14/04/18 Flashcards
1
Q
What is this type of breathing?- high resp rate and shalow breathing?
A
Kussmaul breathing
2
Q
What are the causes a metabolic acidosis with a high anion gap?
A
lactic acidosis; DKA; aspirin overdose; uraemic acidosis-renal failure
3
Q
What are the triggers of DKA?
A
infection; surgery; MI; pancreatitis; chemotherapy; antipyschotics; wrong insulin dose/ non-compliance
4
Q
What are required for a diagnosis of DKA?
A
acidaemia; hyperglycaemia/known DM; ketonaemia or ketonuria
5
Q
What investigations should be done in DKA?
A
CXR; ECG; MSU; capillary and lab glucose; ketones; ph (venous unless decreased GCS/hypoxia); U&Es; bicarb; osmolality; FBC; blood cultures
6
Q
What is the first thing to do with DKA?
A
fluid replacement: 1L 0/9% over 1h
7
Q
What is the fluids in DKA if systolic <90?
A
500ml bolus over 15 mins
8
Q
How much insulin should be given after fluids in DKa?
A
50 units to 50ml 0.9%
9
Q
What is the aim for insulin in DKA?
A
fall in blood ketones of 0.5mmol/L/h or rise in bicarb of 3mmol/L/h with a fall of glucose of 3mmol/L/h
10
Q
When should 10% glucose be started in DKA?
A
when glucose <14 mmol
11
Q
How often should ketones and glucose be checked in DKA?
A
hourly
12
Q
What should be suspected if glucose is normal but significant ketonuria?
A
alcohol
13
Q
What are hte important secondary causes of immunodeficiency?
A
infections e.g HIV; malnutrition; malignancy; drugs; protein-losing states eg nephrotic syndrome; metabolic disease (DM; liver disease)
14
Q
What are hte investigations for suspected immunodeficiency?
A
FBC; liver and renal function; UA; HIV; total serum proteins
15
Q
What type of infection is common in CVID due to mucosal antibody deficiencies?
A
giardia
16
Q
What are the potential complications of CVID?
A
increased AI disease; lung disease; granulomatous disease; cancenr
17
Q
What causes CVID?
A
defect in ability of B cells to differentiate into antibody secreting plasma cells
18
Q
What is normal anion gap?
A
10-18mmol
19
Q
Why does renal tubular acidosis reuslt in nephrolithaisis?
A
acidosis causing increased citrate reabsoportion in the proximal tubule
20
Q
What are the common causes of renal tubular acidosis?
A
autoimmune disease, sickle cells, drugs eg lithium
21
Q
What does a normal anion gap mean?
A
hydrogen is being retained or that bicarb is being lost
22
Q
How is AKI categoried?
A
pre-renal; renal and post-renal
23
Q
What are hte causes of post-renal AKI?
A
bladder outflow obstruction; ureteric calculi; crystal nephropathy; urehtlial neoplasma
24
Q
What are the biochemical abnormalities seen with tumour lysis sundrome?
A
increased urate; hyperkalaemia; hyperphosphataemia and hypocalcaemia
25
What are the symptoms of tumour lysis syndrome?
seizures; arrhytmia; death
26
Why are there increased uric acid in tumour lysis syndrome?
release of nucleic acids which are metabolised to uric acid
27
Why does renal failure occur with tumour lysis syndrome?
urate and phosphate may crystallise and cause intratubular obstruction
28
What is the treatment for hyperuricaemia?
rasburicase which converts uric acid to inacigve metabolits
29
Why is rasburicase preffered over allopurinol in acute hyperuricaemia?
breask down exisitng urate rather than just preventing further formation of urate which is the action of allopurinol
30
What are the 2 broad categories of ambiguous genitalia?
virilised XX female and undervirilised male
31
what are the causes of virilised females?
fetal androgens- CAH or maternal
32
What are the causes of undervirilised males?
reduced foetal androgen production e.g Leydig cells defects; 5 alpha reductase defieicny; deficiency of anti-mullerian hormone ; androgen insufficiency; testicular dysgenesis
33
What is the inheritance of CAH?
AR
34
What is the most common cause of CAH?
21 hydroxylase deficiency
35
What happens in 21 hydroxylase defiency?
needed for the synthesis of aldosterone and cortisol but not adrenal androgens
36
Why is there hyperplasia in CAH?
lack of cortisol stimulates increased ACTH-- hyperplasia of the gland
37
What blood test is done for CAH?
increased 17-hydroxyprogesterone levels
38
What form of CAH may give boys ambiguous genitalia?
