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Resting Energy Expenditure and Substrate Oxidation in Malnourished Patients With Type 1 Glycogenosis.
J Clin Endocrinol Metab. 2019 11 01; 104(11):5566-5572.JC

Abstract

CONTEXT

Type 1a and 1b glycogenosis [glycogen storage disorder (GSD)1a, GSD1b] are rare diseases generally associated with malnutrition. Although abnormal substrate oxidation rates and elevated energy expenditures might contribute to malnutrition, this issue has not been investigated.

OBJECTIVE

To investigate whether abnormal resting energy expenditure (REE) and substrate oxidation rate characterize patients with GSD1.

DESIGN

Cross-sectional study.

SETTING

Outpatient referral center for rare diseases and laboratory of clinical nutrition at the University Hospital of Palermo.

PATIENTS

Five consecutive patients with GSD1 (4 type a, 1 type b; 3 men, 2 women; age range, 19 to 49 years).

MAIN OUTCOME MEASURES

The usual clinical procedures for patients with malnutrition, including REE and basal substrate oxidation rate (both indirect calorimetry), body composition (bioimpedance method), muscle strength (hand-grip test), and the usual laboratory tests, were performed.

RESULTS

Malnutrition was clearly diagnosed in 2 patients (1 GSD1a and 1 GSD1b), with REE elevated in all five patients, and especially, in the two malnourished patients (+124% and +32.1% vs predictive values using Harris-Benedict equations). The two malnourished patients also exhibited lower basal protein oxidation rates (7.7% and 6.6%) than the nourished patients (range, 12.1% to 24.7%), with higher carbohydrate or lipid oxidation rates. Additionally, the two malnourished patients exhibited higher blood concentrations of lactic acid than the nourished patients.

CONCLUSIONS

According to data obtained from our small sample of patients with GSD1, elevated REEs seem to be a common characteristic that might contribute to malnutrition. Low basal protein oxidation rates and elevated blood lactic acid concentrations appear to be associated with malnutrition.

Authors+Show Affiliations

Unit of Malattie Endocrine, del Ricambio e della Nutrizione - Laboratorio di Metabolismo e Nutrizione Clinica, Dipartimento di Promozione della Salute, Materno-Infantile, Medicina Interna e Specialistica di Eccellenza, University of Palermo, Palermo, Italy.Unit of Astanteria/MCAU - Centro di Riferimento Regionale per le Malattie Rare del Metabolismo, Dipartimento di Promozione della Salute, Materno-Infantile, Medicina Interna e Specialistica di Eccellenza, University of Palermo, Palermo, Italy.Unit of Malattie Endocrine, del Ricambio e della Nutrizione - Laboratorio di Metabolismo e Nutrizione Clinica, Dipartimento di Promozione della Salute, Materno-Infantile, Medicina Interna e Specialistica di Eccellenza, University of Palermo, Palermo, Italy.Unit of Malattie Endocrine, del Ricambio e della Nutrizione - Laboratorio di Metabolismo e Nutrizione Clinica, Dipartimento di Promozione della Salute, Materno-Infantile, Medicina Interna e Specialistica di Eccellenza, University of Palermo, Palermo, Italy.Unit of Malattie Endocrine, del Ricambio e della Nutrizione - Laboratorio di Metabolismo e Nutrizione Clinica, Dipartimento di Promozione della Salute, Materno-Infantile, Medicina Interna e Specialistica di Eccellenza, University of Palermo, Palermo, Italy.Unit of Malattie Endocrine, del Ricambio e della Nutrizione - Laboratorio di Metabolismo e Nutrizione Clinica, Dipartimento di Promozione della Salute, Materno-Infantile, Medicina Interna e Specialistica di Eccellenza, University of Palermo, Palermo, Italy.Unit of Astanteria/MCAU - Centro di Riferimento Regionale per le Malattie Rare del Metabolismo, Dipartimento di Promozione della Salute, Materno-Infantile, Medicina Interna e Specialistica di Eccellenza, University of Palermo, Palermo, Italy.Unit of Astanteria/MCAU - Centro di Riferimento Regionale per le Malattie Rare del Metabolismo, Dipartimento di Promozione della Salute, Materno-Infantile, Medicina Interna e Specialistica di Eccellenza, University of Palermo, Palermo, Italy.

