Sinan Ahmed Dheyauldeen Dheyauldeen

• Dheyauldeen, Sinan Ahmed Dheyauldeen; Heimdal, Ketil Riddervold; Osnes, Terje; Akre, Harriet & Olsson, Lars H. (2024). Choanal atresia: A review of surgical outcomes in a tertiary medical center. World Journal of Otorhinolaryngology - Head and Neck Surgery (WJOHNS). ISSN 2095-8811. doi: 10.1002/wjo2.196. Full text in Research Archive Show summary

  Introduction Choanal atresia (CA) is a congenital narrowing or obliteration of the posterior nasal aperture. The condition is rare with an incidence of approximately 1 in 5000 to 9000 live births. Objective The aim of this work was to assess the results of treating this condition at the Otolaryngology Department in Oslo University Hospital-Rikshospitalet. Methods Retrospective review of patients treated at Oslo University Hospital-Rikshospitalet between 2005 and 2015 were performed. The review consisted of surgical techniques and outcomes, types of atresia, uses of stent and mitomycin C, age at operation, laterality of atresia, and associated anomalies. Results Thirty-one patients with CA were referred to Oslo University Hospital-Rikshospitalet during the mentioned observation period. Nine (29%) of the cases had bilateral CA and 22 (71%) had unilateral CA. Ten patients (32%) had bony atresia, 12 (39%) had membranous and eight (26%) had mixed type of atresia. Twenty-one patients (68%) needed revision surgery because of complete or partial restenosis after primary surgery. Stents were applied in 16 cases (52%) of patients and Mitomycin C was only used in the primary surgery of three patients. In the bilateral group, eight of the nine patients were operated on within the first 9 days of life. Sixteen patients (45%) had associated anomalies. Conclusions According to the observations in our sample, the restenosis problem is expected more and may need more revisions in bilateral CA than the unilateral type. The presence of other anomalies is associated with higher incidence of restenosis. Stenting is not essential in preventing restenosis. Key point Risk factors of restenosis in choanal atresia.

• Goutaki, Myrofora; Lam, Yin Ting; Anagiotos, Andreas; Armengot, Miguel; Burgess, Andrea & Campbell, Raewyn [Show all 25 contributors for this article] (2024). Definition of sinonasal and otological exacerbation in patients with primary ciliary dyskinesia: an expert consensus. European Respiratory Journal Open Research (ERJ Open Research). 10(6). doi: 10.1183/23120541.00218-2024. Full text in Research Archive Show summary

  Background Recurrent infections of the nose, sinuses and ears are common problems for people with primary ciliary dyskinesia. While pulmonary exacerbations in primary ciliary dyskinesia are defined, there is no definition for ear-nose-throat exacerbations, a potential outcome for research and clinical trials. Methods We set up an expert panel of 24 ear-nose-throat specialists, respiratory physicians, other healthcare professionals and patients to develop consensus definitions of sinonasal and otological exacerbations in children and adults with primary ciliary dyskinesia for research settings. We reviewed the literature and used a modified Delphi approach with four electronic surveys. Results Definitions for both sinonasal and otological exacerbations are based on a combination of major and minor criteria, requiring three major or two major and at least two minor criteria each. Major criteria for a sinonasal exacerbation are 1) reported acute increase in nasal discharge or change in colour, 2) reported acute pain or sensitivity in the sinus regions and 3) mucopurulent discharge on examination. Minor criteria include reported symptoms, examination signs, doctor's decision to treat and improvement after at least 14 days. Major criteria for the otological exacerbation are 1) reported acute ear pain or sensitivity, 2) reported acute ear discharge, 3) ear discharge on examination and 4) signs of otitis media in otoscopy. Minor criteria are reported acute hearing problems, signs of acute complication, and doctor's decision to treat. Conclusion These definitions might offer a useful outcome measure for primary ciliary dyskinesia research in different settings. They should be validated in future studies and trials together with other potential outcomes, to assess their usability.