17-hydroxylase defiency results in decreased androgens
39
What forms the buffalo hump in Cushings disease?
supraclavicular and cervical fat deposits
40
What is the function of a high-dose dexamethasone suppression test?
suppresses cortisol levels in Cushing's disease but not ectopic ACTH or adrenal tumour
41
What are the endocrine causes of hypertension?
primary aldosteronism; Cushing's syndrone; phaeo; acromegaly; hypo/hyperthyroidism
42
What are the causes of transcellular movement of K causing hypolaemaia?
alkalosis; insulin treatment; beta-agonist; refeeding syndrome
43
What are the complications of hypokalaemia?
cardiac arrhythmia; constipation; ileus; rhabdomyolysys; respiratory muscle weakness-resp failure ; nephrogenic DI
44
What is the other term for primary hypogonadism?
hypergonadotrophic hypogonadism
45
What are the congenital causes of primary hypogonadism?
Klinefelters; testicular ageneis;s enzyme defects
46
What are hte acquired causes of priamry hypogonadism?
mumps orchiditis; bilateral testicualr injury/ torsion; irradiation or cytotoxic drugs
47
What common causes of secondary hypogonadism?
pituitary; hypothalamic; obesity; drugs
48
What is the tumour if there is hypogonadism; reduced libido and erectile dysfunction?
prolactinoma
49
Why should TFTs be done with suspected prolactinomas?
primary hypothyroidism is a common cause of hyperprolactinaemia
50
What is the definition of a microprolactinoma?
<10mm on MRI
51
what is first line treatment for prolactinomas?
bromocriptine or cabergoline
52
What happens as density of lipoproteins increases?
less Tgs with increasing cholesterol and protein
53
What are the causes of hyperlipidaemia?
primary or secondary- hypothyroidism; DM; obesity; nephrotic syndrome; renal and liver failure; drusg and alcohol
54
What drugs are implicated in hyperlipidaemia?
thiazides and antiretrovirals
55
What is the MOA of ezetimibe?
inhibits intestinal cholesterol absoprtion
56
What vessels do chylomicrons go into first?
lynphatics
57
What is cholesterol made from?
acetyl-CoA
58
What else is made from acetyl- CoA?
fatty acids
59
What makes lipoproteins?
golgi apparatus
60
What is found in lipoproteins?
apoprotein; TGs; phospholipids and cholesterol
61
What are the 2 types of lipoproteins the liver makes?
HDL and VLDL
62
How are fatty acids liberated in VLDL?
lipases in endothelial cells
63
What does VLDL become once some fatty acids are liberated?
IDL
64
What does IDL turn into?
LDL
65
What is the main function of LDL?
transportation of cholesterol
66
What is the function of VLDL?
transportation of fatty acids to tissues
67
what are the functions of cholesterol?
to make hormoens and stabilise cell membrane
68
What happens to excess cholesterol?
excreted in bile
69
What receptor is invovled in the uptake of HDL into hepatocytes?
scavenger receptors
70
What is the typical rash seen with graft vs host disease?
erythematous rash taht develops on the palms or soles of the feet
71
What is the chemo regime for hogdkins ?
ABVD
72
How can graft vs host disease occur in autologous grafts?
chemotherapy exposes self-antigens
73
What are the manifestations of GVHD?
maculo-papular rash; jaundice and hepatosplenomeglay and ultimately organ fibrosis
74
What are the common complications of a bone marrow transplant?
infection; bleeding and GVHD
75
What type of hypersensitivity reaction is GVHD?
type 4- T cell mediated
76
What are the 3 types of fragility #?
Colles; neck of femur and vertebral body
77
What tests should be done to look for secondary causes of osteoporosis?
FBC; ESR; bone profile; liver and renal function; thyroid function; screen for myeloma
78
What are the initial signs of allograft transplant rejection?
increased serum bilirubin and deranged LFTs
79
What type of hypersensitivty reaction is hyperacute organ rejection?
type II
80
When does acute organ rejection take place?
a number of weeks after the transplant
81
when does hyperacute organ rejection take palce?
within minutes of graft perfusion in theatre
82
What type of hypersensitivty reaction is acute organ rejection?
type 4
83
What are the calcineurin inhibitiors?
cyclosproin and tacrolimus
84
what is the fucntion of calcineurin inhibitiors?
inihibit calcineurin and production of IL2 by T cells
85
What is hyperacute rejection a result of ?
ABO incompatibility
86
What are the common causes of CKD?
