Functional Impairment and Its Determinants in Adolescents With Inherited Bleeding Disorders: A Single-Centre Cross-Sectional Study.
- 1Internal Medicine, Pakistan Institute of Medical Sciences, Islamabad, PAK.
- 2General Medicine, Pakistan Institute of Medical Sciences, Islamabad, PAK.
- 3Medicine, Shaheed Zulfiqar Ali Bhutto Medical University, Islamabad, Islamabad, PAK.
Abstract
Inherited bleeding disorders (IBDs) require long-term management through adolescence, a period of rapid social and educational development. This study set out to quantify day-to-day functional impairment in adolescents with IBDs attending a tertiary care centre in Islamabad, Pakistan, and to identify the characteristics associated with it. This study aimed to determine the frequency and severity of functional impairment among adolescents with IBDs attending a tertiary care centre and to examine its association with demographic and clinical characteristics. A cross-sectional study was conducted in the Department of Medicine, Pakistan Institute of Medical Sciences (PIMS), Islamabad, between June and September 2025. One hundred and fifty adolescents aged 8-19 years with a confirmed IBD were recruited by consecutive non-probability sampling. Patients who had undergone surgery within the preceding two months or who had an active bacterial infection, malignancy, hepatic impairment, or renal impairment were excluded. Functional impairment was measured using the Columbia Impairment Scale - Youth Version (CIS-Y), a 13-item instrument yielding a total score of 0-52. Scores were classified as minimal or mild (<15), moderate (15-30), and severe (>30). Associations were examined with the Pearson chi-square test, supplemented by Fisher's exact test and an odds ratio for the comparison by sex, with p ≤ 0.05 taken as significant. The mean age was 13.93 ± 3.46 years, and 94 participants (62.7%) were male. Haemophilia A or B was the most common diagnosis (61, 40.7%), followed by platelet function disorders (38, 25.3%), von Willebrand disease (36, 24.0%), and other rare factor deficiencies (15, 10.0%). The mean CIS-Y score was 16.85 ± 11.35 (median 16.0, interquartile range 8.0-24.0). Sixty-six participants (44.0%) had minimal or mild impairment, 73 (48.7%) had moderate impairment, and 11 (7.3%) had severe impairment. Female sex was the only characteristic significantly associated with impairment severity (p = 0.015): 39 of 56 girls (69.6%) scored in the moderate or severe range compared with 45 of 94 boys (47.9%), giving an odds ratio of 2.50 (95% CI 1.24-5.02, Fisher's exact p = 0.011). The median CIS-Y score was correspondingly higher in girls than boys (21.0 vs 14.0, p = 0.004). Age group, body mass index, socioeconomic status, residence, family structure, ethnicity, and type of bleeding disorder showed no significant association. Close to half of the adolescents attending this centre with an IBD had moderate functional impairment, and girls had roughly twice the odds of impairment compared with boys. Severe impairment was uncommon. Functional and psychosocial assessment deserves a routine place in the follow-up of these patients, with particular attention to adolescent girls.
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BibTeX
@article{irfan2026functional,
title = {{Functional Impairment and Its Determinants in Adolescents With Inherited Bleeding Disorders: A Single-Centre Cross-Sectional Study.}},
author = {Tayyaba Irfan and Moneeba Tahir and Valeed B Mansoor and Farwa Rasheed and Fibhaa Syed},
journal = {Cureus},
year = {2026},
month = oct,
doi = {10.7759/cureus.117637},
url = {https://europepmc.org/article/MED/42999927},
note = {CC BY},
}
RIS
TY - JOUR
TI - Functional Impairment and Its Determinants in Adolescents With Inherited Bleeding Disorders: A Single-Centre Cross-Sectional Study.
AU - Tayyaba Irfan
AU - Moneeba Tahir
AU - Valeed B Mansoor
AU - Farwa Rasheed
AU - Fibhaa Syed
PY - 2026
DA - 2026/10/08
JO - Cureus
DO - 10.7759/cureus.117637
UR - https://europepmc.org/article/MED/42999927
AB - Inherited bleeding disorders (IBDs) require long-term management through adolescence, a period of rapid social and educational development. This study set out to quantify day-to-day functional impairment in adolescents with IBDs attending a tertiary care centre in Islamabad, Pakistan, and to identify the characteristics associated with it. This study aimed to determine the frequency and severity of functional impairment among adolescents with IBDs attending a tertiary care centre and to examine its association with demographic and clinical characteristics. A cross-sectional study was conducted in the Department of Medicine, Pakistan Institute of Medical Sciences (PIMS), Islamabad, between June and September 2025. One hundred and fifty adolescents aged 8-19 years with a confirmed IBD were recruited by consecutive non-probability sampling. Patients who had undergone surgery within the preceding two months or who had an active bacterial infection, malignancy, hepatic impairment, or renal impairment were excluded. Functional impairment was measured using the Columbia Impairment Scale - Youth Version (CIS-Y), a 13-item instrument yielding a total score of 0-52. Scores were classified as minimal or mild (<15), moderate (15-30), and severe (>30). Associations were examined with the Pearson chi-square test, supplemented by Fisher's exact test and an odds ratio for the comparison by sex, with p ≤ 0.05 taken as significant. The mean age was 13.93 ± 3.46 years, and 94 participants (62.7%) were male. Haemophilia A or B was the most common diagnosis (61, 40.7%), followed by platelet function disorders (38, 25.3%), von Willebrand disease (36, 24.0%), and other rare factor deficiencies (15, 10.0%). The mean CIS-Y score was 16.85 ± 11.35 (median 16.0, interquartile range 8.0-24.0). Sixty-six participants (44.0%) had minimal or mild impairment, 73 (48.7%) had moderate impairment, and 11 (7.3%) had severe impairment. Female sex was the only characteristic significantly associated with impairment severity (p = 0.015): 39 of 56 girls (69.6%) scored in the moderate or severe range compared with 45 of 94 boys (47.9%), giving an odds ratio of 2.50 (95% CI 1.24-5.02, Fisher's exact p = 0.011). The median CIS-Y score was correspondingly higher in girls than boys (21.0 vs 14.0, p = 0.004). Age group, body mass index, socioeconomic status, residence, family structure, ethnicity, and type of bleeding disorder showed no significant association. Close to half of the adolescents attending this centre with an IBD had moderate functional impairment, and girls had roughly twice the odds of impairment compared with boys. Severe impairment was uncommon. Functional and psychosocial assessment deserves a routine place in the follow-up of these patients, with particular attention to adolescent girls.
SN - 2168-8184
ER -