Nephrology and Urology

Question 1

epidemiology of HSP-henoch schonlein purpura?

Answer 1

usually occurs between ages of 3 and 10 years, commonly between 4 and 6
twice as common in boys than girls
caucasians
peaks in winter mnths-cold exposure may be assoc. environmental factor, and often preceded by URTI-group A streptococci, EBV

Question 2

aetiology of HSP?

Answer 2

systemic vasculitis
unknown aetiology, presumed immune complex mediated disease
thought that genetic predisposition and antigen exposure increase circulating IgA and disrupt IgG synthesis, the IgA and G interact to produce complexes that activate complement and are deposited in affected organs causing inflammatory response with a systemic vasculitis.

Question 3

presenting features of HSP?

Answer 3

symmetrical erythematous maculopapular rash that evolves over 24hrs into purpuric lesions that are characteristically palpable, may recur over several wks, and is 1st clinical feature in about 50%. widespread over buttocks and extensor surfaces of arms and legs, and ankles, with trunk sparing unless trauma induces lesions.
arthralgia-part. knees and ankles, usually resolves before rash disappearance, no permanent joint deformity
periarticular odema
fever?-usually absent
abdo pain, haematemesis, melena-GI petechiae, intussusception-differential for abdo pain, and occurs in 2-3% of HSP patients
micro and macroscopic haematuria-glomerulonephritis, or mild proteinuria, can be nephrotic syndrome-proteinuria more than 3.5g/24hr along with hypoalbuminaemia and oedema, if renal involvement must be f/u for 1 yr to decide who needs LT f/u, as HTN and deteriorating renal function may develop after interval of several years.
can be orchitis and CNS complications, may be headaches.

Question 4

differentials for HSP?

Answer 4

purpuric rash-thrombocytopenia e.g. ITP-low PLT count, meningococcal septicaemia-fever
CTD e.g. SLE
intussuception-abdo pain
other causes of glomerulonephritis e.g. IgA nephropathy-males, haematuria whilst URTI e.g. coughing or sore throat.
acute glomerulonephritis
acute haemorrhagic oedema of infancy-fever, oedema, and rosette shaped, annular or targetoid purpura affecting face, ears and extremities.
arthralgia-juvenile idiopathic arthritis-systemic form-remitting fever, variable rash

Question 5

HSP investigations?

Answer 5

diagnosis is based on clinical picture
FBC: raised WCC with eosinophilia, may be normal or elevated PLT (note reduced PLT in ITP differential)
ESR:raised
serum creatinine elevated in renal involvement
raised serum IgA
autoAb screen-CTD e.g. ANA in SLE
urinalysis-haematuria and proteinuria
abdo USS
barium enema-confirm and treat intussuception
renal BIOPSY if persistent nephrotic syndrome

Question 6

HSP management?

Answer 6

mainly supportive
NSAIDs for arthralgia relief, but use with caution if renal impairment-*as inhibit PG prod. which cause dilation of afferent arteriole for adequate renal perfusion
hosp admission for abdo and renal complication monitoring
possibly corticosteroids for arthralgia and GI dysfunction relief
supportive nephropathy tment

Question 7

what monitoring for renal involvement is advised in f/u of HSP patients?

Answer 7

if no proteinuria, BP and urinalysis at 1 wk, 2 wks and then at 1, 3, 6 and 12 mnths
if proteinuria, monitoring at 1 wk, 2 wks, monthly from 1-6mnths then at 12 mnths.

Question 8

complications of HSP?

Answer 8

renal impairment, but disease only progressive to ESRD in 1% of patients
MI
pleural effusion
CNS complications e.g. seizures, mononeuropathies
GI bleeding
bowel infarct
pulmonary haemorrhage

Question 9

what is long term prognosis in HSP directly dependent on?

Answer 9

severity of renal involvement

Question 10

characteristic blood result findings in patient with nephrotic syndrome?

Answer 10

hypoalbuminaemia: serum albumin less than 25g/L
hypercholesterolaemia: serum cholesterol more than 5.17mmol/L
hyponatraemia
hyperkalaemia
hypocalcaemia-loss of Vit D binding protein in urine, LT risk of bone demineralisation
hypercoagulability-decreased partial thromboplastin time

Question 11

clinical presentation of minimal change disease/idiopathic nephrotic syndrome?

Answer 11

usually younger children (2-5yrs), boys (until puberty, then equal sex incidence)
nephrotic syndrome-oedema-peri-orbital swelling, ascites-everted umbilicus, pitting ankle oedema
proteinuria-low molecular weight anionic proteins e.g. albumin, but some higher molecular weight e.g. IgG also lost.
HTN and haematuria in 10%, but transitory
most respond to corticosteroids

Question 12

usual diagnosis in child with corticosteroid resistant idiopathic nephrotic syndrome?

