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PubMed1982

Colonic polyps in patients with acromegaly.

Seventeen patients with acromegaly were prospectively studied with barium enemas and colonoscopy to investigate the possibility that acromegaly is associated with an increased frequency of colonic polyps and colon cancer. Polyps were identified in nine patients and were removed and examined in eight. In five patients the polyps were adenomatous, and in four of the five there were multiple polyps. The presence of polyps closely correlated with the presence of skin tags (p = 0.041) and also with the age of the patient (p less than 0.01). No new cases of colonic cancer were discovered; however, reviewing the records of 44 patients with acromegaly, four cases were previously diagnosed with colon carcinoma. This study identifies a unique group of patients that are at risk for the development of colonic polyps and perhaps colon cancer.

Acromegaly↗
PubMed1976

Insulin secretion and glucose tolerance in acromegaly.

Thirty patients with active acromegaly were divided into five groups according to the results of their IRI and blood glucose curves in the course of oral glucose tolerance test. Young acromegalics with short duration of acromegaly had grossly exaggerated IRI response with the peak in the first half-an-hour and the flat blood glucose curve. In the second group the peak of IRI response was in the first hour and the blood glucose curve rose to high values in the early stage after the glucose load. The IRI response in the third group was still exaggerated but delayed and the diabetic type of blood glucose curve was found in some of these patients. Acromegalics with overt diabetes mellitus lost their exaggerated IRI response and the IRI curves were flat in those with more advanced disturbance of glucose tolerance. It was suggested that the groups described might represent individual stages in the development of diabetes mellitus in acromegaly. Unclear position in this scheme is kept by the group of active acromegalics with IRI response in normal limits and glucose tolerance unimpaired.

Acromegaly↗
PubMed2000

[Acromegaly in 2000].

Acromegaly is a severe disease often diagnosed late. Morbidity and mortality are high, particularly due to cardiovascular and respiratory complications. Clinical diagnosis is often difficult to confirm, particularly in ectopic forms. In addition, recent reassessment of cure criteria have reduced the number of patients considered to be successfully cured. The therapeutic strategy for acromegaly is thus changing with the advent of somatostatin agonists. Medical treatment is proposed increasingly as the first intention treatment, particularly antitumoral therapy for macroadenomas with surgery or radiotherapy being used as second line treatment. The recent development of GH analogs offers new perspectives for the treatment of acromegaly.

Acromegaly↗
PubMed2000

Surgical treatment of valvular heart disease in patients with acromegaly.

BACKGROUND AND AIM OF THE STUDY: Acromegaly is associated with heart disease in one-third of patients, and diastolic dysfunction may precede global systolic dysfunction. Patients with acromegalic heart disease may have valvular disease, but the role of surgery in such patients has not been established. The purpose of this study was to document the outcome of surgery in a series of these patients from one institution. METHODS: Among 951 patients with the diagnosis of acromegaly seen at our institution since 1972, 10 (eight men, two women) have undergone operation for valvular heart disease. Average patient age was 62.2 +/- 11.5 years; average body weight was 84 +/- 13 kg; average height was 178 +/- 12 cm. The mean duration of acromegaly was 15.2 +/- 12.7 years. At the time of heart surgery, seven patients had active disease, defined by elevation of growth hormone levels, while three had inactive disease. Treatment of pituitary adenomas before valvular surgery included surgical resection in three patients and external-beam radiation treatment in four. The preoperative ejection fraction was 42 +/- 19% (range: 20% to 66%). Valve lesions included aortic stenosis in four patients, aortic regurgitation in four, and mitral regurgitation in three (one patient had double valve disease). RESULTS: Valve replacement was performed in all patients with aortic disease (two bioprostheses, six mechanical), and three patients with mitral regurgitation had repair. Concomitant procedures performed in seven patients included coronary bypass (two), left ventricular aneurysmectomy (two), and ligation of the left atrial appendage, septal myectomy and defibrillator insertion (one each). Early complications included endocarditis, low cardiac output and arrhythmia in one patient each. There were no perioperative deaths. One patient underwent reoperation ten years later for a perivalvular leak. CONCLUSION: Valvular surgery can be performed safely in acromegalic patients, even those with active endocrinopathy.