• Lam, Yin Ting; Papon, Jean-François; Alexandru, Mihaela; Anagiotos, Andreas; Armengot, Miguel & Boon, Mieke [Show all 31 contributors for this article] (2024). Association between upper and lower respiratory disease among patients with primary ciliary dyskinesia: an international study. European Respiratory Journal Open Research (ERJ Open Research). 10(2). doi: 10.1183/23120541.00932-2023. Full text in Research Archive Show summary

  Introduction Nearly all patients with primary ciliary dyskinesia (PCD) report ear–nose–throat (ENT) symptoms. However, scarce evidence exists about how ENT symptoms relate to pulmonary disease in PCD. We explored possible associations between upper and lower respiratory disease among patients with PCD in a multicentre study. Methods We included patients from the ENT Prospective International Cohort (EPIC-PCD). We studied associations of several reported ENT symptoms and chronic rhinosinusitis (defined using patient-reported information and examination findings) with reported sputum production and shortness of breath, using ordinal logistic regression. In a subgroup with available lung function results, we used linear regression to study associations of chronic rhinosinusitis and forced expiratory volume in 1 s (FEV1) accounting for relevant factors. Results We included 457 patients (median age 15 years, interquartile range 10–24 years; 54% males). Shortness of breath associated with reported nasal symptoms and ear pain of any frequency, often or daily hearing problems, headache when bending down (OR 2.1, 95% CI 1.29–3.54) and chronic rhinosinusitis (OR 2.3, 95% CI 1.57–3.38) regardless of polyp presence. Sputum production associated with daily reported nasal (OR 2.2, 95% CI 1.20–4.09) and hearing (OR 2.0, 95% CI 1.10–3.64) problems and chronic rhinosinusitis (OR 2.1, 95% CI 1.48–3.07). We did not find any association between chronic rhinosinusitis and FEV1. Conclusion Reported upper airway symptoms and signs of chronic rhinosinusitis associated with reported pulmonary symptoms, but not with lung function. Our results emphasise the assessment and management of upper and lower respiratory disease as a common, interdependent entity among patients with PCD.

• Lam, Yin Ting; Papon, Jean-François; Alexandru, Mihaela; Anagiotos, Andreas; Armengot, Miguel & Boon, Mieke [Show all 30 contributors for this article] (2023). Sinonasal disease among patients with primary ciliary dyskinesia: An international study. European Respiratory Journal Open Research (ERJ Open Research). 9(3). doi: 10.1183/23120541.00701-2022. Full text in Research Archive Show summary

  Background Sinonasal symptoms are a common feature of primary ciliary dyskinesia (PCD); however, literature about their severity and frequency, particularly during the life course, is scarce. Using baseline data from the Ear, nose and throat (ENT) Prospective International Cohort of PCD patients, we describe sinonasal disease in PCD. Methods We included participants who had a routine sinonasal examination during which they completed a symptoms questionnaire. We compared frequency of reported symptoms and examination findings among children and adults, and identified characteristics potentially associated with higher risk of sinonasal disease using ordinal regression. Results 12 centres contributed 384 participants; median age was 16 years (IQR 9–22), and 54% were male. Chronic nasal problems were the most common feature, reported by 341 (89%). More adults (33; 24%) than children (10; 4%) described hyposmia. Quality of life was moderately affected by rhinosinusitis among 136 participants with completed SNOT-22 questionnaires (median score 31; IQR 23–45). Examinations revealed nasal polyps among 51 of 345 participants (15%) and hypertrophic inferior nasal turbinates among 127 of 341 participants (37%). Facial pain was detected in 50 of 342 participants (15%). Nasal polyps, hypertrophic turbinates, deviated septum and facial pain were found more commonly in adults than children. The only characteristic associated with higher risk of sinonasal disease was age 10 years and older. Conclusions Based on our findings, regular sinonasal examinations are relevant for patients with PCD of all ages. There is a need for improved management of sinonasal disease supported by evidence-based guidelines.

• Lam, Yin Ting; Papon, Jean-François; Alexandru, Mihaela; Anagiotos, Andreas; Armengot, Miguel & Boon, Mieke [Show all 30 contributors for this article] (2023). Lack of Correlation of Sinonasal and Otologic Reported Symptoms With Objective Measurements Among Patients With Primary Ciliary Dyskinesia: An International Study. Clinical and Experimental Otorhinolaryngology. ISSN 1976-8710. 16(4), p. 407–412. doi: 10.21053/ceo.2023.01130. Full text in Research Archive
• Jørgensen, Ole Jakob; Steineger, Johan Edvard; Hillarp, Andreas; Wåland, Erik Pareli; Holme, Pål Andre & Heimdal, Ketil Riddervold [Show all 7 contributors for this article] (2023). Elevated FVIII levels in hereditary hemorrhagic telangiectasia: Implications for clinical management. Laryngoscope Investigative Otolaryngology (LIO). 9(1). doi: 10.1002/lio2.1196. Full text in Research Archive Show summary

