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PubMed1980

Urinary 3',5'-cyclic adenosine monophosphate in relation to serum and urinary calcium in acromegaly and primary hyperparathyroidism.

A comparison of the interrelations between serum and urinary calcium values and the urinary excretion of cAMP in acromegaly (No. of subjects: 26), patients with primary hyperparathyroidism (n = 18) and control subjects (n = 42) is presented. The cAMP excretion was greatest in primary hyperparathyroidism, but acromegalics also exhibited higher values for this parameter than controls. A positive correlation was found between serum calcium values and cAMP in primary hyperparathyroidism, while acromegalics showed no correlation between these parameters. In controls there was a negative correlation between serum calcium and cAMP. Serum calcium levels corrected for variations in total protein concentrations were elevated both in acromegaly and primary hyperparathyroidism, mostly in the latter. Acromegalics and patients with primary hyperparathyroidism exhibited an increase in 24 h calcium excretion. While there was a negative relationship between urinary calcium excretion and cAMP in acromegaly, a positive correlation between these parameters as found in primary hyperparathyroidism. Controls showed a negative correlation between urinary calcium values and cAMP. It is concluded that the role of the parathyroids in the regulation of calcium metabolism in acromegaly is different from that of both normal controls and primary hyperparathyroidism. It is postulated that an active form of Vitamin D plays a major role in the regulation of calcium metabolism in acromegaly.

Acromegaly↗
PubMed1995

Familial acromegaly: a specific clinical entity--further evidence from the genetic study of a three-generation family.

Familial acromegaly is a very rare inherited disorder, characterized by the clustering within a single family of several related cases with somatotroph adenomas and acromegaly. The causes of these dominantly inherited pituitary tumours remain unknown. Although these families have a clinical presentation distinct from that of multiple endocrine neoplasia type 1 (MEN-1), the question of this syndrome as being linked to the MEN-1 locus has remained open. Our aim was to study a three-generation family with cases of acromegaly in a mother and her son, to explore better the clinical presentation of the disease, its pattern of inheritance and to test the hypothesis of a genetic linkage to the MEN-1 locus using closely linked polymorphic genetic markers. The refined analysis of 15 unaffected relatives revealed miscellaneous non-specific endocrine dysfunctions and the presence of multiple lipomata, as noted previously in some cases. Moreover, the notion of acromegalo-gigantism in the maternal grandmother and an incomplete penetrance appeared even more typical, suggesting that familial acromegaly is a specific clinical entity. Finally, under the hypotheses assumed for segregation analysis, no clinical, biological or genetic evidence of linkage to the MEN-1 locus could be retained in this family. However, these conclusions were limited because of incomplete penetrance and uncertain definition of the carrier status. Therefore, we conclude that further identification of the genetic predisposition to familial acromegaly might be obtained from the combined molecular genetic analysis of several families presenting with the same clinical features.

Acromegaly↗
PubMed2002

Growth hormone deficiency and replacement in hypopituitary patients previously treated for acromegaly or Cushing's disease.

OBJECTIVE: To compare baseline characteristics in adult patients with growth hormone (GH) deficiency (GHD) who had previously been treated for Cushing's disease or acromegaly with data from patients with GHD of other aetiologies. To study the effects of GH therapy in those patients who had completed at least 6 months of GH replacement. DESIGN: Data from a large outcomes research database (KIMS (Pharmacia International Metabolic Database)). METHODS: 135 patients were identified with previous Cushing's disease, 40 had had acromegaly, and 1392 had GHD of other aetiologies. The number of additional hormone deficiencies, and the mean age of the patients were similar in the three groups. Similar proportions of patients in each group were treated using surgery, but radiotherapy was used more often in patients with acromegaly than those with other diagnoses. RESULTS: At baseline, the prevalence of diabetes mellitus and hypertension were significantly higher in the group treated for Cushing's disease, and the prevalence of stroke was significantly higher in the group treated for acromegaly. The incidence of coronary heart disease and claudication were similar in all three groups. Patients treated for Cushing's disease had lower bone mineral density and suffered fractures more often than other GHD adults. Body mass index, waist-hip ratio, serum concentrations of lipids and standard deviation scores of serum concentrations of insulin-like-growth factor-I were similar in the three groups. The dose of GH administered was comparable in the three groups and the effects of GH replacement on waist circumference, blood pressure and quality of life were also similar across the groups. The numbers and types of adverse events reported were not different between the groups. CONCLUSIONS: These data suggest that the characteristics of patients in these diagnostic groups depend on the primary disease which resulted in GHD, and that the clinical expression of GHD does not differ between the groups. Patients with previous hypercortisolism showed more long-term effects of their disease, such as diabetes mellitus, hypertension and fractures. A benefit from GH replacement was evident in patients previously treated for acromegaly and Cushing's disease particularly in relation to quality of life.

