Term
Class
MOA
Bacteriocidal or bacteriostatic ?
- Amoxicillin |
|
Definition
Penicillin
B-lactam - inibit cell wall synthesis
Bacteriocidal |
|
|
Term
Class
MOA
Bacteriocidal or bacteriostatic ?
- ciprofloxacin |
|
Definition
Flouroquinilone
inhibits DNA replication via inhibiting activity of DNA GYRASE
Bacteriostatic |
|
|
Term
Class
MOA
Bacteriocidal or bacteriostatic ?
- gentamicin |
|
Definition
Aminoglycoside
Inhibits protein synthesis via blocking 30S subunit
Bacteriocidal |
|
|
Term
Class
MOA
Bacteriocidal or bacteriostatic ?
- teicoplanin/vancomycin |
|
Definition
Glycopeptide abx
disrupt cell wall synthesis via binding to pentapeptide chains and inhibits transglycosylation reaction
bacteriocidal |
|
|
Term
Class
MOA
Bacteriocidal or bacteriostatic ?
- cephalaxin |
|
Definition
cephlasporins
B-lactam - inhibit cell wall synthesis
bacteriocidal |
|
|
Term
Class
MOA
Bacteriocidal or bacteriostatic ?
- clarithromycin |
|
Definition
macrolide
inhibit protein synthesis via 50S ribosome subunit
bacteriostatic |
|
|
Term
Class
MOA
Bacteriocidal or bacteriostatic ?
- trimethoprim |
|
Definition
Sulfonamide
Inhibits bacterial DNA synthesis via preventing folate synthesis
Bacteriostatic |
|
|
Term
Chloramphenicol
- MOA
- why do neonates get grey baby syndrome |
|
Definition
Blocks action of peptidyl transferase and prevents peptide bond synthesis - i.e. inhibits protein synthesis
Grey baby syndrome a result of UGT2B7 enzyme for glucoronidation to water soluble molecule for excretion |
|
|
Term
Codeine
- metabolised by what ? Why is it not recommended for children <12y |
|
Definition
| Metabolised by CYP2D6 enzyme to its active form - highly polymorphic i.e. some people are poor metabolisers, some are ultra-metabolisers - therefore inter individual variability++ |
|
|
Term
What type of receptors are adrenergic receptors ?
How do they work ? |
|
Definition
G protein coupled receptor
Upon activation, the alpha subunit disassociates from delta+gamma subunits, and associates with adenlyate cyclase converts ATP --> cAMP which acts as secondary messenger |
|
|
Term
Opioid receptors are ....
What is the end cellular effect of opioids binding their receptors ? |
|
Definition
G Protein Coupled Receptors
Upon activation alpha subunit disssociates and eventually- cAMP is reduced, Ca channels close and K+ channels open - resulting in hyperpolarised cell and reduced neurotransmitter release |
|
|
Term
| What is the active metabolite of morphine called ? |
|
Definition
| M6G - morphine-6-glucoronide |
|
|
Term
| The enzyme that metabolises morphine to its active form M6G |
|
Definition
|
|
Term
| why are neonates less likely to develop hepatotoxicity from paracetamol ? |
|
Definition
Paracetamol metabolised in three ways
1) glucoronidation
2) sulfide conjugation
3) N-hydroxylation --> forms NAQPI (toxic metablite)
neonates have less (1) and (3) activity, and increased sulfide conjugation to compensate - thus produce less NAQPI which is the toxic bit |
|
|
Term
