Indian J Pediatr (July 2013) 80(7):598–600 DOI 10.1007/s12098-012-0858-x
CLINICAL BRIEF
Pericardial Tamponade in Nephrotic Syndrome: An Uncommon Complication Sunita Namdev & Sriram Krishnamurthy & Niranjan Biswal & Barath Jagadisan
Received: 22 March 2012 / Accepted: 3 July 2012 / Published online: 20 July 2012 # Dr. K C Chaudhuri Foundation 2012
Abstract An 11 y –old boy with steroid resistant nephrotic syndrome (SRNS) secondary to focal segmental glomerulosclerosis (FSGS) developed pericardial tamponade during the course of illness. He was promptly managed with pericardiocentesis. Pericardial tamponade is an extremely rare complication in childhood nephrotic syndrome and very few cases have been reported in literature. Recognition of this rare, yet serious and life threatening complication in childhood nephrotic syndrome (NS) is emphasized to prevent fatalities in children with nephrotic syndrome. Keywords Pericardial tamponade . Nephrotic syndrome . Steroid resistance . Focal segmental glomerulosclerosis
Introduction Fluid retention in nephrotic syndrome (NS) often leads to peripheral edema, ascites, and pleural effusions. Intuitively, pericardial effusions could be anticipated as well, but literature is scarce regarding this complication in NS [1, 2]. The authors herein describe an 11 y-old boy with NS who developed pericardial tamponade.
Case Report An 11 y-old boy presented to the authors’ hospital in August 2011 with anasarca and oliguria. There was no hematuria,
S. Namdev : S. Krishnamurthy : N. Biswal (*) : B. Jagadisan Department of Pediatrics, Jawaharlal Institute of Postgraduate Medical Education and Research (JIPMER), Pondicherry 605006, India e-mail:
[email protected]
rash, joint swelling, fever or jaundice. His blood pressure was 130/90 mm Hg (>95th centile). Cardiovascular and chest examination were normal. Investigations showed massive proteinuria (urine protein 3+, urinary protein/creatinine ratio03.1), hypoalbuminemia (1.8 g/dl) and hypercholesterolemia (780 mg/dl). Blood urea, serum creatinine and T3, T4, TSH levels were normal. He was treated with prednisolone (2 mg/kg) and amlodipine. However, proteinuria (4+) persisted even after 4 wk. In view of steroid resistance, he underwent a renal biopsy, which revealed focal segmental glomerulosclerosis (FSGS), with immunofluorescence showing IgM and C3 deposits. He was then treated with cyclosporine, prednisolone and enalapril. Due to uncontrolled hypertension, he required additional treatment with prazosin and atenolol. After completion of 3 mo of cyclosporine therapy, he continued to have hypoalbuminemia (1.9 g/dl) and massive proteinuria (4+). He was admitted for edema control with diuretics (frusemide and spironolactone), without much success. Albumin infusion could not be given due to persistent hypertension. On the seventh day of admission, he developed tachypnea (respiratory rate of 40/min), pulse rate 100/min with pulsus paradoxus. Heart sounds were muffled. Chest radiograph showed massive cardiac enlargement (Fig. 1); electrocardiogram showed low QRS voltages. Echocardiogram confirmed pericardial tamponade with right atrium and right ventricular collapse (Fig. 2). Blood urea and serum creatinine were normal. Pericardiocentesis was done and 400 ml of fluid drained. Pericardial fluid was clear with no cells; protein was 150 mg/dl, glucose was 90 mg/dl; Gram stain and polymerase chain reaction (PCR) for tuberculosis were negative. The pericardial fluid protein: serum protein was 0.3, while pericardial fluid LDH: serum LDH was 0.4, suggestive of a transudate. Pericardial fluid culture was sterile. He had significant clinical improvement and the
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Proteinuria (4+) continued to persist despite cyclosporine therapy. Thereafter he demonstrated worsening urea and creatinine over a period of 2 wk (blood urea 108 mg/dl, creatinine 3.6 mg/dl) with oliguria. In view of nephrotoxicity, cyclosporine and enalapril were discontinued. Treatment with mycophenolate mofetil and plasmapheresis were initiated which led to improvement in edema and partial remission of proteinuria (2+) on follow up with a weight loss of 3 kg and serum albumin 2.6 g/dL.
Discussion
Fig. 1 Chest x-ray of the patient showing massive cardiac enlargement a before and b after pericardiocentesis
pigtail catheter was removed. Repeat echocardiogram 1 wk later showed no effusion. Anti-nuclear antibody (ANA), dsDNA and anti-neutrophil cytoplasmic antibody (ANCA), C3 levels, HBsAg and HIV serology were negative. Ultrasonogram showed normal size, shape and echotexture of the kidneys and ureters.
Fig. 2 Echocardiogram showing pericardial tamponade with right atrium and ventricular collapse. PE Pericardial tamponade; RA Right atrium; RV Right ventricle; LA Left atrium; LV Left ventricle
Edema, one of the major clinical manifestations of the NS, results from hypoalbuminemia which diminishes the colloid osmotic pressure of plasma leading to seepage of fluid from intravascular into interstitial compartment, with contraction of plasma volume causing salt and water retention [3, 4]. Large pericardial effusions are very rare in childhood idiopathic nephrotic syndrome. Pericardial effusion has been also described to occur in idiopathic NS as well as systemic lupus erythematosus (SLE)- associated NS [2, 5–7]. The index patient did not satisfy biochemical, histopathological or clinical criteria for the diagnosis of SLE. Investigations for secondary etiology (ANA, dsDNA, ANCA, C3 levels, HBsAg and HIV serology) were also negative. Pericardial tamponade is an even rarer complication in NS. On MEDLINE search, the authors could find only two reports of pericardial tamponade in association with childhood NS. The first was an 8 y- old Turkish girl with idiopathic steroid resistant NS (due to FSGS) on cyclosporine therapy, similar to the present case, who developed life threatening pericardial tamponade, attributed to fluid overload, diminished glomerular filtration rate and hypoalbuminemia; managed with pericardiocentesis [1]. The other report describes a 4 y-old Indian girl with steroid-resistant NS (Minimal change disease) who developed chylopericardial tamponade secondary to superior vena cava thrombosis [8]. In the index case , pericardial tamponade was transudative, non-chylous, and occurred possibly due to hypoalbuminemia and fluid overload [7]. There are anecdotal reports of cyclosporine in association with pericardial effusion in patients undergoing cardiac transplantation; this could also have been contributory in the index patient [9]. Other causes such as hypothyroidism, tuberculosis and SLE were ruled out by appropriate investigations. Since the renal function tests at the time of detection of pericardial effusion were normal, it is unlikely that pericardial effusion was a manifestation of uremia. Similarly, pericardial effusions due to viral infections e.g., echovirus or coxsackievirus were unlikely since the nature of the pericardial effusion was transudative.
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Indian J Pediatr (July 2013) 80(7):598–600
In conclusion, the authors emphasize that although pericardial tamponade is exceedingly rare in childhood NS, recognition of this serious complication in children with NS complicated by respiratory distress, is essential to initiate a prompt and life saving pericardiocentesis.
Conflict of Interest None.
Role of Funding Source
None.
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