| Abstract|| |
We report on a 16-year old female patient with biopsy proved minimal change disease and steroid state. She was subsequentlygiven cyclosporin for varying periods of time with inadequate response. She developed four episodes of acute renal failure in the background of severe nephrotic state, 10 years after the onset of her illness. Three of these episodes could be reversed with albumin infusion and judicious use of diuretics while the fourth necessitated six sessions of ultrafiltration. In none of the episodes of ARF could we find any cause other than the nephrotic state itself.
Keywords: Acute renal failure, Minimal change disease, Nephrotic syndrome, Cyclosporine.
|How to cite this article:|
Abdelrahman M, Rafi A, Ghacha R, Kumar SA, Karkar A. Recurrent Acute Renal Failure in a Patient with Minimal Change Disease. Saudi J Kidney Dis Transpl 2001;12:530-3
|How to cite this URL:|
Abdelrahman M, Rafi A, Ghacha R, Kumar SA, Karkar A. Recurrent Acute Renal Failure in a Patient with Minimal Change Disease. Saudi J Kidney Dis Transpl [serial online] 2001 [cited 2019 Jul 23];12:530-3. Available from: http://www.sjkdt.org/text.asp?2001/12/4/530/33795
| Introduction|| |
Acute renal failure (ARF) is an unusual but, serious complication of the nephrotic syndrome (NS) caused by glomerular disease of any type. It may occur during the first presentation of the NS or later on at any stage of the disease. ARF in NS may have many precipitating causes.  These include glomerular diseases such as membranoproliferative glomerulonephritis and focal and segmental glomerulosclerosis, or secondary to renal vein thrombosis.  The other precipitating causes include sepsis, drugs that cause interstitial nephritis  and radiocontrast media. However, in most cases there appears to be no obvious precipitating factor, and ARF may be related to the nephrotic state itself with severe hypoproteinemia which may lead to a hypovolemic state and ARF. The prognosis of this form of ARF varies, with most patients improving with the correction of hypovolemia. A small proportion of patients may need hemodialysis and ultrafiltration, while some others may not recover and develop chronic renal failure. In most cases, control of NS with steroids enhances recovery from ARF. In this study, we report a case of minimal change disease (MCD) that was diagnosed at the age of three years, who relapsed at the age of 14 years and developed four episodes of ARF.
| Case Report|| |
A 14-year-old girl was diagnosed as having MCD (biopsy proved) at the age of three years. This was confirmed by a second renal biopsy at the age of 13 years. She initially responded to steroids, but subsequently had frequent relapses and became steroid resistant at the age of 13. She was treated with cyclosporin for a period of one year and had remission for a total of two years. She subsequently presented with a severe relapse manifested by proteinuria of 12 gm/day and was restarted on cyclosporin following which the proteinuria diminished to 5 gm/day over a period of one month. During this stage, she presented with oliguria and weight gain of 10 kg over a period of two weeks. There was no history of nausea or vomiting, fever, flank pain, hematuria or dysuria. She was not taking any other medication at that time, in particular non-steroidal anti-inflammatory drugs. On physical examination, she had puffiness of face and lower limb edema. She was afebrile, blood pressure was 120/80 mm Hg, pulse rate was 96/minute and the Jugular venous pressure was not raised. There was no other abnormality detected on physical examination.
The laboratory findings showed a hemoglobin of 120 gm/L, hematocrit 35%, white blood cell count 8.33 x 10 9 /L and platelets of 200 x 10 9 /L. Urine analysis showed albumin +++, Pus cells 5-12, red blood cells 3-5 and no casts. Urine sodium was 15 mmol/l, urine osmolality 340 mosmol/kg; 24-hour urine collection showed a total protein of 5.5 gm and creatinine clearance of 15 ml/minute. Blood urea was 95 mg/dl, serum creatinine 2.5 mg/dl, serum albumin 21 gm/L, cholesterol 550 mg/dl, triglycerides 235 mg/dl, serum potassium 5 mmol/L, sodium 137 mmol/L, chloride 100 mmol/L, serum calcium 8.3 mg/dl, phosphate 4.6 mg/dl and uric acid 6 mg/dl. The blood cyclosporin level, which includes its metabolites, was 316 ng/ml (normal 400-800).
