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| Year : 2011 | Volume
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| Issue : 5 | Page : 1028-1029 |
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| An unusual association of primary focal and segmental glomerulosclerosis, distal renal tubular acidosis and secondary erythrocytosis |
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Manjusha Yadla, Sandeep Reddy, P SriramNaveen, C Krishnakishore, VV Sainaresh, V Sivakumar
Department of Nephrology, Sri Venkateswara Institute of Medical Sciences, Tirupati, Andhra Pradesh, India
Click here for correspondence address and email
| Date of Web Publication | 6-Sep-2011 |
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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:
- A patient with hereditary distal RTA with nephrocalcinosis in whom secondary FSGS was found as causing proteinuria. [1]
- A 3-year-old child with Alagille syndrome in whom both FSGS and distal RTA were present. [2]
- 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:
- 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.
- 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 | |  |
| 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. 
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| 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.
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| 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.
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| 4. | Lim CS, Jun KH, Kim YS, et al. Secondary polycythemia associated with idiopathic membranous nephropathy. Am J Nephrol 2000;20: 344-6.
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| 5. | Ardites L, Mezzano S. Erythrocytosis associated to idiopathic membranous nephropathy: Case report. Rev Med Chil 1992;120:430-2.
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| 6. | Stack JI, Zabetaksis PM. Erythrocytosis associated with idiopathic membranous glomerulopathy. Clin Nephrol 1979;12:87-9.
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| 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.
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| 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.
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Correspondence Address:
Manjusha Yadla
Department of Nephrology, Sri Venkateswara Institute of Medical Sciences, Tirupati, Andhra Pradesh
India
 Source of Support: None, Conflict of Interest: None  | Check |
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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| Karunarathne, S. and Udayakumara, Y. and Govindapala, D. and Fernando, H. | | BMC Nephrology. 2012; 13(1) | | [Pubmed] | |
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