Oral manifestations and dental considerations of patients with hereditary hemorrhagic telangiectasia: a scoping review.

OBJECTIVE: We examined the range, nature, and extent of research conducted regarding the oral and dental implications of hereditary hemorrhagic telangiectasia (HHT) to identify gaps in the research and knowledge of the field. STUDY DESIGN: We performed a scoping review according to the Preferred Reporting Items for Systematic Reviews and Meta-Analyses Extension for Scoping Reviews and 2017 Guidance for the Conduct of Joanna Briggs Institute Scoping Reviews. We searched the MEDLINE and Web of Science databases for all full-text articles published in English from December 1946 to October 2022. RESULTS: We identified 103 articles describing oral and dental considerations of patients with HHT, primarily case reports. Most reported oral telangiectasias of the tongue, lips, and palate. Many reported management of bleeding and the use or recommendation of prophylactic antibiotics before dental procedures. CONCLUSIONS: Oral telangiectasias are commonly found in patients with hereditary hemorrhagic telangiectasia, and dental professionals may be the first to diagnose it in their patients. Early detection and diagnosis are important to prevent potentially fatal outcomes, and prophylactic antibiotics before procedures may be warranted.

[Osler's disease - a disease with a wide variety of clinical manifestations deserving multidisciplinary competence]. / Mångfasetterade morbus Osler kräver multidisciplinär kunskap.

Hereditary haemorrhagic telangiectasia (HHT, Osler disease) is an autosomal dominant disease with a prevalence of about 1 in 5 000. The most common symptom is epistaxis in 90 percent of patients, with an average onset at the age of 12 years. Pulmonary arteriovenous malformations are present in 15-35 percent of patients and are associated with embolic complications, such as stroke and cerebral abscesses.  No causative treatment for HHT exists. Iron deficiency anaemia is a common complication. It is treated with oral or intravenous iron replacement depending on the response to tranexamic acid and local treatments. Bevacizumab has been reported to be effective in reducing bleeding complications as well as hepatic and cardiac failure. A multidisciplinary center for the treatment of HHT was established at the University Hospital in Uppsala in 2009.

[Endoscopic nasopharyngectomy, a newly developed surgical technique offering significant clinical benefits in the treatment of nasopharyngeal malignancies]. / Endoskopisk nasofaryngektomi ­ en ny kirurgteknisk möjlighet.

Advances in endoscopic techniques used in sinonasal and skull base surgery have also resulted in the development of endoscopic surgery for malignant tumours in the nasopharynx. The nasopharynx provides great challenges for any surgeon due to its location deep into the cranium  and its close proximity to sensitive structures such as the carotid artery, orbital structures, pituitary gland and the skull base itself. The internationally developed technique of endoscopic nasopharyngectomy has been a clinical treatment option available in Sweden for the past couple of years. In addition to other surgical techniques like 'maxillary swing' the endoscopic approach provides a new option in the difficult management of nasopharyngeal malignancies. The technique is discussed and a clinical case presented to illustrate the method.

Endoscopic assisted insetting of free flaps in anterior skull base reconstruction: A preliminary report of five cases.

INTRODUCTION: Free vascularized tissue may provide a robust reconstruction after anterior skull base surgery. We report our technique and outcomes of the endoscopic inset of free flaps in anterior skull base reconstructions. METHODS: Between 2016 and 2018, endoscopic tumor removal and reconstruction of anterior skull base pathology was performed in five patients aged 20-72 years old (four male, one female). The tumors included three neuroblastomas, a carcinoma, an adenoma, and a melanoma. The median size of the defect was 3.7 × 6.6 cm. Transmaxillary access was gained through the upper sulcus and an anterior and medial maxillectomy. The flap inset was facilitated by the endoscope. The donor vessels were tunneled through the sinus and through the cheek to the facial vessels without the use of the endoscope. RESULTS: In three cases a vastus lateralis flap was used, in one case an adipofascial ALT flap and in one case an adipofascial radial forearm flap. Separation of intracranial and sinonasal spaces was confirmed by radiological and endoscopic examinations. There was no flap failure and one case with partial necrosis. One of the flaps needed to be trimmed as it obliterated the nasal cavity and in one of the cases the flap was repositioned postoperatively. Two cases had infectious complications. The mean follow-up of the patients was 13.8 months. CONCLUSIONS: Endoscopic assisted inset of a free flap in the anterior skull base was feasible in the five cases we present. A dedicated, multidisciplinary approach is mandatory for surgical innovation like this.

Distribution and Molecular Characterization of Human Adenovirus and Epstein-Barr Virus Infections in Tonsillar Lymphocytes Isolated from Patients Diagnosed with Tonsillar Diseases.

