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A Case of Newly Diagnosed Klippel Trenaunay Weber Syndrome Presenting with Nephrotic Syndrome
Klippel Trenaunay Weber syndrome (KTWS) is a rare disease characterized by hemihypertrophy, variceal enlargement of the veins, and arteriovenous (AV) malformations. Renal involvement in KTWS is not known except in rare case reports. Herein, we present a case of KTWS with nephrotic syndrome. A 52-yea...
Autores principales: | , , , , , , , , , , |
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Formato: | Online Artículo Texto |
Lenguaje: | English |
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Hindawi Publishing Corporation
2015
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Acceso en línea: | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4426781/ https://www.ncbi.nlm.nih.gov/pubmed/26000182 http://dx.doi.org/10.1155/2015/704379 |
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author | Cebeci, Egemen Demir, Secil Gursu, Meltem Sumnu, Abdullah Yamak, Mehmet Doner, Barıs Karadag, Serhat Uzun, Sami Behlul, Ahmet Ozkan, Oktay Ozturk, Savas |
author_facet | Cebeci, Egemen Demir, Secil Gursu, Meltem Sumnu, Abdullah Yamak, Mehmet Doner, Barıs Karadag, Serhat Uzun, Sami Behlul, Ahmet Ozkan, Oktay Ozturk, Savas |
author_sort | Cebeci, Egemen |
collection | PubMed |
description | Klippel Trenaunay Weber syndrome (KTWS) is a rare disease characterized by hemihypertrophy, variceal enlargement of the veins, and arteriovenous (AV) malformations. Renal involvement in KTWS is not known except in rare case reports. Herein, we present a case of KTWS with nephrotic syndrome. A 52-year-old male was admitted due to dyspnea and swelling of the body for the last three months. The pathological physical findings were diffuse edema, decreased lung sounds at the right basal site, increased diameter and decreased length of the left leg compared with the right one, diffuse variceal enlargements, and a few hemangiomatous lesions on the left leg. The pathological laboratory findings were hypoalbuminemia, hyperlipidemia, increased creatinine level (1.23 mg/dL), and proteinuria (7.6 g/day). Radiographic pathological findings were cystic lesions in the liver, spleen, and kidneys, splenomegaly, AV malformation on the left posterolateral thigh, and hypertrophy of the soft tissues of the proximal left leg. He was diagnosed to have KTWS with these findings. Renal biopsy was performed to determine the cause of nephrotic syndrome. The pathologic examination was consistent with focal segmental sclerosis (FSGS). He was started on oral methylprednisolone at the dosage of 1 mg/kg and began to be followedup in the nephrology outpatient clinic. |
format | Online Article Text |
id | pubmed-4426781 |
institution | National Center for Biotechnology Information |
language | English |
publishDate | 2015 |
publisher | Hindawi Publishing Corporation |
record_format | MEDLINE/PubMed |
spelling | pubmed-44267812015-05-21 A Case of Newly Diagnosed Klippel Trenaunay Weber Syndrome Presenting with Nephrotic Syndrome Cebeci, Egemen Demir, Secil Gursu, Meltem Sumnu, Abdullah Yamak, Mehmet Doner, Barıs Karadag, Serhat Uzun, Sami Behlul, Ahmet Ozkan, Oktay Ozturk, Savas Case Rep Nephrol Case Report Klippel Trenaunay Weber syndrome (KTWS) is a rare disease characterized by hemihypertrophy, variceal enlargement of the veins, and arteriovenous (AV) malformations. Renal involvement in KTWS is not known except in rare case reports. Herein, we present a case of KTWS with nephrotic syndrome. A 52-year-old male was admitted due to dyspnea and swelling of the body for the last three months. The pathological physical findings were diffuse edema, decreased lung sounds at the right basal site, increased diameter and decreased length of the left leg compared with the right one, diffuse variceal enlargements, and a few hemangiomatous lesions on the left leg. The pathological laboratory findings were hypoalbuminemia, hyperlipidemia, increased creatinine level (1.23 mg/dL), and proteinuria (7.6 g/day). Radiographic pathological findings were cystic lesions in the liver, spleen, and kidneys, splenomegaly, AV malformation on the left posterolateral thigh, and hypertrophy of the soft tissues of the proximal left leg. He was diagnosed to have KTWS with these findings. Renal biopsy was performed to determine the cause of nephrotic syndrome. The pathologic examination was consistent with focal segmental sclerosis (FSGS). He was started on oral methylprednisolone at the dosage of 1 mg/kg and began to be followedup in the nephrology outpatient clinic. Hindawi Publishing Corporation 2015 2015-04-27 /pmc/articles/PMC4426781/ /pubmed/26000182 http://dx.doi.org/10.1155/2015/704379 Text en Copyright © 2015 Egemen Cebeci et al. https://creativecommons.org/licenses/by/3.0/ This is an open access article distributed under the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited. |
spellingShingle | Case Report Cebeci, Egemen Demir, Secil Gursu, Meltem Sumnu, Abdullah Yamak, Mehmet Doner, Barıs Karadag, Serhat Uzun, Sami Behlul, Ahmet Ozkan, Oktay Ozturk, Savas A Case of Newly Diagnosed Klippel Trenaunay Weber Syndrome Presenting with Nephrotic Syndrome |
title | A Case of Newly Diagnosed Klippel Trenaunay Weber Syndrome Presenting with Nephrotic Syndrome |
title_full | A Case of Newly Diagnosed Klippel Trenaunay Weber Syndrome Presenting with Nephrotic Syndrome |
title_fullStr | A Case of Newly Diagnosed Klippel Trenaunay Weber Syndrome Presenting with Nephrotic Syndrome |
title_full_unstemmed | A Case of Newly Diagnosed Klippel Trenaunay Weber Syndrome Presenting with Nephrotic Syndrome |
title_short | A Case of Newly Diagnosed Klippel Trenaunay Weber Syndrome Presenting with Nephrotic Syndrome |
title_sort | case of newly diagnosed klippel trenaunay weber syndrome presenting with nephrotic syndrome |
topic | Case Report |
url | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4426781/ https://www.ncbi.nlm.nih.gov/pubmed/26000182 http://dx.doi.org/10.1155/2015/704379 |
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