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Renal > Tubulo-intestitial disease > Flashcards

Tubulo-intestitial disease Flashcards

1
Q

Definition of tubulo disorders

A

nephritis affecting renal tubules and interstitual disease

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2
Q

Classfication

A

Proximal tubulopathies

  • Renal tubular acidosis – type 2 RTA
  • Fanconi’s syndrome
  • Cystinosis

Loop of henle
-bartters syndrome

Distal tubulopathies
-gitelman syndrome

Collecting ducts
-nephrogenic diabetes insipidous

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3
Q

Renal tubular dysfunction- Where is the problem?

A

Electrolyte disturbance/loss

  • Na, K, Cl, PO4
  • Aminoaciduria
  • Glycosuria

Acid-Base disturbance → metabolic acidosis

  • HCO3 loss – proximal tubule
  • Failure of H+ excretion – distal tubule

Renal concentration defect – collecting duct
-Polyuria

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4
Q

Fanconis syndrome definition

A

Diffuse proximal tubular dysfunction

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5
Q

Clinical feautures of fanconis

A 
Polyuria/polydipsia
Volume depletion
FTT
Constipation
Rickets
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6
Q

Pathophysiology of fanconis

A

Loss of

  • AA
  • Glucose
  • PO4
  • HCO3-

in the urine

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7
Q

Causes of fanconis syndrome

A 
Idiopathic 
Genetic 
-Wilsons
-drugs 
-Multple myelome
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8
Q

Nephropathic cystinosis incidence

A

1 in 100000 children

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9
Q

Genetics of nephropathic cystinosis

A

AR inheritance
CTNS gene mutation on chromosome 17p13
Defect in lysosomal cystine transport leading to excessive intracellular cystine accumulation

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10
Q

Pathophysiology of nephropathic cystinosis

A

Raised white cell cystine levels: diagnostic

Impaired proximal tubular reabsorptive capacity

  • hypokalaemia, hypophosphataemia, metabolic acidosis, glycosuria, and aminoaciduria (Fanconi syndrome)
  • sodium wasting
  • ADH resistance (nephrogenic diabetes insipidus)

Progressive glomerular renal failure

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11
Q

Clinical features of nephropathic cystinosis

A 
Fanconi’s syndrome
Extra-renal disease
-Corneal cystine crystal deposits – photophobia
-Skeletal - Growth retardation
-Hypothyroidism
-Hepato-splenomegaly - hypersplenism
-Neurological: deterioration/cerebral atrophy
-Pubertal delay and infertility
-Pancreatic insufficiency - DM

Renal Failure

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12
Q

management of nephropathic cystinosis

A

Supportive:Fluid and electrolyte replacement

Specific: Mercaptamine – cystine depletion

  • Systemic oral therapy: Monitor white cell cystine levels
  • Topical cysteamine eye therapy

Other: Indomethacin – to reduce polyuria

CKD treatment
NB: Transplantation – does not correct underlying disorder

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13
Q

barrters syndrome genetics

A

AR

Defects in NKCC2; KCNJ1; CLCNKB genes identified

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14
Q

Pathophysiology of barrters syndrome

A

Defect in Na+-K+-2Cl- co-transporter

Thick ascending limb of Loop of Henle

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15
Q

LAB presentation of barrters syndrome

A

Plasma magnesium – normal
Urinary Chloride - high
Urinary Calcium - normal/high
Hyperreninaemia – normal BP

Hypochloraemic hypokalaemic alkalosis

low chloride low potassium alkalotic picture!!

Similar picture with Furosemide
(Loop Diuretics)

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16
Q

clinical features of barters

A

In-utero

  • Polyuria– polyhydramnios
  • IUGR
  • Premature delivery

Neonate

  • Polyuria
  • Electrolyte disturbance
  • Poor growth
  • Hypercalciuria and nephrocalcinosis

Childhood

  • Polyuria
  • Electrolyte disturbance
  • Episodes of dehydration

Adults
-Electrolyte disturbance

17
Q

Management of bartters syndrome

A

K+ replacement
Fluid replacement

Indomethacin

  • PG syntethase inhibitor –
  • Reduces renal salt, water and potassium loss – reducing GFR
18
Q

Psuedo-Bartters syndrome definition

A

Hypocholaemic hypokaelmic alkalosis

But apparently low urinary levels:

  • urine chloride
  • urine sodium
19
Q

Causes of psuedo-bartters syndrome

A

Cystic fibrosis – sweat losses of NaCl and water

Congenital chloride diarrhoea – gut loss

Laxative abuse – gut loss
Cyclical vomiting

20
Q

Gitelman syndrome

A

Compare to Bartter’s syndrome
AR
Defect in distal tubule Na-Cl co-transporter

Hypokalaemic metabolic alkalosis
Plasma magnesium - low
Urinary calcium – low

Similar picture with Thiazide Diuretics

21
Q

Nephrogenic diabetes insipidus genetics

A

X-linked/AR inheritance

22
Q

Nephrogenic diabetes insipidous causes

A

Arginine Vasopressin receptor defect
Aquaporin-2 water channel
defect -Located in collecting duct

Resistance to ADH / Arginine Vasopressin – leading
defective urinary concentration

Hypernatremic dehydration

23
Q

Treatment of nephrogenic diabetes insipidus

A

Fluid intake
Low solute load
Thiazide diuretic/indomethacin

24
Q

Tubulo-interstinital nephritis causes

A

Inflammation of tubulo-interstitium
Presumed to be immune-mediated
Sparing of glomeruli and vessels
Usually secondary to drugs/infections/other causes

Spectrum of pathology –
Acute and reversible nephritis – to – Chronic and irreversible disease

25
Q

Clinical features of tubulo-interstiial nephritis

A

ARF

  • No HT;
  • No proteinuria

CRF

Tubulopathy
Proximal -Fanconi syndrome, Renal tubular acidosis

Distal tubular cell injury -Acidosis and hyperkalaemia

Collecting duct damage -Urinary concentrating defect-Potassium wasting

26
Q

Causes of tubulo-interstitial nephritis

A

Idiopathic
Drugs (NSAIDS, anti0microbials, antiviral)
Infections

Tubulo-interstial nephritis with uveitis – TINU

Systemic disease:Sarcoidosis
Lymphoma/leukaemia, IBD

Other-Heavy metals

DRUGS MOST IMPORTANT CAUSE

27
Q

Management of tubulo-interstitual nepritits

A

Acute

  • remove drug
  • steroids?

Treat primary disease process
Minimise proteinuria
Suppression of inflammation
Management of CKD

Renal (12 decks)

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