A super long-acting LH-RH analogue induces regression of hypothalamic hamartoma associated with precocious puberty. 1995

K Harada, and J Yoshida, and T Wakabayashi, and H Okabe, and K Sugita
Department of Neurosurgery, Nagoya University School of Medicine, Japan.

We treated a 1-year-old female with a hypothalamic hamartoma and precocious puberty with leuprolide acetate depot, a super long-acting hormone-releasing hormone analogue (Tap-144-SR; [D-Leu6-[des-Gly10-NH2] LH-RH ethylamide acetate). The infant's major symptoms were genital bleeding and gynaecomastia. The LH-RH analogue (30 micrograms/kg) was injected subcutaneously once every 4 weeks. Clinical and laboratory manifestations of precocious puberty showed marked improvement. A follow-up after 16 months of treatment, the size of the tumour decreased significantly and remained unchanged for 2 years of further follow-up. To the best of our knowledge, this is the first hypothalamic hamartoma case in whom a decrease of tumour size under treatment with LH-RH analogue has been documented. But, because diagnosis of hamartoma is only based on neuroradiological and not on histological examinations, the possibility of a gangliocytoma cannot be excluded with certainty.

UI MeSH Term Description Entries
D007027 Hypothalamic Diseases Neoplastic, inflammatory, infectious, and other diseases of the hypothalamus. Clinical manifestations include appetite disorders; AUTONOMIC NERVOUS SYSTEM DISEASES; SLEEP DISORDERS; behavioral symptoms related to dysfunction of the LIMBIC SYSTEM; and neuroendocrine disorders. Froehlich's Syndrome,Hypothalamic-Neurohypophyseal Disorders,Pituitary Diencephalic Syndrome,Hypothalamic Dysfunction Syndromes,Hypothalamic Dysinhibition Syndrome,Hypothalamic Overactivity Syndrome,Hypothalamic Pseudopuberty,Hypothalamic-Adenohypophyseal Disorders,Diencephalic Syndrome, Pituitary,Diencephalic Syndromes, Pituitary,Disease, Hypothalamic,Diseases, Hypothalamic,Disorder, Hypothalamic-Adenohypophyseal,Disorder, Hypothalamic-Neurohypophyseal,Disorders, Hypothalamic-Adenohypophyseal,Disorders, Hypothalamic-Neurohypophyseal,Dysfunction Syndrome, Hypothalamic,Dysfunction Syndromes, Hypothalamic,Dysinhibition Syndrome, Hypothalamic,Dysinhibition Syndromes, Hypothalamic,Froehlich Syndrome,Froehlichs Syndrome,Hypothalamic Adenohypophyseal Disorders,Hypothalamic Disease,Hypothalamic Dysfunction Syndrome,Hypothalamic Dysinhibition Syndromes,Hypothalamic Neurohypophyseal Disorders,Hypothalamic Overactivity Syndromes,Hypothalamic Pseudopuberties,Hypothalamic-Adenohypophyseal Disorder,Hypothalamic-Neurohypophyseal Disorder,Overactivity Syndrome, Hypothalamic,Overactivity Syndromes, Hypothalamic,Pituitary Diencephalic Syndromes,Pseudopuberties, Hypothalamic,Pseudopuberty, Hypothalamic,Syndrome, Froehlich's,Syndrome, Hypothalamic Dysfunction,Syndrome, Hypothalamic Dysinhibition,Syndrome, Hypothalamic Overactivity,Syndromes, Hypothalamic Dysfunction,Syndromes, Hypothalamic Dysinhibition,Syndromes, Hypothalamic Overactivity,Syndromes, Pituitary Diencephalic
D007223 Infant A child between 1 and 23 months of age. Infants
D007279 Injections, Subcutaneous Forceful administration under the skin of liquid medication, nutrient, or other fluid through a hollow needle piercing the skin. Subcutaneous Injections,Injection, Subcutaneous,Subcutaneous Injection
