Journal of Integrative Nephrology and Andrology

: 2015  |  Volume : 2  |  Issue : 3  |  Page : 96--98

Idiopathic Distal Renal Tubular Acidosis Presenting as Polymorphic Ventricular Tachycardia

Naveed Mohsin1, Javvid Muzamil Dandroo1, Shabir Ahmed Rather1, Firdousa Nabi2,  
1 Department of Internal Medicine, SKIMS, Soura, Srinagar, India
2 Department of Prosthodonsia, Govt. Dental College, Srinagar, Jammu and Kashmir, India

Correspondence Address:
Dr. Javvid Muzamil Dandroo
Married Hostel, Room Number F16, SKIMS, Soura, Srinagar - 190 011, Jammu and Kashmir


Renal tubular acidosis is a known clinical entity, in which type I distal renal tubular acidosis (dRTA type I), also known as classical dRTA, is second most common type after dRTA type IV. We report 21-year-old female presenting as polymorphic ventricular tachycardia (VT), which on evaluation was found to have the type I dRTA with idiopathic etiology. Polymorphic VT as presentation of type I dRTA has not been reported in the literature in human beings so far but has been reported in animal models.

How to cite this article:
Mohsin N, Dandroo JM, Rather SA, Nabi F. Idiopathic Distal Renal Tubular Acidosis Presenting as Polymorphic Ventricular Tachycardia.J Integr Nephrol Androl 2015;2:96-98

How to cite this URL:
Mohsin N, Dandroo JM, Rather SA, Nabi F. Idiopathic Distal Renal Tubular Acidosis Presenting as Polymorphic Ventricular Tachycardia. J Integr Nephrol Androl [serial online] 2015 [cited 2020 Jul 8 ];2:96-98
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Renal tubular acidosis is a known clinical entity, with type I distal renal tubular acidosis (dRTA type I) the second most common type. This entity is commonly seen in children and mostly diagnosed in early age group. dRTA type I is the classical form of RTA, was first described among all RTA. [1] dRTA type I is characterized by a failure of H + secretion into the lumen of the nephron by the alpha intercalated cells of the medullary collecting duct of the distal nephron. [2],[3],[4] This failure of acid secretion may be due to a number of causes, and it leads to an inability to acidify the urine to a pH of <5.5. [5],[6] Because renal excretion is the primary means of eliminating H + from the body, there is consequently a tendency toward acidemia. There is an inability to excrete H + while as K + cannot be reclaimed by the cell, leading to acidosis and hypokalemia.

Etiologically, many conditions can present or cause RTA. In dRTA type I, Sjogren's syndrome is most common secondary etiology. Other conditions are giving rise to dRTA type I are systemic lupus erythematosus, thyroiditis, primary biliary cirrhosis, chronic active hepatitis, analgesic use, hyperthyroidism, hyperparathyroidism, and many more.

Most common presentation among children is growth retardation. Other presentations are Confusion or decreased alertness, fatigue, increased breathing rate, kidney stones, nephrocalcinosis, osteomalacia, rickets, muscle weakness, bone pain, decreased urine output, Increased heart rate or irregular heartbeat, muscle cramps, pain in the back, flank, or abdomen, and Skeletal abnormalities.

In young adults and elderly, dRTA type I present with myalgia and arthralgia, [7] arrhythmia, mostly ventricular premature contraction (VPC's), osteomalacia, hypokalemic weakness, respiratory muscle weakness, and confusion. Our patient presented with multiple episodes of ventricular tachycardia (VT) with spontaneous terminations.

 Case Report

A 21-year-old married female with no significant past medical history, presented with multiple brief unresponsive spells with abnormal body movements (generalized tonic-clonic seizures [GTCs]) lasting between 2 and 5 min for last 2 years. Patient was evaluated twice before for possible cause of seizures and each time magnetic resonance imaging (MRI) brain and electroencephalogram (EEG) were done; both were reported as normal. Patient was diagnosed as conversion disorder and was put on psychiatric follow-up. However, patient continued to have similar spells. Presently she came to our emergency department with similar multiple brief unresponsive spells with abnormal body movements (GTCs) for few minutes with immediate regaining of consciousness. There was no other significant history. On examination, patient was conscious, pulse 100 per minute and irregular, blood pressure 110/80 mmHg, and the rest of clinical examination was unremarkable.

