tetano
Editor, Senior Moderator
Orphanet J Rare Dis
. 2021 Sep 8;16(1):378.
doi: 10.1186/s13023-021-02000-2.
Characterization of epidemiological distribution and outcome of COVID-19 in patients with hereditary hemorrhagic telangiectasia: a nationwide retrospective multi-centre study during first wave in Italy
Patrizia Suppressa[SUP] 1 [/SUP], Fabio Pagella[SUP] 2 3 [/SUP], Gennaro Mariano Lenato[SUP] 1 [/SUP], Eleonora Gaetani[SUP] 4 [/SUP], Ilaria Serio[SUP] 5 [/SUP], Maristella Salvatora Masala[SUP] 6 [/SUP], Giuseppe Spinozzi[SUP] 2 [/SUP], Roberta Lizzio[SUP] 2 [/SUP], Elina Matti[SUP] 2 [/SUP], Annalisa De Silvestri[SUP] 7 [/SUP], Giulio Cesare Passali[SUP] 8 [/SUP], Maria Aguglia[SUP] 9 10 [/SUP], Claudia Crocione[SUP] 10 [/SUP], Carlo Sabbà[SUP] 11 [/SUP]
Affiliations
Abstract
Background: Coronavirus Disease 2019 (COVID-19) continues to have a devastating impact across the world. A number of pre-existing common clinical conditions were reported to represent risk factors for more severe COVID-19 outcomes. Hereditary Hemorrhagic Telangiectasia (HHT) is a rare vascular heritable disorders, characterized by complications secondary to visceral Arterio-Venous Malformations. The impact of HHT, as well as for many Rare Diseases (RDs) on infection susceptibility profile and clinical adverse outcome risk is an unresolved issue.
Objectives: The main objectives were: to assess the clinical features and outcomes of HHT patients infected with COVID-19; to compare the relative infection risk in these patients with the Italian general population throughout the first pandemic wave; to investigate the factors potentially associated with severe COVID-19 outcome in HHT patients, and the possible impact of COVID-19 infection on HHT-related symptoms/complications. Finally, we aimed to estimate how the lockdown-associated wearing of personal protective equipment/individual protection devices could affect HHT-related telangiectasia bleeding frequency.
Methods: The study is a nation-wide questionnaire-based survey, with a multi-Center retrospective cross-sectional design, addressed to the whole Italian HHT population. COVID-19 cases, occurring throughout the first pandemic wave, were collected by a questionnaire-based semi-structured interview. Only the cases ascertained by laboratory confirmation (molecular/serological) were included for epidemiological estimates. Information concerning eventual SarS-Cov-2 infection, as well as regarding HHT-related manifestations and HHT-unrelated co-morbidities were collected by the questionnaire. Prevalence data were compared to Italian general population in the same period.
Results: The survey disclosed 9/296 (3.04%) COVID-19 cases, 8/9 of them being resident in Lombardy, the main epidemic epicenter. Pneumonia was reported by 4/9 patients, which prompted hospital admission and intensive care management in 2 cases. No fatal outcome was recorded. After careful refinement of epidemiological analysis, the survey evidenced overlapping infection risk in HHT compared to general population.
Conclusions: COVID-19 infection profile parallels geographical distribution of epidemic foci. COVID-19 in HHT patients can lead to highly variable clinical profile, likely overlapping with that of general population. The HHT disease does not seem to involve a different approach in terms of hospital admission and access to intensive care with respect to general population.
Keywords: COVID-19; Hereditary hemorrhagic telangiectasia; PANDEMICS 2020; Rare diseases; SARS-CoV-2 infection.
. 2021 Sep 8;16(1):378.
doi: 10.1186/s13023-021-02000-2.
Characterization of epidemiological distribution and outcome of COVID-19 in patients with hereditary hemorrhagic telangiectasia: a nationwide retrospective multi-centre study during first wave in Italy
Patrizia Suppressa[SUP] 1 [/SUP], Fabio Pagella[SUP] 2 3 [/SUP], Gennaro Mariano Lenato[SUP] 1 [/SUP], Eleonora Gaetani[SUP] 4 [/SUP], Ilaria Serio[SUP] 5 [/SUP], Maristella Salvatora Masala[SUP] 6 [/SUP], Giuseppe Spinozzi[SUP] 2 [/SUP], Roberta Lizzio[SUP] 2 [/SUP], Elina Matti[SUP] 2 [/SUP], Annalisa De Silvestri[SUP] 7 [/SUP], Giulio Cesare Passali[SUP] 8 [/SUP], Maria Aguglia[SUP] 9 10 [/SUP], Claudia Crocione[SUP] 10 [/SUP], Carlo Sabbà[SUP] 11 [/SUP]
Affiliations
- PMID: 34496900
- DOI: 10.1186/s13023-021-02000-2
Abstract
Background: Coronavirus Disease 2019 (COVID-19) continues to have a devastating impact across the world. A number of pre-existing common clinical conditions were reported to represent risk factors for more severe COVID-19 outcomes. Hereditary Hemorrhagic Telangiectasia (HHT) is a rare vascular heritable disorders, characterized by complications secondary to visceral Arterio-Venous Malformations. The impact of HHT, as well as for many Rare Diseases (RDs) on infection susceptibility profile and clinical adverse outcome risk is an unresolved issue.
Objectives: The main objectives were: to assess the clinical features and outcomes of HHT patients infected with COVID-19; to compare the relative infection risk in these patients with the Italian general population throughout the first pandemic wave; to investigate the factors potentially associated with severe COVID-19 outcome in HHT patients, and the possible impact of COVID-19 infection on HHT-related symptoms/complications. Finally, we aimed to estimate how the lockdown-associated wearing of personal protective equipment/individual protection devices could affect HHT-related telangiectasia bleeding frequency.
Methods: The study is a nation-wide questionnaire-based survey, with a multi-Center retrospective cross-sectional design, addressed to the whole Italian HHT population. COVID-19 cases, occurring throughout the first pandemic wave, were collected by a questionnaire-based semi-structured interview. Only the cases ascertained by laboratory confirmation (molecular/serological) were included for epidemiological estimates. Information concerning eventual SarS-Cov-2 infection, as well as regarding HHT-related manifestations and HHT-unrelated co-morbidities were collected by the questionnaire. Prevalence data were compared to Italian general population in the same period.
Results: The survey disclosed 9/296 (3.04%) COVID-19 cases, 8/9 of them being resident in Lombardy, the main epidemic epicenter. Pneumonia was reported by 4/9 patients, which prompted hospital admission and intensive care management in 2 cases. No fatal outcome was recorded. After careful refinement of epidemiological analysis, the survey evidenced overlapping infection risk in HHT compared to general population.
Conclusions: COVID-19 infection profile parallels geographical distribution of epidemic foci. COVID-19 in HHT patients can lead to highly variable clinical profile, likely overlapping with that of general population. The HHT disease does not seem to involve a different approach in terms of hospital admission and access to intensive care with respect to general population.
Keywords: COVID-19; Hereditary hemorrhagic telangiectasia; PANDEMICS 2020; Rare diseases; SARS-CoV-2 infection.