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Table 1 Types of Immunodeficiency, Diagnosis, and Number of Patients

From: Primary Immunodeficiency Diseases in Oman: Five Years' Experience at Sultan Qaboos University Hospital

Type of Immunodeficiency

Diagnosis and No. Patients

Predominantly antibody disorders, 16 patients

X-linked agammaglobulinemia, 3

 

Agammaglobulinemia, 4

 

Common variable immunodeficiency, 5

 

Hyper IgM syndrome, 2

 

Selective IgA deficiency, 1

 

Transient hypogammaglobulinemia of infancy, 1

Combined immunodeficiency, 11 patients

Severe combined immunodeficiency, 5

 

Major histocompatibility Class II deficiency, 2

 

Combined immunodeficiency, 4

Phagocytic disorders, 38 patients

CGD, 31

 

Interferon gamma receptor II deficiency, 2

 

Severe congenital neutropenia, 2

 

Leukocyte adhesion defect type-1, 1

 

Pearson syndrome (infections due to neutropenia), 1

 

Severe neutrophil defect, granules defect, 1

Complement deficiencies, 5 patients

Complement 3 deficiency, 1

 

Complement 7 deficiency, 1

 

C1 Esterase inhibitor deficiency type-1, 2

 

C1 Esterase inhibitor deficiency type-2, 1

Other well defined PID syndromes, 12 patients

Ataxia telangiectasia, 6

 

Di-George syndrome, 1

 

Cartilage hair hypoplasia, 1

 

Chronic mucocutaneous candidiasis, 1

 

Autosomal recessive hyper-IgE syndrome, 2

 

Nijmegen breakage syndrome, 1

Immune dysregulation syndromes, 3 patients

Chediak-Higashi syndrome, 2

 

Autoimmune lymphoproliferative syndrome, 1

Unclassified PIDs, 5 patients

4 patients presented with nonpruritic angioedema without urticaria but C1 esterase inhibitor was normal, and one patient presented with recurrent infections but investigations were normal