Physical Growth of Patients with Hereditary Tyrosinaemia Type I: A Single-Centre Retrospective Study
In a retrospective review, we aimed to assess long-term growth in 17 patients (<i>n</i> = 11 males) with hereditary tyrosinaemia type I (HTI). Median age at assessment was 15.6 years (5.7–26.6 years) and median age at diagnosis was 1 month (range: 0–16 months), with 35% (<i>n</i...
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| Format: | Article |
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MDPI AG
2021-08-01
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| Series: | Nutrients |
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| Online Access: | https://www.mdpi.com/2072-6643/13/9/3070 |
| _version_ | 1797517820294070272 |
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| author | Ozlem Yilmaz Anne Daly Alex Pinto Catherine Ashmore Sharon Evans Girish Gupte Richard Jackson Nurcan Yabanci Ayhan Anita MacDonald |
| author_facet | Ozlem Yilmaz Anne Daly Alex Pinto Catherine Ashmore Sharon Evans Girish Gupte Richard Jackson Nurcan Yabanci Ayhan Anita MacDonald |
| author_sort | Ozlem Yilmaz |
| collection | DOAJ |
| description | In a retrospective review, we aimed to assess long-term growth in 17 patients (<i>n</i> = 11 males) with hereditary tyrosinaemia type I (HTI). Median age at assessment was 15.6 years (5.7–26.6 years) and median age at diagnosis was 1 month (range: 0–16 months), with 35% (<i>n</i> = 6/17) symptomatic on presentation. From the age of 8 years, there was a noticeable change in median height, weight, and body-mass-index [BMI]-z-scores. Median height-for-age z-scores were consistently ≤ −1 (IQR −1.6, −0.5) during the first 8 years of life but increased with age. Weight-for-age z-scores ranged between −1 to 0 (IQR −1.2, 0.1) in the first 8 years; then increased to > 0.5 (IQR −0.3, 1.3) by age 16 years, and BMI-for-age z-scores ranged from 0 to 1 (IQR −0.7, 1.3) up to 8 years, and >1 (IQR −0.2, 1.9) until 16 years. The percentage of overweight and obesity was lowest in children aged < 5 years, and consistently > 40% in patients aged between 7 to 16 years. The prescribed total protein intake was associated with improved height growth (p < 0.01). Impaired growth in early life improved with age achieving normal population standards. Further studies are needed to investigate factors that influence growth outcome in HTI patients. |
| first_indexed | 2024-03-10T07:20:31Z |
| format | Article |
| id | doaj.art-b0031254bda94be3bce7441c9cc482c8 |
| institution | Directory Open Access Journal |
| issn | 2072-6643 |
| language | English |
| last_indexed | 2024-03-10T07:20:31Z |
| publishDate | 2021-08-01 |
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| record_format | Article |
| series | Nutrients |
| spelling | doaj.art-b0031254bda94be3bce7441c9cc482c82023-11-22T14:36:54ZengMDPI AGNutrients2072-66432021-08-01139307010.3390/nu13093070Physical Growth of Patients with Hereditary Tyrosinaemia Type I: A Single-Centre Retrospective StudyOzlem Yilmaz0Anne Daly1Alex Pinto2Catherine Ashmore3Sharon Evans4Girish Gupte5Richard Jackson6Nurcan Yabanci Ayhan7Anita MacDonald8Birmingham Women’s and Children’s Hospital, Birmingham B4 6NH, UKBirmingham Women’s and Children’s Hospital, Birmingham B4 6NH, UKBirmingham Women’s and Children’s Hospital, Birmingham B4 6NH, UKBirmingham Women’s and Children’s Hospital, Birmingham B4 6NH, UKBirmingham Women’s and Children’s Hospital, Birmingham B4 6NH, UKBirmingham Women’s and Children’s Hospital, Birmingham B4 6NH, UKCancer Research UK Liverpool Cancer Trials Unit, University of Liverpool, Liverpool L69 3GL, UKDepartment of Nutrition and Dietetics, Faculty of Health Sciences, Ankara University, Ankara 06290, TurkeyBirmingham Women’s and Children’s Hospital, Birmingham B4 6NH, UKIn a retrospective review, we aimed to assess long-term growth in 17 patients (<i>n</i> = 11 males) with hereditary tyrosinaemia type I (HTI). Median age at assessment was 15.6 years (5.7–26.6 years) and median age at diagnosis was 1 month (range: 0–16 months), with 35% (<i>n</i> = 6/17) symptomatic on presentation. From the age of 8 years, there was a noticeable change in median height, weight, and body-mass-index [BMI]-z-scores. Median height-for-age z-scores were consistently ≤ −1 (IQR −1.6, −0.5) during the first 8 years of life but increased with age. Weight-for-age z-scores ranged between −1 to 0 (IQR −1.2, 0.1) in the first 8 years; then increased to > 0.5 (IQR −0.3, 1.3) by age 16 years, and BMI-for-age z-scores ranged from 0 to 1 (IQR −0.7, 1.3) up to 8 years, and >1 (IQR −0.2, 1.9) until 16 years. The percentage of overweight and obesity was lowest in children aged < 5 years, and consistently > 40% in patients aged between 7 to 16 years. The prescribed total protein intake was associated with improved height growth (p < 0.01). Impaired growth in early life improved with age achieving normal population standards. Further studies are needed to investigate factors that influence growth outcome in HTI patients.https://www.mdpi.com/2072-6643/13/9/3070tyrosinaemiagrowthphysical outcomesweightheightBMI |
| spellingShingle | Ozlem Yilmaz Anne Daly Alex Pinto Catherine Ashmore Sharon Evans Girish Gupte Richard Jackson Nurcan Yabanci Ayhan Anita MacDonald Physical Growth of Patients with Hereditary Tyrosinaemia Type I: A Single-Centre Retrospective Study Nutrients tyrosinaemia growth physical outcomes weight height BMI |
| title | Physical Growth of Patients with Hereditary Tyrosinaemia Type I: A Single-Centre Retrospective Study |
| title_full | Physical Growth of Patients with Hereditary Tyrosinaemia Type I: A Single-Centre Retrospective Study |
| title_fullStr | Physical Growth of Patients with Hereditary Tyrosinaemia Type I: A Single-Centre Retrospective Study |
| title_full_unstemmed | Physical Growth of Patients with Hereditary Tyrosinaemia Type I: A Single-Centre Retrospective Study |
| title_short | Physical Growth of Patients with Hereditary Tyrosinaemia Type I: A Single-Centre Retrospective Study |
| title_sort | physical growth of patients with hereditary tyrosinaemia type i a single centre retrospective study |
| topic | tyrosinaemia growth physical outcomes weight height BMI |
| url | https://www.mdpi.com/2072-6643/13/9/3070 |
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