Answer 12

FSGS

Question 13

typical features of nephrotic syndrome caused by FSGS, membranous glomerulonephritis, SLE or mesangial proliferative glomerulonephritis?

Answer 13

older children (6-15 yrs)
variable sex ratio
greater protein leakage through glomerular filtration barrier, higher molecular weight proteins e.g. Igs may be lost.
haematuria often present
HTN may occur
often not responsive to steroids

Question 14

how can children with minimal change disease be classified?

Answer 14

those in remission: urinary protein excretion less than 4mg/m^2/hr, or no protein trace on dipstick, or protein/creatinine ratio 0.02g/mmol for 3 consecutive days
relapse: proteinuria recurrence as defined in minimal change definition, or 2+ or more on dipstick for 3 consecutive days
frequent relapses: 2 or more within 1st 6 mnths or initial response, or more than 4 in 12 mnths
corticosteroid dependence: 2 consecutive relapses while on prednisolone or within 2 wks of stopping it
corticosteroid resistance: failed response after 8wks of 2mg/kg/day
ciclosporin dependent: relapses when ciclosporin stopped or tapered, steroid dependent children who achieve remission with ciclosporin.

Question 15

why are children with nephrotic syndrome more susceptible to infection, and which types of infection in particular are they more at risk of?

Answer 15

proteinuria-urinary loss of IgG
loss of factors B and D of alternate complement activation path
transferrin loss
altered T cell function
impaired Ab producing ability
loss of opsonising factors*-specifically increases risk of encapsulated organism infection
steroid and immunosuppressive drug tment
mechanical-oedema and ascites

encapsulated bacteria: strep pneumoniae (less so now with vaccination), H.influenzae, E coli, causing cellulitis, UTIs, peritonitis, meningitis, septicaemia.
*peritonitis risk further increased by reduced mesenteric b.flow and increased coagulability, with reduced flow and sludging causing microinfarction.

Question 16

what worrying complication of nephrotic syndrome is more likely in a child suffering acute fluid loss e.g. viral gastroenteritis with diarrhoea and vomiting?

Answer 16

thromboembolism
risk in NS assoc with raised plasma fibrinogen and clotting factors due to increased hepatic synthesis, decreased plasma ATIII and protein S as lost in urine, PLT abnormalities, increased blood viscosity, decreased b.flow and hyperlipidaemia.

Question 17

what raises our concerns for CVD risk in patients with nephrotic syndrome?

Answer 17

hyperlipidaemia plus:
corticosteroid exposure
HTN
hypercoagulability
anaemia

Question 18

why is nephrotic syndrome thought to cause growth disturbance, especially if congenital?

Answer 18

loss of IGF binding protein in the urine
and
corticosteroid use

Question 19

why is hypothyroidism a complication of nephrotic syndrome?

Answer 19

loss of thyroid binding protein in urine

Question 20

current symptoms we want to know in patient presenting with nephrotic syndrome?

Answer 20

general health-appetite loss, weight gain, lethargy, poor height gain
oedema-periorbital, ankle swelling-pitting oedema, ascites, scrotal swelling
may be abdo pain and diarrhoea
urinary-haematuria, oliguria, concentrated urine
other-infections*incresed susceptibility with loss of Igs-IgG and complement activation pathway factors and opsonising factors in urine, and immunosuppressive tment, abdo pain, HTN
?SOB-pleural effusion-CXR

Question 21

investigations needed in nephrotic syndrome?

Answer 21

urinalysis:
proteinuria-3+ or 4+ on dipstick
cellular casts e.g. hyaline or waxy casts in minimal change
microscopic haematuria-may persist in steroid resistant NS
bloods:
Us and Es
FBC
albumin and total protein levels
lipid profile
serum complements C3 and C4-normal in minimal change, low in SLE
Hep B and C serology-B assoc. with membranous, C with mesangial proliferative.

Question 22

indications for renal biopsy in nephrotic syndrome?

Answer 22

steroid resistance-no response within 1 mnth
age under 1 yr
nephritic features-HTN, haematuria, raised creatinine/renal impairment
persistent abnormal serum complement (low C3)
older children, part. african americans where FSGS more common-most common progressive glomerular disease in children

Question 23

best prognostic indicator in nephrotic syndrome?

Answer 23

steroid sensitivity

Question 24

what is acute glomerulonephritis?

Answer 24

sudden development of immunological damage to the glomerulus
commonest form in childhood result of immune complex formation following infection by nephritogenic form of streptococcus-pt has hx of sore throat or skin infection, and 1-2 wks after presents with haematuria= acute post streptococcal glomerulonephritis

Question 25

clinical features of acute glomerulonephritis?

