Prader-Willi Syndrome after age 15 years.

Laurance, B M; Brito, Alfredo Darío Espinosa; Wilkinson, Jane

doi:10.1136/adc.56.3.181

Cited by 131 publications

(84 citation statements)

References 31 publications

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“…Decreased physical exercise and lowered energy expenditure have been identified as exacerbating the problem (Butler, 1990;Davies & Joughin, 1993). Physical health problems associated with weight gain have been reported including: diabetes mellitus (Laurance et al, 1981), coronary artery disease (Lamb & Johnson, 1987) and premature death (Laurance et al, 1981). The psychological impact of being overweight may also be detrimental, contributing to difficulties in forming relationships with peers (Dykens et al, 1992) and in turn affecting an individuals' selfworth (Wolfensberger, 2000).…”

Section: Introductionmentioning

confidence: 99%

The assessment of food‐related problems in individuals with Prader‐Willi syndrome

Russell

Oliver

2003

British J Clinic Psychol

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The development of a twenty item questionnaire, the Food Related Problem Questionnaire (FRPQ), with four subscales (preoccupation with food, impairment of satiety, difficulty with self-control and other food-related 'challenging' behaviour of food-related problems) for use with individuals with Prader-Willi syndrome (PWS) is described.. Test-retest and inter-rater reliability for the total score are .78 and .86 respectively. Subscale test-retest and inter-rater reliability are acceptable for three subscales (range .70 to .85) but not for the self control subscale. To establish criterion validity, scores on the FRPQ for a sample of individuals with PWS were compared to scores for individuals with intellectual disability but not PWS. All reliable subscale scores and the total score differentiated between the groups (p<.001 in all cases). Internal consistency for the total scale is good (Cronbach's Alpha = .87). Finally, some normative data for a limited age range are presented and potential applications of the measure are discussed.Assessing food related problems 3

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Section: Introductionmentioning

confidence: 99%

The assessment of food‐related problems in individuals with Prader‐Willi syndrome

Russell

Oliver

2003

British J Clinic Psychol

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“…Ventilatory failure appears to be a frequent cause of death but this has hardly been investigated. In one series of 33 patients, nine died and in each case cardiorespiratory failure was given as the cause of death, although no details of the clinical features or treatment were available [3].…”

Section: Discussionmentioning

confidence: 99%

Treatment of ventilatory failure in the Prader-Willi syndrome

Smith

King²,

Siklos³

et al. 1998

Eur Respir J

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Hypercapnic respiratory failure is a common cause of death in the Prader-Willi syndrome. Its relationship to sleep-disordered breathing has not been established and there are no reports of its successful treatment. We have retrospectively reviewed the records of four patients with the syndrome, who developed ventilatory failure. Daytime arterial blood gas tensions and overnight oximetry traces before and during treatment were compared. Each patient had severe sleep-disordered breathing in association with daytime ventilatory failure. The median overnight mean arterial oxygen saturation (Sa,O2) was 82% and the median minimum was only 41.5%. Initial treatment was with nasal intermittent positive pressure ventilation, and in each case the daytime arterial blood gas tensions were normalized. The patients were maintained on nasal continuous positive airway pressure at night after discharge. Compliance has been good, and at last follow-up (after a median of 4.8 yrs) the daytime arterial gas tensions remained normal, while the median overnight mean arterial oxygen saturation was 95.5% and the median minimum was 84.5%. This study of patients with the Prader-Willi syndrome shows that daytime ventilatory failure is associated with sleep-disordered breathing. It can be reversed with nocturnal noninvasive ventilation and maintenance treatment with continuous positive airway pressure is well tolerated, with no deterioration in respiratory parameters.

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“…1,[8][9][10][11][12] One study estimated that between 43% and 66% of those with PWS developed scoliosis. 8 The Queen Elizabeth Hospital for Children in London did a retrospective study that demonstrated that 62% of PWS patients had clinical scoliosis.…”

Section: Introductionmentioning

confidence: 99%

“…8 The Queen Elizabeth Hospital for Children in London did a retrospective study that demonstrated that 62% of PWS patients had clinical scoliosis. 9 Body composition studies have shown increased body fat and reduced muscle in PWS, both in infants and adults. These 2 factors are most likely major contributors in the development and prevalence of scoliosis among those with PWS.…”

Section: Introductionmentioning

confidence: 99%

“…Obesity, in addition to the structural and physiologic conditions outlined in Fig 2, has often led to early death due to cardiorespiratory failure. 2,9 However, with early diagnosis, weight control, psychosocial management, GHRT, and use of multidisciplinary physicians, the prognosis is promising. 1 Chiropractors are sometimes forgotten to be included in the care of children with PWS, despite successful management of spine conditions and case reports of managing idiopathic scoliosis with chiropractic care.…”

Section: Introductionmentioning

confidence: 99%

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Inclusion of chiropractic care in multidisciplinary management of a child with Prader-Willi syndrome: a case report

Wittman

Vallone

2009

Journal of Chiropractic Medicine

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Objective: The purpose of this paper is to present a case of a child with Prader-Willi syndrome and the observed improvement in the degree of scoliosis, immune function, and behavior documented during the course of her treatment. Clinical Features: A 7-year-old girl presented to Kentuckiana Children's Center with a 15°l umbar levoscoliosis and diagnosis of Prader-Willi syndrome. Intervention and Outcome: The treatment plan consisted of chiropractic adjustments, craniosacral therapy, movement therapy, and nutritional therapy. Over the course of treatment, her muscle strength, tone, and motor activity increased. She improved in coordination of gait and balance. Over the course of 3 years, her scoliosis decreased to 4°to 5°. Improvements in immune function and a reduction in anxiety type behaviors were documented by the parents and doctor of chiropractic over the course of 5 years. Conclusions: This case report describes the improvements and progression of one female child with Prader-Willi syndrome under chiropractic and multidisciplinary care.

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Prader-Willi Syndrome after age 15 years.

Cited by 131 publications

References 31 publications

The assessment of food‐related problems in individuals with Prader‐Willi syndrome

The assessment of food‐related problems in individuals with Prader‐Willi syndrome

Treatment of ventilatory failure in the Prader-Willi syndrome

Inclusion of chiropractic care in multidisciplinary management of a child with Prader-Willi syndrome: a case report

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