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Acromegaly is a disorder characterized by excessive growth hormone production that primarily affects children before their growth plates close, leading to excessive height.
Answer: False
Acromegaly is characterized by excessive growth hormone production in adults after epiphyseal plate closure. When excessive growth hormone is produced in children before growth plates close, the resulting condition is termed gigantism, not acromegaly.
Acromegaly falls under the medical specialty of cardiology due to its significant impact on heart health.
Answer: False
Acromegaly is primarily managed within the medical specialty of endocrinology, as it involves hormonal imbalance and glandular dysfunction, specifically of the pituitary gland.
Acromegaly is most commonly diagnosed in early adulthood, typically before the age of 25.
Answer: False
Acromegaly typically has an insidious onset and is most commonly diagnosed in middle age, often after years of symptom progression, rather than in early adulthood.
Acromegaly is a relatively common condition, affecting approximately 60 out of every 100,000 people.
Answer: False
Acromegaly is considered a rare endocrine disorder, with an estimated prevalence of approximately 6 cases per 100,000 individuals in the general population.
Nicolas Saucerotte was the first to describe acromegaly in medical literature in the 18th century.
Answer: True
The initial medical description of acromegaly is attributed to Nicolas Saucerotte, who documented the condition in 1772, marking its first appearance in medical literature.
The term 'acromegaly' originates from Latin words meaning 'large extremities'.
Answer: False
The etymology of 'acromegaly' derives from the Greek words 'akron' (extremity) and 'mega' (large), accurately describing the characteristic enlargement of the body's extremities.
Males are affected by acromegaly more frequently than females.
Answer: False
Epidemiological data indicate that acromegaly affects males and females with approximately equal frequency, showing no significant gender predilection.
Which statement accurately describes the primary characteristic of acromegaly?
Answer: Excessive growth of certain body parts due to growth hormone overproduction after growth plates close.
Acromegaly is defined by the excessive growth of certain body parts, resulting from growth hormone overproduction that commences after the closure of epiphyseal growth plates in adulthood.
Which medical specialty is primarily concerned with acromegaly?
Answer: Endocrinology
Acromegaly, being a disorder of hormonal imbalance and glandular function, falls primarily within the purview of endocrinology, the medical specialty dedicated to the study of hormones and endocrine glands.
At what age is acromegaly most commonly diagnosed?
Answer: Middle age
Due to its often gradual and subtle progression, acromegaly is most frequently diagnosed in individuals during their middle age, rather than in earlier life stages.
What is the approximate prevalence of acromegaly in the general population?
Answer: 6 out of every 100,000 people
Acromegaly is a relatively rare endocrine disorder, with an estimated prevalence in the general population of approximately 6 individuals per 100,000.
Who first described acromegaly in medical literature?
Answer: Nicolas Saucerotte
The earliest documented description of acromegaly in medical literature is credited to Nicolas Saucerotte, who published his observations in 1772.
What is the etymological origin of the term 'acromegaly'?
Answer: Greek words 'akron' (extremity) and 'mega' (large).
The term 'acromegaly' is derived from the Greek words 'akron,' signifying 'extremity,' and 'mega,' meaning 'large,' accurately reflecting the characteristic enlargement of the body's distal parts.
What is the gender distribution for acromegaly?
Answer: Males and females are affected with equal frequency.
Acromegaly demonstrates an approximately equal incidence across both male and female populations, indicating no significant gender-based predisposition to the disorder.
The vast majority of acromegaly cases (approximately 98%) are caused by a benign tumor of the pituitary gland.
Answer: True
The overwhelming majority of acromegaly cases, approximately 98%, are attributed to the hypersecretion of growth hormone by a benign tumor, or adenoma, of the pituitary gland.
Pituitary adenomas causing acromegaly always grow rapidly and invade nearby brain areas, making symptoms immediately noticeable.
Answer: False
The growth rate and invasiveness of pituitary adenomas in acromegaly are highly variable. Many grow slowly, leading to a gradual onset of symptoms that may go unnoticed for years, while others can be more aggressive.
Most pituitary tumors that cause acromegaly are genetically inherited, passed down from parents to children.
Answer: False
The majority of pituitary tumors responsible for acromegaly are not inherited but arise spontaneously from a somatic genetic alteration in a single pituitary cell during an individual's lifetime.
In a small number of individuals, acromegaly can be caused by tumors in organs such as the pancreas, lungs, or adrenal glands.
