Home
Keys to Success in Medicine
Cardiovascular System
Respiratory System
Locomotor System
Endocrine and Metabolic System
Kidneys and Urinary System
Gastrointestinal Tract
Central Nervous System
Haematological System
Integumental System
Reproductive and Genital System
Analysing Medical Investigations
Recommended Reading
Forum
Links

amazon astore

ydr

aces for paces

Clinical Skills Blogspot

Type 1 Renal Tubular Acidosis

Title               

Ñ    Type 1 Renal Tubular Acidosis

Causes

Genetic

Ñ    Autosomal dominant

KUS

Ñ    Obstructive uropathy

Ñ    Medullary sponge kidney

Ñ    Pyelonephritis

Ñ    Papillary necrosis

Ñ    Nephrocalcinosis

Ñ    Renal transplantation

LMS

Ñ    Sjögren’s syndrome

Ñ    SLE

GIT

Ñ    Chronic active liver disease

RS

Ñ    Fibrosing alveolitis

HS

Ñ    Sickle-cell anaemia

Ñ    Hypergammaglobulinaemia

Ñ    Dysglobulinaemia

E&M

Ñ    Mineralocorticoid deficiency

Drugs

Ñ    Trimethoprim

Ñ    Amphotericin

Ñ    Analgesic nephropathy:

Papillary necrosis

Pathophysiology

Ñ    Failure of H⁺ ion secretion in the distal tubule

Results in:

Ñ    Acidosis

Ñ    Hypokalaemia:

With a few exceptions

Ñ    Failure to reduce urinary pH to  < 5.3 despite systemic acidosis

Ñ    Low urinary ammonium production

Ñ    Low urinary citrate

Ñ    Hypercalciuria

Clinical Features

History

Age

May present in:

Ñ    Infancy

Ñ    Childhood

Ñ    Adult life

E&M

Ñ    Failure of growth

Ñ    Lethargy

RS

Ñ    Hyperventilation

GIT

Ñ    Anorexia

KUS

Ñ    Calculi 

Due to:

·          Hypercalciuria

·          Hypocitraturia

·          Alkaline urine

Ñ    UTI (urinary tract infection):

Stones

Ñ    Chronic renal failure

CNS

Ñ    Periodic paralysis

LMS

Ñ    Rickets, osteomalacia:

Depletion of calcium from bone due to buffering of hydrogen ions by calcium ions in bone

Investigations

Fluids

Blood

Biochemistry

Ñ    Hyperchloraemic acidosis

Ñ    Hypokalaemia:

Rare exceptions

Imaging

X-ray KUB

Ñ    Nephrocalcinosis

Physiological studies

Acid load test

Ñ    Acid load (100 mg/kg ammonium chloride):

Urine pH remains > 5.3 despite HCO₃^– of 21 mmol/L

Management

Control

Drugs

Ñ    Bicarbonate

Ñ    Potassium supplements

Ñ    Citrate

Ñ    Thiazide diuretics:

Volume contraction with increased proximal bicarbonate reabsorption

Acute Situation

Ñ    Correct hypokalaemia before acidosis

Prognosis

Treatment with bicarbonate

Ñ    Prevents exacerbations of acidosis

Ñ    Prevents potassium loss

Ñ    Hypercalciuria diminishes

Ñ    Hypocitraturia improves

Ñ    Osteomalacia and rickets improve

Ñ    Growth restored in children

Ñ    Halts progression of nephrocalcinosis and nephrolithiasis

[ Up ] [ Next ]

[Up]