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Chronic thromboembolic pulmonary hypertension as the first manifestation of nephrotic syndrome in a 12-year-old child
RATIONALE: Chronic thromboembolic pulmonary hypertension (CTEPH) is rare in children and determining the underlying etiologies is essential for treatment. Venous thromboembolism, a well-known complication in nephrotic syndrome (NS), always occurrs during the treatment in course of the disease. Howev...
Autores principales: | , , , , , , |
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Formato: | Online Artículo Texto |
Lenguaje: | English |
Publicado: |
Wolters Kluwer Health
2018
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Materias: | |
Acceso en línea: | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6203542/ https://www.ncbi.nlm.nih.gov/pubmed/30313030 http://dx.doi.org/10.1097/MD.0000000000012349 |
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author | Ma, Fan Zhou, Kaiyu Hua, Yimin Liu, Xiaoliang Duan, Hongyu Li, Yifei Wang, Chuan |
author_facet | Ma, Fan Zhou, Kaiyu Hua, Yimin Liu, Xiaoliang Duan, Hongyu Li, Yifei Wang, Chuan |
author_sort | Ma, Fan |
collection | PubMed |
description | RATIONALE: Chronic thromboembolic pulmonary hypertension (CTEPH) is rare in children and determining the underlying etiologies is essential for treatment. Venous thromboembolism, a well-known complication in nephrotic syndrome (NS), always occurrs during the treatment in course of the disease. However, CTEPH as the first manifestation of NS has not been reported till now. PATIENT CONCERNS: A 12-year-old boy initially complained of hemoptysis, cough and shortness of breath with exertion, any symptoms regarding NS such as edema were not presented. Due to the identification of P2 enhancement, liver enlargement (2 cm below the rib) and jugular vein distension, pulmonary hypertension (PH) was firstly suspected and ultimately confirmed by detection of enlargement of right atrium (RA) and right ventricle (RV) enlargement (RA = 45mm, RV = 30mm), mild tricuspid valve regurgitation (TR) and elevation of pulmonary arterial pressure (63 mmHg) on echocardiogram. In order to search the underlying causes of PH, series of targeted laboratory evaluation and imaging were conducted, and pulmonary arterial embolism (PE) in inferior lobes of double lungs was found on chest contrast-enhanced computed tomography. DIAGNOSIS: NS was unexpectedly discovered by detection of lower serum albumin level (24.4 g/L), severe proteinuria (+++, 4.62 g/24 h) when we were searching for the predisposing factors causing thromboembolism. INTERVENTIONS AND OUTCOMES: After treatment of NS, the symptom regarding shortness of breath with exertion gradually became less apparent and was relieved one month later. Proteinuria and microscopic hematuria also disappeared. Encouragingly, RA and RV dilation, and the pulmonary arterial pressure almost returned to a normal range half a year later, with alleviation of MR. LESSONS: CTEPH can occur rarely in children and NS is an important predisposing factor. PE could be the first manifestation of NS. When pediatricians encounter children with PE or CTEPH, NS as the underlying etiology should be considered. Except for renal venous thrombosis, the possibility of PE needs to be paid more attention in children with NS. |
format | Online Article Text |
id | pubmed-6203542 |
institution | National Center for Biotechnology Information |
language | English |
publishDate | 2018 |
publisher | Wolters Kluwer Health |
record_format | MEDLINE/PubMed |
spelling | pubmed-62035422018-11-07 Chronic thromboembolic pulmonary hypertension as the first manifestation of nephrotic syndrome in a 12-year-old child Ma, Fan Zhou, Kaiyu Hua, Yimin Liu, Xiaoliang Duan, Hongyu Li, Yifei Wang, Chuan Medicine (Baltimore) Research Article RATIONALE: Chronic thromboembolic pulmonary hypertension (CTEPH) is rare in children and determining the underlying etiologies is essential for treatment. Venous thromboembolism, a well-known complication in nephrotic syndrome (NS), always occurrs during the treatment in course of the disease. However, CTEPH as the first manifestation of NS has not been reported till now. PATIENT CONCERNS: A 12-year-old boy initially complained of hemoptysis, cough and shortness of breath with exertion, any symptoms regarding NS such as edema were not presented. Due to the identification of P2 enhancement, liver enlargement (2 cm below the rib) and jugular vein distension, pulmonary hypertension (PH) was firstly suspected and ultimately confirmed by detection of enlargement of right atrium (RA) and right ventricle (RV) enlargement (RA = 45mm, RV = 30mm), mild tricuspid valve regurgitation (TR) and elevation of pulmonary arterial pressure (63 mmHg) on echocardiogram. In order to search the underlying causes of PH, series of targeted laboratory evaluation and imaging were conducted, and pulmonary arterial embolism (PE) in inferior lobes of double lungs was found on chest contrast-enhanced computed tomography. DIAGNOSIS: NS was unexpectedly discovered by detection of lower serum albumin level (24.4 g/L), severe proteinuria (+++, 4.62 g/24 h) when we were searching for the predisposing factors causing thromboembolism. INTERVENTIONS AND OUTCOMES: After treatment of NS, the symptom regarding shortness of breath with exertion gradually became less apparent and was relieved one month later. Proteinuria and microscopic hematuria also disappeared. Encouragingly, RA and RV dilation, and the pulmonary arterial pressure almost returned to a normal range half a year later, with alleviation of MR. LESSONS: CTEPH can occur rarely in children and NS is an important predisposing factor. PE could be the first manifestation of NS. When pediatricians encounter children with PE or CTEPH, NS as the underlying etiology should be considered. Except for renal venous thrombosis, the possibility of PE needs to be paid more attention in children with NS. Wolters Kluwer Health 2018-10-12 /pmc/articles/PMC6203542/ /pubmed/30313030 http://dx.doi.org/10.1097/MD.0000000000012349 Text en Copyright © 2018 the Author(s). Published by Wolters Kluwer Health, Inc. http://creativecommons.org/licenses/by-nc-nd/4.0 This is an open access article distributed under the terms of the Creative Commons Attribution-Non Commercial-No Derivatives License 4.0 (CCBY-NC-ND), where it is permissible to download and share the work provided it is properly cited. The work cannot be changed in any way or used commercially without permission from the journal. http://creativecommons.org/licenses/by-nc-nd/4.0 |
spellingShingle | Research Article Ma, Fan Zhou, Kaiyu Hua, Yimin Liu, Xiaoliang Duan, Hongyu Li, Yifei Wang, Chuan Chronic thromboembolic pulmonary hypertension as the first manifestation of nephrotic syndrome in a 12-year-old child |
title | Chronic thromboembolic pulmonary hypertension as the first manifestation of nephrotic syndrome in a 12-year-old child |
title_full | Chronic thromboembolic pulmonary hypertension as the first manifestation of nephrotic syndrome in a 12-year-old child |
title_fullStr | Chronic thromboembolic pulmonary hypertension as the first manifestation of nephrotic syndrome in a 12-year-old child |
title_full_unstemmed | Chronic thromboembolic pulmonary hypertension as the first manifestation of nephrotic syndrome in a 12-year-old child |
title_short | Chronic thromboembolic pulmonary hypertension as the first manifestation of nephrotic syndrome in a 12-year-old child |
title_sort | chronic thromboembolic pulmonary hypertension as the first manifestation of nephrotic syndrome in a 12-year-old child |
topic | Research Article |
url | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6203542/ https://www.ncbi.nlm.nih.gov/pubmed/30313030 http://dx.doi.org/10.1097/MD.0000000000012349 |
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