Acromegaly↗
PubMed2004

Remission criteria for the follow-up of patients with acromegaly.

OBJECTIVE: The aim was to evaluate the validity of current remission criteria in acromegaly, a random GH level of <2.5 microg/l, a glucose-suppressed GH level of <1 microg/l and a normal IGF-I level. DESIGN: In forty-one patients treated for acromegaly (23 males and 18 females, 20-69 years) and 94 healthy subjects (50 males and 44 females, 20-78 years), basal GH and IGF-I levels and nadir GH levels after 75 g oral glucose were evaluated in decade blocks; these were assayed by sensitive immunoradiometric assays. RESULTS: Basal GH levels varied widely from 0.022 to 10.4 in healthy subjects and were >2.5 microg/l in 19%. The mean post-glucose GH nadir was 0.067+/-0.009 microg/l (range 0.003-0.4 microg/l) and the upper limit of the GH nadir was 0.26 microg/l (means+2 S.D.) in healthy subjects. Thirty-five patients with acromegaly had high-for-age IGF-I levels in relation to our healthy subjects. In this group, 15 (42.9%) patients had basal GH levels of <2.5 microg/l, 14 (40%) patients had nadir GH levels of <1 microg/l, and three (8.6%) patients had GH suppression to <0.26 microg/l which was defined as normal GH suppression in our healthy subjects. Only six patients with acromegaly had normal-for-age IGF-I levels and all of these patients had basal GH levels of <2.5 microg/l and all but one had nadir GH levels of <0.26 microg/l. CONCLUSIONS: A basal or random GH level of <2.5 microg/l is not a reliable criterion for remission in acromegaly and the currently accepted normal upper limit of 1 microg/l for post-glucose GH suppression is too high. Post-glucose nadir GH levels, measured with sensitive assays, can be <1.0 microg/l in 40% and basal GH levels can be <2.5 microg/l in 43% of the active acromegalic patients. IGF-I levels appeared to correlate better with a nadir GH cut-off of 0.26 microg/l rather than 1 microg/l in the determination of disease activity.

Acromegaly↗
PubMed2005

Effectiveness of adding dopamine agonist therapy to long-acting somatostatin analogues in the management of acromegaly.

BACKGROUND: The excess mortality and morbidity associated with acromegaly are secondary to prolonged elevation of GH and IGF-I. Vigorous control of these biochemical parameters results in improved morbidity and mortality. Somatostatin analogues (SAs) allow adequate control of GH and IGF-I in approximately 65% of subjects, leaving a significant cohort uncontrolled. Dopamine agonists (DAs), a cheap alternative to SAs, allow control of GH and IGF-I in less than 20% of patients with acromegaly. AIMS: To assess the effectiveness of adding DA therapy to SA in the biochemical control of acromegaly. SUBJECTS: One hundred and twenty cases from the Sheffield Acromegaly Register were reviewed; 24 (20%) did not require medical treatment following pituitary surgery alone; 16 (13%) had safe GH levels following surgery and radiotherapy; and 58 (48%) required medical treatment despite having had surgery, radiotherapy or both. The remaining 22 (18%) received only medical treatment. METHODS: In nine subjects a DA (three bromocriptine, six cabergoline) was added to an SA to control active disease. GH day curves and IGF-I levels were compared before and after the addition of a DA to existing SA treatment. All were on stable maximum-dose treatment with an SA, with inadequate biochemical control prior to addition of DA therapy. Mean duration of treatment on a DA before biochemical assessments were made was 10.3 months. Six subjects had previously been treated with either transsphenoidal surgery, radiotherapy or both. In three subjects SA was the primary therapy. RESULTS: All subjects exhibited a fall in median GH and IGF-I levels. Introduction of a DA resulted in a 36.1% reduction in median GH levels (8.3 vs 5.3 mIU/l; P = 0.008) on a GH day curve and a 35.2% reduction in IGF-I levels (387.2 vs 251.0 microg/l; P = 0.018). Only four subjects had elevated prolactin levels prior to the addition of a DA (>368 mIU/l). CONCLUSION: Addition of DAs to SAs is of benefit in the biochemical control of acromegaly and should be considered in those inadequately controlled. Furthermore, the beneficial effects of DAs occur even when pre-treatment prolactin levels are within the normal range.

