Hunter Syndrome Hazardous Growth Hindrance

Hunter syndrome – hazardous growth hindrance

by

Balfour

Hunter syndrome is characterized by missing or malfunctioning enzyme iduronate-2-sulfatase. This enzyme is used by lysosomes to build and repair bones, tendons, cartilages and connecting tissues. They lubricate joints and aid body s renewal process.

When this enzyme is absent or less than necessary, body cannot break down complex sugars called glycosaminoglycan. These molecules eventually get saturated in certain cells and tissues. These build ups cause permanent damage to those specific organs especially brain and joints. Most of the organ functions, mental abilities and physical abilities are severely affected. Damage is progressive and irreversible in nature.

Hunter syndrome is belongs to a group of inherited metabolic disorders namely mucopolysaccharidosis (MPS). It is also known as MPS II. MPS diseases are called lysosomal storage disease.

Symptoms:-

Late development e.g. delayed sitting or walking

Enlarged spleen and liver

Distended abdomen

Facial disorientation

Thickening of lips. Nostrils and tongue

Broad nose and flared nostrils

Protruding tongue

Claws like hands

Respiratory disorders

Sleep apnea

Abnormal bone development

Diarrhea

Progressive vision loss and hearing loss

Carpel Tunnel syndrome

Behavioral changes

Cardiovascular diseases like thickening of heart valves or clogging of blood vessels

Hypertension

Risk factors:-

Family history- It is X-linked recessive disease inherited from mothers. Family history plays an important role in transmission of Hunter disease.

Sex- Men are more prone to this disease. Women inherit two X chromosomes. Hence when one is defective, second one can provide a functioning gene. On the other hand, men contain only one copy of X chromosome and if it is defective, alternative copy of functioning gene is not available.

Complications:-

Respiratory complications: – Thickening of windpipe and nasal passages can cause breathing problems.

Children may develop sinus infection, pneumonia, chronic ear and respiratory infections. If these diseases progresses, they can cause death.

Cardiac complications:-Thickening of heart walls can interfere with normal circulation. Heart and other organs may receive inadequate supply of blood. It often results into heart failure as the disease progresses. Hypertension and pulmonary hypertension are other possibilities.

Skeletal complications: -Joint stiffness is caused by structural abnormalities in bone sand connective tissues. Growth is thwarted and child may become unable to move. Enlargement of spleen and liver can cause hernia.

Brain and nervous system complications: – Hydrocephalus or fluid buildup in brain can cause a variety of neurological complications. These fluid buildups may exert excessive pressure on brain and spinal cord. Other sensory organs like eyes and ears may also get affected. Hypertrophic cervical pachymeningitis or thickening of membranes around spinal cord may weaken the legs as well as cause fatigue. Child may show behavioral problems like inability to pay concentration, hyperactivity and aggression. Some children may develop seizures.

Balfour Morris is a well known content writer with his every article focusing on new and unique topics related to health. The area of expertise of his articles is the accuracy and apt information with reference to normal well being. Also write the article like generic medicines like

Zenegra

, Forzest,

Penegra

, silagra.

Article Source:

ArticleRich.com