Classification of endonasal HHT lesions using digital microscopy
| Autor: | Henrik Schinke, Mattis Bertlich, A Schneider, Fatemeh Kashani, Martin Canis, Frank Haubner, BG Weiss |
|---|---|
| Jazyk: | angličtina |
| Rok vydání: | 2021 |
| Předmět: |
Vascular Endothelial Growth Factor A
medicine.medical_specialty congenital hereditary and neonatal diseases and abnormalities Visual analogue scale VEGF receptors Mucocutaneous zone Laser Clinical manifestation 030204 cardiovascular system & hematology HHT 03 medical and health sciences 0302 clinical medicine medicine otorhinolaryngologic diseases Humans Pharmacology (medical) 030223 otorhinolaryngology Telangiectasia Morbus Osler Genetics (clinical) Hereditary haemorrhagic telangiectasia Microscopy biology business.industry Research Routine laboratory General Medicine Epistaxis Digital microscopy biology.protein Quality of Life Medicine Telangiectasia Hereditary Hemorrhagic Radiology medicine.symptom business |
| Zdroj: | Orphanet Journal of Rare Diseases, Vol 16, Iss 1, Pp 1-8 (2021) Orphanet Journal of Rare Diseases |
| ISSN: | 1750-1172 |
| Popis: | Background Recurrent spontaneous epistaxis is the most common clinical manifestation and the most debilitating symptom in hereditary haemorrhagic telangiectasia (HHT) patients. To this date, there exist only a classification of HHT patients by different genetic mutations. There is no standard classification for the mucocutaneous endonasal manifestations of HHT. The aim of the present study was to document the variety of endonasal HHT lesions using digital microscopy and to propose a clinical classification. Methods We recorded the endonasal HHT lesions of 28 patients using a digital microscope. We reconstructed the 3D images und videos recorded by digital microscope afterwards and classified the endonasal lesions of HHT in two classes: Grade A, presence of only flat telangiectasias in the mucosa level and Grade B, (additional) presence of raised berry or wart-like telangiectasia spots. We investigated also Haemoglobin level by routine laboratory procedures, plasma VEGF level by ELISA, Severity of epistaxis by epistaxis severity score (ESS) and quality of life by a linear visual analogue scale (VAS). Results We found a higher quality of life and a lower severity of epistaxis in Grade A patients in comparison to Grade B patients. No difference in plasma VEGF level and in Haemoglobin between Grad A patients and Grade B patients could be detected. Plasma VEGF levels showed no gender specific differences. It could also not be correlated to the extranasal manifestation. Conclusion The classification for endonasal manifestation of HHT proposed in this study indicates severity of epistaxis und quality of life. Digital microscopy with the ability of 3D reconstruction of images presents a useful tool for such classifications. The classification of endonasal HHT lesions using digital microscopy allows to evaluate the dynamic of HHT lesions in the course of time independent of examiner. This allows also to evaluate the efficacy of the different treatment modalities by dynamic of HHT lesions. Moreover digital microscopy is very beneficial in academic teaching of rare diseases. |
| Databáze: | OpenAIRE |
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