Standard growth curves for Japanese patients with Prader-Willi syndrome

Abstract

We constructed the standard growth (length/height and weight) curves for Japa-nese individuals with Prader-Willi syndrome (PWS). Crude height and weight data were collected from 153 males and 99 females with the syndrome, and the collected data were arranged by a mathematical method to construct the curves. Height growth patterns were quite different between PWS and normal children. Mean height of individuals with the syndrome by puberty is −2 SD for normal children, and it drops off far below −2 SD value after puberty. Final mean height is 141.2 ± 4.8 cm for females (n = 13) and 147.7 ± 7.7 cm for males (n = 17), showing 15.8 and 21.9 cm below the average height for normal Japanese girls and boys, respectively. Thus, the degree of shortness is more pronounced in male than in female patients. There was no difference in height between those with chromosome 15q deletion and those without. Mean weight at birth of girls (n = 88) and boys (n = 131) were 2.70 ± 0.45 Kg and 2.62 ± 0.47 Kg, respectively. These values were smaller than those for normal neonates (P < 0.05, t-test). The weight of PWS children was under the mean value for normal infants by age 2 years, but gradually increase above the mean values for normal children around ages 2-4 years. Overweight in both males and females becomes obvious during prepu-berty. Growth patterns are not different between Japanese and Caucasian children with the syndrome. Short stature is more prominent in boys of both ethnic groups, whereas the degree of overweight appears much more severe in Caucasians. Am. J. Med. Genet. 95:130-134, 2000.

Prader‐Willi syndrome: is there a recognizable fetal phenotype?

“…This growth pattern is also found after birth in European PWS neonates who have a low average weight (2945 ± 570 g in boys and 2782 ± 594 g in girls), contrasting with normal average length (50.2 ± 2.8 cm in boys and 48.9 ± 3.3 cm in girls) (Wollmann et al, 1998). Similar neonatal growth values have been reported for Japanese patients with PWS (Nagai et al, 2000). Hypogonadism in male fetuses is an additional nonspecific feature compatible with the diagnosis of PWS (Le Bris-Quillevere et al, 1990;L'Hermine et al, 2003).…”

Growth references for Japanese individuals with Noonan syndrome

“…At present, there are disease-specific growth charts for several syndromes, such as Prader-Willi syndrome, Down syndrome, Williams syndrome, and Turner syndrome (14)(15)(16)(17)(18). These charts are generally used in the clinical setting for monitoring the growth and development of patients with these syndromes and allow the identification of those with severe growth deficit who may need additional medications.…”

Clinical management of behavioral characteristics of Prader&ndash;Willi syndrome

“…During adolescence, the growth rate declines due in part to inefficient testosterone and estrogen levels, resulting in the absence of the pubertal growth spurt. 9,108 Long-term GH therapy in patients with PWS has been shown to increase: height velocity and final adult height potential, lean body mass, muscle strength, and level of activity, and to decrease fat mass. [109][110][111][112][113][114] As a result of decreased fat mass and increased lean body mass, GH treatment has also shown to improve pulmonary function in children with PWS.…”