Renal -clinical exam

Question 1

Renal short case exam

Answer 1

What is the cause?
What are the complications of disease? ( HTN, volume overload, anemia, bone disease, growth failure)
IHIGVIDEP
-note growth (small vs overweight from steroids), HTN, hearing aids, skin, nutritional status, moon facies, hypertrichosis/hirsuitism

Hands
-tremor
-pallor palmar creases
-koikonichia
-splaying of wrists
-bruising

Head
- conjunctival pallor
- periorbital swelling
-cataracts
-hearing (grossly normal but would check formally)
-gingival hyper trophy, candida

Neck
-scars
-jvp

Chest
-ricketsial rosary
-flow murmur
-crackles
-sacral oedema

Abdo
- scars
-stomas
-Pd site
-distension
-masses, organomegaly
-percuss for ascutes
-auscultate renal bruits

Lower limbs
-oedema
-tibial bowing

I would also like to check tanner staging and hearing

Ix:
URINALYSIS
Renal uss
Renal biospy
Renal function Creatinine , urea
Treatment- hba1c, hypoMg
Bones- ca,phosphate, bicarbonate, vit d, pth, ALP , X-rays hips knees ankles (osteopenia)

Question 2

Hepatorenal syndrome

Question 3

autosomal recessive polycystic kidney disease

Answer 3

renal and liver manifestations

polyuria + polydypsia (impaired urinary concentration)
metabolic acidosis
HTN
recurrent UTIs

hepatomegaly and portal HTN

only thing that would give you BIG palpable kidneys in kids

Question 4

causes of ESRF/transplant in children

Answer 4

1. CAKUT (congenital anomalies of kidney and urinary tract)- Dysplasia/Hypoplasia +/- urological abnormalities (includes PUVs and reflux nephropathy)
   
   2. Glomerulonephritis (eg post strep, lupus)
2. Neurogenic bladder eg Spina bifida
   
   4. Focal segmental glomerulosclerosis
   5. Congenital nephrotic syndrome
   6. Nephronophthisis
   7. Polycystic kidney disease (ARPKD- renal and liver involvement, also leads to cirrhosis and portal HTN)
   8. Alport’s
   9. HUS

Question 5

Anemia in CKD

Answer 5

Managed with darbopoetin (Arenesp)
- IV or subcut or IP
- Iron infusions + B12 + folate

post transplant usually related to MMF and ACE-I; may still require iron and arenesp post transplant

Question 6

Poor growth in CKD

Answer 6

Inadequate intake of protein and calories especially in the first two years of life. Children with dysplasia/obstructive uropathy will need salt, water, bicarbonate supplementation.
There is resistance to GH and low levels of IGF 1 so are eligiable for Growth hormone
· The most important factor in the first couple of years in achieving adequate nutrition – potentially difficult in a small vomiting dialysis dependent infant.

· Important and impossible. As a rule children with obstructive uropathy/dysplasia lose salt and water and need this supplemented. This may require ng feeding ( are they oroaversive) or a gastrostomy tube.
· Reflux is common – may need a fundoplication.
· Uraemia is associated with anorexia – many foods taste odd and it is also associated with high leptin levels.
· 
· In theory should be on low phosphate+/-potassium diet but this basically excludes any food with flavour. Phosphate binders are used – these include caltrate which tastes awful and can increase serum Ca. Sevalamer is a newish drug which reduces phosphate without giving calcium.

Question 7

Development and cognition

Answer 7

ESRF in early childhood associated with global developmental delay (multifactorial). Adult outcome studies show significantly impaired cognition.

Question 8

Bladder issues in CKD/transplant

Answer 8

Obstructive neuropathy (eg PUVs) andneurogenic bladders can have awful bladders. They may require surgery to increase the size, or big baggy sacks of urine which don’t empty properly. Either way they may well end up having to catheterise either per urethra or via a Mitroffanoff.

This is pretty grim and becomes even more important post transplant. Not surprisingly lots don’t do them – end up with wrecked grafts, UTIs, accelerated renal failure etc.

Question 9

Renal bone disease

Answer 9

Renal bone disease begins early and can be exacerbated by acidosis. Initially hypocalcaemic and then go on to develop secondary hyperparathyroidism.

Need to keep Ca up and PO4 down to keep PTH down ( target is about twice upper limit of normal).

Rx: calcitriol.

Question 10

Chronic graft dysfunction > 3 mo post transplant is due to:

Answer 10

• Calcineurin inhibitor toxicity which may progress to CKD
  
  • Nonadherance to immunosupression medications (highest risk of this amongst young adults)
    Recurrence of primary disease esp FSGS

Question 11

Post transplant complications:

Answer 11

• HTN ( secondary to CNI, steroids, recurrance primary disease, obesity)
• CV disease (htn, dyslipidemia) + T2DM
• Infection (cmv, varicella, EBV, HSV, BK virus)
• PTLD
• Malignancy
• Mineral bone disease
• Anemia
• Side effects from tacrolimus, MMF and steroids

Question 12

Questions to ask about dialysis

Answer 12

• PD or HD
• Overnight, how many hours or how many days a week for HD
• limitation on fluids?
• any infections - eg peritonititis?
  who is trained to do PD? burden of care on one parent, means can never ever have a night off
• how do they feel about the catheter?
• HD- access, infections, pain, what they do while on HD eg do they get schoolwork?

Question 13

Family hx in long case

Answer 13

Alports/deafness
IHD
Diabetes

Question 14

Psychosocial impacts of CKD

Answer 14

Dialysis
· Long lasting dependence on parents eg dialysis, appointments.
· Dietary restrictions
· Significant loss of time at school for treatment/appointments
· Loss of time for social interaction eg going to stay with friends
· Do they get school work on haemo?
· Restriction of being on APD for 10+ hours a night – attached to machine/home
· Blood tests
· Adherence issues

Transplant
· Continuing pre transplant morbidity eg bladder management
· Parental dependence/anxiety
· Dependence on long term check ups and blood tests
· Unexpected complications – rejection, infection
· Long term loss of graft
· Have to take drugs with unwelcome side effects (steroids, cyclosporine – ie short, fat and hairy)
· Adherence issues

Adolescent
· Missing school/training time
· Delayed puberty
· Short stature
· Responsibility for own meds
· Responsibility for other care eg catheters
· Adherence
· Smoking
· Contraception – pregnancy can accelerate CRF, is virtually unknown on dialysis but can occur post transplant.
· Have they received advice re genetics if appropriate?
· Plans for transition
· Overall adults with ESRF are more likely to live with their parents, less likely to have a partner than age matched controls
· Do they go to the Kidney Camp ( for kids all round the country, annual)
· Make a Wish Foundation

Effect of the disease on siblings
· Anxiety/ feelings of neglect
· Screening issues
· Some sibs go to camp

Effect of the disease on parents
· Guilt/grief over congenital disease
· Financial
· Work – may need time away from work at any time
· Are they both trained to do PD – if relevant – or does it all fall to one parent? Is any one else trained or can they never have a break from the child
· Respite