Download the Free Unbound MEDLINE PubMed App to your smartphone or tablet.
Available for iPhone, iPad, iPod touch, and Android.
- The role of colonoscopic screening in acromegaly revisited: review of current literature and practice guidelines. [JOURNAL ARTICLE]
- Pituitary 2014 Jul 23.
Acromegaly is a chronic, debilitating and disfiguring condition with a significantly increased morbidity and mortality due to cardiovascular, as well as respiratory complications. Patients with acromegaly are usually diagnosed at the age of 40, however, the duration of symptoms can vary from 5 to 10 years before the formal diagnosis is confirmed. Recent advances in the field of acromegaly have improved survival significantly. A strong association between acromegaly and premalignant colonic lesions and colon cancer has been highlighted. Furthermore, patients with acromegaly have a greater lifetime risk of malignant transformation and a far worse overall prognosis from colorectal cancer, which is now considered a major disease related complication.A comprehensive search strategy was applied for the Medline/PubMed electronic database from its inception until April 2014. We considered all human research articles published in English, not classified as case report, editorial, comment, letter, or news.Specific recommendations for large bowel endoscopic screening in patients with acromegaly have been proposed. In this comprehensive review we discuss the current state of knowledge and evidence on colonoscopic screening in patients with acromegaly illustrated by a case of aggressive colorectal cancer presenting late in a young woman with difficult to control acromegaly.
- Gamma knife radiosurgery for Cushing's disease and Nelson's syndrome. [JOURNAL ARTICLE]
- Pituitary 2014 Jul 10.
This paper presents our 18 years of experience in treating ACTH secreting adenomas (Cushing's disease and Nelson's syndrome) using the Leksell gamma knife (LGK) irradiation.Twenty-six patients with Cushing's disease were followed-up after LGK irradiation for 48-216 months (median 78 months). Seventeen patients had undergone previous surgery, in nine patients LGK irradiation was the primary therapy. Furthermore, 14 patients with Nelson's syndrome were followed-up for 30-204 months (median 144 months).LGK treatment resulted in hormonal normalization in 80.7 % of patients with Cushing's disease. Time to normalization was 6-54 months (median 30 months). The volume of the adenoma decreased in 92.3 % (in 30.7 % disappeared completely). There was no recurrence of the disease. In all 14 patients with Nelson's syndrome ACTH levels decreased (in two patients fully normalized) their ACTH levels. When checked up 5-10 years after irradiation regrowth of the adenoma was only detected in one patient (9.1 %), in 27.3 % adenoma volume remained unchanged, in 45.4 % adenoma volume decreased and in 18.2 % adenoma completely disappeared. Hypopituitarism did not develop in any patient where the critical dose to the pituitary and distal infundibulum was respected.LGK radiation represents an effective and well-tolerated option for the treatment of patients with Cushing's disease after unsuccessful surgery and may be valuable even as a primary treatment in patients who are not suitable for, or refuse, surgery. In the case of Nelson's syndrome it is possible to impede tumorous growth and control the size of the adenoma in almost all patients.
- Persistent self-consciousness about facial appearance, measured with the Derriford appearance scale 59, in patients after long-term biochemical remission of acromegaly. [JOURNAL ARTICLE]
- Pituitary 2014 Jun 26.
Acromegaly is associated with impaired quality of life (QoL) and causes anatomical disproportions, which may contribute to the decreased QoL after successful treatment. The Derriford appearance scale 59 (DAS59) is a questionnaire measuring psychological distress and disruptions to everyday life associated with self-consciousness of appearance.Investigate the psychological distress and dysfunction related to self-consciousness about appearance and its effect on QoL in patients in long-term remission of acromegaly.Patients (>18 years old) treated for acromegaly at the Department of Endocrinology of the Radboud University Medical Center Nijmegen were invited to participate. A gender-, age- and body mass index matched control group was provided by the patients themselves. Participants were asked to complete the modified DAS59-, research and development 36- (RAND-36), acromegaly quality of life questionnaire (AcroQoL) and a sociodemographic questionnaire. Differences between patient- and control groups and correlations between questionnaire scores and clinical characteristics collected from medical records were analyzed.Questionnaire scores.Of the 120 respondents, 73 agreed to participate [all cured or under biochemical control, median remission time 10.5 years (range 2.3-43.6 years)]. Of these, 34 (46.6 %) reported self-consciousness about their appearance. Twenty-nine of these patients (85.3 %) pointed out their face to be a prominent source of self-consciousness. Fifty-seven matched control subjects were included as well. Significant correlations were found between the scores of the DAS59 and the AcroQoL, RAND-36 and VAS in patients.Even after long-term remission of acromegaly, a large number of patients are self-conscious about their appearance, leading to psychological distress and disruptions to everyday life and decreased QoL. Facial features were the most important source of self-consciousness. This stresses the importance of addressing self-consciousness of appearance and the need for additional support in this regard during follow-up in these patients.
