Post-surgery Quality of Life in Patients with Acromegaly Using SF36 Quality of Life Questionnaire-prospective Study

Introduction:Acromegaly is chronic progressive disease with multisystem involvement characterised by an excess secretion of growth hormone and increased circulating insulin like growth factor 1 concentration.Aims and Objectives:To assess surgical outcome of acromegaly patients at tertiary care institute using SF 36 quality of life questionnaire.SF-36 scores comprise 3 components:the physical component summary(PCS),the mental component summary(MCS)and role-social component summary(RCS).30 acromegaly patients admitted in Guwahati medical college were enrolled in study and followed up post operatively for surgical remission.All participants completed the SF-36 preoperatively,1 year and 2 years postoperatively.Material and Method:Out of 30 patients 6 patients had surgical remission post operatively on the basis of postoperative glucose suppressed GH Level done after 12 weeks.Preoperatively subscale scores(physical functioning,role physical,general health)which were below the set standards for the normal population show significant postoperative improvements along with mental health(MH)scores.Similarly,PCS,MCS and RCS scores changed significantly after surgery.We also compared the QOL of 6 patients whose peak GH level was<0.4μg/L during postoperative oral glucose tolerance testing with those patients whose nadir GH level was≥0.4μg/L.There was significant difference between partial and complete remission group in subscale score role physical,social function and mental health.Similarly,PCS and RCS score significantly different in partial and complete remission group than MCS score.Conclusions:QOL is considerably reduced in patients with acromegaly compared to general population which improves significantly after surgical treatment.Patients achieving the new remission criteria had significant improvement in physical and social components than those who did not.

Cost-Effectiveness of Somatostatin Analogues for the Treatment of Acromegaly in Colombia

Background: Somatostatin analogues have shown to be effective in controlling the levels of growth hormone and are recommended for the treatment of acromegaly. These treatments have high costs of acquisition and their use might be restricted in limited resource settings. Objective: To evaluate the cost-effectiveness of somatostatin analogues for acromegaly in Colombia. Setting/Subjects/Intervention: A decision model was developed using a hypothetical cohort of patients with Acromegaly. Patients were treated according to the clinical practice of the country. Response to treatment and transition probabilities were derived from published literature. Costs and resource utilization were extracted from public and private sources in Colombia. Main Outcome Measure(s): Cost-effectiveness ratio, measured in Colombian pesos in a 2 year time-horizon. Results: The total medical treatment costs for the octreotide group were (Colombian Pesos) COP$ 53,807,616, compared to the total costs for the lanreotide group of COP$ 83,126,567. In the octreotide arm 65.30% of the patients and in the lanreotide arm 59.50% of the patients were successfully controlled. The number of deaths was 295 (13.1%) and 302 (13.4%) for octreotide and lanreotide, respectively. Because the costs are lower and the effectiveness is higher for octreotide in comparison with lanreotide, octreotide is more cost-effective than lanreotide. Probabilistic sensitivity analyses were consistent showing octreotide as the most cost-effective option. Conclusions: Costs and effects of octreotide compare favorably to those of lanreotide in the treatment of acromegaly in Colombia. Sensitivity analysis showed that despite the uncertainty in cost-effectiveness ratio this result is robust.