On evaluation her hemoglobin was 11.5 g/dL, leucocyte count 6400/mm 3 , erythrocyte sedimentation rate 21 mm for the 1 st h, random blood sugar 110 mg/dL, blood urea nitrogen 12 mg/dL, serum creatinine 0.9 mg/dL, uric acid 3.2 mg/dL, aspartate aminotransferase 44 IU/L, ALT 43 IU/L, alkaline phosphatase 130 IU/L, creatine kinase 190 IU/L, bilirubin 0.6 mg/dL, albumin 3.6 mg/dL, calcium 8.9 mg/dL, and phosphorous 1.9 mg/dL. Blood gas analysis revealed pH 7.28 (n = 7.35-7.45), serum potassium 1.6 (n = 3.5-5.0) mEq/L, serum sodium 140 (n = 135-148) mEq/L, pCO 2 30 (35-45) mmHg, pO 2 90 (80-100) mmHg, [HCO 3 ]− 10.5 mEq/L, chloride 120 mEq/L and an anion gap of 11.1 suggesting hyperchloremic normal anion gap metabolic acidosis. Electrocardiogram (ECG) [Figure 1] showed polymorphic VPC's with frequent bigeminy pattern. Patient was put on continuous ECG monitoring which showed occurrence of intermittent polymorphic VT [Figure 2], consistent with unresponsive spells.{Figure 1}{Figure 2}

Patient was given intravenous potassium, and magnesium sulfate, and her repeat serum electrolytes normalized. Further evaluation was carried out to look for the cause of VT. In the meantime, patient reverted to sinus rhythm and had no more similar spells.

Echocardiography was normal. MRI brain and awake EEG were normal.

Patient was evaluated for hyperchloremic normal anion gap metabolic acidosis and following parameters were checked. Urinary pH was 6.0, and urinary anion gap was 60 mEq/L with urinary sodium (U Na + ) of 70.0, chloride (UCl− ) of 50 and potassium (Uk + ) of 40. Urine for glucose and amino acids were negative. Bicarbonate loading test was carried out to differentiate from proximal metabolic acidosis. Patient received 7.5% soda bicarbonate intravenous at the rate of 2 mL/min for 2 h till urinary PH reached to 7.5, which was confirmed thrice. Urinary [HCO3]− was 75 mEq/L, urinary creatinine 50 mg/dL, serum creatinine 1.0 mg/dL of and serum [HCO3]− of 35, with fractional excretion of bicarbonate [FEHCO3− ] of 4.2%.

Further investigations were carried out to look for etiology of dRTA. Ultrasound abdomen showed nephrolithiasis [Figure 3]. Her 24-h urinary protein was 280 mg, serum Vitamin D level was 21.86 (normal 9-47) ng/mL, serum parathormone level 54.0 (normal 9-55) pg/mL, thyroid stimulating hormone 5.0 (normal 0.6-5.5) μU/mL, T4 10 (normal 5-12) μg/dL and antinuclear antibody was negative.{Figure 3}

Patient was put on supplemental soda bicarbonate and potassium at the time of discharge. There were no such episodes of unresponsiveness after admission and multiple ECG's were normal.

Finally, diagnosis of idiopathic type I dRTA was made presenting as polymorphic VT. The cause of VT was prolonged severe hypokalemia and acidosis.


Renal tubular acidosis was first described in 1935 by Lightwood and 1936 by Butler et al. in children. [1],[8] Baines et al. first described it in adults in 1945. [9] A normal anion gap pattern is commonly found in patients with both acute and chronic metabolic acidosis. Between 19% [10] and 41% [11] of patients in intensive care units with acute metabolic acidosis and 20-55% of individuals with chronic uremic acidosis have a nongap pattern. [12],[13] This pattern can originate from a number of pathophysiologic mechanisms; therefore, determination of its cause can be challenging.

