Tuesday, November 28, 2017

Albright's syndrome.. Severe bone changes are accompanied by constant persistent pain with satisfactory anatomical manifestations and minor abnormalities



Albright's syndrome:
Causes:
The cause of this unknown syndrome is full syndrome symptoms with early puberty occurring only in females.
Clinical manifestations:
Skin manifestations:
- Skin pigmentation usually occurs between the ages of 4 months and two years, but may occur at birth in the form of light brown complex densities, often with irregular or circular edge, appear mainly on the trunk or buttocks and thighs, but rarely affect the face and neck, Be symmetrical and may be more intense on the sides.
- Bone manifestations: Bone changes are accompanied by severe persistent pain with painful anatomical manifestations and secondary abnormalities.
- Ocular manifestations: Lupus (upper eyelid drop) and a turbulent vision caused by overgrowth of the bone base of the skull.
Blood assets:
Serum calcification and phosphorus are normal, but alkaline phosphatase is high if multiple bone lesions.
General features:
- In females: early attainment of large breasts, vaginal hemorrhage and pubic hair growth. Occur less than 5 years in about 25% of the cases and between 510 years in 30%.
- Growth during childhood is accelerating but the immature unit is immature. Other developmental abnormalities may be associated with this syndrome.
- Good life warning and normal pathological manifestations.
Diagnosis:
- There is no single clinical appearance of pigmented lesions that can differentiate Albright's syndrome from fibroblastoma, where bone disorders and endocrine glands may occur.
- The presence of several smears (coffee spots) with milk nectars in the armpits and nodules on the iris is a characteristic of neurofibromatosis. The pigmented granules are rarely found in Albright syndrome.