Precocious Puberty Syndrome Research Articles

LAUGHING SEIZURES- A RARE DISORDER IN PEDIATRIC PATIENTS

Gelastic epilepsy or laughing seizures have been historically related to children with hypothalamic hamartomas. Gelastic seizures are rare; they are usually associated with other seizure types and can be restricted to a limited period during the evolution of an epileptic syndrome early-onset gelastic epilepsy, hypothalamic harmatoma, and precocious puberty syndrome. This condition typically results in a catastrophic epileptic encephalopathy, which is usually refractory to antiepileptic therapy, but in most cases, it is reversed by surgical excision, radiofrequency thermocoagulation, or gamma-knife surgery of the HH. Therefore, despite its rarity, this syndrome is of clinical relevance and an early diagnosis is important. Moreover, this review provides a model for the study of Gelastic epileptic mechanisms. Keywords: Laugh-induced seizure, Gelastic seizure, Parietal cortical dysplasia

Pituitary-Thyroid Hormone Interactions in States of Hyper- and Hypothyroidism

Circulating thyroxine (T4) and triiodothyronine (T3) are regulated by a negative feedback system involving hypothalamic TRH, pituitary TSH, and the thyroid hormones, T4 and T3. In states of severe primary hypothyroidism, pituitary enlargement may occur as a result of thyrotroph hyperplasia and cause mass effects similar to pituitary adenomas. Conversely, thyrotoxicosis may be caused by pituitary TSH-secreting adenomas. States of thyroid hormone deficiency and excess play important roles in the regulation of all other protein hormones released by the pituitary. In hypothyroidism, alterations in growth hormone, gonadotropins, and prolactin, respectively, can be attended by impaired somatic growth, menstrual irregularities, and galactorrhea. In states of hyperthyroidism, most of these manifestations are reversed, as exemplified by the accelerated growth in children with thyrotoxicosis, delayed menarche, and oligomenorrhea in young thyrotoxic women, with contrasts with the syndrome of precocious puberty in children with 1° hypothyroidism. Hyperthyroidism can be attended by enhanced clearance of cortisol evoking rises in ACTH, and thyrotoxic patients on glucocorticoid replacement therapy may require additional steroid therapy. Finally, hyponatremia from impaired free water excretion can be seen in hypothyroidism. This can lead to lethargy and frank coma. This abnormality in water metabolism is corrected completely by thyroid hormone administration. Awareness of these thyroid hormone-dependent pituitary endocrinopathies should lead to improved diagnosis and management of these important problems. The Endocrinologist 2000; 10: 118-123 Learning Objectives: In what ways do hypothalamic and pituitary hormone release interact with thyroid hormone deficiency or excess to alter body growth, puberty, and reproductive function? Review interactions between hypo- and hyperthyroidism on the one hand, and changes in hypothalamic-pituitary-adrenal axis function and water/electrolyte homeostasis on the other. In patients with pituitary enlargement and abnormal thyroid function, when does the primary fault reside in the pituitary and when in the thyroid?

THE SYNDROME OF PRECOCIOUS PUBERTY, FIBROCYSTIC BONE DISEASE AND PIGMENTATION OF THE SKIN: ELEVEN YEARS' OBSERVATION OF A CASE