D007987 Gonadotropin-Releasing Hormone A decapeptide that stimulates the synthesis and secretion of both pituitary gonadotropins, LUTEINIZING HORMONE and FOLLICLE STIMULATING HORMONE. GnRH is produced by neurons in the septum PREOPTIC AREA of the HYPOTHALAMUS and released into the pituitary portal blood, leading to stimulation of GONADOTROPHS in the ANTERIOR PITUITARY GLAND. FSH-Releasing Hormone,GnRH,Gonadoliberin,Gonadorelin,LH-FSH Releasing Hormone,LHRH,Luliberin,Luteinizing Hormone-Releasing Hormone,Cystorelin,Dirigestran,Factrel,Gn-RH,Gonadorelin Acetate,Gonadorelin Hydrochloride,Kryptocur,LFRH,LH-RH,LH-Releasing Hormone,LHFSH Releasing Hormone,LHFSHRH,FSH Releasing Hormone,Gonadotropin Releasing Hormone,LH FSH Releasing Hormone,LH Releasing Hormone,Luteinizing Hormone Releasing Hormone,Releasing Hormone, LHFSH
D011629 Puberty, Precocious Development of SEXUAL MATURATION in boys and girls at a chronological age that is 2.5 standard deviations below the mean age at onset of PUBERTY in the population. This early maturation of the hypothalamic-pituitary-gonadal axis results in sexual precocity, elevated serum levels of GONADOTROPINS and GONADAL STEROID HORMONES such as ESTRADIOL and TESTOSTERONE. Familial Precocious Puberty,Idiopathic Sexual Precocity,Precocious Puberty,Precocious Puberty, Central,Precocious Puberty, Male Limited,Precocious Puberty, Male-Limited,Pubertas Praecox,Sexual Precocity,Testotoxicosis,Central Precocious Puberties,Central Precocious Puberty,Familial Precocious Puberties,Idiopathic Sexual Precocities,Male-Limited Precocious Puberties,Male-Limited Precocious Puberty,Praecox, Pubertas,Precocious Puberties,Precocious Puberties, Central,Precocious Puberties, Familial,Precocious Puberties, Male-Limited,Precocious Puberty, Familial,Precocities, Idiopathic Sexual,Precocities, Sexual,Precocity, Idiopathic Sexual,Precocity, Sexual,Puberties, Central Precocious,Puberties, Familial Precocious,Puberties, Male-Limited Precocious,Puberties, Precocious,Puberty, Central Precocious,Puberty, Familial Precocious,Puberty, Male-Limited Precocious,Sexual Precocities,Sexual Precocities, Idiopathic,Sexual Precocity, Idiopathic
D003692 Delayed-Action Preparations Dosage forms of a drug that act over a period of time by controlled-release processes or technology. Controlled Release Formulation,Controlled-Release Formulation,Controlled-Release Preparation,Delayed-Action Preparation,Depot Preparation,Depot Preparations,Extended Release Formulation,Extended Release Preparation,Prolonged-Action Preparation,Prolonged-Action Preparations,Sustained Release Formulation,Sustained-Release Preparation,Sustained-Release Preparations,Timed-Release Preparation,Timed-Release Preparations,Controlled-Release Formulations,Controlled-Release Preparations,Extended Release Formulations,Extended Release Preparations,Slow Release Formulation,Sustained Release Formulations,Controlled Release Formulations,Controlled Release Preparation,Controlled Release Preparations,Delayed Action Preparation,Delayed Action Preparations,Formulation, Controlled Release,Formulations, Controlled Release,Prolonged Action Preparation,Release Formulation, Controlled,Release Formulations, Controlled,Sustained Release Preparation,Timed Release Preparation,Timed Release Preparations
D004334 Drug Administration Schedule Time schedule for administration of a drug in order to achieve optimum effectiveness and convenience. Administration Schedule, Drug,Administration Schedules, Drug,Drug Administration Schedules,Schedule, Drug Administration,Schedules, Drug Administration
D005260 Female Females
D006222 Hamartoma A focal malformation resembling a neoplasm, composed of an overgrowth of mature cells and tissues that normally occur in the affected area. Hamartomas
D006801 Humans Members of the species Homo sapiens. Homo sapiens,Man (Taxonomy),Human,Man, Modern,Modern Man

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