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Paediatrics > Renal > Flashcards

Renal Flashcards

1
Q

Proximal tubule..

A

Main site for reabsorbtion

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2
Q

Aldosterone acts on..

A

Na channel in DCT to reabsorb water and sodium (secrete k and H)

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3
Q

Renal angiomyolipomas in what syndrome

A

TS

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4
Q

Peritonitis organisms in dialysis

A

SA 20% CONS 40% strep 20% pseudomonas 5%

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5
Q

Fanconi syndrome renal problem location

A

Proximal tubule dysfunction (RTA2), not reabsorbing all the good stuff so lose sodiuma nd don’t grow well and have tubular dysfunction so drink and wee lots
Causes: cystinosis and Lowes syndrome

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6
Q

In phaeo with HTN don’t use..

A

Bets blocker but use a blocker (phentolamine IV or oral phenoxybenzamine)

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7
Q

Persistently low C3 in…

A

Membranoprolif

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8
Q

RPGN presentation

A

Nephrotic or nephritic
Crescents on biopsy
Tx steroid

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9
Q

Presents with family history and haematuria

Alports genetic test and biopsy result?

A

Hereditary nephritis mainly XL (dominant)
Mutation in COL4 gene encoding type IV collagen which affects BM
Macroscopic haematuria
Biopsy variable thickness and splitting of BM - basket weave appearance
SNHL ears
Eyes cataracts, anterior lenticonus, macular abnormalities
ESRD 50% by 25

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10
Q

IgA nephropathy

A 
Mostly boys
Recurrent (at time of infection)
Macroscopic haematuria
Most are ok by 10% to EsRf
Biopsy IgA deposits 
Tx steroids If bad
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11
Q

Three causes of nephrotic syndrome

A

Minimal change (steroids)
FSGN
Memb snouts nephropathy

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12
Q

Renal stone increased by high ? Diet

A

Na (decreases calcium absorption)

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13
Q

Present with FTT, recurrent episodes dehydration, hypercalciuria, 20% low serum mg, renal stones

Hypokalaemic metabolic acidosis

A

Bartter syndrome:
AR dysmorphic
Nephrocalcinosis
Like frusemide abuse

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14
Q

Present with hypokalaemic metabolic alkalosis, some mild growth failure, pr sent later, hypomagnesemia, hypocalciuria

A

Gitelman syndrome
AR defect na/cl CT
Like thiazide abuse

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15
Q

Prune belly =

A

Abdo wall defect, undescended testes, UT abnormalities

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16
Q

Granular casts in..

A

ATN

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17
Q

GFR gold standard

A

Inulin (gold standard but difficult to measure)

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18
Q

DTPA vs DMSA

A

Parenchyma defects

DMSA for structure/morphology - scarring, static scan, size and % function

DTPA for transit over time and looks for obstruction and drainage, how whole system is working, dynamic

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19
Q

Haematuria excretion rate increases with

A

Age and exercise

Proteinuria with fever, exercise, heart failure

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20
Q

Risk of nephritis in ahSP after 2m

A

2%

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21
Q

6 forms of steroid resistant nephrotic syndrome in childhood

A 
Minimal change
Membranoproliferative glomerulosis
Membranous nephropathy
Secondary (IgA, HSP, Lupus)
FSGS
Congenital
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22
Q

Syndrome causing childhood HTN

A

Liddle due to acting like there is high aldosterone (but it’s normal)

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23
Q

Best drug for reduction BP, proteinuria, preservation of renal function

A

ACEi

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24
Q

MCDK

A

1/4000, non heritable, unilateral nonfunctional cystic mass

Bilateral fatal often other kidney can make up for it but may have VUR - monitoring important

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25
Q

Campomelic dysplasia

A

SOX9, dysplasia, hydronephrosis

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26
Q

Agenesis kidney in what syndromes

A

Fraser
Kallman
Pallister-hall
Rubinstein-taybi

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27
Q

ADPKD

A

Mutation PKD
1/1000
Wide phenotype
Usually asymptomatic in childhood

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28
Q

ARPKD

A

Bilateral large echogenic kidneys
Mutation PKHD1
1/20000
With congenital hepatic fibrosis and hepatomegaly with portal HTN

