Kidneys

Question 1

What is Potter’s syndrome?

Answer 1

A result of renal agenesis causing oligohydramnios

Features:
Facies:
	• Low-set ears
	• Beaked nose
	• Downward slanted eyes
Pulmonary hypoplasia 
Limb deformity

Question 2

What are the features of UTI in an infant?

Answer 2

Fever
Jaundice
Poor feeding
Febrile convulsions 
Vomiting
Offensive urine

Question 3

What are the features of UTI in a child?

Answer 3

Frequency
Dysuria 
Urgency
Abdominal pain
D&V
Haematuria 
Febrile convulsions
Enuresis

Question 4

What is vesicoureteric reflux and why is it important?

Answer 4

The ureters are displaced laterally and enter directly into the bladder, rather than an angle.

This can lead to backflow of urine from the bladder, back into the ureters

This is problematic because:

1) After voiding, the urine in the ureter will return to the bladder, leading to incomplete bladder emptying
2) If the child has a UTI, the infected urine may backflow to the kidney, leading to kidney damage and scarring

Question 5

How is vesicoureteric reflux investigated?

Answer 5

Initial ultrasound to identify potential obstruction or renal defects
Micturating cystourethrogram (MCUG) is best investigation

Question 6

What are features of atypical UTIs?

Answer 6

Sepsis
Crap urine flow
Abdominal mass
Raised creatinine 
Repeated UTI
Failure of Abx

Question 7

What are the investigations for UTI?

Answer 7

Urine dipstick if clinical suspicion

Urine culture if:
• Urine dipstick is leukocyte esterase or nitrite positive
○ If Nitrite +ve and leukocyte -ve, don’t start antibiotics unless there is good clinical evidence
• Child is <3 months
• Suspected upper UTI
• Risk of serious infection
• Recurrent infection

Question 8

What is the management of UTI in infants <3 months?

Answer 8

REFERRAL TO HOSPITAL
IV Antibiotics (Co-amoxiclav) for 5-7 days
Followed by oral prophylaxis

Question 9

What is the management of UTI in children >3 months?

Answer 9

If suspected upper UTI (Fever or loin pain):
Trimethoprim (PO) for 7 days

If suspected lower UTI:
Trimethoprim (PO) for 3 days

Question 10

What are the investigations for atypical UTIs?

Answer 10

• Ultrasound of the urinary tract to identify structural abnormalities
• DMSA and MCUG may also be performed in children < 6 months presenting

Question 11

What is daytime enuresis?

Answer 11

This is lack of bladder control during the day in a child old enough to be continent (over the age of 3-5). Daytime enuresis may also be accompanied by nocturnal enuresis.

Question 12

What are the causes of daytime enuresis?

Answer 12

Causes include:

• Lack of attention to the bladder.
• Detrusor instability - Sudden bladder contractions leading to feeling of urgency and enuresis.
• Neuropathic bladder - Enlarged bladder that fails to empty properly. Associated with spina bifida and other neurologic problems. There may be abnormal perianal sensation and anal tone, abnormal leg reflexes and gait.
• UTI (rarely in the absence of other symptoms)
• Constipation
• Ectopic ureter - Causes constant dribbling, and the child is always damp. Girls may be dry all night, but wet themselves when waking due to pooling of the urine in the vagina, secondary to ectopic ureter.

Question 13

How should daytime enuresis be investigated?

Answer 13

A urine sample should be examined for MC&S. Perform an examination to exclude neurologic causes.

Further investigations may be indicated, including urodynamic studies, ultrasound of bladder, X-ray of spine or MRI.

Question 14

What is the management of daytime enuresis?

Answer 14

○ Children in whom a neurological cause has been excluded, may benefit from:
§ Star charts
§ Bladder training
§ Pelvic floor exercises
○ Anticholinergic drugs (e.g. oxybutynin), which reduces bladder contractions, may be useful

Question 15

What is the most common cause of secondary enuresis?

