Paediatric renal / GU medicine Flashcards

1
Q

What is the most common cause of nephrotic syndrome in children?

How is it diagnosed and what is the treatment?

A

Minimal change disease

Dx = biopsy and microscopy may be normal but urinalysis will show hyaline casts

Tx = corticosteroids

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2
Q

What is the patho/histology of nephrotic syndrome?

A

Basement membrane in glomerulus becomes permeable to protein so protein leaks from blood into urine.

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3
Q

What is the classic triad of nephrotic syndrome?

What are some additional symptoms?

A
  1. Low serum albumin
  2. Proteinuria (>3+ on dipstick)
  3. Oedema

other:

  • Deranged lipids
  • HTN
  • Hypercoagulability
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4
Q

What are some common secondary causes of nephrotic syndrome?

A

Intrinsic kidney disease:

  • Focal segmental glomerulosclerosis
  • Membranoproliferative glomerulonephritis

Systemic illness:

  • HSP
  • Diabetes
  • Infection - HIV, hepatitis, malaria
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5
Q

How is nephrotic syndrome managed?

A

High dose prednisolone 4 weeks then weaning. 80% resolves with steroids

  • low salt diet
  • ?diuretics
  • albumin infusions
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6
Q

What are the main complications of nephrotic syndrome?

A
Hypovolaemia
Thromobosis
Infection (kidneys leak Igs)
Renal failure
Relapse

CNS presentation - high mortality

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7
Q

What features would suggest steroid resistant nephrotic syndrome?

Possible causes?

What would you if atypical / unresponsive to steroids?

A

Elevated BP
Haematuria
Failure to respond to steroids

Possible causes:

  • Focal segmental glomerular scleroosis
  • Mesangiovapillary glomerulonephritis - haematuria
  • Membranous nephropathy - Hep B

–> renal biopsy

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8
Q

What are the main causes of nephritic syndrome?

A
  1. Post infection (Group A beta-haemolytic strep)
  2. Vasculitis - HSP or SLE
  3. IgA nephropathy
  4. Alport syndrome
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9
Q

What is nephritis / nephritic syndrome?

What are the main symptpoms?

A

Inflammation within nephrons of the kidney.

Haematuria
Proteinuria (less than nephrotic)
Decreased urine output
Impaire GFR

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10
Q

What are the 2 most common causes of nephritis in children?

A
  1. Post-streptococcal glomerulonephritis

2. IgA nephropathy (Berger’s disease)

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11
Q

What is post-strep nephritis?

How is a diagnosis made?

A

Follows strep throat (7-21 days later).
Immune complexes of strep antigens, antibodies and complement get stuck in glomeruli and cause inflammatin, causing AKI.

Dx = evidence of recent strep infection (Hx, throat swab, anti-streptolysin antibody titres)

Mx = supportive / general

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12
Q

What is the general treatment for nephritis?

A

Supportive
Low salt diet
Antihypertensives
Diuretics

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13
Q

What is IgA nephropathy / Berger’s disease?

A
  • Related to HSP
  • IgA deposits in nephron causing inflammation
  • More common in teenagers
    Dx = biopsy + histology
    Mx = supportive treatment and immunosuppressants/ steroids
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14
Q

What is Alport syndrome?

A

X-linked recessive
Progresses to end-stage CKD by early adulthood in males
Associated with nerve deafness and ocular defects
Mother may have haematuria

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15
Q

What are the most common causative organisms for UTI?

A
Klebsiella
E. coli
Proteus
Pseudomonas
Streo faecalis
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16
Q

What are the symptoms fo an upper tract UTI?

A
Fever / sepsis
Malaise
Vomiting
Loin/abdo pain in older children
Failure to thrive / jaundice in infants
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17
Q

What are the symptoms fr a lower tract UTI

A
Dysuria
Frequency / urgency
Incontinence
Lower abdo pain
Haematuria
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18
Q

What would dysuria with no other symptoms suggest?

A

Vulvitis (girls)

Balanitis (circumsised boys)

19
Q

What are some common abnormalities associated with uti?

A

VUR - vesico-ureteric reflux
Renal scarring
Stones

20
Q

What is the key feature of chronic pyelonephritis?

A

Histological / radiolgical diagnosis = cortex scarring and dilated calyx

Causes HTN and possible renal failure

21
Q

What are some atypical UTI features that would indicate an USS scan?

A
Poor urine flow
Mass
Seriously unwell
Raised creatinine
Septicaemia
Failure to respond to Abx WITHIN 48 HOURS
Infection with non e. coli
22
Q

What is the relationship between nitrites and leukocytes on urine dipstrick?

A

Nit + Leu = likely UTI

Only Nit = treat as UTI

Only Leu = do not treat as UTI unless clinical indication they have one

23
Q

What is the management for UTI in children?

A

IV ANTIBIOTICS IF:

  1. <3 months + fever (plus full septic screen)
  2. Features of sepsis / pyelonephritis

ORAL ANTIBIOTICS IF:
>3 months and otherwise well

Common abx = cetriaxone, trimethoprim, amoxicillin etc

24
Q

When is an USS scan indicated for UTI?