  Objectives: The objective of this study was twofold: to determine the prevalence of arterial and venous thromboembolic events in the Norwegian Hereditary Hemor- rhagic Telangiectasia (HHT) population, and to explore potential factors linked to such events, with particular emphasis on FVIII. Methods: Patients with an HHT diagnosis attending the Otorhinolaryngology Department at Oslo University Hospital—Rikshospitalet were included consecutively between April 2021 and November 2022. We recorded the participants' medical his- tory with an emphasis on thromboembolic events. Measurements of blood constitu- ents, including FVIII, FIX, vWF, hemoglobin, iron, ferritin, and CRP were performed. Results: One hundred and thirty-four patients were included in the study. The total prevalence of thromboembolic events among the participants was 23.1%. FVIII levels were high (>150 IU/dL) in the majority of HHT patients (n = 84) (68.3%) and were significantly associated with thromboembolic events (p < .001), as was age. Of the patients with high FVIII levels, 28 (33%) had experienced a thromboembolic event. Furthermore, FVIII levels were measured consecutively in 51 patients and were found to fluctuate above or below 150 IU/dL in 25% of these cases. Conclusion: Thromboembolic events are highly prevalent in the Norwegian HHT popula- tion and are significantly associated with FVIII levels. FVIII levels can fluctuate, and measure- ments should be repeated in HHT patients to assess the risk of thromboembolic events. Level of Evidence: 4.

• Goutaki, Myrofora; Lam, Yin Ting; Alexandru, Mihaela; Anagiotos, Andreas; Armengot, Miguel & Boon, Mieke [Show all 30 contributors for this article] (2023). Characteristics of Otologic Disease among Patients with Primary Ciliary Dyskinesia. JAMA Otolaryngology - Head and Neck Surgery. ISSN 2168-6181. 149(7), p. 587–596. doi: 10.1001/jamaoto.2023.0841. Full text in Research Archive
• Jorgensen, Ole Jakob; Steineger, Johan Edvard; Bachmann-Harildstad, G. & Dheyauldeen, S. (2021). A comparative study of two grading systems for epistaxis in hereditary haemorrhagic telangiectasia. Rhinology. ISSN 0300-0729. 59(2), p. 212–218. doi: 10.4193/Rhin20.540. Full text in Research Archive
• Hui, David; Johansen, Edvard; Akre, Harriet; Osnes, Terje & Dheyauldeen, Sinan (2020). Øre-nese-hals-leger og sars-CoV-2-viruset. Tidsskrift for Den norske legeforening (Tidsskriftet). ISSN 0029-2001. 140(10). doi: 10.4045/tidsskr.20.0423. Full text in Research Archive
• Steineger, Johan Edvard; Geirdal, Amy Østertun; Osnes, Terje; Heimdal, Ketil Riddervold & Dheyauldeen, Sinan (2019). Intranasal bevacizumab injections improve quality of life in HHT patients. The Laryngoscope. ISSN 0023-852X. p. 1–5. doi: 10.1002/lary.28179. Full text in Research Archive
• Steineger, Johan Edvard; Osnes, Terje; Heimdal, Ketil Riddervold & Dheyauldeen, Sinan (2018). Long-term experience with intranasal bevacizumab therapy. The Laryngoscope. ISSN 0023-852X. 128(10), p. 2237–2244. doi: 10.1002/lary.27147. Full text in Research Archive
• Eguiluz-Gracia, Ibon; Malmström, Kristiina; Dheyauldeen, Sinan; Lohi, Jouko; Sajantila, Antti & Aaløkken, Ragnhild [Show all 11 contributors for this article] (2018). Monocytes accumulate in the airways of children with fatal asthma. Clinical and Experimental Allergy. ISSN 0954-7894. 48(12), p. 1631–1639. doi: 10.1111/cea.13265. Full text in Research Archive Show summary

  Background Activated T helper type 2 (Th2) cells are believed to play a pivotal role in allergic airway inflammation, but which cells attract and activate Th2 cells locally have not been fully determined. Recently, it was shown in an experimental human model of allergic rhinitis (AR) that activated monocytes rapidly accumulate in the nasal mucosa after local allergen challenge, where they promote recruitment of Th2 cells and eosinophils. Objective To investigate whether monocytes are recruited to the lungs in paediatric asthma. Methods Tissue samples obtained from children and adolescents with fatal asthma attack (n = 12), age‐matched non‐atopic controls (n = 9) and allergen‐challenged AR patients (n = 8) were subjected to in situ immunostaining. Results Monocytes, identified as CD68+S100A8/A9+ cells, were significantly increased in the lower airway mucosa and in the alveoli of fatal asthma patients compared with control individuals. Interestingly, cellular aggregates containing CD68+S100A8/A9+ monocytes obstructing the lumen of bronchioles were found in asthmatics (8 out of 12) but not in controls. Analysing tissue specimens from challenged AR patients, we confirmed that co‐staining with CD68 and S100A8/A9 was a valid method to identify recently recruited monocytes. We also showed that the vast majority of accumulating monocytes both in the lungs and in the nasal mucosa expressed matrix metalloproteinase 10, suggesting that this protein may be involved in their migration within the tissue. Conclusions and clinical relevance Monocytes accumulated in the lungs of children and adolescents with fatal asthma attack. This finding strongly suggests that monocytes are directly involved in the immunopathology of asthma and that these pro‐inflammatory cells are potential targets for therapy.