Gastrointestinal symptoms in acromegaly:A case control study

BACKGROUND Acromegaly is a chronic disease caused by a pituitary somatotroph adenoma resulting in excess secretion of growth hormone,which leads to excess secretion of Insulin like growth factor 1 from the liver,causing abnormal soft tissue growth.There is increasing awareness that diseases affecting connective tissue are associated with an increase in functional gastrointestinal symptoms.Data was collected from patients with a confirmed diagnosis of acromegaly to evaluate the intensity,variety and impact of abdominal symptoms in comparison with a control group who were healthy participants recruited from the local fracture clinic.AIM To evaluate the frequency type and burden of abdominal symptoms in acromegaly in comparison with a control group.METHODS Medical documentation of patients with a diagnosis of acromegaly treated in one tertiary medical centre between 2010 and 2017 has been analysed.Data was collected from patients with confirmed acromegaly,using the Short Form Health Survey(SF36)and Rome IV Diagnostic questionnaire for Functional Gastrointestinal Disorders in Adults(R4DQ)and compared to a sex-and agematched control group,to assess the burden of abdominal symptoms.Microsoft Excel and IBM SPSS v 25 were used for data analysis.RESULTS Fifty patients with acromegaly(24 male and 26 females;age range 23-64 years,mean 43)and 200 controls(96 male and 104 females;age range 18-84,mean 42.4)were recruited.92%(46 out of 50)of patients with acromegaly reported abdominal symptoms and 78%(39 out of 50)had at least one functional gastrointestinal disorder according to the Rome IV diagnostic criteria,compared to 16%of controls(OR>1,P<0.0001).The most commonly reported symptom was constipation(69%acromegaly vs 21%of controls OR>1,P<0.0001,95%CI:4.4–15.8).34 out of 50(68%)respondents met the criteria for functional constipation according to Rome IV.Upper gastrointestinal disorders were also more prevalent in the acromegaly group.There was no statistically significant difference in the prevalence of biliary and anorectal symptoms between the two groups.Patients in acromegaly group scored lower on the mean scores of the eight parameters of SF36 Quality of Life questionnaire(mean scores 60.04 vs 71.23,95%CI:-13.6829 to-8.6971,OR>1,P<0.001)as compared to the control group.CONCLUSION Upper and lower functional gastrointestinal tract disorders(defined by Rome IV diagnostic criteria)are significantly more prevalent in patients with acromegaly compared with healthy age and sex matched controls in our study.Functional constipation is the most commonly reported problem.Poorer quality of life may in part be attributable to the increased prevalence of abdominal symptoms.

Acromegaly as an Expression of a Rare Disease: Description of an Unusual Clinical Case of Carney Complex

Introduction: The Carney complex (CC) is an autosomal dominant syndrome, characterized by multiple endocrine neoplasms, pigmented skin lesions, endocrine hyper-activity and myxomas. Case study: C. E., 71 years old, was addressed to our center in August 1994, following the finding of a sellar enlargement in a skull radiography performed after an accident. A pituitary tomography showed the presence of a lesion extended up to the optic chiasm, a macroadenoma that was treated with transsphenoidal endoscopic surgery. A histological diagnosis of a “GH secreting macroadenoma” was performed. An echocardiographic study highlighted an atrial isolated myxoma that was subsequently removed. A contrast-enhanced abdominal magnetic resonance imaging (MRI) excluded adrenal lesions. In 2001, in relation to relapse of Acromegaly, the patient started therapy with somatostatin analogues which was replaced, in 2008, by Pegvisomant to obtain biochemical control of the disease. Conclusions: At the time of initial screening, it is important not to underestimate the possibility that certain clinical features fall within the context of more complex syndromes. The knowledge of how Acromegaly presents itself in relation to CC and its associated characteristics such as cardiac myxoma or typical skin lesions, will help clinicians to timely diagnose this rare disease and treat it appropriately.

Research Progress of the Influence of Acromegaly on Cardiovascular Diseases

Acromegaly is a progressive disease caused by an increased in the levels of growth hormone(GH),followed by an increased in the levels of insulin-like growth factor-1(IGF-1).Most cases are caused by GH-secreting pituitary adenomas.Long-term exposure to excessive GH would affect all systems of the body,of which the cardiovascular system has the highest incidence rate and mortality rate.This review discusses the influence of acromegaly on cardiovascular diseases and its treatment.

Spontaneous remission of acromegaly or gigantism due to subclinical apoplexy of pituitary growth hormone adenoma