- Bilateral cerebral infarction in the setting of pituitary apoplexy: a case presentation and literature review. [JOURNAL ARTICLE]
- Pituitary 2014 Jun 26.
Pituitary tumor apoplexy (PTA) is a potentially fatal condition caused by hemorrhage and rapid expansion of a pituitary tumor. One rare consequence of PTA is occlusion of the intracavernous carotid arteries, very rarely leading to cerebral infarction.To describe a case of PTA leading to bilateral cerebral infarction and provide an extensive literature review of all previously reported cases of PTA leading to cerebral infarction. We discuss how these cases contribute to our understanding of the management of PTA, and we also discuss the differences between cases associated with the reported mechanism of carotid occlusion (compression vs. vasospasm).A 56-year-old previously healthy woman complained of severe headache and visual loss one day after sustaining a fall from standing. Computed tomography demonstrated an enlarged sellar and suprasellar mass displacing both cavernous ICAs laterally, with multiple bilateral hypodense areas in the ICA distribution consistent with infarction. She clinically deteriorated and underwent endoscopic transsphenoidal gross total resection for suspected PTA within 48 hours after falling. Her prognosis remained poor after 5 days, and support was withdrawn.Twenty-four cases of PTA leading to cerebral infarction have been previously documented-four bilateral, our case being the fifth. Based on our review, the presence of infarction itself does not seem to warrant surgical management in the absence of previously established indications for surgery (such as a deteriorating visual field), despite a 3-5 times mortality increase. No conclusion regarding the role of the mechanism of occlusion can be made at this time.
- Up-to 5-year efficacy of pasireotide in a patient with Cushing's disease and pre-existing diabetes: literature review and clinical practice considerations. [JOURNAL ARTICLE]
- Pituitary 2014 Jun 21.
Pasireotide is a multi-receptor-targeted somatostatin analogue approved in the EU and in the US for the treatment of adults with Cushing's disease (CD). Pasireotide has a safety profile similar to other somatostatin analogues with the exception of hyperglycemia. In this report and literature review, the current understanding of predicting a positive treatment response to pasireotide in CD and the management of diabetes mellitus (DM) during pasireotide treatment are discussed and analyzed.We report a case of a 55-year-old woman with CD and DM who benefitted from long-term pasireotide. The patient, who was enrolled in a phase III trial of the drug, showed early clinical improvements with pasireotide [900 μg subcutaneously twice daily (bid)] but was classified as a non-responder as urinary free cortisol (UFC) levels, were not normalized. Continuation of pasireotide for 12 months at an increased dose (1,200 μg bid) normalized UFC levels and restored cortisol rhythm. The initial deterioration in her blood glucose was managed with insulin and metformin; however, after 12 months' treatment with pasireotide her DM was well controlled with oral hypoglycemic agents. Five years later, the patient is still receiving pasireotide (300 μg bid) with no loss of clinical or biochemical efficacy and with continued glycemic control.This case presentation indicates that uncontrolled UFC levels during the first few months of pasireotide treatment as well as worsening of glycemic control in patients with CD and DM are not always predictive of the efficacy and tolerability and appears to support the long-term continuation of pasireotide.
- The prevalence and associated factors of colorectal neoplasms in acromegaly: a single center based study. [JOURNAL ARTICLE]
- Pituitary 2014 Jun 20.
Colorectal neoplasms are well known to be a complication in cases of acromegaly; however, data on the prevalence of colorectal neoplasms in Asian patients with acromegaly are limited. Further, the factors associated with colorectal neoplasms in cases of acromegaly are controversial. Therefore, we aimed to clarify the prevalence of and factors associated with colorectal neoplasms in Japanese patients with acromegaly in a single center.We analyzed consecutive 57 patients who had undergone full-length colonoscopy at the time of diagnosis at Kobe University Hospital between 1986 and 2012.Of the 57 patients, 22 (38.6 %), 18 (31.6 %) and 3 (5.3 %) patients were diagnosed with hyperplastic polyps, adenomas, and adenocarcinomas, respectively and the prevalence was significantly higher than in a historical control group, Chinese patients with irritable bowel syndrome (The odds ratio was 4.0, 8.7, and 17.5, respectively). The prevalence of adenocarcinomas was also significantly higher in these patients than in the general Japanese population (odds ratio 14.5). Patients with acromegaly who had colorectal neoplasms had longer disease duration than those without colorectal neoplasms. Of note, the area under the growth hormone (GH) concentration-time curve (GH AUC) during the oral glucose tolerance test was significantly higher in patients with adenocarcinomas than in those with no colonic lesion or those with hyperplastic polyps.Japanese patients with acromegaly exhibited an increased risk of colorectal neoplasms, especially colorectal adenocarcinomas. An increased GH AUC was associated with an increased risk for colon adenocarcinomas in patients with acromegaly.
- Stereotactic radiosurgery for acromegaly: outcomes by adenoma subtype. [JOURNAL ARTICLE]
- Pituitary 2014 Jun 13.