• Steineger, Johan Edvard; Ueland, Thor; Aukrust, Pål; Michelsen, Annika; Osnes, Terje & Heimdal, Ketil Riddervold [Show all 7 contributors for this article] (2018). Pentraxin 3 level is elevated in hereditary hemorrhagic telangiectasia and reflects the severity of disease-associated epistaxis. The Laryngoscope. ISSN 0023-852X. 129(1), p. E44–E49. doi: 10.1002/lary.27548. Full text in Research Archive
• Dollner, Ralph; Larsen, Petter Lorentz; Dheyauldeen, Sinan & Steinsvåg, Sverre Karmhus (2017). A multicenter, prospective, noninterventional study in a Norwegian cohort of patients with moderate-to-severe allergic rhinitis treated with MP-AzeFlu. Allergy & Rhinology. ISSN 2152-6575. 8(3), p. e148–e156. doi: 10.2500/ar.2017.8.0216. Full text in Research Archive
• Eguiluz-Gracia, Ibon; Bosco, A; Dollner, Ralph; Melum, Guro Reinholt; Lexberg, Maria Helen & Jones, Anya C. [Show all 10 contributors for this article] (2016). Rapid recruitment of CD14+ monocytes in experimentally induced allergic rhinitis in human subjects. Journal of Allergy and Clinical Immunology. ISSN 0091-6749. 137(6), p. 1872–1881.e12. doi: 10.1016/j.jaci.2015.11.025. Full text in Research Archive Show summary

  Background Activated TH2 cells and eosinophils are hallmarks of the allergic inflammation seen in patients with allergic rhinitis (AR). However, which cells activate and attract T cells and eosinophils to the inflammatory lesion has not been determined. Objective We wanted to assess the role of mucosal mononuclear phagocytes, consisting of monocytes, macrophages, and dendritic cells, in the local allergic inflammatory reaction. Methods Patients with AR and nonatopic control subjects were challenged with pollen extract, and nasal symptoms were recorded. Mucosal biopsy specimens obtained at different time points before and after challenge were used for immunostaining in situ and flow cytometric cell sorting. Sorted mononuclear phagocytes were subjected to RNA extraction and gene expression profiling. Results In an in vivo model of AR, we found that CD14+ monocytes were recruited to the nasal mucosa within hours after local allergen challenge, whereas conventional dendritic cells accumulated after several days of continued provocation. Transcriptomic profiling of mucosal mononuclear phagocytes sorted after 1 week of continued allergen challenge showed an activated phenotype at least partially driven by IL-4 signaling, IL-13 signaling, or both. Importantly, gene expression of several TH2-related chemokines was significantly upregulated by the mononuclear phagocyte population concomitant with an increased recruitment of CD4+ T cells and eosinophils. Conclusion Our findings suggest that the mononuclear phagocyte population is directly involved in the production of proinflammatory chemokines that attract other immune cells. Rapid recruitment of CD14+ monocytes to the challenged site indicates that these proinflammatory mononuclear phagocytes have a central role in orchestrating local allergic inflammation.

