Severe Combined Immunodeficiency (Scid)

SEVERE COMBINED IMMUNODEFICIENCY (SCID)


SEVERE COMBINED IMMUNODEFICIENCY (SCID)

It is caused due to –

  • Deficiency of Adenosine Deaminase (ADA)
  • Adenosine accumulates and converted to ribonucleotides and deoxyribonucleotides (dATP).
  • dATP inhibits ribonucleotidereductase which decreases production of deoxyribose nucleotides.
  • There is decrease in T and B cells leading to immunodeficiency.
  • X Linked Type is the Most Common Pattern of Inheritance

Clinical features-

  •  Chronic diarrhea
  •   failure to thrive.

Treatment-

  • Gene therapy is the first order to be treated.
  • Enzyme Replacement Therapy with Polyethyleneglycol modified bovine adenosine deaminase (PEGADA).

Exam Important

  • There is a decrease in T and B cells.
  • Adenosine accumulation is seen in SCID.
  • DNA synthesis decreases.
  • X Linked Pattren of Inheritance is most common
Don’t Forget to Solve all the previous Year Question asked on SEVERE COMBINED IMMUNODEFICIENCY (SCID)

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