1.A 22-year-old Caucasian male presents to the emergency room
complaining of severe headaches and vomiting. Soon after, he
slips into a coma and dies. Autopsy shows a ruptured cerebral
aneurysm with extensive intracranial haemorrhage. This patient's
condition is most likely associated with:
A. Primum-type atrial septal defect
B. Secundum-type atrial septal defect
C. Ventricular septal defect
D. Patent ductus arteriosus
E. Coarctation of the aorta
F. Tetralogy of fallot
Answer: E
Explanation:
Since there is no history of head trauma, this patient most likely suffered
a spontaneous intracranial haemorrhage (SIGH). The most common
causes of SIGH in young adults are arteriovenous malformations,
ruptured cerebral aneurysms, or abuse of sympathomimetic drugs such
as cocaine.
We are told that this patient had a ruptured cerebra.I aneurysm, and are
asked to identify an associated congenital cardiac anomaly. Although
coarctation of the aorta may occur as a solitary defect, it may also be
associated with other congenital cardiac anomalies or with berry
aneurysms of the Circle of Willis. Berry aneurysms are particularly prone
to rupture when associated with coarctation, because of hypertension in
branches of the aortic arch proximal to the coarct. Subarachnoid
Haemorrhage Is The Result.
(Choices A, B, and C) Isolated atrial or ventricular septal defects are not
commonly associated with spontaneous rupture of intracranial
aneurysms in young adults. The incidence of intracerebral aneurysms is
not increased in patients with isolated septal defects, nor are septal
defects associated with severe systemic hypertension (though they may
,cause severe pulmonary hypertension via left-to-right intracardiac shunt
flow). However, the incidence of thromboembolic stroke as the result of
paradoxical embolism might be increased in patients with an atrial or
ventricular septal defect and late-onset right-to-left shunting.
2. Over the past few months, a 54-year-old Caucasian male has
experienced several episodes of thrombophlebitis involving various sites
in both his arms and legs. The patient's symptoms may indicate the
presence of which of the following?
A. Hyperthyroidism
B. Celiac sprue
C. Polycystic kidneys
D. Visceral cancer
E. Chlamydial infection
Answer: D
Explanation:
Migratory thrombophlebitis should always make you think "cancer."
(Small vessel hypersensitivity type vasculitis affecting the venules is also
possible in this scenario, but is not an answer choice.}
Hypercoagulability is a very common paraneoplastic syndrome seen
most commonly in adenocarcinoma sofa pancreas,colon,or lung.
Hypercoagulability Occurs Because Adenocarcinomas produce a
thromboplastin-like substance that is capable of causing chronic
intravascular coagulations that are both disseminated and tend to
migrate. Migratory superficial thrombophlebitis is "Trousseau's
syndrome."
(Choice A) Hyperthyroidism has been associated with a
hypercoagulable state and occasionally reported in cases of cerebral
venous thrombosis, but evidence of a significant association with venous
thrombotic events is currently lacking.
,(Choice B) Celiac sprue has been associated w ith a hemorrhagic
diathesis (rather than abnormal thrombosis}. The malabsorption resulting
from sprue can result in vitamin K deficiency.
(Choice C) Polycystic kidney disease is not among conditions
associated with an acquired prothrombotic state.
(Choice E) Infections can create a systemic inflammatory state that
favours thrombosis. However, a recent study has shown that lgG
antibody titers against Chlamydia pneumoniae were not correlated with
the concentrations of such prothrombotic inflammatory mediators as IL-6
and IL-8.
3. A 43-year-old Caucasian male is hospitalised with recent-onset
oliguria and a high serum creatinine level. He has been seen in the clinic
several times for an intranasal ulcer that has failed to heal. This patient's
condition is most likely associated with antibodies against which of the
following?
A. Glomerular basal membrane
B. Smooth muscle cells
C. Neutrophils
D. Erythrocytes
E. Platelets
F. Mitochondria
Answer: C
Explanation:
Nasal mucosal ulcerations and glomerulonephritis are most
characteristic of granulomatosis with polyangiitis (Wegener's).
Cytoplasmic-staining antineutrophil cytoplasmic antibodies (c-ANCAs)
, are virtually pathognomonic for granulomatosis with polyangiitis, with a
better than 90% specificity and sensitivity. C- ANCA may also be useful
as a quantitative measure of disease activity.
(Choice A) Goodpasture syndrome is sometimes called anti-glomerular
basement membrane (GBM) antibody disease. Presenting clinical
manifestations of Goodpasture syndrome are hemoptysis, radiographic
focal pulmonary consolidations, and a glomerulonephritis that may
rapidly progress to renal failure. Upper respiratory tract ulceration is not
characteristic.
(Choice B) Smooth muscle cell antibodies are commonly seen in
patients with autoimmune hepatitis.
(Choice D) Anti Erythrocyte antibodies can produce hemolysis of an
intravascular and/or extravascular type. Hemoglobinemia from
intravascular hemolysis can end in acute renal failure, although this is
predominantly seen after transfusion reactions only when other
conditions, such as hypovolemia and/or acidemia, coexist. Hemolytic
anaemia would not cause isolated ulceration of mucosa.
(Choice E) Platelet antibodies can often cause thrombocytopenia by
binding to platelets. Neither these autoantibodies, nor thrombocytopenia
is known to cause acute renal failure.
