Amal Khsiba, Sahar Nasr, Lamine Hamzaoui, Moufida Mahmoudi, Asma Ben Mohamed, Manel Yaakoubi, Mouna Medhioub, Mohamed Moussadek Azouz
{"title":"Osteopetrosis: a rare case of portal hypertension.","authors":"Amal Khsiba, Sahar Nasr, Lamine Hamzaoui, Moufida Mahmoudi, Asma Ben Mohamed, Manel Yaakoubi, Mouna Medhioub, Mohamed Moussadek Azouz","doi":"10.2144/fsoa-2022-0028","DOIUrl":null,"url":null,"abstract":"<p><p>Osteopetrosis is a rare genetic bone disorder characterized by a defect in osteoclasts recruitment and function. Its manifestations are numerous and they mainly include skeletal and dental deformities, cranial nerve entrapment and infections. Over time, osteoclastic expansion invades bone marrow leaving little space for hematopoietic cells. As a result, extramedullary hematopoiesis takes place in the reticular system mainly in the spleen and liver. In these patients, portal hypertension can occur as a result of extramedullary hematopoiesis associated splenomegaly. We are reporting in this article a rare case of spontaneous bacterial peritonitis associated with portal hypertension in a patient with osteopetrosis.</p>","PeriodicalId":12568,"journal":{"name":"Future Science OA","volume":"8 8","pages":"FSO817"},"PeriodicalIF":2.4000,"publicationDate":"2022-11-15","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9704036/pdf/","citationCount":"0","resultStr":null,"platform":"Semanticscholar","paperid":null,"PeriodicalName":"Future Science OA","FirstCategoryId":"1085","ListUrlMain":"https://doi.org/10.2144/fsoa-2022-0028","RegionNum":0,"RegionCategory":null,"ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":null,"EPubDate":"2022/9/1 0:00:00","PubModel":"eCollection","JCR":"Q3","JCRName":"MEDICINE, RESEARCH & EXPERIMENTAL","Score":null,"Total":0}
引用次数: 0
Abstract
Osteopetrosis is a rare genetic bone disorder characterized by a defect in osteoclasts recruitment and function. Its manifestations are numerous and they mainly include skeletal and dental deformities, cranial nerve entrapment and infections. Over time, osteoclastic expansion invades bone marrow leaving little space for hematopoietic cells. As a result, extramedullary hematopoiesis takes place in the reticular system mainly in the spleen and liver. In these patients, portal hypertension can occur as a result of extramedullary hematopoiesis associated splenomegaly. We are reporting in this article a rare case of spontaneous bacterial peritonitis associated with portal hypertension in a patient with osteopetrosis.
期刊介绍：
Future Science OA is an online, open access, peer-reviewed title from the Future Science Group. The journal covers research and discussion related to advances in biotechnology, medicine and health. The journal embraces the importance of publishing all good-quality research with the potential to further the progress of research in these fields. All original research articles will be considered that are within the journal''s scope, and have been conducted with scientific rigour and research integrity. The journal also features review articles, editorials and perspectives, providing readers with a leading source of commentary and analysis. Submissions of the following article types will be considered: -Research articles -Preliminary communications -Short communications -Methodologies -Trial design articles -Trial results (including early-phase and negative studies) -Reviews -Perspectives -Commentaries
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