A CASE REPORT ON DISTAL RENAL TUBULAR ACIDOSIS

Dr. Bantupalli Vinisha, Dr. B. Raghava, Dr. Marina Amarendra

INTRODUCTION INVESTIGATIONS DISCUSSION

Distal RTA (type 1) is due to defective secretion of h+ in Rbs : 128 mg/dl Hb : 9.8g/dl Tlc : 13800 N(68%) L(28%) Primary or secondary causes of distal RTA can result in
distal tubules. M(01%) E(03%) Platelet count : 3.08L/cu mm [Link] : 28 mg/dl damaged or impaired functioning of one or more transporters
The condition is often sporadic but may be inherited [Link] : 0.6mg/dl sodium : 137mmol/l potassium : or proteins involved in the acidification process.
Dominant,Recessive(associated with Sensorneural 2.8mmol/l Because of impaired hydrogen ion excretion, urine pH
Deafness)Or X-Linked. chloride : 120.3mmol/l ph : 7.176 bicarbonate : 12 po2 : 179 cannot be reduced to <5.5,despite the presence of severe
There is an association with systemic diseases mm hg pco2 : 27.2 mm hg urine ph : 6.0 metabolic [Link] to secrete H+ is compensated by
(SLE,sickle cell,wilsons) and drug toxicity albumin : nil sugar : nil microscopy : 1-2 pus cells/HPF increased K+ secretion distally, leading to hypokalemia
(lithium,cisplatin,amphotericin B). urine anion gap : positive Hypercalciuria is usually present and can lead to
Children present with failure to renal u/s : B/L medullary nephrocalcinosis nephrocalcinosis or nephrolithiasis. Bone disease is
thrive,polyuria,hypokalemic muscle weakness,rickets. common, resulting from mobilization of organic components
from bone to serve as buffers to chronic acidosis
Biochemical abnormalities include hyperchloremic metabolic
CASE PRESENTATION acidosis, hypokalemia, increased urinary excretion of
calcium and decreased urinary citrate. Despite moderate to
severe acidosis, patients cannot lower urine pH below 5.3.
AGE - 18months Gender - male Treatment: Hypokalemia should be treated before correction
H/o Fast breathing and recurrent episodes of vomiting of acidosis. Acidosis is treated by administration of sodium
since 2days bicarbonate (initially 2-3 mEq/kg in divided doses); the dose
No h/o fever,cough&cold,diarrhea of alkali can be increased until the blood bicarbonate level is
h/o failure to thrive h/o polyuria normal . Treatment of acidosis reduces potassium losses
past h/o similar episode at 11 months of age and promotes growth and healing of [Link] D
only child born out of second degree consanguinous supplements are not required.
O/E : irritable,breathing fast and deep
afebrile , tachypnea+ , tachycardia+ ,bp normal
no cyanosis,pallor,edema,lymphadenopathy MANAGEMENT
o/e fronto parietal bossing+
rachiatic rosary+ , b/l widening of wrist+ References:
R/S - B/L air entry equal , nvbs+, no added sounds After correcting dehydration and hypokalemia,acidosis Nelson textbook of Paediatrics 21st edition
corrected with oral supplementation of sodium Mantan M. Indian Pediatr. 2005 Apr;42(4):321-8
CVS – s1s2 normal intensity Approach to Renal Tubular Disorders [Indian J Pediatr 2005; 72 (9) : 771-776
P/A – liver 3cm below RCM , spleen tip palpable bicarbonate@2meq/kg/day in divided [Link] of alkali
Bagga textbook of nephrology
CNS – no focal neurological deficit increased upto 5meq/kg/day,until the blood bicarbonate is
normal. Hearing evaluation is normal.