Acromegaly↗
PubMed2005

Acromegaly in a multiple endocrine neoplasia type 1 (MEN1) family with low penetrance of the disease.

Multiple endocrine neoplasia type 1 (MEN1) is an inherited syndrome that is characterised by the occurrence of tumours in the parathyroid glands, the endocrine pancreas, the pituitary gland and the adrenal glands and by neuroendocrine carcinoid tumours, often at a young age. The penetrance of MEN1 among gene carriers is reported to be high; 82-99% at age 50. We present a patient with a history of parathyroid adenomas also showing signs of acromegaly. He turned out to be a carrier of a MEN1 germ-line mutation in intron 3 (IVS3-6C > G). This germ-line mutation was also found in nine of his family members. However, none of these relatives have developed any MEN1-related lesion yet, although several are older than 60 years. To our knowledge, a MEN1 family with as few clinical features as this family has not been reported to date. Because MEN1 patients have an increased risk of developing acromegaly, insulin-like growth factor (IGF-I) levels are monitored periodically. We investigated whether IGF-I levels might serve as a presymptomatic marker for acromegaly; 9% (3/33) of MEN1 patients showed temporary IGF-I elevations. One patient (1/3) later developed clinical signs of acromegaly. Possibly, acromegaly in MEN1 is preceded by a transient preacromegalic state.

Acromegaly↗
PubMed1990

Increased activity of digoxin-like substance in low-renin hypertension in acromegaly.

UNLABELLED: Arterial hypertension is common in acromegaly, but the pathogenesis of this complication remains unknown. To determine the role of an endogenous Na,K pump inhibitor/digoxin-like substance (DLS) in the pathogenesis of hypertension in acromegaly 76 subjects: 28 with acromegaly, 20 with essential hypertension and 28 healthy controls were studied. Serum DLS was measured with the use of radioimmunoassay and bioassay by the inhibition of digoxin-sensitive erythrocyte 86-Rb uptake. In acromegaly, the activity of DLS was significantly increased and plasma renin activity decreased in the hypertensive group, as compared with that of the normotensive group and controls. Moreover, DLS was elevated in the low-renin group of essential hypertension, as compared with that of the normal/high-renin group or controls. The activity of DLS correlated positively with mean arterial pressure and negatively with plasma renin activity, but not with growth hormone levels. IN CONCLUSION: an endogenous sodium pump inhibitor/digoxin-like substance may play a role in the pathogenesis of low-renin hypertension in acromegaly.

Acromegaly↗
PubMed2000

Acromegaly associated with a granular cell tumor of the neurohypophysis: a clinical and histological study. Case report.

