Hypogonadotropic Hypogonadism

Aka: Hypogonadotropic Hypogonadism, Hypogonadotropic Causes of Delayed Puberty, Hypogonadotropic Causes of Primary Amenorrhea, Sexual Infantilism due to Gonadotropin Deficiency, Secondary Hypogonadism

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II. Definition: Hypogonadotropic Hypogonadism

  1. Decreased functional activity of ovaries or Testes
  2. Results from pituitary failure to secrete gonadotropins
  3. Results in delayed Sexual Development and Growth Delay

III. Etiology

  1. Central defect of Hypothalamic-Pituitary Axis
  2. Low gonadotropin

IV. General

  1. Late eunuchoid body proportions
  2. Grow normally during childhood
    1. Fail to have pubertal growth spurt
    2. Growth retardation is recent event
    3. Plot Growth Velocity!!

V. Causes: Endocrine

  1. Multiple tropic Hormone deficiency
  2. Isolated Growth Hormone (Somatotropin) deficiency
    1. Severe growth retardation (>3 SD from mean)
    2. Long duration of growth retardation since childhood
    3. Height Retardation exceeds Weight retardation
    4. Proportionate dwarfism
    5. Immature round face
    6. Mild truncal fat distribution
  3. Isolated Gonadotropin deficiency
    1. Kallmann's Syndrome
    2. Congenital adrenal hypoplasia
    3. Isolated LH or FSH deficiency
  4. Other endocrine disorders
    1. Hypothyroidism
    2. Diabetes Mellitus
    3. Glucocorticoid excess (Cushing Syndrome)
      1. Growth Delay mild if adrenal sex steroid increased
    4. Hyperprolactinemia

VI. Causes: CNS

  1. CNS insult due to infection, radiation or Trauma
  2. Brain Tumor
    1. Craniopharygioma
    2. Astrocytoma
    3. Hypothalamic glioma
    4. Pituitary tumor (e.g. Prolactinoma)

VII. Causes: Miscellaneous

  1. Prader-Willi Syndrome
    1. Obesity
    2. Short Stature
    3. Mild Mental Retardation
  2. Systemic Conditions
    1. Chronic gastrointestinal conditions
    2. Chronic Kidney Disease
    3. Gaucher's Disease
    4. Cystic Fibrosis
    5. Sickle Cell Anemia
    6. Human Immunodeficiency Virus infection
  3. Medications
    1. Chronic Opioid use (86% of users)
  4. Nutritional deficiency OR extreme Energy Expenditure
    1. Female Athlete Triad
    2. Psychogenic
      1. Weight Retardation exceeds Height Retardation
      2. Anorexia
      3. Bulimia
  5. Constitutional Delay in Growth and Development
    1. Compare siblings and parental growth
    2. Calculate target height from parental contribution

VIII. References

  1. Master-Hunter (2006) Am Fam Physician 73:1374-87 +PMID:16669559 [PubMed]

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Related Studies

Ontology: Hypogonadotropic hypogonadism (C0271623)

Definition (NCI) Abnormal ovarian or testicular function due to insufficient hormonal stimulation from the hypothalamic-pituitary axis.(NICHD)
Concepts Disease or Syndrome (T047)
MSH D007006
ICD10 E23.0
SnomedCT 67718002, 33927004
English Hypogonadotropic hypogonadism, HYPOGONADISM, ISOLATED HYPOGONADOTROPIC, hypogonadotrophic hypogonadism (diagnosis), hypogonadotrophic hypogonadism, secondary testicular failure (diagnosis), secondary testicular failure, Hypogonadotrophic hypogonadism, secondary hypogonadism, hypogonadotropic hypogonadism, HH7, Gonadotropin deficiency, HYPOGONADOTROPIC HYPOGONADISM 7 WITH OR WITHOUT ANOSMIA, Secondary hypogonadism (disorder), Hypogonadism, hypogonadotropic, Secondary Hypogonadism, Gonadotrophin deficiency, Secondary hypogonadism, Hypogonadotropic hypogonadism (disorder), deficiency; gonadotropin, gonadotropin; deficiency, Secondary hypogonadism, NOS, Secondary hypogonadism -RETIRED-, Hypogonadotropic Hypogonadism, Hypogonadism, Hypogonadotropic
Italian Deficit di gonadotropina, Ipogonadismo secondario, Ipogonadismo ipogonadotropo
Dutch hypogonadotroop hypogonadisme, deficiëntie; gonadotropine, gonadotropine; deficiëntie, gonadotropinedeficiëntie, secundair hypogonadisme
German hypogonadotropher Hypogonadismus, Gonadotropinmangel, sekundaerer Hypogonadismus
Portuguese Hipogonadismo hipogonadotrófico, Hipogonadismo secundário, Insuficiência de gonadotrofinas
Spanish Hipogonadismo hipogonadotrófico, hipognonadismo hipogonadotrófico, hipogonadismo hipogonadotrófico, hipognonadismo hipogonadotrófico (trastorno), hipogonadismo hipogonadotrófico (trastorno), hipogonadismo secundario - RETIRADO - (concepto no activo), hipogonadismo secundario - RETIRADO -, hipogonadismo secundario, Hipogonadismo secundario, Déficit de gonadotropinas
Japanese ゴナドトロピン欠乏症, 続発性性腺機能低下, 低性腺刺激ホルモン性性腺機能低下症, ｺﾞﾅﾄﾞﾄﾛﾋﾟﾝｹﾂﾎﾞｳｼｮｳ, ｿﾞｸﾊﾂｾｲｾｲｾﾝｷﾉｳﾃｲｶ, ﾃｲｾｲｾﾝｼｹﾞｷﾎﾙﾓﾝｾｲｾｲｾﾝｷﾉｳﾃｲｶｼｮｳ
French Hypogonadisme hypogonadotrophique, Déficit gonadotrope, Hypogonadisme secondaire
Czech Gonadotropinový deficit, Sekundární hypogonadismus, Hypogonadotropní hypogonadismus
Hungarian Gonadotropin hiány, Másodlagos hypogonadismus, Hypogonadotrophiás hypogonadismus
Derived from the NIH UMLS (Unified Medical Language System)

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