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Saudi Journal of Kidney Diseases and Transplantation
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Table of Contents   
CASE REPORT  
Year : 2012  |  Volume : 23  |  Issue : 3  |  Page : 556-558
C1q nephropathy with acute hemolytic uremic syndrome


1 Department of Pathology, Laboratory Medicine, Transfusion Services and Immunohematology, Dr. H. L. Trivedi Institute of Transplantation Sciences (ITS)-Smt Gulabben Rasiklal Doshi and Smt Kamlaben Mafatlal Mehta Institute of Kidney Diseases and Research Centre (IKDRC), Civil Hospital Campus, Asarwa, Ahmedabad, Gujarat, India
2 Department of Nephrology and Clinical Transplantation, Dr. H. L. Trivedi Institute of Transplantation Sciences (ITS)-Smt Gulabben Rasiklal Doshi and Smt Kamlaben Mafatlal Mehta Institute of Kidney Diseases and Research Centre (IKDRC), Civil Hospital Campus, Asarwa, Ahmedabad, Gujarat, India

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Date of Web Publication7-May-2012
 

   Abstract 

C1q nephropathy with hemolytic uremic syndrome (HUS) is an uncommon combi要ation associated with rapidly progressive renal failure. We report a case of C1q nephropathy with HUS in an 18-year-old man associated with rapidly progressive renal failure. The patient did not respond to treatment with steroids and was started on maintenance hemodialysis. C1q nephropathy with HUS is rare and can lead to rapidly progressive renal failure.

How to cite this article:
Kanodia KV, Vanikar AV, Shah P R, Kute VB, Feroz A, Suthar K, Trivedi HL. C1q nephropathy with acute hemolytic uremic syndrome. Saudi J Kidney Dis Transpl 2012;23:556-8

How to cite this URL:
Kanodia KV, Vanikar AV, Shah P R, Kute VB, Feroz A, Suthar K, Trivedi HL. C1q nephropathy with acute hemolytic uremic syndrome. Saudi J Kidney Dis Transpl [serial online] 2012 [cited 2014 Sep 1];23:556-8. Available from: http://www.sjkdt.org/text.asp?2012/23/3/556/95805

   Introduction Top


C1q nephropathy is an immune complex glomerulonephritis defined by the presence of mesangial immunoglobulin and complement deposits, most notably C1q, with absence of clinical and laboratory evidence of systemic lupus erythematosus (SLE). [1] Hemolytic uremic syndrome (HUS) is characterized by the triad of hemolytic anemia, thrombocytopenia and acute renal failure. C1q nephropathy with HUS is rare. We report a case of C1q nephropathy with HUS diagnosed in an 18-year-old man.


   Case Report Top


An 18-year-old male presented with pedal edema, decreased urine output, dyspnea on exertion, nausea, vomiting and decreased ap計etite since one month. On examination, he had a pulse rate of 100/min, normal tempera負ure and blood pressure of 150/100 mmHg. Car-diological and neurological examination was un訃emarkable. Ultrasonography showed right kid要ey measuring 12.2 cm × 4.6 cm, left kidney measuring 11.5 cm × 5 cm, with increased echo茆enicity and preserved cortico-medullary diffe訃entiation. X-ray chest showed bilateral lower zone consolidation with infiltrative changes.

On investigation, urine albumin was 5.84 gm/24 h, microscopy showed 30-40 pus cells and 10-12 red blood cells/high-power field and culture was sterile. Sputum culture showed Klebsiella pneumoniae. The serum creatinine was 4.94 mg%, total protein was 5.7 gm/dL, albumin/globulin ratio was 3/2.2, random blood sugar was 113 mg/dL, sodium was 122 meq/L and potassium was 5.84 meq/L. The patient's hemoglobin was 12 gm/dL; total leukocyte count was 9400/mm 3 , platelet count was 235,000/mm 3 and D Dimer was 1500 ng/mL (normal range:200-500 ng/mL). All serological markers for SLE were negative.

