Term
| what is USS good for in renal tract abnormalities? |
|
Definition
for anatomical assessment
to see dilatations, stones and nephrocalcinosis
cannot detect all real scars |
|
|
Term
DMSA scan
dimercaptosuccinic acid
what is it used for? |
|
Definition
static scan of renal cortex
good for functional defects such as scars
very sensitive so need to wait at least 2 months after UTI to avoid false positives |
|
|
Term
micturating cystourethrogram
MCUG
what is it used for? |
|
Definition
detects vesicoureteric reflux and urethral obstruction
contrast is put into bladder via catheter |
|
|
Term
|
Definition
| dynamic scan. it measures drainage and used to identify vesicoureteric reflux in children who are old enough to cooperate. |
|
|
Term
| from DMSA and MAG3 which scan is dynamic and which is static? |
|
Definition
DMSA is static
MAG3 is dynamic. |
|
|
Term
| features of potters syndrome |
|
Definition
renal a genesis leading to reduced fetal urine.
this causes oligohydramnios and feral compressions
characteristic: low set ears, beaked nose, downward slant to eyes
pulmonary hypoplasia causing respiratory failure
limb deformities: talipes |
|
|
Term
|
Definition
liver and pancreatic cysts
cerebral aneurysms
mitral valve prolapse |
|
|
Term
| what syndrome is characterised by absence or severe deficiency of the anterior abdominal wall? |
|
Definition
absent musculature syndrome AKA prune belly syndrome
large bladder, dilated ureters and cryptorchidism |
|
|
Term
| bilateral hydronephrosis in a new born male infants warrants USS to rule out what? |
|
Definition
posterior urethral valves.
this will need intervention such as cystoscopic ablation |
|
|
Term
| ways to obtain urine sample in a child in nappies |
|
Definition
clean catch when nappy is removed is recommend
absorbant sponge placed in nappies
urethral catheter if urgent
suprapubic aspiration in severely ill children needing urgent diagnosis and rx. |
|
|
Term
| how to obtain a urine sample in older children? |
|
Definition
|
|
Term
| what volume of bacteria suggest a 90% probability of a UTI? |
|
Definition
|
|
Term
| why is proteus infection more commonly diagnosed in boys than girls? |
|
Definition
because of its presence beneath the prepuce.
proteus infection predisposes to phosphate stones. |
|
|
Term
| common organisms causing UTI |
|
Definition
ecoli
klebsiella
Proteus
Psuedomonas
Strep feacalis |
|
|
Term
| in an infant with psuedomonas UTI what should we investigate? |
|
Definition
| ix for a structural abnormality affecting urine drainage. |
|
|
Term
|
Definition
high fluid intake
regular voiding and double voiding
prevent/treat constipation
good personal hygiene
lactobacillus acidophillus |
|
|
Term
| features of nephrotic syndrome |
|
Definition
periorbital oedema
scrotal,vulval oedema and ankle oedema
ascites
breathlessness due to pleural effusions and abdominal distension.
diagnosis: heavy proteinuria and low plasma albumin |
|
|
Term
describe steroid sensitive nephrotic syndrome.
these do not progress to renal failure, |
|
Definition
aged between 1-10 years
no macro heamaturia
normal BP
normal complement
normal renal function |
|
|
Term
| in steroid sensitive minimal change disease what is seen under electron microscopy ? |
|
Definition
|
|
Term
| complications of nephrotic syndrome |
|
Definition
hypovolemia
thrombosis
infection: pneumococcal
hypercholesterolemia |
|
|
Term
| mx of steroid resistant nephrotic syndrome |
|
Definition
refer to peadiatric nephrologists
oedema mx: diuretic, salt restriction, ACE-i and sometimes NSAIDs |
|
|
Term
| list some steroid resistant nephrotic syndromes |
|
Definition
focal segmental
mesangiocapillary
membranous nephropathy |
|
|
Term
| prognosis of focal segmental glomeruloscelrosis? |
|
Definition
30% ESRF in 5 years
20% respond to cyclophosphamide, ciclosporin,tacrolimus or rituximab
recurrence post transplant is common |
|
|
Term
| which steroid resistant nephrotic syndrome is associated with hepatitis B? |
|
Definition
mebranous nephropathy
it may also precede SLE |
|
|
Term
| how to define heamaturia using microscopy? |
|
Definition
| >10 RBC per high power field |
|
|
Term
| features of IgA nephropathy |
|
Definition
macroscopic heamturia
in association with URTI
prognosis in children better than that for adults |
|
|
Term
|
Definition
X linked recessive
ESRF by adult life in males
associated with nerve deafness and ocular defects
Mother may have heavy heamaturia so test mother's urine. |
|
|
Term
features of vasculitis.