Answer 25

haematuria-appearance of smoky or cola-coloured urine (gross haematuria) and microscopic
may be otherwise asymptomatic, although may also be malaise, headache-may occur secondary to HTN, loin discomfort-stretching of renal capsule.
can be oedema around eyes, and dorsum of hands and feet
oliguria
may be proteinuria
may be HTN

Question 26

results of investigations in acute glomerulonephritis?

Answer 26

proteinuria
urine microscopy-gross haematuria, with granular and red cell casts
mild oliguria
may be complicated course with renal failure, HTN, seizures, and heart failure and pulm oedema-*SOB.

Question 27

management of acute glomerulonephritis?

Answer 27

diagnosis of post-streptococcal glomerulonephritis made via throat swab and ASO titre-antistreptolysin O titre-blood test to measure Abs produced against streptolysin O-produced by Group A strep. also low C3.
monitor creatinine clearance and fluid balance, restrict Na+ and H2O if oliguria develops.
diuretics and anti-hypertensives if HTN develops, rarely peritoneal dialysis.
penicillin-eradicate strep infection, but no evidence it affects course of disease. also culture family members and treat if organism found.

Question 28

components to glomerular filtration barrier?

Answer 28

capillary endothelium
glomerular BM-anionic proteins with -ve charge
visceral epithelium-podocyte foot processes-filtration slit diaphragm

*increase in glomerular capillary wall permeability-nephrotic syndrome

Question 29

overall nephrotic syndrome management?

Answer 29

discourage excessive fluid intake
limit Na+ intake to no added salt
advise parents when 1st discharged from hosp to monitor daily fluid input and output and record, and weigh child daily
prednisolone to induce remission, must do daily weighing and monitor proteinuria-remission when no protein for 3 days, low dose continued for 4-6wks
prophylactic penicillin during steroid tment

Question 30

most common organism responsible for UTI in childhood?

Answer 30

E coli

Question 31

clinical features in neonates of a UTI?

Answer 31

prolonged jaundice
apnoea
weight loss
collapse

Question 32

indications of UTI on urine MCS?

Answer 32

growth of more than 10^5 colony forming units
plus greater than 50 WCC
(normal WCC urine culture=0-40)

Question 33

how are children under 6 mnths of age with UTI investigated?

Answer 33

if single confirmed UTI and repsond rapidly to tment, require urinary tract USS.

Question 34

define hypospadias?

Answer 34

abnormal opening of urethra along the inferior aspect of the penis, usually near the glans, along the shaft, or near the base.
rarely may extend along the scrotal raphe

Question 35

how does hypospadias occur?

Answer 35

incomplete fusion of urethral folds:
in normal development, androgen secretion by fetal testes (Leydig cells) stimulates rapid elongation of genital tubercle-now phallus, and during this process, phallus pulls urethral folds forward so they form lateral walls of urethral groove which extends along caudal aspect of elongated phallus but does not reach the glans. epithelial lining of groove forms the urethral plate, and at end of 3rd mnth, urethral folds close over urethral plate to form penile urethra.

?relation to rise in environmental oestrogens

Question 36

other conditions hypospadias may occur in association with?

Answer 36

cryptorchidism (in about 10% of cases)
9-15% also have an open processus vaginalis or inguinal hernia.

sometimes FH of hypospadias

Question 37

problems associated with hypospadias?

Answer 37

problems more common the further posteriorly the urethral opening arises on the inferior aspect of the penis:
urine spraying backwards
chordee (tissue tightening on inferior aspect) pulling penis down, can make erection difficult
premature ejaculation in adulthood, and in those who had surgery in childhood
psychological problems

Question 38

treatment of hypospadias?*

Answer 38

if mild, so urethral opening close to normal location, no treatment required
in most cases, surgery perfomed when child 4-18mnths, may need 2 ops if complicated
position of urethral opening altered and if chordee present then this is corrected
foreskin usually used to make new urethra.

Question 39

most common cause of haematuria in childhood?

Answer 39

UTI

Question 40

acute nephritis vs. nephritic syndrome, and causes?*

Answer 40

post strep glomerulonephritis
IgA nephropathy
vasculitis-wegener’s, HSP, SLE
anti-glomerular BM disease (Goodpasture syndrome)

Question 41

why is early diagnosis of UTIs in children, especially infants, essential?

Answer 41

as most vulnerable age for renal scarring that can lead to later HTN and renal failure

Question 42

how are infant urine specimens collected for dipstick in suspected UTI?

Answer 42

routine clean catch with help of carer

bag urine-U bag, if above fails, this must be checked regularly by opening up nappy.

Question 43

when should urine be sent for culture in children?

Answer 43

as routine in children 3 years and younger, and if child systemically unwell

Question 44

Abx treatment for acute pyelonephritis in children?

Answer 44

cefuroxime for at least 7 days, and single dose of gentamicin

Question 45

lower UTI Abx treatment in children?