Renal biopsy was performed and after paraf苯in embedding, 3-μm sections were taken and stained using hematoxylin and eosin, Periodic Acid Schiff, Jone's silver methane-amine and Gomori's trichrome stains. Indirect immunofluorescence staining was performed using anti-human IgG, IgM, IgA, C1q, C3, albumin and fibrinogen anti-sera (DAKO, USA). Histopathology revealed one core with 11 glomeruli. Most of the glomeruli showed normal dis負ribution of mesangial cells and focal mesangial matrix accentuation [Figure 1]. The capillary tufts had fairly open lumina lined by thickened and wrinkled membranes. Bowman capsules were segmentally thickened. One glomerulus showed afferent arteriolar lumina filled with red blood cells and thrombus [Figure 1]. Tubules were moderately degenerated and focally atro計hied. Interstitium showed focal fibrosis and overlying focal mononuclear cellular infiltra負ion with admixed neutrophils. All the small to medium caliber arteries showed severe mucoidintimal proliferation leading to complete luminal obliteration. One artery showed sub-minimal fibrin deposition [Figure 2].
Figure 1: Renal biopsy sample showing afferent arteriolar lumina filled with red blood cells and thrombus (GT, ×200).

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Figure 2: Renal biopsy sample showing mucointimal proliferation and fibrin deposition in the sub-intima of the artery (GT, ×200).

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Immunofluorescence studies showed fine gra要ular fluorescence (+3) across most of the mesangial regions and adjacent capillaries of all glomeruli on staining with anti-human C1q and (+3) with anti-human IgM antisera (DAKO) [Figure 3].
Figure 3: C1Q staining showing granular fluorescence (+3) in the mesangium (C1Q, ×200).

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The patient was treated with methylprednisolone 500 mg, intravenously for three days following which he was switched over to oral prednisolone, 40 mg/day. Hemodialysis was performed on alternate days for 21 days. Plasmaphresis could not be performed as the pa負ient was suffering from pneumonitis. The res計onse to steroids was not satisfactory and the patient remained on maintenance hemodialysis.


   Discussion Top


C1q nephropathy is characterized by a mild to marked mesangial prominence without seg衫ental sclerosis. C1q nephropathy usually pre貞ents with nephrotic range proteinuria in older children and young adults, and has a poor res計onse to steroids. It was first reported as a variant of focal and segmental glomerulosclerosis in 19 patients. [2]

HUS is an uncommon but serious condition that can cause life-threatening kidney failure. HUS most often affects children and older adults. In children, most cases of HUS develop after several days of diarrhea, often bloody, due to infection with a specific strain of  Escherichia More Details coli (E. coli). In adults, HUS may de赳elop after E. coli infection, but more often the cause is obscure or even unknown.

C1q nephropathy with HUS has not been reported in the native kidneys. [3] We present this young man who developed C1q nephropathy and acute HUS not responding to ste訃oids. He continued on maintenance hemodialysis.

C1q nephropathy with HUS can lead to ra計idly progressive renal failure. This was a rare case noted in a young man.

 
   References Top

1.Srivastava T, Chadha V, Taboada EM, Alon US. C1q nephropathy presenting as rapidly progressive crescentic glomerulonephritis. Pediatr. Nephrol 2000;14::976-9.  Back to cited text no. 1
    
2.Markowitz GS, Schwimmer JA, Stokes MB, et al. C1q nephropathy: a variant of focal seg衫ental glomerulosclerosis. Kidney Int 2003; 64:1232-40.  Back to cited text no. 2
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3.Isaac J, Shihab FS. De novo C1q nephropathy in the renal allograft of a kidney pancreas transplant recipient: BK Virus- induced nephropathy? Nephron 2002;92:431-6.  Back to cited text no. 3
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Correspondence Address:
Kamal V Kanodia
Associate Professor, Department of Pathology, Laboratory Medicine, Transfusion Services and Immunohematology, Dr. H. L. Trivedi Institute of Transplantation Sciences (ITS)-Smt Gulabben Rasiklal Doshi and Smt Kamlaben Mafatlal Mehta Institute of Kidney Diseases and Research Centre (IKDRC), Civil Hospital Campus, Asarwa, Ahmedabad - 380 016, Gujarat
India
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    Abstract
   Introduction
   Case Report
   Discussion
    References
    Article Figures
 

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