Abstract

Background and ObjectiveKaposiform hemangioendothelioma (KHE) is a rare, aggressive and borderline vascular tumor mainly occurring in infants and children. The aim of this study was to determine the health-related quality of life (HRQOL) in children with KHE.MeasuresA total of 91 children with KHE participated in this cross-sectional study. The HRQOL was assessed by the age-specific Pediatric Quality of Life Inventory Version 4.0 (PedsQL™ 4.0) Infant Scales, Family Information Form (FIF), Family Impact Module (FIM) and Generic Core Scales (GCS). For comparison, demographically matched healthy children were recruited as a control group. The main outcome measure of HRQOL was analyzed in the two groups. We determined related factors that influenced the HRQOL in children with KHE and their parents by using a stepwise multiple regression analysis.ResultsThe study found that the scores of each item in the family impact module (FIM) were lower than 75, which suggesting that KHE can make the parents of patients in a state of poor quality of life. The scores of physiological and psychosocial domains in all age groups of patients with KHE were lower than those of normal children (P < 0.01). Activity dysfunction is the factor influencing the physiological function score of all-age patients. KMP is the factor influencing the psychosocial function score of all-age patients.ConclusionsThe findings presented here suggest that patients with KHE have a poor HRQOL. KMP and activity dysfunction are risk factors for poor HRQOL in patients with KHE. However, lesion size, lesion location and education level of the mother and father were not related to the HRQOL.

Highlights

  • Kaposiform hemangioendothelioma (KHE) is a rare, endodermic, aggressive and borderline vascular tumor mainly occurring in infants and children (1)

  • We recruited 0–14-year-old children diagnosed with KHE at the Department of Pediatric Surgery, West China Hospital of Sichuan University, and coetaneous healthy children at the Department of Children Preventive Health Care, All the recruited patients were newly diagnosed with KHE by Abbreviations: KHE, Kaposiform hemangioendothelioma; health-related quality of life (HRQOL), healthrelated quality of life; KMP, Kasabach-Merritt phenomenon; QOL, quality of life; PedsQLTM 4.0, Pediatric Quality of Life Inventory Version 4.0; FIF, Family Information Form; FIM, Family Impact Module; GCS, Generic Core Scales; SD, standard deviation

  • Through the analysis of the influencing factors of HRQOL in patients with KHE, we found that the activity dysfunction was the factor influencing the score of physiological function, and KMP was the factor influencing the score of psychosocial function

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Summary

Introduction

Kaposiform hemangioendothelioma (KHE) is a rare, endodermic, aggressive and borderline vascular tumor mainly occurring in infants and children (1). QOL in Kaposiform Hemangioendothelioma there is currently no appropriate assessment of quality of life (QOL) in patients with KHE. It is still unknown whether KHE affects the QOL in children with KHE and their parents. Kaposiform hemangioendothelioma (KHE) is a rare, aggressive and borderline vascular tumor mainly occurring in infants and children. The aim of this study was to determine the health-related quality of life (HRQOL) in children with KHE. We determined related factors that influenced the HRQOL in children with KHE and their parents by using a stepwise multiple regression analysis

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