title: Nutrient Insufficiencies/Deficiencies in Children With Sickle Cell Disease and Its Association With Increased Disease Severity.
abstract: BACKGROUND
  Sickle cell disease (SCD) is characteristically described as a disease of hemolytic anemia and vaso-occlusive crises (VOCs). However, patients suffer from a multitude of other problems including impaired development, chronic pain, and increased susceptibility to infection. Nutritional deficiency has been implicated as a contributor to these issues.
  
  
  METHODS
  We reported the nutrition status with respect to vitamin D, zinc, B6, B12, folate, and homocysteine serum levels in Canadian children with SCD (n = 91). We also tested for associations between nutrients and markers of disease severity and growth.
  
  
  RESULTS
  Almost half the sample (42%) had multiple nutrient insufficiencies/deficiencies, and a further 27% had a single insufficiency/deficiency. The most common insufficiency/deficiency was zinc in 57% followed by calcidiol (25 dihydroxyvitamin D (25(OH)D)) (52%). Sixteen percent of patients had low vitamin B6 levels, while folate, calcitriol (1,25(OH)D), and homocysteine levels were normal. Increased number of vitamin insufficiencies/deficiencies was associated with increasing disease severity (P = 0.018). Zinc insufficiency/deficiency was significantly associated with an increased number of home pain crises (P = 0.001) and an increased incidence of hospitalizations for VOCs (P = 0.01).
  
  
  CONCLUSIONS
  Our findings show that patients with SCD commonly have multiple nutrient insufficiencies/deficiencies and support the growing evidence for the link between low zinc and increased VOC. It also indicates that increased nutrient insufficiencies/deficiencies are associated with increased disease severity in SCD. Prospective studies with larger samples are needed to further elucidate the relationship between nutrient deficiencies and SCD, and to determine whether nutrient supplementation can improve the disease course.
authors: Faculty of Medicine, University of Ottawa, Ottawa, Ontario, Canada.
authors: David J
authors: DJ
authors: Martyres
authors: Faculty of Medicine, University of Toronto, Toronto, Ontario, Canada.
authors: Abi
authors: A
authors: Vijenthira
authors: Clinical Research Unit, Children's Hospital of Eastern Ontario, Ottawa, Ontario, Canada.
authors: Nick
authors: N
authors: Barrowman
authors: Division of Hematology/Oncology, Children's Hospital of Eastern Ontario, Ottawa, Ontario, Canada.
authors: Sydney
authors: S
authors: Harris-Janz
authors: Division of Hematology/Oncology, Children's Hospital of Eastern Ontario, Ottawa, Ontario, Canada.
authors: Christine
authors: C
authors: Chretien
authors: Division of Hematology/Oncology, Children's Hospital of Eastern Ontario, Ottawa, Ontario, Canada.
authors: Robert J
authors: RJ
authors: Klaassen
mesh_terms: D000755:Anemia, Sickle Cell / Q000150:complications*; D002170:Canada / Q000453:epidemiology; D002648:Child; D002675:Child, Preschool; D005260:Female; D006801:Humans; D008297:Male; D044342:Malnutrition / Q000150:complications* / Q000453:epidemiology*; D009752:Nutritional Status; D012189:Retrospective Studies
keywords: nutrient deficiency; pain; sickle cell disease; vitamin d; zinc