Table 3

Causes of primary hypogonadism in males (low testosterone, high LH)

Congenital

  1. Klinefelter's syndrome and variants (47,XXY, 48XXXY, 49XXXXY, 46,XY and 47,XXY mosaicism)

  2. 46,XY/XO mosaicism

  3. Inactivating mutation of steroidogenic enzymes (STAR Protein, CYP11A1, HSD3B2, CYP17, HSD17B3, SRD5A2)

  4. LH receptor mutations

  5. Uncorrected Cryptorchidism

  6. Myotonic Dystrophy

  7. Bilateral Congenital Anorchia

  8. Testicular Adrenal Rest Tumors

Acquired

  1. Orchitis (infection, radiation)-

  2. Testicular trauma/torsion

  3. Polyglandular Autoimmune disease

  4. Chemotherapy (suramin, alkylating agents)

  5. Drugs (Ketoconazole, Spironolactone, Abiraterone, Alcohol)

  6. Cryptorchidism

  7. Radiation

  8. Bilateral Orchiectomy

  9. Systemic Disorders (Cancer, Lymphoma, Amyloidosis)