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Growth charts for patients with Hunter syndrome ☆

View Article: PubMed Central - PubMed

ABSTRACT

Children with mucopolysaccharidosis II (MPS II), also known as Hunter syndrome, an X-linked disorder, suffer from a multisystem dysfunction caused by the accumulation of glycosaminoglycans. However, there has been no systemic report on the growth of patients with MPS II. The purpose of this study is to describe the growth patterns of patients with MPS II and to compare with the patterns of age-matched controls. Data (height, weight, age, etc.) was collected in a longitudinal study of Japanese male patients with MPS II (n = 111). The mean birth length was 50.31 ± 1.42 cm, while the mean birth weight was 3.35 ± 0.39 kg. The mean final height and weight at 18 years and older were 125.63 ± 9.09 cm and 37.18 ± 8.72 kg; corresponding to a difference of − 46.40 cm and − 25.89 kg lower, when compared with healthy Japanese male controls. The mean birth BMI was 10.84 ± 3.29 kg/m2, while the mean BMI at 18 years was 29.41 ± 6.15 kg/m2. The growth pattern in patients with MPS II was characterized by overgrowth for the first several years, although growth velocity fell below that of the normal healthy controls after one year of age. No statistical difference in height was observed between patients with the attenuated and severe phenotypes in each age class.

In conclusion, this report describes the natural history of growth in patients with MPS II, which can help in monitoring the progression of the disease as well as assessing therapeutic efficacy.

No MeSH data available.


Related in: MedlinePlus

BMI curve for patients with MPS II from birth to 18 years of age. The dotted line shows the mean BMI for healthy males. The solid line shows BMIs for patients with MPS II. Arrows point to their respective percentile curves.
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f0050: BMI curve for patients with MPS II from birth to 18 years of age. The dotted line shows the mean BMI for healthy males. The solid line shows BMIs for patients with MPS II. Arrows point to their respective percentile curves.

Mentions: The average BMI for newborn patients was 10.84 ± 3.30 kg/m2, and the BMI for patients at18 years and older was 29.41 ± 6.15 kg/m2. When compared with the normal control, 97% of patients had a BMI higher than the mean BMI of the normal control, but by the 1 year of age > 50% of patients had BMIs above that of the control. From ages of 5 to 13, 97% of patients had BMIs above the normal control. At the age of 16 years, 75% of patients had BMIs above the normal control (Fig. 10).


Growth charts for patients with Hunter syndrome ☆
BMI curve for patients with MPS II from birth to 18 years of age. The dotted line shows the mean BMI for healthy males. The solid line shows BMIs for patients with MPS II. Arrows point to their respective percentile curves.
© Copyright Policy - CC BY-NC-ND
Related In: Results  -  Collection

License
Show All Figures
getmorefigures.php?uid=PMC4060980&req=5

f0050: BMI curve for patients with MPS II from birth to 18 years of age. The dotted line shows the mean BMI for healthy males. The solid line shows BMIs for patients with MPS II. Arrows point to their respective percentile curves.
Mentions: The average BMI for newborn patients was 10.84 ± 3.30 kg/m2, and the BMI for patients at18 years and older was 29.41 ± 6.15 kg/m2. When compared with the normal control, 97% of patients had a BMI higher than the mean BMI of the normal control, but by the 1 year of age > 50% of patients had BMIs above that of the control. From ages of 5 to 13, 97% of patients had BMIs above the normal control. At the age of 16 years, 75% of patients had BMIs above the normal control (Fig. 10).

View Article: PubMed Central - PubMed

ABSTRACT

Children with mucopolysaccharidosis II (MPS II), also known as Hunter syndrome, an X-linked disorder, suffer from a multisystem dysfunction caused by the accumulation of glycosaminoglycans. However, there has been no systemic report on the growth of patients with MPS II. The purpose of this study is to describe the growth patterns of patients with MPS II and to compare with the patterns of age-matched controls. Data (height, weight, age, etc.) was collected in a longitudinal study of Japanese male patients with MPS II (n = 111). The mean birth length was 50.31 ± 1.42 cm, while the mean birth weight was 3.35 ± 0.39 kg. The mean final height and weight at 18 years and older were 125.63 ± 9.09 cm and 37.18 ± 8.72 kg; corresponding to a difference of − 46.40 cm and − 25.89 kg lower, when compared with healthy Japanese male controls. The mean birth BMI was 10.84 ± 3.29 kg/m2, while the mean BMI at 18 years was 29.41 ± 6.15 kg/m2. The growth pattern in patients with MPS II was characterized by overgrowth for the first several years, although growth velocity fell below that of the normal healthy controls after one year of age. No statistical difference in height was observed between patients with the attenuated and severe phenotypes in each age class.

In conclusion, this report describes the natural history of growth in patients with MPS II, which can help in monitoring the progression of the disease as well as assessing therapeutic efficacy.

No MeSH data available.


Related in: MedlinePlus