Pauci-immune crescentic glomerulonephritis in the Down′s syndrome
Kidney disease is a rare complication in patients with the Down′s syndrome. However, with increased survival, it appears that a growing number of these patients present with glomerulonephritis. Most cases have been reported as case reports and include lesions such as mesangiocapillary glomerulonephr...
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Wolters Kluwer Medknow Publications
2013-01-01
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Series: | Saudi Journal of Kidney Diseases and Transplantation |
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doaj-9b09120e3d0c4e3199580d368ea0785b2020-11-24T21:22:21ZengWolters Kluwer Medknow PublicationsSaudi Journal of Kidney Diseases and Transplantation1319-24422013-01-012461223122710.4103/1319-2442.121311Pauci-immune crescentic glomerulonephritis in the Down′s syndromeMejda CherifHafedh HedriMondher OunissiTaher GergahRim GouchaSamia BarbouchEzzedine AbderrahimHedi Ben MaizAdel KhederKidney disease is a rare complication in patients with the Down′s syndrome. However, with increased survival, it appears that a growing number of these patients present with glomerulonephritis. Most cases have been reported as case reports and include lesions such as mesangiocapillary glomerulonephritis with hypo-complementemia, crescentic glomerulonephritis with anti-neutrophil cytoplasmic antibodies (ANCA), amyloidosis and immunotactoid glomerulopathy. We report the observation of a 38-year-old man with the Down′s syndrome who presented with severe renal failure, proteinuria and microscopic hematuria evolving over two months. There was no history of congenital heart disease or urinary symptoms. Percutaneous renal biopsy revealed fibrous crescents, rupture of Bowman′s capsule and peri-glomerular granuloma; there were no deposits on immunofluorescence study. Thoracic computerized tomography scan showed alveolar congestion. The patient tested negative for ANCA. At the time of reporting, the patient is on regular chronic hemodialysis. Our case illustrates a distinct entity that further expands the spectrum of renal disease known to occur in the Down′s syndrome. Early detection of the renal disorders may prevent or slow down the progression.http://www.sjkdt.org/article.asp?issn=1319-2442;year=2013;volume=24;issue=6;spage=1223;epage=1227;aulast=Cherif |
collection |
DOAJ |
language |
English |
format |
Article |
sources |
DOAJ |
author |
Mejda Cherif Hafedh Hedri Mondher Ounissi Taher Gergah Rim Goucha Samia Barbouch Ezzedine Abderrahim Hedi Ben Maiz Adel Kheder |
spellingShingle |
Mejda Cherif Hafedh Hedri Mondher Ounissi Taher Gergah Rim Goucha Samia Barbouch Ezzedine Abderrahim Hedi Ben Maiz Adel Kheder Pauci-immune crescentic glomerulonephritis in the Down′s syndrome Saudi Journal of Kidney Diseases and Transplantation |
author_facet |
Mejda Cherif Hafedh Hedri Mondher Ounissi Taher Gergah Rim Goucha Samia Barbouch Ezzedine Abderrahim Hedi Ben Maiz Adel Kheder |
author_sort |
Mejda Cherif |
title |
Pauci-immune crescentic glomerulonephritis in the Down′s syndrome |
title_short |
Pauci-immune crescentic glomerulonephritis in the Down′s syndrome |
title_full |
Pauci-immune crescentic glomerulonephritis in the Down′s syndrome |
title_fullStr |
Pauci-immune crescentic glomerulonephritis in the Down′s syndrome |
title_full_unstemmed |
Pauci-immune crescentic glomerulonephritis in the Down′s syndrome |
title_sort |
pauci-immune crescentic glomerulonephritis in the down′s syndrome |
publisher |
Wolters Kluwer Medknow Publications |
series |
Saudi Journal of Kidney Diseases and Transplantation |
issn |
1319-2442 |
publishDate |
2013-01-01 |
description |
Kidney disease is a rare complication in patients with the Down′s syndrome. However, with increased survival, it appears that a growing number of these patients present with glomerulonephritis. Most cases have been reported as case reports and include lesions such as mesangiocapillary glomerulonephritis with hypo-complementemia, crescentic glomerulonephritis with anti-neutrophil cytoplasmic antibodies (ANCA), amyloidosis and immunotactoid glomerulopathy. We report the observation of a 38-year-old man with the Down′s syndrome who presented with severe renal failure, proteinuria and microscopic hematuria evolving over two months. There was no history of congenital heart disease or urinary symptoms. Percutaneous renal biopsy revealed fibrous crescents, rupture of Bowman′s capsule and peri-glomerular granuloma; there were no deposits on immunofluorescence study. Thoracic computerized tomography scan showed alveolar congestion. The patient tested negative for ANCA. At the time of reporting, the patient is on regular chronic hemodialysis. Our case illustrates a distinct entity that further expands the spectrum of renal disease known to occur in the Down′s syndrome. Early detection of the renal disorders may prevent or slow down the progression. |
url |
http://www.sjkdt.org/article.asp?issn=1319-2442;year=2013;volume=24;issue=6;spage=1223;epage=1227;aulast=Cherif |
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