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Angioedema is a localized, self-limited (but potentially life-threatening), nonpitting, asymmetrical edema occurring in the deep layers of the skin and mucosal tissue. The common underlying pathophysiology involves inflammatory mediators triggering significant vasodilation and increased capillary permeability. Clinically, angioedema presents with swelling around the eyes, lips, tongue, mouth, bowel wall, extremities, or genitalia. Angioedema may also compromise the airway. Urticaria will be present when the angioedema is mediated by mast cells, but not when it is due to increases in bradykinin. Diagnosis is usually clinical but additional testing may include skin/serum testing for specific antigens and C4 level assessment. Management depends on the underlying mechanism but may include treatment for anaphylaxis, removing any offending agents, antihistamines, glucocorticoids, and/or therapies that target bradykinin.

T-Cell Deficiency, Severe Combined Immunodeficiency (SCID), Ataxia-Telangiectasia, Wiskott–Aldrich syndrome (WAS) & DiGeorge Syndrome

T-Cell Deficiency causes cell-mediated immunodeficiency. Examples of T cell disorders include Severe Combined Immunodeficiency (SCID), Ataxia-Telangiectasia, Wiskott–Aldrich syndrome (WAS), and DiGeorge Syndrome. SCID causes the disturbing development of functional B and T cells due to a number of genetic mutations. Ataxia telangiectasia is a combined deficiency of T cells, immunoglobulins, and neurocutaneous findings. WAS is caused by WAS gene mutation resulting in a lack of functional Wiskott-Aldrich syndrome protein. DiGeorge Syndrome results from microdeletion in a small segment of chromosome 22.

Chronic Granulomatous Disease (CGD) in Children — Symptoms and Treatment

Chronic granulomatous disease is caused by impaired phagocyte NADPH oxidase complex activity, or complete absence of the enzyme. X-linked and autosomal recessive patterns of inheritance have been described for the condition. Patients usually present with recurrent skin, lung and gastrointestinal infections. Granulomas can form in the skin, gastrointestinal tract or genitourinary tract. Patients should undergo qualitative and quantitative testing of the oxidase complex enzyme from isolated neutrophils. The mainstay treatment of chronic granulomatous disease is antimicrobial therapy and interferon-gamma. Hematopoietic stem cell transplantation is curative, but carries significant risks.

Humoral Immune Deficiency (Humoral Immunodeficiency) in Children — Clinical Presentation and Diagnostic Workup

CVID, also known as humoral immunodeficiency, is characterized by reduced serum levels of immunoglobulins G and A and, in some patients, also immunoglobulin M. Patients present with recurrent infections, an increased risk of autoimmune disorders and malignant disease. Quantification of serum levels of immunoglobulins, in addition to assessment of the functional activity of lymphocytes, is indicated in the diagnostic workup of CVID. Specific culture and sensitivity tests should be performed in patients with infectious complications to guide antibiotic therapy. Treatment of CVID includes antimicrobial therapy, immunoglobulin replacement therapy and immunomodulation therapy in patients with autoimmune disease.

Allergies in Children — Types, Diagnosis and Treatment

Allergies are the reactions mediated by immune system against a specific substance or allergen. Allergen can be certain food, dust, pollen, or medicines. The common allergic conditions are asthma, contact dermitis, hay fever, allergic rhinitis, eczema, food allergies, urticaria and angioedema. Urticaria is characterized by discrete raised and red lesions of the skin due to edema within the dermis and epidermis. When the extravasation of fluid involves the subcutaneous tissue, the term angioedema is used. Child’s medical history and allergy skin test establishes the diagnosis. Avoidance of allergens are recommended in the management.

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Pediatric Allergy and Immunology

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