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29
Q

Most frequent genetic cause CKD

A

Neohropnopthisis (tubuloingerstitial sclerosis - concentrating defect)

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30
Q

Causes of CKD

A

Hypoplasia/dysplasia
Nephronopthisis (normal kidney size)
Obstructive uropathy
Cystic

FSGN
Chronic GN
Alpert
HUS
Cortical necrosis
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31
Q

In chronic tubular defect - concentrating defect causes

A

Polyuria and polydipsia
Enuresis/nocturia
Electrolyte loss in urine

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32
Q

Causes of AKI

A 
Sepsis
HUS
GN
ATN
Drugs
Dehydration
Interstitial nephritis
Rhabdo 
Oncology
Cardiac surgery
Liver transplant
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33
Q

Tubular damage from what drugs

A

Aciclovir

Aminoglycosides

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34
Q

ATN urine results

A

Casts
Low osmolality
<40 urea/cr ratio
High Na

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35
Q

Two other causes HUS

A

Atypical in 10%
I.e.
Strep pneumoniae
Low C3

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36
Q

Anti GBM

A

May have pulmonary haemorrhage

Linear staining

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37
Q

Imaging small vs large echogenic kidney

A

Small structural

Large acute disease (ARF or GN)

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38
Q

Proteinuria

A

Sign of CKD and will progress - needs f/u

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39
Q

Orthostatic proteinuria

A

Normal in morning
Teens
Normalises

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40
Q

Nephrocalcinosis

A

Generalised echogenic appearance due to calcium deposits

  • cortical nephrocalcinosis is often due to cortical necrosis – check renal function, BP etc
  • Medullary and cortical – hypercalcaemia ( seen with excess Vit D) oxalosis (esp if calculi)
  • Medullary alone – very long list of causes
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41
Q

Plasma osmolality

A

(2xplasma Na) + glu + urea

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42
Q

Chloride in urine

A

High in diuretic abuse, Bartters, Gitelmans

Low with vomiting and laxative abuse

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43
Q

Pneumococcal HUS pathophysiology

A

Neuraminidase uncovere T-cryptantigen which binds T IgM

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44
Q

Rituximab

A

Monoclonal antibody against CD20 causing rapid depletion of B cell populations

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45
Q

Mycophenolate mofetil mechanism of action and side effects

A

Inhibits T and B cell proliferation

GI, leukopenia, anaemia (not nephrotoxic)

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46
Q

Six causes of allograft dysfuncTION

A 
DehydraTION
MedicaTION
InfecTION
ObstrucTION
RejecTION
PrefusION problem
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47
Q

High tacrilomus level causes electrolyte low

A

MG

Tremour, alopecia

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48
Q

Cyclosporine SE

A

Hirsuitism and gingival hyperplasia

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49
Q

Post transplant lymohoproliferative disorder

A

Mononucleosis like lesions

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50
Q

Post transplant infection prophylaxis

A

CMV 3m valganciclovir
Cotrimox
Candida 1m nilstat (and with antibiotics)

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51
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)

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52
Q

Type 3 RTA

A

Rare

Children have mental retardation, cerebral calcification and osteoporosis (carbonic anhydride deficiency)

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53
Q

Drug contraindicated in renal artery stenosis

A

ACEi

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54
Q

Renal artery stenosis assoc with what…

A

FiBromuscular dysplasia
Takayasu arteritis
NF1
Williams

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55
Q

Micrhaematuria resolves after how long with PSGN

A

2 years

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56
Q

Follow up renal after HSP for

A

3 months (risk 2% at 2m)

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57
Q

Minimal change presents with nephrotic syndrome and what feat on electromicroscopy

A

The three hallmarks of minimal change disease: diffuse loss of podocyte foot processes, vacuolation, and the appearance of microvilli.

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58
Q

Treating HTN with Ca Channel Blockers Avoid in…

A

polycystic kidney disease

59
Q

Older child polyuria and polydipsia

Ultrasound: bilateral small corticomedullary cysts, tubulointersitial fibrosis. Small or normal sized kidneys.

disruption of TBM, tubulo‐interstitial fibrosis, cyst formation.

