Background: The olfactory and gustatory functions of hereditary hemorrhagic telangiectasia (HHT) patients have not been documented by validated tests. Disorders of the nasal/oral cavity may interfere with the olfactory and gustatory functions. Fifty-four HHT patients were investigated by smell/taste tests.
Methods: HHT patients provided subjective ratings in areas such as ability to perceive smell/taste. "Sniffin' Sticks" were used for smell tests, and taste strips were used for taste tests.
Results: HHT patients rated their subjective olfactory and gustatory function on a visual analog scale from 0 (none) to 100 (high) as 65.3 ± 27.7 and 68.1 ± 25.1, respectively. Comparison of smell test results of HHT patients with normative data of sex- and age-matched controls from Hummel et al. revealed that HHT patients had lower threshold values, whereas there was no difference in identification and discrimination values. HHT patients were hyposmic. In the case of taste qualities, all values (sweet, sour, salty, and bitter) in HHT patients were lower than those in normative data of Mueller et al. However, HHT patients were not hypogeusic. The duration of disease, extranasal manifestation, and treatments did not significantly correlate with smell/taste test values.
Conclusion: Compared with healthy people, HHT patients exhibit reduced olfactory and gustatory function; however, HHT patients are hyposmic and not hypogeusic. This chemosensory deficit may highlight an early sign of disease and has no correlation with disease severity. HHT patients should be informed about these potential disease manifestations, thus enabling them to improve their quality of life.
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http://dx.doi.org/10.2500/ajra.2012.26.3824 | DOI Listing |
Acta Neurol Belg
January 2025
Intensive Care Department, Cliniques Universitaire Saint-Luc (CUSL), Université Catholique de Louvain (UCL), Brussels, Belgium.
Osler-Weber-Rendu syndrome, or hereditary hemorrhagic telangiectasia (HHT), is a rare vascular disorder characterized by arteriovenous malformations (AVMs) in various organs, including the lungs. Pulmonary AVMs (PAVMs) are especially worrisome due to their potential to form right-to-left shunts, resulting in life-threatening complications such as paradoxical embolism and stroke . We present a case of fatal air embolism in a young patient with a known history of HHT and recurring hemoptysis.
View Article and Find Full Text PDFPediatr Pulmonol
January 2025
Department of Pulmonology, St. Antonius Hospital, Nieuwegein, the Netherlands.
Introduction: Hereditary hemorrhagic telangiectasia (HHT) is an autosomal dominant vascular disease and screening to detect pulmonary arteriovenous malformations (PAVMs) is important to prevent complications. In adults, transthoracic contrast echocardiogram (TTCE) is used to screen PAVMs. In children, a conservative screening method seems to be sufficient to rule out major PAVMs and prevent them from PAVM-related complications.
View Article and Find Full Text PDFJ Clin Med
December 2024
Clinic of Diagnostic and Interventional Radiology, Saarland University Medical Center, 66421 Homburg, Germany.
The aim of this study was to evaluate patients with hereditary hemorrhagic telangiectasia (HHT) for the potential reperfusion of pulmonary arteriovenous malformations (PAVM) treated by catheter embolization using coils or embolization plugs and to analyze causes of possible reperfusion in order to further improve treatment. This retrospective study analyzed the data of 345 patients who underwent screening for pulmonary arteriovenous malformations in cases of suspected or confirmed HHT (Osler's disease). Of these, 118 patients with PAVM that underwent catheter embolization and had at least one follow-up study were included in our study and evaluated for potential reperfusion.
View Article and Find Full Text PDFBiomedicines
December 2024
Division of Hematology, Department of Internal Medicine, University Hospital Dubrava and Medical School, University of Zagreb, 10000 Zagreb, Croatia.
Obinutuzumab was approved for front-line treatment of chronic lymphocytic leukemia in combination with chlorambucil pulses administered every 2 wks. Alternative schedules of chlorambucil enable the administration of higher total chlorambucil doses, and have better antileukemia activity. So far, evidence on the feasibility of combining obinutuzumab with alternative chlorambucil schedules is lacking.
View Article and Find Full Text PDFDiagnostics (Basel)
December 2024
Pneumology Department, General University Hospital of Valencia, 46014 Valencia, Spain.
: Angiogenesis is involved in the pathogenesis of hereditary hemorrhagic telangiectasia (HHT). VEGF, ANG2, TGFβ1, and ENG are the most studied angiogenic factors, but their clinical significance in blood samples is still not completely defined. The genetic study of HHT mutations is the test of choice for diagnosing the disease, but this route is expensive, and the causative mutation is not found in up to 10% of cases.
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