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Performance status and deaths among children registered in Kuwait National Primary ImmunoDeficiency Disorders

Waleed Al-Herz1, Mohammad E Zainal, Hamid M Alenezi

  • 1Department of Pediatrics, Al-Sabah Hospital, Kuwait. wemh@hotmail.com

Asian Pacific Journal of Allergy and Immunology
|November 3, 2010
PubMed

Insights

Children with primary immunodeficiency disorders (PIDD) face poor performance and high mortality. Early diagnosis and treatment for early onset and CMV infections can improve their quality of life.

Area of Science:

  • Pediatrics
  • Immunology
  • Public Health

Background:

  • Primary immunodeficiency disorders (PIDD) significantly impact children's physical, social, and psychological well-being.
  • Understanding performance status and mortality factors in pediatric PIDD is crucial for effective management.

Purpose of the Study:

  • To evaluate the performance status and mortality rates of children with PIDD in Kuwait.
  • To identify variables and comorbidities influencing performance and survival in pediatric PIDD patients.

Main Methods:

  • Utilized data from the Kuwait National Primary Immunodeficiency Disorders Registry for 98 pediatric patients.
  • Assessed patient performance using the Lansky Play Performance Scale (LPPS) and analyzed mortality data.
  • Examined the impact of diagnostic delay, comorbidities, and treatments on LPPS scores and mortality.

Main Results:

  • A mean diagnostic delay of 21.2 months was observed.
  • The mean LPPS score was 65.5, with significant variations across PIDD categories.
  • Factors like early onset (<6 months), CMV infection, consanguinity, antimicrobial prophylaxis, and IVIG therapy significantly affected performance and mortality.

Conclusions:

  • Pediatric patients with PIDD exhibit poor performance status and high mortality rates.
  • Early diagnosis and targeted interventions for early symptom onset and CMV infections are vital for improving quality of life in PIDD patients.

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