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Primary immunodeficiency

Immunodeficientia primaria · Primární imunodeficit

What it is

Part of the immune system is missing from birth; infections keep coming back.

Symptoms & signs

  • Recurrent severe infections
  • failure to thrive
  • chronic diarrhoea
  • unusual organisms
  • family history of early deaths

🚩 Get help if…

  • call 112 / 911 now Fever with shivers, fast breathing or confusion
  • see a doctor soon Two or more pneumonias in a year
  • see a doctor soon Infections that need IV antibiotics or keep coming back
  • see a doctor soon A baby who fails to gain weight with ongoing diarrhoea or thrush

Where it strikes

Medical treatment

  • Immunoglobulin replacement
  • prophylactic antibiotics
  • stem-cell transplant
  • gene therapy
  • avoid live vaccines in some forms

Natural & lifestyle

  • Infection prevention
  • vaccination of household contacts
  • nutrition
  • newborn SCID screening

Emerging & experimental

  • Lentiviral gene therapy for ADA-SCID and X-SCID
  • gene editing
  • thymus transplantation

Alternative, off-label & anecdotal

Approaches used outside the guidelines, with what their supporters report and what testing has found so far.

  • Probiotics — small studies of fewer infections early / small studies
  • 'Immune boosters' — cannot replace missing antibodies or cells anecdotal / traditional

ICD codes