Laron syndrome: clinic, diagnostics (а clinical case)
Keywords:Laron’s syndrome, low growth, growth hormone, insulin-like growth factor, clinic, diagnosis
The combination of normal/high levels of somatotropic hormone with low levels of insulin-like growth factor-1 is characteristic of impaired receptor sensitivity to somatotropic hormone, a rare genetically determined syndrome described by Israeli clinician Z. Laron. In addition to the relative deficiency of somatotropic hormone, in contrast to pituitary dwarfism, other functions of the pituitary gland are not changed. Against the background of a sharp lag in growth from an early age, sometimes from the fetal period, children retain normal body proportions. The case of rare endocrinopathy — genetically determined Laron’s syndrome is described. Ethiopathogenesis, features of clinical course of disease and prospects of therapeutic approach are considered. The clinical signs of the main forms of growth retardation are presented. The diagnosis of Laron’s syndrome is made on the basis of short stature in the absence of other endocrinopathies, low levels of insulin-like growth factor-1 with a normal reference value of somatotropic hormone and no effect of treatment with somatotropic hormone. The patient needs further observation by an endocrinologist before the onset of puberty and, if necessary (in the case of the formation of inferiority complex), іn psychological correction. General recommendations on the completeness of the diet, sleep, physical activity and physical therapy are given. Prescribed multivitamins. Attempts to use insulin-like growth factor-1 genetically engineered drugs are encouraging. When making a differential diagnosis between the main forms of growth retardation and stunted growth, the family physician and endocrinologist should be prescribed hormonal blood tests and imaging methods according to the previous indicative diagnosis according to clinical data.
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