https://www.thelancet.com/pdfs/journals/lancet/PIIS0140-6736(14)61375-1.pdf

Cushing's syndrome

Objective: The aim was to formulate practice guidelines for the diagnosis and treatment of hyperprolactinemia. Participants: The Task Force consisted of Endocrine Society-appointed experts, a methodologist, and a medical writer. Evidence: This evidence-based guideline was developed using the Grading of Recommendations, Assessment, Development, and Evaluation (GRADE) system to describe both the strength of recommendations and the quality of evidence. Consensus Process: One group meeting, several conference calls, and e-mail communications enabled consensus. Committees and members of The Endocrine Society, The European Society of Endocrinology, and The Pituitary Society reviewed and commented on preliminary drafts of these guidelines. Conclusions: Practice guidelines are presented for diagnosis and treatment of patients with elevated prolactin levels. These include evidence-based approaches to assessing the cause of hyperprolactinemia, treating drug-induced hyperprolactinemia, and managing prolactinomas in nonpregnant and pregnant subjects. Indications and side effects of therapeutic agents for treating prolactinomas are also presented. (J Clin Endocrinol Metab 96: 273–288, 2011)

Diagnosis and treatment of hyperprolactinemia: an Endocrine Society clinical practice guideline

Mood disorders and allostatic load.

Objective: The aim was to provide guidelines for evaluation, treatment, and genetic testing for multiple endocrine neoplasia type 1 (MEN1). Participants: The group, which comprised 10 experts, including physicians, surgeons, and geneticists from international centers, received no corporate funding or remuneration. Process: Guidelines were developed by reviews of peer-reviewed publications; a draft was prepared, reviewed, and rigorously revised at several stages; and agreed-upon revisions were incorporated. Conclusions: MEN1 is an autosomal dominant disorder that is due to mutations in the tumor suppressor gene MEN1, which encodes a 610-amino acid protein, menin. Thus, the finding of MEN1 in a patient has important implications for family members because first-degree relatives have a 50% risk of developing the disease and can often be identified by MEN1 mutational analysis. MEN1 is characterized by the occurrence of parathyroid, pancreatic islet, and anterior pituitary tumors. Some patients may also develo...

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Clinical practice guidelines for multiple endocrine neoplasia type 1 (MEN1)

OBJECTIVE:The primary objectives of this report were, first, to determine the number and incidence of complications of transsphenoidal surgery performed by a cross-section of neurosurgeons in the United States and, second, to ascertain the influence of the surgeon's experience with the procedure on

Complications of Transsphenoidal Surgery: Results of a National Survey, Review of the Literature, and Personal Experience

This study reports the clinical and biological follow-up 5-11 yr after transsphenoidal selective adenomectomyin 25 patients with acromegaly. Eight patients had microadenomas,and 17 had macroadenomas. Initial normalization ofplasma GH levels (basal values, <5 ng/ml; glucose-suppresedconcentrations, <2.5 ng/ml) was achieved in all 8 patients withmicroadenomas and in 13 patients with macroadenomas. Ofthese, 3 patients with normal GH levels and dynamics hadrelapse of GH hypersecretion after intervals between 1–6 yr aftermicroadenoma removal. Recurrence of pituitary adenoma wasdocumented by surgery in 1 patient and by computed tomographic scanning in 2 others. Normal basal and glucose-suppressedplasma GH concentrations were maintained 7.4 ± 0.5(±SEM) yr after adenomectomy in 7 patients with microadenomasand in all 10 patients with macroadenomas. Thus, 88% ofthe patients with microadenomas and 59% of the patients withmacroadenomas were cured, and the overall cure rate was 68%.We conclude that recurrence of acro...

Acromegaly: biochemical assessment of cure after long term follow-up of transsphenoidal selective adenomectomy.

