PAEDS 5: Renal, GU, Rheum, Ortho Flashcards
RENAL - nephrotic syndrome, HUS, HSP, PCKD, Wilm's tumour. GU - enuresis, cryptochidism, hydrocele, torsion, epidiymitis/orchitis. RHEUM - septic arthritis, reactive arthritis, JIA, transient synovitis. ORTHO - SUFE, Perthes, DDH, bone cancer, osgood-schlatter, achrondroplasia
What is the most common cause of nephrotic syndrome in children?
minimal change disease
What is the triad assoc. with nephrotic syndrome?
- low serum albumin
- high urine protein content (>3+ on dipstick = frothy urine)
- oedema (esp. peri-orbitally or of testes)
OTHER FEATURES: deranged lipid profile, hypercoagulability
How is nephrotic syndrome investigated?
Kidney biopsy
- light microscopy no signs
- electron microscopy = loss of foot processes on podocytes
- immunofluorescence = no immune deposits
How is nephrotic syndrome managed?
- high dose steroids for 4w, gradually weaned over next 8w
- diuretics for oedema
- albumin infusions if severe hypoalbuminaemia
What is Henoch Schonlein Purpura?
IgA vasculitis - autoimmune disease involving IgA immune complexes
Abnormal glycolisation of IgA protein is believed to cause abnormal depositing in blood vessels.
Often occurs after viral or bacterial URTI (as IgA released first in defence)
How does Henoch-Schonlein Purpura present?
- Purpuric rash - from buttocks downwards
- Arthralgia - knees and ankles
- Abdo pain - N&V, bowel haemorrhage/infarction
- Kidney sx e.g. haematuria, proteinuria esp.
Supportive mx
What is haemolytic uraemic syndrome?
Non-autoimmune microangiopathic haemolytic anaemia (MAHA)
Typically caused by E. coli 0157:H7 which produces shiga toxin
How does HUS present and how is it managed?
Sx: diarrhoea followed by MAHA, renal impairment (AKI)
Mx: supportive, plasma exchange, eculizumab (a C5 inhibitor monoclonal antibody)
What is Wilm’s tumour?
A nephroblastoma that typically presents in children
2nd most common childhood malignancy
How does Wilm’s tumour present?
Abdo mass (95% unilateral), occasionally haematuria
How is Wilm’s tumour investigated?
BEDSIDE: examination
USS doppler: rule out IVC and renal vein involvement
Microscopy: small undifferentiated blue cells, primitive renal tubule formation
How is Wilm’s tumour managed?
urgent paeds review within 48h
What is enuresis and how is it classified?
Bedwetting in absence of congenital or acquired defects of nervous system or urinary tract.
Most children achieve day and night dryness by 3-4 yrs old.
PRIMARY = child has never achieved continence (w or w/o daytime sx)
SECONDARY = occurs after child sustained continence for >6 months
What are causes of primary enuresis without daytime symptoms?
Sleep arousal difficulty
Bladder dysfunction
What are causes of primary enuresis with daytime symptoms?
Congenital malformations
Neurological disorders
UTI
Overactive bladder
What are causes of secondary enuresis?
Diabetes
UTI
Constipation
Psychological problems - ask about school, friends
Family problems - vulnerable child, abuse
How is enuresis investigated?
Clinical diagnosis
Urine MC&S to rule out UTI
Urine dip to rule out diabetes
How is enuresis managed?
Primary enuresis w/day + night sx = refer to paeds urology
Night sx only = manage in community
1. Conservative - reduce fluid intake, regular emptying, star chart for actions not outcomes
2. Enuresis alarm - high success rate and lower relapse than meds
3. Desmopressin - synthetic ADH replacement, good for short term control or if above fail
What is vesicoureteral reflux?
Abnormal backflow of urine from bladder into ureter and kidney (ureters laterally displaced = more perpendicular angle)
Found in about 30% of children who present w/UTI.
~35% children develop renal scarring, hence important to investigate
How does vesicoureteral reflux present?
Antenatal period - hydronephrosis on USS
Recurrent childhood UTI
Reflux nephropathy - chronic pyelonephritis secondary to VUR
How vesicoureteral reflux investigated?
Micturating cystourethrogram (MCUG) - assesses how urine flows
DMSA - looks for renal scarring, routine referral within 4-6m
- routine referral 4-6m alongside USS if recurrent UTI sx of <3yo w/atypical UTI
How is vesicoureteral reflux graded?
I = into nondilated ureter
II = into pelvis and calyces w/o dilation
III = mild to moderate dilation of ureter, renal pelvis and calcyces w/minimal blunting of fornices
IV = moderate ureteral tortuosity
V = gross dilation of ureter, pelvis and calyces, loss of papillary impressions, ureteral tortuosity
What is epididymitis/orchitis and how does it present?