Presents with polydipsia and polyuria with failure and small echogenic kidneys and retinitis pigmentosa

AR

A

Nephropnopthisis

60
Q

Fanconi syndrome

A

Growth issues/rickets and concentrating defect
Due to proximal tubule disorder (proximal tubule usually reabsorbs glucose and a.a and bicarb/phosphate causing…
Hyperaminoaciduria
Glycosuria
Hypophosphatemia
Hypokalemia
Metabolic acidosis hyperchloremia

61
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.

62
Q

Normal bladder volume

A

(AGE x 30) X 30 = volume (mls)
330ml in 10 year old

63
Q 
Pale kids with high creatinine and poor growth with 
polyuria&amp; polydipsia
enuresis / nocturia
Always thirsty
Loss of other electrolytes in the urine 
Little haematuria or proteinuria 
Has a...
A

Concentrating defect from tubular +- interstitial cause (non-glomerular)

Also polyuria and hypovolaemia in acute tubular necrosis or interstial insult

64
Q

HUS triad

A

Thrombocytopenia, AKI, haemolytic anaemia

Typical from diarrhoea illness
Atypical - pneumococcal and complement def

65
Q

Most common cause of fanconi syndrome and has crystal deposits on slit lamp eye

A

Cystinosis

66
Q

Presents with obesity, diabetes, retinitis pigmentosa, polydactyly, infertility and renal failure which syndrome

A

Ciliopathic

Bardet–Biedl syndrome

67
Q

Which syndrome?
Presents with renal cystic dysplasia, central nervous system malformations (occipital encephalocele/NTD), polydactyly (post axial), hepatic fibrosis

A

Meckle

68
Q

Treat peritonitis with…

A

After sample
IP vanc/cefazolin and ceftazadime (IV if septic)
Antifungsl prophylaxis

69
Q

Post transplant graft dysfunction and haematuria ?organism

A

BK

70
Q

Flank mass, haematuria and decreased platelet in newborn suggests

A

Renal vein thrombus

71
Q

High what in urine predisposes to kidney stones

A

Calcium

72
Q

IgA deposited where in HSP

A

Mesangium

73
Q

Treating enuresis with drugs (first and second line)

A

First is desmopressin and second oxybutynin

74
Q

Is serum IgA helpful in IgA nephropathy

A

No (only up in 15% paed pt)

75
Q

Mechanism injury in renal HUS e.coli

A

ENDOtoxin

76
Q

Hypercalciuria diet advice

A

Low sodium

77
Q

Type 2 RTA causes

A

Cystinosis, Lowe syndrome, galactosemia, Wilson disease, tyrosinaemia, interstitial nephritis, medication (gent, isofosamide, cyclosporine)

78
Q

Present with nephrotic or nephrotic or mixed

Histology showing deposits (C3) and thick capillary walls (tram tracks present)

A

Membranoproliferative GN (type 1 and 2 - dense deposits)

79
Q

Renal biopsy deposit C3 and IgG in?

Deposit all immunoglobulins and C3 in?

A

psGN

SLE

80
Q

Large placenta

Genetic defect in nephrin or podocin cause

A

Congenital nephrotic syndrome

81
Q

What reduces total body K

A

Resonium

82
Q

Main site of K+ reabsorption

A

Proximal convoluted tubule

83
Q

Carrier freq PKHD1 gene (fibrocystin) involved with ARPKD

A

1:70

84
Q

Anion gap formula

A

Anion Gap = Na+ – (Cl- + HCO3-)

Urinary = UCl-(UNa+UK)

85
Q

Cr clearance not accurate when renal disease worsens because

A

approximately 15% of excreted urine creatinine is derived from proximal tubular secretion. Because of the tubular secretion of creatinine, the creatinine clearance typically overestimates the true GFR by 10% to 15%.

86
Q

MCU should be considered for

A

infants under 3 months with US abnormalities after their first febrile UTI and /or atypical UTI
for children under 6 months with recurrent febrile UTI and / or abnormal US
for male infants where there is concern regarding obstructive uropathy on US

Needs three days treatment dose antibiotics starting day before

87
Q

Thiazides mech of action

A

inhibit reabsorption of sodium (Na+) and chloride (Cl−) ions from the distal convoluted tubules in the kidneys by blocking the thiazide-sensitive Na+-Cl− symporter.

