Bone mineral density, growth, pubertal development and other parameters in Brazilian children and young adults with sickle cell anaemia

Abstract

Abstractobjectives To evaluate the occurrence of low bone mineral density (BMD) and its relationship with clinical and laboratorial characteristics in children and young adults with sickle cell anaemia living in Northeast-Brazil, and to assess the role of radiography in diagnosing low BMD.methods Bone mineral density of lumbar spine was measured by dual energy X-ray absorptiometry (DXA) in 27 patients with Sickle cell anaemia (SCA) aged 7-28 years. Clinical history, calcium and calorie intake, laboratory measurements, anthropometrics and pubertal development were assessed, and X-rays were obtained. Z-scores and T-scores for weight, height, Body Mass Index (BMI) and BMD were calculated using age and gender matched reference data.results Mean lumbar spine BMD Z-scores and T-scores were À1.81 SD in boys and À0.80 SD in girls. BMD Z-scores were below -2 SD in 33.3% of girls and in 46.7% of boys. Low BMD (<À2 SD) occurred significantly more in patients with low height-for-age (P = 0.02), low weight-for-age (P = 0.001) and low BMI-for-age (P = 0.006). No significant relationships were found between BMD and other clinical and laboratory parameters. Radiography had a sensitivity of 75% and a specificity of 36% to detect low BMD, and was considered not useful in this context.conclusions Patients with low height and/or low weight-for-age seem to be at high risk for developing low BMD.

Prevalence and correlates of growth failure in young African patients with sickle cell disease

“…Growth failure in SCD is also believed to be induced by higher energy expenditure (Barden et al, 2000;Buchowski et al, 2002;Rhodes et al, 2009) and hypermetabolic state resulting from chronic haemolysis. This hypothesis is consistent with the correlation we observed between growth failure, anaemia and haemolysis markers, and may explain the difference between the SS-Sb 0 and SC-Sb + phenotypes, which display lower (Meeuwes et al, 2013) levels of haemolysis (Dubert et al, 2017). Furthermore, the high frequency of growth failure we observed in African children with SCD contrasts with the most recent studies on growth status of children with SCD in high-income countries.…”

Osteoporosis and Vitamin D Deficiency in Patients with Sickle Cell Disease

“…In another study on 17 SCA patients, osteopenia frequency was 47% and serum iron and ferritin levels were found higher in osteopenic patients than patients with normal BMD [13]. In the study of M. Meeuwes et al [14] on 27 patients ranging in age from 7-28, low BMD was not correlated with age, gender, calcium, calorie intake, or laboratory measurements. Low BMD was present in 11 patients (4 females, 7 males).…”

Response to low bone mineral areal density in patients with sickle cell anaemia (SCA) and short stature should be interpreted with caution

“…We thank Drs. Spinola-Castro and Siviero-Miachon for their comments on our paper (Meeuwes et al 2013) and present the following considerations/responses:…”