asmi
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Which hormone is involved in laron dwarfism ?
Where is the defect ?
What hormonal and metabolic changes do we see in this disease ?
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| dxtxpx
Forum Guru
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Laron syndrome (LTD1), a rare genetic disorder, is caused by the body's inability to use the growth hormone (GH) that it produces. The problem lies not in the production of growth hormone but rather in a defective GH-receptor. This defect prevents the proper binding of the GH molecule leaving high levels of unbound growth hormone in the plasma.
LTD1 is characterized by short stature, delayed bone age and, less frequently, blue eyes and hip degeneration as well as high levels of circulating growth hormone.
A second form of the disorder known as Laron syndrome type II (LTD2) shows typical clinical features of the Laron syndrome but is due to a defect in the biochemical processing of growth hormone after the hormone has been bound on the cell surface.
Symptoms: severe but proportionate short stature (evident at birth or soon after), growth retardation, delays in tooth eruption, disproportion between the growth of the head and jaw, a saddle nose and deep-set eyes. Sexual development is slow but it does occur. The usual age of sexual maturation in boys with Laron syndrome is about 22 years of age. In females with the disorder, sexual maturation usually takes place between 16 to 19 years of age. Hands and feet are smaller than normal. Obesity and a high-pitched voice may also be present.
There are always high levels of growth hormone in the plasma of people with LTD1. A high percentage of patients have extremely low blood sugar levels (hypoglycemia) that can have very serious consequences if not treated promptly by a doctor.
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| asmi
Forum Hero
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thanx dxtxpx
so, to differentiate it with cretin dwarf....mian point will be mental retardation in cretins.Are there any other points to differentiate cretin from a laron dwarf ?
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