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Saudi Journal of Kidney Diseases and Transplantation
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LETTER TO THE EDITOR  
Year : 2011  |  Volume : 22  |  Issue : 5  |  Page : 1028-1029
An unusual association of primary focal and segmental glomerulosclerosis, distal renal tubular acidosis and secondary erythrocytosis


Department of Nephrology, Sri Venkateswara Institute of Medical Sciences, Tirupati, Andhra Pradesh, India

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Date of Web Publication6-Sep-2011
 

How to cite this article:
Yadla M, Reddy S, SriramNaveen P, Krishnakishore C, Sainaresh V V, Sivakumar V. An unusual association of primary focal and segmental glomerulosclerosis, distal renal tubular acidosis and secondary erythrocytosis. Saudi J Kidney Dis Transpl 2011;22:1028-9

How to cite this URL:
Yadla M, Reddy S, SriramNaveen P, Krishnakishore C, Sainaresh V V, Sivakumar V. An unusual association of primary focal and segmental glomerulosclerosis, distal renal tubular acidosis and secondary erythrocytosis. Saudi J Kidney Dis Transpl [serial online] 2011 [cited 2022 May 25];22:1028-9. Available from: https://www.sjkdt.org/text.asp?2011/22/5/1028/84559
To the Editor ,

The association of primary focal and segmental glomerulosclerosis (FSGS) with distal renal tubular acidosis (RTA) and secondary erythrocytosis is unusual and, hence, is the interest in reporting this entity.

A 25-year-old male patient who was diagnosed as having FSGS and treated with steroids and cytotoxic therapy elsewhere presented to us for the management of hypokalemic quadriparesis. Evaluation at that time revealed hemoglobin of 18 gm/dL, with an increase in red blood cell (RBC) count. The peripheral smear was within normal limits. There was no evidence of leukocytosis or thrombocytosis. Urine analysis revealed 1+ proteinuria, and there was no active sediment. Blood urea and serum creatinine were within normal limits. Serum electrolytes revealed normal sodium and potassium of 1.8 meq/L. Blood gas analysis was suggestive of metabolic acidosis. Ultrasound abdomen showed normal sized kidneys and computerized tomography (CT) scan of the abdomen revealed bilateral medullary nephrocalcinosis. Serum anion gap and urinary anion gap were within normal limits. A 24-hour urinary bicarbonate was within normal limits. Bone marrow showed normal cellular lineages. Serum erythropoietin level was within normal limits.

The above features suggested the presence of distal RTA and secondary erythrocytosis. The patient needed phlebotomy and was started on angiotensin-converting enzyme inhibitors (ACEI). He continues to be on regular follow-up. His proteinuria at last follow-up was 185 mg/day and hemoglobin remained at 18 gm%. Literature review revealed three such situations of co-existence of FSGS with distal RTA:

  1. A patient with hereditary distal RTA with nephrocalcinosis in whom secondary FSGS was found as causing proteinuria. [1]
  2. A 3-year-old child with Alagille syndrome in whom both FSGS and distal RTA were present. [2]
  3. Sjogrens syndrome manifesting FSGS and distal RTA. [3]
Secondary erythrocytosis was reported earlier in association with membranous nephropathy and minimal change disease. [4],[5],[6],[7]

The mechanisms cited were:

  1. Intrarenal hypoxia activating the reninangiotensin system, causing increased sodium reabsorption from proximal tubules resulting in excess oxygen consumption, which would in turn stimulate oxygen-sensitive receptors and thus increase the secretion of erythropoietin, leading to secondary erythrocytosis.
  2. Increased levels of IL-8 in minimal change disease may cause erythrocytosis by stimulating the bone marrow.
The association of distal RTA with nephrocalcinosis and secondary erythrocytosis [8] can be explained as follows: nephrocalcinosis provokes hypoxia in the renal tissue. In addition, nephrocalcinosis sometimes stimulates prostaglandin production, which, in turn, stimulates erythropoietin production, contributing to erythrocytosis.

In this presentation, we found three unusual associations: FSGS, distal RTA and secondary erythrocytosis. The literature review did not reveal any similar association reported in the past. The plausible mechanisms to explain the association of FSGS with distal RTA and secondary erythrocytosis in this patient were discussed.

 
   References Top

1.Balogun RA, Adams ND, Palmisano J, Yamase H, Chughtai I, Kaplan AA. Focal segmental glomerulosclerosis, proteinuria and nephrocalcinosis associated with renal tubular acidosis. Nephrol Dial Transplant 2002;17(2):308-10.  Back to cited text no. 1
    
2.Davis J, Griffiths R, Larkin K, Rozansky D, Troxell M. Glomerular basement membrane lipidosis in Alagille syndrome. Pediatr Nephrol 2010;25(6):1181-4.  Back to cited text no. 2
    
3.Yang J, Li X, Huang Q. Renal involvement in primary Sjögren's syndrome: a clinicopathological study of 26 cases. Zhonghua Nei Ke Za Zhi 1997;36(1):28-31 Chinese.  Back to cited text no. 3
    
4.Lim CS, Jun KH, Kim YS, et al. Secondary polycythemia associated with idiopathic membranous nephropathy. Am J Nephrol 2000;20: 344-6.  Back to cited text no. 4
    
5.Ardites L, Mezzano S. Erythrocytosis associated to idiopathic membranous nephropathy: Case report. Rev Med Chil 1992;120:430-2.  Back to cited text no. 5
    
6.Stack JI, Zabetaksis PM. Erythrocytosis associated with idiopathic membranous glomerulopathy. Clin Nephrol 1979;12:87-9.  Back to cited text no. 6
    
7.Balal M, Seyrek N, Karayaylali I, Paydas S.. A unique form of polycythemia associated with minimal change disease. Med Princ Pract 2004; 13:366-8.  Back to cited text no. 7
    
8.Agroyannis B, Koutsikos D, Tzanatos-Exarchou H, Yatzidis H. Erythrocytosis in type I renal tubular acidosis with nephrocalcinosis. Nephrol Dial Transplant 1992;7(4):365-9.  Back to cited text no. 8
    

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Correspondence Address:
Manjusha Yadla
Department of Nephrology, Sri Venkateswara Institute of Medical Sciences, Tirupati, Andhra Pradesh
India
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Source of Support: None, Conflict of Interest: None


PMID: 21912041

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This article has been cited by
1 Medullary nephrocalcinosis, distal renal tubular acidosis and polycythaemia in a patient with nephrotic syndrome
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[Pubmed]



 

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