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Saudi Journal of Kidney Diseases and Transplantation
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CASE REPORT Table of Contents   
Year : 2007  |  Volume : 18  |  Issue : 4  |  Page : 585-589
A Neglected Case of Renal Tubular Acidosis


Shiraz University of Medical Sciences, Shiraz Nephro-Urology Research Center, Shiraz, Iran

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   Abstract 

In this report, we present a case of a child with distal renal tubular acidosis, severe failure to thrive and profound rickets, who was only 7.8 Kg when presented at 6 years of age. His response to treatment and his follow-up for four years is discussed. Although failure to thrive is a common finding in renal tubular acidosis but the physical and x-ray findings in our case were unique.

How to cite this article:
Derakhshan A, Basiratnia M, Fallahzadeh MH, Hosseini-Al-Hashemi G. A Neglected Case of Renal Tubular Acidosis. Saudi J Kidney Dis Transpl 2007;18:585-9

How to cite this URL:
Derakhshan A, Basiratnia M, Fallahzadeh MH, Hosseini-Al-Hashemi G. A Neglected Case of Renal Tubular Acidosis. Saudi J Kidney Dis Transpl [serial online] 2007 [cited 2020 Jun 2];18:585-9. Available from: http://www.sjkdt.org/text.asp?2007/18/4/585/36516

   Introduction Top


Renal tubular acidosis (RTA) was first described by Lightwood et al, in 1935 [1] and Butler et al, in 1936 [2] in children, and by Baines in 1945 in adults. [3] There are three main types of RTA, each type is either primary or secondary, besides several subdivision for each type.[4],[6] Recent advances of molecular basis of RTA have resulted in a better understanding of the disease and more therapeutic options than the mere oral alkali therapy.[7],[8]

Failure to thrive is a common presentation of distal RTA but rickets is not as common. [9],[10] We report here an interesting case of distal RTA with severe failure to thrive and profound rickets.


   Case report Top


A six-year old boy referred with severe growth retardation and respiratory distress in June 2002. He was apparently well till five months of age, but he failed to thrive after­wards. There was a history of recurrent vomiting. Four siblings died at early life (no data available).

On physical examination, he was tachypnic, irritable, and cyanotic with severe deformity in chest cage and deformity of extremities [Figure - 1],[Figure - 2]. His parameters included weight 7.800 Kg, length 81 cm, head circum­ference 51 cm, blood pressure 80/50 mmHg, respiratory rate 35/min, pulse rate 90/min, and temperature 36.7 o C. Widening of wrist and deformity of knees and ankles were observed. The cardiac and abdomen exam were unremarkable.

The laboratory evaluations are summarized in [Table - 1], and revealed normal liver function test, urinalysis: RBC 4-5, WBC 8-10, Specific Gravity 1.002, Blood +1, urine pH 6.8, 24hr urine calcium 133 mg, serum creatinine 72 mg, and urine Na 26 mmol/L, K 20 mmol/L, Cl 33 mmol/L. Urine culture was negative. Renal ultrasound revealed normal size kidneys with severe increase in medullary echogenicity compatible with nephrocalcinosis, [Figure - 3]. Wrist x-ray revealed profound rickets [Figure - 2] and abdominal plain X-ray revealed nephro­calcinosis [Figure - 3] and severe osteopenia in all visualized bones. According to this presen­tation and investigations we diagnosed renal tubular acidosis.

Since the patient had a very low serum pota­ssium level, potassium infusion and then sodium bicarbonate infusion were initiated as treatment of metabolic acidosis. After three days, his treatment was changed to polycitra (citric acid, sodium citrate and potassium citrate). Vitamin D was started from the beginning of treatment and was continued after discharge.

The course of the patient and follow up are summarized in [Table - 2], and his last follow up photograph was shown in [Figure - 4].


   Discussion Top


Diagnosis of distal RTA is obvious with normal anion gap hyperchloremic metabolic acidosis, alkaline urine pH, positive urine anion gap, hypercalciuria and dramatic response to alkaline therapy in our case. [4],[5],[6],[11] Rickets in distal RTA could be treated with alkali therapy, without vitamin D, but the rickets in our case was so severe that we preferred to treat him with active vitamin D.

