DWARFISM

DWARFISM:

 

Introduction:

·         Dwarfism is a condition of short stature that result from a genetic or medical conditions .

·          It is defined by the advocacy group little people of America (LPA) as an adult height of 4 feet 10 inches or under, as a result of medical or genetic condition.

·         Dwarfism is a condition in which the growth of the individual is very slow or delayed, resulting in less than normal adult stature. It is characterized by decreased bodily growth primarily due to hormone problems. The end result is a proportional little person because the growth of all other structures of the individual is decreased.      

          

Types of Dwarfism:

There are two main categories of dwarfism- Disproportionate and Proportionate.

a.       Disproportionate dwarfism or Disproportionate Short Stature (DSS):

·         If the body size is disproportionate i.e. some parts of the body are small and other parts are of average size or above average size.

·         It is characterized by an average size of torso and shorter arms and legs or a shortened trunk with longer limbs. It inhibits the development of bones.

·         Conditions associated with DSS are usually caused by faulty gene. The affected genes stop the bones and cartilage developing property, leading to restricted growth that is often noticeable from birth.

·         Many children born with DSS have parents of average height and the change to the gene happen by chance. However, people with conditions that cause DSS may pass their condition on to their children.

·         The most common cause of DSS is a condition called achondroplasia, which is estimated to affect around one in every 25,000 people.

·         Other conditions that cause DSS include; hypochodroplasia, MPS disease, dystrophic dysplasia, spondyloepiphyseal dysplasia, Pseudoachondroplasia, Conradi syndrome, Elli- Van crevoid syndrome.

           

b.      Proportionate Dwarfism or Proportionate Short Stature (PSS):

·         In proportionate dwarfism, the body parts are in proportion but are shortened in length. A body is proportionally small to the same degree and appear to be proportioned like a body of average stature.

·         A less common is the body not producing enough hormones, which is estimated to affect one in every 3,500- 4,000 children.

·          In medical condition fat at birth or appearing in early childhood limit the overall growth and development.

·         The main cause of PSS is being born from small parents.

·         This may be present at birth responsible for producing several different or as part of other conditions it can also start in childhood, as the result of an injury, a brain tumour or radiotherapy to the brain.

·         Other possible causes of PSS include; long term conditions- such as those affecting lungs, heart or kidneys, Steroid treatment for chronic condition such as asthma, Genetic abnormality such as  Down’s syndrome, Nooron syndrome, Turner’s syndrome, Proder- Willi syndrome, Malnutrition or poor absorptionof food; for example coelic disease.

Causes of Dwarfism:

More than 300 conditions are known to have short stature in child. Most are caused by a spontaneous genetic change (mutation) in the egg or sperm cells prior to conception. Others are caused by genetic changes inherited from one or both parents.

·         Depending on the type of condition causing the short stature; it is possible for two average sized parents to have child with short stature and is possible for parents to have child with short stature and is possible for parents who are little people to have an average sized child.

·         Dwarfism has other causes including metabolic or hormonal disorders in childhood chromosome abnormalities, pituitary gland disorders (which influence growth and metabolism), absorptive problems (when the body cannot absorb nutrients adequately) and kidney disease can all lead to short stature if a child fails to grow at a normal rate.

·         This includes metabolic and hormonal disorders such as growth hormone deficiency

·         The most common types of dwarfism known as skeletal dysplasia.

Skeletal Dysplasia:

·          It a genetic disease. This is a condition of abnormal bone growth that causes disproportionate dwarfism.

·         Skeletal dysplasia includes following:

        I.            Achondroplasia:

It occurs in about 1 out of 26,000 – 40,000 babies. It is evident at birth People with achondroplasia have a relatively long time and shortened upper parts of their arms and legs. Other features of achondroplasia include:

·         A large head with prominent forehead.

·         A flattened bridge of the nose.

·         A protruding jaw.

·         Crowded and misaligned teeth.

·         Forward curvature of the lower spine.

·         Bowed legs.

·         Flat, short and broad feet.

·         Double jointedness.

          

      II.            Spondyloepiphyseal Dysplasia (SED):

It is a less common form of dwarfism, it affects approximately one in 95 % of babies. Spondyloepiphyseal dysplasia refers to a group of condition characterized by shortened trunk, which may not become apparent until a child is between the age of 5- 10 years. Other features include:

·         Club feet

·         Cleft Palate

·         Severe Osteoarthritis in the hips

·         Weak hands and feet

·         Barrel chested appearance

          

    III.            Diastrophic Dysplasia:

A rare form of dwarfism, Diastrophic dysplasia occurs in about one in 100,000 births. People who have shortened forearms and calves (this is known as mesometric shortening). Other signs include:

·         Deformed hands and feet

·         Limited range of motion

·         Cleft palate

·         Ears with cauliflower appearance

Diagnosis and Treatment:

A diagnosis if dwarfism is based primarily on measurements of the child’s height, weight and head circumference. Other factors taken into consideration include genetics, family, medical history and the results of hormone tests and imaging studies. Treatment cannot improve stature but is aimed at improving complications of the condition. Some types of dwarfism can be identified through prenatal testing if a doctor suspects a particular condition and tests for it. But most cases are not identified until after the child is born. In these instances, the doctor make a diagnosis based on the body’s appearance, growth pattern and X- rays of the bones. Depending on the type of dwarfism the child carries, diagnosis often can be made almost immediately after birth.

Some type of dwarfism can be identified through prenatal testing if a doctor suspects a reticular condition and tests for it.  But most cases are not identified until after the child is born. In these instances, the doctor make a diagnosis based on the type of dwarfism the child has diagnosis often can be made almost immediately after birth.

·         Once a diagnosis is made, there is no treatment for most of the conditions that lead to short stature. Hormonal or metabolic problems may be treated with hormonal injections or special diets to increase a child’s growth, but skeletal dysplasia cannot be cured.

·         People with skeletal dysplasia can get medical care for some of the health complications associated with their short stature.

·         Some form of dwarfism also involves issues in other body systems. Such as vision or hearing and require careful monitoring.

Surgery is also a method of treatment in dwarfism. Some example of the surgical procedures used to address abnormal bone growth. It includes:

·         The insertion of metal plates in the growth plates of bones.

·         The straightening of bones and addition of metal plates to hold them in place..

·         The insertion of rods and staples  to correct the spine’s shape

·         Lengthening of limbs which involve dividing the bone and separating the section.

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