. IMMUNODEFICIENCY DISEASES  Primary immunodeficiencies, rare disorders with genetic origins are seen primarily in infants and young children. To date, more than 95 immunodeficiencies of genetic origin have been identified(Buckley, 2000).  Symptoms usually develop early in life after protection from maternal antibodies decreases. Without treatment, infants and children with these disorders seldom survive to adulthood.  These disorders may involve one or more components of the immune system. Symptoms of immune deficiency diseases are related to the role that the deficient component normally plays
  • 3. IMMUNODEFICIENCY DISEASES  Immunodeficiency disorders may be caused by a defect or deficiency in phagocytic cells, B lymphocytes, T lymphocytes, or the complement system. The specific symptoms and their severity, age of onset, and prognosis depend on the immune system components affected and their degree of functional impairment.  Regardless of the underlying cause, the cardinal symptoms of immunodeficiency include chronic or recurrent severe infections, infections caused by unusual organisms or organisms that are normal body flora, poor response to treatment of infections, and chronic diarrhea.  In addition, the patient is susceptible to a variety of secondary disorders, including cancer.
  • 4. IMMUNODEFICIENCY DISEASES Immunodeficiencies may be classified as either primary or secondary and by the components of the immune system that are affected. Primary immunodeficiency: abnormalities in development of immune mechanisms Secondary immunodeficiency: Consequences of disease, drugs, nutritional inadequacies & other processes interfere with proper functioning of mature immune system
  • 5. PRIMARY IMMUNODEFICIENCY DISORDERS Immune component Disorder Major symptoms Treatment Phagocytic cells Hyperimmunoglo bulinemia E (HIE) syndrome Bacterial, fungal, and viral infections; deep-seated cold abscesses Antibiotic therapy and treatment for viral and fungal infections Granulocyte-macrophage colonystimulating factor (GM-CSF); granulocyte colony-stimulating factor (G-CSF) B lymphocytes Sex-linked agammaglobuline mia (Bruton’s disease) Common variable immunodeficienc y (CVID) Severe infections soon after birth Bacterial infections, infection with Giardia lamblia Pernicious anemia Chronic respiratory infections Passive pooled plasma or gammaglobulin IV immunoglobulin Metronidazole (Flagyl) Quinacrine HCl (Atabrine) Vitamin B12 Antimicrobial therapy
  • 6. PRIMARY IMMUNODEFICIENCY DISORDERS Immune component Disorder Major symptoms Treatment B lymphocytes Immunoglobulin A (IgA) Deficiency IgC2 deficiency Predisposition to recurrent infections, adverse reactions to blood transfusions or immunoglobulin, autoimmune diseases,hypothyroidism Heightened incidence of infectious diseases None Pooled immunoglobulin T lymphocytes Thymic hypoplasia (DiGeorge syndrome) Recurrent infections; hypoparathyroidism; hypocalcemia, tetany, convulsions; congenital heart disease; possible renal abnormalities; abnormal facies Thymus graft
  • 7. PRIMARY IMMUNODEFICIENCY DISORDERS Immune component Disorder Major symptoms Treatment T lymphocytes Chronic mucocutaneous candidiasis Candida albicans infections of mucous membrane, skin, and nails; endocrine abnormalities (hypoparathyroidism, Addison’s disease) Antifungal agents: Topical: miconazole Oral: clotrimazole, ketoconazole IV: amphotericin B B and T lymphocytes Ataxia- telangiectasia Ataxia with progressive neurologic deterioration; telangiectasia (vascular lesions); recurrent infections; malignancies Antimicrobial therapy; management of presenting symptoms; fetal thymus transplant, IV immunoglobulin
  • 8. PRIMARY IMMUNODEFICIENCY DISORDERS Immune component Disorder Major symptoms Treatment B and T lymphocytes Nezelof’s syndrome Severe infections; malignancies Antimicrobial therapy; IV immunoglobulin, bone marrow transplantation; thymus transplantation; thymus factors Wiskott-Aldrich syndrome Thrombocytopenia, resulting in bleeding; infections; malignancies Antimicrobial therapy; splenectomy with continuous antibiotic prophylaxis; IV immunoglobulin and bone marrow transplantation
  • 9. PRIMARY IMMUNODEFICIENCY DISORDERS Immune component Disorder Major symptoms Treatment B and T lymphocytes Severe combined immunodeficiency disease (SCID) Overwhelming severe fatal infections soon after birth (also includes opportunistic infections) Antimicrobial therapy; IV immunoglobulin and bone marrow transplantation Complement system Angioneurotic edema Paroxysmal nocturnal hemoglobinuria (PNH) Episodes of edema in various parts of the body, including respiratory tract and bowels Lysis of erythrocytes due to lack of decay-accelerating factor (DAF) on erythrocytes Pooled plasma, androgen therapy None
  • 10. THE 10 WARNING SIGNS OF PRIMARY IMMUNE DEFICIENCY 1. Eight or more new ear infections within 1 year. 2. Two or more serious sinus infections within 1 year. 3. Two or more months on antibiotics with little effect. 4. Two or more pneumonias within 1 year. 5. Failure of an infant to gain weight or grow normally. 6. Recurrent, deep skin or organ abscesses. 7. Persistent thrush in mouth or elsewhere on skin, after age 1.
