title: Hypothalamic growth hormone deficiency and supplementary GH therapy in two patients with mitochondrial myopathy, encephalopathy, lactic acidosis and stroke-like episodes.
abstract: Two pediatric patients with mitochondrial myopathy, encephalopathy, lactic acidosis, and stroke-like episodes were diagnosed with growth hormone deficiency with the primary lesion identified as the growth hormone-releasing factor producing cells of the hypothalamus. Stimulation tests with insulin, levodopa and sleep did not overcome the deficient pattern of growth hormone secretion. By comparison, the growth hormone-releasing factor stimulation test generated a normal growth hormone response in these two patients. Growth hormone supplementary therapy was effective in terms of growth gain without adverse effects.
authors.affiliation: Department of Pediatrics, Tokyo Women's Medical University, School of Medicine, Tokyo, Japan. ikyoku@ped.twmu.ac.jp
authors.forename: M
authors.initials: M
authors.lastname: Matsuzaki
authors.forename: T
authors.initials: T
authors.lastname: Izumi
authors.forename: K
authors.initials: K
authors.lastname: Shishikura
authors.forename: H
authors.initials: H
authors.lastname: Suzuki
authors.forename: Y
authors.initials: Y
authors.lastname: Hirayama
mesh_terms: D002648:Child; D002675:Child, Preschool; D005260:Female; D019382:Human Growth Hormone / Q000097:blood / Q000172:deficiency* / Q000627:therapeutic use*; D006801:Humans; D007028:Hypothalamic Hormones / Q000097:blood / Q000172:deficiency*; D017241:MELAS Syndrome / Q000097:blood / Q000150:complications* / Q000188:drug therapy*; D008297:Male; D008659:Metabolic Diseases / Q000097:blood / Q000188:drug therapy* / Q000209:etiology*; D011994:Recombinant Proteins / Q000627:therapeutic use*
chemical_list: D007028:Hypothalamic Hormones; D011994:Recombinant Proteins; D019382:Human Growth Hormone