Biomedicine and diseases: the Klippel-Trenaunay syndrome, vascular anomalies and vascular morphogenesis

Timur, Ayse Anil; Driscoll, David J.; Wang, Qing Kenneth

doi:10.1007/s00018-005-4523-7

Cited by 81 publications

(72 citation statements)

References 106 publications

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“…It can either be congenital or acquired. The congenital form of angiomatosis may be seen sporadically or accompanying certain types of syndromes such as Klippel-Trenaunay-Weber syndrome, 5) Sneddon's syndrome, 6) or Gorham disease. 7) In these syndromes, the lesions may be observed in any tissue and are clinically extensive, covering large parts of the body in a continuous pattern.…”

Section: Discussionmentioning

confidence: 99%

Primary Left Ventricular Angiomatosis First Description of a Rare Vascular Tumor in the Left Heart

et al. 2006

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SUMMARYA 24 year-old male who presented with palpitations and presyncopal attacks had monomorphic ventricular tachycardia and a well-defined huge mass within the inferoposterior wall of the left ventricle proved by transthoracic echocardiography. The mass was completely resected and shown to be proliferative angiomatosis by histopathologic examination. This is the first reported case of primary left ventricular angiomatosis in the literature. (Int Heart J 2006; 47: 469-474)

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Section: Discussionmentioning

confidence: 99%

Primary Left Ventricular Angiomatosis First Description of a Rare Vascular Tumor in the Left Heart

et al. 2006

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“…However, it is considered that thrombus formation is causally related to disturbance of mobility due to a prolonged bedridden state related to cerebral palsy from young childhood, especially due to paralysis of the lower extremities, which leads to contracture deformities of the lower extremities and concomitant narrowing of the vessels by vascular underdevelopment and immaturity. 22,23) Consequently, it has been suggested that thrombus forms in the conduct veins centrally in patients with SMID. We only detected it mainly in the common femoral and superficial veins, there were no associations with significantly elevated levels of D-dimer caused by reduced vessel volume due to vascular underdevelopment and immaturity.…”

Section: Discussionmentioning

confidence: 99%

Deep Vein Thrombosis in Patients with Severe Motor and Intellectual Disabilities, Especially Diagnosis and Prevention of Recurrence for Chronic Thrombosis—Serial Changes of Sonography and D-Dimer

Ohmori

Kanaoka²,

Murata

et al. 2015

Annals of Vascular Diseases

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This article is a translation of Jpn J Phlebol 2014; 25: 34-42. Most patients with severe motor and intellectual disabilities (SMID) have restricted mobility capability and have been bedridden for long periods because of paralysis of the extremities caused by abnormal muscular tonicity due to cerebral palsy and developmental disabilities. Such patients are associated with a high risk of complications like deep vein thrombosis (DVT). Here, we report twelve patients (42.9%) with DVT among 28 patients with SMID during prolonged bed rest. However, we did not detect thrombosis in the soleal veins, finding it mostly in the femoral and common femoral veins. We applied anticoagulant therapy (warfarin), and carefully followed up the cases with DVT, regulating the warfarin dosage at prothrombin time-international normalized ratio (PT-INR) values around two to prevent recurrence of chronic thrombosis. Regarding laboratory data for the coagulation system, there were no cases above 5 µg/ml for the D-dimer and there were significant differences between the DVT and non-DVT groups in the D-dimer levels. The plasma levels of D-dimer in patients with DVT diminished to less than 1.0 µg/ml after warfarin treatment. Concerning sudden death (4.2%) in patients with SMID, we have to be very careful of the possibility of pulmonary thromboembolism due to DVT. Therefore, we should consider the particularity of the underdeveloped vascular system from underlying diseases for the evaluation of DVT. A detailed study of DVT as a vascular complication is very important for the smooth medical care of SMID, and serial assessment of compression Doppler ultrasonography of the lower extremities, as a noninvasive examination and measurement of D-dimer, is very helpful. (This article is a translation of Jpn J Phlebol 2014; 25: 34-42.)

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“…There is some evidence that it may be associated with a translocation at (8;14) (q22.3;q13). Some researchers have suggested that VG5Q (Vasculogenesis gene on 5q protein) has an association (7)(8)(9)(10)(11).…”

Section: Discussionmentioning

confidence: 99%

Klippel-Trenaunay Syndrome Accompanied by the Findings of Scoliosis and Spinal Nerve Root Compression Due to AVM

Özkan¹,

Şanal²

2015

Turk J Phys Med Rehab

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Klippel-Trenaunay Syndrome (KTS) is one of the rare congenital hyperplasia syndromes generally involving a single extremity characterized by port-wine-stain-type cutaneous vascular malformations, bone and soft tissue hypertrophy, and varicose veins. It was described by the French doctors Maurice Klippel and Paul Trenaunay for the first time in 1900s. Later, the investigators Parkes and Weber described the accompanying arterial abnormalities, arteriovenous fistula, and arterial aneurysms. The disease affects men and women at an equal rate and is not limited to any racial groups. The purpose of this article is to introduce an extremely rare case of KTS with three hypertrophic extremities, presenting with the findings of spinal nerve root compression due to arteriovenous malformation located in the lumbar spinal canal with a literature review.

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Biomedicine and diseases: the Klippel-Trenaunay syndrome, vascular anomalies and vascular morphogenesis

Cited by 81 publications

References 106 publications

Primary Left Ventricular Angiomatosis First Description of a Rare Vascular Tumor in the Left Heart

Primary Left Ventricular Angiomatosis First Description of a Rare Vascular Tumor in the Left Heart

Deep Vein Thrombosis in Patients with Severe Motor and Intellectual Disabilities, Especially Diagnosis and Prevention of Recurrence for Chronic Thrombosis—Serial Changes of Sonography and D-Dimer

Klippel-Trenaunay Syndrome Accompanied by the Findings of Scoliosis and Spinal Nerve Root Compression Due to AVM

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