The septic work-up including urine culture was negative. Chest X-ray and electrocardiogram were within normal limits. Ultrasound examination showed the right kidney measuring 9.5 x 4 cm and the left kidney measuring 9.9 x 4.5 cm, with no morphological abnormalities.
The patient was treated with albumin infusion (20% human albumin 100 ml intravenous (i.v.) daily for five days) and diuretics (frusemide i.v. 80 mg twice a day). She slowly improved with normalization of her renal functions. She was instructed to continue treatment with cyclosporin, but she discontinued the drug on her own.
One month later, she developed a second episode of ARF, necessitating hospitalization. She had a similar history of oliguria and weight gain of 10 kg. Her blood pressure was 130/70 mm Hg. Her renal functions were impaired with serum creatinine 2.4 mg/dl and blood urea 99 mg/dl. The serum albumin was 24 gm/L, serum protein 44 gm/L, urine sodium 24 mmol/L and urine osmolality was 300 mosmol/kg. She was treated with albumin infusion and diuretics with which she recovered over a 10 days period. Her serum creatinine on recovery returned down to 0.8 mg/dl. However, she refused any further investigations including the assessment of her proteinuria.
About one month later, she presented again with a similar history of oliguria and weight gain. Her blood pressure was 120/70 mm Hg. She had impaired renal functions with serum creatinine 2.5 mg/dl, blood urea 76 mg/dl. Her serum albumin was 19 gm/L and serum protein 42 gm/L. Her urine sodium was 30 mmol/l and urine osmolality was 290 mosmol/kg. Her clinical condition improved again with albumin infusions and diuretics. Her serum creatinine came down to 0.9 mg/day. She again refused any further investigations.
A month later she developed her fourth episode of ARF with almost similar history and physical and laboratory findings. Her blood pressure was 120/80 mm Hg. The serum creatinine was 2.6 mg/dl and serum albumin was 18 gm/L. Her urine sodium was 25 mmol/l, urine osmolality 350 mosmol/kg. At this presentation, she did not respond well to albumin infusions and diuretics. However, her clinical situation improved and her renal functions returned to normal following six sessions of ultrafiltration. Subsequently, she was restarted on 4 mg/kg cyclosporin and showed a good response with remission of proteinuria over three-months period. Two years later, the patient is still in remission with normal renal functions; serum creatinine 0.6 mg/dl, serum albumin 44 gm/L and ever since had no more episodes of ARF. Currently, she is continuing her treatment with 4 mg/kg cyclosporin.
| Discussion|| |
Acute renal failure is well known to occur in the NS, particularly in the early phase of the disease. However, in our patient the four episodes of ARF occurred at a later stage of the disease, and when the patient reached the age of 14 years. Most reported cases have shown that ARF is more common in adults with an estimated frequency of 3%,  whereas, only few cases have been reported in children. , Functional and pathological changes that have been described in these cases include reduced glomerular filtration rate, increased tubular osmotic pressure, tubular obstruction by albumin, tubular necrosis with luminal debris, renal edema and interstitial nephritis. Our patient developed four episodes of ARF over four consecutive months. There were no precipitating factors for ARF during these episodes except for the NS itself.
The variability of clinical presentation of ARF in the NS is well reported. ,,,,,,,,,, Clinical features from these studies and case reports show that most patients were adults (39 out of 42), and that MCD was the most common lesion (28 out of 42). Total serum protein and serum albumin levels were very low, and 24-hour proteinuria was massive with an average of 11.4 gm. Blood pressure was normal or high in most cases. ARF occurred in 28 out of 45 patients at the onset of the disease. There was no precipitating factor for ARF in 28 out of 45 patients. Clinical course showed complete recovery in 28 patients (66%). In most of cases it was accompanied by resolution of proteinuria induced by steroids. Ten patients died (24%) and four were left with chronic renal impairment. Hemodialysis was needed in eight patients for management of ARF. The clinical profile of our patient showed many similarities with the above picture although there were few differences in the clinical presentation. These include her maintenance of normal blood pressure, the occurrence of ARF late in the course of her disease (10 years from the onset) and its recurrence on four occasions within a short period of time. The cause of ARF in our patient was most likely severe NS (MCD) with hypoproteinemia. She responded well to albumin infusion and diuretics, though she required ultrafiltration in the last episode.