DM; HT; GN; renovascular disease; chronic obstruction; hereditry
87
What occurs in renal osteodystrophy?
kidney failure- phosphate retenion and hypocalcaemia with reduced vitamin D--osteomalacia and hyperaprathyroidism; and in acidosis excess hydrogen ions are buffered by bone- further demineralisation
88
What is adrenoleukodystrophy?
x-linked disorder with accumulation of ver long chain fatty acids in several organs including the adrenal gladn
89
what is the treatment for Marfans?
regular screening with echo; beta blockers; surgery
90
Why are patients with marfans at increased risk of aortic aneurysm and dissection?
aortic root dilatation
91
What are the mx options for women with the BRCA mutations?
primary prevention- prophylactic mastectomy and/or oopherectomy; chemoprevention with tamoxifen or surveillance- self-examination with annual mammography and breast MRI
92
What is the risk of breast cancer with BRCA1?
80% lifetime
93
What is the risk of breast cancer with BRCA2?
40%
94
What is the risk of ovarian cancer in BRCA1 compared to BRCA2?
40% with BRCA1 and 10% with BRCA2
95
What are the most common causes of neonatal seizure?
hypoxic-ischaemic encephalopathy; intracranial haemorrhage; CNS infections; metabolic disorders
96
What is the most common cause of hypocalcaemia?
hypoalbuminaemia
97
What are the features of tetralogy of fallot?
ventricular septal defect; pulmonary stenosis; over-riding aorta; RV hypertrophy
98
What is the function of the 3rd branchial pouch?
development of hte inferior parathyroid glands and thymus
99
What is the function of the 4th branchial pouch?
superior parathyroid glands
100
What happens in DiGeorge syndrome?
defect in neural crest cell migration and defects in 3rd and 4th branchial pouch
101
What does a defect in neural crest migration result in?
defects in development of the heart, ascending aorta and pulmonary trunk
102
What is the second most common casue of congenital cardiac anomalies ?
DiGeorge (second to Down's)
103
What is the usual inheritnace of DiGeorge syndrome?
usually denovo but can be AD
104
What are the features of DiGeorge syndrome?
```
Cardiac defects
Abnormal facies
Thymus hypoplasia
Cleft palate
Hypocalcaemia (parathyroid hypoplasia)
```
105
How long does the serological response to HIV take ?
4-8 weeks
106
What is AIDS?
dramatic increase in viral load with breakdown of host defences and appearance of serious opportunistic infections, secondary neoplasma and neurologica disease
107
What are the specific anti-retroviral investigations for newly diagnosed HIV?
CD4 coutn; HIV viral load; serological tests for toxoplasma; CMV; tuberculin skin test; ECG; CXR
108
When should prophylactic co-trimoxazole be started?
CD4 count <200
109
When should mycoplasma prophylaxis with azitrhomycin be used?
CD4 <50
110
What vaccines should all HIV patietns recieve?
strep; influenza and hepB
111
What type of hypersensitivity with Rheumatic fever?
type II
112
Why is there a delay in presentation of cardiac symptoms following rheumatic fever?
continued immunologic damage and fibrosis progresses over years to cause deformity of the heart valves
113
What are the pathognomic histologic features of Rheumatic fever?
Aschoff bodies - granulomas with giant cells (macrophages)-- which can become activated = Anitschkows cells
114
what are the complications of rheumatic heart disease?
cardiac failure; AF and VTE; infective endocarditis
115
What is the common mechanism of death in SCD?
lethal arrhythmia triggered by irritability of the myocardium
116
What is sudden cardiac death defiend as?
death within 1 hour of onset of cardiac sypmtosm
117
What is Dresslers syndrome?
recurrent pericarditis; pleural effusions; fever; anaemia; and increased ESR 1-3 weeks psot MI
118
What is hte treatment for pericarditis?
NSAIDs
119
What is Kussmauls sign?
JVP rises during inspiration
120
What are the causes of mitral regurgitation following an MI?
papillary muscle dysfunction or papillary/choradal muscle rupture secondary to ischaemia
121
How does VSD present?
pansystolic murmur, increase JVP; cardiac failure
122
What would persisten ST elevation signify 4-6 weeks post-MI?
left ventricular aneurysm
123
What is the definition of an aneurysm?
abnormal permanent focal dilatation of all layers of a blood vessel
124
What conditions are associated with thoracic aneurysms?
Marfans; late stage syphilis
125
What condition is assocaited with coronary artery aneurysms?
Kawasaki's
126
What is a false aneurysm?
contained collections of blood immediately outside a vessel but where there is still direct comunication thorugh a defect in the vessel wall
127
What condition is assocaited with panacinar distribution emphysema?
alpha-1 antitrypsin deficiency
128
What is cor pulmonale?
pure right sided heart failure
129
What are the complications of COPD which can result in sudden death?
respiratory failure; cor pulmonale; pneumothorax
130
What do red cell casts mean?
