title: Impairment of bone health in pediatric patients with hemolytic anemia.
abstract: BACKGROUND
  Sickle cell anemia and thalassemia result in impaired bone health in both adults and youths. Children with other types of chronic hemolytic anemia may also display impaired bone health.
  
  
  METHODS
  To assess bone health in pediatric patients with chronic hemolytic anemia, a cross-sectional study was conducted involving 45 patients with different forms of hemolytic anemia (i.e., 17 homozygous sickle cell disease and 14 hereditary spherocytosis patients). Biochemical, radiographic and anamnestic parameters of bone health were assessed.
  
  
  RESULTS
  Vitamin D deficiency with 25 OH-vitamin D serum levels below 20 ng/ml was a common finding (80.5%) in this cohort. Bone pain was present in 31% of patients. Analysis of RANKL, osteoprotegerin (OPG) and osteocalcin levels indicated an alteration in bone modeling with significantly elevated RANKL/OPG ratios (control: 0.08+0.07; patients: 0.26+0.2, P = 0.0007). Osteocalcin levels were found to be lower in patients compared with healthy controls (68.5+39.0 ng/ml vs. 118.0+36.6 ng/ml, P = 0.0001). Multiple stepwise regression analysis revealed a significant (P<0.025) influence of LDH (partial r2 = 0.29), diagnosis of hemolytic anemia (partial r2 = 0.05) and age (partial r2 = 0.03) on osteocalcin levels. Patients with homozygous sickle cell anemia were more frequently and more severely affected by impaired bone health than patients with hereditary spherocytosis.
  
  
  CONCLUSIONS
  Bone health is impaired in pediatric patients with hemolytic anemia. In addition to endocrine alterations, an imbalance in the RANKL/OPG system and low levels of osteocalcin may contribute to this impairment.
authors.affiliation: Department of Pediatric Hematology and Oncology, Kinderklinik III, Universitätsklinikum-Essen and the University of Duisburg-Essen, Essen, Germany.
authors.forename: Michael M
authors.initials: MM
authors.lastname: Schündeln
authors.affiliation: Department of Pediatric Hematology and Oncology, Kinderklinik III, Universitätsklinikum-Essen and the University of Duisburg-Essen, Essen, Germany.
authors.forename: Sarah C
authors.initials: SC
authors.lastname: Goretzki
authors.affiliation: Department of Pediatric Hematology and Oncology, Kinderklinik III, Universitätsklinikum-Essen and the University of Duisburg-Essen, Essen, Germany.
authors.forename: Pia K
authors.initials: PK
authors.lastname: Hauffa
authors.affiliation: Department of Pediatric Hematology and Oncology, Kinderklinik III, Universitätsklinikum-Essen and the University of Duisburg-Essen, Essen, Germany.
authors.forename: Regina
authors.initials: R
authors.lastname: Wieland
authors.affiliation: Department of Pediatric Endocrinology and Diabetology, Kinderklinik II, Universitätsklinikum-Essen and the University of Duisburg-Essen, Essen, Germany.
authors.forename: Jens
authors.initials: J
authors.lastname: Bauer
authors.affiliation: Department of Pediatric Endocrinology and Diabetology, Kinderklinik II, Universitätsklinikum-Essen and the University of Duisburg-Essen, Essen, Germany.
authors.forename: Lena
authors.initials: L
authors.lastname: Baeder
authors.affiliation: Department of Pediatrics, Division of Oncology and Hematology, Charité - Universitätsmedizin, Berlin, Germany.
authors.forename: Angelika
authors.initials: A
authors.lastname: Eggert
authors.affiliation: Department of Pediatric Endocrinology and Diabetology, Kinderklinik II, Universitätsklinikum-Essen and the University of Duisburg-Essen, Essen, Germany.
authors.forename: Berthold P
authors.initials: BP
authors.lastname: Hauffa
authors.affiliation: Department of Pediatric Endocrinology and Diabetology, Kinderklinik II, Universitätsklinikum-Essen and the University of Duisburg-Essen, Essen, Germany.
authors.forename: Corinna
authors.initials: C
authors.lastname: Grasemann
mesh_terms: D000293:Adolescent; D000743:Anemia, Hemolytic / Q000097:blood* / Q000503:physiopathology*; D015519:Bone Density / Q000502:physiology; D001842:Bone and Bones / Q000503:physiopathology*; D016022:Case-Control Studies; D002648:Child; D002675:Child, Preschool; D003430:Cross-Sectional Studies; D005260:Female; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D008297:Male; D015675:Osteocalcin / Q000097:blood; D053244:Osteoprotegerin / Q000097:blood; D053245:RANK Ligand / Q000097:blood
chemical_list: D053244:Osteoprotegerin; D053245:RANK Ligand; D015675:Osteocalcin