Please use this identifier to cite or link to this item:
10.1186/s13256-017-1413-1
Title: | Klippel-Trenaunay-Weber syndrome with atypical presentation of hypersplenism and nephrotic syndrome : A case report |
Authors: | Kundzina, Linda Lejniece, Sandra Department of Internal Diseases |
Keywords: | Hypersplenism;Klippel-Trenaunay-Weber syndrome;Nephrotic syndrome;3.2 Clinical medicine;1.1. Scientific article indexed in Web of Science and/or Scopus database;General Medicine |
Issue Date: | 21-Aug-2017 |
Citation: | Kundzina , L & Lejniece , S 2017 , ' Klippel-Trenaunay-Weber syndrome with atypical presentation of hypersplenism and nephrotic syndrome : A case report ' , Journal of Medical Case Reports , vol. 11 , no. 1 , 243 . https://doi.org/10.1186/s13256-017-1413-1 |
Abstract: | Background: Klippel-Trenaunay-Weber syndrome is a rare syndrome; unfortunately, very few studies of the connection between hypersplenism, nephrotic syndrome, and Klippel-Trenaunay-Weber syndrome have been published. Case presentation: We report the case of a 40-year-old white man with a typical clinical presentation of Klippel-Trenaunay-Weber syndrome, including "port-wine stains," varicose veins, hypertrophy of lower extremities, and arteriovenous fistula, as well as an unfortunate development of hypersplenism and nephrotic syndrome. Conclusions: This case report described considerable atypical relevance of Klippel-Trenaunay-Weber syndrome and hypersplenism together with nephrotic syndrome. A multidisciplinary approach was made. Unfortunately, hypersplenism is characterized by pancytopenia that suggests splenectomy, whereas nephrotic syndrome is an indication for renal biopsy; the splenectomy and renal biopsy were delayed due to our patient's severe condition. Deeper analysis including study of other patients with Klippel-Trenaunay-Weber syndrome would help us to understand the connection between elevated spleen and liver sizes, nephrotic syndrome, and Klippel-Trenaunay-Weber syndrome. |
Description: | Publisher Copyright: © 2017 The Author(s). |
DOI: | 10.1186/s13256-017-1413-1 |
ISSN: | 1752-1947 |
Appears in Collections: | Research outputs from Pure / Zinātniskās darbības rezultāti no ZDIS Pure |
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Klippel_Trenaunay_Weber_syndrome_withatypical_presentation.pdf | 1.66 MB | Adobe PDF | View/Open |
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