Paediatric renal problems Flashcards

1
Q

What is a common antenatal presentation of renal disease?

A

Renal Pelvis dilatation

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2
Q

What are possible signs of the abnormal kidney (visible on investigations)?

A

– Echo bright

– Small

– Cystic

– Wrong place

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3
Q

What si clinical significance of renal congenital malformation? (3)

A
  • infections -> may cause renal damage
  • renal function (creatinine, BP, proteinuria)
  • growth and development
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4
Q

What’s that?

A

Cystic kidney disease

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5
Q

What’s CAKUT?

A

congenital anomalies of the kidney and urinary tract

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6
Q

What is a characteristic feature of renal hypodysplasia?

A

Renal hypodysplasia

  • small kidneys with a reduced number of nephrons and dysplastic features
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7
Q

What’s renal aplasia?

A

Renal aplasia (agenesis) – Congenital absence of kidney(s)

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8
Q

What’s congenital renal hypoplasia?

A

Congenitally small kidneys with a reduced number of nephrons but normal architecture

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9
Q

What’s renal dysplasia?

A

Renal dysplasia

  • the presence of malformed renal tissue elements, including primitive tubules, interstitial fibrosis, and/or the presence of cartilage in the renal parenchyma
  • dysplastic kidneys often contain cysts
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10
Q

What the birth weight tends to be in patients with congenital renal abnormalities?

A

The birth weight (BW) is often below the normal mean because of the association with intrauterine growth restriction (IUGR)

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11
Q

Possible presentaiton of congenital renal abnormalities in neonate

A

In the neonatal period, patients may present with one or more of the following:

●Pneumothorax

●Feeding difficulties

●Metabolic acidosis

●Urinary sodium losses

●Impaired renal function based on elevated serum/plasma creatinine level

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12
Q

Possible presentation of congenital renal disease in first year of life

A
  • anorexia
  • vomiting
  • failure to thrive
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13
Q

What’s possibl presentation of congenital renal disease ater 1st year of life

A
  • proteinuria
  • possible polyuria nad polydypsia
  • failure to grow
  • anaemia
  • osteodystrophy (secondary to hyperparathyroidism - as PTH tries to compensate for low Ca++ reabsorption at the tubules)
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14
Q

What renal abnormalities (in general) would indicate poor renal prognosis?

A

Patients with malformations involving a reduction in kidney numbers or size are most likely to have a poor renal prognosis

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15
Q

Causes of CAKUT

A
  • genetic abnormalities - mutations in some genes and syndromes
  • environmental factors -> malnutrition, exposure to teratogenic drugs
  • deficiency of vitamin A (will interfere with kidney development)
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16
Q

What do we need to monitor in unilateral kidney problems?

A

Follow-up care

  • the contralateral normal kidney is expected to undergo compensatory hypertrophy in patients with unilateral renal problems (e.g. renal agenesis)
  • serial ultrasonography is recommended to monitor for renal compensatory growth
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17
Q

If there is no compensatory hypertrophy (of normal functioning kidney), what further tests do we need to do?

A
  • If compensatory hypertrophy is not observed -> ongoing monitoring of the patient is recommended -> yearly assessment of blood pressure and urinalysis
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18
Q

What tests are recommended in patient with elevated BP and proteinuria?

A

In patients with elevated blood pressure or urinary protein excretion -> renal function should be assessed by obtaining a serum creatinine to estimate the GFR

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19
Q

Disruption of the normal embryologic migration of the kidneys may result in what conditions? (2)

A
  • renal ectopia (eg, pelvic kidney)
  • fusion anomalies (eg, horseshoe kidney)
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20
Q

What’s vesicoureteral reflux?

A

Vesicoureteric reflux (VUR)

  • abnormal backflow of urine from the bladder into the ureter and kidney
  • relatively common abnormality of the urinary tract
  • predisposes to urinary tract infection (UTI)
  • found in around 30% of children who present with a UTI
  • complications: 35% of children develop renal scarring it is important to investigate for VUR in children following a UTI
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21
Q

A child presents with UTI, what do we need to investigate for?

A

Vesicoureteric reflux (VUR)

  • abnormal backflow of urine from the bladder into the ureter and kidney
  • relatively common abnormality of the urinary tract
  • predisposes to urinary tract infection (UTI)
  • found in around 30% of children who present with a UTI
  • complications: 35% of children develop renal scarring it is important to investigate for VUR in children following a UTI
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22
Q

Pathophysiology of Vesicoureteric reflux

A

Pathophysiology of VUR

  • ureters are displaced laterally, entering the bladder in a more perpendicular fashion than at an angle
  • therefore shortened intramural course of ureter
  • vesicoureteric junction cannot therefore function adequately
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23
Q

Investigations in Vesico-ureteral reflux

A

Investigation

  • VUR is normally diagnosed following a micturating cystourethrogram
  • a DMSA scan* may also be performed to look for renal scarring

*DMSA scan = radionuclide scan to assess renal morphology

24
Q

What ‘big’ kidney on USS may indicate?

A

Polycystic kidney disease - kidney is filled with cysts/ above the centile that is expected for a child at given age

25
Q

DMSA test - what is it done for?

A

Injection of the die - to look at each kidney’s uptake of it and their shape

26
Q

MSUG test - what is it done for?

