Brainscape's Knowledge GenomeTM

Browse over 1 million classes created by top students, professors, publishers, and experts.


See full index

Paeds > Renal > Flashcards

Renal Flashcards

1
Q

3 layers of filtration in Glomerulus

A
  1. Endothelium (glomerular capillary)
  2. Basement membrane
  3. Epithelium (podocytes that extend into BM)
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
2
Q

Haematuria Ddx

A

Glomerular (red cell casts, dysmorphic RBC, +/- Proteinuria)
- Glomerulonephritis - primary or secondary
PSGN/MPGN/RPGN/HSP/HUS
- Inherited GBM problem (Alports, Goodpastures, thin BM)
- Nephrotic

Non Glomerular

  • infective (UTI, Viral - Adeno/BK, TB, schisto)
  • Urological (stone/trauma/PUJO - pain)
  • tumour/vascular - Wilms
  • Sickle cell
  • Hypercalciuria (check urine ca/cr)
  • Renal V thrombosis
  • Cystic disease
  • Med (Aspirin, cyclophosphamide)
  • Vascular - nutcracker (compression left renal being between aorta and SMA), coagulopathy
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
3
Q

Haematuria investigations

A

FBC, U&E, Complement, Strep serology
ANA, Anti-dsDNA
Urine Protein:Cr ratio, urine calcium/cr ratio
Renal US

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
4
Q

Glomerulonephritis

A

Post infectious GN
IgA / HSP
SLE
RPGN (ANCA +ve, Anti-GBM, HSP - crescents on biopsy)
Inherited collagen disorders
- Alports (80%XLR COL4A5 gene, defect type 4 collagen)
- Thin GBM disease (benign familial haematuria)

HUS (Oliguria / anuria ; MAHA, thrombocytopenia)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
5
Q

Onset PSGN

A

7-14 days after throat infection

3-6 weeks after skin infection

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
6
Q

Timing of symptom resolution PSGN

A 
Gross haematuria - 2 weeks
Hypertension - 4 weeks
Low C3 - 8 weeks
Persistent proteinuria - 6 months
Intermittent proteinuria - 12months
Microsopic haematuria - 2 years
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
7
Q

Hypocomplementaemic GN

A

Post infectious GN
MPGN
SLE (low c3 and c4)
Shunt nephritis

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
8
Q

GN with normal complement

A 
IgA Nephropathy
HSP
ANCA vasculitis
Alports
Goodpastures
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
9
Q

Post infectious GN

A

Age 2-12

Onset 1-2 weeks post Strep throat
3-6 weeks post strep skin infection

Low C3 with normal c4 ; complement normalises by 8 weeks

IF - deposit C3 and IgG, granular deposits

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
10
Q

HUS (typical / D+ HUS)

A

most common type >90%

  • Caused by shiga toxin producing E.Coli 0157:H7 or shigella ; most frequently <3yrs age
  • Rare can be caused by severe invasive pneumococcal disease in patients <18months ; pneumomococcal meningitis or pneumonia with empyema. COOMBS +ve

Triad
AKI
Thrombocytopenia
MAHA (low Hb with red cell fragments (normal Fibrinogen differentiates from DIC), Low Hb, high retics, high LDH, low haptoglobin

Clinical
Diarrhoea by D3
Bloody diarrhoea by D5 (in 80%)
10-15% patients with STEC develop HUS by D7

Poor prognostic factors
Haemoconcentration
Neutrophilia or leucocytosis
CNS disease (10-15% get seizures ; Intracranial haemorrhage)
Haemorrhagic colitis

50% require RRT
5-10% ESRD
5-7% mortality
20-60% have long term HTN, Proteinuria, CKD

IVF in first 4 days illness reduce risk severe
Abx and Loperamide increase risk severe HUS

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
11
Q

Atypical HUS

A

Complement mediated HUS - familial or genetic
<10% HUS ; alternative complement pathway overactivaton

Recurrent episodes of haemolysis and renal failure

Rx - plasmapheresis +/- FFP

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
12
Q

IgA nephropathy

A

Abnormally glycosylated IgA1 which forms immune complexes in glomerular mesangium

Recurrent gross haematuria ; occurs within 5 days of URTI (synpharyngitic)
Can progress to ESRD

IgA mesangial deposits on IF
HSP nephritis looks same as IgA on biopsy

Normal complement
<20% have elevated IgA

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
13
Q

HSP - small vessel vasculitis

A

Occurs age 3-15years ; M:F=2:1
Deposition IgA in glomeruli

Skin(100%): Symmetrical purpura lower limbs
Joint(80%) - oligoarthritis
GI (50-75%) Abdo pain ; risk intussusception
Renal (20-60%) - Micro haematuria, gross haematuria, hypertension, nephritic/nephrotic
Orchitis (25%boys)
CNS(2%) - seizures

Renal involvement occurs within 2 months of disease
1/3 <2weeks symptoms
1/3 2-4weeks symptoms
1/3 >4weeks symptoms

2/3 recur (within 4 months)
Urinalysis weekly for 3/12 then monthly for 6/12
2% risk nephritis after 2/12

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
14
Q

MPGN

A

Primary
Secondary - to Hep B/C, shunt nephritis, SBE

Type 1, most common ; IF C3+ve, IgG-ve
Type 2 - dense deposit disease

2nd decade life

4 presentations

  1. Nephrotic (40-70%)
  2. Acute Nephritic (20-30%)
  3. Asymptomatic proteinuria, haematuria (20-30%)
  4. Recurrent gross haematuria
Low c3 (remains low)
Idiopathic MPGN, poor prognosis, 50% ESRD 10 years after diagnosis
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
15
Q

RPGN

crescents seen on biopsy

A

ANCA +ve (GPA/wegners =cANCA PR3 ; granulomas, URTI, sinusitis, saddle nose ; MPA=pANCA MPO ILD; pauci immune GN -no immune deposits)

HSP
Goodpastures (antibodies to type 4 collage - pulmonary haemorrhage and GN)

