Hypogonadism in a pediatric patient may present with delayed puberty. One classification is based on serum levels of gonadropins. Low concentrations gonadotropins (hypogonadotropic) may be associated with a wide range of conditions.
Patient selection: delayed puberty with clinical evidence of hypogonadism
Initial testing: decreased serum levels of FSH and LH (hypogonadotropic)
Categories:
(1) permanent, associated with a genetic disorder affecting the CNS
(2) permanent, acquired disorder affecting the CNS
(3) functional, with transient delay in the hypothalamic-pituitary-gonadal (HPG) axis
Permanent, genetic conditions:
(1) Kallmann syndrome
(2) septo-optic dysplasia
(3) Prader-Willi syndrome
(4) Laurence-Moon and Bardet-Biedl syndromes
(5) CHARGE syndrome
(6) idiopathic
Permanent, acquired conditions:
(1) growth hormone deficiency
(2) tumor (pituitary, hypothalamic, central nervous system)
(3) Langerhans histiocytosis
(4) granulomatous or infectious CNS disorder
(5) CNS trauma or surgery
(6) CNS radiation
Functional conditions:
(1) hypothyroidism (not due to pituitary cause)
(2) diabetes mellitus
(3) cystic fibrosis
(4) inflammatory bowel disease (IBD)
(5) celiac disease
(6) juvenile rheumatoid arthritis
(7) systemic lupus erythematosus (SLE)
(8) hemoglobinopathy (sickle cell, thalassemia)
(9) chronic renal disease
(10) anorexia nervosa
(11) malnutrition
(12) intense exercise