Answer: True
While pituitary adenomas are the predominant cause, a minority of acromegaly cases can be attributed to ectopic tumors in organs like the pancreas, lungs, or adrenal glands, which either produce growth hormone or growth hormone-releasing hormone.
If excess growth hormone is produced during childhood, before the growth plates have closed, the resulting condition is still called acromegaly.
Answer: False
Excessive growth hormone secretion occurring during childhood, prior to the fusion of epiphyseal growth plates, leads to the condition known as gigantism, characterized by proportional overgrowth and increased height, distinct from acromegaly which affects adults.
The GNAS gene mutation is associated with pituitary adenomas and diffuse somatomammotroph hyperplasia in acromegaly.
Answer: True
Somatic activating mutations in the GNAS gene are recognized as a genetic basis for the development of pituitary adenomas and diffuse somatomammotroph hyperplasia, which are key pathological features in a subset of acromegaly cases.
What is the primary cause of acromegaly in approximately 98% of cases?
Answer: Overproduction of growth hormone by a benign pituitary tumor
The predominant etiology of acromegaly, accounting for approximately 98% of cases, is the excessive secretion of growth hormone by a benign pituitary adenoma.
What is the typical genetic origin of most pituitary tumors that cause acromegaly?
Answer: They result from a genetic alteration acquired during life in a single pituitary cell.
The majority of pituitary tumors causing acromegaly originate from a somatic genetic alteration acquired during an individual's lifetime within a single pituitary cell, rather than being inherited germline mutations.
Besides pituitary adenomas, what other types of tumors can cause acromegaly in a small number of individuals?
Answer: Tumors in the pancreas, lungs, or adrenal glands.
In a minority of cases, acromegaly can be caused by ectopic tumors located in organs such as the pancreas, lungs, or adrenal glands, which either directly secrete growth hormone or growth hormone-releasing hormone.
What condition results if excess growth hormone is produced during childhood, before the growth plates have closed?
Answer: Gigantism
When excessive growth hormone is secreted during childhood, prior to epiphyseal plate fusion, the resulting condition of generalized overgrowth and increased stature is known as gigantism.
Which gene mutation is associated with pituitary adenomas and diffuse somatomammotroph hyperplasia in acromegaly?
Answer: GNAS gene
Somatic activating mutations in the GNAS gene are implicated in the pathogenesis of pituitary adenomas and diffuse somatomammotroph hyperplasia, which are underlying causes of acromegaly.
The initial symptom of acromegaly is typically the enlargement of the hands and feet.
Answer: True
The initial clinical manifestation of acromegaly is typically the progressive enlargement of the hands and feet, often noticed through changes in ring or shoe size.
Type 2 diabetes, sleep apnea, and an increased risk of cancerous tumors are all potential complications associated with acromegaly.
Answer: True
Acromegaly is associated with a range of systemic complications, including metabolic disorders like type 2 diabetes, respiratory issues such as sleep apnea, and a slightly elevated risk of certain cancerous tumors.
Without treatment, the life expectancy of individuals with acromegaly is typically reduced by 10 years.
Answer: True
Untreated acromegaly is associated with a significant reduction in life expectancy, typically by approximately 10 years, primarily due to cardiovascular, respiratory, and metabolic complications.
The life expectancy of individuals with acromegaly is directly related to how early the disease is detected and successfully treated.
Answer: True
The prognosis and life expectancy for individuals with acromegaly are significantly influenced by the timeliness of diagnosis and the efficacy of subsequent treatment, with early and successful intervention capable of normalizing life expectancy.
Sleep apnea, a common complication of acromegaly, typically resolves after successful treatment of growth hormone levels.
Answer: False
While many symptoms and complications of acromegaly improve with successful growth hormone normalization, sleep apnea, a highly prevalent comorbidity, often persists even after effective treatment of the underlying hormonal imbalance.
Acromegaly is associated with a significantly elevated risk of various types of cancer.
Answer: False
While acromegaly is associated with an increased risk of certain types of cancer, this risk is generally considered to be slightly elevated rather than significantly elevated across all cancer types.
Hypogonadism associated with acromegaly is always irreversible, even with successful treatment of the underlying condition.
Answer: False
Hypogonadism occurring in the context of acromegaly is often reversible with successful treatment of the underlying growth hormone excess, provided that the gonads themselves have not sustained irreversible damage.
Which of the following is typically the initial symptom of acromegaly?
Answer: Enlargement of the hands and feet
The insidious onset of acromegaly often manifests initially as a noticeable enlargement of the hands and feet, which may be observed through changes in glove, ring, or shoe sizes.
