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Une forme particulière de pancytopénie

Primary hyperoxaluria is a rare disease whose incidence is estimated at less than 1 cases/million inhabitants/year. This is a congenital abnormality of hepatic metabolism leading to an endogenous overproduction of oxalate with excess urinary excretion. We report the case of a 43-year-old patient, wa...

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Detalles Bibliográficos
Autores principales: Chahdi, Hafsa, Oukabli, Mohamed
Formato: Online Artículo Texto
Lenguaje:English
Publicado: The African Field Epidemiology Network 2018
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6080970/
https://www.ncbi.nlm.nih.gov/pubmed/30100963
http://dx.doi.org/10.11604/pamj.2018.29.209.14055
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author Chahdi, Hafsa
Oukabli, Mohamed
author_facet Chahdi, Hafsa
Oukabli, Mohamed
author_sort Chahdi, Hafsa
collection PubMed
description Primary hyperoxaluria is a rare disease whose incidence is estimated at less than 1 cases/million inhabitants/year. This is a congenital abnormality of hepatic metabolism leading to an endogenous overproduction of oxalate with excess urinary excretion. We report the case of a 43-year-old patient, was followed to end-stage renal disease hemodialysis, consulting for anemic syndrome with mucocutaneous pallor. Laboratory tests found pancytopenia with aplastic anemia. Bone marrow was difficult to achieve, bringing medullary blood hyperdilué and uninterpretable . Radiographs showed a homogeneous splenomegaly and small dedifferentiated a kidney marrow biopsy was performed. Histological examination revealed a myelofibrosis and birefringent crystals in polarized light, diagnosis retained: spinal oxalosis.
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spelling pubmed-60809702018-08-10 Une forme particulière de pancytopénie Chahdi, Hafsa Oukabli, Mohamed Pan Afr Med J Images in Medicine Primary hyperoxaluria is a rare disease whose incidence is estimated at less than 1 cases/million inhabitants/year. This is a congenital abnormality of hepatic metabolism leading to an endogenous overproduction of oxalate with excess urinary excretion. We report the case of a 43-year-old patient, was followed to end-stage renal disease hemodialysis, consulting for anemic syndrome with mucocutaneous pallor. Laboratory tests found pancytopenia with aplastic anemia. Bone marrow was difficult to achieve, bringing medullary blood hyperdilué and uninterpretable . Radiographs showed a homogeneous splenomegaly and small dedifferentiated a kidney marrow biopsy was performed. Histological examination revealed a myelofibrosis and birefringent crystals in polarized light, diagnosis retained: spinal oxalosis. The African Field Epidemiology Network 2018-04-09 /pmc/articles/PMC6080970/ /pubmed/30100963 http://dx.doi.org/10.11604/pamj.2018.29.209.14055 Text en © Hafsa Chahdi et al. http://creativecommons.org/licenses/by/2.0/ The Pan African Medical Journal - ISSN 1937-8688. This is an Open Access article distributed under the terms of the Creative Commons Attribution License which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.
spellingShingle Images in Medicine
Chahdi, Hafsa
Oukabli, Mohamed
Une forme particulière de pancytopénie
title Une forme particulière de pancytopénie
title_full Une forme particulière de pancytopénie
title_fullStr Une forme particulière de pancytopénie
title_full_unstemmed Une forme particulière de pancytopénie
title_short Une forme particulière de pancytopénie
title_sort une forme particulière de pancytopénie
topic Images in Medicine
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6080970/
https://www.ncbi.nlm.nih.gov/pubmed/30100963
http://dx.doi.org/10.11604/pamj.2018.29.209.14055
work_keys_str_mv AT chahdihafsa uneformeparticulieredepancytopenie
AT oukablimohamed uneformeparticulieredepancytopenie