Q1:
You are evaluating a 16-year-old boy whom you have known since early childhood. He is
complaining of headaches over the past 2 weeks. He has a history of asthma, which has been
well controlled, and he is an otherwise healthy member of the varsity football team at school. He
has had a significant weight gain of 30 lb (13.5 kg) since his visit to you 1 year ago. He denies
using illicit or prescription drugs. On physical examination, he appears very muscular and has a
blood pressure of 180/120 mm Hg. You repeat the measurement using a leg cuff to ensure
adequate cuff size and obtain the same result.
Of the following, the BEST management plan is
A. angiotensin-converting enzyme inhibition as an outpatient
B. beta blocker therapy as an outpatient
C. diuretic therapy as an inpatient
D. repeat blood pressure measurement in 1 to 2 weeks
E. vasodilator therapy as an inpatient
A nswer
E
The patient described in the vignette has a significantly elevated blood pressure that
involves marked and reproducible systolic and diastolic hypertension. The best management
plan is to monitor his blood pressure while the cause is ascertained and treatment begun, which
involves admission to the hospital and initial treatment with an intravenous antihypertensive
agent. The goal of such therapy is to reduce the blood pressure by 25% or less over the first 8
hours and gradually normalize it over the next 48 hours to avoid complications (eg,
cerebrovascular accident).
The choice of chronic antihypertensive therapy depends, in part, on the cause of the
hypertension, but for immediate short-term management, vasodilators (eg, calcium channel
blockers, hydralazine, nitroprusside) are useful. These agents reduce the afterload against
which the left ventricle pumps, thereby reducing its work and oxygen consumption. Alternatively,
short-acting beta blockers could be used in the acute setting. When using beta blockers,
however, the clinician must bear in mind their potential complications, including exacerbation of
underlying asthma. Of importance, pharmacologic management of severe hypertension and
hypertensive crisis should use medications that can be titrated to effect readily and have a fast
onset of action. Diuretics, particularly the thiazide class, often are used as first-line
antihypertensive agents for those who have mild or moderate hypertension that can be
controlled on an outpatient basis. These may be used in combination with other agents, including
but not limited to angiotensin-converting enzyme inhibitors or angiotensin receptor blockers, if
adequate control is not obtained with a single agent. The significant hypertension reported for the
boy in the vignette requires immediate action; repeating the blood pressure measurement in 1 to
2 weeks is not appropriate.
Q2 :
Before antihypertensive therapy begins, secondary causes of hypertension should be ruled out.
Laboratory findings that suggest an underlying cause of hypertension include:
A. a decreased serum potassium level.
B. an increased urinary catecholamine level.
C. an increased blood cortisol level.
D. All of the above.
Answer
D
All these investigations are needed.
Low serum potassium levels in a hypertensive patient suggest primary aldosteronism. Elevated urinary catecholamines suggest a pheochromocytoma; other signs and symptoms of this tumor include weight loss, episodic flushing, and sweating. Elevated serum cortisol levels suggest Cushing syndrome; the patient is also
likely to have a round (moon) face and truncal obesity.
Secondary hypertension requires treatment of the underlying cause; supplementary antihypertensive drug
therapy may also be needed.
-------------------
Q2- 3:
A 12-year-old boy presented with a 2-year history of intermittent tachycardia. More recently he had developed headaches, hot flushes and weight loss. He denies any drug misuse. He was hypertensive with a blood pressure of 218/ 134 mmHg. Investigations showed a normal cardiac ECHO
and ECG showed sinus tachycardia of 130/minutes and ventricular ectopics.
Full blood count, Thyroid function and LFTs were normal. Urinary normetadrenaline levels were raised.
Abdominal ultrasound scan showed an abdominal mass. CT scan identified this as a 5 cm 4 cm vascular mass arising from Organ of Zuckerkandl. Meta-iodobenzylguanidine (MiBG) scan showed increased uptake in an isolated avid lesion at the bifurcation of the aorta.
(Q 2) What is the most likely diagnosis?
A- Grave’s disease
B- Phaeochromocytoma
C- Cushing’s disease
D-Neuroblastoma
(Q 3) What is the prognosis?
A- Good outcome but requires lifelong hormone replacement
B- 5-year survival rates of <10%
C- Potentially curable with surgical resection
D- Invariably fatal, no known cure
Answer :
2 : B
3: C
Phaeochromocytoma is a tumour of the sympathetic nervous system that presents with episodes of tachycardia/ palpitations, headaches, sweating and pallor. These are precipitated because of catecholamine-releasing properties of this tumour. Symptomatic relief can be achieved with the use of alphaand
betablockers, such as bisoprolol and phenoxybenzamine respectively. Doses should be titrated up to effect
.
Tumours are most commonly located in the adrenal glands, but as in this case, have a number of extra-renal
sites where they may be found. The Organ of Zuckerkandl is not normally visualized on scanning, and if seen, is suggestive of pathology such as neuroblastoma or phaeochromocytoma.
In this age group, phaeochromocytoma is a much more likely diagnosis. Undiagnosed, the tumour can be fatal due to the cardiovascular risk. Early diagnosis lends itself to curative treatment by surgical resection.
