Paediatric renal / GU medicine Flashcards by Laura Johnson

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

How is it diagnosed and what is the treatment?

Minimal change disease

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

Tx = corticosteroids

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What is the patho/histology of nephrotic syndrome?

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

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What is the classic triad of nephrotic syndrome?

What are some additional symptoms?

Low serum albumin
Proteinuria (>3+ on dipstick)
Oedema

other:

Deranged lipids
HTN
Hypercoagulability

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What are some common secondary causes of nephrotic syndrome?

Intrinsic kidney disease:

Focal segmental glomerulosclerosis
Membranoproliferative glomerulonephritis

Systemic illness:

HSP
Diabetes
Infection - HIV, hepatitis, malaria

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How is nephrotic syndrome managed?

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

low salt diet
?diuretics
albumin infusions

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What are the main complications of nephrotic syndrome?

Hypovolaemia
Thromobosis
Infection (kidneys leak Igs)
Renal failure
Relapse

CNS presentation - high mortality

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What features would suggest steroid resistant nephrotic syndrome?

Possible causes?

What would you if atypical / unresponsive to steroids?

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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What are the main causes of nephritic syndrome?

Post infection (Group A beta-haemolytic strep)
Vasculitis - HSP or SLE
IgA nephropathy
Alport syndrome

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What is nephritis / nephritic syndrome?

What are the main symptpoms?

Inflammation within nephrons of the kidney.

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

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

Post-streptococcal glomerulonephritis

2. IgA nephropathy (Berger’s disease)

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What is post-strep nephritis?

How is a diagnosis made?

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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What is the general treatment for nephritis?

Supportive
Low salt diet
Antihypertensives
Diuretics

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What is IgA nephropathy / Berger’s disease?

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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What is Alport syndrome?

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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What are the most common causative organisms for UTI?

Klebsiella
E. coli
Proteus
Pseudomonas
Streo faecalis

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What are the symptoms fo an upper tract UTI?

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

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What are the symptoms fr a lower tract UTI

Dysuria
Frequency / urgency
Incontinence
Lower abdo pain
Haematuria

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What would dysuria with no other symptoms suggest?

Vulvitis (girls)

Balanitis (circumsised boys)

What are some common abnormalities associated with uti?

VUR - vesico-ureteric reflux
Renal scarring
Stones

What is the key feature of chronic pyelonephritis?

Histological / radiolgical diagnosis = cortex scarring and dilated calyx

Causes HTN and possible renal failure

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

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

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

Nit + Leu = likely UTI

Only Nit = treat as UTI

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

What is the management for UTI in children?

IV ANTIBIOTICS IF:

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

ORAL ANTIBIOTICS IF:
>3 months and otherwise well

Common abx = cetriaxone, trimethoprim, amoxicillin etc

When is an USS scan indicated for UTI?

All children under 6 months with first UTI within 6 weeks of infection
Recurrent UTI - within 6 weeks
Atypical UTI - during illness

What is a DMSA scan used for?

Assess for damage 4-6 months after recurrent or atypical UTI | - uses radioactive material to assess kidney function

What is Vesico-ureteric reflux? What are the causes? How is it diagnosed?

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 ```

What is the management for VUR?

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

What is the difference between primary and secondary nocturnal enuresis?

Primary = child never achieved continence Secondary = dry for at least 6 months previous

Causes of nocturnal enuresis? When is urinalysis indicated?

``` Genetic Stress Organic (uncommon) - UTI - Constipation - Osmotic diuresis (diabetes, ckd) - Developmental delay ``` -- urinalysis if during day / infection / ill health

What is the management of nocturnal enuresis?

Explain Star chart Enuresis alarm - wake child so they finish in toilet Desmopressin in children over 7

What are the main features of AKI?

Rapid rise in creatinine | Oliguria / anuria

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

Hypovolaemia - nephrotic syndrome - gastroenteritis, burns, sepsis, haemorrhage Circulatory failure

What are the main renal causes of AKI in children? - Vascular - Tubular - Interstitial

Vascular - *Haemolytic uraemia syndrome* - Vasculitis - SLE, HSP Tubular - Acute tubular necroosis (burns, shocks, sepsis, crush injury) - Ischaemic, toxic, oobstructive Interstitial - Interstitial nephritis, pyelonephritis

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

Obstruction - congenital - acquired - eg. blocked catheter

What is the presentation of haemolytic uraemia syndrome?

1. Haemolytic anaemia 2. Thrombocytocytopenia 3. AKI Typical HUS = Associated with diarrhoea Atypical HUS = no diarrhoea

What are the signs of TYPICAL HUS? What is the associated organism? What is the clinical course?

- 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 ```

What is the management for Haemolytic uraemic syndrome?

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

What are the main renal malformations in children?

- 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

What is Wilm's tumour?

Nephroblastoma Children <5 years Features: - unilateral abdo mass = most common - painless haematuria - Flank pain, anorexia, fever, anaemia Tx = nephrectomy, chemo, radio

What is hypospadias?

``` 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

What is the most common polycystic kidney disease in children? What are the main pathological features? What is the genetic mutation responsible?

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

What is multicystic dysplastic kidney?

Different from PCKD - one cystic kidney and one normal kidney, diagnosed in antenatal USS Cystic kidney will often atrophy and disappear by age 5

What are the complications of undescended testes? What are the risk factors?

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