(Q78.1) Polyostotic fibrous dysplasia

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153 430 in individuals diagnosis polyostotic fibrous dysplasia confirmed
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2 369 deaths with diagnosis polyostotic fibrous dysplasia
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2% mortality rate associated with the disease polyostotic fibrous dysplasia

Diagnosis polyostotic fibrous dysplasia is diagnosed Men are 15.04% more likely than Women

88 252

Men receive the diagnosis polyostotic fibrous dysplasia

1 500 (1.7 %)

Died from this diagnosis.

100
95
90
85
80
75
70
65
60
55
50
45
40
35
30
25
20
15
10
5
0
65 178

Women receive the diagnosis polyostotic fibrous dysplasia

869 (1.3 %)

Died from this diagnosis.

Risk Group for the Disease polyostotic fibrous dysplasia - Men aged 10-14 and Women aged 5-9

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In Men diagnosis is most often set at age 0-79
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Less common in men the disease occurs at Age 80-95+Less common in women the disease occurs at Age 75-84, 90-95+
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In Women diagnosis is most often set at age 0-74, 85-89

Disease Features polyostotic fibrous dysplasia

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Absence or low individual and public risk
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Polyostotic fibrous dysplasia - what does this mean

Polyostotic fibrous dysplasia is a disorder of the bones caused by abnormal growth of fibrous connective tissue. this tissue replaces normal bone and can lead to deformities and fractures. it is caused by a mutation in the gnas gene, which is responsible for controlling the production of proteins involved in bone formation.

What happens during the disease - polyostotic fibrous dysplasia

Polyostotic fibrous dysplasia is a genetic disorder caused by a mutation in the gnas1 gene, which is responsible for the production of the gαs protein. this mutation results in abnormal bone formation, as the gαs protein is involved in the regulation of bone-forming cells. as a result, abnormal fibrous tissue replaces normal bone, leading to weakened bones and an increased risk of fracture.

Clinical Pattern

More details coming soon

How does a doctor diagnose

  • Physical examination
  • X-rays
  • CT scan
  • MRI scan
  • Bone scan
  • Blood tests
  • Biopsy

Treatment and Medical Assistance

Main Goal: To reduce pain and improve quality of life.
  • Prescription of pain medications
  • Physical therapy
  • Surgery to remove or replace affected bones
  • Radiation therapy
  • Hormone therapy
  • Osteotomies to correct deformities
  • Joint replacement
  • Bone grafting
  • Stem cell therapy
  • Rehabilitation
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10 Days of Hospitalization Required
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Average Time for Outpatient Care Not Established

Polyostotic fibrous dysplasia - Prevention

The best way to prevent polyostotic fibrous dysplasia is to maintain a healthy lifestyle. eating a balanced diet, exercising regularly, and avoiding smoking and excessive alcohol consumption can help reduce the risk of developing this condition. additionally, it is important to have regular check-ups with your doctor to ensure that any changes in bone structure can be detected early and treated appropriately.