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Background:C1qnephropathy (C1qN)is a rare glomerulopathy,with a very low prevalenceworld widevarying from 0.2 to 2.5%. Even thoughmore than three decades have passed since this entity was first explained, still,it remains a dilemma for many due to the rarityof this lesion. This study was carried out principally to determine theclinical presentation, morphologic features and distribution of C1qN in our region based on renal biopsies studied by light microscopy (LM), and immunofluorescence (IF) so that this entity is better understood both by nephrologists and pathologistsas no such study has ever been conducted in Pakistan to our knowledge.Methods:It was a cross-sectional study carried out from 1st January 2012 to 30th December 2016in Histopathology department, Shifa International Hospital.All cases diagnosed as C1q nephropathy were retrieved from thehospital’scomputerized database. Their clinical profiles, morphology and immunohistochemical profiles were studied.Results:Overthis period a total of 31 cases were diagnosed withC1qN. Mean age of the patients was 32.09±18.66 years. The most common clinical presentation was nephrotic syndrome seenin 22(71%) patients. The most frequentmorphologicalpattern seen was minimal change disease(MCD) in13(41.9%)cases. All cases showed dominant 22(71%) or codominant 9 (42.9%) mesangial±membranousC1q deposition. No correlation was found (p-value >0.05) between morphological pattern and clinical presentation of the disease or immunofluorescencefindings.Conclusion:C1qN isa rare entity which is primarily diagnosed on the basis of immunofluorescencefindings with a dominant or codominant fluorescent intensity for C1q. It is recommended that C1qNissought for preferably with immunofluorescencestaining of biopsies forimmune reactants, especiallyfor C1q. Studies from this part of the world are strongly recommended to predict clinical outcome and treatment options

Dr. Naima Tariq,, Humaira Nasir, Tahir Aziz Ahmed, Mariam Usman, Khawaja Sayeed Ahmed. (2019) C1Q NEPHROPATHY: A MULTIFACETED DISEASE WITH INFREQUENTDIAGNOSIS, JOURNAL OF AYUB MEDICAL COLLEGE ABBOTTABAD, Volume 31, Issue 3.
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