Rx immunosuppression
-Steroids, cyclophosphamide, rituximab

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
16
Q

Alports

A

Mutation type 4 collage

X linked 80% (COL4A5 mutation)
Others AD or AR

Recurrent microscopic haematuria
Kidneys: ESRD in early 20s
Hearing: SNHL males adolescence (50%by 25)
Eyes: Anterior lenticonus pathognomonic, only present 25%

Basket weave appearance GBM (laminated)
ACEi reduces risk ESRD

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
17
Q

Indications Dialysis (AEOIU)

A 
Acidosis / Ammonia
Electrolyte (High K)
Ingestion toxic
Overload (pulmonary oedema)
Uraemia
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
18
Q

Insensible losses neonates

Older kids

A 
<1500g = 30-60ml/kg/day
1500-2500g = 15-35ml/kg/day
>2500 = 15-25ml/kg/day

Baby - 40ml/kg/day
Infant - 30ml/kg/day
Preschool 20ml/kg/day
> 5 yrs 15ml/kg day

Older kids 400ml/m2/day

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
19
Q

Evaluation Paediatric HTN - MONSTER

A 
Medications - steroids, Tac/ciclosporin, OCP, Ritalin
Obesity
Neonatal Hx - umbi lines, asphyxia
Symptoms/signs
Trends in family
Endocrine
Renal
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
20
Q

Lupus nephritis

A

More common girls 9:1 ; Indian/maori/pi

Lymphopenia
Low C3&C4

Nephritis is commonest presentation
Grades 1-5
RENAL Biopsy needed for confirmation

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
21
Q

Proteinuria

Glomerular vs tubular

A

Glomerular - increased filtrations macromolecules such as albumin. Urine ACR (Urine A:P ratio >0.4)
Rx ACEi

Tubular - LMWP, smaller proteins not getting reabsorbed by tubules
Urine A:P ration<0.4 ; have normal serum, albumin
Causes Proximal tubule dysfunction
- Drugs (Cisplat, aminoglycosides, anticonvulsants)
- Cystinosis (lysosomal storage disorder, renal issues 3-6mo age, Vit D resistant rickets and PO4 wasting
- LOWE
- DENT - XLR nephrolithiasis / stones / hypercalciuria
-Wilsons
- ATN, reflux nephropathy, PCKD, TIN

Dipsticks mostly detect albumin
False +ves - alkaline urine, concentrated, blood/pyuria
False -ves - LMW proteinuria, dilute, polyuria

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
22
Q

Nephrotic range proteinuria

A

Urine protein: creatinine ratio >200mg/mmol
>40mg/m2/hr protein

Normal protein excretion = <4mg/m2/hr (150mg/day)
Normal PCR <23 mg/mmol

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
23
Q

Nephrotic syndrome

A

Nephrotic range proteinuria
Low albumin (<25)
Oedema
Hyperlipidemia

Cause
Primary
- MCD most common 75%, average age 2.5yr ; effacement of podocytes
-FSGS. 7%, average age 6yrs (50% have HTN and gross haematuria)
-MPGN
-Membranous (Hep B)

Systemic - SLE/HSP
Syndrome - Denys drash (assoc DSD), Nail patella , Frasier, Pierson

Biopsy if steroid resistant or red flags at presentation such as <12months or >12 years diagnosis, HTN, gross haematuria, persistent renal insufferable

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
24
Q

Nephrotic definitions

A 
Remission= Trace on dipstick for 3 consec. days(PCR<30)
Relapse = 3+ on dip for 3 consec days (PCR>200)

Freq relapse = >2 in 6mo or >4 in 12mo

Steroid dep = 2 consec relapses within 2/52 stopping steroid or whilst on steroids

Steroids Resistant = no remission after 4 weeks on 60mg/m2/day

80%respond to steroids

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
25
Q

Steroid sensitive nephrotic syndrome

A

25% no relapse
25% infrequent relapse
50% (FR/SD/SR)

75% relapse so need urine monitoring
Alternative - steroids - > cyclophosphamide -> MMF–> CSA/Tac - > Ritux

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
26
Q

FSGS outcome

A

1/3 improve
1/3 persistent proteinuria
1/3 ESRD by 5 years

1/3 idiopathic have recurrent disease in transplant

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
27
Q

Nephrotic syndrome complications

A

HTN (FSGS + MPGN > MCD)
Hypovolaemia
Thrombosis (DVT)

Infection - low IgG ; encapsulated organisms
Primary peritonitis - strep pneumo 60-75%
Disseminated VZV/measles
Cellulitis

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
28
Q

Congenital nephrotic syndrome

A

present <3months - 90% genetic
present 3-12mo(Infantile) - 50% genetic

NPHS1 or NPHS2 - Finnish type, AR
WT1 - Denys drash (nephrotic, DSD, risk wilms)
Nail patella syndrome
Pierson - LAMB2 gene mutation - bilateral microcoria(fixed narrowing of pupil)

Antenatal may have elevated AFP

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
29
Q

Autosomal recessive polycystic kidney disease

A

AR - 1:10,000-40,000 ; PKHD1 gene
Cystic dilations collecting ducts
Hepatic fibrosis

Antenatal - oligohydramnios, causing pulmonary hypoplasia ; echogenic kidneys
Large palpable renal masses
HTN
Renal impairment (normal in 20%)
Intrahepatic bile duct dilation (Caroli syndr), can cause portal HTN and hepatomegaly, risk varies and cholangitis

Rx supportive
30% die neonatal period due to pulmonary hypoplasia

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
30
Q

ADPKD

A 
Mutation PKD1(85%)/PKD2(15%) ; 1:1,000
Variable phenotype
-Asymptomatic children, onset 20-30s often
-HTN, proteinuria, HTN
Renal US: Macroscopic cysts

