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Our subject had hypertension since 10 years and may not be a manifestation of CG

Our subject had hypertension since 10 years and may not be a manifestation of CG. view of comparable histological findings but without skeletal abnormalities.[4,5] The patients range in age from 2 to 66 years, with no sex predilection.[6] The most common clinical presentation is proteinuria with or without associated nephrotic syndrome, with minor alterations in renal function.[7] Only 17 cases have been reported from India.[6] We report a case of this rare entity. Case Statement A 63-year-old man, hypertensive for ten years, nondiabetic, a vegan, and with no significant recent or family history, presented with anasarca since 2? months. On physical examination, his blood pressure was 160/100 mm Hg and fundus showed grade two hypertensive retinopathy. Laboratory investigations revealed macrocytic anemia (hemoglobin 10.7 g/dl), 24 h urine protein of 3.9 g, and serum albumin of 2.7 g/dl. The serum creatinine (0.8 mg/dl), lipid profile, complement levels, thyroid profile were within normal range. There was an associated Vitamin D deficiency (9.6 ng/ml) and Vitamin B12 deficiency (135 mcg/dl). Myeloma screening was unfavorable. Ultrasound of stomach showed normal sized kidneys with increased echotexture. Viral screen for HIV, hepatitis B computer virus, and hepatitis C computer virus was unfavorable, and coagulation Radezolid profile was normal. The renal biopsy [Physique 1] showed 15 glomeruli, two of whom were sclerosed. Viable glomeruli were enlarged in size with deposition of pale eosinophilic material in the mesangium forming nodules. This material was unfavorable for Periodic Acid Schiff (PAS), Congo reddish and positive with silver methenamine. Masson trichrome showed increased collagen within the nodules. The glomerular basement membrane (GBM) was mildly thickened and showed focal reduplication. There was no evidence of tubule-interstitial chronicity or inflammation. Direct immunofluorescence of renal biopsy with IgM, IgA, IgG, C3, C1q, and kappa and lambda light chains were unfavorable ruling out the presence of immune complex deposits. These features were suggestive of a possible CG, which was further confirmed with electron microscopy. Electron microscopy [Physique 2] showed markedly enlarged glomerulus with lobular arrangement and diffuse foot process effacement. The mesangium and capillary lumina revealed subendothelial deposits of large fibers (97 nm width) which are curvilinear and with disorganized arrangement and periodicity suggestive of collagen fibers; confirming the diagnosis of collagenous glomerulopathy. Open in a separate window Physique 1 (a) The glomerular enlargement with mesangial growth with the formation of nodules. The nodules were (b) positive for silver stains (c) unfavorable with Periodic Acid Schiff stain and (d) collagen deposition with Masson trichrome stain Open in a separate window Physique 2 The electron microscopic study of renal biopsy specimen fixed using glutaraldehyde shows the presence of subendothelial deposits of large HOX1 fibers which are curvilinear and with disorganized arrangement suggestive of collagen fibers; confirming the diagnosis of collagenous glomerulopathy No electron dense deposits were recognized. The lamina densa of GBM was unremarkable. All these features confirmed the diagnosis of CG. Conversation CG is recognized as a distinct entity and Radezolid is relatively common in Asian countries.[6] Radezolid Ethnic/genetic factors play an important role in etiopathogenesis. You will find two fundamental theories regarding the genesis of this disease. First is usually that normal human glomeruli lack collagen III, and it is postulated that mesangial cells are the endogenous source of collagen III. The second theory is usually that this disease is usually a systemic disorder with abnormal metabolism of collagen III.[8] The incidence of CG is highest between the fourth and seventh decades, notably among Asian patients; while those reported in Europe are children.[9] The most common clinical presentation is edema and/or persistent proteinuria that Radezolid may be a nephrotic range (about 60% of patients). Our individual is usually a 63-year-old who presented with generalized edema and experienced nephrotic range proteinuria. Hypertension is seen in the two-third of cases at the time of presentation. Our subject experienced hypertension since 10 years and may not be a manifestation of CG. Anemia may be noticed even before the development of renal dysfunction. Occasionally, microangiopathic hemolytic anemia has been documented in children. Our patient experienced macrocytic anemia secondary to Vitamin B12 deficiency as he was a rigid vegan. Only 17 cases of CG have been reported from India to the best of our knowledge. Table 1 summarizes the clinical profile of published cases of CG in literature. Extrarenal symptoms are absent, unlike in cases of NailCPatella syndrome. The natural history of the disease is usually variable, but it is usually progressive in most patients. Children are more likely to progress to end-stage renal failure. Successful renal transplantation without a recurrence has been documented in one case.[7] The only specific test for CG is an estimation of procollagen III N-terminal peptide levels. Procollagen peptide levels or immunohistochemistry for collagen typing was not.