Brainscape's Knowledge GenomeTM

Browse over 1 million classes created by top students, professors, publishers, and experts.


See full index

endo > Acromegaly > Flashcards

Acromegaly Flashcards

1
Q

What is Acromegaly characterized by?

A

Answer: Excessive growth hormone (GH) production and elevated insulin-like growth factor 1 (IGF-1) levels.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
2
Q

What distinguishes Acromegaly from Gigantism?

A

Answer: Acromegaly occurs in adults after the closure of growth plates, whereas Gigantism results from GH excess before the closure of growth plates and has its onset before puberty.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
3
Q

What is the primary cause of GH excess in Acromegaly cases (>95%)?

A

Answer: A GH-producing pituitary adenoma (somatotropinoma or GH and prolactin co-secreting mammosomatotropinoma).

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
4
Q

In very rare cases, what can cause Acromegaly apart from pituitary adenomas?

A

Answer: Ectopic (paraneoplastic) secretion of GH or growth hormone-releasing hormone (GHRH) responsible for pituitary hyperplasia.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
5
Q

What is the most common familial cause of Acromegaly?

A

Answer: Familial isolated pituitary adenoma (FIPA) syndrome.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
6
Q

What is the role of the aryl hydrocarbon receptor-interacting protein (AIP) gene in FIPA cases?

A

Approximately one-fifth of FIPA families have germline mutations in the AIP gene.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
7
Q

How do pituitary tumors in FIPA differ from sporadic cases in terms of disease onset and tumor characteristics?

A

Answer: Pituitary tumors in FIPA have an earlier disease onset and are diagnosed at a younger age than their sporadic counterparts, and these tumors are usually large and invasive.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
8
Q

What are the main symptoms of Acromegaly due to elevated GH and IGF-1 levels?

A

Answer: Progressive somatic disfigurement, mainly involving the face and extremities.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
9
Q

What complications are associated with Acromegaly due to chronic GH and IGF-1 elevation?

A

Answer: Cardiovascular, metabolic, respiratory, neoplastic, endocrine, articular, and bone complications.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
10
Q

How does effective treatment of Acromegaly impact the condition and quality of life?

A

Answer: Effective control of GH and IGF-1 hypersecretion, as well as the ablation or stabilization of the pituitary tumor mass, leads to improved comorbidities and enhances life expectancy and quality of life.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
11
Q

What are some local tumor effects associated with acromegaly?

A

Answer: Headache, impaired vision, cranial nerve palsy, and hypopituitarism are local tumor effects seen in acromegaly.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
12
Q

How might acromegaly manifest in terms of cranial nerve function?

A

Answer: Acromegaly can lead to cranial nerve palsy.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
13
Q

What is hypopituitarism, and how is it related to acromegaly?

A

Answer: Hypopituitarism refers to reduced pituitary gland function and can occur as a result of a pituitary tumor in acromegaly.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
14
Q

What are the musculoskeletal and soft tissue effects of acromegaly?

A

Answer: Acromegaly is associated with enlarged hands and feet, enlarged tongue and organs, oily and thickened skin, excessive sweating and body odor, skin tags, fatigue, muscle weakness, joint pain, limited mobility, and facial changes like prognathism.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
15
Q

How does acromegaly impact the size of the hands and feet?

A

Answer: Acromegaly causes the enlargement of hands and feet.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
16
Q

What cardiovascular effects can occur in individuals with acromegaly?

A

Answer: Cardiovascular effects include left ventricular hypertrophy, arrhythmias, hypertension, cardiomyopathy, and congestive heart failure.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
17
Q

What pulmonary effects are observed in individuals with acromegaly?

A

Answer: Acromegaly can lead to sleep apnea and severe snoring due to the obstruction of the upper airway.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
18
Q

What voice-related changes can occur in individuals with acromegaly?

A

Answer: Acromegaly can cause a deepened voice due to enlarged vocal cords and sinuses.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
19
Q

What neoplastic conditions are associated with acromegaly?

A

Answer: Acromegaly is associated with colon polyps and carcinoma.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
20
Q

What endocrine and metabolic effects can occur in individuals with acromegaly?

A

Answer: Acromegaly can lead to menstrual cycle irregularities in women, erectile dysfunction in men, insulin resistance, hyperinsulinemia, impaired glucose tolerance, diabetes mellitus, hypertriglyceridemia, and hypercalciuria.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
21
Q

What is goiter, and how is it related to acromegaly?

A

Answer: Goiter is an enlarged thyroid gland, and it can be associated with acromegaly.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
22
Q

What is macroglossia?

A

Answer: Enlarged tongue.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
23
Q

What is the main goal of the diagnostic steps in Acromegaly?

A

Answer: The main goal of the diagnostic steps is to verify the GH and IGF-1 overproduction and to find the source of the GH excess.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
24
Q

Why random GH measurements is not the gold standard for the diagnosis of Acromegaly?

A

Answer: random GH measurement is not the gold standard for the diagnosis of acromegaly, as serum levels may
fluctuate from undetectable to high levels because of its episodic secretion.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
25
Q

What happens to GH levels in healthy subjects after glucose administration?

