Name
FERTILE EUNUCH SYNDROME
DESCRIPTION
DETAIL
CAUSED BY INCOMPLETE FORM OF GONADOTROPIN RELEASING HORMONE DEFICIENCY IN MEN D.D. : - FROHLICH SYNDROME - PRADDER WILLI SYNDROME - KLINEFELTER SYNDROME - XX MALE - PRIMARY HYPOPITUITARISM OR SECONDARY TO HYPOTHALAMIC LESIONS - PANHYPOPITUITARISM - ISOLATED GONADOTROPIN DEFICIENCY - ADRENAL HYPOPLASIA CONGENITA - CUSHING SYNDROME - HYPERPROLACTINEMIA - HEMOCHROMATOSIS - VIRAL ORCHITIS - TRAUMA - RADIATION - DRUGS - SPIRONOLACTONE, ALCOHOL, KETOCONAZOLE , CYCLOPHOSPHAMIDE - ENVIRONMENTAL TOXINS - AUTOIMMUNITY - GRANULOMATOUS DISEASE 2. HYPOGONADISM ASSOCIATED WITH SYSTEMIC DISEASE : - CIRRHOSIS LIVER - CHR RENAL FAILURE - SICKLE CELL ANEMIA - PROTEIN - ENERGY MALNUTRITION - ADVANCED HODGKINS DISEASE & CANCERS - AMYLOIDOSIS - ANDROGEN RESISTANCE - IMMUNE DISEASES LIKE AIDS , RHEUMATOID ARTHRITIS - NEUROLOGIC DISEASES LIKE MYOTONIC DYSTROPHY , SPINOBULBAR MUSCULAR ATROPHY , PARAPLEGIA -------------------------------------------------------------------------- ANOTHER CLASSIFICATION OF CAUSES : 1. PRIMARY HYPOGONADISM ( DUE TO TESTICULAR DISORDERS ) A. CONGENITAL CAUSES - KLINEFELTER SYNDROME - MICRODELETIONS OF AZOOSPERMIA FACTOR ( AZF ) REGIONS OF YP TELOMERE( 15 % OF MEN WITH NONOBSTRUCTIVE AZOOSPERMIA; 5-10% OF THOSE WITH OLIGOSPERMIA - CRYPTORCHIDISM - MYOTONIC DYSTROPHY - CONGENITAL ADRENAL DYSPLASIA DUE TO * 21- HYDROXYLASE DEFICIENCY ( MOST COMMON DEFECT ) * 11-BETA-HYDROXYLASE DEFICIENCY * 3-BETA HYDROXYSTEROID DEHYDROGENASE DEFICIENCY - ANDROGEN RECEPTOR GENE MUTATION ( QUALITATIVE OR QUANTITATIVE ) - LH RECEPTOR MUTATIONS( MALE PHENOTYPE, IF MILD; FEMALE PHENOTYPE, IF SEVERE) B. ACQUIRED CAUSES - CANCER THERAPY : CHEMO THERAPY OR RADIOTHERAPY - DRUGS ( KETOCONAZOLE ) - TRAUMA - INFILTRATIVE DISEASE ( HEMOCHROMATOSIS ) - INFECTIONS ( HIV, MUMPS ORCHITIS ) - SYSTEMIC ILLNESS LIKE UREMIA, CIRRHOSIS 2. SECONDARY HYPOGONADISM A . CONGENITAL : SEXUAL DIFFERENTIATION IS NORMAL. IN MEN PHALLIC DEVELOPMENT MAY BE SUBNORMAL - ISOLATED IDIOPATHIC HYPOGONADOTROPIC HYPOGONADISM - KALLMANN SYNDROME - IDIOPATHIC HYPOGONADOTROPIC HYPOGONADISM ASSOCIATED WITH MENTAL RETARDATION * PRADER-WILLI SYNDROME - CRANIOPHARYNGIOMA - COMBINED PITUITARY HORMONE DEFICIENCY - FERTILE EUNUCH SYNDROME - ABNORMAL BETA SUBUNIT OF LH - ABNORMAL BETA SUBUNIT OF FSH - CONGENITAL ADRENAL HYPERPLASIA B. ACQUIRED CAUSES - VARIOUS ANATOMIC LESION OF PITUITARY LIKE * BENIGN TUMORS OR CYSTS, MALIGNANT TUMORS, VASCULAR ANEURYSM * INFILTRATIVE DISEASES LIKE HEMOCHROMATOSIS, SARCOIDOSIS, HISTIOCYTOSIS, LYMPHOMA * PITUITARY HAEMORRHAGE * PITUITARY TRAUMA * RADIATION INJURY * INFECTIONS LIKE MENINGITIS * IATROGENIC LIKE USE OF NARCOTIC ANALGESICS OR ABUSE OF ANABOLIC STEROIDS BY ATHLETES,GONADOTROPIN RELEASING HORMONE ANALOGS IN PROSTATE CANCER * GLUCOCORTICOID EXCESS : EXOGENIC OR ENDOGENIC ( CUSHING SYNDROME ) * HYPERPROLACTINEMIA * PRIMARY HYPOTHYROIDISM * EXCESSIVE EXCERCISES * SEX-STEROID SECRETING TUMORS * EMPTY SELLA SYNDROME * CHR SYSTEMIC DISEASES * ANOREXIA NERVOSA * ACUTE ALCOHOL INGESTION * IDIOPATHIC SERUM GONADOTROPIN RELEASING HORMONE - LOW * SERUM LH LEVELS - LOW * SERUM FSH LEVELS - NORMAL * SERUM TOTAL TESTOSTERONE LEVELS - LOW * SEMEN ANALYSIS - LOW OR NORMAL SPERM COUNT
TYPENOTES
RELATED DISEASE
Not Available Disease
DISEASE
INVESTIGATION
SEMEN ANALYSIS, FSH ( MALE ), LH ( MALE ), SERUM TESTOSTERONE( TOTAL ) FOR FEMALE, COMPLETE BLOOD COUNT