renal Flashcards

1
Q

causes of Potter sequence (syndrome)

A
  1. ARPKD
  2. obstructive uropathy (posterior urethral valves)
  3. bilateral renal agenesis
  4. chronic placental insuficiency
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2
Q

Potter sequence (syndrome) - mnemonic

A
POTTER 
Pulmonary hypoplasia
Oligohydramnios (trigger)
Twisted face 
Twisted skin
Extremitiy defects 
Renal failure (in utero)
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3
Q

MC congenital renal abnormality

A

Horseshoe kidney

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4
Q

Horseshoe kidney - defnition

A

interior poles of both kidneys fuse abnormally

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5
Q

Horseshoe kidney - location

A

As they ascend from pelvis during fetal development, horseshoe kidneys get trapped under inferior mesenteric artery and remain low in the abdomen

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6
Q

Horseshoe kidney is associated with

A
  1. hydronephrosis (eg. ureteropelvic junction)
  2. renal stones
  3. infection
  4. chromosomal aneuploidy syndromes (Turner syndrome, trisomies 13, 18, 21)
  5. renal cancer (rarely)
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7
Q

Unilateral renal agenesis - definition

A

complete absence of kidney and ureter

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8
Q

Multicystic dysplstic kidney - definiton

A

nonfuctonal kidney consisting of cysts and connective tissue

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9
Q

Duplex collecting system - mechanism/defintion

A
  • Bifurcation of ureteric bud before it enters the metanephric blastema creates a Y-shaped bifid ureter.
  • Duplex collecting system can alternatively occur through two ureteric buds reaching and interacting with metanephric blastema
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10
Q

Duplex collecting system - is strongly associated with

A
  1. vesicoulateral refelx
  2. ureteral obstruction
  3. high risk of UTIs
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11
Q

Congenital solitary functional kidney - clinical presentation

A

Majority asymptomatic with compensatory hypertrophy of contralateral kidney, but anomalies in contralateral kidney are common –> increased risk of rena failure later in life

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12
Q

Horseshoe Kidney - chromosomal aneuploidy syndromes

A

13, 18, 21

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13
Q

Wimls tumor (nephroblastoma) - mechanism

A

Loss of function mutation of tumor suppressor genes WT1 or WT2 on CH 11. MAY be part of several syndromes:

  1. WAGR complex
  2. Denys-Drash
  3. Beckwith-Wiedemann
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14
Q

WAGR complex - manifestation

A

Wilms tumor
Aniridia
Genitourinary malformations
mental Retardation/intellectual disability

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15
Q

Denys-Drash syndrome - manifestation

A
  1. Wilms tumor
  2. early onset nephrotic syndrome
  3. male pseudohermaphroditism
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16
Q

Beckwith-Wiedemann syndrome manifestations

A
  1. Wilms
  2. macroglossia
  3. organomegaly
  4. hemihypertrophy (one side of the body or a part of one side of the body is larger than the other)
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17
Q

Renal cyst disorders - types

A
  1. Autosomal dominant polycystic kidney disease (ADPKD)
  2. Autosomal recessive polycystic kidney disease (ARPKD)
  3. Medullary cystic disease
  4. Simple renal cysts
  5. complex renal cysts
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18
Q

Autosomal dominant polycystic kidney disease - gross appearance

A

Numerous cysts in cortex and medulla –> bilateral enlarged kidneys ultimately destroy kidney parenchyma

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19
Q

Autosomal dominant polycystic kidney disease - present with (clinical presentation)

A
  1. flank pain
  2. hematuria
  3. hypertension
  4. urinary infection
  5. Progressive renal failure (50%)
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20
Q

Autosomal dominant polycystic kidney disease - Mechanism

A
PKD1 mutation (85% - ch 16)
PKD2 mutation (15% - ch 4)
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21
Q

Autosomal dominant polycystic kidney disease is associated with

A
  1. berry aneurysm
  2. mitral valver prolapse
  3. benign hepatic cysts
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22
Q