| Toxic metabolite of paracetamol is ______. It is cleared by ___________ with ______________. This is depleted in overdose. NAC works by increasing levels of ___________ so that excess _______ can be ___________ and excreted |
|
Definition
| Toxic metabolite of paracetamol is NAQPI. It is cleared by conjugation with glutathione. This is depleted in overdose. NAC works by increasing levels of glutathione so that excess NAQPI can be conjugated and excreted |
|
|
Term
|
Definition
| oxidation/hydrolysis/reduction - usually with cytochrome enzymes etc |
|
|
Term
|
Definition
Conjugation
- glucoronidation
- sulfalation
- methylation
- acetylation |
|
|
Term
| Two times when ceftriaxone should not be used |
|
Definition
1. with calcium containing infusions
2. jaundice - causes biliary sludging |
|
|
Term
| How does paracetamol reduce fever |
|
Definition
| Reduction in formation of PGE2 (via COX inhibition of arachadonic acid pathway). The reduced PGE2 lowers hypothalamic set point |
|
|
Term
| How do shock and hypothermia affect metabolism of drugs |
|
Definition
|
|
Term
| WHO definition of adverse drug reaction |
|
Definition
| noxious and unintended response at a normal dose |
|
|
Term
| How might CF affect clearance of some drugs |
|
Definition
| clearance of some drugs is increased therefore higher doses may be required for theraputic effect |
|
|
Term
| Name a drug that is excreted by renal tubules |
|
Definition
|
|
Term
|
Definition
| Chloramphenical (due to decreased glucoronidation) |
|
|
Term
Inhibitor/inducer:
- MACROLIDES |
|
Definition
|
|
Term
|
Definition
|
|
Term
Inhibitor/inducer:
- KETOCONAZOLE |
|
Definition
|
|
Term
Inhibitor/inducer:
- PHENOBARBITONE |
|
Definition
|
|
Term
Inhibitor/inducer:
- CARBEMAZEPINE |
|
Definition
|
|
Term
|
Definition
| Associated with vancomycin - is a flushing of upper body and face - caused by non specific mast cell degranulation - solution is to slow the infusion |
|
|
Term
Class
MOA
Bacteriocidal or bacteriostatic ?
- Tobramycin |
|
Definition
Aminoglycoside
Inhibits protein synthesis via blocking 30S subunit
Bacteriocidal |
|
|
Term
| When is PCP prophalyxis given to children with HIV ? |
|
Definition
1) Baby HIV+ PCR - start PCP prophalyxis age 4 weeks (until 1 year of age, or until subsequent testing excludes)
2) when CD4 count low enough to necessitate it in HIV+ child >1y |
|
|
Term
| Which children with HIV will definately get ART therapy |
|
Definition
everyone <1y
everyone with significant disease
Co-infection with TB/hep C
Children with dropping CD4 count (before its too low! - 350 is cut off for low, should consider ART if <500)
consider if high viral load/sexually active/age1-3/hepB coinfection |
|
|
Term
| typical antiretroviral regime |
|
Definition
at least three drugs
2x NRTI backbone
Plus PI (protease inhibitor) or NNRTI (non-neoclioside reverse transcriptase inhibitor) |
|
|
Term
HIV
- structure
- how does it work (simply) - i.e. how does it replicate etc
- how does NRTI work ?
