الفهرس 
ThalassemiaOnly 14 pages are availabe for public view
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Abstract
The thalassemia syndromes constitute the most prevalent
of all known genetic diseases. About 3% of world’s population
carry 13-thalassemia genes (Lukens, 1993).
Each year, 100,000 children throughout the world are
born with thalassemia major (Esposito, 1992).
Along the last 15 years, thalassemia represented 40% of
the hematological problems in children attending the
Hematology/ Oncology Clinic, Children Hospital, Ain Shams
University (Imam, 1994).
Despite regular blood transfusion and chelation therapy,
growth failure and delayed sexual maturation continued to be
observed in a significant proportion of adolescent patients
with j3--thalassemia major (Borgna-Pignatti eta/., 1993).
Homozygous 13-thalassemia is associated with significant
skeletal abnonmalities including osteopenia (Orvietto
eta/., 1992).
As a consequence of chronic iron overload, many
endocrinopathies may occur in 13-thalassemia major patients.
The most frequent endocrine dysfunction is hypogonadotropic
hypogonadism (Kwan eta/., 1995).
Hypogonadism seems to play an important role in the
development of osteopenia-osteoporosis in 13-thalassemia
major patients (Anapliotou eta/., 1995). Brandle et a/. (1996) found that hypogonadotropic
hypogonadism is mainly responsible for osteopenia in as
much as 80% of thalassemia patients.
Although the etiology of bone disease is still debatable,
many factors can adversely affect bone accretion m
13-thalassemia major patients (Soliman et at., 1998).
Osteoporosis is generally thought to develop because of
inadequate bone formation and I or excessive bone
resorption. In thalassemia major, the exact mechanisms
operating in the development of osteopenia are unclear and
probably multifactorial (Anapliotou eta/., 1995).
Anapliotou et a/. (1995) concluded from their study that
hypogonadism plays an important role in the development of
osteopenia-osteoporosis in thalassemia major and
continuous hormone replacement with transdermal estrogen
for females or human chorionic gonadotropine for males best
improves the bone density parameters