if ANCA positive what are we thinking?
if presence of renal aneurysms what are you thinking?
what is the rx? |
|
Definition
ANCA positive = wegeners
renal aneurysms = Polyarteritis nodosa
rx: steroids, plasma exchange and IV cyclophosphamide. this may need to be continued for months. |
|
|
Term
|
Definition
mainly presents in adolescent girls
antibodies to dsDNA
C3 and C4 levels are low particularly during active phases of disease
if heamaturia and proteinuria then do a renal biopsy.
rx: immunosuppression. |
|
|
Term
| causes of bilateral palpable kidneys |
|
Definition
AR PKD
AD PKD
tuberous sclerosis
renal vein thrombosis |
|
|
Term
what are the commonest types of renal stones in children?
what are they associated with? |
|
Definition
phosphate stones
associated with proteus infection
more common in boys as they are found under the prepuce |
|
|
Term
| fanconi syndrome. features that should make you suspect this include? |
|
Definition
polydipsia and polyuria
salt depletion and dehydration
hyperchloreamic metabolic acidosis
rickets
failure to thrive/poor growth |
|
|
Term
a child is obese, has a depressed nasal bridge and has 2nd, 4th and 5th short fingers.
what are you thinking? |
|
Definition
|
|
Term
| name the triad of haemolytic uremic syndrome |
|
Definition
Acute renal failure
hemolytic anemia
thrombocytopenia |
|
|
Term
| features of haemolytic uraemia syndrome |
|
Definition
triad: AKI, heamolytic anemia and thrombocytopenia
can be typical and atypical
typical HUS is secondary to GI infection from ECOLI 0157 strain or less often Shigella.
mx: supportive therapy including dialysis.
prognosis is good for typical HUS
atypical: no diarrhoea prodrome. can be familial with frequent relapses. high risk of HTN, chronic renal failure and high mortality. |
|
|
Term
| how would you prevent renal osteodystrophy? |
|
Definition
dietary phosphate restriction by limiting milk products
calcium carbonate to bind phosphate
activate vit D supplements |
|
|
Term
gap fill
the testes are formed from the ........ ridge on the posterior abdominal wall |
|
Definition
|
|
Term
| which mesenchymal tissue guides the descent of the testes towards the inguinal canal? |
|
Definition
the gubernaculum.
probz under the influence of anti mullein hormone. |
|
|
Term
gap fill
inguinal hernias in children are almost always....
they are due to a patent ..... .......
much more frequent in .....
hernias are more common on the ....... hand side |
|
Definition
INDIRECT
processus vaginalis
boys
right |
|
|
Term
| on which hand side are varicoceles commonly found? |
|
Definition
|
|
Term
|
Definition
ventral urethral meatus
chordee: ventral curvature of shaft
glanular variation: sole concern in this form may be cosmetic
mx: surgery
undertaken before 2 years of age
caution: theses with hypospadias must not be circumsised as the foreskin is needed for later reconstructive surgery |
|
|
Term
|
Definition
glanular
coronal
midshaft
penoscrotal |
|
|
Term
| medical indications of circumscision |
|
Definition
phimosis
recurrent balanoposthisis
recurrent UTI |
|
|
Term
| in prolonged jaundiced if the billirubin is conjugated what are you thinking? |
|
Definition
|
|
Term
| what is the kasai procedure used for? |
|
Definition
| in billiary atresia. part of the jejunum is anastamosed to the surface of the port hepatis |
|
|
Term
| alpha 1 anti trypsin deficiency features |
|
Definition
AR
ch 14
prolonged jaundice and liver disease as child
emphysema as adult |
|
|
Term
| what does TIBIDA scan do and show? |
|
Definition
radionuclide scan, the liver will show good uptake but no excretion.
the finding suggests extra hepatic biliary obstruction or atresia. |
|
|
Term
| you find galactose, a reducing substance in the urine of a baby who has prolonged jaundice. what are you thinking? |
|
Definition
|
|
Term
| features of Allagille syndrome |
|
Definition
AD
triangular facies, skeletal abnormalities
congenital heart diseasE: peripheral pulmonary stenosis
renal tubular disorders
defects in eye
intrahepatic biliary hypoplasia with severe pruritus and FTT
variable prognosis
50% children survive into adulthood w/o liver transplantation. |
|
|
Term
| features of wilsons disease |
|
Definition
AR
ch 13
rx: penicillamine to chelate copper or trientine
dx: liver biopsy
extra: zinc is given to reduce copper absorption |
|
|
Term
| what does vitamin D resistant rickets indicate? |
|
Definition
|
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