DR. RICHARD JORDAN

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DR. RICHARD JORDAN

• PROFESSOR OF MEDICINE AND PROGRAM DIRECTOR
• CHIEF, DIVISION OF ENDOCRINOLOGY AND METABOLISM

J. H. QUILLEN VA MEDICAL CENTER EAST TENESSEE
STATE UNIVERSITY JOHNSON CITY, TN
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MALE HYPOGONADISM
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Secondary Hypogonadism(Hypogonadotrophic
Hypogonadism)

• Kallmann Syndrome
• Idiopathic Gonadotrophin Deficiency
• Selective Gonadotrophin Deficiency
• Prader-Will Syndrome
• Acquired Gonadotrophin Deficiency
• (Continued)

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Secondary Hypogonadism(Hypogonadotrophic
Hypogonadism)

• Kallmann Syndrome
• Absent or Deficient GnRH (Mild to Severe)
• Eunuchoid with Variable Expression of
  Hypogonadism, Anosmia, or Hyposmia, R/G Color
• Blindness, Cleft Palate, GU Tract Abnormalities,
  Neurosensory Deafness
• Genetics Sporadic, Dominant, Recessive, X-linked
• Etiology Absent neural cell adhesion molecule
  (anosmin) in 0-14, KAL Gene Point Mutation
• (Continued)

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Secondary Hypogonadism(Hypogonadotrophic
Hypogonadism)

• Idiopathic Gonadotrophin Deficiency
• No Somatic Abnormalities, No Ansomia, No KAL,
• Gene Mutation, Abnormal GnRH Receptor in a Few
• Selective Gonadotrophin Deficiency
• Isolated LH Deficiency Fertile Eunuch, Absence
  Virilization with Spermatogenesis
• Isolated FSH Deficiency Somewhat Small Testis,
  Oligospermia to Azospermia, Normal Virilization
• (Continued)

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Secondary Hypogonadism(Hypogonadotrophic
Hypogonadism)

• Prader-Willi Syndrome
• Obesity, Hypotonia, Hypogonadotrophic
  Hypogonadism, Retardation, Small Hands and Feet
• (Continued)

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Secondary Hypogonadism(Hypogonadotrophic
Hypogonadism)

• Acquired Gonadotrophic Deficiency
• Prolactinoma
• Other Pituitary Tumors
• Hypothalamic Tumors
• Infiltrative Disorders
• Hemochromatosis
• Eosinophilic Granuloma
• Sarcoidosis

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Clinical Evaluation

• History
• Diminished Libido, Weakness, Erectile
  Dysfunction,
• Behavior Abnormalities, Systemic Illness,
  Headache,
• Visual Changes, Multiple Hormone Deficiencies,
• Chemotherapy, Radiation Rx, Orchitis, Toxin
  Exposure,
• Anosmia, Family History of Delayed Puberty
• (Continued)

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Clinical Evaluation

• Physical Examination
• Eunuchoid Proportions, Size of Testis,
  Gynecomastia,
• Characteristic Dysmorphic Features, Secondary Sex
  
• Characteristics Slow to Change

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Laboratory EvaluationGonadal Function

• Semen Analysis
• Sperm Count 20 million/ml, 40
  million/ejaculate, gt50 normal motility, gt30
  normal morphology
• TestosteroneTotal or Free?
• Decreased BindingObesity, Hypothyroidism,
  Acromegaly
• Increased BindingOld Age, Cirrhosis,
  Thyrotoxicosis
• Testosterone Lower in the Afternoon
• Gonadotropins
• LH Elevated with Leydig Cell Dysfunction
• FSH Elevated with Germinal Cell Dysfunction or
  Leydig Cell Dysfunction
• Gonadotrophins Normal or Low with Secondary
  Hypogonadism

