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Clinical and pathological findings in 100 cases of acromegaly were reviewed. Great individual variation in manifestations of the disease was apparent. The chronic course was evidenced by 30 patients presenting symptoms unrelated to acromegaly. Headache, visual disturbances, arthritis and elevated blood pressure were common. The incidence of diabetes mellitus, thyroid and adrenal dysfunction was similar to that reported in other series. Gonadal disturbances were frequent. Two patients had galactorrhea and two had hyperparathyroidism. Radiologically, pituitary fossae were normal in 24. Pituitary histology in 29 cases revealed four eosinophilic adenomas, but most were mixed eosinophilic-chromophobic. Fifty-two patients received radiation therapy. Rhinorrhea and optic nerve fibrosis were seen in this group. Twenty-four patients were operated upon, with no operative deaths since 1945 and no morbidity since 1952. Visual failure was the clearest indication for surgery. Treatment will be difficult to evaluate until an index of growth hormone secretion is generally available.
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PMID:Acromegaly: a review of 100 cases. 1394 86

Octreotide long-acting release (LAR) is a somatostatin analogue designed for once monthly intramuscular injection. As with endogenous somatostatin, octreotide LAR inhibits secretion of growth hormone (GH) as well as various other peptide hormones. In the treatment of acromegaly, octreotide LAR effectively controlled the secretion of GH and insulin-like growth factor-1 (IGF-1) in about 55-70% of patients (n > 100) who had previously been treated with somatostatin analogues, a similar degree of control to that observed with subcutaneous octreotide and lanreotide slow release (SR). Progressive control of serum levels of GH and IGF-1 was achieved with octreotide LAR in clinical studies of up to 4 years' duration. In addition, primary therapy with octreotide LAR provided effective control of GH and IGF-1 secretion, particularly in patients with a pretreatment GH level <20 microg/L. The percentage of patients achieving a target serum GH level of <2-2.5 micro g/L or normal IGF-1 levels was significantly greater with octreotide LAR 10, 20 or 30 mg every 28 days than with lanreotide SR 30 mg every 7-14 days in a large (n = 125) sequential, 6-month study, but was not significantly different between treatment groups in a small, randomised, nonblind, parallel group study of previously untreated patients. The volume of pituitary tumour shrinkage achieved with octreotide LAR or lanreotide SR was also similar ( approximate, equals 33% after 24 months). Acromegaly symptoms, such as headache, increased perspiration, paraesthesia, fatigue and osteoarthralgia were improved during treatment with octreotide LAR or lanreotide SR. Overall, octreotide LAR is generally well tolerated by most patients. The incidence of gastrointestinal symptoms is about 30% but, in most cases, events are transient and mild to moderate. Gallbladder abnormalities (sediment, sludge, microlithiasis and gallstones) can occur, but only 1% have become symptomatic to date. The prevalence of biliary abnormalities did not change after switching from subcutaneous octreotide, or from lanreotide SR, to octreotide LAR. Glucose metabolism can be affected by octreotide LAR in some patients; about 15% become hyperglycaemic, usually mild in severity. In summary, octreotide LAR controls GH and IGF-1 secretion in about 55-70% of patients with acromegaly. Octreotide LAR is administered intramuscularly every 28 days, offering improved patient compliance and convenience over three-times-daily subcutaneous octreotide. Long-term therapy provides progressive control of serum GH and IGF-1 levels, and is generally well tolerated by most patients. Thus, for the medical management of acromegaly, octreotide LAR is an effective, well tolerated and convenient treatment option.
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PMID:Octreotide long-acting release (LAR): a review of its use in the management of acromegaly. 1460 59