All of the following are potential complications of acromegaly EXCEPT:
Answer: Significantly reduced risk of cancerous tumors
Acromegaly is associated with an *increased* or *slightly elevated* risk of cancerous tumors, not a significantly reduced risk. Other listed options are indeed known complications.
How do pituitary adenomas contribute to symptoms like headaches and visual disturbances in acromegaly?
Answer: By compressing surrounding brain tissues as they enlarge.
As pituitary adenomas enlarge, they can exert mass effect by compressing adjacent brain structures, including the optic chiasm, leading to symptoms such as headaches and visual field defects.
How does successful treatment impact the life expectancy of individuals with acromegaly?
Answer: It can normalize life expectancy to that of the general population.
Effective and timely treatment of acromegaly can significantly improve patient outcomes, potentially normalizing life expectancy to that of the general population, thereby mitigating the disease's impact on longevity.
Which of the following symptoms of acromegaly does NOT tend to resolve even with successful treatment of growth hormone levels?
Answer: Sleep apnea
Among the various complications of acromegaly, sleep apnea is notable for its tendency to persist even after successful biochemical control of growth hormone hypersecretion, often requiring continued management.
What is the prognosis for hypogonadism in acromegaly if it is not caused by gonad destruction?
Answer: It is reversible with successful treatment of the underlying acromegaly.
Hypogonadism associated with acromegaly, when not attributable to irreversible gonadal damage, typically resolves or significantly improves following successful treatment and normalization of growth hormone levels.
Acromegaly is diagnosed by measuring growth hormone levels after a person consumes a glucose solution or by measuring insulin-like growth factor I (IGF-I) in the blood.
Answer: True
The diagnosis of acromegaly relies on biochemical confirmation, typically involving the measurement of growth hormone levels following an oral glucose tolerance test or the assessment of elevated insulin-like growth factor I (IGF-I) concentrations in the blood.
Pseudoacromegaly is a condition that presents with similar features to acromegaly but is characterized by significantly elevated levels of growth hormone (GH) and insulin-like growth factor I (IGF-I).
Answer: False
Pseudoacromegaly is a clinical entity that mimics the physical features of acromegaly but is biochemically distinguished by the absence of elevated growth hormone (GH) and insulin-like growth factor I (IGF-I) levels.
Which of the following is a key diagnostic method for acromegaly?
Answer: Measuring growth hormone levels after consuming a glucose solution.
A definitive diagnostic approach for acromegaly involves measuring growth hormone levels after an oral glucose load, as glucose typically suppresses GH secretion in healthy individuals but not in those with acromegaly.
What is the defining characteristic that differentiates pseudoacromegaly from true acromegaly?
Answer: Pseudoacromegaly presents with similar features but without elevated GH and IGF-I levels.
Pseudoacromegaly is distinguished from true acromegaly by the absence of elevated serum growth hormone (GH) and insulin-like growth factor I (IGF-I) levels, despite presenting with similar acromegaloid physical features.
Surgery to remove the pituitary tumor is usually the preferred initial treatment for acromegaly, with success rates higher for larger tumors.
Answer: False
Surgical resection of the pituitary adenoma is generally considered the primary initial treatment for acromegaly, with optimal success rates observed in cases involving smaller tumors and performed by experienced neurosurgeons.
The older transsphenoidal surgery method is generally preferred over endonasal transsphenoidal surgery because it is less invasive and offers a shorter recovery time.
Answer: False
Endonasal transsphenoidal surgery is generally favored over the older transsphenoidal approach due to its minimally invasive nature, reduced recovery period, and often superior outcomes in terms of tumor removal and complication rates.
A potential complication of surgery for acromegaly is damage to the surrounding normal pituitary tissue, which might require lifelong hormone replacement therapy.
Answer: True
One significant potential complication of pituitary surgery for acromegaly is iatrogenic damage to the adjacent normal pituitary tissue, which can result in hypopituitarism and necessitate lifelong hormone replacement therapy.
Radiation therapy for acromegaly typically lowers growth hormone levels immediately and completely normalizes them within a few weeks.
Answer: False
Radiation therapy for acromegaly is characterized by a gradual onset of effect, typically lowering growth hormone levels by approximately 50% over a period of 2 to 5 years, with full normalization often taking even longer, rather than immediate resolution.
What is generally considered the preferred initial treatment for acromegaly?
Answer: Surgical removal of the pituitary tumor
Surgical resection of the pituitary adenoma via a transsphenoidal approach is widely regarded as the preferred initial therapeutic modality for most patients with acromegaly, aiming for complete tumor removal and biochemical remission.
