Addison's Disease Research Today is a free monthly online journal that collates and summarizes the latest research about Addison's Disease, including details on chronic adrenal insufficiency, treatment, causes, medication. | ||||||
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Intracranial compound odontome.Faria PR, Cardoso SV, Rocha A, Gomes DC, Castro SC, Loyola AM Departamento de Morfologia, Instituto de Ciências Biomédicas, Universidade Federal de Uberlândia, Uberlândia, Minas Gerais, Brazil. An exceedingly rare case of an extragnathic odontome is described arising within the brain. A 10-year-old boy complained of progressive frontal headache for 5 years. Axial computerized tomography the head revealed a solid, calcified lesion with well-defined borders localized in the sellar and suprasellar region composed of multiple calcified structures resembling teeth. The diagnosis was compound odontome. Physical examination and blood analysis revealed hypopituitarism. The patient was submitted for radical tumour resection. He developed persistent diabetes insipidus, hypothyroidism and adrenal insufficiency for which appropriate replacement therapy has been necessary. This case demonstrates that an odontogenic lesion may arise in brain tissues due to the embryological relationship between primordial stomodeum and Rathke's pouch. Its development could be associated with endocrine disturbances. Published 22 June 2009 in J Craniomaxillofac Surg. Articles on Addison's Disease published 18 June 2009: Targeted disruption of Sonic Hedgehog in the mouse adrenal leads to adrenocortical hypoplasia. Genesis. Development of the mammalian adrenal gland is regulated by a diverse network of growth and transcription factors. Disruptions in these pathways often result in adrenal insufficiency because of adrenal hypoplasia. Several lines of evidence have suggested that the Hedgehog signaling pathway, which controls many aspects of tissue and organ patterning, may play a direct role in adrenal morphogenesis as well. Therefore, we examined the role of Sonic Hedgehog (Shh), a member of the Hedgehog family, ... [Abstract] [Full-text] Articles on Addison's Disease published 15 June 2009: Primary adrenal lymphoma is extremely rare, accounting for <1% of non-Hodgkin lymphomas, and lymphoma-associated chromosomal translocations have yet to be reported in this entity. We performed a retrospective study of 10 cases in immunocompetent patients including 4 males and 6 females with a median age of 68 years. The most common presenting symptoms were abdominal pain and fever; unexpectedly, clinically evident adrenal insufficiency was detected only in one patient. The mean tumor size at ... [Abstract] [Full-text] Articles on Addison's Disease published 10 June 2009: Clinical and genetic heterogeneity of congenital adrenal hypoplasia due to NR0B1 gene mutations. Clin Endocrinol (Oxf). Introduction: X-linked adrenal hypoplasia congenita (AHC) is a rare disorder caused by mutations or complete deletion of the NR0B1 gene that encodes the DAX-1 protein, an orphan member of the nuclear receptor superfamily. AHC is characterized by adrenal insufficiency in infancy and early childhood. Later, hypogonadotropic hypogonadism (HH) manifests as pubertal failure. Patients and methods: We evaluated the clinical, endocrine and molecular characteristics of 12 AHC patients from 5 families ... [Abstract] [Full-text] Articles on Addison's Disease published 4 June 2009: Effects of dehydroepiandrosterone replacement on vascular function in primary and secondary adrenal insufficiency: a randomized crossover trial. J Clin Endocrinol Metab, 94(6): 1966-72. CONTEXT: Patients with Addison's disease and hypopituitarism have increased mortality, chiefly related to vascular disease. Both diseases are characterized by dehydroepiandrosterone (DHEA) deficiency, yet this is not usually corrected. It is unclear whether treatment of these conditions with DHEA improves cardiovascular risk. OBJECTIVE: The aim of the study was to evaluate the effects of DHEA on arterial stiffness and endothelial function in subjects with Addison's disease and hypopituitarism. ... [Abstract] [Full-text] Articles on Addison's Disease published 3 June 2009: Beclomethasone dipropionate enema in ulcerative colitis: Is it safe? Endocr Pract. Objective: To evaluate the pituitary-adrenal axis (PA axis) reserve in patients with ulcerative colitis (UC) treated with steroid enemas, we performed 1mug ACTH test.Methods: Patients with UC (n=8) given beclomethasone dipropionate enema (Betnesol) for 4 weeks. Fasting serum cortisol and the response at 30 and 60 minutes following an IV 1mug ACTH injection were determined after 2 weeks of Betnesol enemas. Patients were retested, one month after discontinuation of enemas.Results: Fasting and ... [Abstract] [Full-text] Articles on Addison's Disease published 29 May 2009: Steroid hormone analysis by tandem mass spectrometry. Clin Chem, 55(6): 1061-6. BACKGROUND: New high-performance liquid chromatography/tandem mass spectrometry (LC-MS/MS) methods are among the most successful approaches to improve specificity problems inherent in many immunoassays. CONTENT: We emphasize problems with immunoassays for the measurement of steroids and review the emerging role of LC-MS/MS in the measurement of clinically relevant steroids. The latest generation of tandem mass spectrometers has superior limits of quantification, permitting omission of ... [Abstract] [Full-text] Articles on Addison's Disease published 28 May 2009: Effects of short-term nocturnal cortisol replacement on cognitive function and quality of life in patients with primary or secondary adrenal insufficiency: a pilot study. Appl Psychophysiol Biofeedback, 34(2): 113-9. Cortisol replacement in patients with adrenal insufficiency usually consists of hydrocortisone (HC) given orally during day time. Due to the short half-life of hydrocortisone, cortisol levels between midnight and early morning are very low in contrast to the physiological rise of cortisol serum levels during this time. We investigated whether short-term cortisol replacement during the night improves cognitive function and well-being in these patients. Fourteen patients with adrenal ... [Abstract] [Full-text] Articles on Addison's Disease published 22 May 2009: Premature ovarian failure. Obstet Gynecol, 113(6): 1355-63. Premature ovarian failure is the term usually used to describe women aged younger than 40 years who present with amenorrhea, hypergonadotropinism, and hypoestrogenism. Such women may ovulate and even conceive after the diagnosis is made, so it may be more appropriate to refer to these patients as having "primary ovarian insufficiency," or alternatively, as having "hypergonadotropic hypogonadism" or "primary hypogonadism." The clinical presentation is diverse, and ... 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