Acromegaly↗
PubMed2000

[Combined therapy of acromegaly with a combination of lanreotide and terguride].

BACKGROUND: The aim of the treatment of acromegaly is to normalise the hormonal activity. Besides the surgical and radiation treatment, medical therapy can be used. The project was set to determine the value of combined therapy with lanreotide and terguride in patients with active acromegaly. METHODS AND RESULTS: Nine patients previously treated with lanreotide for at least one year without normalisation of hormonal activity were included in the study. Terguride was added to lanreotide for one month. The combined treatment brought about reduction of growth hormone (GH) levels, however, with marginal significance only. GH-BP, IGF-I and IGFBP-3 serum levels were not changed significantly. Considering the individual cases, the combined treatment resulted in normalisation of GH levels in one patient and that of IGF-I in another one. Substantial decrease of GH levels (> 50%) was found in three patients and that of IGF-I (> 20%) in another one patient. CONCLUSIONS: The combined treatment of acromegaly appears to be more effective than monotherapy with lanreotide only in a subset of acromegalic patients.

Acromegaly↗
PubMedDate not supplied

Diabetic ketoacidosis in a patient with acromegaly.

Abnormalities of glucose metabolism are a common feature of acromegaly. Overt diabetes mellitus develops in about 10-15% of patients. We present an unusual complication of acromegaly: a 37-year old man with a 2-year history of acromegaly developed diabetic ketoacidosis 3 weeks after transsphenoidal adenomectomy. No specific cause for this sudden metabolic derangement could be demonstrated. Insulin need was very high in the first days after the onset of ketoacidosis, but was considerably reduced after initiation of treatment with octreotide and after successful re-operation.

Acromegaly↗
PubMed2001

Focal segmental glomerulosclerosis associated with acromegaly.

Experimental studies in rats have demonstrated an association between focal segmental glomerulosclerosis (FSGS) and growth hormone, but patients with FSGS complicating acromegaly are very rare. In this report we present a case of FSGS associated with acromegaly. With a long history of soft tissue swelling of hands and feet, elevated plasma growth hormone levels and other biochemical abnormalities, a 53-year old male had suffered from acromegaly for over 15 years. He had moderate proteinuria for 6 years, but never evidenced nephrotic syndrome. A renal biopsy specimen revealed FSGS and glomerular hypertrophy. Trans-sphenoidal surgical removal of the pituitary adenoma resulted in the normalization of elevated growth hormone and insulin-like growth factor I levels, but proteinuria continued. This case suggests that the overproduction of growth hormone may participate, at least in part, in the development of human FSGS. It is possible that once FSGS is present in an acromegalic patient, cessation of GH overproduction may not be enough to reverse it.

Acromegaly↗
PubMed2001

[Two cases of B-cell disease coexisting with acromegaly].

The first two Danish cases of a B cell disease co-existing with acromegaly are reported, although over the last twelve years similar cases have been reported worldwide. We can only conclude that acromegaly might predispose to B cell diseases and therefore it is still important to report new cases. It is relevant to consider acromegalic patients as having an elevated risk of developing such diseases. Any signs of acromegaly should be taken seriously in patients with B cell diseases. We also suggest that abuse of GH, as seen in certain sports, may increase the risk of developing malignant B cell diseases.

Acromegaly↗
PubMed2002

[Acromegaly: a disorder with distinguished features yet delayed diagnosis].

The authors review the historical and epidemiological aspects, as well as the distinguishing clinical features and complications of acromegaly to emphasize the importance of early diagnosis and treatment of patients with this disorder. Acromegaly is a rare disorder with a prevalence of 55-69 cases per million population and an incidence of 3-4 newly detected cases per million per year. Recent estimates indicate, that its slow progression and insidious course leads to a considerable time lag of 7-12 years between the first symptoms and the diagnosis of the disease, although younger patients with more severe disease may be detected earlier. In addition to the disfigurement and disability which develop progressively from the onset of the disease, acromegaly is associated with a number of complications resulting in a two- or three-fold increase of mortality and a decrease of life expectancy by about 10 years. The major causes of death include cardiovascular and cerebrovascular events, respiratory disease and malignancy. Because the duration of the disease before effective therapy may be one of the major predictors of increased mortality, practicing doctors need to be particularly vigilant to recognize undiagnosed cases in order to avoid the serious complications of the disease.