Which surgical procedure is generally preferred for removing pituitary tumors in acromegaly due to its less invasive nature and shorter recovery time?
Answer: Endonasal transsphenoidal surgery
Endonasal transsphenoidal surgery is the preferred surgical approach for pituitary adenoma removal in acromegaly, offering advantages such as minimal invasiveness, reduced postoperative discomfort, and a more rapid recovery compared to traditional methods.
What is a potential complication of surgery for acromegaly that might necessitate lifelong hormone replacement therapy?
Answer: Damage to the surrounding normal pituitary tissue
A significant surgical complication in acromegaly treatment is iatrogenic damage to the surrounding healthy pituitary tissue, which can lead to panhypopituitarism and require lifelong exogenous hormone replacement.
What happens if hormone levels do not fully normalize after successful surgery for acromegaly?
Answer: Patients usually require additional treatment, often with medications.
Even following successful surgical debulking of a pituitary adenoma, if growth hormone and IGF-I levels do not achieve complete normalization, patients typically require subsequent medical therapy and diligent long-term monitoring to manage residual disease activity.
When is radiation therapy typically used in the treatment of acromegaly?
Answer: For individuals who still have tumor remnants after surgery.
Radiation therapy is generally reserved as a secondary treatment modality for acromegaly, primarily indicated for patients with residual tumor tissue after surgical resection or for those who are not candidates for or do not respond to medical therapy.
Somatostatin analogues treat acromegaly by stimulating the pituitary gland to produce less growth hormone.
Answer: False
Somatostatin analogues, synthetic forms of the natural hormone somatostatin, exert their therapeutic effect in acromegaly by directly inhibiting the pituitary gland's production and secretion of growth hormone.
Long-term use of somatostatin analogues can cause digestive problems and, in about 25% of patients, may lead to the development of gallstones.
Answer: True
Common adverse effects associated with the long-term administration of somatostatin analogues include gastrointestinal disturbances such as loose stools, nausea, and gas, and a notable incidence of gallstone formation in approximately 25% of treated patients.
Dopamine agonists are primarily used as a first-line monotherapy for all acromegaly patients due to their high efficacy and minimal side effects.
Answer: False
Dopamine agonists are typically employed as an adjunctive or second-line therapy in acromegaly, particularly for patients who do not respond adequately to somatostatin analogues or when co-secretion of prolactin is present, rather than as a universal first-line monotherapy.
Growth hormone receptor antagonists like pegvisomant work by blocking the action of growth hormone molecules at the receptor level.
Answer: True
Growth hormone receptor antagonists, exemplified by pegvisomant, exert their therapeutic effect by competitively binding to growth hormone receptors in peripheral tissues, thereby preventing endogenous growth hormone from initiating its biological actions.
How do somatostatin analogues like octreotide treat acromegaly?
Answer: By inhibiting the production of growth hormone.
Somatostatin analogues, such as octreotide, function by binding to somatostatin receptors on pituitary adenoma cells, thereby inhibiting the synthesis and release of growth hormone.
Which of the following is a common side effect of long-term somatostatin analogue treatment for acromegaly?
Answer: Development of gallstones in approximately 25% of patients
A frequently observed adverse effect of chronic somatostatin analogue therapy for acromegaly is the formation of gallstones, which occurs in approximately 25% of patients, often due to altered gallbladder motility and bile composition.
When might pasireotide, a second-generation somatostatin analogue, be used in acromegaly treatment?
Answer: For tumor control in aggressive adenomas resistant to octreotide.
Pasireotide, a more potent somatostatin analogue, is typically considered for the management of aggressive pituitary adenomas in acromegaly that have demonstrated resistance or inadequate response to conventional somatostatin analogue therapies like octreotide.
Dopamine agonists are particularly effective in acromegaly when pituitary tumors also secrete which other hormone?
Answer: Prolactin
Dopamine agonists exhibit enhanced efficacy in acromegaly patients whose pituitary adenomas co-secrete prolactin, as these medications are potent inhibitors of prolactin secretion and can also reduce growth hormone levels in such cases.
How do growth hormone receptor antagonists, such as pegvisomant, primarily work to treat acromegaly?
Answer: They block the action of growth hormone molecules at the receptor level.
Growth hormone receptor antagonists, exemplified by pegvisomant, function by competitively binding to growth hormone receptors on target cells, thereby preventing the binding of endogenous growth hormone and inhibiting its downstream signaling pathways.