IMPE2023 Poster Presentations Bone, Growth Plate and Mineral Metabolism (19 abstracts)
Serum alkaline phosphatase activity is correlated with the arm span to height ratio in the children with hypophosphatasia
Makoto Fujiwara 1,2 , Takeshi Ishimi 1 , Chieko Yamada 1 , Shinji Takeyari 1 , Kenichi Yamamoto 1 , Yukako Nakano 1 , Hirofumi Nakayama 1 , Yasuhisa Ohata 1 , Taichi Kitaoka 1 , Tomoyuki Akiyama 3 & Keiichi Ozono 1
1Department of Pediatrics, Osaka University Graduate School of Medicine, Suita, Japan. 2The First Department of Oral and Maxillofacial Surgery, Osaka University Graduate School of Dentistry, Suita, Japan. 3Department of Child Neurology, Okayama University Graduate School of Medicine, Dentistry and Pharmaceutical Sciences, Okayama, Japan
Hypophosphatasia (HPP) is a congenital skeletal disorder characterized by failure of bone calcification, which is caused by deficiency of the tissue non-specific alkaline phosphatase (TNSALP) enzyme. There is a remarkable range of severity in the phenotype, and some pediatric HPP patients show failure to thrive depending on their severity. In this study, we aim to elucidate the association between serum ALP activity and growth in HPP children. Twenty-six HPP patients (16 males, 10 females), followed at least 1 years in Osaka University Hospital from 2000 to 2021, were enrolled and retrospectively reviewed. All participants were aged under 15 years at the time of examination (mean age: 2.7 years) and the clinical classification was as follows: 1 perinatal severe, 5 perinatal benign, 3 infantile, 4 childhood, and 13 odonto type. The serum ALP activity was converted to ratio of the measured value to 2.5-percentile lower limit of normal values (ALP/LLN) or to median of normal values (ALP/MN) according to age and gender (reference data: Tanaka T et al. J Jpn Pediatr Soc, 2008). Patients’ heights and weights were converted to age- and sex-specific standard deviation score (SDS) on the basis of reference data from the Japanese Society for Pediatric Endocrinology. In the patients with non-odonto type, ALP/LLN and ALP/MN was significantly lower compared to that in odonto type patients. ALP/LLN and ALP/MN revealed significant positive correlation with arm span to height ratio (AHR) (r= 0.664, P= 0.01 and r=0.692, P= 0.01, respectively) but not with the height- and weight-SDS. Consistent to this result, pyridoxal 5'-phosphate to pyridoxal ratio (PLP/PL) showed significant negative correlation with ARH (r = -0.671, P= 0.03). In addition, the AHR measured during follow-up (after 6.1 years on average) was still correlated with the first measurement of ALP/LLN and PLP/PL, respectively. The predictive model of AHR determined by multiple regression analysis (R2 = 0.882, P= 0.01) consisted of several associated variables including ALP/MN, which was the strongest effector (standard β coefficient = 0.924, P= 0.01). These findings suggest that the AHR in HPP children reflect the impairment of ALP activity, which might be through the mechanism of the specific effect of TNSALP on the elongation of long bones.
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