Infection of epididymitis/testis due to STI (chlamydia/gonorrhoea, teens) or bladder (e. coli, younger kids)
Sx = unilateral testicular pain and swelling, discharge
How is epididymitis/orchitis investigated?
MUST EXCLUDE TORSION (via surgical exploration if indicated by clinical assessment)
External genitalia examination
Urine MC&S
NAAT
USS should not be arranged if scrotal swelling is of acute onset w/red flags - urgent hospital admission or referral is indicated.
How is How is epididymitis/orchitis managed?
Urinary = treat empirically w/quinolone for 2/52
STI = cause unknown IM Cef 500mg + PO Doxy BD 10-14d
What are hypospadias?
Congenital abnormality of penis usually identified on newborn baby check.
Normally isolated disorder but can be associated w/cryptorchordism and inguinal hernias
Presents w/ventral urethral meatus +/- hooded prepuce/foreskin, chordee (ventral curvature)
How are hypospadias managed?
- refer to specialist service (usually paeds urology)
- corrective surgery ~12 months
- DO NOT CIRCUMCISE - foreskin may be used as part of corrective procedure
What are the types of hypospadias?
Normal
Glanural
Coronal
Penile shaft
What is phimosis?
Inability to retract foreskin covering glans penis
- non-retractile foreskin +/- ballooning during micturition and <2yo = physiological phimosis
DON’T attempt to retract foreskin to clean under it if it is still fixed.
What is balanitis/prosthitis?
Inflammation of glans penis +/- underside of foreskin
Typically caused by bacterial or candidal infection, but can also be dermatological
Sx = redness, soreness, dysuria, itchiness, phimosis
How is balanitis and prosthitis investigated and managed?
Ix = swab under foreskin and do STI screen esp. if high risk
Mx = clean w/lukewarm water regularly, no soap or baby wipes. Change nappies frequently, NHS leaflet for balanitis care
Consider hydrocortisone for severe symptoms and specific antibiotics/clotrimazole if organism identified
What is cryptorchidism?
Incomplete descent of one or both testes from abdomen, through inguinal canal and into scrotum
What are the types of cryptorchidism?
True undescended testis = testis lies along normal path of descent in the abdomen or inguinal region but has never been present in scrotum
Ectopic testis = testis lies outside of normal path of descent and outside the scrotum e.g. in femoral region
Ascending testis = testis prev. present in scrotum but has then moved to a higher position over time
Absent or atrophic testis = testis in scrotum at birth but later disappears
What are complications of cryptorchidism?
Impaired fertility, testicular cancer in undescended testis, testicular torsion and inguinal hernia
How is cryptorchidism investigated?
EXAMINATION:
- hypospadias = disorder of sexual development
- bilateral + ambiguous genitalia = CAH
- can attempt to manipulate inguinal testes into scrotum, if it moves back into canal = retractile testis
- if unpalpable to unable to manipulate = undescended testis
- contralateral testis is atrophied = testicular absence or atrophy
How is cryptorchidism managed?
Disorder or sexual development or bilateral at birth = urgent referral to paeds in 24hrs (endocrine and genetics ix)
Unilateral at birth - review at 6-8w
Bilateral at 6-8w = urgent 2w referral to paeds urology
Unilateral at 6-8w = re-examine at 4-5 months - still unilateral - urgent referral to paeds urology to be seen by 6m old
What is testicular torsion?
Testicle rotates twisting spermatic cord that brings blood to scrotum causing ischaemia. Most common M’s 12-18 yo
How does testicular torsion present?
v tender, hot, swollen single testis
cremasteric reflex lost (doesn’t exclude TT if unseen)
elevation of testis doesn’t ease pain (negative Prehn’s sign)
How is testicular torsion investigated and managed?
Urgent surgical exploration
Mx = bilateral fixation (orchidopexy) and possible orchidectomy (salvage rate 90-100% if <6hrs)
What is septic arthritis and how is it investigated?
Single painful swollen joint = septic arthritis until proven otherwise
Always send to paeds A&E urgently
Most common organism is S. Aureus
Ix =
BEDSIDE - obs, examination
BLOODS - FBC, U&E, blood cultures
IMAGING - joint aspiration (in theatre, purulent), XR, MRI considered
How is septic arthritis managed?
Broad spectrum antibiotics according to trust policy then switch to pathogen targeted after consulting micro for advice.
Analgesia for pain
Refer to ortho if complications
Summarise Juvenile idiopathic arthritis
Sx = <16yo, lasts >6wks
- spiking fever, salmon pink rash, ANA marker present, arthritis develops after a few months
Mx = NSAIDs, steroids
Summarise transient synovitis?
Sx = acute joint pain (usually hip) following viral infection, ages 3-8yo
If child is well, afebrile, sx<72hrs = considered for community management
Summarise reactive arthritis?
