GIT review Flashcards

1
Q

congenital malformations of the kidney

A

20% of renal failure in kids is due to renal dysplasia or hypoplasia

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2
Q

agenesis of the kidney

A

complete absence of one or both kidneys
results from a major disruption of metanephric development at an early stage (interaction between the ureteric bud and metanephric blastema
mix of genetic and environmental factors including infections

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3
Q

bilateral agenesis

A

potter’s syndromes

  • incompatible with life
  • oligohydrammois (fetal urine produced around week 16, usually contributes to amniotic fluid)
  • hypoplastic lungs
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4
Q

patter’s syndrome also called

A

bilateral renal agenesis

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5
Q

potters facies

A
large low set ears 
wide set eyes 
flattening of the nose 
receding chin 
prominent skin folds over the eyes
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6
Q

unilateral agenesis

A
  • usually asymptomatic
  • many have other abnormalities of the genitourinary tract like vesicoureteric reflux
  • ipsilateral ureter is absent or rudimentary
  • the contralateral kidney undergoes compensatory hypertrophy
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7
Q

hypoplasia of the kidneys

A

small but normally developed

usually occurs unilaterally

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8
Q

ectopic kidney

A
  • A Kidney not located in its usual position
  • usually just above the pelvic brim or within the pelvis (resulting from failure of the kidney to ascend during embryogenesis)
  • rarely seen in the thoracic cavity
  • normal or slightly small, flatenned
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9
Q

horseshoe kidney

A

fusion of the upper or lower poles of the two kidneys
usually lower poles
gets hooked at the inferior mesenteric artery (lower in the abdomen than normal kidneys)
common and usually incidentally detected

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10
Q

polycystic disease of the kidney

A

polycystic disease

  • autosomal dominant PKD
  • autosomal recessive PKD
  • glomerulocystic kidney disease
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11
Q

cystic disease of the renal medulla

A
  • nephronophthisis

- medullary sponge kidney

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12
Q

simple renal cysts

A
  • common finding in normal kidneys
  • usually cause no symptoms or signs
  • occasionally can rupture, cause haematuria, pain, abdominal mass, infection, hypertension
  • major issue is excluding a more serious disorder eg. malignancy
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13
Q

clues to malignancy in cystic renal lesions

A
  • multiple septa
  • thicket cyst wall
  • solid areas within or around the cyst
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14
Q

cystic renal dysplasia

A
  • malformation of the kidney
  • unilateral or bilateral
  • often associated with obstruction/reflux
  • consists of irregular cysts or varying sizes
  • common cause of an abdominal mass in infants
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15
Q

histology of cystic renal dysplasia

A

persistence of abnormal structures like cartilage, immature mesenchyme, immature collecting ductules, with abnormal lobular organisation

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16
Q

polycystic kidney disease

A
  • cystic genetic disorders of the kidneys
  • two types - autosomal dominant and recessive
    both types are characterised by numerous fluidd filled cysts in the kidneys, causing massive enlargement
17
Q

autosomal dominant polycystic kidney disease

A
  • common
  • 10% of end stage renal failure patients having dialysis have ADPKD
  • autosomal dominance with high penetrance
  • proteins called polycystins which are important in the function of cilia
18
Q

ADPKD development

A

cyst formation begins in utero
cysts grow overtime, compress surrounding renal tissue, cause renal failure
kidneys become overgrown

19
Q

presenting complaint of ADPKD

A
  • patients typically present in 30s-50s
  • flank pain due to abdominal mass or renal stones
  • pain from haemorrhage into a cyst
  • haematuria
  • hypertension
20
Q

associated congenital abnormalities of ADPKD

A
  • cysts in the liver, pancreas, spleen , lungs
  • intracranial berry aneurysms
  • mitral valve prolapse
21
Q

autosomal recessive polycystic kidney disease

A
  • rare
  • autosomal recessive
  • PKHD1 gene - protein called fibrocystic in cilia
  • also causes liver cysts
  • smooth kidneys, smaller cysts
  • requires kidney transplant for survival
22
Q

obstructive uropathy

A
  • obstruction to the urinary tract
  • acute or chronic
  • may be unilateral or bilateral
  • any level from urethra to pelvis
  • intrinsic, extrinsic
  • enlargement of the prostate is a common cause
23
Q

causes of obstructive uropathy

A
  • congenital anomalies
  • urinary calculi
  • benign prostatic hypertrophy
  • tumours of the prostate, bladder, lymph nodes
  • inflammation
  • sloughed renal papillae/blood clots
  • normal pregnancy
  • uterine prolapse and cystocoele
  • functional
24
Q

symptoms of obstructive uropathy

A
  • kidney enlargement
  • dilatation of the renal pelvis.calyces
  • hydronephrisis
  • thick walled bladder - muscle hypertrophies
  • hydroureter - ureter expands
25
Q

hydropnephrosis

A

water inside the kidney
distension and dilatation of the renal pelvis and calyces, caused by obstruction to the free flow of wine from the kidney
leads to atrophy of the kidney

26
Q

obstructive uropathy clinical presentation

A
  • pain from dilatation of the renal capsule/ureter/bladder
  • urinary tract infections
  • urinary calculi
  • renal failure
  • partial bilateral obstruction - inability to concentrate urine
  • complete bilateral obstruction - fatal
27
Q

urolithiasis

A

common
men >women
starts 20-30
often have family history

28
Q

types of kidney stones

A
  • calcium oxalate
  • uric acid
  • struvute
  • calcium phosphate
  • others
29
Q

calcium containing stones (usually calcium oxalate) risk factors

A
  • dehydration
  • hypercalciuria (with or without hypercalcaemia)
  • hyperoxaluric - genetic defects (increased oxalate excretion), dietary causes
30
Q

hypercalcaemia

A

increase absorption in the GI tract, hormonal causes

31
Q

hypercalciuria

A

impaired renal tubules don’t reabsorb Ca effectively

32
Q

hyperoxaluric

A

genetic defects (increased oxalate excretion), dietary causes - rhubarb, spinach, chocolate, nuts and beer

33
Q

uric acid stones - risk factors

A

dehydration
acidic urine
hyperuricaemia - diet rich in shellfish, red meat
increased urinary excretion of uric acid - rapid cell turnover eg,. leukaemia, chemotherapy

34
Q

struvite stones

A
  • magnesium ammonium phosphate
  • after infection with urea-splitting bacteria
  • urea becomes ammonia, making urine more alkaline, precipitation of magnesium ammonium phosphate
35
Q

complications of urolithiasis

A
  • silent
  • pass into the ureters - pain and obstruction
  • ulceration and bleeding - haematuria
  • infection
36
Q

renal colic

A

excruciating pain
often constant rather than intermittent
radiates from the flank to the groin
urinary urgency, restlessness, haematuria, sweating, nausea, vomting

pain associated with a ureteric stone

37
Q

summary of obstructive disease

A
  • structural or functional hindrance of urine flow, sometimes leads to renal dysfunction
  • can occur at any point in the urinary tract, can affect one or both kidneys
  • common cause is calculi
  • common presentation is renal colic
  • complications are infection, renal failure