Octreotide is a somatostatin analog that inhibits growth hormone release showing higher potency than natural somatostatin so it has proved to be effective in acromegaly treatment. The objective of present study was to establish the effects of octreotide LAR (long acting release) preparation in patients with active acromegaly. The following parameters were assessed: clinical response, safety of medication, GH and IGF-1 serum concentrations and pituitary tumor size. Eleven patients (6 men and 5 women) range 41.4 years old with diagnosis of active acromegaly were included. Octreotide was administered at 0.1 mg subcutaneusly dose three times daily for four weeks to test the drug tolerability. Afterwards patients received octreotide LAR 20 mg intramuscularly separated by 28 days periods with an option to continue for 8 months. Basal average GH serum concentrations was 27.6 ng/mL. After 6 months treatment reduction to 5.03 +/- 5.38 ng/mL in 9 patients (p < 0.001) was observed. Basal IGF-1 average serum concentration was 889.55 +/- 167.29 ng/mL with a reduction value to 483.00 +/- 239.71 ng/mL in 9 of 11 patients after 6 months treatment (p < 0.005). The drug was well tolerated with few adverse effects Diarrhea, flatulence and steatorrhea were observed during the administration of subcutaneous octreotide in 18.2% of patients. Two patients had symptomatic biliary lithiasis that was successfully removed by surgery. Clinical symptoms improved and some of them dissapeared such as headaches and sweatings. Tumor shrinkage was observed in 66.7% of cases. Monthly injections of 20 mg of octreotide LAR were effective to reduce GH and IGF-1 levels in patients with active acromegaly accompanied by improvement of clinical symptoms and significant tumor size reduction.
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PMID:Improvement of acromegaly after octreotide LAR treatment. 1467 21

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.
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PMID:[Spontaneous remission of acromegaly after meningitis: a case report]. 1472 37

Normal ageing is associated with a decline in spontaneous growth hormone (GH) secretion, and although elderly hypopituitary adults demonstrate an increase in total and central fat compared with age-matched controls and are distinguishable from control subjects in terms of GH responsiveness on dynamic testing, there are few data available on the response to GH replacement in older subjects. We have studied the baseline characteristics of 295 patients (173 males and 122 females) aged >65 years of age who began GH replacement therapy at the time of entry into the KIMS program (Pfizer International Metabolic Database) and the effects of GH replacement in 125 patients who completed at least 12 months of GH replacement therapy. Data were compared with those of 2469 (1249 males and 1220 females) patients aged <65 years with adult-onset GH deficiency (GHD). The patients were selected using strict criteria in accordance with the recommendations from the Growth Hormone Research Society. There was a higher proportion of pituitary adenoma relative to craniopharyngioma in the older age group (P<0.001), but there was no difference between groups in the degree of hypopituitarism (number of additional hormone deficiencies). Blood pressure, cholesterol and low-density lipoprotein (LDL) cholesterol levels were positively correlated with age, and older patients had a predictably higher prevalence of diabetes mellitus, coronary heart disease, stroke and history of hypertension. Quality of life (Assessment of Growth Hormone Deficiency in Adults (AGHDA) score) was impaired in both groups before the start of GH therapy. GH replacement doses were lower in older patients with GHD as compared with patients <65 years old. After 12 months of GH replacement, significant improvements were evident in waist circumference, waist/hip ratio, lean body mass, diastolic blood pressure, total and LDL cholesterol levels and AGHDA scores in patients aged <65 years. Similar significant reductions were evidenced in patients >65 years old compared with those observed in younger patients. The total number of adverse events was similar in younger and older patients with GHD. However, younger patients had more fluid retention-related adverse events such as headache, oedema and arthralgia; whereas, older patients with GHD had more adverse events related to glucose metabolism, cardiovascular events and neoplasms. These data indicate a positive benefit from GH replacement in older patients with hypopituitarism - particularly in relation to quality of life - using a lower dose of GH for replacement and with appropriate age-related safety controls.
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PMID:Aspects of growth hormone deficiency and replacement in elderly hypopituitary adults. 1513 78

Rathke's cleft cysts arise from remnants of Rathke's pouch and are usually found incidentally on MRI or autopsy. In childhood, the most common presenting symptoms of Rathke's cleft cysts are endocrine abnormalities, such as reduced growth hormone secretion, hyperprolactinemia, or diabetes insipidus. Non-specific symptoms, such as headache and visual disturbance, may also occur. Although precocious puberty has occasionally been described in association with suprasellar lesions, such as hamartomas, arachnoid cysts, and craniopharyngiomas, to our knowledge there have been no documented cases secondary to Rathke's cleft cysts. We report here two patients, both of whom presented with precocious puberty, and were found to have Rathke's cleft cysts.
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PMID:Rathke's cleft cyst in two girls with precocious puberty. 1523 14

