Mineral Intake and Clinical Symptoms in Adult Patients with Hypophosphatasia

verfasst von

Katinka Kühn, Andreas Hahn, Lothar Seefried

Abstract

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.

Organisationseinheit(en)

Institut für Lebensmittelwissenschaft und Humanernährung
Abteilung Ernährungsphysiologie und Humanernährung

Externe Organisation(en)

Julius-Maximilians-Universität Würzburg

Typ

Artikel

Journal

Journal of Clinical Endocrinology and Metabolism

Band

105

Seiten

E2982-E2992

ISSN

0021-972X

Publikationsdatum

08.2020

Publikationsstatus

Veröffentlicht

Peer-reviewed

Ja

ASJC Scopus Sachgebiete

Endokrinologie, Diabetes und Stoffwechsel, Biochemie, Endokrinologie, Klinische Biochemie, Biochemie, medizinische

Ziele für nachhaltige Entwicklung

SDG 3 – Gute Gesundheit und Wohlergehen, SDG 7 – Erschwingliche und saubere Energie

Elektronische Version(en)

https://doi.org/10.1210/clinem/dgaa324 (Zugang: Geschlossen)