Autosomal recessive polycystic kidney disease is associated with

A
  1. congenital hepatic fibrosis

2. Significant oliguric renal failure in utero –> Potter sequence

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23
Q

Autosomal recessive polycystic kidney disease - concern beyond neonatal period include

A
  1. hypertension
  2. progressive renal insufficiency
  3. portal hypertension (from congenital hepatic fibrosis)
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24
Q

Medullary cystic disease - histology and mechanism

A

Inherited disease causing tubulointerstitial fibrosis and progressive renal insufficiency with inability to concentrate urine

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25
Q

Medullary cystic disease - gross

A

Medullary cysts usually not visualised

shrunken kidneys on ultrasound

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26
Q

Medullary cystic disease - US

A

shrunken kidneys

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27
Q

Medullary cystic disease - prognosis / complications

A

poor –> progressive renal insufficiency with inability to concentrate urine

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28
Q

kidney stones - types and urine crystals

A
  1. Calcium oxalate –> shaped like envelope or dumbbell
  2. Caclicum phosphate –> wedge-shaped prism
  3. Ammonium magnesium phosphate –> coffin lid
  4. Uric acid –> Rhomboid or rosettes
  5. Cystine –> hexagonal
29
Q

kidney stones - types/precipitating factors/x-ray/CT

A
  1. calcium oxalate –> low ph, radiopaque in both
  2. caclicum phosphate –> high ph, radiopaque in both
  3. Ammonium magnesium phosphate, high ph, radiopaque in both
  4. Uric acid –> low ph, radiolucent in x-ray, minimally vissible in CT
  5. Cystine –> low ph, radiolucent in xray, sometimes vissuble in CT
30
Q

causes of Cystine stones

A

Hereditary (AR) condition in which Cystine -reabsorbing PCT transporter loses function causing cysteinuria (also poor reabsorption of ornithine, lysine, arginine) –> cystine is poorly soluble, thus stones form in urine
(mneomnic: COLA: Cystine, Ornithine, Lysine, Arginine)

31
Q

kidney stones - types and urine crystals

A
  1. Calcium oxalate –> shaped like envelope or dumbbell
  2. Caclicum phosphate –> wedge-shaped prism
  3. Ammonium magnesium phosphate –> coffin lid
  4. Uric acid –> Rhomboid or rosettes
  5. Cystine –> hexagonal
32
Q

enuresis - definition

A

urinary incontinense in children 5 or older than 5

33
Q

enuresis - treatment

A

education, enuresis alarms, dietary modification (no fluids near bedtime), desmopressin or imipramine n refractory cases
(also urinalysis to rule out 2ry causes)
desmopresin has high rate of relpase after stopped when is monotherapy

34
Q

MCC of urinary tract obstruction in newborn boys

A

posterior urethral valves

35
Q

children enuresis - DM1 vs DI

A

DI is very rare in children

36
Q

enuresis with polyurea, polydepsia weight loss

A

DM1

37
Q

2ry enuresis definition

A

enuresis after 6 months of dryness

38
Q

hbv, hcv nephritis - types

A

nephrotic –> membranous

nephritic –> membranoproliferative

39
Q

definitive diagnosis of vesiculateral reflex

A

voiding cystourethrogram

40
Q

vesiculateral reflex - grades

A
  1. into nondilated ureter
  2. into pelvis + calyces without dilation
  3. mild to moderate dilatation ureter, pelivis, calyces with minimal blunting
  4. moderate ureteral tortuosity + dilation of the pelvis + calyces
  5. gross dilation of ureter, pelvis, calc=cyes, loss of papillary impressions, ureteral tortuosity
41
Q

renal tubular acidosis - types

A

type 1. distal
type 2. proximal
type 4

42
Q

type 1 (distal) tubular acidosis (mechanism, urine ph, K+, causes)

A
  • poor hydrogen secretion into urine
  • urine ph higher than 5.5
  • low/normal serum potasium
  • causes: genetic, medication, autoimmune
43
Q

type 2 (proximal) acidosis (mechanism, urine ph, K+, causes)

