I got this on listserve. Finally, a PKU Elder post, it's a scary thought; osteoporosis and i see it everyday living in the independent area of a nursing home. I would guess it's due to lack of calcium in foods. Dairy foods that aren't allowed:
This topic is
clearly a huge concern for every PKU patient and the families that love and care
for them. For those of you that know me personally, know that I will continue to
learn all I can about the implications of PKU and challenge myself and my
company to do whatever it takes to optimize appropriate nutrition interventions.
Below is some info and a few links that may be of interest.
Nutritional
Management of Phenylketonuria
Osteopenia
Long-term complications of low bone mass
(i.e. osteopenia, osteoporosis and fractures) are becoming evident in the
growing population of adolescents and adults with PKU. Studies of young adults
with PKU found low peak bone mass in more than 40% of subjects [37,38]. The etiology of bone complications in PKU remains unclear and
may reflect the PKU genotype and/or the composition of the AA-based diet, in
particular reduced absorption and utilization of calcium phosphorus and vitamin
D. For genotype effects, the bone cells (osteoblasts and osteoclasts) or other
tissues involved in calcium uptake and vitamin D metabolism (e.g. kidney and
intestines) may be sensitive to elevated phe levels, just as brain cells are. In
support of this, evidence from the PKU mouse model (PAHenu2) suggests
that increased circulating phe concentrations adversely affect bone development
[39]. The current diet may provide an excess acid load [31] and a less than optimal supply of AA needed to maintain
adequate protein synthesis and collagen turnover for formation of bone matrix
[40]. Studies in humans with PKU are needed to investigate the
complication of low bone mass in a longitudinal manner with careful assessment
of dietary intake.
The links below, though
dense provide published papers on low bone strength in the PKU mice models. I
believe they provide some useful information for my adult PKU
children.
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