(Choice F) Antimitochondrial antibodies are seen iin primary biliary
cirrhosis.
complaining of severe headaches and vomiting. Soon after, he
slips into a coma and dies. Autopsy shows a ruptured cerebral
aneurysm with extensive intracranial haemorrhage. This patient's
condition is most likely associated with:
A. Primum-type atrial septal defect
B. Secundum-type atrial septal defect
C. Ventricular septal defect
D. Patent ductus arteriosus
E. Coarctation of the aorta
F. Tetralogy of fallot
Answer: E
Explanation:
Since there is no history of head trauma, this patient most likely suffered
a spontaneous intracranial haemorrhage (SIGH). The most common
causes of SIGH in young adults are arteriovenous malformations,
ruptured cerebral aneurysms, or abuse of sympathomimetic drugs such
as cocaine.
We are told that this patient had a ruptured cerebra.I aneurysm, and are
asked to identify an associated congenital cardiac anomaly. Although
coarctation of the aorta may occur as a solitary defect, it may also be
associated with other congenital cardiac anomalies or with berry
aneurysms of the Circle of Willis. Berry aneurysms are particularly prone
to rupture when associated with coarctation, because of hypertension in
branches of the aortic arch proximal to the coarct. Subarachnoid
Haemorrhage Is The Result.
(Choices A, B, and C) Isolated atrial or ventricular septal defects are not
commonly associated with spontaneous rupture of intracranial
aneurysms in young adults. The incidence of intracerebral aneurysms is
not increased in patients with isolated septal defects, nor are septal
defects associated with severe systemic hypertension (though they may
,cause severe pulmonary hypertension via left-to-right intracardiac shunt
flow). However, the incidence of thromboembolic stroke as the result of
paradoxical embolism might be increased in patients with an atrial or
ventricular septal defect and late-onset right-to-left shunting.
2. Over the past few months, a 54-year-old Caucasian male has
experienced several episodes of thrombophlebitis involving various sites
in both his arms and legs. The patient's symptoms may indicate the
presence of which of the following?
A. Hyperthyroidism
B. Celiac sprue
C. Polycystic kidneys
D. Visceral cancer
E. Chlamydial infection
Answer: D
Explanation:
Migratory thrombophlebitis should always make you think "cancer."
(Small vessel hypersensitivity type vasculitis affecting the venules is also
possible in this scenario, but is not an answer choice.}
Hypercoagulability is a very common paraneoplastic syndrome seen
most commonly in adenocarcinoma sofa pancreas,colon,or lung.
Hypercoagulability Occurs Because Adenocarcinomas produce a
thromboplastin-like substance that is capable of causing chronic
intravascular coagulations that are both disseminated and tend to
migrate. Migratory superficial thrombophlebitis is "Trousseau's
syndrome."
(Choice A) Hyperthyroidism has been associated with a
hypercoagulable state and occasionally reported in cases of cerebral
venous thrombosis, but evidence of a significant association with venous
thrombotic events is currently lacking.
,(Choice B) Celiac sprue has been associated w ith a hemorrhagic
diathesis (rather than abnormal thrombosis}. The malabsorption resulting
from sprue can result in vitamin K deficiency.
(Choice C) Polycystic kidney disease is not among conditions
associated with an acquired prothrombotic state.
(Choice E) Infections can create a systemic inflammatory state that
favours thrombosis. However, a recent study has shown that lgG
antibody titers against Chlamydia pneumoniae were not correlated with
the concentrations of such prothrombotic inflammatory mediators as IL-6
and IL-8.
3. A 43-year-old Caucasian male is hospitalised with recent-onset
oliguria and a high serum creatinine level. He has been seen in the clinic
several times for an intranasal ulcer that has failed to heal. This patient's
condition is most likely associated with antibodies against which of the
following?
A. Glomerular basal membrane
B. Smooth muscle cells
C. Neutrophils
D. Erythrocytes
E. Platelets
F. Mitochondria
Answer: C
Explanation:
Nasal mucosal ulcerations and glomerulonephritis are most
characteristic of granulomatosis with polyangiitis (Wegener's).
Cytoplasmic-staining antineutrophil cytoplasmic antibodies (c-ANCAs)
, are virtually pathognomonic for granulomatosis with polyangiitis, with a
better than 90% specificity and sensitivity. C- ANCA may also be useful
as a quantitative measure of disease activity.
(Choice A) Goodpasture syndrome is sometimes called anti-glomerular
basement membrane (GBM) antibody disease. Presenting clinical
manifestations of Goodpasture syndrome are hemoptysis, radiographic
focal pulmonary consolidations, and a glomerulonephritis that may
rapidly progress to renal failure. Upper respiratory tract ulceration is not
characteristic.
(Choice B) Smooth muscle cell antibodies are commonly seen in
patients with autoimmune hepatitis.
(Choice D) Anti Erythrocyte antibodies can produce hemolysis of an
intravascular and/or extravascular type. Hemoglobinemia from
intravascular hemolysis can end in acute renal failure, although this is
predominantly seen after transfusion reactions only when other
conditions, such as hypovolemia and/or acidemia, coexist. Hemolytic
anaemia would not cause isolated ulceration of mucosa.
(Choice E) Platelet antibodies can often cause thrombocytopenia by
binding to platelets. Neither these autoantibodies, nor thrombocytopenia
is known to cause acute renal failure.
(Choice F) Antimitochondrial antibodies are seen iin primary biliary
cirrhosis.