Case Reports1 January 1945THE SYNDROME OF PRECOCIOUS PUBERTY, FIBROCYSTIC BONE DISEASE AND PIGMENTATION OF THE SKIN: ELEVEN YEARS' OBSERVATION OF A CASEBERNARD M. SCHOLDERBERNARD M. SCHOLDERSearch for more papers by this authorAuthor, Article, and Disclosure Informationhttps://doi.org/10.7326/0003-4819-22-1-105 SectionsAboutPDF ToolsAdd to favoritesDownload CitationsTrack CitationsPermissions ShareFacebookTwitterLinkedInRedditEmail ExcerptIn 1922 Weil1 first described the syndrome of precocious puberty, fibrocystic bone disease and pigmentation of the skin. Since then there have been reports of some 18 cases.2, 3, 4, 5, 6, 7, 8, 9, 10, 11, 12, 13, 14 These are presented in table 1. Although the syndrome is interesting because it is uncommon and bizarre, its chief appeal arises from attempts to explain the various phenomena in the light of present day knowledge of neuro-endocrine mechanisms.The patient to be presented has been under constant medical scrutiny since the onset of symptoms at the age of three, and...Bibliography1. WEIL : Pubertas praecox und Knochenbrüchigkeit, Klin. Wchnschr., 1922, i, 2114. Google Scholar2. GAUPP V: Pubertas praecox bei Osteodystrophia fibrosa, Monatschr. f. Kinderh., 1932, liii, 312. Google Scholar3. FREEDMAN HJ: Disturbances of function of the suprarenal glands in children, Am. Jr. Dis. Child., 1932, xliv, 1285. Google Scholar4. STALMANN A: Nerven-, Haut-, und Knochenveränderungen bei der Neurofibromatosis Recklinghausen und ihre entstehungsgeschichtlichen Zusammenhänge, Virchow's Arch. f. path. Anat., 1933, cclxxxix, 96. CrossrefGoogle Scholar5. SNAPPERPARISEL IC: Xanthomatosis generalisata ossium, Quart. Jr. Med., 1933, ii, 407. Google Scholar6. GOLDHAMMER K: Osteodystrophia fibrosa unilateralis (kombiniert mit Pubertas praecox und mit gleichseitigen osteosklerotischen Veränderungen des Schädels), Fortsch. a. d. Geb. d. Röntgenstrahlen, 1934, xlix, 456. Google Scholar7. MCCUNEBRUCH DJH: Osteodystrophia fibrosa, Am. Jr. Dis. Child., 1937, liv, 806. CrossrefGoogle Scholar8. ALBRIGHTBUTLERHAMPTONSMITH FAMAOP: Syndrome characterized by osteitis fibrosa disseminata, areas of pigmentation and endocrine dysfunction, with precocious puberty in females, New Eng. Jr. Med., 1937, ccxvi, 727. CrossrefGoogle Scholar9. MONDORDUCROQUETLEGERLAURENCE HRLG: Sur un cas d'ostéite fibrokystique unilatérale avec pigmentation et puberté précoce, Rev. gén. de clin. et de thérap., 1938, lii, 823-824. Google Scholar10. SUMMERFELDTBROWN PA: Osteodystrophia fibrosa, Am. Jr. Dis. Child., 1939, lvii, 90-101. Google Scholar11. ROBSONTODD KJW: Fibrocystic bone disease with skin pigmentation and endocrine dysfunction, Lancet, 1939, i, 372-380. Google Scholar12. BRAID F: Osseous dystrophy following icterus gravis neonatorum. Generalized osteitis fibrosa with areas of pigmentation of skin and precocious puberty in female, Arch. Dis. Child., 1939, xiv, 181-202. CrossrefGoogle Scholar13. DIEZ J: La osteitis fibrosa diseminada con puberbad precoz y pigmentaciones cutaneas (sindrome de Albright), Prensa med. argent., 1939, xxvi, 1870-1897. Google Scholar14. DOCKERTYMEYERDINGWALLACE MBHWGT: Albright's syndrome (fibrous dysplasia of bones with cutaneous pigmentation in both sexes and gonadal dysfunction in females), Proc. Staff Meet. Mayo Clin., 1944, xix, 81-88. Google Scholar15. LICHTWITZ L: Functional pathology, 1941, Grune and Stratton, New York. Google Scholar16. STERNBERGJOSEPH WHV: Osteodystrophia fibrosa combined with precocious puberty and exophthalmic goiter, Am. Jr. Dis. Child., 1942, lxiii, 748-783. CrossrefGoogle Scholar17. NOVAK E: The constitutional type of female precocious puberty with a report of 9 cases, Am. Jr. Obst. and Gynec., 1944, xlvii, 20-42. CrossrefGoogle Scholar18. WEINBERGERGRANT LMFC: Precocious puberty and tumors of the hypothalamus. Report of a case and review of the literature with a physiologic explanation of the precocious sexual syndrome, Arch. Int. Med., 1941, lxvii, 762-792. CrossrefGoogle Scholar19. BINGGLOBUSSIMON JFJHHJ: Pubertas praecox: a survey of the reported cases and verified anatomical findings, with particular reference to tumors of the pineal body, Jr. Mount Sinai Hosp., 1938, iv, 935. Google Scholar20. SMITHDORTZBACH PEC: The first appearance in the anterior pituitary of the developing pig fetus of detectable amounts of the hormones stimulating ovarian maturity and general body growth, Anat. Rec., 1929, xliii, 277. CrossrefGoogle Scholar21. FORDGUILD FRH: Precocious puberty following measles encephalomyelitis and epidemic encephalitis, Bull. Johns Hopkins Hosp., 1937, lx, 192. Google Scholar22. BESTTAYLOR CHNB: The physiological basis of medical practice, 1943, Williams and Wilkins, Baltimore. Google Scholar23. BREMER JL: Osteitis fibrosa localisata, Arch. Path., 1941, xxxii, 200-210. Google Scholar This content is PDF only. To continue reading please click on the PDF icon. Author, Article, and Disclosure InformationAffiliations: M.C. U.S.N.R. Mount Vernon, New York*Received for publication July 11, 1944.The opinions and assertions contained herein are those of the writer and are not to be construed as official or reflecting the views of the Navy Department or of the Naval Service at large. PreviousarticleNextarticle Advertisement FiguresReferencesRelatedDetails Metrics Cited byHormonale Osteopathien im ErwachsenenalterHormonale Osteopathien im ErwachsenenalterFibrosarcoma complicating polyostotic fibrous dysplasiaOsteopathienDie Röntgendiagnostik der SchädelbasisDie Röntgendiagnostik der SchädelbasisUNILATERAL DYSPLASIA OF BONE IN AN ELDERLY WOMANSonstige Erkrankungen der Schädelknochen von gewisser neurochirurgischer BedeutungDie Keimdrüsen und ihre KrankheitenPolyostotische fibröse DysplasiePrecocious sexual development 1 January 1945Volume 22, Issue 1Page: 105-118KeywordsBoneGratitudePuberty Issue Published: 1 January 1945 PDF downloadLoading ...