Approximately 4% of paediatric tumours are malignant, they can be locally invasive or metastazise to lung, bone, liver and brain. It is more common in boys than girls with a ratio of 2:1.
The differential diagnosis should include primary hypertension, thyrotoxicosis, co-arctation of the aorta and
neuroblastoma. Investigations should be performed in order to make a comprehensive diagnosis, to include blood pressure monitoring, cardiac assessment, radiological imaging with ultrasound or CT and urinary catecholamines.
A nswer
E
The patient described in the vignette has a significantly elevated blood pressure that
involves marked and reproducible systolic and diastolic hypertension. The best management
plan is to monitor his blood pressure while the cause is ascertained and treatment begun, which
involves admission to the hospital and initial treatment with an intravenous antihypertensive
agent. The goal of such therapy is to reduce the blood pressure by 25% or less over the first 8
hours and gradually normalize it over the next 48 hours to avoid complications (eg,
cerebrovascular accident).
The choice of chronic antihypertensive therapy depends, in part, on the cause of the
hypertension, but for immediate short-term management, vasodilators (eg, calcium channel
blockers, hydralazine, nitroprusside) are useful. These agents reduce the afterload against
which the left ventricle pumps, thereby reducing its work and oxygen consumption. Alternatively,
short-acting beta blockers could be used in the acute setting. When using beta blockers,
however, the clinician must bear in mind their potential complications, including exacerbation of
underlying asthma. Of importance, pharmacologic management of severe hypertension and
hypertensive crisis should use medications that can be titrated to effect readily and have a fast
onset of action. Diuretics, particularly the thiazide class, often are used as first-line
antihypertensive agents for those who have mild or moderate hypertension that can be
controlled on an outpatient basis. These may be used in combination with other agents, including
but not limited to angiotensin-converting enzyme inhibitors or angiotensin receptor blockers, if
adequate control is not obtained with a single agent. The significant hypertension reported for the
boy in the vignette requires immediate action; repeating the blood pressure measurement in 1 to
2 weeks is not appropriate.
Q2 :
Before antihypertensive therapy begins, secondary causes of hypertension should be ruled out.
Laboratory findings that suggest an underlying cause of hypertension include:
A. a decreased serum potassium level.
B. an increased urinary catecholamine level.
C. an increased blood cortisol level.
D. All of the above.
Answer
D
All these investigations are needed.
Low serum potassium levels in a hypertensive patient suggest primary aldosteronism. Elevated urinary catecholamines suggest a pheochromocytoma; other signs and symptoms of this tumor include weight loss, episodic flushing, and sweating. Elevated serum cortisol levels suggest Cushing syndrome; the patient is also
likely to have a round (moon) face and truncal obesity.
Secondary hypertension requires treatment of the underlying cause; supplementary antihypertensive drug
therapy may also be needed.
-------------------
Q2- 3:
A 12-year-old boy presented with a 2-year history of intermittent tachycardia. More recently he had developed headaches, hot flushes and weight loss. He denies any drug misuse. He was hypertensive with a blood pressure of 218/ 134 mmHg. Investigations showed a normal cardiac ECHO
and ECG showed sinus tachycardia of 130/minutes and ventricular ectopics.
Full blood count, Thyroid function and LFTs were normal. Urinary normetadrenaline levels were raised.
Abdominal ultrasound scan showed an abdominal mass. CT scan identified this as a 5 cm 4 cm vascular mass arising from Organ of Zuckerkandl. Meta-iodobenzylguanidine (MiBG) scan showed increased uptake in an isolated avid lesion at the bifurcation of the aorta.
(Q 2) What is the most likely diagnosis?
A- Grave’s disease
B- Phaeochromocytoma
C- Cushing’s disease
D-Neuroblastoma
(Q 3) What is the prognosis?
A- Good outcome but requires lifelong hormone replacement
B- 5-year survival rates of <10%
C- Potentially curable with surgical resection
D- Invariably fatal, no known cure
Answer :
2 : B
3: C
Phaeochromocytoma is a tumour of the sympathetic nervous system that presents with episodes of tachycardia/ palpitations, headaches, sweating and pallor. These are precipitated because of catecholamine-releasing properties of this tumour. Symptomatic relief can be achieved with the use of alphaand
betablockers, such as bisoprolol and phenoxybenzamine respectively. Doses should be titrated up to effect
.
Tumours are most commonly located in the adrenal glands, but as in this case, have a number of extra-renal
sites where they may be found. The Organ of Zuckerkandl is not normally visualized on scanning, and if seen, is suggestive of pathology such as neuroblastoma or phaeochromocytoma.
In this age group, phaeochromocytoma is a much more likely diagnosis. Undiagnosed, the tumour can be fatal due to the cardiovascular risk. Early diagnosis lends itself to curative treatment by surgical resection.
Approximately 4% of paediatric tumours are malignant, they can be locally invasive or metastazise to lung, bone, liver and brain. It is more common in boys than girls with a ratio of 2:1.
The differential diagnosis should include primary hypertension, thyrotoxicosis, co-arctation of the aorta and
neuroblastoma. Investigations should be performed in order to make a comprehensive diagnosis, to include blood pressure monitoring, cardiac assessment, radiological imaging with ultrasound or CT and urinary catecholamines.
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