II. Epidemiology
- Most commonly affects children
III. Pathophysiology
- Proximal Tubule defect of bicarbonate reabsorption
- Results in bicarbonate wasting- Initially distal tubule attempts to reabsorb
- Later distal tubule mechanisms overcome
 
IV. Causes
- Medications
- Fanconi's Syndrome
- Medullary cystic disease
- Multiple Myeloma
- Nephrotic Syndrome
- Renal Transplantation
V. Signs (Presentation)
- Failure to Thrive
- Growth retardation
- Vomiting
- Dehydration
- Lethargy
VI. Labs
- 
                          Arterial Blood Gas
                          - Mild to moderate Non-Anion Gap Metabolic Acidosis
 
- Serum bicarbonate decreased- Usually not lower than 15 meq/L
 
- 
                          Urine pH
                          - Exceeds 5.5 except in severe Metabolic Acidosis
 
- 
                          Fractional Excretion of Bicarbonate
                          - FE-HCO3 exceeds 15% if serum bicarbonate >20 meq/L
- FE-HCO3 <5% in Distal RTA
 
VII. Radiology: XRay
- Children: Rickets
- Adults: Osteopenia
VIII. Management
- High dose Bicarbonate Supplementation- Oral Bicarbonate 10-25 meq/kg/day
 
- Observe for Hypokalemia
- Treat Osteomalacia in adults
- Treat Rickets in children- Vitamin D Supplementation
- Sodium phosphate 1.6 grams per day
 