Acromegaly is usually caused by a growth hormone (GH)-secreting pituitary adenoma, and hypersecretion of GH-releasing hormone (GHRH) from a hypothalamic or neuroendocrine tumor accounts for other cases. The authors report on the unusual association of acromegaly with a granular cell tumor of the neurohypophysis. A 42-year-old woman with a 10-year history of acral enlargement, headache, and menstrual abnormalities was referred to our department for a suspected GH-secreting pituitary adenoma. The patient's basal GH levels were mildly elevated at 4.8 microg/L, were not suppressed in response to an oral glucose tolerance test, and increased paradoxically after administration of thyrotropin-releasing hormone. The patient's insulin-like growth factor-1 (IGF-1) level was elevated at 462 microg/L, whereas a magnetic resonance image of the sella turcica revealed an intra- and suprasellar lesion that was compatible with a diagnosis of pituitary adenoma. A transsphenoidal approach to remove the lesion, which was mainly suprasellar, was successful during a second operative attempt, resulting in the clinical and biochemical regression of the patient's acromegaly. Four months postoperatively, the patient's basal GH level was 0.9 microg/L and her IGF-1 level was 140 microg/L. Histological analysis of the operative specimen demonstrated a granular cell tumor of the neurohypophysis, which when stained proved negative for pituitary hormones and GHRH. This case represents the first reported association between a granular cell tumor of the neurohypophysis and acromegaly. Granular cell tumor of the neurohypophysis could be added to the restricted list of neoplastic causes of acromegaly secondary to hypersecretion of a GH-releasing substance.

Acromegaly↗
PubMed2003

Assessment of long-term remission of acromegaly following surgery.

OBJECT: The criteria for remission of acromegaly following transsphenoidal adenoma resection are in evolution. In the present study the authors evaluate the utility of predicting long-term remission by reference to a single fasting growth hormone (GH) level on the 1st postoperative day. METHODS: A retrospective analysis was conducted on 181 patients with acromegaly who underwent transsphenoidal resection between 1973 and 1990 and completed a 5-year follow-up period. Fasting serum GH levels were obtained in all patients on the 1st postoperative day in the absence of exogenous glucocorticoids. All patients participated in a follow-up evaluation lasting at least 5 years, which included measurements of serum insulin-like growth factor-I (IGF-I) levels as an index of acromegalic activity. Among the 181 patients, GH levels ranged from 0 to 8 ng/ml in 131 (72%) on the 1st postoperative day, suggesting biochemical remission. This group included 107 (84%) of the 127 patients with microadenomas, but only 24 (44%) of the 54 with macroadenomas. Nevertheless, 15 (11%) of the 131 patients who initially had attenuated GH levels displayed recurrent acromegaly within the first 2 years (with elevated levels of IGF-I in all cases, and abnormalities appearing on magnetic resonance images in nine cases). Only one of 116 patients in whom the initial postoperative GH level was lower than 2 ng/ml experienced a recurrence, whereas 14 (93%) of the 15 patients with postoperative GH levels between 2.2 and 8 ng/ml subsequently displayed biochemical evidence of acromegaly. CONCLUSIONS: The findings indicate that a fasting morning serum GH level lower than 2 ng/ml on the 1st postoperative day portends long-term biochemical remission of acromegaly, whereas higher levels are a significant marker for recurrent disease.

Acromegaly↗
PubMed2006

Presurgical treatment with somatostatin analogs in patients with acromegaly: effects on the remission and complication rates.

OBJECT: The question of whether preoperative therapy with somatostatin analogs can improve surgical outcome in acromegaly has not been definitively answered. In this paper, the authors report the effects of preoperative treatment with somatostatin analogs in a large sample of patients with acromegaly. METHODS: Between 1990 and 2003, 399 consecutive patients with acromegaly underwent surgery at the Istituto Scientifico San Raffaele. Thirty-three patients who had previously undergone surgery or radiation treatment, 48 patients treated with somatostatin analogs for fewer than 3 months, and patients who had stopped therapy for too long a time before surgery were excluded from the study. One hundred forty-three patients who had received somatostatin analogs prior to surgery (Group 1) were randomly matched to 143 patients who had never been treated with somatostatin analogs (Group 2). Matching criteria were tumor size and invasiveness into the cavernous sinus. Before surgery, Group 1 patients showed reduction of growth hormone levels to less than 50% of baseline in 64% of cases, but insulin-like growth factor-I was normalized in only 19.5%. Surgical remission occurred in 81 Group 1 patients (56.6%) and in 91 Group 2 patients (63.6%; p = 0.28). No significant difference in the remission rate was observed when cases were analyzed according to tumor size or invasiveness. Logistic regression analysis confirmed that pretreatment with somatostatin analogs was not associated with surgical outcome. Surgical morbidity was mild and similar in Group 1 and Group 2 patients (7 and 5.6%, respectively; p = 0.81). Surgical remission and complication rates in patients with acromegaly who received treatment with somatostatin analogs prior to surgery were not significantly different from those of matched patients who did not receive these agents. CONCLUSIONS: At present, the routine use of presurgical therapy with somatostatin analogs for patients with acromegaly cannot be recommended.

