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Kowloon, Hong Kong

Soo E.,Center for Pediatric and Adolescent Medicine | Soo E.,Paediatric Nephrology Center | Sanders A.,University of Heidelberg | Heckert K.,Center for Pediatric and Adolescent Medicine | And 3 more authors.
Pediatric Nephrology | Year: 2016

Background: In children acute liver failure is a rare but life-threatening condition from which two-thirds do not recover with supportive therapy. Treatment is limited by the availability of liver transplants. Molecular adsorbent recirculating system (MARS) dialysis is a bridge to transplantation that enhances the chances of survival during the waiting period for a transplant, although it cannot improve survival. Open albumin dialysis (OPAL) is a new mode of albumin dialysis developed to further improve dialysis efficiency. Case diagnosis/treatment: We report a paediatric case of acute-on-chronic liver failure and compare the two modes of albumin dialysis, namely, the MARS and OPAL, used to treat this patient’s cholestatic pruritus. Removal of total and direct bilirubin, ammonia and bile acids were measured by serial blood tests. There was an increased removal of bile acids with the OPAL mode, whereas the removal of total and direct bilirubin and ammonia was similar in both modes. The patient reported better improvement in pruritus following OPAL compared to dialysis with the MARS. Conclusion: OPAL may offer a better solution than the MARS in the treatment of refractory pruritus in liver failure. © 2016 IPNA

Ma A.L.-T.,Paediatric Nephrology Center | Lai W.-M.,Paediatric Nephrology Center | Chiu M.-C.,Paediatric Nephrology Center
Clinical and Experimental Nephrology | Year: 2010

We report a 14-year-old girl with nephrotic syndrome and renal vein thrombosis (RVT) on initial presentation. The patient tested positive for antinuclear antibodies but only weakly positive for anti-double-stranded DNA (anti-dsDNA). Her C3 level was normal. Treatment with low molecular weight heparin resulted in resolution of RVT. Renal biopsy showed membranous glomerulonephritis with segmental sclerosis. Tissue immunostaining showed diffuse granular C3 and immunoglobulin (Ig)G staining along the capillary wall with focal segmental IgM staining deposits in the mesangium. No C1q, IgA, or fibrinogen was noted on immunofluorescence assay. With cyclosporin A and prednisolone, the patient went into remission and corticosteroids were tapered off gradually. Two years later, she had a relapse of proteinuria, hypocomplementemia, and extremely high anti-dsDNA. Systemic lupus erythematosus (SLE) was diagnosed, and she was promptly started on steroid and immunosuppressive agents, which resulted in reduction of proteinuria. Her renal function has been normal all along. Membranous nephropathy is uncommon in Chinese children and could be a possible early presentation of SLE. © 2009 Japanese Society of Nephrology.

Ma A.L.-T.,Paediatric Nephrology Center | Lai W.-M.,Paediatric Nephrology Center | Chiu M.-C.,Paediatric Nephrology Center | Tse N.K.-C.,Paediatric Nephrology Center
Clinical and Experimental Nephrology | Year: 2012

Lupus vasculopathy (LV) is one of the complications of lupus nephritis. No definite therapy has been recommended for the management of LV, and patient outcomes are variable. We present the case of a 16-year-old girl who recovered from stage V chronic kidney disease due to lupus nephritis with vasculopathy with active treatment including pulse methylprednisolone, cyclophosphamide, rituximab and plasma exchange. © 2012 Japanese Society of Nephrology.

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