In a recent review, 5.6% of hospital admissions and/ or pediatric consultation were due to renal tubular acidosis. [12] Cases of distal RTA with hypokalemic paralysis and bony deformities and multiple bone fractures has been reported in the literature. [12],[13],[14] Although RTA is not a common renal cause of failure to thrive, it should be considered when we are confronted with children who have developed significant growth retardation, tubular dysfunction, polyuria, refractory rickets and hypokalemic metabolic acidosis. [15]

Our case is unique in that at his six years of age, he was just 7.8 Kg in weight, 81 cm in length and did not seek medical advice so far.


   Acknowledgement Top


The authors would like to thank Dr. Davood Mehrabani, Miss Gholami and Mrs. Ghorbani at Center for Development of Clinical Research of Nemazee Hospital for editorial and typing assistance.

 
   References Top

1.Lightwood R. Calcium infarction of kidney in infants. Arch Dis Child 1935;10:205-10.  Back to cited text no. 1    
2.Butler AM, Wilson JL, Farber S. Dehydration and acidosis with calcification of renal tubules. J Pediatr 1936;8:489-99.  Back to cited text no. 2    
3.Baines GH. Barclay JA, Cooke WT. Nephro­calcinosis associated with hyperchloremia and low plasma bicarbonate. Q J Med 1945;14: 113-22  Back to cited text no. 3    
4.Herrin TJ. Renal tubular acidosis. In: Avner ED, Harmon WE, Niaudet P, eds. Pediatric Nephrology. 5 th ed. Baltimore; Lippincot Williams and Wilkins, 2004:757-76.  Back to cited text no. 4    
5.Rodriguez-Soriano J, Vallo A. Renal tubular acidosis. Pediatr Nephrol 1990;4:268-75.  Back to cited text no. 5    
6.Laing CM, Unwin RJ. Renal tubular acidosis. J Nephrol 2006;19Suppl9:S46-52.  Back to cited text no. 6    
7.Laing CM, Toye AM, Capasso G, Unwin RJ. Renal tubular acidosis: developments in our understanding of the molecular basis. Int J Biochem Cell Biol 2005;37(6):1151-61.  Back to cited text no. 7    
8.Cheidde L, Vieira TC, Lima PR, Saad ST, Heilberg IP. A novel mutation in the anion exchanger 1 gene is associated with familial distal renal tubular acidosis and nephro­calcinosis. Pediatrics 2003;112:1361-7.  Back to cited text no. 8    
9.Chang CY, Lin CY. Failure to thrive in children with primary distal type renal tubular acidosis. Acta Paediatr Taiwan 2002;43(6):334-9.  Back to cited text no. 9    
10.Russell WC. Rickets associated with renal tubular acidosis. In: Behrman RE, Kliegman RM, Jenson HB, eds. Nelson Textbook of Pediatrics. 17 th ed. Saunders. 2004:1762.  Back to cited text no. 10    
11.Bajpai A, Bagga A, Hari P, Bardia A, Mantan M. Long-term outcome in children with primary distal renal tubular acidosis. Indian Pediatr 2005;42:321-8.  Back to cited text no. 11    
12.Orta-Sibu N, Lopez M, Moriyon JC, Chavez JB. Renal diseases in children in Venezuela, South America. Pediatr Nephrol 2002;17:566-­9.  Back to cited text no. 12    
13.Bresolin NL, Grillo E, Fernandes VR, Carvalho FL, Goes JE, da Silva RJ. A case report and review of hypokalemic paralysis secondary to renal tubular acidosis. Pediatr Nephrol 2005;20:818-20.  Back to cited text no. 13    
14.Bagga A, Bajpai A, Gulati S, Singh A. Distal renal tubular acidosis with severe bony defor­mities and multiple fractures. Indian Pediatr 2001;38:1301-5.  Back to cited text no. 14    
15.Bagga A, Bajpai A, Menon S. Approach to renal tubular disorders. Indian J Pediatr 2005; 72:771-6.  Back to cited text no. 15    

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Correspondence Address:
Ali Derakhshan
Pediatric Office, Nemazee Hospital, Shiraz University of Medical Sciences, Post -Code: 71937-11351, Shiraz
Iran
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PMID: 17951947

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    Figures

  [Figure - 1], [Figure - 2], [Figure - 3], [Figure - 4]
 
 
    Tables

  [Table - 1], [Table - 2]



 

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    Abstract
    Introduction
    Case report
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    Acknowledgement
    References
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