Acromegaly↗
PubMed2000

[Dilated cardiomyopathy secondary to acromegaly].

In acromegaly, dilated cardiomyopathy is rare. Indeed, arterial hypertension, coronary artery disease and hypertrophic cardiomyopathy are more common. The authors report a case of a 42-years-old man with dyspnea (class III NYHA) and palpitations. The cardiac echography showed a dilated cardiomyopathy. The investigations revealed an acromegaly as an etiology. Diagnosis was based on patient morphotype, a high level of growth hormone (GH) and a pituitary adenoma detected by magnetic resonance imagery. This case is characterized by the absence of arterial hypertension and hypertrophic cardiomyopathy. The coronary angiography was normal. Consequently, in this particular case, dilated cardiomyopathy seems specific to acromegaly.

Acromegaly↗
PubMed2003

[Spontaneous remission of acromegaly after meningitis: a case report].

There have been several reports describing the cases of acromegaly, which show reduction in size of tumor in due to pituitary apoplexy or lymphocytic hypophysitis. We have encountered a patient of acromegaly, who developed panhypopituitarism after suffering from meningitis and showed the reduction of tumor in size. The results of imaging examination suggested the presence of pituitary apoplexy and lymphocytic hypophysitis. The patient was a 27-year-old woman, who visited a local physician with complains of headache and fever. After performing lumbar puncture, she was diagnosed as viral meningitis, and conservative therapy was initiated. The results of biochemical test of blood revealed hyponatremia. Because facial appearance of the patient was similar to that of acromegaly, endocrine dysfunction was suspected. The result of pituitary hormone tests showed high levels of growth hormone (GH) and somatomedin C (IGF-1) and low levels of the other hormones. At the same time, sign of diabetes insipidus was noted, and the patient was referred to our hospital. In the examination at the admission, GH and IGF-1 showed the trends to decrease, and the reduction in size of tumor was also detected. From the results of imaging examination, pituitary apoplexy and lymphocytic hypophysitis were suspected. Operation was performed, and pathological examination revealed inflammation of pituitary adenoma.

Acromegaly↗
PubMed2003

[Clinical case of the month. Anterior mediastinal mass in a patient with acromegaly].

The discovery of an anterior asymptomatic mediastinal mass with acromegaly creates a dilemma for the clinician. As acromegaly can be due to a pituitary tumor but also to an ectopic production of GHRH, the first diagnostic step consists of finding the etiology of the disease in order to use adequate treatment. This can be complicated by the fact that acromegaly can be associated with other tumors (for instance in MEN-1 disease and Carney Complex) and because chronic GH hypersecretion can stimulate tumor overgrowth and perhaps tumor formation. We describe the case of a 44-year-old acromegalic woman with an anterior mediastinal mass. We present the different diagnoses and a review of the literature.

Acromegaly↗
PubMed1992

Ectopic acromegaly.

Ectopic acromegaly is a rare syndrome (less than 1% of acromegalic patients) caused by ectopic growth hormone-releasing hormone (GHRH) or growth hormone (GH)-producing tumors. Its recognition is clinically important because acromegaly may be a symptom of an aggressive tumor, and different therapeutic approaches are required. Most cases are caused by either extra- or intracranial GHRH-producing tumors, whereas in rare instances the underlying disease is an ectopic GH-secreting tumor. The routine evaluation of circulating GHRH in all acromegalic patients may allow its early recognition, because plasma levels greater than 0.3 ng/mL are virtually diagnostic of a GHRH-producing tumor (frequently a bronchial or pancreatic carcinoid), whereas suppressed levels may suggest an ectopic GH-producing tumor. In addition to classic imaging techniques, whole body scintiscan with labeled octreotide may help in the localization of ectopic tumors. Surgical removal of the ectopic tumor is the therapy of choice, but it is not always feasible because patients often present with widespread metastases. Patients with GHRH-induced acromegaly benefit from the administration of the long-acting somatostatin analog, octreotide, which reduces GH, IGF-I, and GHRH, and may shrink the ectopic tumor, its metastases, and the secondary pituitary enlargement.