Assoc with cerebral aneurysms and SAH and cysts in brain/liver/pancreas ; MVP

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
31
Q

Nephronophthisis

A

Autosomal recessive disorder, 1:80,000
Tubular cysts

Polyuria, growth delay, anaemia
Cause ESRD children and adolescents

Associated findings
Retinal degeneration (Senior loken synd)
Cerebellar ataxia (Joubert)
Hepatic fibrosis (Boichis)
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
32
Q

Conditions associated with renal cysts

A
  1. Tuberous Sclerosis ( AD ; TSC1 OR TSC2 gene ; angiomyolipomas 80%, cystic disease 20%, risk RCC)
  2. VHL (AD, Cysts, renal cell ca)
  3. Bardet-Biedl (AR, obesity, syndactyly)
  4. NF1
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
33
Q

Hypertension

A

normal BP <90th gentile for age or <120/80 for >13yrs

Elevated BP 90-<95th centile fo age or 120-129/80 for >13

Stage 1 HTN 95th-95+12mmHg or 130-139 / 80-90

Stage 2 HTN >95+12mmHg or >140/90

HTN urgency = Stage 1 +30mmHg ( >180/120 >13yr)

HTN emergency = Stage 2 HTN with symptoms/end organ damage

Symptoms - encephalopathy, seizures, headaches, epistaxis, vomiting, hemiplegia, diplopia, lethargy

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
34
Q

HTN Treatment

A

Elevated BP: Lifestyle modification (diet, exercise, sleep) ; repeat 6/12

Stage 1: :lifestyle, recheck 2/52 ; check upper/lower limb ; if still elevated at 3rd visit ABPM monitor

Stage 2 - check upper and lower, Lifestyle, recheck 1 week ; if still high, ABPM, renal referral

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
35
Q

Ddx HTN by age

A

<1month

  • Renal artery thrombosis
  • CoA
  • Congenital renal disease (PUV, Renal hypoplasia or dysplasia, Reflux nephropathy, Cystic disease)
  • BPD

1mo-6years

  • renal parenchymal disease (GN, PKD, TIN, Renal scarring or dysplasia)
  • Renovascular (? umber lines, neonatal asphyxia)
  • CoA

> 6years

  • Essential HTN
  • Renal parenchymal and renovascular
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
36
Q

HTN workup

A

ABPM
Urine - blood/protein/WCC ; metanephrines
Bloods - U&Es, catecholamines, TFTs, Cortisol, Renin/aldosterone
US kidneys +/- doppler

Organ damage
Echo for LVH (ECG not recommended)
Retinopathy - opthal
Microalbuminuria

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
37
Q

HTN treatment

A

ACEi - first line in DM, CKD, proteinuria ;
CI if renal after stenosis
CCB - avoid in PKD
Thiazides

38
Q

Nephrotic Management

A 
Fluid restrict
IV Albumin if hypovolaemia, severe oedema
Low salt diet
Penicillin prophylaxis
Steroids
-60mg/m2/day x 4 weeks
- 40mg/m2 alt days x 4 weeks
- wean over the next 4-6 weeks (3/12 total)

Relapse - 60mg/m2/day until remission and then 40mg/m2 alt days xs28days then stop

second line
Steroid resistant - CSA / tac, then Ritux
Steroid resp - consider cyclophosphamide >mmf>ritux

39
Q

AKI - failure of kidneys to regulate water and electrolytes

Hyperkalemia / HTN/ Oedema

A

Pre-renal

  • Low FeNa (<1%), Urine Na <20
  • True volume depletion (bleeding, GI/Skin loss)
  • Effective renal hypo perfusion: Hypotension - CHF, Septic shock, cirrhosis

Intrinsic (FeNa >1%, urine Na>40)

  • Vasc - renal V thrombosis
  • GN
  • Interstitial - pyelo ; Tubulointerstitial nephritis
  • Tubular - ATN (Aminoglycosides, tumor lysis,)

Post renal (obstructive)

  • to cause AKI needs to be bilateral
  • Stones / trauma / clots / tumour
  • Bladder outlet obstruction
40
Q

AKI management

A

Treat cause
Fluid overload is independent mortality RF
Fluid management - insensible losses + urine output

Nutrition - prevent catabolism, electrolyte control

41
Q

Causes Paediatric CKD

A

Glomerular (oliguria, haematuria, proteinuria, HTN)

  • Chronic GN
  • FSGS
  • Congenital nephrotic
  • Alports
  • HUS
  • Cortical necrosis

Non glomerular
- Reflux nephropathy
- Hypoplasia/dysplasia
- Cystic - PKD, nephronopthisis
- Obstructive uropathy (PUV, prune belly, neurogenic bladder - UTIs, Type 4 RTA, pseudohypoaldosteronism - hyperkalemia, hyperchloraemic acidosis)
- Tubular / tubuloinstestital disorders - polyuria
eg cystinosis - fair skin, blue eyes, phosphate wasting

42
Q

Metabolic bone disease / renal osteodystrophy

A

Reduced 1 alpha hydroxylase activity caused reduction in active vitamin D

  • leads to reduced calcium ; hypocalcemia stimulates PTH production (PTH increases calcium reabsorption and PO4 excretion)
  • Excess PTH causes bone resorption

Also GFR falls, reduced PO4 excretion - leads to hyperphosphatemia which further causes hypocalcemia and increased PTH

Weakness, bone pain, fractures ; Rickets in growing children

Rx: Activated Vitamin D (calcitriol) suppresses PTH (monitor for hyeprcalcemia)

43
Q

Multicystic dysplastic kidneys

A 
1/4000 live births ; M:F= 2:1
Unilateral non functional cystic mass
No identifiable parenchyma or renal shape
Atretic proximal ureter
Often involute if <5cm by 2yrs

Often get VUR in contralateral kidney (25%)

44
Q

Acute interstitial Nephritis

A

immune mediated infiltration kidney interstitium by inflammatory cells

Hypersensitivity to Drugs - NSAIDs, penicillins, cephalosporins, sulphonamides
(Occurs 1-2 weeks after exposure)