Background Subclinical apoplexy of pituitary functional adenoma can cause spontaneous remission of hormone hypersecretion. The typical presence of pituitary growth hormone （GH） adenoma is gigantism and/or acromegaly. We investigated the clinical characteristics of patients with spontaneous partial remission of acromegaly or gigantism due to subclinical apoplexy of GH adenoma. Methods Six patients with spontaneous remission of acromegaly or gigantism were enrolled. The clinical characteristics, endocrinological evaluation and imageological characteristics were retrospectively analyzed. Results In these cases, the initial clinical presences were diabetes mellitus or hypogonadism. No abrupt headache, vomiting, visual function impairment, or conscious disturbance had ever been complained of. The base levels of GH and insulin growth factor-1 （IGF-1） were normal or higher, but nadir GH levels were all still 〉1 μg/L in 75 g oral glucose tolerance test. Magnetic resonance imaging detected enlarged sella, partial empty sella and compressed pituitary. The transsphenoidal surgery was performed in 2 cases, and the other patients were conservatively managed. All the patients were in clinical remission. Conclusions When the clinical presences, endocrine evaluation, biochemical examination and imageology indicate spontaneous remission of GH hypersecretion in patients with gigantism or acromegaly, the diagnosis of subclinical apoplexy of pituitary GH adenoma should be presumed. To these patients, conservative therapy may be appropriate.

Acromegaly in a domestic short-haired cat: First report from Iran

A 12-year-old cat, weighting 7.4 kg, suffering from fatigue, exercise intolerance, polyphagia, polyuria and polydipsia was presented. Obesity, a massive head, inferior prognathia and widened inter-dental spaces were noted on examination. Radiographic surveys showed organs enlargement. Labaratory results revealed hyperglycemia and glycosuria. Based on fasting hyperglycemia, concurrent hyperglycemia and glycosuria, diagnosis of diabetes mellitus was made. However, according to the poor diabetic regulation, clinical signs and the absence of other diseases, a tentative diagnosis of acromegaly was confirmed by increased plasma levels of growth hormone. Managing diabetes mellitus with increasing doses of insulin was the only possible therapeutic strategy.

Growth hormone and gastrointestinal malignancy:An intriguing link

Growth hormone(GH)excess is associated with several systemic complications,one of which is the increased risk of neoplastic processes particularly of the gastrointestinal(GI)tract.Among the GI neoplasms,the most reported association is with benign and malignant neoplasms of the colon.In the majority of published literature,an increased incidence of GI neoplasms,both colonic adenomas as well as colorectal carcinoma is reported.However,the studies on colon cancer-specific mortality rate are conflicting with recent studies reporting similar cancer-specific mortality rates in comparison to controls.Many studies have reported an association of colorectal neoplasms with GH levels.Pathogenic mechanisms put forward to explain this association of GH excess and GI neoplasms primarily involve the increased GH-insulin-like growth factor 1(IGF-1)signaling.Both GH and IGF-1 have proliferative,anti-apoptotic,and angiogenic effects on the systemic tissues leading to cellular proliferation.Other contributing factors to the increased risk of GI neoplasms include slow intestinal transit with a redundant large bowel,altered bile acids,deranged local immune response,shared genetic susceptibility factors and hyperinsulinemia.In view of the increased risk association,most guidelines for the care of acromegaly patients recommend an initial screening colonoscopy.Recommendations for further follow-up colonoscopy differ but broadly,the guidelines agree that it depends on the findings at first colonoscopy and state of remission of GH excess.Regarding the concern about the risk of colorectal cancers in patients receiving recombinant GH therapy,most cohort studies do not show an increased risk.

Respiratory Disorders in Acromegalic Patients

Purpose: To evaluate the prevalence and clinical performance of obstructive sleep apnoea syndrome (OSAS) in acromegalic patients, focusing on the possible correlation between alterations of pulmonary microcirculation and patient’s clinical and hormonal parameters. Methods: We enrolled 22 acromegalic patients with apnea hypopnea index (AHI) ≥ 5 if symptomatic, or ≥15 in the absence of sleep related symptoms. Patients underwent the following evaluations: GH and IGF-1 serum levels, arterial blood gas test, spirometry, carbon monoxide diffusing capacity (DLCO), home-based cardiorespiratory sleep and pulmonary function test. Results: The prevalence of OSAS was 66.6%. Patients with a severe form of OSAS appeared to be affected from acromegaly for more years than those with a lower severity of disease and those with acromegaly duration > 10 years had significantly higher levels of glycated hemoglobin. We observed a positive correlation between GH level at diagnosis and DLCO value, showing that acromegalic patients have an increase in static and dynamic respiratory volumes and alveolar-capillary exchange surface compared to the general population. We also observed a positive correlation between baseline serum IGF-1 level and DLCO/VA and between years of disease and DLCO/VA, showing the association of increased lung volume and increase of the exchange surface. Conclusions: The results of this study add new information on lung volume and alveolar gas exchange in acromegaly. Our findings highlight the role of SSA as therapy with positive impact on complication and comorbidities of acromegaly.