Answer 15

Loss of previously attained bladder control

Most common cause is emotional upset

Other causes include UTI and polyuria from diabetes

Question 16

When is nocturnal enuresis a disorder?

Answer 16

Treatment is rarely undertaken for children younger than 5 years; at 5 years of age most children should be continent. Infrequent bedwetting is common in children. Bedwetting becomes a disorder when it is more frequent (2 or more times a week), which is present in 6% of 5 year olds, and 1.5% of 10 year olds

Question 17

What tends to cause primary nocturnal enuresis?

Answer 17

Rarely medical
Mixture of developmental delay and genetic factors which determine bladder control
2/3 of children with enuresis have a first-degree relative who has shared the issue.

Question 18

What is the management of nocturnal enuresis?

Answer 18

If child is <5 = Reassure that it should stop by the time they’re 5

If child is >5 and infrequent bedwetting (<2 times a week) = Watchful waiting, provide advice on reducing fluid intake before sleep, increase access to toilets, pee before sleep etc

If child is >5 and frequent bedwetting (>2/week) =
• Enuresis alarm with positive reward system
• Desmopressin

If short term relief is needed, prescribe desmopressin

If after 2 courses of treatment there is no response, refer to specialist

If there is nocturnal enuresis with daytime enuresis too, refer to secondary care

Question 19

What are causes of transient proteinuria?

Answer 19

Febrile illness
Exercise
Orthostatic (postural) proteinuria - Normally the result of a child being upright all day

Does not require investigation

Question 20

What are the causes of pathological proteinuria?

Answer 20

Minimal change glomerulonephritis
Glomerulonephritides
Increased GFR
Hypertension

Question 21

What are the clinical features of nephrotic syndrome?

Answer 21

Triad:
• Proteinuria
• Hypoalbuminaemia
• Oedema

Hyperlipidaemia and lipiduria
Earliest sign is periorbital oedema on waking
Breathlessness due to pleural effusion
Ascites

Secondary causes: HSP, SLE, infections (e.g. malaria) or allergens (e.g. bee stings

NOTE: Same presentation for minimal change nephropathy

Question 22

What is the management of nephrotic syndrome?

Answer 22

Oral steroids (Prednisolone) 
After 4 weeks, beginning reducing dose
If after 6 weeks, no improvement, renal biopsy is necessary

NOTE: Same management for minimal change nephropathy

Question 23

What are the complications of nephrotic syndrome?

Answer 23

• Hypovolaemia
○ Abdominal pain/Feeling faint
○ Treat with IV saline
• Thrombosis
○ Urinary loss of antithrombin III
○ Steroid therapy can worsen thrombocytosis
• Infection
○ Vaccinations against pneumococcus/ influenza recommended
○ Aciclovir treatment of chicken pox
• Hypercholesterolaemia
○ Inversely proportional to serum albumin

Question 24

What is the management of steroid-resistant nephrotic syndrome?

Answer 24

Oedema is managed with diuretics, salt restriction and ACE inhibitors

Unlike steroid sensitive, haematuria would be seen
Most common cause is focal segmental glomerulosclerosis

Question 25

How do you differentiate glomerular and lower urinary tract haematuria?

Answer 25

Glomerular - LUT Brown urine - Red urine Proteinuria - No proteinuria Glomerular has deformed cells LUT haematuria seen at start or end of urine stream

Question 26

What are the symptoms of Alport syndrome?

Answer 26

Kidney disease Hearing loss Eye abnormality X-linked recessive

Question 27

What investigations are conducted for haematuria?