Transsphenoidal selective adenomectomy is the most efficient primary treatment for acromegaly. However, management of persistent or recurrent disease remains controversial. The objective of the present study was to evaluate the early and long-term efficacy and safety of a second transsphenoidal surgery performed in those cases. The results of a retrospective study of 16 patients undergoing reoperation by the senior author (J.H.) between 1970 and 1991 are reported. Reoperation was performed for persistent or progressive acromegaly in 11 patients, visual impairment in four, and disease recurrence in one. Normalization of growth hormone (GH) was defined as a basal GH level of less than 5 micrograms/L and suppression to less than 2 micrograms/L during the oral glucose tolerance test. Long-term follow-up data were available in 15 patients. The second transsphenoidal surgery induced a greater than 50% decrease of GH level in 11 patients. Three (19%) of 16 patients were cured according to the authors' criteria and remained so after 2, 7, and 20 years. Two more patients had a postoperative basal GH level of less than 5 micrograms/L but incomplete suppression during the oral glucose tolerance test. Thus, a total of five patients (31%) achieved a basal GH of less than 5 micrograms/L. One other patient who had no initial improvement after the second transphenoidal surgery had spontaneous normalization of his GH level after 13 years. The following complications of the second surgery occurred in three patients: one subarachnoid hemorrhage, two new visual field defects, one cranial nerve palsy, and one meningitis. Moreover, 10 patients (62.5%) developed one or more new pituitary hormone deficiencies. In conclusion, reoperation for persistent or recurrent acromegaly has low success and high complication rates. According to the authors' experience, this procedure should be reserved for patients unresponsive to other forms of therapy or with progressive visual impairment despite medical therapy.

Surgical outcome after repeated transsphenoidal surgery in acromegaly

Transsphenoidal adenomectomy for microprolactinomas: 10 to 20 years of follow-up

The PRL inhibitory effect of dopamine (DA) in human in vivo studies has been previously demonstrated with DA infusions at rates generally exceeding 2 μg/kg-min. We report here the effects of a DA infusion administered at a rate of 0.02 μg/kg-min for 180 min to 10 normal subjects and 25 hyperprolactinemic patients with pituitary tumors (13 microprolactinomas, 8 macroprolactinomas, and 4 expanding nonsecreting pituitary adenomas). Serum free DA concentrations during the 3-h DA infusion reached an average of 0.8 ± 0.1 ng/ml (about an 8- to 10-fold rise from basal levels). DA produced a significant (P μ 0.001) decline in plasma PRL levels in both normal subjects and hyperprolactinemic patients. There was a negative linear correlation between the serum DA concentrations and the percent PRL variation from basal levels (r = −0.58; Pμ 0.001). The comparison of PRL responses between the different groups revealed that the mean percent overall PRL inhibition was significantly lower in patients with microprolactinoma...

Differential effects of a low dose dopamine infusion on prolactin secretion in normal and hyperprolactinemic subjects.

Three patients with Kallmann's syndrome, 1 hyposmic man, 24 yr old, and 2 anosmic women, 17 and 26 yr old, have had studies of hypothalamo-pituitary function ACTH, TSH, and GH pituitary reserves were found normal when evaluated respectively by metyrapone test, TSH increase after the intravenous administration of 200 μg of TRH and GH response to insulin-induced hypoglycemia The subcutaneous administration of 100 μg of synthetic LRH produced an 8- to 18-fold increase in plasma LH, and an increase of plasma FSH varying between 320% and 830% These responses are of greater magnitude than those hitherto reported Finally, pituitary secretory reserve of prolactin was found normal as evidenced by an increase of plasma prolactin levels of 250% after the intravenous administration of 200 or 400 μg of synthetic TRH However, when 25 mg of chlorpromazine were administrated intramuscularly, the patient with hyposmia responded with a 450% increase of plasma prolactin while no increase was observed in the tw

M. Somma

Papers

Acromegaly: biochemical assessment of cure after long term follow-up of transsphenoidal selective adenomectomy.

Surgical outcome after repeated transsphenoidal surgery in acromegaly

Transsphenoidal adenomectomy for microprolactinomas: 10 to 20 years of follow-up

Differential effects of a low dose dopamine infusion on prolactin secretion in normal and hyperprolactinemic subjects.

Hypothalamic-Pituitary Function in the Olfacto-Genital Syndrome