Cutaneous adverse reactions to drugs are common in pediatric practice and often present a diagnostic challenge.
Pathogenesis:
The pathogenesis of most drug eruptions is not well understood. With few exceptions, eg, fixed drug eruption, the diagnosis cannot be based solely on the morphology of the eruption.

Clinical Features:
A drug rash may manifest as urticaria, a morbilliform exanthem, erythroderma, Stevens-Johnson syndrome (SJS), toxic epidermal necrolysis (TEN), photosensitivity, lichen planus, or vasculitis, all of which have other potential causes. A high index of suspicion for drug causation is important so that an offending drug is discontinued and avoided in the future, particularly in the case of life-threatening reactions such as anaphylaxis, the drug (anticonvulsant) hypersensitivity syndrome, SJS, and TEN. Conversely, it is important not to err by labeling a child as “allergic” to a widely used medication, such as penicillin. There are no standardized laboratory investigations that are diagnostic for drug allergy, and the value of allergy testing is largely restricted to cases of IgE-mediated penicillin hypersensitivity. Therefore, a detailed history, evaluation of the morphology of the rash, consideration of a differential diagnosis, and careful clinical judgment are essential.
The timing of the reaction may be helpful. Medications begun recently, particularly within the past Continue reading »
VN:F [1.9.2_1090]
Rating: 10.0/10 (1 vote cast)
VN:F [1.9.2_1090]
Rating: +1 (from 1 vote)
  • Share/Bookmark
Both cell-mediated (T-cell) and humoral (B-cell) immunity are deficient or absent in severe combined immunodeficiency disease (SCID). This results in susceptibility to infection from all classes of microorganisms during infancy.
At least three types of SCID exist: reticular dysgenesis, the most severe type, in which the hematopoietic stem cell fails to differentiate into lymphocytes and granulocytes; Swiss-type agammaglobulinemia, in which the hematopoietic stem cell fails to differentiate into lymphocytes alone; and enzyme deficiency, such as adenosine deaminase (ADA) deficiency, in which

the buildup of toxic products in the lymphoid tissue causes damage and subsequent dysfunction.
Incidence
SCID affects more males than females; its estimated incidence is 1 in every 100,000 to 500,000 births. Most untreated patients die from infection within 1 year of birth.

Causes
SCID is usually transmitted as an autosomal recessive trait, although it may be X-linked. In most cases, the genetic defect seems associated with failure of the stem cell to differentiate into T and B lymphocytes.
Many molecular defects, such as mutation of the kinase ZAP-70, can cause SCID. X-linked SCID results from a mutation of a subunit of the interleukin-2 (IL-2), IL-4, and IL-7 receptors. Less commonly, it results from an enzyme deficiency.
Signs and symptoms
An extreme susceptibility to infection becomes obvious in the infant with SCID in the first months of life. The infant fails to thrive and develops chronic otitis, sepsis, watery diarrhea (associated with Salmonella or Escherichia coli), recurrent pulmonary infections (usually caused by Pseudomonas, cytomegalo-virus, or Pneumocystis carinii), persistent oral candidiasis (sometimes with esophageal erosions), and possibly fatal viral infections (such as chickenpox).
P. carinii pneumonia usually strikes a severely immunodeficient infant in the first 3 to 5 weeks of Continue reading »
VN:F [1.9.2_1090]
Rating: 10.0/10 (1 vote cast)
VN:F [1.9.2_1090]
Rating: +1 (from 1 vote)
  • Share/Bookmark
© 2012 Easy Pediatrics Suffusion WordPress theme by Sayontan Sinha