Strömungsgeräusch im Oberbauch

Rose, C. Sainte; Wessel, Anne; Scholz, K-H.; Pankau, R.

doi:10.1007/s001120050586

Cited by 4 publications

(3 citation statements)

References 0 publications

Supporting

Mentioning

Contrasting

Order By: Relevance

“…It remains often unsuccessful [1]. We have reported one successfully dilated RAS [12]. Since there is no causal therapy for increased wall stiness, the antihypertensive treatment is dicult and often ineective.…”

Section: Discussionmentioning

confidence: 97%

“…The pathology shows elastic disorganisation, hypertrophied smooth muscle cells and collagen bundles [8]. The most common locations of vascular stenosis are peripheral pulmonary arteries, aorta ascendens and the aortic arch [12]. Coarctation of the abdominal aorta has also been described [2,11,12], but there is no information about prevalence and features of abnormalities of the abdominal aorta.…”

Section: Introductionmentioning

confidence: 99%

See 1 more Smart Citation

Anomalies of the abdominal aorta in Williams-Beuren syndrome – another cause of arterial hypertension

Rose¹,

Wessel²,

Pankau³

et al. 2001

Eur J Pediatr

View full text Add to dashboard Cite

show abstract

Section: Discussionmentioning

confidence: 97%

Section: Introductionmentioning

confidence: 99%

Anomalies of the abdominal aorta in Williams-Beuren syndrome – another cause of arterial hypertension

Rose¹,

Wessel²,

Pankau³

et al. 2001

Eur J Pediatr

View full text Add to dashboard Cite

show abstract

“…The main symptoms are distinctive facial appearance , psychomotor retardation with friendly behavior [Gosch and Pankau, 1994], typical growth pattern [Pankau et al, 1992;Partsch et al, 1999], and supravalvular aortic stenosis (SVAS) combined with pulmonary artery stenoses [Wessel et al, 1994]. In addition, anomalies of the aorta [Wessel et al, 1997b;Rose et al, 2001] and the renal arteries are common [Pankau et al, 1996;Rose et al, 2000], often leading to arterial hypertension [Wessel et al, 1997a]. Coronary arteries are also involved, probably leading to sudden death in 21 documented cases [Bird et al, 1996;Suares-Mier and Morentin, 1999;Imashuku et al, 2000].…”

Section: Introductionmentioning

confidence: 98%

Risk of sudden death in the Williams–Beuren syndrome

Wessel

Gravenhorst

Buchhorn

et al. 2004

American J of Med Genetics Pt A

118

View full text Add to dashboard Cite

Williams-Beuren syndrome (WBS) is a genetic disorder characterized by a distinctive facial gestalt, mental retardation, mild growth deficiency, and cardiovascular disease. The occurrence of sudden death in the WBS is known from several case reports, but information about the risk of sudden death as derived from the data of a large cohort of patients is lacking. We analyzed the data of 293 WBS patients who had been treated for 43 years at the same two institutions. We thus collected 5,190 patient years without loss to follow-up. During this period ten patients died. Five of them died from: reticulosarcoma (1), after accident (1), heart failure (1), following heart surgery (2). Of the remaining five patients, four died suddenly and one died of unknown cause suggestive of sudden cardiac death. Thus, the incidence of sudden death in our WBS cohort amounts to 1/1,000 patient years. This risk of sudden death is comparable to that following surgery for congenital heart disease, and is 25-100-fold higher compared to the age-matched normal population.

show abstract