6- Paediatric Nephrology Flashcards
Causes of Chronic kidney disease
IN ADULTS:
- HTN
- T2DM
Causes of Chronic kidney disease
IN CHILDREN:
- Congenital – Renal dysplasia, obstructive uropathies like PUV, reflux nephropathy – presents with uti
- Genetic syndromes– Bardet-Biedl syndrome, Joubert syndrome
- Hereditary – ARPKD, ADPKD, hereditary nephritis like Alport syndrome, familial nephronophthisis
- Tubulopathies- Cystinosis, Dent’s disease
- Glomerulonephritis – FSGS, MPGN, lupus nephritis, congenital nephrotic syndrome, ANCA vasculitis
- Tumours/Vascular – Wilms tumour ( bilateral), renal venous thrombosis, renal artery stenosis
- Infection – Hemoltic uraemic syndrome (E.coli)
- AKI leading to CKD/misc – Significant hypoxia, medications, cortical necrosis of the newborn, prematurity with LBW, severe obesity
define Chronic kidney disease
CKD is defined as the presence of kidney damage, manifested by abnormal albumin excretion or decreased kidney function, quantified by measured or estimated GFR that persists for more than three months.
classification of CKD
KDIGO
Looks at
- eGFR
- albumin/creatinine ratio
complications of CKD
- Anaemia of Chronic Kidney Disease
- Chronic Kidney Disease – Mineral & Bone Disease
- Secondary & Tertiary hyperparathyroidism
- Hypertension
- Cardiovascular Disease – No 1 cause of Mortality
- Malnutrition/sarcopenia
- Dyslipidaemia
As CKD progresses
* Electrolyte disturbances
* Fluid overload
* Metabolic acidosis
* Uraemic pericarditis
* Uraemic encephalopathy
CKD staging ins paediatrics is
the same as adults
investigations for CKD
- History, development, family history
- Growth and nutrition
- Fluid status, blood pressure, urine output
- Examination, blood pressure, urine analysis
- Blood tests
- Urine
- renal biopsy
- imaging
- urinary bladder assessments
blood tests for CKD
FBC
U&Es
- Na
- K
- Urea
- Creatinine
- bicarb
Bone profile (calcium, vit D, PTH)
LFT
Growth and nutrition in CKD
- often infants will have salt losing nephropathy, polyuria, acidosis, are unable to handle the potassium load
- Infants are at the greatest risk, children with CKD may require Growth Hormone therapy (avoid in severe MBD)
- First 2 years of life – growth is principally dependent on nutrition
o Often they have vomiting, reflux, have a poor appetite – need calorie supplementation
o Fluid overload makes assessment difficult
MDT management of CKD
- Renal physicians
- General practitioners
- Renal specialist nurses/ home care team
- Dieticians
- Pharmacists
- Vascular/transplant surgeons
manaagement of CKD principles
outpatient based
- treat udnelrying disease
- reduce CVD risk
- reduce progression of CKD
- prevent or treat complications of CK
- plan for the future
CKD management: treat underlying disease
- Treat and monitor diabetic control
- Treat hypertension
- Treat infections promptly
- Tolvaptan if meets criteria for ADPKD
- Immunosuppression for GN if appropriate
CKD management: Reduce cardiovascular risk
- Start on statin
- Control BP
- Improve control of diabetes
- Advise weight loss
- Advise exercise
- STOP SMOKING
CKD management: Reduce progression of CKD
- Reduce proteinuria – ACEi/ARB
- Monitor blood tests
- Control BP
CKD management: Prevent or treat complications of CKD
- Dietary advice regarding low phosphate/low potassium diet
- Phosphate binders
- IV Iron/Folate/Vit B12 replacement
- EPO (Erythropoesis stimulating agent)
- Replace Vitamin D deficiency
- Consider Calcimimetics for tertiary hyperparathyroidism
- Dietician input
CKD management: Plan for the future
- Start discussions of what options they have if they reach ESRF
- Home care team input
- Discuss disadvantages & advantages of types of RRT
o Home therapies – APD, CAPD, Home HD
o Unit-based therapies – Nocturnal HD, conventional HD
o Active conservative management
o Transplant - Refer for fistula
o Venous mapping - Refer for PD tube insertion
- Work-up for transplant
o Further tests
o Refer to Transplant work-up clinic
CKD management: Plan for the future
- Start discussions of what options they have if they reach ESRF
- Home care team input
- Discuss disadvantages & advantages of types of RRT
o Home therapies – APD, CAPD, Home HD
o Unit-based therapies – Nocturnal HD, conventional HD
o Active conservative management
o Transplant - Refer for fistula
o Venous mapping - Refer for PD tube insertion
- Work-up for transplant
o Further tests
o Refer to Transplant work-up clinic
Renal replacement therapy
- Haemodialysis
- Peritoneal dialysis
- Renal transplant
haemodialysis indications
- Acute kidney injury.