Acromegaly↗
PubMed1992

Clinical manifestations of acromegaly.

Acromegaly is an uncommon disorder and may present in a variety of ways, leading to considerable delay in diagnosis. Unlike other pituitary tumors, tumors associated with acromegaly tend to be fairly large in most patients. Thus, symptoms may be commonly due to the tumor mass as well as to hormone oversecretion. Mortality is two- to threefold increased due to cardiovascular, respiratory, and neoplastic causes. An increase in diabetes mellitus and hypertension may contribute to the first of these. Early treatment may reverse the diabetes, soft tissue changes, sleep apnea, cardiovascular disease, and neuromuscular disease. The effect of early treatment on neoplasia is unclear, and patients probably should continue to be screened, especially for colon neoplasia, even after appropriate therapy for the acromegaly. Hypopituitarism may be present initially as a result of tumor mass but may also develop as a result of ablative therapy.

Acromegaly↗
PubMed2004

[Acromegaly and sleep apnea].

OBJECTIVE: Acromegaly is often associated with sleep apnea-hypopnea syndrome (SAHS). The purpose of this study was to understand the prevalence of SAHS in patients with acromegaly and define the characteristics of acromegalic patients with and without SAHS. PATIENTS AND METHODS: The study enrolled 17 patients (11 women and 6 men) residing in the province of Ourense, Spain, who were diagnosed with acromegaly. All patients underwent overnight polysomnography in a sleep laboratory. In addition, growth hormone and insulin-like growth factor 1 levels were assessed. Sixteen of the patients underwent cephalometric study. RESULTS: The average age of the patients was 58 years (95% confidence interval [CI], 52-63). The average body mass index was 31 (95% CI, 29-34) and average neck circumference was 41 (95% CI, 39-43). Ten patients (58.8%) had an apnea-hypopnea index (AHI) greater than 10. Nine had obstructive apnea and one had central apnea. Seven (5 with an AHI>10 and 2 with an AHI<10) reported excessive daytime sleepiness with Epworth scores greater than 10 (41.2%). Five patients (29.4%) were diagnosed with SAHS (AHI>10 and Epworth>10). No correlation was found between an AHI greater than 10 and hormonal activity (P=.082). The mean growth hormone level for patients with an AHI greater than 10 was 4.8 (95% CI, 0.5-9) and the mean for those with an AHI less than 10 was 12 (95% CI, 2-27). Fifty percent of the patients were treated with a somatostatin analog and half of those treated exhibited apnea (P=.302). No cephalometric differences related to the presence of apneas were found. CONCLUSIONS: We found a high prevalence of sleep apneas (58.8%) and SAHS (29.4%), and central apneas were rare. We found no correlation between hormone activity level and the presence of SAHS. The incidence of SAHS was the same in somatostatin analog treated and untreated patients. Cephalometric variables did not distinguish between acromegalic patients with and without SAHS.

Acromegaly↗
PubMed2004

Current diagnostic guidelines for biochemical diagnosis of acromegaly.

Acromegaly is a rare and chronic disease that, in the majority of cases, is due to the presence of a benign growth hormone (GH)-producing tumor of the pituitary. In the past, the diagnosis of acromegaly was established basically on physical changes, and only the patients with a severe clinical picture were brought to medical attention. The development of a radioimmunoassay for detecting GH allowed for the first time to confirm the diagnosis biochemically. Subsequently, methods for measuring insulin-like growth factor 1 (IGF-I) became available and added another important biochemical marker for the diagnosis and follow-up of these patients. Progressive improvements in assay methods have allowed for progressively better definitions of normality and, as a result, have permitted the diagnosis to be biochemically established in patients with only mild forms of the disease. Moreover, new potential markers of disease activity, such as other GH-dependent IGF system parameters, have been investigated and proposed in the diagnostic work-up and for monitoring the therapeutic outcome. Optimal assessment of disease activity, for both diagnostic and follow-up purposes, is mandatory. This subject has been strongly debated regarding proper cut-off values using highly sensitive GH assays as well as the problems linked to IGF system components measurement. Consequently, several consensus reports, as well as original studies, have been issued giving special attention to diagnostic procedures, cut-off revisions and definition of disease activity. The present review discuss principally the biochemical diagnosis of acromegaly based on these articles and on the experience collected in an endocrinological unit considered as reference center for pituitary diseases.