About seven to ten percent of all brain tumours are neoplasias of the pituitary gland. Pituitary gland tumours can cause different clinical symptoms often making it difficult to come to the correct diagnosis. They can lead to severe complications such as hypopituitarism with secondary hypogonadism, hypothyroidism, and adrenocortical insufficiency, compression of the optic tract or obstructive hydrocephalus. We report on two patients with hormone-secreting pituitary tumours that were unknown prior to pregnancy. The first woman suffered from a growth hormone-producing pituitary adenoma, causing persistent headaches after birth. The second woman showed a significant loss of vision and visual field defects in the 32nd week of gestation, caused by a prolactin-producing pituitary tumour.
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PMID:[Primary diagnosis of hormone-secreting pituitary adenoma during pregnancy and after birth -- a rare occurrence]. 1532 57

Idiopathic intracranial hypertension, characterized by headache, visual disturbance, papilledema, and increased intracranial pressure in the absence of space-occupying lesions, has been reported in pediatric recipients several months to years following kidney transplantation (KTx). We describe the development of idiopathic intracranial hypertension in a 10-year-old girl in the first few hours after KTx. We hypothesize that this event was associated with thymoglobulin administration, perhaps on the background of growth hormone therapy. Awareness of this possibility could lead to earlier diagnosis and treatment of children with similar risk factors.
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PMID:Idiopathic intracranial hypertension immediately after kidney transplantation in a pediatric recipient. 1537 20

We report the case of 19-year-old man with pituitary gigantism due to growth hormone-producing pituitary macroadenoma. The patient complained of recurrent headache and excessive growth spurt since age 15. Octreotide administration was initiated following transsphenoidal pituitary adenomectomy. Octreotide injection for 4 years efficaciously reduced the size of remnant adenoma as well as serum growth hormone levels. Notably, octreotide exhibited a potent analgesic effect on his intractable cluster headache that has continued even after reduction of the adenoma volume. The analgesic effect lasted 2 to 6 hours after each injection and no tachyphylaxis to octreotide appeared during 4-year treatment. To characterize the headache and the pain intensity, analgesic drugs including octreotide, lidocaine, morphine and thiopental were tested using a visual analogue scale (VAS) evaluation, with the result that octreotide exhibited a prompt and complete disappearance of the headache. Headache relief was in part reproduced by morphine injection (56% reduction) but not by lidocaine or thiopental. The present case suggests that the intractable headache associated with pituitary gigantism is possibly related to the endogenous opioid system. Thus, the headache control by octreotide is clinically helpful for continuation of the self-injection regimen.
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PMID:Long-term effects of octreotide on pituitary gigantism: its analgesic action on cluster headache. 1551 76

Acromegaly is a slowly developing disfiguring disease characterized by chronic growth hormone (GH) and insulin-like growth factor-I (IGF-I) excess and caused by a pituitary somatotroph adenoma. It is associated to 2- to 3 fold increased mortality, compared to normal population, mostly due to cardiovascular and cerebro-vascular diseases, and to several co-morbid systemic illnesses, such as diabetes mellitus, hypertension, severe arthropathies, a specific cardio-myopathy, goitre, sleep-apnoea, intractable headache. The morbidity and excess mortality of acromegaly are usually the consequence of the metabolic actions of excess GH and IGF-I secretion, while only in rare patients mortality is due to the mass effects of the pituitary tumour. Since, serum IGF-I concentrations within age-adjusted normal range, and a tight GH control have to be achieved to normalize life-expectancy in these patients, an aggressive, and often multi-modality treatment is required for acromegaly. In recent years, new drugs, and new formulations of old drugs, have been developed that are able to effectively inhibit GH secretion or GH action, and may represent important adjuncts or even alternatives to the traditional approaches of surgery and radiotherapy. This review briefly summarizes the therapeutic options nowadays available for acromegaly. A brief note about innovative drugs under study, is also given.
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PMID:New therapeutic options for acromegaly. 1576 31


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