Exclusive Sertoli Cell Syndrome (ESCS) is a rare condition that has male infertility as its main consequence. It is one of the most serious forms of non-obstructive azoospermia, with a poor reproductive prognosis

专属支持细胞综合征(ESCS)是一种罕见疾病,其主要后果是男性不育。它是最严重的非梗阻性无精子症之一,生育预后不良。

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Abstract

Exclusive Sertoli Cell Syndrome (ESCS) is a rare condition that has male infertility as its main consequence. It is one of the most serious forms of non-obstructive azoospermia, with a poor reproductive prognosis. In some cases, however, such as the type II of the syndrome, sperm can be recovered through testicular puncture and subsequent ICSI, with a 13% success rate. This article aims to report the case of an azoospermic 35-year-old patient, with no other significant changes in complementary exams. 35 years old man with history of primary infertility, he was married since 17 years with normal sexual activity and and average built. Hematologic examination and biochemistry lab data were within normal limits. LH and total testosterone values were also within normal range, together with slightly elevated FSH value (119.88mUI/ml. There was azoospermia in the first semen analysis. Repeated semen analysis, 3 months later, revealed azoospermia. The karyotype of the patient is 46, XY with no Y chromosome micro deletions. Scrotal Color Doppler ultrasound and MRI examinations revealed that both testicles were in the left hemiscrotum. The lower testis had 53×26×43 mm dimensions and the posterosuperior located testis had 27×16×25 mm dimensions. Total abdominal ultrasonic examination revealed no developmental anomaly of urinary system. Family history of the patient revealed that mother and father were cousins no family history of infertility, the picture suggestive a case of sertoli only cell syndrome.

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