Markers of bone turnover are associated with growth and development in young subjects with sickle cell anemia

Fung, Ellen B.; Kawchak, Deborah A.; Zemel, Babette S.; Rovner, Alisha J.; Ohene‐Frempong, Kwaku; Stallings, Virginia A.

doi:10.1002/pbc.21147

Cited by 28 publications

(28 citation statements)

References 16 publications

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“…In a study of 32 adults with SCD (mean age of 34 years), 72% had low BMD at 1 or more anatomic sites and 40% were classified as osteoporotic [33]. Markers of bone formation are elevated whereas bone resorption is decreased in children with SCD compared with healthy children [34]. Additional mechanisms and risk factors for osteopenia in SCD include delayed puberty and low accrual of peak bone mass, bone microinfarcts resulting from repeated sickle crises, chronic illness with immobilization, and calcium, vitamin D, and other nutritional deficiencies [35,36].…”

Section: Discussionmentioning

confidence: 99%

Frequency and Risk Factors of Endocrine Complications in Turkish Children and Adolescents with Sickle Cell Anemia

Özen¹,

Ünal

Erçetin³

et al. 2013

tjh

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Objective: To define frequency and risk factors of abnormalities in growth, puberty, thyroid function, and bone and carbohydrate metabolisms in children and adolescents with sickle cell disease (SCD).Materials and Methods: Endocrine problems including short stature, puberty and thyroid disorders, and carbohydrate and bone metabolisms in 50 Turkish children and adolescents with SCD were evaluated. Relationships among sex, disease type, blood transfusions, exchange and exacerbation frequency, ferritin levels, and endocrine pathologies were investigated.Results: The mean age of the study group was 13.1±2.9 years. Weights and heights of 12 participants (24%) were below -2 standard deviations and 4 participants (8%) had malnutrition. Mean difference (±standard deviation) between bone and chronological age of patients was -1.73±1.86 years. Fifty percent of patients had at least one endocrine abnormality other than vitamin D deficiency and insufficiency. Hypergonadotropic hypogonadism in 3 patients (6%), hypogonadotropic hypogonadism in 1 female patient (2%), and small testicular volume in respect to age in 3 male patients (8.5%) were seen. Growth hormone deficiency was detected in 1 (2%) female patient, and hypothyroidism was diagnosed in 3 patients (6%; 1 central case, 2 cases of primary hypothyroidism). At vertebral level, 5 patients (11.1%) had osteopenia and 1 patient (2.2%) had osteoporosis, while 5 patients (11.1%) had osteopenia at femur neck level. The most common endocrine abnormality was vitamin D deficiency. 25-Hydroxyvitamin D was deficient in 63.2% and insufficient in 18.4% of patients. Sex, disease type, blood transfusion frequency, exacerbation frequency, and ferritin levels were not related to endocrine pathologies. As the age was increased, standard deviation scores of femur neck bone mineral density was decreased (r =-0.56; p<0.05). Vitamin D was lower in patients whose weights and/or heights were below -2 standard deviations from the mean (p<0.05).Conclusion: Endocrine organ dysfunctions are commonly detected in children and adolescents with SCD, and vitamin D deficiency is the most commonly encountered endocrine disorder. Regular follow- ups of patients for endocrine complications, starting from early ages of patients, and initiation of appropriate treatments will elongate expectancy and quality of life.Conflict of interest:None declared.

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Section: Discussionmentioning

confidence: 99%

Frequency and Risk Factors of Endocrine Complications in Turkish Children and Adolescents with Sickle Cell Anemia

Özen¹,

Ünal

Erçetin³

et al. 2013

tjh

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“…Alkaline phosphatase is a biochemical marker of bone formation and its level may be related to growth impairment or associated with zinc deficiency. 39 Measurement of the bone-specific isoform would be preferable. Zinc deficiency was not assessed in this study.…”

Section: Discussionmentioning

confidence: 99%

Growth in children and adolescents with sickle cell disease in Yemen

Al-Saqladi

Bin-Gadeen

Brabin

2010

Annals of Tropical Paediatrics

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“…Several factors, including reduced haemoglobin levels, chronic haemolytic anaemia, hyperplasia of the bone marrow, abnor- mally low body mass index (BMI), increased ferritin levels, vitamin D deficiencies and low plasma zinc or sex steroid levels, have been previously correlated with reduced BMD in adult patients with SCD (17)(18)(19). Furthermore, reduced physical activity and decreased circulating GH are likely contributing factors (20,21).…”

Section: Sickle Cell Disease and The Bonesmentioning

confidence: 99%

Bone Disease in Haemoglobin Disorders

Voskaridou

Terpos

2013

Thalassemia Reports

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Bone disease represents a prominent cause of morbidity in patients with thalassaemia and other haemoglobin disorders. The delay in sexual maturation, the presence of diabetes and hypothyroidism, the parathyroid gland dysfunction, the haemolytic anaemia, the progressive marrow expansion, the iron toxicity on osteoblasts, the iron chelators, and the deficiency of growth hormone or insulin growth factors have been identified as major causes of osteoporosis in thalassaemia. Adequate hormonal replacement, effective iron chelation, improvement of hemoglobin levels, calcium and vitamin D administration, physical activity, and smoking cessation are the main to-date measures for the management of the disease. During the last decade, novel pathogenetic data suggest that the reduced osteoblastic activity, which is believed to be the basic mechanism of bone loss in thalassemia, is accompanied by a comparable or even greater increase in bone resorption. Therefore, potent inhibitors of osteoclast activation, such as the aminobisphosphonates, arise as key drugs for the management of osteoporosis in thalassaemia patients and other haemoglobin disorders.

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Markers of bone turnover are associated with growth and development in young subjects with sickle cell anemia

Cited by 28 publications

References 16 publications

Frequency and Risk Factors of Endocrine Complications in Turkish Children and Adolescents with Sickle Cell Anemia

Frequency and Risk Factors of Endocrine Complications in Turkish Children and Adolescents with Sickle Cell Anemia

Growth in children and adolescents with sickle cell disease in Yemen

Bone Disease in Haemoglobin Disorders

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