- Uremic encephalopathy.
- Pericarditis.
- Life-threatening hyperkalemia.
- Refractory acidosis.
- Hypervolemia causing end-organ complications (e.g., pulmonary edema)
- Failure to thrive and malnutrition.
haemodialysis
How it works
The dialysis machine pumps blood from the patient, through disposable tubing, through a dialyser, or artificial kidney, and back into the patient. Waste solute, salt and excess fluid is removed from the blood as it passes through the dialyser.
Advantages
o Efficient form of dialysis
o Unit-based – plenty of support from staff
Disadvantages/Complications
o Dialysis access needs to be secured
o Infection/Bacteraemia
o Haemodynamic instability
o Reactions to dialysers
o Haematomas/risk of bleeding
o Muscle cramps
o Anaemia due to clotted lines/Haemolysis
o AVF steal syndrome
o SVCO from central lines
types of haemodialysis
Home HD – offer training at home for more frequent HD
Nocturnal HD– Overnight slow, long HD
CRRT – continuous renal replacement therapy mainly used in acute setting (ITU/HDU)
Peritoneal dialysis
How it works
* Home based therapy
* Reliant on the patients’ own peritoneal membrane acting as the dialysis membrane.
* Solutes (electrolytes, urea, creatinine) move from the patient’s blood, across the peritoneal membrane, down the concentration gradient into the dialysate fluid.
* Osmotic gradient is created by high concentration of glucose (occasionally amino acid or glucose polymer solutions are used) in the dialysate fluid, which removes water from the patient.
Advantages
* Quality of life
* It is often an excellent first choice for patients starting dialysis, particularly when they still have some residual native renal function
* PD regimes are designed on a much more individualised basis than patients on HD.
Disadvantages
* Patients need to be able to manage technical aspects of dialysis
* Unsuitable in patients with stoma/previous surgery
* Risk of infection (PD peritonitis)
* Complications – drainage problems, malposition, leaks, herniae, hydrothorax, long term use associated with encapsulating peritoneal sclerosis
types of PD
Automated PD
* Carried out with an automated cycler machine performed at night.
* 10-12L usually exchanged, over 8-10 hours.
* Lifestyle advantages – Leaves the daytime free
Continuous Ambulatory PD
* Usually consisting of 4-5 dialysis exchanges per day (usually 2 litres each)
* Exchanges are performed at regular intervals throughout the day, with a long overnight dwell.
Assisted Automated PD
Trained healthcare assistants visit the patient’s home to help with setting up APD.
Transplantation
Treatment of choice for most patients with ESRF
Advantages
* Near normal lifestyle
* Better mortality/morbidity
Disadvantages
* Criteria to meet suitability to safely undergo operation
* Compliance with medication lifelong
* Risk of rejection
* Risk of malignancies over time
* Risk of infection (on immunosuppression)
o CMV, hepatitis B, herpes, varicella zoster, EBV, aspergillus, pneumocystis jiroverccii, listeria, MTB
* Long waiting times for cadaveric organ
* New onset diaebetes
types of transplanation
Can be live related/non-related or deceased donor
Living related donor
* Best
* Good compatibility
* Time to transplantation can happen in months
Living unrelated
4 forms:
- live-donor paired exchange,
- live- donor/deceased-donor exchange,
- live-donor chain,
- altruistic donation
Usually have comparable outcomes to live-related donors
Time to transplantation can happen in months
Deceased donor
- Approximately 60% of the transplants in the UK fall into this category
- Patients receive a kidney (or two from the same donor) with little time for preparation, so transplant protocols are important to keep updated regularly
- Time to transplantation happen in years
- Survival of kidney allograft and patients are significantly low compared to live donor transplantation.
