Past test Flashcards

1
Q

What is considered portal hypertension?

A

then the pressure in the portal vein is > 12mmHg

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2
Q

What is the wedged hepatic venous pressure?

A

The wedged hepatic venous pressure is the pressure recorded by a catheter wedged in a hepatic vein - it most accurately reflect the portal venous pressure in the hepatic sinusoids.
Wedged hepatic venous pressure is elevated in sinusoidal and post sinusoidal portal hypertension but remains normal in pre-sinusoidal portal hypertension

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3
Q

What test can be used to elucidate a mechanical reason for elevated hemidiaphragm?

A

The diagnosis of unilateral paralysis, suggested bu asymmetric elevation of the affected hemidiaphragm on XR can be confimred by fluoroscopy

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4
Q

What is pituitary apoplexy?

A

Pituitary apoplexy is a rare yet potentially life-threatening clinical syndrome characterized by acute haemorrhagic infarction of the pituitary gland, typically within a pre-existing pituitary adenoma.

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5
Q

How does pituitary apoplexy present?

A

an abrupt onset of severe headache, visual disturbances, ophthalmoplegia due to cranial nerve palsies (particularly CN III, IV, and VI), altered mental status, and potential endocrine dysfunction such as adrenal insufficiency or hypopituitarism

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6
Q

How is pituitary apoplext diagnosed?

A

MRI

Laboratory evaluation should include assessment of anterior pituitary hormonal function (e.g., serum cortisol, thyroid-stimulating hormone, free thyroxine, prolactin, insulin-like growth factor-1) as well as electrolytes to evaluate for diabetes insipidus.

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7
Q

ECG changes in pericarditis?

A

ST elevation with concavity upward in all leads facing the epicardial surface i.e anterior, inferior and lateral

Only cavity leads - aVR, V1 and rarely V2 show ST depression

This is followed by the return of ST segments to baseline and flat or inverted T waves

PR depression - the most specific marker for pericarditis

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8
Q

What is Pseudoxanthoma elasticum?

A

Pseudoxanthoma elasticum is an inherited condition (usually autosomal recessive*) characterised by an abnormality in elastic fibres

Features
retinal angioid streaks
‘plucked chicken skin’ appearance - small yellow papules on the neck, antecubital fossa and axillae
cardiac: mitral valve prolapse, increased risk of ischaemic heart disease
gastrointestinal haemorrhage

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9
Q

What is inclusion body myositis?

A

It typicall presents with slowly progressive weakness and wasting of the finger flexors and quadriceps.
Slow disease progression
Leads to diffiuclty in walking, frequent falls
Dysphagia is a common manifestation
CK is normal or up to 5x the normal range

There is no available treatment

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10
Q

What does EMG show in inclusion body myositis?

A

inflammatory myopathic changes, increased insertional and spontaneous activity as well and small amplitude, short duration polyphasic unit

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11
Q

MEN type 1?

A

3ps
Parathyroid - hyperparathyroid due to parathyroid hyperplasia

Pituitary

ancreas - insulinoma and gastrinoma
Also adrenal and thyroid

MEN 1 gene

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12
Q

How does MEN present?

A

Most commonly presents with hypercalcaemia

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13
Q

MEN typa IIa?

A

Medullary thyroid cancer (70%)
Parathyroid (60%)
Phaechromocytoma

RET oncocogene

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14
Q

MEN type IIb?

A

Medullary thyroid cancer
Phaeochromocytoma

Marfanoid habitus
Neuromas

RET oncogene

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15
Q

What type of MEN is Hirshprung’s disease associated with?

A

MEN 2

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16
Q

What is mucosal neuroma?

A

This is a dermatological unique to MEN2B
Mucosal neuromas are the most consistent and distinctive feature
Yellow white sessile painless nodules on the lips or tongue

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17
Q

How does metoclopramide cause galactorrhoea?

A

Metoclopramide is a dopamine antagonist that binds to D2- receptors on pituitary lactotropes. This stimulates the release of prolactin by anterior pituitary resulting in galacotthoea.

Prolactin inhibits GNRH and gonadotopin secretion and so oligomenorrhoea or amenorrhoea can occur.

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18
Q

Microscopic polyangitis

A

Microscopic polyangiitis is a small-vessel ANCA vasculitis.

Features
renal impairment: raised creatinine, haematuria, proteinuria
fever
other systemic symptoms: lethargy, myalgia, weight loss
rash: palpable purpura
cough, dyspnoea, haemoptysis
mononeuritis multiplex

Investigations
pANCA (against MPO) - positive in 50-75%
cANCA (against PR3) - positive in 40%

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19
Q

What is the genetic link between MND and frontotemporal dementia?

A

It is a hexanucleotide repeat expansion of the GGGGCC repeat in the C9orf72 gee

20
Q

How many trinucleotide repeat are there in Huntingtons?

A

Cases have been reported with repeats ranging from 37 to 120

21
Q

Polymyositis

A

symmetrical proximal muscle weakness
T cell mediated - against muscle fibres

Features
- proximal muscle weaakness, raynaud’s, ILD, dysphagia, dysphonia

Ix - elevated CK, EMG, muscle biopsy, anti-Jo-1 (seen in pattern of disease associated with lung involvement, Raynaud’s and fever)
Best diagnostic test - muscle biopsy

tx - high dose steroids
azathioprine

dermatomyositis is a variant of the disease where skin manifestations are prominent, for example a purple (heliotrope) rash on the cheeks and eyelids

22
Q

what is membranoproliferative glomerulonephritis?

A

also known as mesangiocapillary glomerulonephritis

23
Q

What is types 1 membranoproliferative glomerulonephritis?