The subtypes of somatotroph-cell pituitary adenomas have been correlated with clinical and histopathological variables. Densely granulated somatotroph-cell (DG) adenomas are typically highly responsive to somatostatin analog drugs, whereas sparsely granulated somatotroph-cell (SG) are less responsive. The aim of the study is to determine the effect of stereotactic radiosurgery (SRS) on remission and development of new pituitary deficiency according to the different subtypes of growth hormone (GH) secreting adenomas.A total of 176 patients underwent SRS for acromegaly at the University of Virginia. Diagnosis of acromegaly was based on the combination of clinical features and biochemical assessment including the serum GH level, and age- and gender-matched serum insulin-like growth factor-1 level. All patients underwent endocrine and neuro-imaging evaluations before and after SRS. Histological specimens were available in 73 patients.The histopathological examination showed 34 patients had a DG adenoma, 19 had a SG adenoma, eight had a mixed DG/SG pattern, while other rare mixed subtypes were present in 12 patients. Patients who had a SG adenoma were more likely to be younger and female, and the SG adenomas appeared to be more invasive into the cavernous sinus. With a median follow-up of 67 months (range 6-188 months), 55/73 patients (75.3 %) achieved remission. The median time to remission was 26 months (range 6-102 months). The actuarial remission rates in the DG adenoma group at 2, 4, and 6 years post-radiosurgery were 35.1, 71.4, and 79.3 %, respectively, while those in SG adenoma group were 35.4, 73.1, and 82.1 %, respectively.While patients who had a SG adenoma may be less responsive to medical therapy, they exhibited similar responses to SRS as patients with a DG adenoma. For SG adenomas, which respond less well to medical therapy, earlier SRS may be reasonable for consideration.
- Low expression of secreted frizzled-related protein 4 in aggressive pituitary adenoma. [JOURNAL ARTICLE]
- Pituitary 2014 Jun 11.
The secreted frizzled-related proteins (sFRPs) are reported to be antagonists of a number of tumors. This study was designed to investigate the relationship of sFRP4 with aggressiveness of pituitary adenomas.Specimens were classified into three groups: normal control (n = 10), non-aggressive group (n = 42) and aggressive group (n = 26) according to preoperative magnetic resonance imaging (MRI)/computed tomography. sFRP4 were investigated by PCR, Western blotting, and immunohistochemistry (IHC). The methylation status of the sFRP4 promoter region was observed by MassArray. Cell culture and 5-aza-2-deoxycytidine treatment was performed to observe the relationship of downregulation of sFRP4 with methylation of the sFRP4 gene.PCR and Western blot results showed that sFRP4 expression was downregulated in aggressive pituitary adenomas, which was confirmed by IHC. Methylation of the sFRP4 promoter was increased in aggressive pituitary adenomas. And methylation of the sFRP4 promoter lead to downregulation of sFRP4 expression.sFRP4 expression is inversely related to the aggressiveness of pituitary adenomas, and act as a tumor suppressor.
- Importance of cannulated prolactin test in the definition of hyperprolactinaemia. [JOURNAL ARTICLE]
- Pituitary 2014 May 31.
Recent guidelines suggest that a single prolactin measurement is adequate to confirm hyperprolactinaemia. This may lead to unnecessary investigation of artefactual hyperprolactinaemia. Prolactin measurement drawn from an indwelling cannula after rest removes stress as a confounding variable. The objective was to determine the frequency of true hyperprolactinaemia amongst patients referred following a single prolactin measurement.A cannulated study was considered if prolactin on referral ('Referral Prolactin') was <5,500 mU/L (260 ng/mL) but >410 mU/L (19 ng/mL) in males or >510 mU/L (24 ng/mL) in females, irrespective of clinical context. Case-notes of 267 patients undergoing cannulated prolactin measurement over a 10-year period (2000-2010) were reviewed. Pre-existing pituitary disease, dopamine antagonist use, and macroprolactinaemia were excluded. Morning ante-cubital vein cannulation was followed immediately by withdrawal of 'Repeat Prolactin' sample. After 120-min bed-rest, 'Resting Prolactin' was withdrawn through the cannula.235 patients were included for analysis. 64 (27 %) were within normal range; following Repeat Prolactin in 41 (17 %) and Resting Prolactin in 23 (9 %) cases. Referral Prolactin was higher in patients with true hyperprolactinaemia, 1,637 ± 100 mU/L (77.2 ± 4.7 ng/mL) than with artefactual hyperprolactinaemia, 1,122 ± 68 mU/L (52.9 ± 3.2 ng/mL; P < 0.001) but there was substantial overlap. 21 out of 171 cases (12 %) with true hyperprolactinaemia had a macroadenoma. Presenting symptoms did not predict true hyperprolactinaemia. Referral Prolactin of 2,000 mU/L (94 ng/mL) had 97 % specificity to identify true hyperprolactinaemia.Reliance on a single, non-rested prolactin value may lead to over-diagnosis of hyperprolactinaemia. A resting sample should be considered with random values <2,000 mU/L (94 ng/mL).