Acromegaly↗
PubMedDate not supplied

Some clues and pitfalls in the diagnosis of acromegaly.

OBJECTIVE: To describe a case of acromegaly in a young woman using oral contraceptives who had nonspecific symptoms and persistent hyperphosphatemia and to analyze the reasons for delay in diagnosis. METHODS: A 31-year-old woman underwent evaluation for failure of menstrual flow to resume after discontinued use of oral contraceptives. Clinical and laboratory findings are summarized, and atypical manifestations of acromegaly are discussed. RESULTS: The patient complained of weight gain, acne, and increased facial hair growth, and laboratory tests showed a low estradiol level and hyperinsulinemia. "Post-pill amenorrhea" and polycystic ovary syndrome were considered possible diagnoses. During subsequent endocrinology consultation, hyperphosphatemia was present on three occasions during a 7-month period. Hormonal studies for evaluation of pituitary function revealed increased insulin-like growth factor-I and growth hormone (GH) levels. Acromegaly was suspected, and the increased GH level failed to suppress after a glucose load. Magnetic resonance imaging revealed a pituitary macro-adenoma with suprasellar extension. Visual field testing showed bitemporal abnormalities. Transsphenoidal resection successfully removed the GH-producing tumor, and hormone replacement therapy was initiated postoperatively. CONCLUSION: The textbook presentation of GH excess is a late feature of acromegaly. The diagnosis in a young woman can be easily missed when clinicopathologic findings are not classic and the picture is further clouded by factors such as oral contraceptive use. Features may also resemble polycystic ovary syndrome, a more common condition in women of childbearing age. This case also illustrates that hyperphosphatemia, overlooked in this patient, can precede the full-blown clinical manifestations of acromegaly and serve as a subtle clue to the underlying disease. Awareness of these situations and screening for high GH levels in patients with unexplained, persistent hyperphosphatemia are advisable for making an early correct diagnosis and providing appropriate therapy.

Acromegaly↗
PubMedDate not supplied

Longitudinal assessment of economic burden and clinical outcomes in acromegaly.

OBJECTIVE: To determine costs and outcomes over time for surgical and various medical regimens in the management of patients with acromegaly. METHODS: We studied a sample of 53 consecutive Canadian patients with acromegaly who underwent a transsphenoidal pituitary surgical procedure only (N = 27) or in conjunction with medical therapy (N = 26). Outcomes were analyzed as person-months spent in various health state outcomes, which were defined on the basis of growth hormone and insulin-like growth factor I levels. Costs are reported in 1998 Canadian dollars. RESULTS: The mean duration of follow-up was 49 months. Of the 53 patients, 25 (47%) had microadenomas at admission. Patients spent as much as 65% of the time in "uncured" health states. Patients with less extensive disease had better outcomes. The mean annual cost per patient was $8,111 (95% confidence interval, $5,848 to $10,374). Medications were the largest contributor to overall cost (38%). Although per patient surgical costs themselves were high (ranging from approximately $2,800 to $9,200), when averaged over the 4 years the mean annual cost was approximately $2,400, less than the cost of medications. Treatment of macroadenomas cost more than treatment of microadenomas ($11,425 versus $4,442 annually). The treatment of acromegaly costs $14.7 million annually in Canada (95% confidence interval, $10.6 to $18.8 million) and, if patterns of care are similar, about $139 million annually in the United States. CONCLUSION: Treatment of acromegaly is no more costly than therapy for other chronic diseases, especially those with a surgical component. Early diagnosis (at the stage of microadenoma) resulted in better outcomes and lower costs. Thus, from the standpoint of economics and well-being, a continued aggressive attitude toward screening programs and treatment of persistently active acromegaly seems warranted.

Acromegaly↗
PubMed1991

Sleep apnea in acromegaly.