A
  1. All children under 6 months with first UTI within 6 weeks of infection
  2. Recurrent UTI - within 6 weeks
  3. Atypical UTI - during illness
25
Q

What is a DMSA scan used for?

A

Assess for damage 4-6 months after recurrent or atypical UTI

- uses radioactive material to assess kidney function

26
Q

What is Vesico-ureteric reflux?
What are the causes?

How is it diagnosed?

A

Urine flows back from bladder into ureters.
Causes:
- Familial
- 2nd to pathology (obstruction, neuropathic bladder)
- temporarily after UTI

Dx = Micturating cystourethrogram (MCUG)
Mild = only into ureter
Severe = gross dilatation
27
Q

What is the management for VUR?

A

Most resolves with age
Avoid constipation / full bladder
Prophlactic Abx
Surgery

28
Q

What is the difference between primary and secondary nocturnal enuresis?

A

Primary = child never achieved continence

Secondary = dry for at least 6 months previous

29
Q

Causes of nocturnal enuresis?

When is urinalysis indicated?

A
Genetic
Stress
Organic (uncommon)
- UTI
- Constipation
- Osmotic diuresis (diabetes, ckd)
- Developmental delay

– urinalysis if during day / infection / ill health

30
Q

What is the management of nocturnal enuresis?

A

Explain
Star chart
Enuresis alarm - wake child so they finish in toilet

Desmopressin in children over 7

31
Q

What are the main features of AKI?

A

Rapid rise in creatinine

Oliguria / anuria

32
Q

What are the main pre-renal causes of AKI in children?

A

Hypovolaemia

  • nephrotic syndrome
  • gastroenteritis, burns, sepsis, haemorrhage

Circulatory failure

33
Q

What are the main renal causes of AKI in children?

  • Vascular
  • Tubular
  • Interstitial
A

Vascular

  • Haemolytic uraemia syndrome
  • Vasculitis - SLE, HSP

Tubular

  • Acute tubular necroosis (burns, shocks, sepsis, crush injury)
  • Ischaemic, toxic, oobstructive

Interstitial
- Interstitial nephritis, pyelonephritis

34
Q

What are the main post-renal causes of AKI in children?

A

Obstruction

  • congenital
  • acquired - eg. blocked catheter
35
Q

What is the presentation of haemolytic uraemia syndrome?

A
  1. Haemolytic anaemia
  2. Thrombocytocytopenia
  3. AKI

Typical HUS = Associated with diarrhoea
Atypical HUS = no diarrhoea

36
Q

What are the signs of TYPICAL HUS?

What is the associated organism?

What is the clinical course?

A
  • summer months
  • children under 3 years

E.coli 0157 (shiga toxin)

1. Colitis / bloody diarrhoea
After 5 days:
- Reduced urine output
- Haematuria / dark urine
- Lethargy
- Confusino
- Oedema
- HTN
37
Q

What is the management for Haemolytic uraemic syndrome?

A

Emergency!
Supportive - antihypertensives, diuretics
Refer to renal paeds unit - may need dialysis oor blood transfusions

38
Q

What are the main renal malformations in children?

A
  • Epispadias
  • Horseshoe kidney - silent/obstructive uropathy / renal infections
  • Ectopic kidney - pelvic mass on USS
  • Renal agenesis - causes oligohydromnias, Potter’s Facies, and death if bilateral

Potter’s Facies = oligohydromnias –> intrauterine compression -> low set ears, beaked nose, epicanthic folds, lung hypoplasia – often stillborn

39
Q

What is Wilm’s tumour?

A

Nephroblastoma
Children <5 years

Features:

  • unilateral abdo mass = most common
  • painless haematuria
  • Flank pain, anorexia, fever, anaemia

Tx = nephrectomy, chemo, radio

40
Q

What is hypospadias?

A
Common abnormality
Abnormal position of external urethral meatus on distal ventral penis
3 features:
1. Ventral urethral meatus
2. Ventral curvature
3. Hooded appearance of foreskin

Complications - hard to direct urine, cosmetic, sexual dysfunction

41
Q

What is the most common polycystic kidney disease in children?

What are the main pathological features?

What is the genetic mutation responsible?

A

Autosomal recessive polycystic kidney disease
Presents in neonates, can be picked up on antenatal USS

Main pathology:

  • Cystic enlargement of collecting ducts
  • Oligohydromnias –> pulmonary hypoplasia, Potter syndrome
  • Congenital liver fibrosis

poor survival

Mutation = PKD1 no chromosome 6

42
Q

What is multicystic dysplastic kidney?

A

Different from PCKD - one cystic kidney and one normal kidney, diagnosed in antenatal USS

Cystic kidney will often atrophy and disappear by age 5

43
Q

What are the complications of undescended testes?

What are the risk factors?

A

Testicular torsion, infertility and testicular cancer

FHx, LBW, small for gest age, prematurity, maternal smoking

  • usually will resolve within 6 months or surgery by 12 months