How are complications of Chédiak-Higashi syndrome (CHS) prevented?

Updated: Aug 08, 2019
  • Author: Roman J Nowicki, MD, PhD; Chief Editor: Dirk M Elston, MD  more...
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Answer

Chédiak-Higashi syndrome (CHS) patients may exhibit an increased bleeding tendency owing to platelet dysfunction caused by delta storage pool deficiency. Preventative measures include avoidance of drugs that interfere with platelet functions such as aspirin, other nonsteroidal anti-inflammatory agents, or serotonin reuptake inhibitors. Intramuscular injections are prohibited, but subcutaneous injections are authorized. [14]

Hygiene should be meticulous to avoid bacterial infections. The skin should be washed 2 times a day with disinfectant soap to prevent skin infections. Cutting the fingernails to a short length helps to reduce autoinoculation.

Excessive operative blood loss should be anticipated during the operation secondary to quantitative and qualitative defects in platelet function, and certain techniques (eg, epidural anesthesia, intramuscular injections) should be avoided.


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