OBJECTIVE: To provide information on the nature, prevalence, and severity of sleep apnea in patients with acromegaly. DESIGN: Consecutive case series. SETTING: Tertiary referral hospital. PATIENTS: Fifty-three patients with acromegaly were consecutively referred: 33 patients were referred because of clinical suspicion of sleep apnea and 20 patients were referred without suspected apnea. MEASUREMENTS: Sleep studies as well as growth hormone and insulin-like growth factor 1 (IGF-1) measurements were done. MAIN RESULTS: Thirty-one patients (93%; 95% Cl, 85% to 100%) referred because of suspicion of sleep apnea had sleep apnea compared with 12 patients (60%; Cl, 37% to 83%) referred without suspected sleep apnea. Patients with sleep apnea did not have biochemical evidence of increased disease activity (random growth hormone, 12.7 +/- 4.4 micrograms/L; mean growth hormone at 24-hour sampling, 10.8 +/- 8.4 micrograms/L; IGF-1, 90.0 +/- 7.5 nmol/L) compared with patients without sleep apnea (random growth hormone, 14.2 +/- 4.9 micrograms/L, P greater than 0.2; mean growth hormone, 12.4 +/- 3.5 micrograms/L, P greater than 0.2; IGF-1, 90.0 +/- 10.0 nmol/L, P greater than 0.2). Central sleep apnea was the predominant type of apnea in 33% (Cl, 18% to 47%) of patients and was associated with higher random growth hormone and IGF-1 levels than was obstructive apnea (random growth hormone, 23.4 +/- 3.9 compared with 8.8 +/- 3.1 micrograms/L, P less than 0.001; IGF-1, 126 +/- 17.5 compared with 72.5 +/- 7.5 nmol/L, P less than 0.01). CONCLUSIONS: Sleep apnea is common in acromegaly. The rate of central sleep apnea was unexpectedly high in patients with acromegaly, and biochemical evidence of increased disease activity was associated with the presence of central apnea rather than with the degree of sleep apnea. Altered respiratory control is a possible mechanism producing sleep apnea in acromegaly.

Acromegaly↗
PubMed2000

The pathology of median neuropathy in acromegaly.

BACKGROUND: Median neuropathy is commonly associated with acromegaly, although its pathology is uncertain. OBJECTIVE: To study the pathology of median neuropathy in acromegaly by using magnetic resonance imaging (MRI). DESIGN: Case series. SETTINGS: Outpatient clinic and MRI unit. PATIENTS: Nine patients with acromegaly, four of whom had clinical symptoms of neuropathy. MEASUREMENTS: At presentation and 6 months after treatment, median nerve size, its signal intensity, and the volume of the carpal tunnel contents were measured. RESULTS: At presentation, patients with symptoms of neuropathy had increased nerve size and signal intensity compared with asymptomatic patients, but the two groups did not differ in volume of carpal tunnel contents. These measures improved with treatment of acromegaly in symptomatic patients; asymptomatic patients experienced no change or worsening. CONCLUSION: The predominant pathology of median neuropathy in acromegaly seems to be increased edema of the median nerve within the carpal tunnel rather than extrinsic compression from increased volume of the carpal tunnel contents.

Acromegaly↗
PubMed2002

Filter paper collection of plasma for IGF-I test in patients with acromegaly.

The concentration of insulin-like growth factor I (IGF-I) was determined in paired specimens of liquid plasma and of plasma that had been applied to a filter paper disk, dried, and eluted; the measurements were performed by an immunochemiluminometric assay (ICMA). The 129 paired assays showed good correlation: y = 1.04x + 4.99; R2 = 0.934, where y is the result on dried samples and x is that on liquid samples. In 18 patients with acromegaly who lived on an island off the West Pacific Rim, plasma specimens were collected, applied to filter paper, dried, and mailed to a laboratory in the Upper Midwest of the United States. IGF-I was eluted from the filter paper disks and measured by ICMA. IGF-I concentrations ranged from 101 to 254 ng/ml in 11 patients with newly diagnosed but untreated acromegaly, 138 to 503 ng/ml in 4 patients with recurrent acromegaly, and 27 to 88 ng/ml in 3 patients with surgically cured acromegaly. These data demonstrate that plasma specimens of patients with acromegaly can be collected on filter paper disks and mailed to a laboratory on a distant continent for measurement of IGF-I. Such use of laboratory resources in one continent to help another continent, "Laboratories Without Borders," is somewhat analogous to the program "Doctors Without Borders."