88
Q

Fractional excretion sodium =

A

Fractional excretion sodium = 100 x (sodium urine x Cr blood / sodium blood x Cr urine)

89
Q 
reabsorption of most bicarbonate (HCO3) from the glomerular ultrafiltrate is by: 
A. diffusion of carbon dioxide (CO2). 
B. intercellular transport. 
C. pinocytosis. 
D. primary active transport. 
E. transcellular aquapores.
A

A

90
Q

Aldosterone and ANP role

A

ANP stimulates sodium loss
Aldosterone stimulates sodium/H20 retention by increased aquaporins luminal surface DCT and CD (loss of K+ and H+ in exchange)

91
Q

Multicystic dysplastic kidney risks

Bunch of grapes on USS

A

Not likely cancer or HTN
Some involute
Other kidney having Reflux (PUJ obstruction)

92
Q

Salt wasting syndromes/hypokalaemic metabolic alkalosis

  1. Salt (Na and Cl and K) wasting nephropathy more severe (thick ascending loop) like thiazides diuretic (high calcium and chloride in urine)
  2. Hypokalaemic, hypomagnesiaemia, hypocalciuria (distal tubule) less severe
A

Bartter syndrome

Gitelman

93
Q

NEphronophthisis with coloboma/ cerebella =

A

Jouberts syndrome

94
Q

vasodilator in renal afforestation arterioles

A

Prostacyclin (prostaglandins)

95
Q

Cresenteric glomerulonephritis with IgG and C3 deposits and lord positive anti-GBM antibodies is

A

Goodpastures

96
Q

Schistocyte on bloodfilm (=helmet cell) may indicate

A

HUS

97
Q

Gene affected in alports

A

Col4A5

98
Q

Wilms tumour, proteinuria (diffuse mesangial sclerosis) and ambiguous genitalia capsule be from

A

Denys-drash syndrome

99
Q

Prune belly =

A

Boys
Absent abdominal wall muscle
Bilateral crypdorchidsim
Gu tract abnormality –> kidney failure

100
Q

Three central causes of micropenis

A

Kallman
Prader Willi
Panhypopit

101
Q

Behaviours to delay voiding such as bounding up and down on soles of feet or squatting with urinary incontinence suggests or urge incontinence

A

Detrusor instability (can’t confirm without invasive test of muscle)

102
Q

Daytime wetting with urgency and increased voiding frequency consider

A

overactive bladder, low awareness bladder sensation, poor attention / concentration
Renal USS all day wetters
Treatment needs to relax pelvic floor - behavioural management and can use oxybutinin (antichol)

103
Q

IgA levels only elevated in what percent of IgA nephropathy

A

50%

104
Q

Cerebral salt wasting ANP directly inhibits ADH so ADH is?

A

Low

105
Q

Most common cause of nephrotic syndrome

A

Minimal change

106
Q

Most common cause of gross haematuria

A

IgA nephropathy

107
Q

Renin measurement in HTN - if low suggests

High means renal cause

A

Mineralocorticoid excess

108
Q

Electron microscopy: diffuse thickening, thinning, splitting and layering of
glomerular and tubular basement membranes.
Which condition

A

Alports

109
Q

Drug that improves calcium in urine

A

Thiazides (block cl na reabsorption distal tubule)

110
Q

Most common genetic cause of kidney failure

A

nephropnopthisis

111
Q

Stop correcting for BSA GFR what age

A

2y

112
Q

Bright kidney on USS causes

A 
Potential causes include:
•Dysplasia/ Glomerulocystic disease
•ARPKD ( large echogenic kidneys cysts too small to see)
•ADPKD
•Congenital nephrotic syndrome
•Insult such as ischaemia or infection
•Beckwith- Wiedeman syndrome
•Tuberous sclerosis
113
Q

Nephropnopthisis and retinal dystrophy two syndromes

A

Leber and senior loken syndrome

114
Q

What are the causes of an increased AG metabolic acidosis?

A

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

115
Q

Low chloride in urine when?

A

Vomiting and laxative abuse

116
Q

Beta 2 microglobulin used for

A

In someone with signs of kidney disease, increased levels of B2M in the blood and low levels in the urine indicate that the disorder is associated with glomeruli dysfunction. If B2M is low in the blood and high in the urine, then it is likely that the person has renal tubule damage or disease.

117
Q

HSP in Armenian may be early indicator of what?

