Document Details

Document Type : Article In Journal 
Document Title :
بحث عن أختين مصابتين بالمتلازمة الكلوية الغير مستجيبة للبيدنزلون بسبب التغييرات في سي1 كيو C1q nephropathy in two young sisters.
بحث عن أختين مصابتين بالمتلازمة الكلوية الغير مستجيبة للبيدنزلون بسبب التغييرات في سي1 كيو C1q nephropathy in two young sisters.
 
Document Language : English 
Abstract : C1q nephropathy (C1qNP) is a controversial and uncommon form of glomerulonephritis, characterized by mesangial immunoglobulin and complement deposits, predominantly C1q, with no evidence of systemic lupus erythematosus. Clinically, it may present as nephrotic syndrome and non-nephrotic proteinuria per se or associated with microhematuria, hypertension, or renal insufficiency. We describe two sisters with C1qNP, who presented with steroid-resistant nephrotic syndrome. Both sisters presented before the age of 2 years, and they showed a poor response to other immunosuppressive therapy. Both girls had normal serum complement levels, negative antinuclear antibodies (ANAs) and negative hepatitis B antigen. Renal biopsy in both patients showed histological features of mesangioproliferative glomerulonephritis, with diffuse "full-house" positive immunofluorescence reaction in the mesangial area. The immunofluorescence reaction for C1q was most intense and co-dominant with IgG in both patients. Correspondingly, electron microscopy demonstrated dense deposits mainly in the mesangial areas too. We report on two young sisters with the characteristic features of C1qNP presented in early childhood. To the best of our knowledge, this is the first report of C1qNP in siblings. 
ISSN : 8832-1012 
Journal Name : Pediatr Nephrol. 
Volume : 3 
Issue Number : 23 
Publishing Year : 2008 AH
2008 AD 
Article Type : Article 
Added Date : Wednesday, March 10, 2010 

Researchers

Researcher Name (Arabic)Researcher Name (English)Researcher TypeDr GradeEmail
جميلة قاريKari, Jameela ResearcherDoctorate 

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