Pediatric Nephrology Flashcards
How much of the cardiac output does the nephron in the neonate receive ?
20-30ml/min
What are the 5 functions of the kidney ?
Waste handling (Urea and creatinine) Water handli9Sodium and potassium)ng Salt balance Acid base balance Endocrine (Red cells/blood pressure/bone health)
In glomerular filtration what is the purpose of the endothelial cells ?
Fenestrated
Filter
Vulnerable to immune mediated injury
What 2 proteins are found in the basement membrane ?
Type IV collagen (COL4) and Laminin
What cells in the basement membrane of the glomerulus synthesize the proteins ?
Podocytes and endothelial cells
What is the role of mesangial cells in the glomerulus ?
They are smooth muscle cells within the basement membrane which are structural support of the glomerulus.
They regulate blood flow of the glomerular capillaries.
Proteinuria signifies what ?
Glomerular injury
What is the cause of Haematuria and what is the name of the syndrome ?
Increasing haematuria and intravascular overload - Nephritic syndrome
What is the cause of Proteinuria and what is the name of the syndrome?
Increasing proteinuria and intravascular depletion - Nephrotic syndrome
In an acquired Glomerulopathy which disease/condition attacks the epithelial ells (podocytes) ?
Lupus
Focal segmental glomerulitis
Minimal change disease (MCD)
In an acquired Glomerulopathy which disease/condition attacks the basement membrane ?
Membranous glomerulopathy
Membranoproliferative Glomerulonephritis.
Post infectious Glomerulonephritis.
In an acquired Glomerulopathy which disease/condition attacks the endothelial cells ?
Infection associated glomerulonephritis (PIGN), Haemolytic Uraemic Syndrome (HUS), Membranoproliferative Glomerulonephritis (MPGN), Lupus
In an acquired Glomerulopathy which disease/condition attacks the mesangial cells ?
Henoch-Schonlein purpura (HSP)
IgA nephropathy
Lupus
What is nephrotic syndrome?
Nephrotic range proteinuria which leads to Hypoalbuminaemia and finally oedema
What are the 3 ways in which we would test for proteinuria in children ?
Dipstix
Protein Creatinine Ratio (Early morning urine is best)
24 hour urine collection
What are the clinical signs and symptoms of nephrotic syndrome ?
Oedema
Proteinuria
Increased protein creatinine ratio
Low albumin
What are the typical features of minimal change disease?
Age 2-5
Normal BP
Resolving microscopic haematuria
Normal renal function
What are the atypical features of Minimal change syndrome ?
Suggestions of autoimmune disease
Abnormal renal function
Steroid resistance
What would be the treatment of minimal change disease if the child present with typical signs and symptoms ?
Prednisalone for 8 weeks
What are some of the symptoms to glucocorticoid treatment ?
GI side effects Glucose intolerance Hypertension Risk of adrenal crisis Growth issues
What is the pathological process of nephrotic syndrome ?
Interaction between lymphocytes and podocytes - The glomerular filtration barrier changes size (shrinks) .
What is the second line treatment for nephrotic syndrome ?
Immunosupression
What is a form of acquired steroid resistant nephrotic syndrome ?
Focal segmental Glomerulosclerosis (FSGS)
Podocyte loss
Progressive inflammation and sclerosis
What is a forms/s of congenital steroid resistant nephrotic syndrome ?
Presents <3 months of age
Mutations in nephrin and podocin genes
i.e. HPHS 1 and 2
What are the types of idiopathic nephrotic syndromes ?
Minimal change
FSGS
MPGN
What are the types of acquired nephrotic syndromes ?
HSP/IgA
Lupus
Post infectious
What are the clinical signs of glomerulonephritis ?
Haematuria and proteinuria Reduced GFR Oliguria Fluid overload (Raised JVP and oedema) Hypertension
What are the causes of GN ?
Post infectious GN HSP/IgA nephropathy Membraniproliferative GN Lupus nephritis ANCA +ve vasculitis
What are the causes of Acute Post Infectious Golmerulonephritis ?
Group A Strep (Throat)
Beta hemolytic
How do you diagnose acute post-infectious glomerulonephritis ?
Bacterial culture
Positive ASOT
Low C3 normaises
What is the treatment for acute post-infectious glomerulonephritis ?
Antibiotic
Support renal function
Overload/hypertension
What is the most common glomerulonephritis ?
IgA Nephropathhy
What are the clinical signs and symptoms of IgA nephropathy ?
Usually occurs 1-2 days after URTI Usually in older children and adults Recurrent macroscopic haematuria Chronic microscopic haematuria Varying degree of proteinuria
What is the pathogenesis/steps of IgA nephropathy ?
- Increasing IgA in circulation
- Production of Anti-IgA antibodies
3a. Immune complexes form in the circulation.
3b. Immune complexes form in situ - Immune complexesin the mesangium cause local activation and injury
How do you confirm the diagnosis of IgA nephropathy ?
Confirmation via biopsy
What is the treatment for IgA nephropathy ?
Mild disease - Proteinuria with ACEi
Moderate/severe disease - Immunosuppression
What is the clinical picture for a child with IgA related vasculitis ?
Age of onset 5-15 Mandatory palpable purpura and one of the following 4: 1. Abdominal pain 2. Renal involvement 3. Arthritis or arthralgia 4. Biopsy (IgA deposition)
What is HSP ?
IgA vasculitis with Nephritis
What is the treatment for IgA Vasculitis ?
Symptomatic (Joints and gut)
Glucocorticoid therapy
Immunosuppression
Hypertension and proteinuria screening
Define acute kidney injury (AKI)
abrupt loss of kidney function, resulting in the retention of urea and other nitrogenous waste products and in the dysregulation of extracellular volume and electrolytes
What are the 3 cardinal symptoms of an AKI ?
Anuria/oliguria
Hypertension with fluid overload
Rapid rise in plasma creatinine
What is the management of AKI ?
3 M’s
Monitor (Urine output)
Maintain (good hydration)
Minimise (Drugs)
What are the causes of AKI ?
Pre-renal
Perfusion problem
What are some of the intrinsic renal problems which cause AKI ?
Glomerular disease (HUS and GN) Tubular injury (Acute tubular necrosis) Interstitial nephritis (NSAID or autoimmune)
What is a post-renal cause of an AKI ?
Obstructive
When is Haemolytic-Uraemic Syndrome (HUS) typical ?
Post-diarrhoea (Caused by E.coli)
What are the presenting symptoms of HUS ?
Bloody diarrhoea Triad of: 1. Microangiopathic haemolytic anaemia 2. Thrombocytopenia 3. Acute renal faulire/AKI
What is the treatment of HUS ?
3 M's: 1. Monitor (5 kidney functions) Fluid balance Electrolytes Acidosis Hypertension 2. Maintain IV Normal saline and fluid Renal relacement therapy 3. Minimise No antibiotics
What are the long-term consequences of AKI ?
Blood pressure
Proteinuria monitoring
Evolution to CKD
What are the congenital causes of CKD ?
Congenital Anomalies of the Kidney and Urinary Tract (CAKUT)
Reflux nephropathy
Dysplasia
What kinds of investigations are appropriate in analyzing the kidneys ?
USS
DMSA (Isotope scan)
MAG 3 Scan
What is the treatment for lower tract infection of the urinary tract?
3 days PO antibiotics
What is the treatment for upper tract infections/pyelonephritis ?
Antibiotics for 7-10 days
How do we prevent urinary infections in children ?
Fluids
Hygiene
Relive constipation
Voiding dysfunctin
What is the management of CKD ?
Waste handling Water handling Salt balance Acid base control Endocrine
