a Record control number 000284910500005

a ISSN 0301-1623

a Original cataloging agency NEU GrandLibrary

a Language code of text/sound track or separate title eng

a Classification number WJ 300

b Item number .G57 2010

a Personal name Turkmen, Mehmet.

h Medium Electronic Document

a Title A girl of Klippel-Trenaunay Weber syndrome coexistence of recurrent bloody vaginal discharge.

c Statement of responsibility, etc Mehmet Turkmen, Salih Kavukçu, Handan Çakmakci, Alper Soylu, Sebnem Aktan, Yeliz Çağan.

b Name of publisher, distributor, etc Springer,

c Date of publication, distribution, etc 2010.

a Summary, etc Klippel-Trenaunay Weber syndrome (KTWS) is the coexistence of capillary vascular malformations, varicose veins, dilated arteries and arteriovenous fistulas, soft tissue and/or bone hypertrophy. We present a girl of KTWS associated with hypertrophied left kidney, enlargement in venous structures of the left kidney, recurrent bloody vaginal discharge and angiokeratomas. A 6-year-old girl was admitted to our department with complaints of recurrent bloody vaginal discharge and swelling in the left inguinal region. Physical examination revealed hypertrophy of the left lower extremity. Vaginoscopy and cystoscopy revealed normal findings. Abdominal ultrasound revealed an enlarged left kidney. Enlargement in the venous structures of the hypertrophied left kidney was detected by abdominal magnetic resonance imaging, and arteriovenous fistulas were revealed by conventional angiography. The patient was diagnosed KTWS. Ophthalmic examination was normal. Galactosidase A (GLA) level was found to be at the lower limit of the normal range, and mutation was not detected in the GLA gene. In conclusion, we have emphasized that the girls with recurrent vaginal discharge might be KTWS. Angiokeratoma may be considered as a dermatological finding of KTWS. KTWS may also have enlarged kidney and enlargement in venous structures of the kidney in hypertrophied side.

a Topical term or geographic name as entry element Urology.

a Topical term or geographic name as entry element Near East University Article

a Topical term or geographic name as entry element Yakın Doğu Üniversitesi Makale

a Topical term or geographic name as entry element Urology & Nephrology

a Main entry heading Internatıonal Urology And Nephrology

g Relationship information SEP 2010, Vol 43 Issue 3, p575-578

x International Standard Serial Number 03011623

u Uniform Resource Identifier http://library.neu.edu.tr:2048/login?url=http://dx.doi.org/10.1007/s11255-009-9640-z