pathology 2 Flashcards

1
Q

which condition causes:
-systolic murmur loudest at the back below left scapula
-rib notching on CXR
-radiofemoral delay
-can have syncope/ claudication

A

Aortic coarctation

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2
Q

Why do you see rib notching on CXR of aortic coarctation?

A

The intercostal vessels act as collaterals and are dilation

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3
Q

At which site is aortic coarctation most common

A

Site of ductus arteriosus

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4
Q

Groups likely to get aortic coarctation

A

Boys
Girls w/ turners

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5
Q

Mx for aortic coarctation

A

-angioplasty
-surgical resection

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6
Q

what are the tumours found in patients with MEN 1

A

3 P’s + thyroid + adrenal adenoma

-Pituitary (secretes ACTH, GH, prolactin)
-Parathyroid
-pancreatic (islet cells/ ZEllison)

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7
Q

what is the gene and chromosome mutation in MEN 1

A

MENIN gene
Chr 11

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8
Q

what are the tumours found in patients with MEN 2A

A

-Phaeochromocytoma
-Medullary thyroid carcinoma
-Hyperparathyroidism

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9
Q

what are the tumours found in patients with MEN 2b

A

-Phaeochromocytoma
-Medullary thyroid
-hyperparathyroid
+
marfanoid features
+
mucosal neuromas

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10
Q

what is the gene and chromosome mutation in MEN 2a/b

A

RET-oncogene
Chr 10

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11
Q

which cells of the thyroid produce the hormone that reduces calcium levels

A

Parafollicular cells/ C-cells produce calcitonin

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12
Q

what is the inhertience pattern for MEN

A

Autosomal dominant

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13
Q

What are the colonic and extracolonic features seen in Gardners syndrome

A

Colonic:
colonic polyps

Extra:
jaw/ skull osteomas
epidermal/ sebaceous cysts
desmoid tumours
Thyroid cancer

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14
Q

T/F gardners syndrome is a variant of FAP

A

True

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15
Q

What are the gene/ chromosome affected in Gardners syndrome

A

APC gene
Chr 5

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16
Q

What is the inheritence pattern for Gardners?

A

Autosomal dominant

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17
Q

What are the features of Lynch syndrome

A

Colonic + endometrial cancer

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18
Q

What is the criteria used to identify high risk patients for Lynch syndrome

A

Amsterdam criteria:

-needs 3 or more members of the family affected with 1 being a first degree relative of the other 2

-2 successive generations affected

-1 or more colon cancers diagnosed under 50 years

-FAP has been excluded

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19
Q

What is the inheritence pattern for Lynch?

A

Autosomal dominant

20
Q

What are the characteristic malignancies in Li-Farumani syndrome

A

Leukaemias
Sarcomas
breast cancers
brain Ca
adrenalCa

21
Q

What gene mutation is seen in Li-Farumani syndrome?

A

p53 tumour suppressor gene

22
Q

What is the inheritence pattern for Li-Farumani syndrome?

A

Autosomal dominant

23
Q

What is the management of AAA according to its size

A

<2cm nothing
<5cm surveillance imaging
5.5-6cm surgery

24
Q

What are the indications for surgical management of AAA

A

-size 5.5-6cm and asymptomatic
-symptomatic
-ruptured AAA

25
Q

What age does screening for AAA start

A

65 yrs

26
Q

What characteristics of the aneurysm are required for an EVAR

A

-long neck
-straight iliac vessels
-healthy groin vessels

27
Q

What is resected in a right hemicolectomy

A

caecum + ascending colon +/- right half of transverse colon
ileocolic + right colic + right branch of middle colic vessels

27
Q

Complications following AAA repair

A

-ITU stay
-cardiac: reperfusion injury, complications from cross clamp
-renal injury/ AKI
-infections
-aorto-enteric fistula
-embolism (gut/ feet)

27
Q

What are the common sites of rupture of a AAA and which is more common?

A

anteroperitoneal
retroperitoneal (more common)

27
Q

What is resected in a left hemicolectomy

A

descending colon +/- left half of transverse

left colic + left branch of middle colic + IMA

28
Q

What is resected in a anterior resection

A

high rectum

29
Q

What is resected in a Hartmann’s

A

sigmoid + rectum + colostomy + close rectal stump

30
Q

How would you treat a DALM

A

panproctocolectomy

31
Q

What is the diagnosis for
PC: cyclical pyrexia, cough, haemoptysis, weightloss, asymptomatic lymphadenopathy

A

Hodgkins lymphoma

32
Q

What is the staging for hodgkins

A

Ann Arbor

1-single lymph node region
2- multiple nodes on same side of diaphragm
3- nodes on both sides of diaphragm
4-extranodal sites

33
Q

what are the subtypes of hodgkins

A

Nodular sclerosing Hodgkin lymphoma (NSHL)
Mixed-cellularity Hodgkin lymphoma (MCHL)
Lymphocyte-depleted Hodgkin lymphoma (LDHL)
Lymphocyte-rich classical Hodgkin lymphoma (LRHL)

34
Q

Tx of Hodgkins

A

both chemo and radiotherapy

35
Q

Which oncovirus causes both Burkitss and Hodgkins?

A

EBV

36
Q

What are the cellular changes in blood that are seen following a splenectomy (in time order)

A

granulocytosis
thrombocytopenia
target cells + siderocytes + reticulocytes
lymphocytosis
monocytosis

37
Q

What is the rule of thirds for carcinoid syndrome

A

1/3 multiple cancers
1/3 second tumour
1/3 small bowel
1/3 metastasize

38
Q

Where do carcinoid tumours originate

A

neuroendocrine cells
usually in the gut (ileum/ appendix)

39
Q

What do carcinoid tumours secrete

A

serotonin

40
Q

T/F: carcinoid tumours on the appendix can present with carcinoid syndrome even without mets to the liver/ lung

A

False

tumours need to metastasize outsid eof the gut to cause the carcinoid syndrome

41
Q

Investigations for carcinoid syndrome

A

-5-HIAA in a 24-hour urine collection
-Somatostatin receptor scintigraphy
-CT scan
-Blood testing for chromogranin A

42
Q

Mx of carcinoid syndrome

A

octreotide
surgery

43
Q

Clinical features of carcinoid syndrome

A

flushing
palpitations
diarrhoea
asthma
pul valve stenosis + tricuspid regurg

insidious onset