Cyclosporin therapy in steroid resistant NS has had disappointing results. In a summary of nine studies, only 20% of patients had complete remission, while many had a relapse with cessation of therapy.  Cyclosporin in association with prednisolone has been found to be more effective in inducing remission than cyclosporin alone.  Our patient responded well to cyclosporin on two occasions but relapsed on discontinuation. However, long-term use of cyclopsorin in such patient raises the concern of nephrotoxicity.
In conclusion, our case illustrates many features of ARF in the NS. The ARF may have many precipitating factors but most of the time it is related to the nephrotic state itself. It may be recurrent especially if NS is not well managed. The most important therapeutic measure in this form of ARF is correction of hypovolemia. Diuretics, ultrafiltration and/or hemodialysis may be needed. Control of nephrotic state with steroids and other drugs is needed to hasten recovery from ARF and to prevent its recurrence.
| References|| |
|1.||Cameron S, Davison AM, Grunfeld JP, et al. Oxford Textbook of Clinical Nephrology Oxford University Press, Oxford 1992;1034-40. |
|2.||Cameron JS, Ogg CS, Wass VJ. Complications of nephrotic syndrome in kidney disease, ed. SJ Cameron and Glassock RJ, Mareel Dekker New York 1988;849-92. |
|3.||Clive DM, Stoff JS. Renal syndromes associated with non-steroidal anti-inflammatory drugs. N Engl J Med 1984;310: 563-72. |
|4.||Cases records of the Massachusetts General Hospital. Weekly clinicopathological exercises. Case 28-1978. N Engl J Med 1978;299:136-45. |
|5.||Cases records of the Massachusetts General Hospital. Weekly clinicopathological exercises. Case 4-1982. Sudden onset of renal failure and the nephrotic syndrome in a middle-aged woman. N Engl J Med 1982;306:221-31. |
|6.||Springate JE, Coyre JF, Karp MP, Feld LG. Acute renal failure in minimal change nephrotic syndrome. Pediatrics 1987;80:946-8. |
|7.||Steele BT, Bacheyie GS, Baumal R, Rance CP. Acute renal failure of short duration in minimal lesion nephrotic syndrome of child. Int J Pediatr Nephrol 1982;3:59-62. |
|8.||Yamauchi H, Hopper J. Hypovolumic shock and hypopotension as a complication in nephrotic syndrome. Ann Int Med 1984;60: 242-54. |
|9.||Chamberlain MJ, Prungle A, Wrong DM. Oliguric renal failure in nephrotic syndrome. Q J Med 1996;35:215-35. |
|10.||Curt GA, Kaldany A, Whitley LG, et al. Reversible rapidly progressive renal failure with nephrotic syndrome due to fenoprofen calcium. Ann Intern Med 1980;92:72-3. |
|11.||Cameron JS, Turner DR, Ogg-CS, Sharpstone P, Brown CB. The nephrotic syndrome in adults with minimal change glomerular lesions. Q J Med 1974;43:461-88. |
|12.||Raij L, Keane WF, Leonard A, Shapiro FL. Irreversible acute renal failure in idiopathic nephrotic syndrome. Am J Med 1976;61:207-14. |
|13.||Esparza AR. Kahn SI, Garella S, Abuelo JG. Spectrum of acute renal failure in nephrotic syndrome with minimal (or minor) glomerular lesions. Role of hemodynamic factors. Lab lnvest 1981;45:510-21. |
|14.||Imbasciati E, Ponticelli C, Case N, et al. Acute renal failure in idiopathic nephrotic syndrome. Nephron 1981;28:186-91. |
|15.||Holdsworth DR, Stephenson P, Dowling JP, Atkins RC. Reversible acute renal failure in the nephrotic syndrome with minimal glomerular pathology. Med J Aust 1977;2:532-3. |
|16.||Hulter HN, Bonner EL Jr. Lipoid Nephrosis appearing as acute oliguric renal failure. Arch Intern Med 1980;140:403-5. |
|17.||Niaudet P, Habib R. Cyclosporin in the treatment of idiopathic nephrosis. J Am Soc Nephrol 1994;5:1049-56. |
Department of Nephrology, Dammam Central Hospital, Dammam