A
  • poor HCO3-
  • urine ph lower than 5.5
  • low/normal K+
  • fanconi syndrome (glucosuria, Phosphaturia, aminoaciduria)
44
Q

type 4 acidosis (mechanism, urine ph, K+, causes

A
  • aldosterone resistance
  • urine ph LOWER than 5.5
  • serum K high
  • obstructive uropathy, congenital adrenal hyperplasia
45
Q

hyposthenuria - definition, RF

A
  • inability of kidneys to concentrate urine
  • in SC anemia or trait
  • polyuria, low urine specific gravity, normal serum sodium
46
Q

isolated proteinuria in children

A
  • MCC: transient proteinuria
  • reevaluate with repat urine dipstick testing on 2 separate occasions to rule out persistent proteinuria
  • by fever exercise, seizures, stress, volume depletion
47
Q

proteinuria on children - types and MC - (management)

A
  • transient (MC)
  • orthostatic
  • persistent
48
Q

proteinuria - when we need further check

A

still proteinuria on the repeat sample –> 24 urine collection, U/S, and maybe biopsy

49
Q

MCC of nephrotic syndrome in children under 10/ when to biopsy

A
  • minimal changes disease

- biopsy if no response to steroids or progressive disease, older children, adolescents

50
Q

complications of constipation in urinary system

A

enuresus or stasis

51
Q

indications of renal + bladder U/S

A
  1. infatns and children under 24 months with 1st febrile UTI
  2. reccurent febrile UTI in children of any age
  3. UTI in child of any age with family history of renal or urologic disease, hypertension or poor growth
  4. no respond to appropriate antibiotic treatment
52
Q

how to check for UTI in children with diapers

A

cathetirization (to avoid contamination from stools)

if older: mid stream

53
Q

renal complications of sickle cell anemia trait

A
  1. hyposthenurua
  2. hematuria
  3. UTI
  4. medullary carcinoma
54
Q

Henoch - Schonlein - manifestation / treatment

A

triad: arthralgia, abd pain, palpable purpura on legs and buttocks + IGA NEPHROPATHY
- supportive (NSAID and hydration)
- if severe: hospitalization + glucocorticoids

55
Q

severe hypovolemic hpernatremia - initial treatment

A

isotonic: 0.9 saline or Ringer (never hypotonic)

56
Q

Alport - manifestations

A
  • recurrent episodes of hematuria
  • sensorineural deafness
  • family history of renal failure
57
Q

wimls tumor - treatment + prognosis

A

tumor excision or nephrectomy, chemo, +/- radiaton

prognosis: 90% in 5 years with treatment

58
Q

wilms vs neuroblastoma regarding age

A

wilms: 2-5
neurob: 0-1

59
Q

pyloric stenosis - what to do before surgery

A

correct electrolytic abnormalities as alkalosis is related with postoperative apnea

60
Q

wilms - site of metastasis

A

lung: but rare on presentation

61
Q

RF for pediatric constipation

A
  1. initiation of solid food and cow milk
  2. toilet training
  3. school entry
62
Q

RF of UTI in small children

A
  1. gilrs
  2. renal anomaly
  3. uncircumcised boy
63
Q

non renal complication on SC trait

A
spenic infraction (esp at higher altitudes, venous thromboembolism, priapism
- exertional rhabdomyolysis
64
Q

serum Na2+ in hyposthenuria due to SC trait

A

normal (due to normal ADH)

65
Q

pediatric etiologies of nephritic syndrome

A
  1. PSGN

2. Hemolytic uremic syndrome

66
Q

the preferred modality for long term evaluation for renal scarring

A

renal scintigraphy with dimercaptosuccinic acid

67
Q

indications for voiding cystourethogram

A
  1. hydronephrosis or scarring in U/S
  2. newborn with UTI
  3. less than 2 years with recurrent UTI
  4. UTI from organism other than E.coli
68
Q

pyurea in children means

A

5 or more WBC/hpf