Hypogammaglobulinemia

ALSANGEDY
 May be asymptomatic if mild
BULLETS™
FOR PACES
 Growth retardation &
Developmental abnormalities

 Adenoids and peripheral FOR EASY PASSING PACES FROM

THE 1ST ATTEMPT
lymphadenopathy
Dr. Ibrahim Alsangedy
 Rashes or livedo reticularis MRCP-UK, MRCEM-UK, MRCPE, AMC
CAT (AU), IDHA (USA), M.SC (ICU),
 Tympanic membrane ADDM, HCQM
perforation, purulent nasal
discharge and nasal exudate
 Bronchiectasis and lung
fibrosis with rales, rhonchi,
and wheezing
 Cardiovascular abnormalities
associated with DiGeorge or
CHARGE syndrome
 Bronchitis, ear infections,
meningitis, pneumonia,
sinus infections, and skin
infections
 Chronic diarrhea
 Complications from receiving
live vaccines
Workup Causes Management
 Laboratory Studies (1) Primary or congenital B-  Major goals of therapy
cell disorders
 Levels of serum  Common variable  Provide adequate replacement
immunoglobulin immunodeficiency (CVID) the immunoglobulins by monthly
 Antibody response after most common intravenously, subcutaneously,
immunization  Severe combined or weekly self-administered
 Isohemagglutinins immunodeficiency (SCID) the hypodermoclysis
 Peripheral blood lymphocyte most severe  Antibiotics are also frequently
immunophenotyping  X-linked agammaglobulinemia used
 Evaluation of cellular immunity (XLA)  Hematopoietic stem cell
 Autosomal recessive transplantation
agammaglobulinemia (ARA)  Gene therapy
 Radiologic Studies  Hyper-IgM syndromes
 Isolated non-IgG  The stepwise approach
 Chest radiograph immunoglobulin deficiencies
 High-resolution computed  IgG replacement therapy is the
(IgM, IgA)
tomography (HRCT) treatment of choice for most
 Nuclear scanning primary immunodeficiency
(2) Secondary or acquired syndromes
diseases  Aggressive and prolonged
 Nephrotic syndrome
 Complete septic  Protein-losing enteropathy
antibiotic therapy covering S
pneumoniae and H influenza is
workup for diagnosing  Catabolic disorders
indicated
acute septic  Immunosuppressive therapy  Treatment of secondary
 Lymphoproliferative is
presentations malignancies
hypogammaglobulinemia
directed at the underlying
 Prematurity in infants cause
 Drug-related
 HIV