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physical and mental changes that will occur during puberty, adolescence and adulthood


A large number of families and caregivers, therefore, have multiple questions about both the physical and mental changes that will occur during puberty, adolescence and adulthood. Previously, our answers as healthcare providers had been based on personal experiences with individual adult patients of different ages.

Although CdLS was originally described in the early 1900’s, the syndrome has been well characterized only during the last quarter of the century. Many individuals with CdLS, diagnosed when they were infants, are now entering into adulthood. There are few publications addressing adolescent and young adulthood issues for any genetic syndrome, although these are of great interest to both primary care providers and geneticists.

In the past several years we have collected specific information about puberty and adolescence from families, as part of an ongoing global project to evaluate aging in CdLS. A puberty questionnaire was originally distributed to member families of the CdLS Foundation through the USA office eight years ago. Seventy‐four have been evaluated for this part of the aging project, and the collated results follow.

  • There were almost twice as many surveys received on females than on males. 
  • Both males and females experienced worsening gastroesophageal reflux during adolescence, often requiring treatment. 
  • Sixty percent reported circulation problems with their hands and/or feet. 
  • Other medically related issues include;
    • scoliosis (curvature of the spine), 
    • nasal polyps (growth in the nose), 
    • seizures, 
    • and psychiatric disorders. 
  • Of the responses received, the average age of puberty onset was 13 years in females and 14 years in males, both slightly later than in unaffected children.
  • There was a growth spurt reported during puberty in both height (in 40% of the females and half of the males) 
  • and weight (in 60% of the females, and in 40% of the males). 
  • Pubic hair developed in most of the individuals (in 85% of the females and 90% of the males); 
  • however axillary hair (under the arms) was infrequent (in 20% of the females and 35% of the males).
  • Of the survey answers received on the females with CdLS, 80% reported breast development, and only 75% ever “got” their period. 
  • Premenstrual syndrome was common; 
  • irregularity of the periods was present but less common. 
  • Various treatments for menstruation included, with varying degrees of success: hysterectomy, hormonal replacement (e.g. taking hormones via a pill), and gynecology procedures.
  • Of the surveys received on the males with CdLS, two‐thirds reported facial hair, 
  • and half had increased size of genitalia (penis, testicles).

Three‐quarters of the surveys discussed that behavioral issues were very common and worsened with the onset of puberty. Thirty‐eight percent reported moodiness, 25% had obsessivecompulsive disorder. Other behavioral concerns included change in sleep patterns and masturbation. Management of these issues provides the most difficult challenge to the caregivers.

There was a growth spurt reported during puberty in both height (in 40% of the females and half of the males)

There was a growth spurt reported during puberty in both height (in 40% of the females and half of the males

)

weight increase (in 60% of the females, and in 40% of the males)

weight increase (in 60% of the females, and in 40% of the males

)

Sixty percent reported circulation problems with their hands and/or feet.

Sixty percent reported circulation problems with their hands and/or feet.

The average age at which puberty started was 13 years for girls and 14 years for boys, for both boys and girls only slightly later than for other children.

The average age at which puberty started was 13 years for girls and 14 years for boys, for both boys and girls only slightly later than for other children.

80% of the girls with CdLS reported breast development, and 75% got their periods.

80% of the girls with CdLS reported breast development, and 75% got their periods.

In 75% of the adolescents there were behavioural problems, which became worse during puberty, in 38% of the adolescents there were depressive feelings.

In 75% of the adolescents there were behavioural problems, which became worse during puberty, in 38% of the adolescents there were depressive feelings.

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TonieKline

Medical Director, CdLS Foundation

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Last modified by Gerritjan Koekkoek on 2022/08/05 11:45
Created by Gerritjan Koekkoek on 2022/08/05 11:45
translated by renee van Tuyll

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