Acromegaly↗
PubMed2005

Role of medical therapy in the management of acromegaly.

Medical therapy plays an important role in the management of patients with acromegaly and is commonly used adjunctively after surgical resection of the pituitary tumor. Generally, surgery alone provides a 50 to 70% rate of cure; however, the outcome depends on the experience and ability of the surgeon and the characteristics of the tumor. The role of postsurgical medical therapy is to achieve long-term biochemical control of the growth hormone (GH)/insulin-like growth factor I (IGF-I) axis. In some patients, medical therapies may be implemented sooner as primary or preoperative therapy. Somatostatin analogs have been the mainstay of medical therapy for acromegaly. The somatostatin analog octreotide produces normalization of IGF-I in approximately 50% of patients but is associated with gastrointestinal adverse effects, including the development of gallstones. Octreotide requires thrice-daily subcutaneous administration. Long-acting formulations of somatostatin analogs (octreotide LAR, lanreotide) are at least as effective and as well tolerated as short-acting octreotide. Unfortunately, some patients are suboptimally responsive to or become intolerant of these agents. Pegvisomant belongs to a new class of agents known as GH-receptor antagonists. This novel agent competitively binds to the GH receptor, blocking IGF-I production. Pegvisomant is highly effective in achieving normal IGF-I concentrations and in reducing signs and symptoms of acromegaly, even in patients resistant to previous treatments. Pegvisomant has been proved safe and well tolerated and has no effect on gallbladder motility. GH levels remain elevated. Transient elevations in liver enzyme levels require monitoring but rarely necessitate termination of therapy. Normalizing IGF-I concentrations with pegvisomant also may have a beneficial effect on carbohydrate metabolism and cardiovascular risk.

Acromegaly↗
PubMed2006

Canadian consensus guidelines for the diagnosis and management of acromegaly.

Acromegaly is a chronic condition associated with considerably increased morbidity and mortality if left unchecked. In December 2004, a national meeting was held to discuss the diversity in clinical practice across the country in diagnosing and treating patients with acromegaly, as well as to seek consensus on a number of management principles. The group reviewed recent guidelines and discussed issues of diagnosis, treatment, monitoring and treating comorbidities to seek a Canadian consensus on the management of this rare disorder. Consensus was that diagnosis should include clinical and biochemical findings, but is hinged on establishing GH hypersecretion with IGF-I and OGTT testing. Treatment has traditionally included surgical resection or debulking, along with adjunctive medical therapy (primarily somatostatin analogues), if necessary, to normalize GH levels. The option of primary medical therapy in managing this condition has recently emerged and can be justified for non-surgical candidates or for those in whom surgery is not expected to be curative. Overall, improved screening practices and superior epidemiological data are required, since timely diagnosis and appropriate treatment are crucial for reducing the potentially debilitating effects of this chronic, progressive disease. The current evidence also supports the need for long-term follow-up of disease activity and comorbidities in diagnosed patients. A national meeting was held to discuss the diversity in clinical practice across the country in diagnosing and treating patients with acromegaly, as well as to seek consensus on a number of management principles. After brief reviews of the most recent Canadian guidelines and the 2004 guidelines published by the American Association of Clinical Endocrinologists, the group was asked to specifically examine the issues of diagnosis, treatment, monitoring and treating comorbidities and seek a Canadian consensus on practice. This paper summarizes the working group's findings and the points of consensus that were achieved.

Acromegaly↗