Insufficiency of the anterior pituitary gland , which arose in childhood .Pituitary dwarfism , pituitary microsomia
Endocrinology / / May 20, 2016
pituitary dwarfism - disease characterized by low growth and lack of puberty due to insufficient production in the anterior pituitary gonadotropins and somatotropin.
Etiology pituitary dwarfism
reason for failure of the anterior pituitary in children, as in adults, may be damaged dientsefalo-pituitary region tumors (kraniofaringeomoy, chromophobe adenoma, glioma), infectious process, infectious and non-infectious granuloma (Gunma,ksantamatozom) injury.However, only a small number of patients with pituitary dwarfism is possible to identify the pathological process, which was the reason for the lack of production of pituitary hormones.It can be assumed that those patients who are unable to establish a pathological process leading to the anterior pituitary failure, the cause of the disease is not the fault of the pituitary gland, and the defeat of diencephalic area, such as encephalitis.
pathogenesis of pituitary dwarfism
The pathogenesis of pituitary dwarfism is insufficient production of gr
high content of growth hormone found in the umbilical cord blood of newborns, and its number is significant in the first 8 weeks of life.Clinically stunting in pituitary dwarfs is often noticeable only over the age of 1-3 years.This allowed suggest that early childhood growth can not depend on the growth hormone, but it is largely as a result of internal growth trend fabrics.
The term "pituitary dwarfism" embedded picture of growth retardation associated mainly with the production of growth hormone deficiency.However, we can assume that at least some patients in the pathogenesis of the disease is important as implicit or explicit failure of production of thyroid-stimulating, adrenocorticotropic and gonadotropin-releasing hormone.
Pathology pituitary dwarfism
When morphological study in a number of cases identified anatomical changes associated with the process that caused the destruction of the anterior pituitary gland - tumor, granuloma, inflammation, bleeding.However, in many cases it is possible to detect only the pituitary hypoplasia;anecdotal in anatomical changes in the pituitary and hypothalamus nonexistent.
thyroid gland, gonads and adrenal glands hypoplastic or are in a state of atrophy.
internal organs are reduced in size in accordance with the general growth retardation.
When X-ray examination revealed delayed skeletal maturation.
clinical picture pituitary dwarfism
Most patients with pituitary dwarfism are born with a low height and weight and growth retardation observed in them in the first years of life.However, some patients are born with normal weight and growth retardation is their noticeable only after 1-4 years of life.
Build pituitary dwarfs proportional, but retains the proportions typical of children age - the predominance of the body length of the limb length.The degree of growth delay varies.It is considered dwarf growth of less than 130 cm. The height of the pituitary dwarfs is often significantly lower, not reaching 100 cm.
In children, the skin is usually thin and delicate, but in older children and adults pituitary dwarfs skin is usually pale, yellowish, dry,flabby and wrinkled.Hair thin head.Beard and mustache adult male dwarfs, as well as the vegetation on the body in patients of both sexes are missing.
development of subcutaneous fat in childhood is generally weak, and body weight corresponds to an increase or reduced.At older ages, it is often a moderate overweight (see Figure) At the same time damage the pituitary gland and hypothalamus, may develop obesity.
muscular system is underdeveloped, reduced muscle strength.
Bones thin and relatively short, keeps baby's skull proportions.There is a progressive skeletal maturation lag - "bone" age of the "passport".
heart, lungs, digestive organs of small size, but the signs of their features are usually absent.Blood pressure is often reduced, there is a tendency to bradycardia.Mental development is normal pituitary dwarfs.However, on their psyche affects the fact that due to the low growth in childhood they are playing with younger children, meet patronizing attitude from others both in childhood and in later life.Therefore psyche pituitary dwarfs retains the features of infantilism.
dramatically underdeveloped reproductive system.The dimensions of the external genitalia in males remain as children of the corresponding growth, sometimes nespuschenie testicles - cryptorchidism.The internal reproductive organs in women strongly hypoplastic.Secondary sexual characteristics and menstruation absent.Voice remains high, childish.
Despite the fact that the production of thyroid-stimulating hormone and adrenocorticotropic in pituitary dwarfs is usually reduced, clinical manifestations of hypothyroidism and adrenal insufficiency usually are mild or absent, and in some patients, they can only be detected using special hormonal studies.
deficiency of thyroid clinically in some patients bradycardia, and dry skin, adrenal insufficiency - hypotension and decreased resistance to infection and injury.
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Highlight 17-ketosteroidov and urine iodine levels associated with blood proteins and capture of radioactive iodine by the thyroid gland is usually located on the lower limit of normal, or below it.Adrenocortical insufficiency due to the reduced production of ACTH, many patients exhibit reduced tolerance to water stress.Research adrenocorticotropic pituitary reserves produced in 13 gipopituitarnyh Aarskog dwarfs et al.(1965), found in 10 of them the reduced reaction to the introduction of SU-4885.However, 12 of 13 patients had improvement of 17 glucocorticoids in the blood and increase their excretion in urine after administration of pyrogens.These data show that, although the ability dientsefalo-pituitary system of these patients respond to an increase in ACTH production to reduce cortisol in the blood is reduced, they are usually stored adrenocorticotropic sufficient reserves to ensure a stress reaction.
if damaged diencephalic region, some patients develop symptoms of diabetes insipidus.
Along with the symptoms associated with hormone deficiency, patients pituitary dwarfism in which the disease is associated with tumor or inflammation in dientsefalo-pituitary region, there are headaches due to increased intracranial pressure and bitemporal visual field loss due to pressure on the opticnerves.
pituitary dwarfs have often hypercholesterolemia.In the group of patients, 26 people in 18 blood cholesterol was above 300 mg.In some cases it is fasting hypoglycemia, increased insulin sensitivity.