Autoimmune with uveitis (TINU)
Infection - HIV, Hep B

Polyuria, often have rash/fever
Urine: White cell casts ; eosinophils

45
Q

Side effects Calcineurin inhibitors

Tac / CSA - inhibit T cell activation

A

Ciclosporin: Nephrotoxic, HTN, gingival hyperplasia, hirsutism, hyperuricemia, high cholesterol

Tacrolimus: tremors, low magnesium, diarrhoea, alopecia, DM

46
Q

Peritoneal Dialysis

A

Indications AEOIU

Increase ultrafiltration

  • Increase dwell/fill volume
  • increase no cycles
  • increase glucose concentration dialyse

Risk PD peritonitis ; fluid >100WCC
CONS>Staph aureus > strep > pseudo
Rx IP Abx x 3 weeks

47
Q

Paediatric AKI

pRIFLE

A

Risk: Reduce GFR by 25% ; UO <0.5ml/kg/hr x 8 hours

Injury: Reduce GFR 50% ; UO <0.5ml/kg/hr x 16hours

Failure: Reduce GFR by 75% ; UO <0.3ml/kg/hr 24hours or anuria for 12 hours

Loss : Persistent failure for >4weeks

End stage: Persistent failure >3mo

Stage 1: Cr 1.5x baseline
Stage 2: Cr 2-3x baseline
Stage 3: Cr >3x baseline

Neonatal AKI, serum cr>130

48
Q

VUR

A

Diagnosed on MCUG
30% patients who have had febrile UTI
5-15% infants with congenital hydronephrosis

  1. Reflux into non dilated ureter
  2. Reflux into upper collecting system with no dilation
  3. Reflux into dilated ureter +/or blunting of calyces fornices
  4. Reflux into grossly distended ureters
  5. Massive VUR with significant ureter dilation and tortuosity, loss of papillary impression
High Pressure = reflux on passing urine
Low pressure = reflux on bladder filling
Genetic component (AD with variable penetrance)
49
Q

Renal imaging

A

US - anatomy and cysts

MCUG - assess VUR and PUV

DTPA - dynamic scan, assess function - use for investigation of obstruction follow up post op for dilated kidneys. Excreted by GFR

MAG3 - assess function. Secreted by tubules

DMSA - static scan, assess cortical scars

50
Q

Mycophenolate mofetil (Cellcept)

A

antimetabolite, blocks purine synthesis, inhibits proliferation B/T cells

SE Blood (cytopenias), GI (N+V), dose related SE

51
Q

Medications that elevate Tacrolimus levels and risk toxicity

A 
Macrolides
Fluoroquinolones (Cipro)
NSAIDs
Antifungals
Omeprazole
52
Q

Medications that lead to reduction Tacrolimus levels and risk rejection

A

AEDs

Rifampicin

53
Q

FeNa

A

urine na x serum cr / urine cr x serum na

FENa <1% suggests pre renal AKI, a FENa above 2% suggests ATN, a FENa between 1 and 2% is non-diagnostic.

54
Q

ATN urine results

A

Casts
Low osmolality
<40 urea/cr ratio
High Na

55
Q

Chloride in urine

A

High in diuretic abuse, Bartters, Gitelmans

Low with vomiting and laxative abuse

56
Q

Six causes of allograft dysfuncTION

A 
DehydraTION
MedicaTION
InfecTION
ObstrucTION
RejecTION
PrefusION problem
57
Q

Normal anion gap metabolic acidosis (not due to diarrhoea) causes

A

RTA
Type 1: unable to excrete H+ (urine mor alkaloid making a higher urine pH) low hCO3 level
Type 2: reduced bicarb reabsorption (assoc w Fanconi syndrome), acidic urine (no bicarb buffer)
Type 4: hyperkalaemia major feature, lose sodium in urine and keep potassium from mineralocorticoid def/insensitivity (hx obstructive uropathy or due to adrenal disease)

58
Q

Bladder capacity child

A

(Age x 30) + 30

59
Q

Nephrogenic DI

A

90% are mutations in the ADH receptor (AVPR2)
gene; X‐linked.
• Over 180 mutations so variable affects.
• Remaining 10% are mutations in the aquaporin 2
(AQP2) channel; autosomal recessive/dominant

60
Q

What are the causes of an increased AG metabolic acidosis?

A

MUDPILES Methanol, Uremia, DKA, Paraldehyde, Iron or Isoniazid, Lactate, Ethylene Glycol, Salicylates

61
Q

What are the renal manifestations of tuberous sclerosis?

A
  • Renal angiomyolipomas 80% (if >4cm tx sirolimus)
  • Cystic disease 20%
  • Renal cell carcinoma <1%
62
Q

What is the pRIFLE criteria?

A

Used for AKI

  • Risk - decr GFR by 25%, <0.5ml/kg/hr for 8 hrs
  • Injury - decr GFR by 50%, <0.5ml/kg/hr for 12 hrs
  • Failure - decr GFR by 75%, anuric 12hrs
  • Loss - persistent failure > 4 weeks
  • End stage - persistent failure > 3 months
63
Q

What is the 10-4 rule with albumin?

A

A 10g/L drop in albumin leads to a 4mmol/L drop in the anion gap

64
Q

nephrocalcinosis

A
  • USS: diffuse speckled calcification
  • Calcification of renal tissue
  • Causes of nephrocalcinosis: distal RTA, ex-prems (frusemide, steroids), Vit D treatment for phosphatemic rickets, oxalosis
65
Q

What are the causes and complications of neurogenic bladder?

A
  • Spina bifida, sacral agenesis (maternal diabetes), tumour, trauma, transverse myelitis
  • Can lead to renal damage, incontinence, UTI (incomplete emptying), high pressure VUR, progressive renal scarring, detrusor-sphincter dyssynergia (leading to bladder hypertrophy and trabeculation, hydronephrosis), atonia (large, chronically distended, poor emptying)
  • Ix: video urodynamic assessment, kidney function/scarring
  • Tx: bladder relaxation with oxybutynin if unstable contractions, and clean intermittent catheterisation, augmentation cystoplasty (larger capacity, low pressure)
66
Q

What are the dietary considerations in CKD?

