scholarly journals Body Composition and Fatness Patterns in Prader-Willi Syndrome: Comparison with Simple Obesity*

Obesity ◽  
2006 ◽  
Vol 14 (10) ◽  
pp. 1685-1690 ◽  
Author(s):  
Mariana F. Theodoro ◽  
Zohreh Talebizadeh ◽  
Merlin G. Butler
Keyword(s):  
Body Composition ◽  
Prader Willi Syndrome ◽  
Simple Obesity

scholarly journals Comparison of Frequency and Severity of Sleep-Related Breathing Disorders in Children with Simple Obesity and Paediatric Patients with Prader–Willi Syndrome

2021 ◽  
Vol 11 (2) ◽  
pp. 141
Author(s):  
Agnieszka Lecka-Ambroziak ◽  
Marta Wysocka-Mincewicz ◽  
Anna Świercz ◽  
Małgorzata Jędrzejczak ◽  
Mieczysław Szalecki
Keyword(s):  
Insulin Resistance ◽  
Apnea Hypopnea Index ◽  
Oral Glucose ◽  
Prader Willi Syndrome ◽  
Model Assessment ◽  
Sleep Related Breathing Disorders ◽  
Breathing Disorders ◽  
Simple Obesity ◽  
Rhgh Treatment ◽  
Group 1

Sleep-related breathing disorders (SRBDs) can be present in children with simple obesity and with Prader–Willi syndrome (PWS) and influence an individual diagnostic and treatment approach. We compared frequency and severity of SRBDs in children with simple obesity and with PWS, both without and on recombinant human growth hormone (rhGH) treatment, and correlation of SRBDs with insulin resistance tests. A screening polysomnography-polygraphy (PSG), the oral glucose tolerance test (OGTT) and homeostasis model assessment of insulin resistance (HOMA-IR) were analysed in three groups of patients—with simple obesity (group 1, n = 30, mean age 14.2 years), patients with PWS without the rhGH therapy (group 2, n = 8, mean age 13.0 years) and during the rhGH treatment (group 3, n = 17, mean age 8.9 years). The oxygen desaturation index (ODI) was significantly higher in groups 2 and 3, compared to group 1 (p = 0.00), and hypopnea index (HI) was higher in group 1 (p = 0.03). Apnea–hypopnea index (AHI) and apnea index (AI) results positively correlated with the insulin resistance parameters in groups 1 and 3. The PSG values worsened along with the increasing insulin resistance in children with simple obesity and patients with PWS treated with rhGH that may lead to a change in the patients’ care.

scholarly journals Time for a general approval of growth hormone treatment in adults with Prader–Willi syndrome

2021 ◽  
Vol 16 (1) ◽  
Author(s):  
Charlotte Höybye ◽  
◽  
Anthony J. Holland ◽  
Daniel J. Driscoll
Keyword(s):  
Growth Hormone ◽  
Body Composition ◽  
Transition Period ◽  
Neurodevelopmental Disorder ◽  
Hormone Treatment ◽  
Psychomotor Development ◽  
Prader Willi Syndrome ◽  
Gh Treatment ◽  
Beneficial Effects ◽  
Positive Effects

AbstractPrader-Willi syndrome (PWS) is a complex, multi-system, neurodevelopmental disorder characterised by neonatal muscular hypotonia, short stature, high risk of obesity, hypogonadism, intellectual disabilities, distinct behavioural/psychiatric problems and abnormal body composition with increased body fat and a deficit of lean body mass. Growth hormone (GH) deficiency and other hormone deficiencies are common due to hypothalamic dysfunction. In children with PWS GH treatment has been widely demonstrated to improve body composition, normalise height and improve psychomotor development. In adults with PWS, GH’s main effects are to maintain normal body structure and metabolism. The positive effects of GH treatment on body composition, physical fitness and beneficial effects on cardiovascular risk markers, behaviour and quality of life in adults with PWS are also well established from several studies. GH treatment is approved for treatment of children with PWS in many countries, but until recently not as a treatment in young adults in the transition period or for adults in general. In this commentary we want to draw attention to the uneven global use of GH treatment, specifically in adults with PWS, and advocate for GH treatment to be approved internationally, not just for children, but also for adults with PWS and based only on the diagnosis of genetically confirmed PWS.

Two Year of Growth Hormone Therapy Improves Body Composition in Adults with Prader-Willi Syndrome.

2010 ◽  
pp. OR25-6-OR25-6
Author(s):  
R Sode-Carlsen ◽  
S Farholt ◽  
K FR Rabben ◽  
J Bollerslev ◽  
T Schreiner ◽  
...  
Keyword(s):  
Growth Hormone ◽  
Body Composition ◽  
Hormone Therapy ◽  
Growth Hormone Therapy ◽  
Prader Willi Syndrome

scholarly journals Changes of Body Weight and Body Composition in Obese Patients with Prader–Willi Syndrome at 3 and 6 Years of Follow-Up: A Retrospective Cohort Study

2020 ◽  
Vol 9 (11) ◽  
pp. 3596
Author(s):  
Giorgio Bedogni ◽  
Graziano Grugni ◽  
Sabrina Cicolini ◽  
Diana Caroli ◽  
Sofia Tamini ◽  
...  
Keyword(s):  
Weight Loss ◽  
Body Composition ◽  
Cohort Study ◽  
Retrospective Cohort Study ◽  
Retrospective Cohort ◽  
Density Lipoprotein ◽  
Rehabilitation Program ◽  
Long Term Results ◽  
Prader Willi Syndrome

Few short-term studies of weight loss have been performed in adult patients with Prader–Willi syndrome (PWS) undergoing metabolic rehabilitation. We performed a retrospective cohort study of 45 adult obese PWS patients undergoing a long-term multidisciplinary metabolic rehabilitation program based on diet and physical activity. Body composition was evaluated by dual-energy X-ray absorptiometry in 36 (80%) patients. The mean (95% CI) weight change was −3.6 (−7.6 to 0.4, p = 0.08) kg at 3 years and −4.6 (−8.5 to −0.8, p = 0.02) kg at 6 years, and that of BMI was −1.7 (−3.4 to 0.1, p = 0.06) kg/m2 at 3 years and −2.1 (−3.8 to −0.4, p = 0.02) kg/m2 at 6 years. A decrease of about 2% in fat mass per unit of body mass was observed, which is in line with the expectations for moderate weight loss. A possibly clinically relevant decrease in total and low-density lipoprotein cholesterol was also observed. These long-term results are important for patients with PWS, which is characterized by severe hyperphagia, behavioral disturbances, and cognitive impairment and is generally considered “resistant” to classical weight loss interventions.

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