- how does NNRTI work ? |
|
Definition
single stranded RNA virus - 2 VIRAL RNA strands in single capsid
Uses reverse transcriptase to convert ssRNA --> ssDNA by joining up neuclosides (complementary base pairings)
- this is the target of NRTI's - the reverse transcriptase
enzyme takes the NRTI and tries to insert it into the chain
thinking it is a nucleoside (its actually a neucleoside
analogue)- as a consequence, no more nucleosides can
be added to the chain ! formation of ssDNA is halted -
AKA CHAIN TERMINATION
- NNRTI works by blocking viral RT function - hence enzyme
is not even able to start converting ssRNA to ssDNA
after HIV makes ssDNA - it undergoes SECOND ROUND OF REVERSE TRANSCRIPTASE to make dsDNA, the dsDNA then goes into nucleus via use of INTEGRASE ENZYME |
|
|
Term
|
Definition
HIV uses reverse transcriptase to convert its ssRNA --> ssDNA by joining up neuclosides (complementary base pairings)
- this is the target of NRTI's - the reverse transcriptase
enzyme takes the NRTI and tries to insert it into the chain
thinking it is a nucleoside (its actually a neucleoside
analogue)- as a consequence, no more nucleosides can
be added to the chain ! formation of ssDNA is halted -
AKA CHAIN TERMINATION |
|
|
Term
which cells does HIV infect and via which receptor
what else is involved in it getting in ??? |
|
Definition
T-Helper cells via CD4 receptor
CCR5 co-receptor |
|
|
Term
| role of integrase enzyme in HIV |
|
Definition
after reverse transcriptase ssRNA --> ssDNA
then second reverse transcriptase ssDNA --> dsDNA
integrase then helps dsDNA insert into host DNA in nucleus |
|
|
Term
| what enzyme makes messenger RNA (mRNA) from DNA |
|
Definition
|
|
Term
| after dsDNA is made in HIV infection and taken to nucleus and integrated with help of integrase enzyme ... what happens next - what is the drug taregt here? |
|
Definition
translation and protein synthesis occurs at ribosomes - making viral proteins that will eventually form the mature virons with 2 x single strands of viral RNA and enzymes (RT and integrase) necessary for infection of cell)
Enzyme protease chops up these proteins to allow for assembly of virons - PROTEASE INHIBITORS TARGET THIS |
|
|
Term
|
Definition
Zidovudine
ABC & 3TC
(3TC = lamivudine
ABC = abacavir) |
|
|
Term
| Protease inhibitor example |
|
Definition
| LPV/r = lopinavir/ritonavir |
|
|
Term
|
Definition
|
|
Term
| which conducts heat better ? Wet or Dry? |
|
Definition
| Wet - hence wet heat burns worse than dry heat burns |
|
|
Term
|
Definition
| limited to epidermis only e.g. sunburn |
|
|
Term
| Define superficial partial thickness burn |
|
Definition
affects epidermis and up to 1/3rd of dermis - this is the most painful type of burn.
Fluid leaks from damaged capillaries = blister formation
This type of burn often appears pink and wet |
|
|
Term
| Define deep partial thickness burn |
|
Definition
all of epidermis, most of dermis
white and dry burn - intact but reduced sensation |
|
|
Term
full thickness burn - define
and subdermal ? |
|
Definition
all of epidermis and all of dermis - these are painless
subdermal involves fat/muscle/ tendon/bone |
|
|
Term
RE: Burns
Zone of hyperaemia
Zone of stasis
Zone of coagulation |
|
Definition
Zone of hyperaemia - warm and red
Zone of stasis - fluids leak into interstitial space
Zone of coagulation - microcirculation obstructed, hence humoral immunity cant get here - part most at risk of infection |
|
|
Term
| What must always be used with a LABA ? Why ? |
|
Definition
| LABA must always be in combination with steroid - increased risk of sudden death in pts taking LABA w/o steroid |
|
|
Term
| When does RSV infection become most common and why ? |
|
Definition
> 2 months of age
Decrease in passive immunity at this stage |
|
|
Term
| How frequently is adrenalin given in cardiac arrest ? |
|
Definition
| Every 4 minutes (i.e. every 2nd cycle - rhythm check is done every 2 minutes) |
|
|
Term
| Toxoplasma vs CMV on brain scan |
|
Definition
Toxoplasma diffuse calcification
CMV periventricular calcification |
|
|
Term
|
Definition
| basal, non insulin mediated uptake of glucose into cells (esp CNS - remember GLUT-1 deficiency) |
|
|
Term
|
Definition
| insulin mediated uptake of glucose into cells |
|
|
Term
|
Definition
| glucose sensing in beta cells (non insulin mediated) |
|
|
Term
Best test for Chedak-Higadashi syndrome
How might they present ? |
|
Definition
NK function - is essentially a problem with phagocytosis, hence get lots of pyogenic infections
Fair hair, ataxia (from peripheral neuropathy), pyogenic infections |
|
|
Term
|
Definition
| a fungal abscess - treat with systemic antifungal |
|
|
Term
|
Definition
1. Wound debridement
2. Tetanus toxoid
3. Penicillin
4. Consider ITU |
|
|
Term
Lyme disease
- what pathogen ?