A

Armenian children with FMF had higher than expected frequency of vasculitis (4.3%). We suppose, that in children with FMF: 1) HSP and PFM vasculitis might be considered as markers of severe FMF and early disease onset 2) M694V homozygous genotype is a risk factor for the development of PFM. These results are consistent with data on the susceptibility of FMF patients in ethnically matched populations in the development of HSP and PFM [Lange-Sperandio B. et al., 2004].MEFV mutation genetic screening is recommended for Armenian children with HSP, PFM vasculitis for early diagnosis of FMF, treatment and prevention of complications.

118
Q

Renal cysts and MODY gene linked?

A

Hepatocyte Nuclear Factor 1β

119
Q

Instead of creatinine what marker can be better and less influenced by muscle mass, age, illness etc

A

Cystatin C

120
Q

Tacrilomus potent anti T cell in renal transplant SE (not to be given with cyclosporine)

A

Tremors, headaches, diarrhoea, hypertension, nausea, renal impairment.

121
Q

Mycophenilate motefil renal transplant mechanism and SE

A

inhibits the de novo pathway of guanosine nucleotide synthesis without incorporation into DNA. B and T lymphocyte proliferation depends on de novo guanosine synthesis whereas other cell types can utilise salvage pathways

Diarrhoea and increased cancer risk (lymphoma)

122
Q

Basiliximab mechanism and side effects

A

specifically binds to the CD25 antigen on activated t-cells from starting the rejection process

SE low platelets and high lipids

123
Q

Prevention CMV in transplant

A

Valgancyclovir 3-6m

124
Q

Calcium with diuretics (frusemide vs thiazides)

A

Calcium excretion is increased by loop diuretics and diminished by thiazide-type diuretics and amiloride

125
Q

What is characterized by weakness, dizziness, headache, and in severe cases, mental status changes in dialysis patient

A

Dialysis dysequilibrium syndrome is a common neurologic complication seen in dialysis patients

126
Q

Pseudohyperaldosteronism syndrome

HTN and low K

A

Liddle

127
Q

Hypoaldosteronism cause?

A

CAH

128
Q

Paroxysmal nocturnal haemoglobinuria mechanism

A

Sensitive RBC membrane to lysis by complement

129
Q

Posterior urethral valves and pelvicoureteric junction obstruction differ on MCU by?

A

PUV has a trabeculated bladder

130
Q

Name four Causes of false negatives of protein on urine dipstick

A

captopril, vitamin C, proteinuria, acidic urine

131
Q

Podocytes are negatively charged and keep albumin in tight junction gene affected in congenital nephrotic syndrome?

A

Nephrin (NPHS1)
Podocin (NPHS2)
Deny-Drash (WT1)

132
Q

Hyaline cast in urine may indicate what?

A

ATN

133
Q

Boy with kidney stones and proteinuria with calcium in urine and renal impairment from?

A

Dents syndrome
OCRL1
XL
Affected endocytotic process in PCT

134
Q

Cataracts, developmental delay, hypotonia and OCRL1 gene affected

A

Lowe syndrome

135
Q

Mineralocorticoid from adrenal called

A

Aldosterone

136
Q

Pseudoaldosterone with HTN and increased K in?

A

WNK1
Upregullates NCC
Gordon’s

137
Q

Present with kidney stone and low potsssium, chloride and metabolic alkalosis
FTT with tubular dysfunction
Like frusemide abuse (LOH)
What is affected and what syndrome

A

Bartter
(Bart says F and has 4 spikes on head)
Na-K-2cl cotransporter affected
MG and Ca between cell junctions due to gradient

138
Q

Giggle incontinece treatment

A

Methylphenidate because centrally mediated

139
Q

When wee interrupt flow by wctivatiaphincter against flow called

A

Dysfunctional voiding

140
Q

Night wetting treatment

A

Alarm
Desmopressin for sleep over or impacting life
(Oxybutinin)

141
Q

Lethal ciliopathy with triad occipital encephalocoelenlarge cystic kidneys and polydactyly cause

A

Meckle-Gruber

142
Q

How many renal cysts are you allowed per 10 years

A

1

143
Q

Caroline disease is?

A

Hepatic involvement in ARPCKD

Paediatrics (19 decks)

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