Past test Flashcards
What is considered portal hypertension?
then the pressure in the portal vein is > 12mmHg
What is the wedged hepatic venous pressure?
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
What test can be used to elucidate a mechanical reason for elevated hemidiaphragm?
The diagnosis of unilateral paralysis, suggested bu asymmetric elevation of the affected hemidiaphragm on XR can be confimred by fluoroscopy
What is pituitary apoplexy?
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.
How does pituitary apoplexy present?
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
How is pituitary apoplext diagnosed?
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.
ECG changes in pericarditis?
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
What is Pseudoxanthoma elasticum?
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
What is inclusion body myositis?
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
What does EMG show in inclusion body myositis?
inflammatory myopathic changes, increased insertional and spontaneous activity as well and small amplitude, short duration polyphasic unit
MEN type 1?
3ps
Parathyroid - hyperparathyroid due to parathyroid hyperplasia
Pituitary
ancreas - insulinoma and gastrinoma
Also adrenal and thyroid
MEN 1 gene
How does MEN present?
Most commonly presents with hypercalcaemia
MEN typa IIa?
Medullary thyroid cancer (70%)
Parathyroid (60%)
Phaechromocytoma
RET oncocogene
MEN type IIb?
Medullary thyroid cancer
Phaeochromocytoma
Marfanoid habitus
Neuromas
RET oncogene
What type of MEN is Hirshprung’s disease associated with?
MEN 2
What is mucosal neuroma?
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
How does metoclopramide cause galactorrhoea?
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.
Microscopic polyangitis
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%
What is the genetic link between MND and frontotemporal dementia?
It is a hexanucleotide repeat expansion of the GGGGCC repeat in the C9orf72 gee
How many trinucleotide repeat are there in Huntingtons?
Cases have been reported with repeats ranging from 37 to 120
Polymyositis
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
what is membranoproliferative glomerulonephritis?
also known as mesangiocapillary glomerulonephritis
What is types 1 membranoproliferative glomerulonephritis?
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
What is types 2 membranoproliferative glomerulonephritis?
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’