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Therapy

• Secondary Hypogonadism
• 1. HCG1000u 3 times weekly
• HMG75u 3 times weekly
• 2. GnRH pulses (pulse q 90 min)
• Primary Hypogonadism
• 1. Testosterone Ester Injection
• 2. Testosterone Patch5 mg/day
• 3. Testosterone GelUpper Arms, Shoulders,
  Abdomen
• Side Effects
• Exacerbation of BPH, Occult Prostate Cancer,
  Erythrocytosis,
• Sleep Apnea

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Primary Hypogonadism(Hypergonadotrophic
Hypogonadism)

• Klinefelter Syndrome
• XX Male (Sex Reversal)
• Noonan Syndrome (Male Turner Syndrome)
• Myotonic Dystrophy
• Congenital Anorchia (Vanishing Testis Syndrome)
• Sertoli-Cell-Only Syndrome
• Acquired Germinal Cell Aplasia
• Orchitis
• Chronic Illnesses
• (Continued)

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Primary Hypogonadism(Hypergonadotrophic
Hypogonadism)

• Klinefelter Syndrome
• 46 XXY, 46 XY/XXY, 48 XXXY
• Eunuchoid lower segment, Taller than Average,
  Gynecomastia,
• Gynecoid Features, Very Small Testis, Normal to
  Low
• Testosterone, FSH increase gtLH, Modest Elevation
  of Estradiol,
• Severe Oligospermia to Azospermia
• Associated Conditions COPD, Cancer of Breast,
  Germ Cell Tumors, Autoimmune Diseases, Diabetes
  Mellitus, Osteopenia, Mitral Valve Prolapse,
  Mental Slowness, Antisocial Behavior
• (Continued)

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Primary Hypogonadism(Hypergonadotrophic
Hypogonadism)

• XX Male (Sex Reversal)
• Translocation of the SRY gene, Shorter than
  Average, Normal
• Intelligence, Gynecomastia, Small Testis,
  Azospermia
• Noonan Syndrome (Male Turner Syndrome)
• 46 XY, Short Stature, Webbed Neck, Shield
• Chest, Small Testis, Impaired
• Spermatogenesis, May Have Low Testosterone
• Associated Conditions Mental Retardation,
  Pulmonary Stenosis, Hypertrophic Cardiomyopathy,
  Tyrosine Phosphase Activation?
• (Continued)

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Primary Hypogonadism(Hypergonadotrophic
Hypogonadism)

• Myotonic Dystrophy
• Autosomal Dominant
• Inability to Relax Striated Muscle, Frontal
  Balding,
• Ptosis, Cataracts, Atrophy of Facial Muscles,
  Distal Muscle
• Wasting, Testicular Atrophy after Puberty
• Associated Conditions Cardiomyopathy with
  Conduction
• Abnormalities, Type II Diabetes Mellitus, Mental
  Retardation,
• Decreased Myotonin (transfers phosphate to ATP)
• (Continued)

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Primary Hypogonadism(Hypergonadotrophic
Hypogonadism)

• Congenital Anorchcia (Vanishing Testis Syndrome)
• 46XY, No Discernable Testicular Tissue in Most,
  Bilateral Testicular Torsion in Utero?
• HCG StimulationDetect Testicular Remnants
• Sertoli-Cell-Only Syndrome
• 46XY, Germinal Cell Aplasia, FSHgtLH
• Testosterone Normal
• Sertoli Cells VacuolatedFunctional Abnormality?
• (Continued)

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Primary Hypogonadism(Hypergonadotrophic
Hypogonadism)

• Acquired Germinal Cell Aplasia
• Chemotherapy, Radiation, Environmental Toxins
• (Dibromodichloralpropane)
• Orchitis
• Post-Pubertal Mumps 40 have Orchitis, 40 with
  Orchitis have Varying Degrees of Testicular
  Atrophy, Sperm Count Lower in Most with Atrophy
  but True Impaired Fertility in 15
• Autoimmune Orchitis Type I and II endocrine
  deficiency
• Chronic Illnesses
• Cirrhosis, Chronic Renal Failure, Long-Term
  Glucocorticoid Therapy