Renal disease in a child (nephrotic syndrome, renal failure, acute glomerulonephritis) Flashcards

1
Q

List some conditions with a raised anion gap.

A
  • DKA
  • Renal failure
  • Poisoning with salicylate, ethanol/methanol, paraldehyde
  • Inborn errors of metabolism

NB: There will be a normal anion gap in:

  • Intestinal loss of base e.g. diarrhoea
  • Renal loss of base e.g. renal tubular acidosis types 1 and type 2
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2
Q

Which electrolyte is high in renal failure? What other blood investigations will be raised?

A
  • Potassium (normal range 3.5-4.7 mmol/L)
  • Plasma urea often starts rising before creatinine

Should measure: serum Cr, urea, electrolytes, acid-base status, calcium and phosphate.

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3
Q

Apart from renal failure, what are the other reasons for a raised urea?

A
  • High protein diet
  • Catabolic state
  • GI bleeding
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4
Q

What are the main non-radiological investigations for assessing kidney function in a child?

A
  • Plasma creatinine concentration
  • Calculating eGFR from formula using height and creatinine
  • Inulin or EDTA GFR
  • Creatinine clearance
  • Plasma urea concentration
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5
Q

What radiological investigations of the kidneys and urinary tract are useful in children?

A

US - visualises urinary tract dilatation, stones and nephrocalcinosis

DMSA - detects functional defect, nucelar scan

MCUG - contrast introduced into bladder through catheter

MAG3 renogram - isotope-labelled scan to measure drainage

Plain abdo XR - spinal abnormalities and stones mainly

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6
Q

How are the causes of AKI classified? Give examples of causes in each group.

A
  1. prerenal: the most common cause in children
  2. renal: there is salt and water retention; blood, protein, and casts are often present in the urine; and there may be symptoms specific to an accompanying disease [e.g. haemolytic uraemic syndrome (HUS)]
  3. postrenal: from urinary obstruction.
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7
Q

What is the brief management of AKI based on cause (prerenal/renal/postrenal)?

A

Prerenal failure - suggested by hypovolaemia, low fractional excretion of sodium is as the body tries to retain volume. Correct hypovolaemia urgently with _fluid replacement and circulatory suppor_t to avoid ATI and necrosis.

Renal failure - if circulatory overload, restriction of fluid intake and challenge with a diuretic to increase urine output sufficiently to allow gradual correction of sodium and water balance. A high-calorie, normal protein feed will decrease catabolism, uraemia, and hyperkalaemia. If the cause of renal failure is not obvious, a renal biopsy should be performed to identify rapidly progressive glomerulonephritis, as this may need immediate treatment with immunosuppression.

Postrenal failure - assess the site of obstruction and relieve by nephrostomy or bladder catheterization.

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8
Q

What are the two most common renal causes of acute renal failure in children

A

The two most common renal causes of acute renal failure in children in the UK are:

  • HUS
  • ATN

the latter usually in the setting of multisystem failure in the intensive care unit or following cardiac surgery.

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9
Q

How do you correct these metabolic abnormalities in renal failure?

A
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10
Q

When is dialysis indicated in AKI?

A

BMJ general indications for dialysis:

  1. intractable hyperkalaemia;
  2. acidosis;
  3. uraemic symptoms (nausea, pruritus, malaise);
  4. therapy-resistant fluid overload;
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11
Q

What is HUS?

A

Triad of:

  1. Acute renal failure
  2. Microangiopathic haemolytic anaemia
  3. Thrombocytopenia

Usually secondary to GI infection with verocytotoxin producing E. coli O157:H7 or sometimes Shigella. Follows a prodrome of bloody diarrhoea. Toxin enters the endothelial kidney cells and causes intravascular thrombogenesis so platelets are consumed.

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12
Q

What are the long-term complications of HUS if left untreated?

A

Persistent proteinuria

Hypertension

Progressive chronic kidney disease

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13
Q

How do you grade the severity of CKD?

A
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14
Q

How common is CKD in children?

A

10 per million (i.e. very uncommon)

Congenital and familial causes are more common in childhood than acquired causes

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15
Q

What are the clinical features of stage4/5 CKD in children?

A
  • anorexia and lethargy
  • polydipsia and polyuria
  • faltering growth/growth failure
  • bony deformities from renal osteodystrophy (renal rickets)
  • hypertension
  • acute-on-chronic renal failure (precipitated by infection or dehydration)
  • incidental finding of proteinuria
  • unexplained normochromic, normocytic anaemia
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16
Q

What is stage 5 CKD by definition?