  • 11. THE 10 WARNING SIGNS OF PRIMARY IMMUNE DEFICIENCY 8. Need for intravenous antibiotics to clear infections. 9. Two or more deep-seated infections such as meningitis, osteomyelitis, cellulitis, or sepsis. 10. A family history of primary immune deficiency.
  • 12. ALERT  Though the primary immune deficiency diseases can be serious, they are rarely fatal and can generally be controlled. Primary immune deficiency should not be confused with AIDS.  Primary immune deficiency can be diagnosed through blood tests and should be detected as soon as possible to prevent avoidable permanent damage. As with all disease, only direct examination by a physician should be used to determine the presence of primary immune deficiency.
  • 13. MANAGING AN IV IMMUNOGLOBULIN INFUSION  Weigh the patient before treatment.  Obtain vital signs before, during, and after treatment.  Administer the prescribed pretreatment prophylactic aspirin or IV antihistamine, such as diphenhydramine (Benadryl).  Be aware that corticosteroids may be used to prevent possible severe reactions.  Administer the IV infusion at a slow rate, not to exceed 3 mL/minute.  Assess the patient for adverse reactions, including the early signs of anaphylactic shock; prepare to slow the infusion rate if necessary.
  • 14. MANAGING AN IV IMMUNOGLOBULIN INFUSION  Be aware that patients with low gammaglobulin levels have more severe reactions than those with normal levels (eg, patients who receive gammaglobulin for thrombocytopenia or Kawasaki disease).  Keep in mind that patients who have an immunoglobulin A (IgA) deficiency have IgE antibodies to IgA, which requires administration of plasma or immunoglobulin replacement from IgA-deficient patients. Because all IV immunoglobulin preparations contain some IgA, they may cause an anaphylactic reaction in patients with IgE anti-IgA antibodies.  Remember that the risk for transmission of hepatitis, HIV, or other known viruses is extremely low.
  • 15. ADVERSE EFFECTS  Complaints of flank pain, shaking chills, and tightness in the chest, terminating with a slight rise in body temperature  Hypotension (possible with severe reactions)  Anaphylactic reactions
  • 16. SECONDARY IMMUNODEFICIENCY DISORDERS Category Examples Endocrine Diabetes mellitus GI Hepatic insufficiency, hepatitis, intestinal lymphangiectasia, protein-losing enteropathy Hematologic Aplatic anemia, cancer, graft-vs-host disease, sickle cell disease Iatrogenic Certain drugs: chemotherapeutic drugs, immunosuppressants, corticosteroids, radiation therapy; splenectomy Infectious Cytomegalovirus, Epstein-Barr virus, HIV, measles virus, varicella-zoster virus
  • 17. SECONDARY IMMUNODEFICIENCY DISORDERS Category Examples Nutritional Alcoholism, undernutrition Physiologic Physiologic immunodeficiency in infants due to immaturity of immune system, pregnancy Renal Nephrotic syndrome, renal insufficiency, uremia Rheumatologic RA, SLE Other Burns, chromosomal abnormalities (eg, Down syndrome), congenital asplenia, critical and chronic illness, histiocytosis, sarcoidosis
  • 18. INFECTION PREVENTION FOR THE PATIENT WITH IMMUNODEFICIENCY  Identify signs and symptoms of infection to report to the health care provider, such as fever, chills; wet or dry cough; breathing problems; white patches in the mouth; swollen glands; nausea; vomiting; persistent abdominal pain; persistent diarrhea; problems with urination; red, swollen, or draining wounds; sores or lesions on the body; and persistent vaginal discharge with or without itching.  Demonstrate correct handwashing procedure.
  • 19. INFECTION PREVENTION FOR THE PATIENT WITH IMMUNODEFICIENCY  State rationale for thorough hand washing before eating, after using the bathroom, and before and after performing health care procedures.  State rationale for use of cream and emollients to prevent or manage dry, chaffed, or cracked skin.  Demonstrate recommended personal hygiene in bathing and foot care to prevent bacterial and fungal diseases.  State rationale for avoiding contact with people who have known illness or who have recently been vaccinated.
  • 20. INFECTION PREVENTION FOR THE PATIENT WITH IMMUNODEFICIENCY  Verbalize understanding of ways to maintain a well- balanced diet and adequate calories.  State the reason for avoiding the eating of raw fruits and vegetables, cooking all foods thoroughly, and immediately refrigerating all leftover food.  Identify the rationale for frequent cleaning of kitchen and bathroom surfaces with disinfectant.  Identify rationale and benefits of avoiding alcohol, tobacco, and unprescribed medications.  State rationale for taking prescribed medications as directed.  Verbalize ways to cope with stress successfully, plans for regular exercise, and rationale for obtaining adequate rest.