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Renal tubular disease (Proximal tubular dysfunction (Fanconi syndrome)…
Renal tubular disease
Proximal tubular dysfunction
(Fanconi syndrome)
Pathophysiology
PCT dysfunction resulting in loss of aa, glucose, phos, and bicarb into the urine
Dehydration, metabolic acodisis, rickets/osteomalacia, electrolyte disturbances
Diagnosis
History: congenital disease, autoimmune disease
Examination: rickets, dehydration
Investigations: bedside (nil), bloods (ABG [metab acidosis], FBC, U+E [deranged; low HCO3- and phos]), urine (high HCO3-, high phos])
Aetiology
Congenital/genetic, cystinosis, Wilsons
Drugs: heavy metals, gentamycin, cisplatin
Autoimmune: amyloidosis, Sjogrens
Neoplastic: myeoloma
Clinical presentation
Faltering growth, rickets/osteomalacia, polydipsia, polyuria
Management
Conservative: fluids
Medical: replace losses (glucose, HCO3-, phos)
Renal tubular
acidosis (RTA)
Type 1
(distal)
Complications
Renal calculi
ESRF
Management
Conservative: fluids
Medical: PO bicarbonate/citrate
Diagnosis
History: rickets, renal stones, UTIs
Examination: rickets, abdo pain
Investigations: bedside (nil), bloods (ABG [metab acidosis], FBC, U+E, Ca [high]), urine (pH>5.5, stones, low citrate)
Pathophysiology
Inability to secrete H+ in DCT
Alkaline urine and metabolic acidosis
Ca from bone used to buffer H+
Clinical presentation
Failure tothrive
Rickets/osteomalacia
Renal stones (ca phosphate)
Recurrent UTIs
Aetiology
Genetic: genetic RTA type 1
Autoimmune: SLE, Sjogrens
Drug: amphoteracin
Idiopathic: famailial
Degenerative: tubulointerstitial disease
Type 4
(hyper-K)
Diagnosis
History: abdo pain if stone, systemic disease signs
Examination: signs of systemic disease
Investigations: bedside (nil), bloods (ABG [metab acidosis], FBC, U+E (low K), urine 9low K, low H)
Pathophysiology
Low renin and aldosterone, thus low K+ and H+ excretion
High serum K+ and H+ (metabolic acidosis)
Management
Conservative: fluids
Medical: replace losses (K+, bicarbonate, Vit D, phospherus)
Aetiology
Drugs: K+ sparing diuretics, NSAIDs
Metabolic: DM nephropathy
Autoimmune: Addisons, SLE
Type 2
(proximal)
Aetiology
Idiopathic: familial
Degenerative: tubulointerstitial disease
Genetic: Fanconi syndrome
Pathophysiology
Defect in HCO3- reabs in PCT
Metabolic acidosis and alkaline urine
Lack of reabs in PCT causes inc flow rate to DCT and inc secretion of K (hypo-K)
Diagnosis
History: renal stones
Examination: abdo pain if stone
Investigations: bedside (nil), bloods (ABG [metab acidosis], FBC, U+E [low K]), urine (pH>5.5)
Management
Conservative: fluids
Medical: IV bicarbonate (high dose)
Hereditary hypo-K
tubulopathies
Gitelman syndrome
Mutation of K+ transporter
Low K+, low Ca+, low Mg, metabolic alkalosis
Per thiazide diuretic action
Bartter syndrome
Mutation of K+/Na+/2Cl- transporter
LOw K+, high Ca, metabolic alkalosis
Per loop diuretic action
Cystinosis
Management
Replace missing electrolytes and cysteine
Pathophysiology
AR mutation and accumulation
of cysteine in lysosomes
Fanconi syndrome, visual impairment,
myopathy, hypothyroidism