TALL STATURE

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  1.  Etiologies

        a. Familial tall stature (parents and other close relatives are tall)

        b. Exogenous obesity (tall during early years, but do not typically end up tall)

        c  Endocrinological cause

          (1) Sexual precocity (again tall early on if untreated, but end up short if either

          (2) Hyperthyroidism (grow rapidly prior to institution of treatment and then, with

                 establishment of  euthyroidism, grow slowly returning to genetic height channel; may actually lose height potential if hyperthyroid for substantial period of time)

    (3) Gigantism (GH excess secondary to pituitary tumor - very rare)

    (4) Males with either estrogen deficiency (aromatase deficiency) or

    estrogen receptor deficiency (implies that estrogen action is

    more important than androgen action insofar as ultimate

    fusion of epiphyses is concerned)

    d. Genetic disorders

    (1) Kilnefelter syndrome [usually 47,XXY, with reduced IQ; testes <5 mL in volume as adults leading to decreased testosterone

     

    roduction by Leydig cells and infertility with seminiferous

    tubules (bulk of testes) replaced by hyalinization and fibrosis; and gynecomastia]

    (2) Marfan syndrome (arachnodactyly, joint laxity, scoliosis, upward lens dislocation, and aortic dilatation and aneurysm)

    (3) Martinoid body habitus may occur in association with type 2 multiple endocrine adenomatosis (familial medullary thyroid cancer, parathyroid adenoma and hyperplasia, and mucosal neuromas)

    (4)  Homocystinuria (mental retardation, joint contractures, downward lens dislocation, propensity for thromboembolic phenomena, in

    association with excessive urinary homocystine excretion)

    (5) Sotos syndrome (cerebral gigantism): autosomal dominant overgrowth (>97th %ile) characterized by macrocrania, large

    hands and feet, and mental retardation with no known etiology

    2. Treatment

    a. Treat underlying cause if possible, e.g., obesity - lose weight; sexual precocity - gonadotropin releasing-hormone (GnRH) analog; hyperthyroidism - thionamide medication (PTU or methimazole); gigantism with surgery

    b. High-dose estrogen (for females) or testosterone (for males) to cause rapid epiphysial maturation (however, potential high for side effects)