A

eGFR <15 ml/min per 1.73m2

end-stage renal failure

renal replacement therapy required

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17
Q

What are the most common causes of CKD in children?

A

Cause - %

  • Renal dysplasia ± reflux - 34
  • Obstructive uropathy - 18
  • Glomerular disease - 10
  • Congenital nephrotic syndrome - 10
  • Tubulointerstitial diseases - 7
  • Renovascular disease - 5
  • Polycystic kidney disease - 4
  • Metabolic - 4
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18
Q

When do symptoms usually begin with CKD?

A

When renal function falls to less than one third of normal or CKD stage 4

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19
Q

How is CKD managed?

A

Aims to prevent symptoms, metabolic abnormaities and allow normal growth.

Diet - calorie supplements and NG tube, sufficient protein to maintain growth but not to accumlate toxix metabolic by-products

Phosphate restriction/binder and activated vitamin D - prevents renal ostedystrophy (from secondary parathyroidism)

Salt and water balance - may need salt supplements and water, with bicarbonate supplements to prevent acidosis

Recombinant human EPO - prevents anaemia and the toxic metabolites which circulate in anaemia and are toxic to BM

Hormonal abnormalities - GH resistance may be found which can be treated with GH up to 5yrs of age, delayed puberty, subnormal pubertal growth spurt

Dialysis and transplantation - all children can have RRT when CKD stage 5 is reached but transplantation is better (although weight of 10kg needs to be reached first to avoid renal vein thrombosis)

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20
Q

What are the chances of success with renal transplants in children in CKD?

A

Kidneys from live donors have better success rate than those from deceased and 5yr graft survival is 94% vs 86% respectively

21
Q
A
22
Q

List some causes of proteinuria

A
  • Orthostatic proteinuria
  • Glomerular abnormalities - minimal change disease, glomerulonephritis, abnormal glomerular basement membrane (familial nephritides)
  • Increased glomerular filtration pressure
  • Reduced renal mass in CKD
  • Hypertension
  • Tubular proteinuria
23
Q

What are the clinical features of nephrotic syndrome?

A
  • periorbital oedema (particularly on waking) which is often the earliest sign - heavy proteinuria results in a low plasma albumin and oedema
  • scrotal or vulval, leg, and ankle oedema
  • ascites
  • breathlessness due to pleural effusions and abdominal distension
  • infection such as peritonitis, septic arthritis, or sepsis due to loss of protective immunoglobulins in the urine.
24
Q

What are the causes of nephrotic syndrome?

A
  • The cause of the condition is unknown,
  • But a few cases are secondary to..,
    • Systemic diseases such as Henoch–Schönlein purpura and other vasculitides, e.g. SLE (systemic lupus erythematosus),
    • Infections (e.g. malaria)
    • Allergens (e.g. bee sting).
25
Q

Name 3 types of nephrotic syndrome.

A

Steroid sensitive nephrotic syndrome - 85-90% of cases

Steroid-resistant nephrotic syndrome - refer to paediatric nephrologist; will require ACEi and diuretic therapy and maybe genetic testing

Congenital nephrotic syndrome - presents in first 3 months of life; recessive inheritance

26
Q

What investigations should you do for nephrotic syndrome?

A
  • Urine protein – on test strips (dipstick)
  • Full blood count and erythrocyte sedimentation rate
  • Urea, electrolytes, creatinine, albumin
  • Complement levels – C3, C4
  • Antistreptolysin O or anti-DNAse B titres and throat swab
  • Urine microscopy and culture
  • Urinary sodium concentration
  • Hepatitis B and hepatitis C screen
  • Malaria screen if travel abroad
27
Q

What is the prognosis for nephrotic syndrome?

A

80-95% of cases of nephrotic syndrome are steroid-sensitive and do not progress to CKD

28
Q

What are the clinical features of steroid-sensitive nephrotic syndrome?

A
  • age between 1–10 years
  • no macroscopic haematuria
  • normal blood pressure
  • normal complement levels
  • normal renal function.
29
Q

What is a common cause of steroid-sensitive nephrotic syndrome?

A

Often precipitated bu respiratory infections

Association to atopy

30
Q

What is the management of steroid-sensitive nephrotic syndrome?

A

Oral corticosteroids (60mg/m2 per day of prednisolone) for 4 weeks, then reduction to 40 mg/m2 on alternate days for 4 weeks then weaned or stopped

31
Q

What is the prognosis with steroid-sensitive nephrotic syndrome?

A

No progression to CKD

Proteinuria resolves in ~11 days

  • 1/3 resolve directly
  • 1/3 have infrequent relapses
  • 1/3 have frequent relapses
32
Q

What should you do if a child presents oedematous?

