Pediatric Patients With Sickle Cell Disease at a Public Hospital: Nutrition, Compliance and Early Experience With L-Glutamine Therapy.

Autor:	Gotesman M; Division of Pediatric Hematology-Oncology, Department of Pediatrics, Harbor-UCLA Medical Center, Torrance, CA, U.S.A.; moran.gotesman@lundquist.org.; The Lundquist Institute for Biomedical Innovation, Torrance, CA, U.S A., Elgar G; The Lundquist Institute for Biomedical Innovation, Torrance, CA, U.S A., Santiago LH; David Geffen School of Medicine, University of California Los Angeles, Los Angeles, CA, U.S.A., Alvarez A; Division of Pediatric Hematology-Oncology, Department of Pediatrics, Harbor-UCLA Medical Center, Torrance, CA, U.S.A., Pak Y; The Lundquist Institute for Biomedical Innovation, Torrance, CA, U.S A., Lin HJ; Division of Pediatric Hematology-Oncology, Department of Pediatrics, Harbor-UCLA Medical Center, Torrance, CA, U.S.A.; The Lundquist Institute for Biomedical Innovation, Torrance, CA, U.S A., Lasky JL; Division of Pediatric Hematology-Oncology, Cure 4 The Kids Foundation, Las Vegas, NV, U.SA., Panosyan EH; Division of Pediatric Hematology-Oncology, Department of Pediatrics, Harbor-UCLA Medical Center, Torrance, CA, U.S.A.; The Lundquist Institute for Biomedical Innovation, Torrance, CA, U.S A.
Jazyk:	angličtina
Zdroj:	In vivo (Athens, Greece) [In Vivo] 2022 Jul-Aug; Vol. 36 (4), pp. 1761-1768.
DOI:	10.21873/invivo.12889
Abstrakt:	Background/aim: Hydration and hydroxyurea (HU) can modify sickle cell disease (SCD) severity. Optimal nutrition and L-glutamine (Gln) may provide further amelioration. Patients and Methods: Reviews of medical records and nutrition surveys were used to investigate severity of pediatric patients with SCD in relation to nutrition, growth, hematologic parameters, and diseasemodifying agents. Results: Among 25 females and 25 males (9.1±7 years), beta-globin genotypes were: HbSS/Sβ°, 60%; HbSC, 32%; HbSβ + , 8%. The mean number of annual pain crises (APC) was 0.97±1.1. APCs increased ≥2-fold as HbF dropped to <10% with age. Proper hydration and nutrition correlated with younger ages and fewer APCs. Height and weight Z-scores were ≤-1SD in 20% of 35 surveyed patients (12±7.8 years), who had more APCs (2.5±2.5 vs. 1±1.3, p=0.03). Prealbumin levels were overall low. Twenty-two of 28 patients on HU reported ≥90% adherence - with higher mean corpuscular volume (92±9.6 vs. 74±10 f/l, p<0.01). Seventy percent of Gln prescriptions were filled. Compliance over 23 months was ≥70% in 12 patients, including 2 on chronic transfusion. Of 10 evaluable patients, 6 (8.8±2.2 years) had fewer APCs with Gln (mean 0.2 vs. 0.9, p=0.016), with increasing prealbumin levels (14.1 to 15.8 mg/dl, p=0.1). Conclusion: Younger, and well-nourished, well-hydrated patients have a milder clinic course. Disease severity was the worse in undernourished teenagers with suboptimal compliance. L-Glutamine with prealbumin monitoring should be considered for further evaluation in pediatric SCD. (Copyright © 2022, International Institute of Anticancer Research (Dr. George J. Delinasios), All rights reserved.)
Databáze:	MEDLINE
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