- Light Wood-Albright syndrome.

Light Wood-Albright syndrome
Lightwood syndrome
Renal tubular acidosis (RTA) (Type-2)
(Proximal RTA)

-Pathophsiology:-

it is caused by failure of the proximal tubular cells to reabsorb filtered bicarbonate from the urine, leading to urinary bicarbonate wasting and subsequent metabolic acidosis.

-Clinical picture:-

• Rickets(in children).
  
• Osteomalacia (in adults).
• Metabolic acidosis.
  
• Vomiting ( due to acidosis).
• Polyuria and polydepsia.
• Episodes of fever and dehydration.
• Muscle weakness and constipation.
• Growth retardation.

-Investigation:-

• Ph (decrease).
• Ca++ (Normal).
• Alkaline phosphatase (increased).
• increase urinary phosphate,bicarbonate,glucose, amino acids and may be Potassium.

-Treatment:-

• Oral phosphate 1-3 gm/day divided into 5 doses.
• Vitamin D: 2000 IU/day.
• Sodium bicarbonate tablets or Sodium citrate and Potassium citrate syrup for metabolic acidosis.
• Potassium chloride 2-3 mEq/kg/day for cases of hypokalemia.
  

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