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Health-related quality of life and cognitive function in children with Crigler-Najjar syndrome type 1.
El-Anwar, Noha; El-Shabrawi, Mortada; Shahin, Ola Omar; Abdel Kareem, Reem; Salama, Ahmed Mohamed; Baroudy, Sherif.
Afiliación
  • El-Anwar N; Department of Paediatrics, Faculty of Medicine, Cairo University, Cairo, Egypt.
  • El-Shabrawi M; Department of Paediatrics, Faculty of Medicine, Cairo University, Cairo, Egypt.
  • Shahin OO; Clinical and Chemical Pathology Department, Faculty of Medicine, Cairo University, Cairo, Egypt.
  • Abdel Kareem R; Paediatrics department, Ministry of Health and Population, Cairo, Egypt.
  • Salama AM; Paediatrics department, Ministry of Health and Population, Cairo, Egypt.
  • Baroudy S; Department of Paediatrics, Faculty of Medicine, Cairo University, Cairo, Egypt.
Paediatr Int Child Health ; 44(1): 18-23, 2024 05.
Article en En | MEDLINE | ID: mdl-38334259
ABSTRACT

BACKGROUND:

The aim of the study was to assess the health-related quality of life (HRQOL) and cognitive function in patients with Crigler-Najjar syndrome (CNS) type I and its impact on their lives.

METHODS:

Twenty-one patients diagnosed with CNS type I aged 1 month to 18 years in the Paediatric Hepatology Unit of Cairo University Children's Hospital were enrolled in this cross-sectional observational study. The patients' health-related quality of life (HRQOL) was assessed using the World Health Organization Quality Of Life BREF questionnaire (WHOQOL-BREF) and the Short Form 36 Health Survey Questionnaire (SF-36). Cognitive function was assessed using the Stanford-Binet Intelligence Scale Fifth Edition (SB5).

RESULTS:

All patients had a history of admission to a neonatal intensive care unit, 17 were managed by phototherapy only and 5 also underwent exchange transfusion. According to the WHOQOL questionnaire, 11 cases (52.4%) had a low QOL score, and 7 of 13 patients had an average score for their total IQ test. Cases with poor compliance to phototherapy had statistically significantly lower QOL scores (p=0.001), while, according to the SF36 survey, cases who received exchange transfusion had statistically significantly higher cognitive function (p=0.03). There was a positive correlation between the neurological effect as a complication of the disease and poor physical QOL.

CONCLUSION:

Paediatric patients with CNS have significantly lower HRQOL, especially physically, psychologically and environmentally. It is recommended that assessment of HRQOL should be a routine part of follow-up in CNS patients. Patients whose HRQOL is affected receive regular psychiatric counselling, social support and rehabilitation.Abbreviations CNS Crigler-Najjar syndrome; HRQOL health-related quality of life; IQ intelligence quotient; NICU neonatal intensive care unit; QOL quality of life; SB5 Stanford-Binet intelligence scale 5th edition; SF-36 Short Form 36 Health Survey Questionnaire; UDGT uridine diphosphate glucuronosyl transferase; UGT1A1 uridine 5'-diphosphate glucuronosyltransferase; WHOQOL-BREF World Health Organization Quality of Life Brief Version.
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Texto completo: 1 Colección: 01-internacional Base de datos: MEDLINE Asunto principal: Calidad de Vida / Síndrome de Crigler-Najjar Tipo de estudio: Observational_studies Aspecto: Patient_preference Límite: Adolescent / Child / Child, preschool / Humans / Infant Idioma: En Revista: Paediatr Int Child Health Año: 2024 Tipo del documento: Article País de afiliación: Egipto Pais de publicación: Reino Unido
Texto completo
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Texto completo: 1 Colección: 01-internacional Base de datos: MEDLINE Asunto principal: Calidad de Vida / Síndrome de Crigler-Najjar Tipo de estudio: Observational_studies Aspecto: Patient_preference Límite: Adolescent / Child / Child, preschool / Humans / Infant Idioma: En Revista: Paediatr Int Child Health Año: 2024 Tipo del documento: Article País de afiliación: Egipto Pais de publicación: Reino Unido