- what vector ? |
|
Definition
Borrelia Burgdorferi
Ixodes tick vector |
|
|
Term
| Mechanism of action of Nystatin and Amphotericetan B |
|
Definition
Are POLYENES
Bind to ergosterol in fungal cell wall and cause cell lysis |
|
|
Term
| Mechanism of action of azoles |
|
Definition
| block synthesis of ergesterol |
|
|
Term
| Drug of choice to treat CMV ? |
|
Definition
|
|
Term
|
Definition
| is a prodrug - requires activation by viral thymidine kinase enzyme - prevents DNA assembly |
|
|
Term
| What is meant by the term "THERAPUTIC INDEX?" |
|
Definition
likelihood of causing harm
The higher the number the less likely it is going to cause toxicity |
|
|
Term
What is a protozoa ?
Give some examples |
|
Definition
Protozoa is a unicellular organism with a plasma cell membrane
e.g. Toxoplasma
Flagellites - guardia & tricominosis
Amoebia - e.g. enteromoeba histolytica |
|
|
Term
| Difference between gram negative and gram positive organisms |
|
Definition
Gram positive have thick peptidoglycan cell wall with no outer membrane - they appear blue/purple on gram stain because they retain the dye
Gram negative bacteria have a thin peptidoglycan cell wall WITH AN OUTER MEMBRANE |
|
|
Term
| Define an encapsulated organism and give three examples |
|
Definition
surrounded by polysaccaride capsule - require a spleen to clear
e.g. strep pneumonae, neiserria, klebsiella, e-coli, group B strep |
|
|
Term
| Why is norovirus more stable than RSV ? |
|
Definition
RSV has a lipoprotein envelope which makes it vulnerable to detergents, alcohols, and dry environments
Norovirus does not have such an envelope, this makes it more stable |
|
|
Term
Which layer is affected in EB simplex ?
Inheritance ? |
|
Definition
Epidermal (basal)
AD inheritance
lesions heal without scars |
|
|
Term
Junctional EB - where is problem ?
Inheritance ? |
|
Definition
Dermo-epidermal junction
AR inheritance |
|
|
Term
Dystrophic EB - where is the problem ?
Inheritance |
|
Definition
| Blister formation in upper dermis (Papillary dermis) |
|
|
Term
| Name 4 causes of blisters that affect the epidermis |
|
Definition
1. Bullous impetigo
2. Staph Scalded Skin
3. Pemphigus
4. EB simplex |
|
|
Term
| Atopic dermitits is associated with defect of which gene ? |
|
Definition
|
|
Term
| Where does vernix come from ? |
|
Definition
|
|
Term
Where do melanocytes come from (embryologically speaking) and where do they go to ?
What happens if there is failure in this migration ?
