Moran Gotesman1,2, Guy Elgar2, Laura Hernandez Santiago3, Abigail Alvarez4, Youngju Pak2, Henry J Lin4,2, Joseph L Lasky5, Eduard H Panosyan4,2. 1. Division of Pediatric Hematology-Oncology, Department of Pediatrics, Harbor-UCLA Medical Center, Torrance, CA, U.S.A.; moran.gotesman@lundquist.org. 2. The Lundquist Institute for Biomedical Innovation, Torrance, CA, U.S A. 3. David Geffen School of Medicine, University of California Los Angeles, Los Angeles, CA, U.S.A. 4. Division of Pediatric Hematology-Oncology, Department of Pediatrics, Harbor-UCLA Medical Center, Torrance, CA, U.S.A. 5. Division of Pediatric Hematology-Oncology, Cure 4 The Kids Foundation, Las Vegas, NV, U.SA.
Abstract
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.
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.
Authors: Yutaka Niihara; Scott T Miller; Julie Kanter; Sophie Lanzkron; Wally R Smith; Lewis L Hsu; Victor R Gordeuk; Kusum Viswanathan; Sharada Sarnaik; Ifeyinwa Osunkwo; Edouard Guillaume; Swayam Sadanandan; Lance Sieger; Joseph L Lasky; Eduard H Panosyan; Osbourne A Blake; Tamara N New; Rita Bellevue; Lan T Tran; Rafael L Razon; Charles W Stark; Lynne D Neumayr; Elliott P Vichinsky Journal: N Engl J Med Date: 2018-07-19 Impact factor: 91.245
Authors: Sarah B Schuchard; Jennifer R Lissick; Amanda Nickel; David Watson; Kristin L Moquist; Rae M Blaylark; Stephen C Nelson Journal: Pediatr Blood Cancer Date: 2019-02-07 Impact factor: 3.167
Authors: Robert I Liem; Sophie Lanzkron; Thomas D Coates; Laura DeCastro; Ankit A Desai; Kenneth I Ataga; Robyn T Cohen; Johnson Haynes; Ifeyinwa Osunkwo; Jeffrey D Lebensburger; James P Lash; Theodore Wun; Madeleine Verhovsek; Elodie Ontala; Rae Blaylark; Fares Alahdab; Abdulrahman Katabi; Reem A Mustafa Journal: Blood Adv Date: 2019-12-10