21/02/2009 Nutritional Issues in Turner Syndrome A presentation to the TCGI Annual Conference 2009 Jessica Sheppard

Overview Features with nutritional impact Short stature Tendency toward overweight High blood pressure High blood lipids Risk of endocrine problems; hypothyroidism, diabetes Risk of impaired bone health No special diet, no foods that must be eaten or must be avoided

Short Stature Turner girls are typically 8-10cm shorter than other girls. Height and weight should be relatively proportional Adequate nutrition important for growth and development, especially when on hormone treatment

Short Stature/Weight Lower energy requirements Modern obesogenic environment Most food portions are standard

Weight Management Any unhealthy trend in growth will be picked up at clinic. Can be easily addressed with moderate lifestyle changes No less effective in Turner girls/women

Weight Management ~40 TS girls attending OLCH Endocrine clinic 10 currently linked in with dietetic service All for weight management Similar degree of overweight as non TS girls at first assessment Seem to experience same level of success with weight management as non TS

Weight Management Approach has changed over last decade Focus on behaviour change, SMART goal setting Sustainable Measurable Achievable Rewarded Time limited

Weight Management Individual advice for each client Based on current lifestyle No unrealistic targets set Weight loss rarely appropriate Progress assessed at each visit based change in BMI relative to population

Weight Management Several programmes available Variable results Measurement of success not necessarily consistent with client expectations Consistent attendance a problem for all programmes

High blood pressure Puts stress on circulatory system and kidneys Regular screening recommended Weight management, exercise can help

Blood lipids Cholesterol, Triglycerides Regular screening recommended Healthy levels important for everybody Weight management, exercise can help

Endocrine system Diabetes Mellitus Increased risk in TS Maintenance of a healthy weight can greatly reduce risk Regular screening recommended Hypothyroidism Affects up to 40% of Turner women by age 40 Regular screening recommended

Bone Health Two main types of bone Cortical ‘Long’ bones e.g. limbs, fingers Thinner in TS Does not appear to be related to lack of oestrogen Trabecular Vertebrae, hips Thinner in any oestrogen-deficient condition

Bone Health Dietary factors: Vitamin D Found in and fish, dairy products, vegetable oils Calcium Found in dairy products, some fruit and veg, some cereal products Irish RDAs are higher across all age groups Phosphate Found in dairy products, wholegrains

Bone Health Other protective factors Oestrogen Deficiency reduces density of trabecular bone No effect on cortical bone Weight bearing exercise Strengthens bones, muscles, connective tissue Could reduce risk of falls by improving balance and dexterity

Role of Phytoestrogens Plant chemicals found mainly in legumes e.g. soya, clover, chickpeas; also in flaxseed Structurally similar to human oestrogens

Oestradiol (human)

Genistein (phyto)

Phytoestrogens Believed to have an oestrogen-like effect Sometimes marketed as a supplement/alternative to hormone replacement Claims beneficial effects on bone health, hot flushes, reduced risk of hormone related cancers

Phytoestrogens: Evidence Animal studies:Genistein and Daidzein (found in soy) have been shown to preserve bone mass Humans: Lower incidence of hip fracture in South-East Asia, where intake of soy is high. However, incidence of other fractures is not reduced

Phytoestrogens: Evidence Studies have been of small size and short duration One study showed that enterodiol (found in flaxseed) can limit hot flushes in menopausal women but high dose of flaxseed caused abdominal side effects Reduced risk of hormone related cancers is not supported by the scientific evidence

Role of Phytoestrogens No specific benefits, so no need to actively include unless vegan or milk intolerant. Important to choose calcium enriched products Perfectly safe to consume, no need to avoid