A

Answer: In healthy subjects, GH levels fall after glucose administration.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
26
Q

How do GH levels in acromegalic patients differ from those in healthy individuals during an OGTT?

A

Answer: In acromegalic patients, the suppressive effect of glucose on GH levels is missing.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
27
Q

What is the most common cause of acromegaly?

A

Answer: The majority of patients with acromegaly have a GH-secreting pituitary adenoma.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
28
Q

Why is magnetic resonance imaging (MRI) performed in patients with acromegaly?

A

Answer: MRI is performed to find a pituitary adenoma and visualize its extension.

29
Q

In what very rare cases might acromegaly be caused by conditions other than pituitary adenomas?

A

Answer: In very rare cases, acromegaly can be caused by GH-secreting pituitary carcinoma, hypothalamic GHRH-secreting tumors, or ectopic GH/GHRH secretion.

30
Q

Which hormone test can be used for screening for acromegaly?

A

Answer: IGF-1 should be first measured in patients who are suspected to suffer from acromegaly. If IGF-1 is normal, the chance for acromegaly is very low.

31
Q

Why is the evaluation of residual pituitary function essential in patients with pituitary tumors?

A

Answer: Pituitary tumors might cause hypopituitarism by compressing the surrounding normal pituitary tissue, so evaluating pituitary function is crucial.

32
Q

Which hormone levels should be measured to assess pituitary function in patients with pituitary tumors?

A

Answer: The following hormone levels should be measured: basal prolactin (PRL), FSH, LH, TSH, fT4, fT3, estradiol (in women), testosterone (in men), and cortisol levels.

33
Q

Why might prolactin levels be elevated in patients with pituitary tumors?

A

Answer: Prolactin levels might be elevated due to pituitary stalk compression by the tumor, preventing the inhibitory effect of hypothalamic dopamine, or due to co-secretion of GH and PRL by the pituitary adenoma.

34
Q

What are the primary causes of enhanced mortality in individuals with acromegaly?

A

Answer: Enhanced mortality in acromegaly is mainly due to cardiovascular disease, respiratory disorders, and dysregulated glucose metabolism.

35
Q

What should active acromegaly patients be screened for in terms of cardiovascular complications?

A

Answer: Active acromegalic patients should be screened for cardiovascular complications, including hypertension, arrhythmia, and cardiomyopathy.

36
Q

What are the endocrine and metabolic disorders that active acromegaly patients should be screened for?

A

Answer: Active acromegaly patients should be screened for endocrine and metabolic disorders, such as hypopituitarism and glucose metabolism impairment.

37
Q

What is one of the respiratory complications that active acromegaly patients should be screened for?

A

Answer: Active acromegalic patients should be screened for sleep apnea.

38
Q

Which musculoskeletal disorder should be included in the screening for active acromegaly patients?

A

Answer: Musculoskeletal disorders, including vertebral fractures, should be screened for.

39
Q

What neoplastic condition should active acromegaly patients be screened for?

A

Answer: Active acromegaly patients should be screened for colon polyps and cancer.

40
Q

Does acromegaly increase the risk of certain types of tumors?

A

Colon and thyroid cancer

41
Q

What screening procedure is recommended for colon cancer in individuals with acromegaly?

A

Answer: Screening colonoscopy should be performed at diagnosis and then repeated as needed based on the result, family history, and disease activity.

42
Q

When is thyroid ultrasound suggested for patients with acromegaly?

A

Answer: Thyroid ultrasound is suggested for patients with a palpable nodule or an increased risk for thyroid cancer.

43
Q

What is the leading cause of death in acromegaly patients?

A

Answer: Cardiovascular disease is the leading cause of death in acromegaly patients.

44
Q

What are the two other significant causes of death in individuals with acromegaly?

A

Answer: The other significant causes of death in acromegaly patients are respiratory and cerebrovascular disease.

45
Q

What are the main goals of treatment in acromegaly?

A

Answer: The main goals of treatment in acromegaly are to achieve biochemical control, which includes age- and gender-matched normalized IGF-1 levels and controlled GH levels, and to reduce tumor mass.

46
Q

What is the first-choice treatment for acromegaly, provided there are no contraindications?

A

Answer: The first-choice treatment for acromegaly is surgical treatment, except in cases with contraindications or when total tumor resection is not possible or the patient refuses surgery.

47
Q

In what situations might surgical treatment for acromegaly be contraindicated?

A

Answer: Surgical treatment may be contraindicated in cases of severe heart failure, respiratory disease, tumors that cannot be completely removed without affecting vital structures, or when the patient refuses surgery.

48
Q

What is the primary surgical approach used in pituitary surgery for acromegaly, and when is transcranial resection necessary?

A

Answer: The primary approach is the transphenoidal approach. However, transcranial resection is necessary in some cases.

49
Q

What changes can be observed right after a successful surgical intervention for acromegaly?

A

Answer: After a successful surgical intervention, GH levels fall, and improvements in metabolic dysfunction and soft tissue swelling begin to occur.

50
Q

When should clinical and biochemical evaluation be performed following acromegaly surgery, and what parameters should be measured?