A
  • Aggressive nutrition, many need NGT/PEG
  • Generous H20 intake, Na+ supps (wasting in polyuria) or restriction (if oedema + water retention)
  • No protein restriction
  • Phosphate restriction, use of phosphate binders (calcium carbonate) to control secondary hyperparathyroidism
  • Restriction of potassium (fresh fruit, potatoes)
  • Sodium bicarb supps for acidosis
67
Q

Lowe syndrome (oculocerebrorenal syndrome of Lowe)

A
  • X-linked
  • Congenital cataracts, mental retardation, and Fanconi syndrome
  • Mutations in the OCRL1 gene, abnormal transport of vesicles within the Golgi apparatus
  • Present in infancy with cataracts, progressive growth failure, hypotonia, and Fanconi syndrome
  • Significant proteinuria is common
  • Blindness and renal insufficiency often develop
  • Characteristic behavioral abnormalities: tantrums, stubbornness, stereotypy (repetitive behaviors), and obsessions
  • There is no specific therapy for the renal disease or neurologic deficits
68
Q

features and causes of Fanconi syndrome

A

Diffuse proximal tubular dysfunction leading to excess urinary loss of:

  • Glucose - glycosuria, normal BSL
  • Phosphate - low phos, low TRP, rickets
  • Amino acids - no obvious consequence
  • Bicarb - proximal RTA
  • K+ - hypokalaemia
  • Na, Cl, H2O - polyuria, polydipsia, chronic decr ECF volume, faltering growth
  • Tubular proteins - LMW e.g. retinol-binding + N-acetylglucosamine

Polyuria, polydipsia, faltering growth, constipation, rickets

  • Causes:
  • Metabolic - cystinosis, tyrosinemia, Lowe syndrome (oculocerebrorenal syndrome)
  • Galactosemia
  • Wilson disease
  • heavy metal toxicity (lead, mercury, cadmium)
  • idiopathic, ifosfamide, cisplatin, azathioprine
  • ATN, tubulointerstitial nephritis
  • Tx: replacement of fluid, bicarb
69
Q

Discuss X-linked hypophosphatemic rickets

A
  • Vitamin D resistant rickets
  • Mutation in PHEX gene on X-chromosome
  • Defect in phosphate resorption, low TRP (<85%), normal PTH and calcitriol level, hypophosphatemia
  • Age 3-4m - inc ALP
  • Age 6-9m - decr phosphate
  • Age 12m - delayed growth, low phos, inc ALP, x-ray signs of rickets, delayed dentition, recurrent dental abscesses
  • Tx: phos + calcitriol supps, watch for hypercalcaemia and nephrocalcinosis, may need growth hormone
70
Q

What molecules act on the kidney to increase renal blood flow?

A
  • ANP and BNP (from heart) - cause afferent dilation and efferent constriction - inc GFR
  • Prostaglandin I2 and E2 (from kidney) - cause afferent and efferent dilation - inc GFR
  • Dopamine (from brain and kidneys) - cause afferent and efferent dilation - inc GFR
71
Q

Vit D synthesis

A

Vit D (cholecalciferol) from UV light - hydroxylated in liver to 25 (OH) vitamin D3 (by 25-hydroxylase)

  • Production of 1-25 (OH) Vit D3 (calcitriol) via renal 1-hydroxylase in kidney = most biologically active Vit D metabolite
  • 24-alpha hydroxylase in kidney converts Vitamin D to an inactive form
72
Q

hypocalcaemia

A

Rickets, seizures, tetany, stridor, cramps, paresthesia
- Treatment: IV 10% calcium gluconate with ECG monitoring, PO calcium supps, Vit D/alfacalcidol

causes:

  • Low calcitriol: Vitamin D def, renal failure or liver failure
  • Iatrogenic: frusemide
  • Hypoparathyroidism - transient neonatal, DiGeorge, PT removal
  • Acute pancreatitis
  • Acute alkalosis or correction of acidosis in context of normal-low calcium
  • Hyperphosphatemia (complexes free calcium): RF, rhabdo, tumour lysis
  • Pseudohypoparathyroidism e.g. Albright’s hereditary osteodystrophy
73
Q

hypercalcemia

A

Constipation, renal stones, nausea, lethargy, confusion, headaches, muscle weakness, polyuria, dehydration
- IV hydration, loop diuretics, rarely bisphosphonates (stop bone resorption)

  • Familial hypocalciuric hypercalcaemia, Williams (rarely persists >1y age)
  • Hyperparathyroidism - neonate or MEN1+2
  • Iatrogenic: Vit D excess
  • Macrophage production of calcitriol (sarcoidosis, subcut fat necrosis)
  • Malignancy
74
Q

influence of pH on calcium

A

As pH decreases (acidosis), H+ displaces Ca2+ from binding sites and the amount of iCa2+ increases
- Conversely, as the blood pH increases (alkalosis), albumin and the globulins become more negatively charged and bind more calcium, causing the amount of iCa2+ circulating to decrease. Therefore always correct acidosis prior to giving albumin, otherwise will cause hypocalcaemia as albumin will bind to the increased free calcium

75
Q

hypophosphatemia

A
  • Hyperparathyroidism (increased urinary excretion of phosphate)
  • Dietary deficiency. Appropriately high TRP (low urine phosphate)
  • Hypophosphatemic rickets, Fanconi syndrome. Inappropriately low TRP (high urine phosphate)
  • Tx: PO phosphate, Vit D replacement
76
Q

hyperphosphatemia

A
  • High urine phosphate, low TRP (appropriate) - rhabdomyolysis, tumour lysis
  • Low urine phosphate, high TRP (inappropriate) - CRF, hypoparathyroidism, pseudohypoparathyroidism
  • Tx: phosphate binders (ec calcium carbonate), dialysis
77
Q