A

Tests for proteinura

?nephrotic syndrome

33
Q

Why is nephrotic syndrome called minimal change disease?

A

Normal histology on light microscopy but fusion of the specialised epithelial cells that invest the glomerular capillaries (podocytes) are seen on electron microscopy

34
Q

What are the complications of nephrotic syndrome?

A

Hypovolaemia - intravascular comparment may become depleted during oedema; usually presents as abdominal pain and faintness

Thrombosis - hypercoagulable state, due to urinary loses of antithrombin III, thrombocytosis may also be exacerbated by steroids, increased blood viscosity, increased synthesis of clotting factors,

Infection - especially with encapsulated bacteria e.g. Pneumococcus. Risk of spontaneous peritonitis, chickenpox and shingles.

Hypercholesterolaemia - correlates inversely with serum albumin but not fully understood why

35
Q

How is severe oedema managed? What is a risk of treatment?

A

IV 20% albumin infusion + furosemide.

Care must be taken with the use of 20% albumin as it may precipitate pulmonary oedema and hypertension from fluid overload, and also with diuretics which can cause or worsen hypovolaemia.

36
Q
A
37
Q

What is the management for steroid-resistant nephrotic syndrome?

A
  1. Refer to paediatric nephrologist
  2. Manage oedema with diuretics, salt restriction, ACEi, and sometimes NSAIDs
  3. Genetic testing for possible causes
38
Q

What is the presentation and management of congenital nephrotic syndrome?

A

Rare cause that presents before 3 months; mostly recessively inherited and frequent in Finns or consanguineous families

High mortality usually due to hypoalbuminaemia rather than progressive CKD.

  1. May be so severe that unilateral nephrectomy may be necessary for control
  2. Followed by dialysis for stage 5 CKD which is continued until child is no longer nephrotic and old enough for renal transplantation
39
Q

List some causes of acute nephritis.

A
  1. Post-infectious (including streptococcus)
  2. Vasculitis (Henoch–Schönlein purpura or, rarely, SLE (systemic lupus erythematosus), Wegener granulomatosis, microscopic polyarteritis, polyarteritis nodosa)
  3. IgA nephropathy and mesangiocapillary glomerulonephritis
  4. Antiglomerular basement membrane disease (Goodpasture syndrome) – very rare
40
Q

What is the pathophysiology of acute nephritis?

A

Increased glomerular cellularity –> restricted glomerular blood flow –> decreased glomerular filtration –>

  1. decreased UO and volume overload
  2. HTN (may cause seizures)
  3. oedema which is initially periorbital
  4. haematuria and proteinuria
41
Q

What are the consequences of acute nephritis if left untreated?

A

Irreversible CKD over weeks or months

SO renal biopsy and subsequent treatment with immunosuppresson and plasma exchange may be necessary

42
Q

How is post-streptococcal nephritis diagnosed?

A
  1. Evidence of a recent streptococcal infection (culture of the organism, raised ASO/anti-DNAse B titres)
  2. Low complement C3 levels that return to normal after 3 weeks to 4 weeks.
43
Q

Where is post-streptococcal nephritis common? What is the prognosis?

A

Common in developing countries

Uncommon in developed countries

Long-term prognosis good

44
Q

What are the clinical features of Henoch-Schonlein purpura?

A
  • characteristic skin rash on extensor surfaces
  • arthralgia
  • periarticular oedema
  • abdominal pain
  • glomerulonephritis
45
Q

What is the epidemiology of Henoch-Schonlein purpura?

A

Ages 3-10yrs

boys: girls 2:1

peaks in winter months

46
Q

What is the pathophysiology of acute nephritis in Henoch-schonlein purpura?

A

Often preceded by an upper respiratory infection.

Despite much research, the cause is unknown.

It is postulated that genetic predisposition and antigen exposure increase circulating IgA levels and disrupt IgG synthesis.

The IgA and IgG interact –> complexes that activate complement –> deposited in affected organs –> precipitating an inflammatory response with vasculitis

47
Q

What are the features of renal invovlement in Henoch-Schonlein purpura? What is the management?

A
  • Rarely the first symptom on HSP
  • Over 80% have microscopic or macroscopic haematuria or mild proteinuria
  • Usually make a complete recovery but some may progress to nephrotic syndrome if severe
  • Should be followed for a year to see if proteinuria or haematuria persists, to prevent CKD and hypertension
48
Q

What is the most common familial nephrotis syndrome? What are the other features of this syndrome?

A

Alport syndrome - X linked recessive disorder that progresses to end stage CKD

Nerve deafness and ocular defects also occur

49
Q
A