• Heimdal, Ketil Riddervold; Dalhus, Bjørn; Rødningen, Olaug Kristin; Kroken, Mette; Eiklid, Kristin Louise & Dheyauldeen, Sinan Ahmed Dheyauldeen [Show all 9 contributors for this article] (2016). Mutation analysis in Norwegian families with hereditary hemorrhagic telangiectasia: Founder mutations in ACVRL1. Clinical Genetics. ISSN 0009-9163. 89(2), p. 182–186. doi: 10.1111/cge.12612. Full text in Research Archive
• Geirdal, Amy Østertun; Dheyauldeen, Sinan Ahmed Dheyauldeen; Bachmann-Harildstad, Gregor & Heimdal, Ketil Riddervold (2013). Living with hereditary haemorrhagic telangiectasia: Coping and psychological distress – A cross-sectional study. Disability and Rehabilitation. ISSN 0963-8288. 35(3), p. 206–213. doi: 10.3109/09638288.2012.690500. Full text in Research Archive
• Geirdal, Amy Østertun; Dheyauldeen, Sinan Ahmed Dheyauldeen; Bachmann-Harildstad, Gregor & Heimdal, Ketil Riddervold (2012). Quality of life in patients with hereditary hemorrhagic telangiectasia in Norway: A population based study. American Journal of Medical Genetics. Part A. ISSN 1552-4825. 158A(6), p. 1269–1278. doi: 10.1002/ajmg.a.35309. Full text in Research Archive
• Dheyauldeen, Sinan Ahmed Dheyauldeen; Geirdal, Amy Østertun; Osnes, Terje; Vartdal, Liv Sofie & Dollner, Ralph (2012). Bevacizumab in hereditary hemorrhagic telangiectasia-associated epistaxis: Effectiveness of an injection protocol based on the vascular anatomy of the nose. The Laryngoscope. ISSN 0023-852X. 122(6), p. 1210–1214. doi: 10.1002/lary.23303. Full text in Research Archive
• Dheyauldeen, Sinan Ahmed Dheyauldeen; Abdelnoor, Michael & Bachmann-Harildstad, Gregor (2011). The natural history of epistaxis in patients with hereditary hemorrhagic telangiectasia in the Norwegian population: A cross-sectional study. American Journal of Rhinology & Allergy. ISSN 1945-8924. 25(4), p. 214–218. doi: 10.2500/ajra.2011.25.3616. Full text in Research Archive
• Dheyauldeen, Sinan Ahmed Dheyauldeen & Bachmann-Harildstad, Gregor (2008). A grading scale for epistaxis in hereditary haemorrhagic teleangectasia. Rhinology. ISSN 0300-0729. 46(4), p. 281–284. Full text in Research Archive

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• Wiedmann, Markus; Steineger, Johan Edvard; Dahlberg, Daniel & Dheyauldeen, Sinan Ahmed Dheyauldeen (2026). How I do it: exoscopic minimally invasive pericranial flap harvest for endoscopic anterior skull base reconstruction. Acta Neurochirurgica. ISSN 0001-6268. 168(1). doi: 10.1007/s00701-026-06828-0. Full text in Research Archive
• Wiedmann, Markus; Olesrud, Ida; Lashkarivand, Aslan; Dheyauldeen, Sinan Ahmed Dheyauldeen; Heck, Ansgar & Berg-Johnsen, Jon [Show all 7 contributors for this article] (2023). Endoskopisk firehåndsteknikk ved hypofysesvulster. 143(9). doi: 10.4045/tidsskr.22.0693. Full text in Research Archive
• Steineger, Johan Edvard; Merckoll, Else; Slåstad, John Magnar; Eriksen, Erik Fink; Heimdal, Ketil Riddervold & Dheyauldeen, Sinan (2017). Osteonecrosis after intranasal injection with bevacizumab in treating hereditary hemorrhagic telangiectasia: A case report. The Laryngoscope. ISSN 0023-852X. 128(3), p. 593–596. doi: 10.1002/lary.26722. Full text in Research Archive
• Dheyauldeen, Sinan Ahmed Dheyauldeen & Geirdal, Amy Østertun (2015). HHT Associated Nosebleeds and Quality of Life. Full text in Research Archive
• Geirdal, Amy Østertun; Dheyauldeen, Sinan Ahmed Dheyauldeen; Bachmann-Harildstad, Gregor & Heimdal, Ketil Riddervold (2014). Quality of Life in HHT. Full text in Research Archive
• Fodstad, Philip; Dheyauldeen, Sinan Ahmed Dheyauldeen; Rinde, Marit & Bachmann-Harildstad, Gregor (2011). Anti-VEGF with 3-week intervals is effective on anemia in a patient with severe hereditary hemorrhagic telangiectasia. Annals of Hematology. ISSN 0939-5555. 90(5), p. 611–612. doi: 10.1007/s00277-010-1063-5. Full text in Research Archive
• Bachmann-Harildstad, Gregor; Fodstad, Philip; Rinde, Marit; Dheyauldeen, Sinan Ahmed Dheyauldeen & Heimdal, K (2011). Bevacizumab with 3-weeks intervals is effective on anemia in a patient with severe hereditary hemorrhagic telangiectasia. Full text in Research Archive

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