what occurs if theres a tyrosine problem in melanocytes themselves ? |
|
Definition
Migrate from neural crest cells to epidermis
Failure to get to epidermis - means they are in the dermis, amd you can have dermal melanocytosis - i.e. mongolian blue spot
tyrosine problem in melanocytes = albinism |
|
|
Term
| What cells are antigen presenting in the skin ? which layer of skin do they sit in ? |
|
Definition
| langerhans cells - in epidermis |
|
|
Term
| Name the two layres of dermis |
|
Definition
1. Papillary
2. Reticular - deepest |
|
|
Term
| At what age gestation is skin keritinised ? |
|
Definition
|
|
Term
How many cilia on a ciliated epithelial cell
Frequency of beats
When does primary ciliary diskinesia first cause problems |
|
Definition
200 per cell
15-20Hz
problems at birth - clila play an important role in clearing lungs of fluid at birth |
|
|
Term
| How could you screen for primary ciliary dyskinesia ? |
|
Definition
| Measure nasal nitrous oxide - this will be low in PCD |
|
|
Term
DDX of retinal and subdural haemorrhages that can mimic shaken baby syndrome
what is measured on NBBS to screen for this condition |
|
Definition
glutaric aciduria
glutarylcarnitine (C5-DC) on NBBS |
|
|
Term
| Tropiclamide/cyclopentolate/atropine bring about pupilary dilation via what receptor |
|
Definition
|
|
Term
| What growth factor is important in vasculariation of retina in normal infant, and what drives it ? |
|
Definition
|
|
Term
Pathyphysiology of ROP development
- two phases |
|
Definition
1st phase - HYPEROXIC phase - decreases VEGF-A (which is necessary for normal retinal vascularisation) - hence halts progression of vascular tissue on developing retina
2nd phase - hyperoxic phase - the unvascularised anterior retina becomes increasingly hypoxic THEN VEGF is upregulated = neovascularisation = abnormal vascular network |
|
|
Term
|
Definition
|
|
Term
|
Definition
SV2A BINDER
Binds to a synaptic vesicle glycoprotein, SV2A, and inhibits presynaptic calcium channels reducing neurotransmitter release and acting as a neuromodulator. |
|
|
Term
|
Definition
|
|
Term
|
Definition
|
|
Term
|
Definition
|
|
Term
|
Definition
|
|
Term
|
Definition
Multiple channels:
- Na+ blocker
- Ca+ blocker
- some action at GABA receptors |
|
|
Term
|
Definition
|
|
Term
|
Definition
Na & Ca channel blocker
Carbonic anhydrase inhibitor
NMDAR antagonist
Causes renal stones |
|
|
Term
| Why would you want to do an MRI scan with spectroscopy ?! |
|
Definition
|
|
Term
|
Definition
Dominant CNS cell
Can transform into neurons
form BBB |
|
|
Term
|
Definition
Support and insulate axon
Basically do what schwann cells do in PNS, but in CNS |
|
|
Term
|
Definition
produce myelin
Sub type called ependymal cells - produce and regulate CSF |
|
|
Term
| The blood brain barrier favours which type of molecules ? |
|
Definition
| Lipid soluble molecules e.g. unconjugated biliruben, ammonia |
|
|
Term
| How does glutamine cross BBB |
|
Definition
| Facilitated transport (passive process) |
|
|
Term
| Most common nerve roots involved in an Erb's palsy ? |
|
Definition
|
|
Term
| Pattern of complement in post strep GN ? |
|
Definition
|
|
Term
| Red cell casts are seen in .. |
|
Definition
|
|
Term
| hyaline casts are seen in .... |
|
Definition
| can be normal, especially after exercise |
|
|
Term
| tubular casts are seen in ... |
|
Definition
|
|
Term
| coarse granular casts are seen in ... |
|
Definition
| glomerular/tubular disease |
|
|
Term
Features of Fanconi Syndrome
List some causes |
|
Definition
Disorder of proximal tubule function
Inherited or acquired
Loss of - glucose, phosphate, amino acids, sodium, potassium, bicarbonate and water
Presented with FTT/polyuria/polydipsia, rickets, hypokalaemia
Causes:
- cystinosis
- lowe's syndrome
- drugs |
|
|
Term
Defect in chloride reabsorption - leads to JGA hyperplasia and raised renin+aldosterone levels - but absence of hypertension .... what am I ? What is my biochemical picture ??
What might you get as a complication?
