Disease of bone: The Osteopetrosis

The osteopetrosis is the increase in density (consistency) bones, this disease causes various skeletal abnormalities, which can be generated from mild annoyances to disability or death of the patient. Adults can handle it, but children face major problems.

Our bone system has a cycle of bones, reconstruction in destroys the old fabric to make way for another new; this process must always balance, because when it is altered puts at risk the health of the skeleton and skull.

When dealing with decomposition (called osteoclasts) bone cells tend to be superior in number wings that produce it (osteoblasts), there is considerable decrease bone mineral density (composed by calcium and collagen, mainly), which causes bones to become porous, thin and fragile, therefore susceptible to fracture is with minimal effort. This problem is called osteoporosis and affects the elderly.

Also you can raise him the opposite phenomenon, i.e. that the osteoblasts number exceeds osteoclasts, allowing the accumulation of minerals, becoming too dense bones, called osteopetrosis, disease or bones of stone. The osteopetrosis, also known as Albers-Schφnberg disease is very rare, to the degree that has impact one between 20 thousand births, or occurs in one of every adult 200mil.

The most common cause is a genetic defect that causes lack of substance carbonic anidrasa II, to allow that osteoclasts are sufficient and/or work correctly. What happens then is that new bone accumulates and causes the skeleton is too heavy to be one of the most serious consequences leave little room for bone marrow (substance giving rise to the formation of blood cells) in each of its vertebrae.

It should be noted that this condition is inherited in two ways:

Recessive: Occurs when both parents are carriers of the defective gene, and characterized by manifest themselves in the newly born and be progressive, to the extent that leads to the death, closes the bones hosting bone marrow cavity, causing anemia very severe, deformations in the skull and compression of the nerves that staying there.

Dominant: One parent transmits the disease gene to their offspring, their effects more mild and with later events, allowing the patient to reach adulthood. Those who suffer have stature and bones that break easily.

The osteopetrosis has classified in three ways, which depends on desu severity and age in which presents:

• Evil children. This type is severe; manifests from birth and often ends with the lives of those affected before the age of 10.
• Intermediate. It is less serious than the previous and affects children under 10 years old.
• Adult. It is lighter than the previous; is evident between 20 and 40 years of age and rarely reduces life expectancy.

Common damage present in all three types of the disease include:

• Fractures, despite what you might think in the long bones (of the extremities) and ribs; this explains why the bone is hard has trouble moving, which hinders to the affected and endanger its balance and displacement.
• Anemia. It decreased hemoglobin in blood (protein found in red blood cells and carries oxygen from the lungs to the body of veins and arteries) levels. Due to bone marrow is found in very small footprint and this prevents the proper formation of blood cells.
• Frequent infections due to reduction and white blood cells (cells that fight against invading microorganisms); inactivity are often severe and can include pneumonia (affects the lungs) or sepsis (in blood), even in teeth and jaw.
• Absence or inadequate eruption of dental handpieces.
• Intracranial pressure increased, causing headache and loss of function of the cranial nerve, as a result, blindness, deafness and facial nerve paralysis.
• Broadening of the front of the skull.
• Hydrocephalus. Abnormal growth of head due to excess water.
• Poor growth or delayed development, which is usually related to the weight of the skeleton, causing difficulty to sit, walk and talk, all this despite having normal intelligence.
• An enlarged spleen, body located at the top of the abdomen, whose functions are delete blood waste materials and protect the body from external aggressions.

When you have more of the symptoms, this is confirmed by x-rays, which show very dense bones; also requested additional studies to detect other alterations include Visual, and auditory tests blood and tomography (shows the structures of certain organs at different levels) brain.

Once the endocrinologist (glands health specialist) or the hematologist (his specialty is blood) confirms the diagnosis of the disease, may recommend different types of treatment, which include:

Interferon Gamma. Substance of lymphocytes (white blood cells variant) that stimulates immune response or defence, delaying the time to progression of the disease and infection.
Bone marrow transplant. This procedure abnormal, osteoclasts can be replaced as they are in the marrow.
Cortisone. Administered orally increases the amount of cells in patients with anemia, and that can reduce the destruction of the same speed.
Occupational and physical therapy. Includes a number of specific exercises that encourage the development of children.

The osteopetrosis is a serious disease that only you can control, being affected adult patients have increased life expectancy, because less than 30% of children with malignant form survive his tenth birthday.


 

   

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