Answer 27

``` ○ ALL patients § Urine MC&S § Protein and calcium excretion § Kidney and urinary tract ultrasound § U&Es, albumin § FBC, platelets, coagulation, sickle cell screen ``` ○ If suggestive of GLOMERULAR haematuria § ESR, complement and anti-dsDNA antibodies § Throat swab, antistreptolysin O/anti-DNAse B titres § Hepatitis B and C screen § Renal biopsy § Test mother's urine for blood (if Alport syndrome is suspected) § Hearing test (if Alport syndrome is suspected) ○ NOTE: indications for renal biopsy: § Significant persistent proteinuria § Recurrent macroscopic haematuria § Renal function is abnormal § Complement levels are persistently abnormal

Question 28

What are signs of acute nephritis?

Answer 28

In acute nephritis, increased glomerular cellularity restricts glomerular blood flow, and therefore GFR is decreased. This leads to: ○ Decreased urine output and volume overload ○ Hypertension (may cause seizures) ○ Oedema (initially periorbital) ○ Haematuria + proteinuria

Question 29

What are the features of post-streptococcal glomerulonephritis?

Answer 29

Develops 1-2 weeks after URTI infection (IgA nephropathy is 1-2 days) Diagnosed by evidence of recent streptococcal infection: • Anti-streptolysin O titre • Anti-DNAse B titres

Question 30

What are the clinical features of Henoch-Schonlein purpura?

Answer 30

• Purpuric & petechial rash over extensor surfaces ○ Most commonly the buttocks/legs ○ Symmetrical distribution • Joint pain and swelling (periarticular oedema) • Abdominal pain ○ Can cause melaena or haematemesis • Haematuria

Question 31

What is the management of Henoch-Schonlein purpura?

Answer 31

Spontaneously resolves after 4 weeks Joint pain managed with paracetamol/ibuprofen Severe oedema managed with oral prednisolone IV corticosteroids in those with nephrotic syndrome levels of proteinuria or declining renal function

Question 32

What are the features of IgA nephropathy?

Answer 32

Macroscopic haematuria Develops 1-2 days after URTI infection (Compared to post-strep which develops after 1-2 weeks) Managed the same as HSP

Question 33

What are the clinical features of haemolytic-uraemic syndrome?

Answer 33

Acute renal failure MAHA Thrombocytopaenia Typically triggered by gastrointestinal infection, most commonly E.coli infection. This normally presents with bloody diarrhoea The toxins from this infection travels directly to kidney endothelium, resulting in intravascular thrombogenesis. The coagulation cascade is activated, leading to platelet consumption. There is haemolysis due to the RBCs coming in contact with the thrombuses and breaking

Question 34

What is the management of haemolytic uraemic syndrome?

Answer 34

Hospitalisation Monitor urine output and hydration status Avoid antibiotics, anti-diarrhoeals, narcotic opioids and NSAIDs Monitor BP, treat with CCBs if elevated

Question 35

What are the clinical features of chronic kidney disease?

Answer 35

○ Anorexia and lethargy ○ Polydipsia and polyuria ○ Faltering growth/growth failure ○ Bony deformities from renal osteodystrophy ○ Hypertension ○ Acute-on-chronic renal failure (precipitated by infection or dehydration) ○ Incidental finding of proteinuria ○ Unexplained normochromic, normocytic anaemia

Question 36

What is the management of chronic kidney disease?

Answer 36

• Diet ○ Anorexia and vomiting are common ○ Calorie supplements or NG/gastrostomy feeding ○ Protein intake enough to maintain growth and albumin but preventing accumulation of toxic metabolic by-products • Prevention of renal osteodystrophy ○ Decreased activation of vitamin D leads to phosphate retention and hypocalcaemia. Leads to hyperparathyroidism and eventually osteitis fibrosa cystica and osteomalacia ○ Phosphate restriction (by reducing milk), using calcium carbonate as a phosphate binder and activated vitamin D supplements can help • Control of salt and water balance, and acidosis ○ Salt supplements and a lot of water ○ Treatment with bicarbonate supplements is needed to prevent acidosis • Acidosis ○ Reduced EPO production and circulation of marrow-toxic metabolites leads to anaemia ○ Responds well to the administration of SC recombinant human erythropoietin • Hormonal abnormalities ○ Growth hormone resistance, characterised by a high GH level but poor growth, is a feature of CKD ○ Recombinant human GH is effective in improving growth for up to 5 years of us ○ Many children with stage 4/5 CKD will have delayed puberty or subnormal pubertal growth spurt