X-linked hypophosphatemic rickets

A
  • Vitamin D resistant rickets
  • Mutation in PHEX gene on X-chromosome
  • Defect in phosphate resorption, low TRP (<85%), normal PTH and calcitriol level, hypophosphatemia
  • Age 3-4m - inc ALP
  • Age 6-9m - decr phosphate
  • Age 12m - delayed growth, low phos, inc ALP, x-ray signs of rickets, delayed dentition, recurrent dental abscesses
  • Tx: phos + calcitriol supps, watch for hypercalcaemia and nephrocalcinosis, may need growth hormone
78
Q

Nocturnal enuresis

A 
Monosymptomatic nocturnal enuresis is defined as urinary incontinence occurring during sleep, without any other lower urinary tract symptoms and without a history of bladder dysfunction, in children > 5 years of age.
• It is very common, affects boys more than girls, and resolves spontaneously at 15%/year.
• 5 years – 15 % of children affected 
• 6years–13%
• 7years–10%
• 8years–7%
• 10years–5%
• 12to14years–2to3% 
• ≥15years–1to2%
79
Q

Prune-belly (Eagle-Barrett) syndrome (PBS)

A

Incidence 1 in 40,000 to 50,000 births, males > females

congenital disorder w clinical triad of:
Abdominal wall muscle deficiency –complete/partial (thus wrinkled belly)
Severe urinary tract abnormalities
Bilateral cryptorchidism in males

May be a/w CHD (ASD, VSD, TOF), GI anomalies

Consequences of oligohydramnios: pulmonary hypoplasia, hip dislocation/subluxation, talipes

80
Q

Nephrocalcinosis

A

• Distal RTA
• Ex-premature neonates:
• Furosemide – hypercalciuria
• Steroids – hypercalciuria
• Vitamin D treatment for hypophosphataemic rickets:
• Enhances tubular reabsorption of Ca2+
• Oxalosis:
- Autosomal recessive disorder
- Primary hyperoxaluria associated with defect in alanine:glyoxylate aminotransferase (AGT) enzyme, which leads to excess oxalate production and urinary oxalate excretion
- Calcium oxalate precipitates, nephrocalcinosis and obstructing stones form, renal failure ensues
- Systemic oxalosis – joints, heart, blood vessels
- Treatment – liver transplantation alone if renal function only moderately reduced; sequential liver then kidney transplantation if renal failure established, with intense dialysis therapy between the two operations to lower the systemic oxalate burden (simultaneous liver–kidney transplantation presents high risk of oxalate deposition in newly transplanted kidney)

81
Q

Nutcracker syndrome

A

compression of left renal vein between aorta and proximal SMA, causes microscopic haematuria

82
Q

Renal Stones

A

Composition of stones
• radioopaque stones (90%): CaPO4, CaO..
• relatively radiolucent: cystine, struvite…
• radiolucent stones: uric acid, xanthine, indinavir)

Causes

Calcium stones - most common
• hypercalciuria (polygenic, may be autosomal dominant in some families)
o idiopathic
o absorptive - high Ca or Na diet (NB: dietary K is protective), Vit D excess, Vit C (oxalate precursor),
o renal - renal leak, distal RTA type 1, frusemide,
o resorptive - hyperparathyroidism, immobilisation, sarcoid, corticosteroids, Cushing

• hyperoxaluria {progress to ESRF in 20s]
o primary (AR),
o secondary (Vit C, malabsorption, pyridoxine deficiency)
o enteric (IBD, pancreatic insufficiency, biliary disease)
o NB: CF ~5% – pancreatic insufficient, malabsorption and NaCl failure to excrete
• hypocitruria (chronic diarrhoea, malabsorption, idiopathic)
• renal tubular acidosis (RTA) – Type I (alkaline urine, hyperchloraemic hypokalaemic metabolic acidosis,
• hyperuricosuria
• cystinuria (heterozygous)

Cystine stones
• cystinuria

Struvite stones (Magnesum ammonium phosphate)
• UTI (urea splitting organisms such as Proteus, Klebsiella, E coli, Pseudomonas)
• Foreign body
• Urinary stasis

Uric acid stones (RADIOLUCENT)
• Hyperuricosuria
o Inborn errors of metabolism - Lesch-Nyhan syndrome, G-6-PD,
o Rapid purine turnover - myeloproliferative disorders, post-chemotherapy,
o Short bowel, IBD

Indinavir stones – HIV Rx, 4% of patients develop stones

Nephrocalcinosis
• Commonest - prem neonates receiving frusomide
• Other common - medullary sponge kidney, distal RTA, hyperparathyroidism, hypophosphataemic rickets, sarcoid, cortical necrosis, hyperoxaluria, prolonged immobilisation, Cushing syndrome, hyperuricosuria, renal candidiasis.

Inihibitors of stone formation
• Citrate, Magnesium – inhibits crystallization and aggregation.
• Glycosaminoglycans, Tamm-Horsfall protein
• Proximal RTA protective – decreased HCO3 resorption U citrate (vs dRTA –ass stones)

Stones work up
Urinalysis - ?infection
Renal USS ?obstructed system – need urological input
Stone analysis if possible (?Cystine, Uric acid, Struvite, CaO/CaPO4)
-if likely CaO/CaPO4 (most are):
Urine: urinalysis, pH, M/C/S
Ca:Crt ratio (usually <0.2, if fasting should be <0.7), Oxalate:Crt ratio
Citrate, L-gllyceric acid
+/- Urate:Crt, Cystine
24hour: Ca, Oxalate, Uric acid, citrate, crt. ?Na
Plasma: UEC, C/M/P, HCO3, Cl, PTH +/- Urate
-if cystine – microscopy/cyanide-nitropresside/urine aa chromatography as above.