A

Dye is injected into the bladder through the catheter - to see if there is any backflow into the urinary tract

* normally bladder should be able to hold the dye, but if dye goes up through the urethra -> abnormal (posterior-ureteral valve)

*post-dilatation seen + abnormally shaped bladder (bumpy rather than smooth)

27
Q

What’s the diagnosis?

What happens/ how kidney would be damaged?

A

Vesicoureteric Reflux

Dye is backflowing to the ureters and kidney = so we assume urine is doing the same -> infections -> renal scarring

28
Q

What’s wrong with left ureter?

A

It is dilated

29
Q

What’s the abnormality?

A

Dye uptake is asymmetrical - problem with one of the kidneys

30
Q

A clinical trial of Nephrotic syndrome

A
  • proteinuria
  • low albumin
  • oedema
31
Q

What is the most common cause of nephrotic syndrome?

What do we treat it with?

A
  • Idiopathic nephrotic syndrome*
  • treat with steroids -> usually for minimum 8 weeks
32
Q

How do we measure proteinuria on the urinalysis?

A
33
Q

What is parental advice after we discharge a child home after nephrotic syndrome?

A

To do urinalysis daily, to measure the protein - in order to treat before they get oedema again

34
Q

Triad of Nephritic syndrome

A
  • haematuria (visible or on dipstick)
  • hypertension
  • high creatinine
35
Q

What’s the common cause of nephritis?

A

Henoch Schonlein Purpura

  • type of vasculitis -> inflamed blood vessels

*characteristic rash

* self-limiting course -> but may cause long lasting kidney damage

36
Q

What’s Henoch Schonlein Purpura?

A

Henoch-Schonlein purpura (HSP)

  • an IgA mediated small vessel vasculitis
  • HSP is usually seen in children following an infection
37
Q

Features of Henoch Schonlein Purpura

A
  • palpable purpuric rash (with localized oedema) over buttocks and extensor surfaces of arms and legs
  • abdominal pain
  • polyarthritis
  • features of IgA nephropathy may occur e.g. haematuria, renal failure
38
Q

Treatment and prognosis for Henoch Schonlein Purpura

A

Treatment

  • analgesia for arthralgia
  • treatment of nephropathy is generally supportive. There is inconsistent evidence for the use of steroids and immunosuppressants

Prognosis

  • usually excellent, HSP is a self-limiting condition, especially in children without renal involvement
  • around 1/3rd of patients have a relapse
39
Q

Stages of CKD

A
40
Q

What is the minimum height and weight for a child to be able to ‘fit’ transplanted adult kidney?

A

10-12 kg

90 cm

*renal dietician needed to help a child grow to the point when they can have a transplant

41
Q

What’s the 1st line of dialysis in younger children?

A

Peritoneal dialysis

  • this is because there is no need to use their blood vessels (like in haemodialysis)
42
Q

How does peritoneal dialysis work?

A
  • can be given overnight (8-12 hours) - 10 cycles of dialysis each night done while a child asleep
  • Dextrose fluid concentration - removes fluid by osmotic force (via peritoneal membrane)
43
Q

What’s that?

A

This child has extra feeds via NG tube

44
Q

What children is haemodialysis usually done in?

A

Over 10 kg

(this is for the circulation to cope well enough)

45
Q

What’s that?

A

Haemodialysis

46
Q

Typical location of renal transplant

A

R iliac fossa -> as transplant is usually implanted into iliac vessel

*on the picture an adult kidney is inserted into a child - massive amount of space taken - therefore a child need to be a certain weight to cope with it and enable the surgical wound clousure

47
Q

Where in the kidney do these diuretics work?

  • thiazide
  • potassium sparing
A
  • Thiazide - DCT
  • Potassium-sparing - collecting duct
48
Q

Clinical presentation of a patient with Proximal Renal Tubule problem

A

Problems due to tubules not being able to reabsorb the substances -> lost in the urine

  • acidosis -> biocarb are lost
  • aminoaciduria -> amino-acid loss
  • phosphoturia -> phosphate loss
  • glycosuria -> gluose loss
  • low molecular weight proteinuria
49
Q

What is cystinosis?

A

Inherited disorder -> cystine deposits within proximal renal tubule

  • failure to thrive
  • features of proximal renal tubule problems -> different electrolyte imbalance
50
Q

What is a syndrome that may cause problems in Loop of Henle?

A

Batter’s syndrome

  • mimics the effect of Furosemide
  • severe dehydration
  • loss of potassium in the urine
  • hyponatraemia due to dehydration
  • failure to thrive

-

51
Q

Gitelman’s syndrome

  • what part of the kidney is affected?
A

Gitelman’s syndrome = Distal Tubule Disorder -> mimics Thiazide diuretic effects

  • relatively mild in effects
  • low magnesium (as distal tubules have a role in Mg+ regulation)
  • dehydration may also be present -> due to water loss
52
Q

(3) disorders (names) that occur within the collecting duct

A
  • Pseudohypoaldosteronism
  • Liddle’s Syndrome
  • Nephrogenic Diabetes Insipidus
53
Q

What electrolyte abnormality would Pseudohypoaldosteronism present with?

A

Pseudohypoaldosteronism

  • salt wasting
  • high K+

*disorder in collecting duct

54
Q

What electrolyte abnormality is seen in Liddle’s syndrome?

A

Liddle’s syndrome

  • hypertension
  • low K+

* disorder in collecting duct

55
Q

What happens to the urine in nephrogenic diabetes insipidus?

A

Urine is dilute (not able to be concentrated)