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