A Case of Ectopic Growth Hormone Releasing Hormone (GHRH) from Pancreatic Neuroendocrine Tumor Resistant to Therapy

Introduction: Ectopic secretion of GHRH is a rare cause of acromegaly. However, its recognition is clinically important because different therapeutic approaches are required. Case Presentation: We present a challenging case of acromegaly secondary to ectopic GHRH secretion from pancreatic neuroendocrine tumor in a 52-year-old female. The patient is treated with different modalities which include pegvisomant in an attempt to control the stimulated GH-axis considering the limited data about its use in treatment of ectopic acromegaly. Conclusion: GHRH-secreting tumor is a rare cause of acromegaly. Surgical resection of the tumor is the therapy of choice whenever possible. However, further studies are warranted for unresectable tumor or resistant cases.

Abducens nerve palsy during pegvisomant therapy in an acromegalic patient

Pegvisomant is a recent treatment modality of acromegaly, which is most effective in regulating IGF-1 levels. Tumor growth has been reported in 5% of the acromegaly cases treated with pegvisomant. Herein we report a case of acromegaly resistant to surgical treatment, radiotherapy and medical therapy with a somatostatin analogue and cabergolin. Due to presence of pituitary tumor with cavernous sinus invasion and unremitting disease activity pegvisomant therapy was initiated. She developed left abducens nerve palsy after pegvisomant treatment. The objective of this report is to emphasize the value of close follow-up of the acromely cases under pegvisomant therapy, especially if they have cavernous sinus infiltrating tumor. A 44-year-old woman with acromegaly presented with visual defect and diplopia on left lateral gaze. Her complaint developed 3 years after initiation of pegvisomant therapy. Her neurologic examination was consistent with mild abducens nerve palsy on left side. Other causes for abducens nerve palsy were excluded. Pegvisomant was discontinued upon patient request. Although sella MRI did not reveal a tumor growth, even minimal growth within cavernous sinus can cause cranial nerve involvement. Therefore close and careful follow-up of cavernous sinus infiltrating tumors after pegvisomant therapy is crucial to early recognition of complications.

Comparative Study between Microscopic and Endoscopic Trans-Sphenoidal Surgery for Growth Hormone-Secreting Pituitary Adenomas with Super-Sellar Extension

Background: Advances in the era of modern micro-neurosurgery enabled a reduction of surgical invasiveness and brain retraction which has been defined as minimally invasive or keyhole surgery. Sinonasal endoscopy has brought radical changes in the concepts of pathophysiology and treatment of sinonasal aliments as well as surgical techniques. Aim of the Study: To compare between the use of endoscopic and microscopic trans-sphenoidal approach in resection of growth hormone-secreting pituitary adenomas with Suprasellar Extension. Patients and Methods: This is a prospective study. It had been conducted upon 20 patients having growth hormone secreting pituitary adenoma admitted to Neurosurgery department in Alzar University hospitals and Nasr City Insurance hospital from 2015 to 2018, divided into 2 groups;group A (10 cases) underwent endoscopic endonasal trans-sphenoidal pituitary adenoma resection, while group B (10 cases) operated upon using the standard microscopic trans-sphenoidal pituitary adenoma resection. The inclusion criteria were included: All patients with growth hormone secreting pituitary adenomas showing manifestations of acromegaly, mass effect or hormonal disturbance. Results: This study showed that improvement in outcome was higher in endoscopic group opposed to microscopic group (100% vs. 71% improved headache, 80% vs. 60% visual improvement, 75% vs. 20% fundus improvement and 60% vs. 30% field improvement). Conclusion: We concluded that fully endoscopic procedure result in improved rates of complete tumor removal and a reduced incidence of complications, when compared to the microscopic approach.