Katinka Kuehn 1 , Andreas Hahn 1 , Lothar Seefried 2 Show Affiliations »
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BACKGROUND: Hypophosphatasia (HPP ) is a rare inherited metabolic disorder characterized by deficient activity of the tissue-nonspecific alkaline phosphatase entailing impaired turnover of phosphorus metabolites. Dietary mineral intake is suspected to influence clinical symptoms of HPP, but scientific evidence is missing. METHODS: Cross-sectional matched-pairs study collecting comprehensive data on nutrient intake in 20 HPP patients and 20 unaffected, age- and gender-matched controls . Dietary information and clinical symptoms were documented in detail over 7 consecutive days using structured diaries. RESULTS: Baseline data and type of energy-supplying nutrients were balanced between both groups. Median nutritional intake of phosphorus and calcium were significantly lower in HPP patients versus controls, which is partially attributable to lower energy consumption in HPP patients . Differences regarding phosphorus and calcium (Ca/P) ratio and uptake of magnesium, zinc, and vitamin B6 were not statistically significant. Both high (≥ 1375 mg/d) and low intakes (< 1100 mg/d) of phosphorus were significantly associated with an increased frequency of neuropsychiatric symptoms (P = 0.02). Similarly, very high and very low intake of calcium was significantly associated with musculoskeletal (P < 0.01), gastrointestinal (P = 0.02), and neuropsychiatric (P < 0.001) symptoms. An increased Ca/P ratio was associated with increased tiredness/fatigue (P < 0.01), whereas a decreased Ca/P was associated with gastrointestinal issues (P = 0.01). CONCLUSION: Phosphorus and calcium intake seem reduced in HPP patients along with reduced total energy consumption. Particularly high as well as very low absolute or unbalanced phosphorus and calcium intake are associated with an increased frequency of clinical symptoms. © Endocrine Society 2020. All rights reserved. For permissions, please e-mail: journals.permissions@oup.com.
RCT Entities: Population
Interventions
Outcomes
BACKGROUND: Hypophosphatasia (HP P ) is a rare inherited metabolic disorder characterized by deficient activity of the tissue-nonspecific alkaline phosphatase entailing impaired turnover of phosphorus metabolites. Dietary mineral intake is suspected to influence clinical symptoms of HP P , but scientific evidence is missing. METHODS: Cross-sectional matched-pairs study collecting comprehensive data on nutrient intake in 20 HP P patients and 20 unaffected, age- and gender-matched controls. Dietary information and clinical symptoms were documented in detail over 7 consecutive days using structured diaries. RESULTS: Baseline data and type of energy-supplying nutrients were balanced between both groups. Median nutritional intake of phosphorus and calcium were significantly lower in HP P patients versus controls, which is partially attributable to lower energy consumption in HP P patients . Differences regarding phosphorus and calcium (Ca/P ) ratio and uptake of magnesium, zinc, and vitamin B6 were not statistically significant. Both high (≥ 1375 mg /d) and low intakes (< 1100 mg /d) of phosphorus were significantly associated with an increased frequency of neuropsychiatric symptoms (P = 0.02). Similarly, very high and very low intake of calcium was significantly associated with musculoskeletal (P < 0.01), gastrointestinal (P = 0.02), and neuropsychiatric (P < 0.001) symptoms. An increased Ca/P ratio was associated with increased tiredness/fatigue (P < 0.01), whereas a decreased Ca/P was associated with gastrointestinal issues (P = 0.01). CONCLUSION: Phosphorus and calcium intake seem reduced in HP P patients along with reduced total energy consumption. P articularly high as well as very low absolute or unbalanced phosphorus and calcium intake are associated with an increased frequency of clinical symptoms. © Endocrine Society 2020. All rights reserved. For permissions, please e-mail: journals.permissions@oup.com.
Entities: Chemical
Disease
Gene
Species
Keywords:
calcium-phosphorus ratio; hypophosphatasia; nutritional intake; phosphorus intake
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Year: 2020
PMID： 32502243 DOI： 10.1210/clinem/dgaa324
Source DB: PubMed Journal: J Clin Endocrinol Metab ISSN： 0021-972X Impact factor: 5.958