What is the differential diagnosis of this biochemical picture? |
|
Definition
Bartters Syndrome
Metabolic alkalosis, hypokalaemia, hypochloraemia
Might get renal stones - calcium stones, 2' to hypercalcuria
DDX of hypochloraemic, hypokalaemic metabolic alkalosis
- vomiting++, diuretic abuse, chloridorrhoea |
|
|
Term
| Gold standard investigation for investigation of VUR |
|
Definition
|
|
Term
| Test for renal parenchymal scaring |
|
Definition
|
|
Term
| Type of renal scan useful for demonstrating obstruction |
|
Definition
|
|
Term
| Difference between DMSA and MAG3 |
|
Definition
| MAG 3 is dynamic, DMSA is static |
|
|
Term
| Which bit of kidney is responsible for secretion of H+ ? |
|
Definition
|
|
Term
| Name the two main buffers of urine |
|
Definition
|
|
Term
| Type 4 renal tubular acidosis |
|
Definition
| impaired formation of ammonia (is a buffer for pH) |
|
|
Term
T2 RTA is AKA
What is the problem |
|
Definition
proximal RTA
Failure to reabsorb HCO3-
usually part of the fanconi syndrome |
|
|
Term
T1 RTA is AKA
What is the problem |
|
Definition
Distal RTA
Failure to excrete H+ |
|
|
Term
| Renal agenesis occurs because of what embryological problem ? |
|
Definition
|
|
Term
| Multicystic dysplastic kidney occurs because of what embryological problem ? |
|
Definition
| failure of secretory and connecting bits to communicate --> cyst formation |
|
|
Term
| Multiple kidneys occur because of what embryological problem ? |
|
Definition
| early splitting of ureteric bud |
|
|
Term
| Pelvic kidney occurs because of what embryological problem ? |
|
Definition
| Failure to ascend to correct position due to obstruction by sickle shaped fold of peritoneum |
|
|
Term
| Horseshoe kidney occurs because of what embryological problem ? |
|
Definition
| arrest of ascent due to position of inferior mesenteric artery |
|
|
Term
| Duplex kidney occurs because of what embryological problem ? |
|
Definition
| two seperate ureteric buds arising from single wolffian duct |
|
|
Term
| palpable liver, raised lactate ..... makes you think of ... |
|
Definition
|
|
Term
| H pylori eradication regime |
|
Definition
1 week
- omeprazole
- amoxicillin
- clarithromycin |
|
|
Term
Autosomal dominant condition
Pulmonary stenosis
conjugated hyperbilirubinaemia
what feature on ophthalmology exam ? |
|
Definition
Alagilles
posterior embryotoxin |
|
|
Term
What is the mode of inheritance of alpha-1-antitrypsin deficiency
What is the most severe genotype ? |
|
Definition
co-dominant - i.e. each allele is responsible for 50% of the protein production
PiZZ is most severe phenotype |
|
|
Term
What type of hyperbilirubinaemia is seen in criglar-nijar syndrome ?
What are the two types
What is a possible treatment for the second type ? |
|
Definition
Unconjugated hyperbilirubinaemia+++
type 1 - absent UDP-glucaranosyltransferase
type 2 - decreased UDP-g.....
In type 2 you can use phenobarb (an enzyme inducer) to induce CYP450 |
|
|
Term
| Embryologically, the liver arises from the ... |
|
Definition
|
|
Term
|
Definition
unconj bili --> conj bili
conj bili --> bile --> gut
bile in gut
---> reabsorbed as bile
---> stercobilinogen --> poo
---> urobilinogen ---> urine |
|
|
Term
|
Definition
| aminosaliscylate comprised of 5-ASA and a sulfa group - antiinflammatory |
|
|
Term
| When would you consider stool electrolytes |
|
Definition
| in chronic diarrhoea to see if secretory or osmotic |
|
|
Term
| faecal reducing substances ? |
|
Definition
| if high, suggest CHO malabsorption e.g. lactose intolerance |
|
|
Term
|
Definition
| H. Pylori - urease producing bacteria - high levels of H2 exhaled after ingestion of carbon14urea |
|
|
Term
| Coeliac disease is associated with which HLA types ? |
|
Definition
|
|
Term
| Composition of oral rehydration solution ? Why ? |
|
Definition
Na and glucose (75mmol of each)
the glucose is absorbed and the Na is co-transported - then the water follows. Without the glucose the Na wont be transported ! |
|
|
Term
| Pancreatic exocrine function is under control of which two hormones? |
|
Definition
1. secretin - produced in response to acidic chyme in duodenum
2. cholecystokinin |
|
|
Term
| Role of bile salts in fat absorption - overview of process |
|
Definition
Bile salts emulsify fats --> form myceles
Myceles are absorbed with ADE+K
Then re-converted to triglycerides and transported as chylomicrons at the other side ! |
|
|
Term
| Role of acinir cells in pancreas |
|
Definition
