Prader-Willi Syndrome: Clinical aspects and effects of growth hormone treatment

This thesis presents a detailed description of several studies on growth, metabolism, psychomotor development, cognition, behaviour and breathing in PWS children. In chapter 2, growth, body composition and body proportions before and during growth hormone (GH) treatment are evaluated. In chapter 3, the effects of GH treatment on psychomotor development, as measured with Bayley Scales of Infant Development in PWS infants and toddlers are reported In chapter 4, studies on sleep-related breathing, as measured with polysomnography in pre-pubertal children with PWS are summarized, including the effects of GH treatment and upper respiratory tract infections on sleep-related breathing. Chapter 5 describes the associations between sleep-related breathing, cognition, and neurobehavioral abnormalities. Chapter 6 describes in infants whether psychomotor development is affected by sleep-disordered breathing, particularly obstructive sleep apnea syndrome (OSAS), as was recently shown for healthy infants. In chapter 7, thyroid hormone function is reported in a large group of pre-pubertal PWS children, including effects of GH treatment on thyroid hormone function. In chapter 8, serum adiponectin levels are evaluated both before and during GH-treatment. Adiponectin is strongly related to insulin sensitivity and is considered cardioprotective. In chapter 9, the general discussion our data are discussed in relation to the present literature. Chapter 10 summarizes our findings in English, and chapter 11 presents a Dutch summary.

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