Treatment
• Address specific underlying disorder if possible
o Uric acid stones -> allopurinol (xanthine oxidase inhibitor)
o Cystine stones -> urine alkalinisation, D-penicillamine (chelating agent), NAC?
o RTA I – correct acidosis, replace K and Na.
o Hyperoxaluria primary – pyridoxine, liver transplantation (ideally before renal failure)

• General prevention
o Increase fluid intake (Increase urine volume), night and day
o Reduce Na intake, normal Ca intake (not reduced for growing kids),
o Reduce animal protein intake/vegetarian diet, limit sucrose/fructose, small quantity of wine!
o Thiazide diuretics (reduce renal calcium excretion)
o Potassium citrate (inhibitor of stone formation), or lemonade/lemons
o Urinary alkalisers – sodium bicarbonate/citrate,
• Surgical/endoscopic/lithotripsy removal/stent – consider if large, causing significant obstruction or infection.
• Enhance passage – alpha-adrenergic blockers

A high sodium diet is one of the strongest contributors to stone disease in western society. High sodium diet leads to increased sodium excretion from kidney. Increased sodium excretion is accompanied by increased calcium excretion.
Magnesium is an inhibitor of calcium oxalate stone formation. Hypomagnesuria therefore predisposes to stone formation.
Citrate is an inhibitor of calcium stone formation. Therefore, LOW urinary citrate (hypocitruria) predisposes to stone formation.

83
Q

Renal stones in pancreatic insufficiency

A

Oxalate

84
Q

Uric acid stones

A

Lesch Nyan, G6PD, post chemo

85
Q

Juvenile Nephronopthisis

A
  • AR inheritance
  • Paediatric disease (clinical similar but genetically distinct from MCD)

• Signs & symptoms
o Polyuria, polydipsia
o Anaemia due to failure of EPO production
o EPO made by cells at corticomedullary junction (this is the site of maximal pathology) => EPO insufficiency occurs early (c/f CKD)
o Growth retardation
 Due to salt deficiency (rather than due to renal failure)
o ESRF - major cause of ESRF in kids
o Urinalysis is often normal/minimally abnormal

• Pathology
o Kidneys normal size
o Corticomedullary cysts - Small, not seen distinctly on USS, No extra-renal cysts

•	Histopathology
o	Tubules
	Basement membrane thickening/attenuation
	Distal tubular atrophy
	Cysts
o	Interstitium
	Lymphocytes
	Fibrosis
o	Glomeruli
	Periglomerular fibrosis
•	Genetics
o	NPH (AR)
o	NPH1 – juvenile form (common – probably what this kid has)
o	NPH2 – infantile (uncommon)
o	NPH3 and NPH4
o	Syndromic (many related to cilia dysfunction)
	Oculomotor apraxia (large NPH1 deletion)

 Senior-Loken
• Retinitis pigmentosa by 10y
• Present with blindness/coarse nystagmus by age 2 (not related to NPH1)
• Liver fibrosis

	Joubert type B (cerebello-oculo-renal syndrome)
•	NPH
•	Coloboma
•	Retinal degeneration
•	Aplasia of cerebellar vermis
•	Polydactyly
•	Developmental delay
	Jeune
•	Asphyxiating thoracic dysplasia
•	Neonatal RDS
•	Limb shortening
•	Polydactyly, nail abnormalities
•	Other skeletal abnormalities
•	Liver disease
•	Nephronophthisis
•	With heavy proteinuria
•	Not linked to NPH1, 2 or 3 genes
	Ellis-van Creveld
•	Skeletal abnormalities same as Jeune
	Bardet-Biedl
•	Group of 6 diseases
•	Polydactyly
•	Truncal obesity
•	Renal dysfunction (15%  ESRF)
•	Pigmentary retinopathy
•	Short stature
•	Intellectual disability
•	MCKD (AD – adults)
•	MCKD1 and MCKD2
•	NPH1 (Juvenile Nephronophthisis)
•	80% of nephronophthisis
•	2q13 – large deletion in 2/3
•	NPH1 – codes for nephrocystin – protein that fits into lateral tubular cell wall and basement membrane
•	Early onset polyuria, polydipsia, dilute urine
•	Anaemia later
•	Growth retardation is later
•	Hypertension is rare
•	Diagnosis:
o	Clinical picture fits  renal ultrasound  presumptive diagnosis
o	Confirmatory genetic testing, renal biopsy
o	Normal kidney size on US
o	Check family history
o	?any extra-renal manifestations
o	NPH2 (infantile)
	Rare
	Enlarged kidneys

• Differential diagnosis of nephronophthisis
o Renal dysplasia (USS)
o Hypoplastic kidneys (USS)
o VUR with reflux nephropathy (hypertension, proteinuria)
o Obstructive uropathy (DMSA, US)

86
Q

Posterior urethral valves

A

• Most common cause of severe obstructive uropathy in children
o 1:8000 boys
o Valves = tissue leaflets fanning distally from prostatic urethra to external urinary sphincter, with slit-like opening between leaflets
• Severity of obstruction varies
o 30% ESRF/CRF
o 50% VUR
• Renal sequelae vary along spectrum
o Mild hydronephrosis
o Up to severe renal dysplasia
o Severity probably relates to severity of obstruction and time of onset during development
o Oligohydramnios with consequent pulmonary hypoplasia

• Diagnosis
o Antenatal scans – bilateral hydronephrosis, distended bladder, +/- oligohydramnios
o Palpable, distended bladder with weak urinary stream in male neonate
o If unrecognised may present later with FTT (uraemia, sepsis) if severe; if less severe present later in childhood with difficulty toilet-training or UTI
o Confirmatory investigations
 Voiding cystourethrogram
 Perineal US
o Other investigations
 Renal function
 Delineate upper tract anatomy
• Management
o Well neonate – insert small feeding tube into bladder
 If Cr improves  transurethral valve ablation
 If urethra too small  temporary vesicotomy
 If doesn’t improve (Cr  or static)
 Consider renal dysplasia, irreversible renal damage, secondary urethral obstruction
 Vesicodomy
o Sick baby
 Manage sepsis, stabilize electrolyte balance, resuscitate
 May need temporary percutaneous nephrostomy (to drain upper tract) and dialysis
o Older kid
 Primary valve ablation