| Produce PRECURSOR pancreatic enzymes (to stop autodigestion), also secrete HCO3- to neutralise acid from stomach and help pancreatic enzymes work |
|
|
Term
| what enzyme is produced by the pancreas to digest CHO? |
|
Definition
|
|
Term
Epidemiology of pyloric stenosis
What is the carter effect ? |
|
Definition
Males > females
But offspring of affected female much more likely to be affected - this is carter effect. Affected females have a higher threshold for symptoms than affected males, thus for a female to be affected she must have higher genertic predisposition - thus if shes affected she also passes on higher predisposition |
|
|
Term
| Chief cells secrete what ? |
|
Definition
| Pepsinogen (the precursor to pepsin) |
|
|
Term
| Parietal cells secrete what ? |
|
Definition
HCL and intrinsic factor
(HCL + pepsinogen --> pepsin) |
|
|
Term
|
Definition
| gastrin - which in turn stimulated parietal and chief cells |
|
|
Term
| enterochromaffin cells produce what ? |
|
Definition
|
|
Term
|
Definition
|
|
Term
| What scan might you use to diagnose a meckels diverticulum ? |
|
Definition
|
|
Term
| What is a meckels diverticulum ? |
|
Definition
| remnant of omphalomesenteric duct - when this fails to close it can cause an umbilical fistula |
|
|
Term
| Why does hirschprungs cause bowel obstruction ? |
|
Definition
FUNCTIONAL bowel obstruction
absence of ganglionic cells in a variable segment of colon cause inability of bowel to relax
suspect if BNO >48h in newborn |
|
|
Term
| The gut, embryologically, is derived from which layer of cells |
|
Definition
|
|
Term
| Raised Succynalacetone is pathognomic of what ? |
|
Definition
|
|
Term
| Sulphites positive on urine dip |
|
Definition
MOCOD
Molybdenum cofactor deficiency - dreadful, irretractible seizures in neonatal period |
|
|
Term
|
Definition
| exadurated startle reflex |
|
|
Term
| CSF plasma glucose ratio in GLUT-1 deficiency |
|
Definition
|
|
Term
| Specialised feed for CMPA |
|
Definition
| extensively hydrolysed formula - nutramigen |
|
|
Term
| Neonate with PKU - appropriate formula feed ? |
|
Definition
|
|
Term
| Suitable formula for 3yo child with multiple food allergies on a restricted diet |
|
Definition
| Neocate advanced - amino acid formula |
|
|
Term
|
Definition
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Term
| High energy formula for infant <1y |
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Definition
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Term
| High energy formula for child > 1y |
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Definition
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Term
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Definition
lactose free
hydrolysed caesin
progestimil |
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Term
| name a protease inhibitor |
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Definition
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Term
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Definition
Emtricitabine
Zidovudine
ABC - Abacavir |
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Term
| Postnatal diuresis is __________ in neonates with RDS |
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Definition
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Term
| What characteristic of diarrhoea may help differentiate secretory from osmotic ? |
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Definition
| secretory persists when child is NBM, osmotic stops/slows |
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Term
| Haem --> bilirubin, takes place in which cells ? |
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Definition
| Kupffer cells - are specialised macrophages |
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Term
| Melanosis coli on colonoscopy might make you suspect ... |
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Definition
| ... laxitive abuse (esp senna) |
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Term
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Definition
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Term
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Definition
Hodgkins slower growing, painless lymphadenopathy - 80% neck, reed sternberg cells
NHL - rapidly enlarging, more unwell than hodgkins - high risk of tumour lysis
NHL more common than HL |
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Term
Where do dorsal columns decussate ?
