Relationship between Biochemical Bone Markers and Bone Mineral Density in Patients with Phenylketonuria under Restricted Diet

Sherif  M. Zaki; Hala  M. Koura; Nagwa  A. Ismail; Emad  E. Salama; Dalia H. El Lebedy H. El Lebedy; Laila  K. Effat

Innovative Journal of Pediatrics

The Scientific Journal of Growth & Development Research Center

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Relationship between Biochemical Bone Markers and Bone Mineral Density in Patients with Phenylketonuria under Restricted Diet

Author(s):

Sherif M. Zaki^1,*,

Hala M. Koura²,

Nagwa A. Ismail³,

Emad E. Salama⁴,

Dalia H. El Lebedy H. El Lebedy⁵,

Laila K. Effat⁶

Innovative Journal of Pediatrics:Vol. 24, issue 1; 23-28

Published online:Dec 31, 2013

Article type:Research Article

Received:Jan 18, 2013

Accepted:Nov 16, 2013

How to Cite:M. Zaki S, M. Koura H, A. Ismail N, E. Salama E, H. El Lebedy DHEL, et al. Relationship between Biochemical Bone Markers and Bone Mineral Density in Patients with Phenylketonuria under Restricted Diet. Inn J Pediatr. 2014;24(1):. doi:

Abstract

Objective: Most of phenylketonuria (PKU) develops bone turnover impairment and low bone mineral density (BMD). Measurements of BMD reï¬ect only bone mineral status but not the dynamics of bone turnover. Bone markers are a noninvasive tool useful for the assessment of bone formation and bone resorption processes. Our study was to assess the levels of bone markers in PKU in order to select a screen marker and detect the most specific marker which can be combined with BMD for appropriate follow up. Methods: Thirty three classic PKU patients were studied. BMD and bone mineral content (BMC) were measured. Total alkaline phosphatase (ALP), osteocalcin (OC) and carboxy-terminal propeptide of type I collagen (CICP), osteoprotegerin (OPG), receptor activator of nuclear factor κβ ligand (RANKL) and Deoxypyridinoline (DPD) were measured. Findings: Nineteen (57.6%) male and fourteen (42.4 %) female PKU patients were involved in the current study. Their mean age was 8.4±4.6 yrs and the age range 3-19 yrs. The control group consisted of twenty two (52.4%) males and twenty (47.6%) females. Their mean age was 8.5±3.3 yrs and th age range 2-17 yrs. Using the Z score values, there was a significant decrease of total BMC (TBMC-Z), BMD of the femoral neck BMD-FN-Z, BMD of lumbar vertebrae (BMD-L-Z), BMD-FN and DPD while RANKL increased. There was a negative correlation between CICP and TBMC and between CICP and BMD-L in these patients. Also, a negative correlation between ALP and TBMC and between ALP and BMD-L was observed. It was concluded that the ALP provides a good impression of the new bone formation in the PKU patients and it has a highly significant negative correlation with the many parameters of the bone mineral status beside the wide availability of inexpensive and simple methods. So a screening test and/or follow up for the PKU patients using ALP would be available. Once the level of ALP decrease is detected, one can combine it with BMD to explore the bone mineral status and with specific bone markers (OC, RANKL and DBD), to verify the dynamics of bone turnover. Conclusion: This schedule will reduce the risk of exposure of these patients to the risk hazards of DXA and limit its use only to a limited number of the highly suspected cases.

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Body Composition Profile of Young Patients With Phenylketonuria and Mild Hyperphenylalaninemia

Artemis Doulgeraki,

Astrinia Skarpalezou,

Areti Theodosiadou,

Ioannis Monopolis,

Kleopatra Schulpis

Doulgeraki A, Skarpalezou A, Theodosiadou A, Monopolis I, Schulpis K. Body Composition Profile of Young Patients With Phenylketonuria and Mild Hyperphenylalaninemia. Int J Endocrinol Metab. 2014;12(3):e16061. doi: https://doi.org/10.5812/ijem.16061

17

May

2023

Prevalence of Overweight and Obesity in Children Diagnosed with Phenylketonuria

Hoda Atef Abdelsattar Ibrahim,

Gihan Fouad Ahmed,

Khaled Mohamed Abdelhamid ElKhashab,

Aya Ahmed Amin,

Aya Farag Attia Elsebaey,

Enas Sayed Abbas

Atef Abdelsattar Ibrahim H, Fouad Ahmed G, Mohamed Abdelhamid ElKhashab K, Amin AA, Farag Attia Elsebaey A, et al. Prevalence of Overweight and Obesity in Children Diagnosed with Phenylketonuria. J Compr Ped. 2023;14(3):e136499. doi: https://doi.org/10.5812/compreped-136499

23

Dec

2015

Intellectual and Developmental Status in Children With Hyperphenylalaninemia and PKU Who Were Screened in a National Program

Javad Mahmoudi-Gharaei,

Parisa Aghasi,

Arya Setoodeh,

Azadeh Sayarifard,

Maryam Rashidiyan,

Fatemeh Sayarifard

,et al.

Mahmoudi-Gharaei J, Aghasi P, Setoodeh A, Sayarifard A, Rashidiyan M, et al. Intellectual and Developmental Status in Children With Hyperphenylalaninemia and PKU Who Were Screened in a National Program. Inn J Pediatr. 2015;25(6):e3033. doi: https://doi.org/10.5812/ijp.3033

15

Nov

2022

An Asymptomatic Patient of Phenylketonuria: A Case Report of 2 Siblings

Shahin Koohmanaee,

Afagh Hassanzadeh Rad,

Amir Mohammad Ghanbari,

Seyede Tahoura Hakemzadeh,

Reza Bayat,

Setila Dalili

Koohmanaee S, Hassanzadeh Rad A, Ghanbari AM, Hakemzadeh ST, Bayat R, et al. An Asymptomatic Patient of Phenylketonuria: A Case Report of 2 Siblings. Arch Neurosci. 2022;9(4):e132081. doi: https://doi.org/10.5812/ans-132081

18

Nov

2017

Microsomal Liver Function in Patients with Phenylketonuria Assessed by 13C-Methacetin Breath Test

Renata Mozrzymas,

Monika Dus-Zuchowska,

Lukasz Kaluzny,

Anna Miskiewicz-Chotnicka,

Aleksandra Lisowska,

Jaroslaw Walkowiak

Mozrzymas R, Dus-Zuchowska M, Kaluzny L, Miskiewicz-Chotnicka A, Lisowska A, et al. Microsomal Liver Function in Patients with Phenylketonuria Assessed by 13C-Methacetin Breath Test. Inn J Pediatr. 2017;27(6):e12535. doi: https://doi.org/10.5812/ijp.12535

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Author(s):

Sherif M. Zaki:[PubMed][Scholar]
Hala M. Koura:[PubMed][Scholar]
Nagwa A. Ismail:[PubMed][Scholar]
Emad E. Salama:[PubMed][Scholar]
Dalia H. El Lebedy H. El Lebedy:[PubMed][Scholar]
Laila K. Effat:[PubMed][Scholar]

The Scientific Journal of Growth & Development Research Center

Outlines

Relationship between Biochemical Bone Markers and Bone Mineral Density in Patients with Phenylketonuria under Restricted Diet

Abstract

Fulltext

References

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