dr. richard jordan
DESCRIPTION
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. MALE HYPOGONADISM. Secondary Hypogonadism (Hypogonadotrophic Hypogonadism). Kallmann Syndrome - PowerPoint PPT PresentationTRANSCRIPT
DR. RICHARD JORDANDR. 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
MALE MALE HYPOGONADISMHYPOGONADISM
Secondary HypogonadismSecondary Hypogonadism(Hypogonadotrophic Hypogonadism)(Hypogonadotrophic Hypogonadism)
Kallmann Syndrome
Idiopathic Gonadotrophin Deficiency
Selective Gonadotrophin Deficiency
Prader-Will Syndrome
Acquired Gonadotrophin Deficiency
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Secondary HypogonadismSecondary Hypogonadism(Hypogonadotrophic 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
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Secondary HypogonadismSecondary Hypogonadism(Hypogonadotrophic 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
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Secondary HypogonadismSecondary Hypogonadism(Hypogonadotrophic Hypogonadism)(Hypogonadotrophic Hypogonadism)
Prader-Willi Syndrome
Obesity, Hypotonia, Hypogonadotrophic Hypogonadism, Retardation, Small Hands and Feet
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Secondary HypogonadismSecondary Hypogonadism(Hypogonadotrophic Hypogonadism)(Hypogonadotrophic Hypogonadism)
Acquired Gonadotrophic Deficiency
Prolactinoma
Other Pituitary Tumors
Hypothalamic Tumors
Infiltrative Disorders
Hemochromatosis
Eosinophilic Granuloma
Sarcoidosis
Clinical EvaluationClinical 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
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Clinical EvaluationClinical Evaluation
Physical Examination
Eunuchoid Proportions, Size of Testis, Gynecomastia,
Characteristic Dysmorphic Features, Secondary Sex
Characteristics Slow to Change
Laboratory EvaluationGonadal Function
Semen AnalysisSperm Count – 20 million/ml, 40 million/ejaculate, >50% normal motility, >30% normal morphology
Testosterone—Total or Free?Decreased Binding—Obesity, Hypothyroidism, AcromegalyIncreased Binding—Old Age, Cirrhosis, ThyrotoxicosisTestosterone Lower in the Afternoon
GonadotropinsLH: Elevated with Leydig Cell DysfunctionFSH: Elevated with Germinal Cell Dysfunction or Leydig Cell DysfunctionGonadotrophins: Normal or Low with Secondary Hypogonadism
TherapyTherapySecondary Hypogonadism1. HCG—1000u 3 times weekly HMG—75u 3 times weekly2. GnRH pulses (pulse q 90 min)
Primary Hypogonadism1. Testosterone Ester Injection2. Testosterone Patch—5 mg/day3. Testosterone Gel—Upper Arms, Shoulders, Abdomen
Side EffectsExacerbation of BPH, Occult Prostate Cancer, Erythrocytosis,Sleep Apnea
Primary Hypogonadism(Hypergonadotrophic Hypogonadism))
Klinefelter SyndromeXX Male (Sex Reversal)Noonan Syndrome (Male Turner Syndrome)Myotonic DystrophyCongenital Anorchia (Vanishing Testis Syndrome)Sertoli-Cell-Only SyndromeAcquired Germinal Cell AplasiaOrchitisChronic Illnesses
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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 >LH, 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
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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?
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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)
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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 Stimulation—Detect Testicular Remnants
Sertoli-Cell-Only Syndrome
46XY, Germinal Cell Aplasia, FSH>LH
Testosterone Normal
Sertoli Cells Vacuolated—Functional Abnormality?
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Primary Hypogonadism(Hypergonadotrophic Hypogonadism)
Acquired Germinal Cell AplasiaChemotherapy, Radiation, Environmental Toxins(Dibromodichloralpropane)
OrchitisPost-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 IllnessesCirrhosis, Chronic Renal Failure, Long-Term Glucocorticoid Therapy