Long case Flashcards
A common complication of hypoplastic left heart repair is protein losing enteropathy.
How do you diagnose and manage this?
Dx: Low albumin, and 24 hour stool A1AT to diagnose
Mx: High protein, high MCT, low sodium and low fat diet
What is the most common cancer associated with Hypoplastic left heart sx and how do you monitor/surveillance for this?
Hepatocellular carcinoma
Liver imaging every 3-5 years -> if abnormal monitor AFP and consider Q6mo imaging.
What regular assessments are important in a child with Congenital heart disease?
Exercise tolerance test, BP monitoring, lung function tests, consider BMD
What are four common complications post cardic surgery?
Post op Horner’s, RLN palsy, diaphragmatic palsy and protein losing enteropathy
Why do you get restrictive lung disease post cardiac surgery?
Die to the sternotomy and thoracotomy related scoliosis
What emergency planning might be important in a child with Congenital heart disease?
ED letter stating congenital anomaly with baseline observations +
Consider a MedicAlert bracelet
What are you told to avoid in children with hypertrophic cardiomyopathy?
Avoid competitive sports and dehydration
What further investigations are required with marfan’s syndrome?
1) annual echo to review aortic root
2) MRI spine for spinal canal enlargement
3) Spinal XRAY for scoliosis
Opthal assessments
6 medical management points for Marfan’s syndrome?
1) Beta blockers and ARBs to slow progression of aortic dissection
2) opthal review for lens subluxation, cataracts and glaucoma
3) Review joints due to laxity, review scoliosis and consider orthopaedic referral
4) Individualised sports program, no scuba dividing , no sit ups, weights, contact sports or strenuous exercise
5) Avoid - Stimulants, vasoconstrictors (triptans) and avoid laser eye surgery
6) pregnancy prophylaxis due to risk of aortic rupture
How would you optimise nutrition and growth in a child with CKD?
1) monitor: centile charts, yearly wrist XRAY for bone age (rickets)
2) Adequate protein/energy; Ix and Mx salt losing; Manage acidosis (bicarb supplementation?)
3) Monitor for hypothyroidisim
4) Consider feed supplementation; link with renal dietitian
5) Consider GH for older children (refer to endocrine team)
How do you manage bone mineral disease in CKD?
1) Monitor for bone pain and deformity (osteitis fibrosa cystica and rickets)
2) Low phosphate diet
3) Consider phosphate binders to prevent secondary hyperparathyroidisim
4) Vit D supps
5) Monitor PTH and ALP;
6) DEXA 2 yearly especially post transplant/chronic nephrotic syndrome
Which allied health services might be important in a child with CKD/Nephrotic syndrome?
1) Nutrition/dietician - ensure adequate protein/energy/low phosphate diet
2) Social work - impact on parents, link with Kidney health Australia, dialysis/transplant associations
3) psychology or neuropsych as needed
What are the four primary features of Nephrotic syndrome?
Proteinuria + hypoalbuminaemia + hypercholestrolaemia + oedema
What are other secondary features of nephrotic syndrome?
Hypocalcaemia, hyperkalaemia, hyponatraemia, hypercoagulability, hypothyroidism
What emergency management plans do you need for a child on dialysis?
Plan for:
- What if catheter falls out?
- What if the peritoneal fluid becomes cloudy?
What workup is necessary prior to transplant?
- Ensure all live vaccines are done prior
What are the major side effects associated with Mycophenolate?
Myelosuppression, malignancy, gut effects
What are major side effects associated with Tacrolimus?
Htn, GI sx, nephrotoxicity, infection, malignancy, low Mg
What infections do you need to monitor for pre-post/transplant?
1) PJP proph - Bactrim or pentamidine
2) screening for CMV, EBV, PCP, BK
3) Valgancyclovir for CMV (usually first 3-6 month)
4) Annual flu vax
5) Consider UTI prophylaxis
What are the four domains of routine follow up post renal transplant?
1) Cardiovascular - increased incidence of Htn and hyperlipidaemia
2) Bones - osteoporosis from steroids, vitamin D deficiency; bone density monitoring bi-annually
3) Cancer risk - skin protection and regular review. HPV vaccine
4) Disease recurrence
- MPGN, HSP, IgA nephropathy, FSGS, HUS
- Monitor proteinuria and biopsy for recurrence
What surveillance is required for post renal transplant?
1) Monthly - growth, BP, oedema, EUC, immunosuppressant levels, UA
2) Second yearly US, eye/skin/bone density reviews
3) Careful dosing of nephrotoxic drugs
What future planning is required after a transplant?
1) managing fertility issues and genetic counselling
2) need for re-transplant
What other things should you screen for when reviewing a patient with T1DM?
Thyroid, coeliac, adrenal issues
What Ix should you ask about for hx of T1DM?
OGTT or Insulin antibodies
Ask about any liver US and any other testing related to other autoimmune conditions
