Hereditäre hämorrhagische Teleangiektasie (Morbus Osler)

Geisthoff, Urban W.; Schneider, Gerald; J, Fischinger; Plinkert, Peter K.

doi:10.1007/s001060100537

Cited by 32 publications

(17 citation statements)

References 36 publications

(59 reference statements)

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“…Hereditary haemorrhagic telangiectasia (HHT), also known as Osler-Weber-Rendu disease, is an autosomal dominant disease with penetrance that is incomplete but increases with age [1]. The prevalence is assumed to be approximately 1:10,000 [2].…”

Section: Introductionmentioning

confidence: 99%

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A retrospective analysis of low dose, intranasal injected bevacizumab (Avastin) in hereditary haemorrhagic telangiectasia

Rohrmeier

Sachs²,

Kuehnel

2011

Eur Arch Otorhinolaryngol

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The constantly recurring epistaxis means a great reduction of quality of life for patients with hereditary haemorrhagic telangiectasia (HHT). As yet, an ideal treatment has not been found. Vascular endothelial growth factor (VEGF) has been described as a possible new therapy. In particular, the success of submucosal doses <100 mg has not been analysed before. We injected bevacizumab (Avastin) submucosally in addition to Nd:YAG laser therapy. Doses <7.5 mg were used. To investigate the effect of these additional injections in comparison to laser therapy alone, a retrospective analysis was done. For this purpose a standardized patient questionnaire was completed, which included recording the patients' Epistaxis Severity Score (ESS) before and after the antibody treatment. Besides, patient files were analysed to collect objective data like haemoglobin levels and the number of blood transfusions needed. Data for eleven patients could be analysed. A significant improvement in the ESS resulting from additional bevacizumab therapy was observed (p < 0.01). In particular, the frequency of epistaxis (p = 0.011), duration of epistaxis (p < 0.01), severity of epistaxis (p < 0.01) and the need for acute medical treatment (p = 0.014) decreased significantly. The haemoglobin levels increased significantly (p = 0.011) and the number of blood transfusions declined. There were no side effects caused by the antibody treatment. Additional injections of a low dose of bevacizumab seem to be superior to Nd:YAG laser therapy alone. These results justify further studies.

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Section: Introductionmentioning

confidence: 99%

“…These comprise recurrent, spontaneous epistaxis, the appearance of telangiectasias, the presence of visceral manifestations in the form of haemorrhages or AV malformations and a positive family history. A diagnosis of HHT can be regarded as conWrmed if at least three of these four criteria are fulWlled [1,4].…”

Section: Introductionmentioning

confidence: 99%

A retrospective analysis of low dose, intranasal injected bevacizumab (Avastin) in hereditary haemorrhagic telangiectasia

Rohrmeier

Sachs²,

Kuehnel

2011

Eur Arch Otorhinolaryngol

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“…2 Die auf der klinischen Symptomatik basierenden Cura ç ao-Kriterien zur Diagnosestellung der HHT [7] . In Klammern sind zus ä tzlich die Inzidenzen der wichtigsten Manifestationen aufgef ü hrt [1,8] Tab. 3 Ü bersicht ü ber eine Auswahl an Therapieoptionen zur Behandlung des Nasenblutens beim Morbus Osler unter Gebrauch eines didaktischen mechanistischen Modells zur Genese der Epistaxis [2] .…”

Section: Wie Kann Man Fehldiagnosen Und -Therapien Von Seltenen Erkraunclassified

Hereditäre hämorrhagische Teleangiektasie (Morbus Rendu-Osler-Weber) als Beispiel einer seltenen Erkrankung des HNO-Fachgebietes

Geisthoff

Maune²,

Schneider³

2011

Laryngo-Rhino-Otol

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Diagnosis and treatment of rare diseases are difficult. Hereditary hemorrhagic telangiectasia (HHT, Rendu-Osler-Weber disorder) is a relatively common rare disease. Because epistaxis is the most frequent manifestation of this disease, affected individuals often approach otorhinolaryngologists first. The latter ones often have a key position for early diagnosis and screening for visceral involvement which can prevent life-threatening complications. Treatment of epistaxis can improve quality of life of affected persons. Diagnosis and treatment of rare diseases can be complex and it can be very helpful to use information resources to ensure a correct management. Various possibilities to access these resources are presented in general and in relation to HHT.

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“…Hepatic involvement is encountered in about 8-31% of the patients with HHT, of which about half may be symptomatic [1][2][3][4][5][6][7]. Whereas some symptomatic patients can be managed conservatively, the treating physician is often in a dilemma when confronted with a patient whose symptoms are refractory to all forms of conservative therapy.…”

Section: Introductionmentioning

confidence: 99%

Hepatic artery embolization for treatment of patients with hereditary hemorrhagic telangiectasia and symptomatic hepatic vascular malformations

et al. 2004

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At present there is no established therapy for treating patients with hereditary hemorrhagic telangiectasia (HHT) and symptomatic hepatic involvement. We present the results of a prospective study with 15 consecutive patients who were treated with staged hepatic artery embolization (HAE). Branches of the hepatic artery were selectively catheterized and embolized in stages using polyvinyl alcohol particles (PVA) and platinum microcoils or steel macrocoils. Prophylactic antibiotics, analgesics and anti-emetics were administered after every embolization. Clinical symptomatology and cardiac output were assessed before and after therapy as well as at the end of follow-up (median 28 months; range 10-136 months). Five patients had abdominal pain and four patients had symptoms of portal hypertension. The cardiac output was raised in all patients, with cardiac failure being present in 11 patients. After treatment, pain resolved in all five patients, and portal hypertension improved in two of the four patients. The mean cardiac output decreased significantly ( P<0.001) from 12.57+/-3.27 l/min pre-treatment to 8.36+/-2.60 l/min at the end of follow-up. Symptoms arising from cardiac failure resolved or improved markedly in all but one patient. Cholangitis and/or cholecystitis occurred in three patients of whom two required a cholecystectomy. One patient with pre-existent hepatic cirrhosis died as a complication of the procedure. Staged HAE yields long-term relief of clinical symptoms in patients with HHT and hepatic involvement. Patients with pre-existing hepatic cirrhosis may be poor candidates for HAE.

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Hereditäre hämorrhagische Teleangiektasie (Morbus Osler)

Abstract: Physicians should be especially aware of gastrointestinal telangiectases, arteriovenous malformations (AVM) of the lungs, liver, and brain. A screening is recommended at least for pulmonary AVM.

Cited by 32 publications

References 36 publications

A retrospective analysis of low dose, intranasal injected bevacizumab (Avastin) in hereditary haemorrhagic telangiectasia

A retrospective analysis of low dose, intranasal injected bevacizumab (Avastin) in hereditary haemorrhagic telangiectasia

Hereditäre hämorrhagische Teleangiektasie (Morbus Rendu-Osler-Weber) als Beispiel einer seltenen Erkrankung des HNO-Fachgebietes

Hepatic artery embolization for treatment of patients with hereditary hemorrhagic telangiectasia and symptomatic hepatic vascular malformations

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