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Turner syndrome

Codes

IDC-10: Q96.9

ORPHA: 881

Report from the Questionnaire Report from the Observation Chart

General information

Estimated occurrence
1:2,000 girls born.
Cause
Girls with Turner syndrome are either missing one X chromosome (45,X) or part of it. Sometimes parts of the body have normal cells that contain two X chromosomes, while other cells are missing one X chromosome (mosaicism). Why this chromosomal aberration occurs is not known today. Turner syndrome is not hereditary.
General symptoms
Short stature. Many of these girls have loss of ovarian function and they often do not enter puberty. Cardiac defects are common. Often there are frequent ear infections. Learning disability and behavioral problems can sometimes occur.

Orofacial/odontological symptoms

Characteristic facial features are associated with the diagnosis. In contrast to skeletal development, which tends to be delayed by more than two years, dental development tends to be early, and teeth may erupt earlier than normal. Teeth may have shape aberrations. Common anomalies relate to the proportions between the root and the crown of the teeth, with relatively short roots on the incisors, canines and premolars. Teeth may be small, with thin enamel. They may also be widely-spaced. There are reports of arched palate and a small lower jaw. Various malocclusions may occur, with a particular frequency of post normal bite and cross bite. Malocclusions appear early. Feeding problems are common during the first years of life. Some girls have difficulties with speech and language acquvisition. Women who has not been treated with growth hormone can have a high frequent voice.

Advice on follow-up and treatment

  • Regular check-ups of dental and jaw development. Orthodontist should be consulted when needed.
  • A specialist in pediatric dentistry or orthodontia may be a useful resource in this respect, and should also be consulted if treatment with growth hormone is being considered.
  • Children with eating disorders often required extra dental care, including assistance with oral hygiene and fluoride treatments.
  • Feeding and swallowing difficulties are investigated and treated by a specialist team at the hospital or multidisciplinary treatment centre.
  • Speech and language difficulties should be diagnosed and treated by a speech therapist.
  • When treating medically compromised patients always contact their doctors for medical advice.

Sources

The intervention program for Turner syndrome, 2000, published by the Swedish Turner Academy.
Updated: 0001-01-01 00:00