What sensations? |
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Definition
midbrain
light touch and vibration and propioception |
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Term
| pain and temperature - what spinal tracts ? and where do they decussate |
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Definition
| cross at the spinal level, spinothalamic tracts |
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Term
| A urea cycle defect that presents in neonate |
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Definition
NAGS deficiency
(OTC presents later) |
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Term
| How is ammonia normally cleared - what is formed in brain if this does not happen - and whats the problem ? |
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Definition
normally converted to urea --> excreted renally
if urea cycle defect - NH3 is converted into glutamine, which is neurotoxic (glutamine is an ammonia scavenger) |
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Term
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Definition
| calcitriol ! i.e. active vitamin D |
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Term
| How is vitamin D synthesised in skin ? |
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Definition
| cholesterol -- UVB --> cholecalciferol (then goes to be hydroxylated in liver and kidney before being 'active' vitamin D |
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Term
| What proportion of vitamin D is synthesised by skin ? |
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Definition
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Term
Which type of histamine receptors are reponsible for:
- tachycardia
- flushing
- hypotension
- pruritis
- Bronchospasm |
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Definition
- tachycardia H1
- flushing H2
- hypotension H2
- pruritis H1
- Bronchospasm H1 |
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Term
| What is the role of MRD assessment in ALL treatment ? |
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Definition
| MRD = minimal residual disease, (measured via PCR) - if present after initial induction, poorer prognosis |
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Term
Prognostication of ALL
- what features indicate poorer prognosis |
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Definition
age <1, >10
MRD presence
high WCC at presentation
**list incomplete** |
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Term
| What happens in G1 - name a drug that targets this |
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Definition
Gap phase - cell preparing for S phase - i.e. in G1 the components necessary for DNA synthesis are synthesised
Drug example Aspiriginase -degrades amino acid L-Aspirigine |
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Term
S phase of cell cycle
- what happens ?
- name a drug that targets this phase |
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Definition
DNA synthesis !
Methotrexate
Mercaptopurine |
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Term
G2 phase of cell cycle
- what happens? |
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Definition
Perparation for mitosis
Follows S phase - preceeds mitosis |
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Term
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Definition
| G1, S, G2 (i.e. every part of cell cycle other than mitosis) - and in non dividing cells G0 |
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Term
| Name a drug that targets cell during mitosis |
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Definition
| Vincristine - blocks microtubule assembly, hence prevents formation of mitotic spindle required for chromosome division |
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Term
| What is the composition of surfactant |
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Definition
Complex suspen sion of phospholipids (85%) and proteins
DPPC is one of the phospholipid types - which is suited to forming a stable mono layer generating the lower surface tension which prevents alveolar collapse at end of expiration
SP-B (surfactant protein B) & SP-C (surfactant protein C) are important proteins that maintain the low surface tension after dynamic compression (you can have deficiency of these proteins, SPB deficiency presents v early and is lethal, SPC deficiency presents much later with interstitial lung disease)
SPA and SP-D are less important than B+C in maintaining surface tension, but play a role in immune defence and recycling of surfactant |
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Term
| What is a porencephalic cyst ? |
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Definition
| cyst that develops following a grade IV IVH |
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Term
| What, seen on imagng of neonate, confers a high risk of development of CP ? |
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Definition
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Term
| What is the most common type of OA/TOF ? |
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Definition
| oesophageal atresia with distal tracheosophageal fistula |
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Term
| Expected arterial O2 tension for neonate |
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Definition
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Term
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Definition
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Term
| What volatile marker could be measured as a marker of bilirubin production ? |
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Definition
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Term
a one off big sentinel hypoxic event is most likely to cause injury to which brain area ??
In contrast, chronic partial hypoxia results in damage to which area? |
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Definition
Sentinel event - basal ganglia and thalamus + PLIC.
Chronic partial hypoxia - watershed areas
PLIC damage is best predictor for inability to walk at age 2 years |
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