Acromegaly↗
PubMed1992

[Familial acromegaly. Apropos of a case. Review of the literature].

Two brothers and a sister with acromegaly--one of them with acromegalogigantism--are described. They all presented pituitary adenomas, and the diagnosis was made between the ages of 17 and 29. There was no feature of multiple endocrine neoplasia. The familial investigation consisted of detailed interrogation, basal hormonal evaluation and HLA determination (haplotypes A and B) in 14 members, including the patients themselves. Despite basal plasma GH levels ranging from 8.00 to 10.00 ng/ml in 3 other family members, the normality of both GH response to induced-hyperglycemia and CT-imaging of the pituitary gland excluded the diagnosis of acromegaly in these subjects. No correlation with the HLA haplotypes was observed. Differential diagnosis with familial multiple endocrine neoplasia (MEN type 1) is discussed. Previous reports of familial acromegaly are reviewed and analysed. It appears that familial acromegaly may be considered as a specific entity for two main reasons: 1) owing to the low incidence of acromegaly in the general population, familial cases are unlikely to be fortuitous. 2) most of the reported cases share some common clinical features, such as a male predominance, a high incidence of acromegalogigantism, and the presence of a pituitary macroadenoma. However, the primitive disorder and the genetic transmission of the disease are still unknown.

Acromegaly↗
PubMed1992

Bone mineral density of the axial skeleton in acromegaly.

Acromegaly is characterized by growth hormone (GH) hypersecretion and insulin-like growth factor-I (IGF-I) excess, both of which stimulate osteoblast proliferation. At diagnosis, GH excess has usually been present for years. Furthermore, impaired gonadotropin secretion with hypogonadism is frequent. To date, studies of changes in bone mineral density (BMD) in acromegaly have been limited and the available data inconsistent. To investigate the effects of GH excess on proximal femur and lumbar spine BMD, a case series of 25 patients with acromegaly (8 eugonadal, 17 hypogonadal) documented by high plasma GH and IGF-I concentrations was studied. BMD was measured using dual-photon absorptiometry, hormonal and biochemical measurements, which included GH, IGF-I, serum calcium, phosphate, alkaline phosphatase, 1,25 dihydroxy vitamin D and urinary calcium and hydroxyproline excretion. Seven patients were re-studied after IGF-I was suppressed for six months by the somatostatin analog 201-995 (five patients) or pituitary adenomectomy (two patients). BMD was normal in 22 patients and was decreased at one site each in one eugonadal and two hypogonadal patients. BMD was similar between the eugonadal and hypogonadal groups at all sites. Urinary hydroxyproline excretion was equally increased in both groups. There was no correlation between any of the hormonal or biochemical parameters and the age, sex, race and body mass index matched Z-scores of BMD at any site. Following normalization of IGF-I for 6 mo in seven patients, there was no significant change of BMD. We conclude that proximal femoral and lumbar spine BMD is normal in most patients with active acromegaly, including those who are hypogonad. Successful treatment of acromegaly does not result in major short-term changes in BMD.

Absorptiometry, Photon↗
PubMed2004

Cardiovascular complications in acromegaly.

Cardiovascular morbidity and mortality are increased in acromegaly. In fact, GH and IGF-I excess induces a specific cardiomyopathy. The early stage of acromegaly is characterized by the hyperkinetic syndrome (high heart rate and increased systolic output). Frequently, concentric biventricular hypertrophy and diastolic dysfunction occur in acromegaly, leading to an impaired systolic function ending in heart failure if the disease is untreated or unsuccessfully untreated. Besides, abnormalities of cardiac rhythm and of valves have been also described in acromegaly. The coexistence of other complications, such as arterial hypertension and diabetes, aggravates the acromegalic cardiomyopathy. The suppression of GH/IGF-I following an efficacious therapy could decrease left ventricular mass and improve cardiac function. In conclusion, a careful evaluation of cardiac function, morphology and activity seems to be mandatory in acromegaly.

Acromegaly↗