Pub Type(s)

Journal Article

Language

eng

PubMed ID

31322653

Citation

Buscemi, Silvio, et al. "Resting Energy Expenditure and Substrate Oxidation in Malnourished Patients With Type 1 Glycogenosis." The Journal of Clinical Endocrinology and Metabolism, vol. 104, no. 11, 2019, pp. 5566-5572.
Buscemi S, Noto D, Buscemi C, et al. Resting Energy Expenditure and Substrate Oxidation in Malnourished Patients With Type 1 Glycogenosis. J Clin Endocrinol Metab. 2019;104(11):5566-5572.
Buscemi, S., Noto, D., Buscemi, C., Barile, A. M., Rosafio, G., Settipani, V., Giammanco, A., & Averna, M. (2019). Resting Energy Expenditure and Substrate Oxidation in Malnourished Patients With Type 1 Glycogenosis. The Journal of Clinical Endocrinology and Metabolism, 104(11), 5566-5572. https://doi.org/10.1210/jc.2019-00585
Buscemi S, et al. Resting Energy Expenditure and Substrate Oxidation in Malnourished Patients With Type 1 Glycogenosis. J Clin Endocrinol Metab. 2019 11 1;104(11):5566-5572. PubMed PMID: 31322653.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - Resting Energy Expenditure and Substrate Oxidation in Malnourished Patients With Type 1 Glycogenosis. AU - Buscemi,Silvio, AU - Noto,Davide, AU - Buscemi,Carola, AU - Barile,Anna Maria, AU - Rosafio,Giuseppe, AU - Settipani,Valentina, AU - Giammanco,Antonina, AU - Averna,Maurizio, PY - 2019/03/12/received PY - 2019/07/15/accepted PY - 2019/7/20/pubmed PY - 2020/6/4/medline PY - 2019/7/20/entrez SP - 5566 EP - 5572 JF - The Journal of clinical endocrinology and metabolism JO - J. Clin. Endocrinol. Metab. VL - 104 IS - 11 N2 - CONTEXT: Type 1a and 1b glycogenosis [glycogen storage disorder (GSD)1a, GSD1b] are rare diseases generally associated with malnutrition. Although abnormal substrate oxidation rates and elevated energy expenditures might contribute to malnutrition, this issue has not been investigated. OBJECTIVE: To investigate whether abnormal resting energy expenditure (REE) and substrate oxidation rate characterize patients with GSD1. DESIGN: Cross-sectional study. SETTING: Outpatient referral center for rare diseases and laboratory of clinical nutrition at the University Hospital of Palermo. PATIENTS: Five consecutive patients with GSD1 (4 type a, 1 type b; 3 men, 2 women; age range, 19 to 49 years). MAIN OUTCOME MEASURES: The usual clinical procedures for patients with malnutrition, including REE and basal substrate oxidation rate (both indirect calorimetry), body composition (bioimpedance method), muscle strength (hand-grip test), and the usual laboratory tests, were performed. RESULTS: Malnutrition was clearly diagnosed in 2 patients (1 GSD1a and 1 GSD1b), with REE elevated in all five patients, and especially, in the two malnourished patients (+124% and +32.1% vs predictive values using Harris-Benedict equations). The two malnourished patients also exhibited lower basal protein oxidation rates (7.7% and 6.6%) than the nourished patients (range, 12.1% to 24.7%), with higher carbohydrate or lipid oxidation rates. Additionally, the two malnourished patients exhibited higher blood concentrations of lactic acid than the nourished patients. CONCLUSIONS: According to data obtained from our small sample of patients with GSD1, elevated REEs seem to be a common characteristic that might contribute to malnutrition. Low basal protein oxidation rates and elevated blood lactic acid concentrations appear to be associated with malnutrition. SN - 1945-7197 UR - https://www.unboundmedicine.com/medline/citation/31322653/Resting_energy_expenditure_and_substrate_oxidation_in_malnourished_patients_with_type_1_glycogenosis L2 - https://academic.oup.com/jcem/article-lookup/doi/10.1210/jc.2019-00585 DB - PRIME DP - Unbound Medicine ER -