A

Answer: Clinical and biochemical evaluation should be performed 12 weeks after surgery. Parameters measured include IGF-1, random GH, and GH levels during OGTT (Oral Glucose Tolerance Test).

51
Q

What are the main potential side effects or complications associated with surgery for acromegaly?

A

Answer: The main potential side effects or complications include newly developed hypopituitarism, permanent diabetes insipidus, hemorrhage, meningitis, and cerebrospinal fluid leak.

52
Q

What is the next therapeutic option if acromegaly persists after surgery?

A

Answer: The next therapeutic option is medical therapy, which includes dopamine agonists, somatostatin analogues (SSAs), and GH receptor antagonists or combination therapy.

53
Q

What is the first-line medical therapy for acromegaly, and which types of medications are used?

A

Answer: The first-line medical therapy for acromegaly includes dopamine agonists and first-generation SSAs. Medications used include cabergoline (dopamine agonist), octreotide, and lanreotide (first-generation SSAs).

54
Q

Which dopamine agonist is considered the most effective with the best side effect profile in treating acromegaly?

A

Answer: Cabergoline is considered the most effective dopamine agonist with the best side effect profile in treating acromegaly.

55
Q

What determines the efficacy of first-generation SSA treatment in acromegaly patients?

A

Answer: The efficacy of first-generation SSA treatment is determined by factors such as pretreatment GH levels, tumor expression of somatostatin receptor 2 (SSTR2), presence of mutations in the AIP gene, tumor granularity, and intensity on MRI.

56
Q

In what situations can SSA therapy be applied in acromegaly treatment, and what are some common side effects?

A

Answer: SSA therapy can be applied after surgery to achieve biochemical control, preoperatively to reduce complications of surgery, or after radiotherapy until its effect starts to develop. Common side effects include mainly gastrointestinal side effects and gallbladder sludge or stones.

57
Q

How should biochemical control be evaluated during first-line medical therapy for acromegaly?

A

Answer: Biochemical control during first-line medical therapy is evaluated by measuring IGF-1 levels and the average of multiple GH levels.

58
Q

What are the second-line medical therapy options for acromegaly if surgery and first-line medical therapy are not effective?

A

Answer: Second-line medical therapy options include the second-generation SSA pasireotide and the GH receptor antagonist pegvisomant.

59
Q

What is the main side effect of pasireotide, and why should patients on pasireotide LAR therapy be carefully monitored?

A

Answer: The main side effect of pasireotide is hyperglycemia. Patients on pasireotide LAR therapy should be carefully monitored for impaired glucose tolerance or diabetes mellitus.

60
Q

What is the role of radiotherapy in acromegaly treatment, and how long does it take for its effects to become maximal?

A

Answer: Radiotherapy is usually a third-line therapy for acromegaly when surgery and medical therapy are not sufficient. The maximal effect of radiotherapy starts to develop after approximately 5-10 years.

61
Q

What are some potential side effects of radiotherapy in acromegaly treatment?

A

Answer: Potential side effects of radiotherapy include hypopituitarism, second brain tumor, visual deterioration, and cranial nerve palsies.

62
Q

How is disease control evaluated during second-line medical therapy with pasireotide and GH receptor antagonist pegvisomant?

A

Answer: Disease control during second-line medical therapy with pasireotide is evaluated similarly to first-generation SSAs. For pegvisomant, GH levels are not informative, and only IGF-1 levels are measured.

63
Q

Can acromegaly be inherited, and in what rare cases does it occur as part of endocrine tumor syndromes?

A

Answer: Very rarely, acromegaly can be inherited as part of endocrine tumor syndromes such as multiple endocrine neoplasia types 1 and 4 (MEN1, MEN4), Carney complex, or familial isolated pituitary adenoma (FIPA) and SDH-related syndromes

64
Q

What is the role of pegvisomant in acromegaly treatment, and how does it work?

A

Answer: Pegvisomant is a GH receptor antagonist used in acromegaly treatment. It blocks postreceptor GH signaling and inhibits IGF-1 production, effectively reducing GH action.

65
Q

What should be monitored during pegvisomant therapy, and what are the main side effects associated with this medication?

A

Answer: During pegvisomant therapy, liver enzymes should be screened as elevated hepatic transaminases are one of the main side effects. Another important side effect is lipodystrophy on the local injection site.

66
Q

In what situations is pegvisomant the preferred choice for second-line medical therapy in acromegaly treatment?

A

Answer: Pegvisomant is preferred when patients have impaired glucose metabolism, as it enhances insulin sensitivity. It is also chosen when there is clinically relevant residual tumor and/or clinical concern of tumor growth.

67
Q

What is the mechanism of action of pegvisomant in acromegaly treatment?

A

Answer: Pegvisomant works as a GH receptor antagonist by blocking postreceptor GH signaling and inhibiting IGF-1 production.

68
Q

What is the effectiveness of pegvisomant in normalizing IGF-1 levels in acromegaly patients?

A

Answer: Pegvisomant can normalize IGF-1 levels in approximately 60-90% of acromegaly patients.

endo (2 decks)

Brainscape helps you reach your goals faster, through stronger study habits.
© 2024 Bold Learning Solutions. Terms and Conditions