Inflammatory arthritis that occurs 1-4w post GU/GI infection
Mx = supportive, pain mx
What is Ehlers-Danlos syndrome?
Autosomal dominant condition affecting type 3 collagen.
More elastic tissue = joint hypermobility
Suspect if recurrent joint dislocation occurs.
What conditions are assoc. w/Ehlers-Danlos?
aortic regurgitation, mitral valve prolapse, aortic dissection, easy bruising, SAH
What is SUFE?
Slipped upper femoral epiphysis = displacement of femoral head epiphysis posteriorly and inferiorly
More common in obese and male children
Sx = pain, usually following acute incident, loss of internal rotation on leg flexion
How is SUFE investigated and managed?
Ix = AP and lateral frog-leg views bilaterally
- Trethowan’s sign +ve = when Klein’s line extending along superior femoral neck doesn’t intersect physeal region
Mx = internal fixation with cannulated screw
What is Perthes disease?
Degenerative disease affecting hips of children, 5x more common in boys
Avascular necrosis of femoral head
Sx = progressive hip pain over a few weeks, limp, reduced ROM
Ix = XR bilaterally shows widened joint space, small/flat femoral head
How is Perthes disease managed?
<6yo = observation, often self-resolving, clinic every 3-4mths for examination and X-rays
> 6yo = braces/cast/crutches to keep femoral head in acetabulum and encourage healing, osteotomy considered in older children to help achieve this if severe debilitation or deformity
NOTE: most cases resolve w/conservative management
What is developmental dysplasia of the hip?
Abnormal formation of hip joint, making ball of joint loose in socket allowing it to dislocate easily
What are RFs for DDH?
Female
Breech
FHx
Oligohydramnios
LBW
How is DDH investigated?
Normally picked up in neonatal screening
If missed sx = abnormal gait, leg length discrepancy, asymmetrical skin folds
NEWBORN EXAM + 6w CHECK w/ Barlow and Ortolani’s - ortho referral w/USS in 2-4w
Routine US scanning even if B+O -ve in 4-6w if:
- 1st degree FHx of early hip problems
- breech 36w+ gestation regardless of patient at birth or MOD
- multiple pregnancy
If child >4-5months XR used first line - when ossification of femoral head occurs and abnormalities become more apparent
What is Barlow’s test?
examiner adducts hip while applying a posterior force on the knee to promote dislocation
What is Ortolani’s test?
examiner abducts hip while applying anterior force on femur to reduce hip joint
How is DDH managed?
Dependent on age
Most unstable hips spontaneously stabilise by 3-6w of age
Pavlik harness (dynamic flexion-abduction orthosis) in children younger than 4-5 months
Older children may require surgery
What are osteochondritides?
Trauma-induced focal disturbances of cartilage, either in joints or periarticular epiphyseal plate or at tendon or ligament insertion.
Typically occur in active children and respond to bio-mechanical modifications and pain relief
What is chondromalacia patellae?
softening of patella cartilage
common in teenage girls
characteristically anterior knee pain on walking up and down stairs from prolonged sitting
usually responds to PT
What is Osgood-Schlatter disease? (tibial apophysitis)
sporty teens usually after exercise
pain, tenderness and swelling over tibial tubercle
What is osteochondritis dissecans?
pain after exercise
intermittent swelling and locking
What is patellar subluxation?
medial knee pain due to lateral subluxation of patella
knee may give way
What is patellar tendonitis?
common in athletic teen boys
chronic anterior knee pain worse after running
tender below patella on examination
What is osteosarcoma?
Most common in children
Most common in knee
Replacement of bone marrow w/trabecular bone
XR = sunburst appearance w/elevated periosteum (Codman’s triangle)
What is Ewing’s sarcoma?
Most common in <20yo
HIGHLY malignant, in long bones and pelvis
XR = onion skinning periosteum
Summarise osteomyelitis?
Infection in bone, haematogenous subtype is most common in children (staph aureus most common)
Sx = warmth over bone, local pain/swelling, febrile
Ix = blood culture, bone biopsy, MRI
Mx = IV abx according to trust policy
What is achondroplasia?
AD disorder assoc. w/short stature
Caused by mutation in fibroblast growth factor receptor 3 (FGFR-3) gene = abnormal cartilage
What are symptoms of achondroplasia?
- short limbs w/shortened fingers
- large head w/frontal bossing and narrow foramen magnum
- midface hypoplasia w/flattened nasal bridge
- ‘trident’ hands
- lumbar lordosis
What is rickets?
Inadequately mineralised bone, usually due to vit D deficiency
RFs = calcium deficiency, unsupplemented cow’s milk formula, lack of sunlight
How does rickets present?
Genu varum (bow legs) in toddlers
Genu valgum (knock knees) in older children
Aching bones/joints
