Friday, October 3, 2008

DOWN'S SYNDROME / MONGOLIANISM / MONGOLISM / TRISOMY 21






Down syndrome (DS), also called Trisomy 21, is a condition in which extra genetic material causes delays in the way a child develops, both mentally and physically.
It affects about 1 in every 800 babies.

The physical features and medical problems associated with Down syndrome can vary widely from child to child.
While some kids with DS need a lot of medical attention, others lead healthy lives.

Though Down syndrome can't be prevented, it can be detected before a child is born. The health problems that can go along with DS can be treated, and there are many resources within communities to help kids and their families who are living with the condition.



Normally, at the time of conception a baby inherits genetic information from its parents in the form of 46 chromosomes: 23 from the mother and 23 from the father.
In most cases of Down syndrome, a child gets an extra chromosome 21 — for a total of 47 chromosomes instead of 46.
It's this extra genetic material that causes the physical features and developmental delays associated with DS.

Although no one knows for sure why DS occurs and there's no way to prevent the chromosomal error that causes it, scientists do know that women age 35 and older have a significantly higher risk of having a child with the condition.
At age 30, for example, a woman has about a 1 in 900 chance of conceiving a child with DS.
Those odds increase to about 1 in 350 by age 35.
By 40 the risk rises to about 1 in 100.




Kids with Down syndrome tend to share certain physical features such as a flat facial profile, an upward slant to the eyes, small ears, and a large or protruding tongue.



Low muscle tone (called hypotonia) is also characteristic of children with DS, and babies in particular may seem especially "floppy." Though this can and often does improve over time, most children with DS typically reach developmental milestones — like sitting up, crawling, and walking — later than other kids.


At birth, kids with DS are usually of average size, but they tend to grow at a slower rate and remain smaller than their peers.
For infants, low muscle tone may contribute to sucking and feeding problems, as well as constipation and other digestive issues.
Toddlers and older kids may have delays in speech and self-care skills like feeding, dressing, and toilet teaching.


Down syndrome affects kids' ability to learn in different ways, but most have mild to moderate intellectual impairment.
Kids with DS can and do learn, and are capable of developing skills throughout their lives.
They simply reach goals at a different pace — which is why it's important not to compare a child with DS against typically developing siblings or even other children with the condition.

Kids with DS have a wide range of abilities, and there's no way to tell at birth what they will be capable of as they grow up.


Dental association with DS
Introduction:
The orofacial and skeletal development associated with Down's Syndrome contribute to dental problems. It is important to be aware of the type of anatomical soft tissue and dental anomalies which are part of the typical developmental pattern of people with Down's Syndrome, which have influence on dental problems.

Anatomical development changes the cranial base, the mid third of the face and the proportion between the maxilla and mandible. This alteration of the skeleton leads to people with Down's Syndrome having a recognisable facial appearance. The soft tissue feature most affected is the tongue, which is fissured and protrusive. The tongue appears large because it has to rest in a narrow dental arch. The tonsils and adenoids are also enlarged.

Dental anomalies are related to the tooth morphology in that there is:

* Decreased root to crown ratio
* Decreased tooth size
* Hypodontia or partial anodontia
* Delayed eruption


DENTAL PROBLEMS:


The normal development of oral structure and function is altered leading to compromised development of suckling, swallowing, mastication and speech; and to drooling unless there is effective intervention.

The degree of difficulty varies from person to person:




Preventive measures and therapy are needed to ameliorate the problems found in swallowing and mastication. Here an integrated approach can be adopted with the Speech and Language Therapist.




DENTAL DISEASE


People with Down's Syndrome are prone to the same degree of dental disease as the general population.

Periodontal disease
: People with Down's Syndrome develop more severe forms of periodontal disease than the general population.
This may be related to immunological deficiency factors.
This disease is most rampant in young people between 16 and 20 years old.
The progression of the disease gives rise to periods of acute infection and pain, which may result in changes in behavior, refusal to eat or swallowing food whole.

Caries: Various studies have shown a reduced incidence of caries in children and young adults with Down's Syndrome.
This may be due to the fact that many of these children are under supervision in regard to their diet in order to prevent their tendency to obesity.
This is where the dentist and the dietician can work together to make sure the food being consumed is working towards oral and general health improvement.

Risk associated with infection is raised in people with Down's Syndrome as the incidence of congenital cardiac disease is increased in this group (3% to 40%), resulting in a serious risk of endocarditis.

The gag reflex can occur even in the anterior portion of the oral cavity. Any further back than the premolars a gag reflex may be accompanied by a gastro-oesophegal reflux. Children find this most uncomfortable.

Bruxism occurs in people with Down's Syndrome and may be triggered by a state of chronic anxiety, dental malocclusion, temporo mandibular joint dysfunction due to laxity of the supporting ligaments, and/or underdeveloped nervous control.

Dental trauma is frequently experienced due to lack of motor development.
Fracture or luxation of the anterior teeth is frequent and often involves loss of tooth vitality.

TREATMENT & PREVENTION

* Good oral hygiene and supervised tooth brushing programmes
* Education, e.g. via videotapes
* Diet, communication and use of oral muscles. This requires an integrated approach to care, as it involves a team of professionals and carers.
* Management of any malocclusion requires a multi-disciplinary team to carry out diagnosis and treatment planning (e.g. Orthodontist, Restorative and Oral Maxillo-Facial Surgeons)

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