Among all types of RTA, type IV is most common, followed by the type I dRTA. The causes of type I dRTA in adults are autoimmune disorders (Sjogren's syndrome, rheumatoid arthritis), hypercalciuria, recreational toluene sniffing and drug-induced (amphotericin B) and marked volume depletion and many more. In children, type I RTA is most often a hereditary condition. Genetic mutations in the chloride-bicarbonate co-transporter and in the apical H + -ATPase have been identified. [14],[15],[16]

Renal tubular acidosis has diverse presentations. In children, it presents mostly with growth failure and in young adults and elderly, dRTA type I present with myalgia and arthralgia, arrhythmia, osteomalacia, hypokalemic weakness, respiratory muscle weakness, confusion, etc. Our patient presented with multiple episodes of nonsustained polymorphic VT. Adults can present with arrhythmias in the form of VPC's, APC's, blocks and fibrillations. All arrhythmias are likely due to hypokalemia and acidosis. [17] In our review of the literature, till date there is no report of the type I RTA with polymorphic VT in humans but animal (horse) models do have reports. [18]

Hence, in the light of the present patient every unresponsive spell is not seizure and cardiac arrhythmia should be definitely ruled out when a patient gets multiple brief self-terminating unresponsive spells. It is also important to look for RTA as a possibility if one presents with cardiac arrhythmias, hypokalemia, and metabolic acidosis.


1Lightwood R. Communication no. 1. Arch Dis Child 1935;10:205-6.
2Stanton BA. Renal potassium transport: Morphological and functional adaptations. Am J Physiol 1989;257:R989-97.
3Young DB. Quantitative analysis of aldosterone's role in potassium regulation. Am J Physiol 1988;255:F811-22.
4Wingo CS, Smolka AJ. Function and structure of H-K-ATPase in the kidney. Am J Physiol 1995;269:F1.
5Wrong O, Davies HE. The excretion of acid in renal disease. Q J Med 1959;28:259-313.
6DuBose TD, McDonald GA. Renal tubular acidosis. A Companion to Brenner and Rector's the Kidney. Philadelphia: WB Saunders; 2002. p. 189-206.
7Harrington TM, Bunch TW, Van den Berg CJ. Renal tubular acidosis. A new look at treatment of musculoskeletal and renal disease. Mayo Clin Proc 1983;58:354-60.
8Butler AM, Wilson JL, Farber S. Dehydration and acidosis with calcification at renal tubules. J Pediatr 1936;8:489-99.
9Baines AM, Barelay JA, Cooke WT. Nephrocalcinosis associated with hyperchloremia and low plasma-bicarbonate. Q J Med 1945;14:113-23.
10Gunnerson KJ, Saul M, He S, Kellum JA. Lactate versus non-lactate metabolic acidosis: A retrospective outcome evaluation of critically ill patients. Crit Care 2006;10:R22.
11Brill SA, Stewart TR, Brundage SI, Schreiber MA. Base deficit does not predict mortality when secondary to hyperchloremic acidosis. Shock 2002;17:459-62.
12Lee HY, Joo HY, Han DS. Serum electrolyte and acid base composition in patients with graded degrees of chronic renal failure. Yonsei Med J 1985;26:39-43.
13Wallia R, Greenberg A, Piraino B, Mitro R, Puschett JB. Serum electrolyte patterns in end-stage renal disease. Am J Kidney Dis 1986;8:98-104.
14Wrong O, Bruce LJ, Unwin RJ, Toye AM, Tanner MJ. Band 3 mutations, distal renal tubular acidosis, and Southeast Asian ovalocytosis. Kidney Int 2002;62:10-9.
15Karet FE. Inherited distal renal tubular acidosis. J Am Soc Nephrol 2002;13:2178-84.
16Shayakul C, Alper SL. Defects in processing and trafficking of the AE1 Cl-/HCO3-exchanger associated with inherited distal renal tubular acidosis. Clin Exp Nephrol 2004;8:1-11.
17Helfant RH. Hypokalemia and arrhythmias. Am J Med 1986;80:13-22.
18MacLeay JM, Wilson JH. Type-II renal tubular acidosis and ventricular tachycardia in a horse. J Am Vet Med Assoc 1998;212:1597-9.