Answer 45

if aged over 6months and not sick then cefalexin or trimethoprim for 3-5 days
if aged under 6mnths or sick then cefuroxime for at least 7 days and single dose of gentamicin.

Question 46

indications for US imaging within 6 wks of UTI?

Answer 46

child under 6 months
recurrent UTIs-3 or more lower UTIs, 2 or more with acute pyelonephritis or 1 acute pyelonephritis and 1 or more lower UTIs.

Question 47

atypical UTI features?

Answer 47

seriously unwell
poor urinary flow
abdo or bladder mass
non E-coli organism
raised serum creatinine
septicaemia
failure to respond to suitable Abx within 48hrs

Question 48

predisposing factors to a 1st UTI in a child?

Answer 48

incomplete bladder emptying
vesicoureteric reflux
constipation

Question 49

what specific examinations should be performed in the child who presents with enuresis?*

Answer 49

abdominal
neurological (CNS), including lower limb reflexes
spine

Question 50

how should the problem of nocturnal enuresis in a 6yr old be explained to parents?

Answer 50

that at an age around 6 years this is normal
child is normal and healthy
child will grow out of it naturally
the problem in beyond the child’s conscious control so patience from parents is required, this is a developmental issue

Question 51

what advice can be given regarding simple management of nocturnal enuresis?

Answer 51

drink plenty of fluid during the day
pass urine regularly during the day
don’t drink too large amount of fluid close to going to bed
pass urine before going to bed
praise child when wakes with dry bed e.g. use of star charts
may consider enuresis alarm if no response to star chart
minimise stresses at home

Question 52

how can short term relief of nocturnal enuresis be provided e.g. for a child going on holiday?

Answer 52

with PO desmopressin-synthetic analogue of ADH, only taken temporarily as achieves a suppressant effect rather than lasting cure.

Question 53

name of the fatal syndrome that results from bilateral renal agenesis?

Answer 53

Potter syndrome-result of severe oligohydramnios due to absence of fetal urine
oliohydramniosis causes intrauterine compression of the fetus, causing characteristic facies-low set ears, beaked nose, downward slanting eyes, lung hypolasia and limb deformities including severe talipes.

Question 54

what causes a horseshoe kidney?

Answer 54

failure of kidneys to ascend from the pelvis due to blockage by inferior mesenteric artery, lower poles of the kidneys then fuse in the midline.

Question 55

risks associated with a pelvic or horseshoe kidney?

Answer 55

due to abnormal position, increased risk of infection or urinary obstruction.

Question 56

what causes a duplex kidney?

Answer 56

premature division of the ureteric bud

may be a bifid renal pelvis or complete division with 2 ureters

Question 57

problems with duplex kidney?

Answer 57

if 2 ureters, usually have abnormal drainage so ureter from lower pole moiety often refluxes, and upper pole ureter may drain ectopically into urethra or vagina, or may prolapse into the bladder (ureterocele) and urine flow may be obstructed.

Question 58

causes of bilateral hydronephrosis?

Answer 58

bladder neck obstruction due to neuropathic bladder

posterior urethral valves-mucosal folds or a membrane in boys

Question 59

why might mild outflow tract obstruction not be visible in neonates in 1st few days of life?

Answer 59

as newborn kidney has low GFR, so there is low urine flow anyway

Question 60

management protocol for antenatally diagnosed urinary tract anomalies?

Answer 60

propylactic Abx started at birth
if bilateral hydronepohrosis and/or dilated lower urinary tract in a male-US within 48hr to exclude posterior urethral valves-if abnormal do MCUG, and then surgery if required, if normal stop Abx and rpt US after 2-3mnths.

if unilateral hydronephrosis in male, or any anomal in female, US at 4-6wks, if abnormal further investigate, if normal stop Abx and rpt US at 2-3mnths.

Question 61

what is vesicoureteric reflex (VUR)?

Answer 61

developmental anomaly where ureters are displaced laterally and enter directly into the bladder rather than at an angle, with a shortened or absent intramural course.
familial
may also occur with bladder pathology e.g. neuropathic bladder or urethral obstruction, or temporarily after a UTI.

Question 62

problems with VUR?

Answer 62

may only cause mild reflux into ureters on micturition, or may reflux during bladder filling and voiding with a distended ureter, renal pelvis and clubbed calyces so spectrum of disease.
reflux means not all urine expelled on micturition causing stasis, and risk of UTIs-there is part. high risk of renal scarring after UTIs if intrarenal reflux with urine backflow into papillary CDs.
infection can be LUTIs and pyelonephritis
high bladder voiding pressures transmitted to renal papillae may cause renal damage.
renal scarring can cause a shrunken, poorly functioning segment of kidney=reflux nephropathy.
if bilateral and severe scarring, can cause CKD.

Question 63

What diagnosis is suggested by a child over 5 yrs who is never dry?

Answer 63

Ectopic ureter