GN or vasculitis
131
what do white cell casts mean?
acute interstitial nephritis
132
What do fatty casts mean?
nephrotic syndrome; minimal change disease
133
What do muddy casts mean?
acute tubular necrosis
134
What do renal tubular epithelial cell casts mean?
damage to the tubules- eg renal tubular necoriss
135
What is the penumbra?
area at the periphery of an infarct
136
What are the main macroscopic abnormalities in Alzhemiers?
widening of gyris; atrophy of sulci neurofibrillary tangles; senile plaques and amyoid angiopathy
137
What is a pancreatic gastrinoma associatedi wth peptic ulceration known as ?
Zollinger-Ellison syndrome
138
How does H.pylori survive the acidic stomach environment?
produces urease- which splits urea into CO2 and ammonia which neutralises smtomach acid
139
Why are U&Es done in a upper GI bleed?
increase urea out of proportion to creatinine suggests large blood meal
140
What type of ulcer is at highest risk of rebleeding?
psoterior duodenal ulcer as nearest to gastroduodenal artery
141
What are the alarm symptoms associated with dyspepsia?
anaemia; loss of weight; anorex;a recent onset/progressive; malaena/haematemesis; swallowing difficulty
142
What are typical tirgger foods with peptic ulceration?
coffee; chocolate; tomatoes; fatty or spicy foods
143
What is the treatment for H.pylori infection?
PPI and 7 days of amoxicillin and clarithromycin /metronidazole
144
What is the treatment for H.pylori infection is penicillin allergic?
PPI and 7 days of clarithromycin and metronidazole
145
What conditions are associated with gallstones?
haemolytic disorders; pregnancy; parenteral nutrition; rapid weight loss; loss of bile salts due to terminal ileal resection
146
What is Murphy's sign?
sudden cessation of inspiration while hand is placed on right subcostal area
147
What is a gallstone ileus?
gallstoen erosed through the GB into the duodenum. which obstructs the terminal ileum
148
What is Mirrizi's syndrome?
obstructive jaundice from common bile duct compression by a gallsteon impacted in the cystic duct
149
What HLA is assocaited with coeliac disease?
HLA DQ2 and DQ8
150
What is seen on biopsy with coeliac disease?
subtotal villous atrophy; increased intra-epithelial WBCs and crypt hyperplasia
151
What are the complications of coeliac disease?
anaemia; dermatitis herpetiformis; osetoporosis; hyposplenism; GI T-cell lymphoma; increased malignany- lymphoma; gastric; oesophageal; colorectal and neuropathies
152
What is the function of apo-B100?
binds to LDL receptors allowing uptake of VLDLs; IDLs and LDLs
153
Why would thiazides be given to a patient with oseoporosis instead of CCBs??
increase calcium reabsorption
154
Why is ability to abduct the thumb preserved in carpal tunnel syndrome?
action of abductor pollicis longus (radial nerve)- although it is weakened
155
What is seen in central cord syndrome?
bilateral weakness- upper more than lower and proximal more than distal
156
What is seen classically with cervical central cord syndrome?
cape distribtuion
157
When does papillary thyroid cancer present?
3rd and 4th decades in women
158
What is the CD4 count aim?
>350
159
Why is there an increased ferritin in anaemia of chornic disease?
ferritin is a serum reactant protein
160
What type of zoster is considered AIDS defining?
multidermatomal
161
Is Felty's syndrome assocaited with leukaemia?
No
162
What is HTLV-1 virus associated with?
adult T-cell leukaemia
163
What malignancy is assocaited wiht polycythaemia vera?
AML
164
What is benzene exposure associated with?
AML
165
Which type of leukaemia is assocaited with PAS positive blast cells?
ALL
166
What does cytoplasmic grnaulation in blasts indicate?
degree of myeloid differentiation-AML
167
What type of leukaemia is associated with DIC?
AML
168
What is the most common type of ALL?
pre-B phenotype
169
What is a saddle PE?
at the bifurcation of the pulmonary veins
170
What is the appearnce of glioblastoma multiforme on CT and why?
butterfuly-crosses over the corpus callosum
171
What other name is glioblastoma multiforme give?
type 4 astrocytoma
172
Waht is the appearnce of hte meningioma?
grows from teh periphery of the brain
173
What conditions are associated with meningiomas?
NFII;
174
What condition is situs inversus assocaited with?
kartageners syndrome- pulmonary infection;s difficulty conceiving
175
What is horseshoe kidney assocaited with?
45XO
176
If there is white out and shift of structures toward the side of opacification what does this mean?
atelectasis
177
What is a jones #?
fracutre of the base of the 5th metatarsal