Contraindications for kidney transplantation
- Active infection or malignancy
- Severe heart disease not suitable for correction
- Severe lung disease
- Reversible renal disease
- Uncontrolled substance abuse, psychiatric illness
- On-going treatment non-adherence
- Short life expectancy
Contraindications for kidney transplantation
- Active infection or malignancy
- Severe heart disease not suitable for correction
- Severe lung disease
- Reversible renal disease
- Uncontrolled substance abuse, psychiatric illness
- On-going treatment non-adherence
- Short life expectancy
induction treatment for transplanation
Immunosuppressive drugs to create tolerance of the graft
Methylprednisolone with any of the following:
- Basiliximab
- Thymoglobulin
maintenance treatment transplantation
Prevent acute rejection
- Steroids: prednisolone
- Calcineurin inhibitors (CNI): tacrolimus, cyclosporine, voclosporin
- Antimetabolite medications: mycophenolate, azathioprine
- T cell regulation: belatacept and belimumab
long term care transplantation
Regular follow ups
- GFR
- Calcineurin (CNI) levels
- Proteinuria
- Calcium, phosphate and PTH
- Lipids
- Glucose
- Screen for infections
- Vaccination (except live or live attenuated)
- Monitor and control CVD
- Screen for malignancies
- Contraception obligatory in first yes and counsel about pregnancy one year after
Transplantation: mortality related to
- Cardiovascular disease
- Infections
- Malignancy
Glomerulonephritis
Glomerulonephritis (GN) denotes glomerular injury and applies to a group of diseases that are generally, but not always, characterised by inflammatory changes in the glomerular capillaries and the glomerular basement membrane (GBM).
- nephrotic syndrome
- nephritis syndrome
Nephrotic Syndrome
- Pathology to the basement membrane of the glomerulus causes excessive loss of protein
- Most common between ages of 2 and 5
Pathophysiology nephrotic syndrome
When the basement membrane becomes highly permeable to protein, allowing proteins to leak from the blood into the urine
presentation of nephrotic syndrome
3 cardinal findings
* Low serum albumin <30
* High urine protein content (>3+ protein on urine dipstick)
* Oedema
Other symptoms
- Frothy urine
- Pallor
- Hypercholesteremia
- High BP
- Hypercoagulability- risk of blood clots
Complications of Nephrotic syndrome include
- Higher risk of Infection
- Venous thromboembolism - DVT
- Progression of CKD
- Hypertension
- Hyperlipidaemia
Causes of Nephrotic syndrome in children
1) Minimal Change Disease – most common form of GN in children
- >90% of cases in <10s
- Occurs in isolation- without any clear underlying cause
2) Secondary causes
- Focal Segmental Glomerulosclerosis – Idiopathic or secondary to infection, malignancy, drugs etc.
- Membranoproliferative Glomerulonephritis (more commonly presents as nephritic syndrome)
- Systemic illness
Henoch schonlein puprua (HSP)
Diabetes
Infection e.g. HIV, hep and mal
General management of nephrotic syndrome
- Corticosteroids i.e. prednisolone
o Given for 4 then gradually weaned over next 8 weeks - Low salt diet
- Diuretics may be used to treat oedema
- Albumin infusion if severe hypoalbuminaemia
- Antibiotic prophylaxis in severe cases
Minimal change disease
- Occurs in otherwise healthy children, without any clear risk factors
- Not clear why is occurs in most cases
Prognosis
- Most children make a full recovery
investigations for minimal change disease
Investigations
- Do not renal biopsy – usually not able to detect abnormality
o Causes 90% of cases so no need for unnecessary invasive tests
- Urinalysis
o Protein in urine
o Hyaline casts
management of minimal change disease
- Corticosteroids i.e. prednisolone
- Given for 4 then gradually weaned over next 8 weeks
If steroid resistant
- ACEi
- Immunosuppressants e.g. cyclosporine, tacrolimus
