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Syndrome del Castillo
Last reviewed: 23.04.2024
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Syndrome del Castillo (Sertoli-cell syndrome) is an infrequent disease.
Patients in sexual and physical development do not differ from healthy men. Karyotype 46, XY.
Causes of the syndrome del Castillo
The cause of the development of the del Castillo syndrome has not yet been elucidated. Some researchers consider this syndrome to be congenital, others do not find any data on the role of genetic factors in its etiology. An unknown damaging factor selectively acts on the hermetic elements of testicles, which leads to atrophy of the seed epithelium. Sertoli cells are not damaged in this case. A similar pattern is observed in cases of severe diseases of the nervous system (eg, multiple sclerosis ), vertebral fractures, skull injuries, radiation injuries.
Symptoms of the syndrome del Castillo
Symptoms of the del Castillo syndrome - secondary sexual signs are developed normally. The reason for going to the doctor are complaints about infertility.
Forms
There are 2 variants of the syndrome: idiopathic and developing after combined radio- and chemotherapeutic treatment of tumors. In idiopathic syndrome, the testicles are hypoplastic due to a decrease in the size of the seminiferous tubules, whose diameter varies from 120 to 200 μm. Occasionally there are completely atrophied tubules, tunica propria thickens due to an increase in the number of collagen fibers. The bulk of the tubules is devoid of lumen, and the walls are covered with highly differentiated Sertoli cells.
Some of them are at different stages of degeneration. Leydig cells are extremely polymorphic: on average, 50% of patients have a normal number, the remaining 50% is more or less increased; from 40 to 80% of Leydig cells unchanged; 10-25% - are hypoplastic with a large number of cytoplasmic lipids; part of them with pronounced degenerative changes. Pathologically differentiated and immature Leydig cells are rare.
In the second version of the syndrome, the diameter of the tubules is from 150 to 190 μm, they are surrounded by an undulating basal membrane that wedges into the cytoplasm of the basal part of Sertoli cells. Tunica propria slightly thickened. Characterized by significant hyperplasia, often - hypertrophy of Leydig cells. Less often than in the first version, there are hypoplastic Leydig cells.
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Treatment of the syndrome del Castillo
Treatment of the syndrome del Castillo - usually patients in hormonal therapy do not need. Androgens are prescribed only if androgen deficiency is detected.
Forecast
Prognosis for fertility is unfavorable.
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