Hereditary hyperhomocysteinemia associated with nephrotic syndrome complicated by artery thrombosis and chronic thromboembolic pulmonary hypertension: A case report
We present here the case of a 30-year-old man with a long term history of nephrotic syndrome (NS) who developed an episode of acute left main pulmonary artery thrombosis complicated by a lung abscess. During the hospital admission was also identified a concomitant hyperhomocysteinemia. After an atyp...
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| Format: | Article |
| Language: | English |
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PAGEPress Publications
2017-12-01
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| Series: | Monaldi Archives for Chest Disease |
| Subjects: | |
| Online Access: | https://www.monaldi-archives.org/index.php/macd/article/view/880 |
| _version_ | 1818522147030564864 |
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| author | Paolo Ruggeri Federica Lo Bello Francesco Nucera Michele Gaeta Francesco Monaco Gaetano Caramori Giuseppe Girbino |
| author_facet | Paolo Ruggeri Federica Lo Bello Francesco Nucera Michele Gaeta Francesco Monaco Gaetano Caramori Giuseppe Girbino |
| author_sort | Paolo Ruggeri |
| collection | DOAJ |
| description | We present here the case of a 30-year-old man with a long term history of nephrotic syndrome (NS) who developed an episode of acute left main pulmonary artery thrombosis complicated by a lung abscess. During the hospital admission was also identified a concomitant hyperhomocysteinemia. After an atypical resection of the left upper pulmonary lobe and the starting of long term anticoagulation the patient was discharged but did not attend the planned follow up visits until one year later when he was seen again for severe dyspnea and exercise intolerance. At this time chronic thromboembolic pulmonary hypertension (CTEPH) was diagnosed by lung perfusion scintigraphy and right heart catheterization. He initially refused the surgical treatment but, after six months, for the presence of worsening dyspnea was referred for bilateral pulmonary endarterectomy followed by a cardio-thoracic rehabilitation program. After a follow-up of seven years the patient is alive and in stable conditions. NS and hyperhomocysteinemia are both known risk factors for pulmonary embolism (PE), but their association with CTEPH is extremely rare. We discuss here the possible mechanisms linking these conditions. CTEPH must be suspected in any patient with NS, with or without hyperhomocysteinemia, and unexplained dyspnea. |
| first_indexed | 2024-12-11T05:29:28Z |
| format | Article |
| id | doaj.art-4974f3a56fa2476e8f0745f7ff80ac7e |
| institution | Directory Open Access Journal |
| issn | 1122-0643 2532-5264 |
| language | English |
| last_indexed | 2024-12-11T05:29:28Z |
| publishDate | 2017-12-01 |
| publisher | PAGEPress Publications |
| record_format | Article |
| series | Monaldi Archives for Chest Disease |
| spelling | doaj.art-4974f3a56fa2476e8f0745f7ff80ac7e2022-12-22T01:19:28ZengPAGEPress PublicationsMonaldi Archives for Chest Disease1122-06432532-52642017-12-0187310.4081/monaldi.2017.880Hereditary hyperhomocysteinemia associated with nephrotic syndrome complicated by artery thrombosis and chronic thromboembolic pulmonary hypertension: A case reportPaolo Ruggeri0Federica Lo Bello1Francesco Nucera2Michele Gaeta3Francesco Monaco4Gaetano Caramori5Giuseppe Girbino6University of MessinaUniversity of MessinaUniversity of MessinaUniversity of MessinaUniversity of MessinaUniversity of MessinaUniversity of MessinaWe present here the case of a 30-year-old man with a long term history of nephrotic syndrome (NS) who developed an episode of acute left main pulmonary artery thrombosis complicated by a lung abscess. During the hospital admission was also identified a concomitant hyperhomocysteinemia. After an atypical resection of the left upper pulmonary lobe and the starting of long term anticoagulation the patient was discharged but did not attend the planned follow up visits until one year later when he was seen again for severe dyspnea and exercise intolerance. At this time chronic thromboembolic pulmonary hypertension (CTEPH) was diagnosed by lung perfusion scintigraphy and right heart catheterization. He initially refused the surgical treatment but, after six months, for the presence of worsening dyspnea was referred for bilateral pulmonary endarterectomy followed by a cardio-thoracic rehabilitation program. After a follow-up of seven years the patient is alive and in stable conditions. NS and hyperhomocysteinemia are both known risk factors for pulmonary embolism (PE), but their association with CTEPH is extremely rare. We discuss here the possible mechanisms linking these conditions. CTEPH must be suspected in any patient with NS, with or without hyperhomocysteinemia, and unexplained dyspnea.https://www.monaldi-archives.org/index.php/macd/article/view/880Chronic thromboembolic pulmonary hypertensionpulmonary embolismnephrotic syndromehyperhomocysteinemia. |
| spellingShingle | Paolo Ruggeri Federica Lo Bello Francesco Nucera Michele Gaeta Francesco Monaco Gaetano Caramori Giuseppe Girbino Hereditary hyperhomocysteinemia associated with nephrotic syndrome complicated by artery thrombosis and chronic thromboembolic pulmonary hypertension: A case report Monaldi Archives for Chest Disease Chronic thromboembolic pulmonary hypertension pulmonary embolism nephrotic syndrome hyperhomocysteinemia. |
| title | Hereditary hyperhomocysteinemia associated with nephrotic syndrome complicated by artery thrombosis and chronic thromboembolic pulmonary hypertension: A case report |
| title_full | Hereditary hyperhomocysteinemia associated with nephrotic syndrome complicated by artery thrombosis and chronic thromboembolic pulmonary hypertension: A case report |
| title_fullStr | Hereditary hyperhomocysteinemia associated with nephrotic syndrome complicated by artery thrombosis and chronic thromboembolic pulmonary hypertension: A case report |
| title_full_unstemmed | Hereditary hyperhomocysteinemia associated with nephrotic syndrome complicated by artery thrombosis and chronic thromboembolic pulmonary hypertension: A case report |
| title_short | Hereditary hyperhomocysteinemia associated with nephrotic syndrome complicated by artery thrombosis and chronic thromboembolic pulmonary hypertension: A case report |
| title_sort | hereditary hyperhomocysteinemia associated with nephrotic syndrome complicated by artery thrombosis and chronic thromboembolic pulmonary hypertension a case report |
| topic | Chronic thromboembolic pulmonary hypertension pulmonary embolism nephrotic syndrome hyperhomocysteinemia. |
| url | https://www.monaldi-archives.org/index.php/macd/article/view/880 |
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