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
Medullary cystic disease - gross
Medullary cysts usually not visualised | shrunken kidneys on ultrasound
26
Medullary cystic disease - US
shrunken kidneys
27
Medullary cystic disease - prognosis / complications
poor --> progressive renal insufficiency with inability to concentrate urine
28
kidney stones - types and urine crystals
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
kidney stones - types/precipitating factors/x-ray/CT
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
causes of Cystine stones
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
kidney stones - types and urine crystals
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
enuresis - definition
urinary incontinense in children 5 or older than 5
33
enuresis - treatment
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
MCC of urinary tract obstruction in newborn boys
posterior urethral valves
35
children enuresis - DM1 vs DI
DI is very rare in children
36
enuresis with polyurea, polydepsia weight loss
DM1
37
2ry enuresis definition
enuresis after 6 months of dryness
38
hbv, hcv nephritis - types
nephrotic --> membranous | nephritic --> membranoproliferative
39
definitive diagnosis of vesiculateral reflex
voiding cystourethrogram
40
vesiculateral reflex - grades
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
renal tubular acidosis - types
type 1. distal type 2. proximal type 4
42
type 1 (distal) tubular acidosis (mechanism, urine ph, K+, causes)
- poor hydrogen secretion into urine - urine ph higher than 5.5 - low/normal serum potasium - causes: genetic, medication, autoimmune
43
type 2 (proximal) acidosis (mechanism, urine ph, K+, causes)
- poor HCO3- - urine ph lower than 5.5 - low/normal K+ - fanconi syndrome (glucosuria, Phosphaturia, aminoaciduria)
44
type 4 acidosis (mechanism, urine ph, K+, causes
- aldosterone resistance - urine ph LOWER than 5.5 - serum K high - obstructive uropathy, congenital adrenal hyperplasia
45
hyposthenuria - definition, RF
- inability of kidneys to concentrate urine - in SC anemia or trait - polyuria, low urine specific gravity, normal serum sodium
46
isolated proteinuria in children
- 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
proteinuria on children - types and MC - (management)
- transient (MC) - orthostatic - persistent
48
proteinuria - when we need further check
still proteinuria on the repeat sample --> 24 urine collection, U/S, and maybe biopsy
49
MCC of nephrotic syndrome in children under 10/ when to biopsy
- minimal changes disease | - biopsy if no response to steroids or progressive disease, older children, adolescents
50
complications of constipation in urinary system
enuresus or stasis
51
indications of renal + bladder U/S
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
how to check for UTI in children with diapers
cathetirization (to avoid contamination from stools) | if older: mid stream
53
renal complications of sickle cell anemia trait
1. hyposthenurua 2. hematuria 3. UTI 4. medullary carcinoma
54
Henoch - Schonlein - manifestation / treatment
triad: arthralgia, abd pain, palpable purpura on legs and buttocks + IGA NEPHROPATHY - supportive (NSAID and hydration) - if severe: hospitalization + glucocorticoids
55
severe hypovolemic hpernatremia - initial treatment
isotonic: 0.9 saline or Ringer (never hypotonic)
56
Alport - manifestations
- recurrent episodes of hematuria - sensorineural deafness - family history of renal failure
57
wimls tumor - treatment + prognosis
tumor excision or nephrectomy, chemo, +/- radiaton | prognosis: 90% in 5 years with treatment
58
wilms vs neuroblastoma regarding age
wilms: 2-5 neurob: 0-1
59
pyloric stenosis - what to do before surgery
correct electrolytic abnormalities as alkalosis is related with postoperative apnea
60
wilms - site of metastasis
lung: but rare on presentation
61
RF for pediatric constipation
1. initiation of solid food and cow milk 2. toilet training 3. school entry
62
RF of UTI in small children
1. gilrs 2. renal anomaly 3. uncircumcised boy
63
non renal complication on SC trait
``` spenic infraction (esp at higher altitudes, venous thromboembolism, priapism - exertional rhabdomyolysis ```
64
serum Na2+ in hyposthenuria due to SC trait
normal (due to normal ADH)
65
pediatric etiologies of nephritic syndrome
1. PSGN | 2. Hemolytic uremic syndrome
66
the preferred modality for long term evaluation for renal scarring
renal scintigraphy with dimercaptosuccinic acid
67
indications for voiding cystourethogram
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
pyurea in children means
5 or more WBC/hpf