Bone Health in Pediatric Chronic Illness
Bone Health Assessment in Pediatrics
Zinc Deficiency in Pediatric Hemoglobinopathies
Nutritional Deficiencies in Thalassemia
A primary area of research has focused on the prevention of osteoporosis in children, particularly in those with chronic disease. Osteoporosis is and, with our aging population, will continue to be, one of the major public health concerns of our time. Despite what the pharmaceutical industry has led us to believe, osteoporosis is not just treatable, but preventable. When strong dense bones are built in childhood, it lessens the likelihood of bone fragility in late adulthood. Therefore I have sought to describe, prevent and improve bone health in children particularly those at greatest risk for future bone fragility such as those with thalassemia, cerebral palsy, Rett syndrome, vitamin D deficiency rickets, sickle cell disease, and mucopolysaccaridosis. The majority of these children come from ethnically diverse families often with limited financial or social resources. The beauty of the nutritional and exercise interventions we have proposed are that they are both inexpensive and feasible. My overarching goal is to first characterize bone deficits in pediatric disorders and then to determine how optimal nutrition and exercise interventions can maximize bone density in children. These types of interventions have a lasting impact not only on the quality of life of the child, but on the future well-being of a population.
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Deficiencies in bone mass and strength have been identified as significant contributors to the risk of childhood and adult fractures. The epidemic of osteoporosis in adulthood is well known. In addition, the literature reflects that childhood fracture rates are increasing as are the number of children determined to have low bone mineral density. The greatest opportunity to affect bone health is in the period of greatest acquisition which is during childhood and adolescence. However, clinicians require tools for accurate assessment of bone health in children which has lead us to publish two editions of a textbook on the subject, Bone Health Assessment in Pediatrics (2016, Springer). Other areas of interest include exploration of optimal solutions for the assessment of bone health in children with various chronic illness’ and special health care needs.
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The gold standard way to characterize zinc deficiency in children is to supplement with zinc and measure growth outcome. Therefore, through the NHLBI funded Sickle Cell Center at CHOP we conducted a prospective 12 month zinc supplementation study in children with SS type SCD. Our findings suggested that children with SCD have a growth limiting zinc deficiency (Zemel et al. AJCN 2002;75:300-7.) Later we hypothesized that some patients have low bone mass, in part, due to zinc deficiency. To test this hypothesis, an 18 month randomized placebo-controlled trial of zinc supplementation (25 mg Zn/day) was conducted in 42 young patients (10-30 yrs) with Thalassemia. A significant increase in bone mineral content and density at the spine and whole body was observed in those on zinc compared to placebo (Fung EB et al. AJCN, 2013). This was the first trial of zinc supplementation with the intention to enhance bone mass in any clinical patient population. The results from this study convinced us that there was a functional zinc deficiency in patients with Thal. In
other populations, zinc deficiency is known to aggravate diabetes, a well known co-morbidity in patients with Thal. These observances lead to a short term interventional trial of the effects of zinc supplementation on glucose tolerance in Thal funded by the Cooley’s Anemia Foundation. The preliminary results from this study suggest that zinc supplementation may enhance glucose homeostasis, particularly in patients who are heavily iron loaded.
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Patients with Thalassemia have a number of morbidities including cardiomyopathy, osteoporosis, diabetes, impaired immune function, growth failure and delayed pubertal development. Many if, not all, have some association with altered nutritional status, though few scientists are focused on the issue. Our work over the past decade has been to educate the hemotologists in the field to the importance of nutrition in the quality of life of the patients with thalassemia. Though at least a handful of studies have investigated dietary intake in patients with Thal, the data are limited by either a small sample size, or by the lack of corresponding circulating levels of nutrients in the patients. Our research has expanded to multicenter data sampling, and added research quality nutritional assessment techniques. Nutrition information is now shared directly with the Thal community through our thalassemia website here at BCHO: http://thalassemia.com/nutrition-and-diet.aspx#gsc.tab=0.
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April 16, 2019 2:46 PM