A

accounts for 90% of cases
cause: cryoglobulinaemia, hepatitis C
renal biopsy
electron microscopy: subendothelial and mesangium immune deposits of electron-dense material resulting in a ‘tram-track’ appearance

24
Q

What is types 2 membranoproliferative glomerulonephritis?

A

Type 2 - ‘dense deposit disease’
causes: partial lipodystrophy (patients classically have a loss of subcutaneous tissue from their face), factor H deficiency
caused by persistent activation of the alternative complement pathway
low circulating levels of C3
C3b nephritic factor is found in 70%
an antibody to alternative-pathway C3 convertase (C3bBb)
stabilizes C3 convertase
renal biopsy
electron microscopy: intramembranous immune complex deposits with ‘dense deposits’

25
Mechanism of action of trimethoprim?
interferes with DNA synthesis by inhibiting dihydrofolate reductase may, therefore, interact with methotrexate, which also inhibits dihydrofolate reductase
26
Methotrexate interactions?
Interactions avoid prescribing trimethoprim or co-trimoxazole concurrently - increases risk of marrow aplasia high-dose aspirin increases the risk of methotrexate toxicity secondary to reduced excretion Methotrexate toxicity the treatment of choice is folinic acid
27
What is Truvada?
A conmbination medicadtion containing emtricitabine and tenofavir ## Footnote recommended as first line therapy in the UK It can lead to renal tubular damage
28
What is medullary sponge kidney?
Condition characterised by dilation of the collecting ducts in the papillae with accompanying cystic changes. cyst formation is commonly associated with the development of small calculi in the papillary zones with surounding increased density - this is because the dilated collecting ducts are filled with contrast medium,
29
What are the symptoms of bronchial carcinoid?
Recurrent haemoptysis with segmental collapse is a typical presentation of bronchial carcinoid. Recurrent pneumonia Can rarely cause carcinoid syndrome
30
Invesitgations for carcinoid tumours?
urinary 5-HIAA plasma chromogranin A y
31
Management of carcinoid syndrome?
somatostatin analogues e.g. octreotide diarrhoea: cyproheptadine may help
32
mechanism of action of methotrexate?
an antimetabolite that inhibits dihydrofolate reductase, which interferes with tetrahydrofolate stores required for synthesis of purines and thymidylate in inflammatory cells.
33
What is McCune-Albright syndrome?
McCune-Albright syndrome is not inherited, it is due to a random, somatic mutation in the GNAS gene. Features precocious puberty cafe-au-lait spots polyostotic fibrous dysplasia short stature
34
Ehlers-Danlos syndrome?
Ehler-Danlos syndrome is an autosomal dominant connective tissue disorder that mostly affects type III collagen. This results in the tissue being more elastic than normal leading to joint hypermobility and increased elasticity of the skin. Features and complications elastic, fragile skin joint hypermobility: recurrent joint dislocation easy bruising aortic regurgitation, mitral valve prolapse and aortic dissection subarachnoid haemorrhage angioid retinal streaks
35
When should pleural effusions be drained?
When the fluid is infected when the pH <7.2 when the gram stains shows the presence of organisms when the fluid is frankly purulent when clinical improvement is slow despite abx therapy
36
What is seen on menopausal blood tests?
Persistently high FSH and low oestrogen
37
what is fragile X syndrome?
Fragile X syndrome is a trinucleotide repeat disorder. Features in males learning difficulties large low set ears, long thin face, high arched palate macroorchidism hypotonia autism is more common mitral valve prolapse Features in females (who have one fragile chromosome and one normal X chromosome) range from normal to mild Diagnosis can be made antenatally by chorionic villus sampling or amniocentesis analysis of the number of CGG repeats using restriction endonuclease digestion and Southern blot analysis
37
what conditions are associoated wiht premature ovarian insufficiency?
Addisons disease autoimmune thyroid disease All woman should be tested for the FMR1 premutation (fragile X) regardless of family history
38
what is St John's wort taken for?
shown to be as effective as tricyclic antidepressants in the treatment of mild-moderate depression mechanism: thought to be similar to SSRIs (although noradrenaline uptake inhibition has also been demonstrated) **Inducer of the P450 system
39
Tetrad of symptoms in narcolepsy?
hypersomnia cataplexy hypnagogic/hypnopompic hallucinations sleep paralysis
40
when should multiple doses of activated charcoal be given?
to aid elimination of some drugs that are prone to enterohepatic circulation - charcoal adsorbs drug excreted in bile and prevents reabsorption in the small bowel. typical examples include aspirin, quinine, carbamezapin, phenobarbital and theophylline
41
Ratnauds and digital ulcers and calcinosis?
Systemic sclerosis (ulcers very unusual in primary raynaud's phenomenon)
42
Antibodies seen in systemic sclerosis?
Limited scleroderma with systemic involvement -anti-centromere antibody Diffuse scleroderma - scleroderma involving the trunk and proximal limbs as well as face and distal limbs (anti-Scl70 aka anti-topoisomerase-1
43
Investigations of avascular necrosis of the hip?
MRI is the investigation of choice
44
which anti-eplieptic causes haeorrhagic disease of the newborn
Phenytoin induces vitamin K metabolism, which can cause a relative vitamin K deficiency, creating the potential for heamorrhagic disease of the newborn
45
What are the types of autoimmune hepatitis?
## Footnote associated with HLA B8, DR3
46
differential for primary hyperparathyroidism?
It is important to consider the rare but relatively benign condition of benign familial hypocalciuric hypercalcaemia, caused by an autosomal dominant genetic disorder. Diagnosis is usually made by genetic testing and concordant biochemistry (urine calcium : creatinine clearance ratio <0.01-distinguished from primary hyperparathyroidism).