•	Prognosis
Favorable
Normal 18-24/40 USS
Cr <0.08-0.1 after bladder decompression
Corticomedullary junction visualized on renal USS	Oligohydramnios in utero
Unfavorable
Hydronephrosis identified <24/40
Cr >0.1 after bladder decompression
Bilateral cortical renal cysts
Persistence of diurnal incontinence >5y of age

• In neonates:
o Prognosis relates to degree of pulmonary hypoplasia and potential for recovery of renal function
o In very severe disease – may be stillborn
o If survive neonatal period – 30% have persistent renal insufficiency and may require transplant
• Post-definitive treatment

87
Q

Sodium regulation

A 
There are 4 main sites of sodium transport
	proximal tubule (60%)
	ascending loop of Henle (25%)
	distal tubule (15%)
	collecting tubule

Major hormonal mechanisms mediating sodium balance:

Renin-angiotensin-aldosterone axis
 angiotensin II increases sodium reabsorption in proximal tubule
 aldosterone increases sodium reabsorption in distal tubul, therefore suppression of renin promotes sodium excretion

Atrial natriuretic factor
 produced in response to atrial distension, angiotensin II stimulation and sympathetic stimulation
 therefore, increased in hypervolaemic states
 promotes sodium excretion (increases GFR, decreases renin release)

Noradrenaline
 released in response to volume depletion
 decreases renal blood flow, therefore decreases the amount of sodium filtered and stimulates renin release (reduction in afferent arteriole pressure causes release of renin from JG cells)
 therefore indirectly increases sodium concentration
Na-K ATPase is ATP-dependent pump system essential for maintaining Na and K concentrations across sarcolemmal membranes (e.g. cadiac myocytes)

Tubulo-glomerular feedback
 macula densa cells sense Na levels in tubular fluid and alter GFR (i.e. high Na concentration indicates high GFR and vice versa)
 detection of high Na levels triggers release of signalling molecules from macula densa which drops GFR, problaby by constriction of afferent arteriole

88
Q

Juvenile Nephronophtisis

A
  • AR inheritance
  • Paediatric disease (clinical similar but genetically distinct from MCD)

• Signs & symptoms
o Polyuria, polydipsia
o Anaemia due to failure of EPO production
o EPO made by cells at corticomedullary junction (this is the site of maximal pathology) => EPO insufficiency occurs early (c/f CKD)
o Growth retardation
 Due to salt deficiency (rather than due to renal failure)
o ESRF - major cause of ESRF in kids
o Urinalysis is often normal/minimally abnormal

• Pathology
o Kidneys normal size
o Corticomedullary cysts - Small, not seen distinctly on USS, No extra-renal cysts

•	Histopathology
o	Tubules
	Basement membrane thickening/attenuation
	Distal tubular atrophy
	Cysts
o	Interstitium
	Lymphocytes
	Fibrosis
o	Glomeruli
	Periglomerular fibrosis
•	Genetics
o	NPH (AR)
o	NPH1 – juvenile form (common)
o	NPH2 – infantile (uncommon)
o	NPH3 and NPH4
o	Syndromic (many related to cilia dysfunction)
	Oculomotor apraxia (large NPH1 deletion)
	Senior-Loken
•	Retinitis pigmentosa by 10y
•	Present with blindness/coarse nystagmus by age 2 (not related to NPH1)
•	Liver fibrosis
	Joubert type B (cerebello-oculo-renal syndrome)
•	NPH
•	Coloboma
•	Retinal degeneration
•	Aplasia of cerebellar vermis
•	Polydactyly
•	Developmental delay
	Jeune
•	Asphyxiating thoracic dysplasia
•	Neonatal RDS
•	Limb shortening
•	Polydactyly, nail abnormalities
•	Other skeletal abnormalities
•	Liver disease
•	Nephronophthisis
•	With heavy proteinuria
•	Not linked to NPH1, 2 or 3 genes
	Ellis-van Creveld
•	Skeletal abnormalities same as Jeune
	Bardet-Biedl
•	Group of 6 diseases
•	Polydactyly
•	Truncal obesity
•	Renal dysfunction (15%  ESRF)
•	Pigmentary retinopathy
•	Short stature
•	Intellectual disability
  • MCKD (AD – adults)
  • MCKD1 and MCKD2
  • NPH1 (Juvenile Nephronophthisis)
  • 80% of nephronophthisis
  • 2q13 – large deletion in 2/3
  • NPH1 – codes for nephrocystin – protein that fits into lateral tubular cell wall and basement membrane
  • Early onset polyuria, polydipsia, dilute urine
  • Anaemia later
  • Growth retardation is later
  • Hypertension is rare
•	Diagnosis:
o	Clinical picture fits, then renal ultrasound and presumptive diagnosis
o	Confirmatory genetic testing, renal biopsy
o	Normal kidney size on US
o	Check family history
o	?any extra-renal manifestations
o	NPH2 (infantile)
	Rare
	Enlarged kidneys

• Differential diagnosis of nephronophthisis
o Renal dysplasia (USS)
o Hypoplastic kidneys (USS)
o VUR with reflux nephropathy (hypertension, proteinuria)
o Obstructive uropathy (DMSA, US)

89
Q

Metanephric mesenchyme gives rise to

A

Majority of nephron - Bowmans capsule, proximal tubule, Loop of Henle, distal tubule

90
Q

Ureteric bud gives rise to

A

Collecting duct, renal pelvis, ureter

91
Q

Fe NA

A

(UNa/SNa) / (Ucr/SCr) x 100%

= (UNaXSCr) / (SNaxUcr) x 100%

Pre renal <1% ; post renal >2%
Cant use if on diuretics, use Fe Urea (<35% pre-renal)

Paeds (25 decks)

Brainscape helps you reach your goals faster, through stronger study habits.
© 2024 Bold Learning Solutions. Terms and Conditions