Surgically removed palatine tonsils provide a conveniently accessible source of T and B lymphocytes to study the interplay between foreign pathogens and the host immune system. In this study we have characterised the distribution of human adenovirus (HAdV), Epstein-Barr virus (EBV) and human cytomegalovirus (HCMV) in purified tonsillar T and B cell-enriched fractions isolated from three patient age groups diagnosed with tonsillar hypertrophy and chronic/recurrent tonsillitis. HAdV DNA was detected in 93 out of 111 patients (84%), while EBV DNA was detected in 58 patients (52%). The most abundant adenovirus type was HAdV-5 (68%). None of the patients were positive for HCMV. Furthermore, 43 patients (39%) showed a co-infection of HAdV and EBV. The majority of young patients diagnosed with tonsillar hypertrophy were positive for HAdV, whereas all adult patients diagnosed with chronic/recurrent tonsillitis were positive for either HAdV or EBV. Most of the tonsils from patients diagnosed with either tonsillar hypertrophy or chronic/recurrent tonsillitis showed a higher HAdV DNA copy number in T compared to B cell-enriched fraction. Interestingly, in the majority of the tonsils from patients with chronic/recurrent tonsillitis HAdV DNA was detected in T cells only, whereas hypertrophic tonsils demonstrated HAdV DNA in both T and B cell-enriched fractions. In contrast, the majority of EBV positive tonsils revealed a preference for EBV DNA accumulation in the B cell-enriched fraction compared to T cell fraction irrespective of the patients' age.

BDNF as otoprotectant in toxin-induced hearing loss.

CONCLUSION: Brain-derived neurotrophic factor (BDNF) can prevent auditory brainstem response (ABR) threshold shift changes caused by Pseudomonas aeruginosa exotoxin A (PaExoA). OBJECTIVE: Peptides of the neurotrophin family are known to prevent neuronal death during embryonic development by interacting with specific membrane receptors. The purpose of this study was to investigate whether a single dose of BDNF is an effective protectant against toxic effects of PaExoA-induced ABR threshold shifts. MATERIALS AND METHODS: Eight groups of Sprague-Dawley rats were used. There were five control groups (n = 20) as follows. Group A (n = 4) received NaCl solution; group B (n = 3) received 4 µg BDNF; group C (n = 4) received 1 µg/20 µl PaExoA; group D (n = 4) received 2 µg/20 µl PaExoA; group E (n = 5) received 10 µg/20 µl PaExoA injected into the round window niche. Three treatment groups (n = 13) received a single dose of PaExoA and 4 µg of BDNF simultaneously. Group 1 (n = 3) received 1 µg/20 µl PaExoA + 4 µg of BDNF; group 2 (n = 5) received 2 µg/20 µl PaExoA + 4 µg BDNF; group 3 (n = 5) received 10 µg/20 µl PaExoA + 4 µg BDNF. ABR was used to measure efficacy by analyzing threshold shifts before and after injections. RESULTS: A single dose of BDNF prevented changes in ABR thresholds following exposure to increasing concentrations of PaExoA injected into the middle ear.

Early hearing protection by brain-derived neurotrophic factor.

CONCLUSION: Brain-derived neurotrophic factor (BDNF) protects the inner ear from PaExoA (exotoxin A from Pseudomonas aeruginosa)-induced sensory neural hearing loss when administered 12 h after exotoxin, but not after 72 h. OBJECTIVE: BDNF is a peptide in the neurotrophin family with protective effects against noise-induced hair cell loss and toxic inner ear damage following exposure to cisplatin. The exotoxin A (PaExoA) from P. aeruginosa, the most common microorganism in chronic suppurative otitis media, induces sensorineural hearing loss in rats. Previous study showed that, when given simultaneously with the exotoxin, BDNF protected the inner ear from damage. The aim of this study was to determine if BDNF has a protective effect when given 12-72 h after PaExoA. MATERIALS AND METHODS: Five groups of Sprague-Dawley rats were used. The three control groups (n = 16) were as follows. Group 1 (n = 8) received 15 µg/20 µl PaExoA; group 2 (n = 5) received 20 µg/20 µl PaExoA; and group 3 (n = 3) received 25 µg/20 µl PaExoA injected into the round window niche. There were two treatment groups (n = 12): group A (n = 6) received 15 µg/20 µl PaExoA and 4 µg/20 µl BDNF 12 h later; group B (n = 6) received 15 µg/20 µl PaExoA and 4 µg/20 µl BDNF 72 h later. Brainstem response audiometry (ABR) was performed on day 0 (control), and repeated on days 7, 14, 21, 28, and 35 to analyze the thresholds shifts. RESULTS: Exposure to 15 µg/20 µl PaExoA caused persistent and significant ABR impairment in controls when measured after 35 days. A single dose of BDNF given 12 h after PaExoA reduced hearing loss significantly, but when BDNF was given 72 h after PaExoA no protective effect was evident.

Protective effect of edaravone against tobramycin-induced ototoxicity.

CONCLUSION: It is suggested that simultaneous treatment with the radical scavenger edaravone has an effective protective effect against tobramycin ototoxicity in rat. Even if the edaravone treatment is postponed for 7 days, it can still prevent hearing loss, but a 14 day delay cannot protect from ototoxicity. OBJECTIVES: With the aim of alleviating hearing loss caused by aminoglycoside ototoxicity, we performed a trial to assess the hearing protective efficacy of the radical scavenger edaravone. MATERIALS AND METHODS: In part one of the study, 21 male Sprague-Dawley albino rats were used; 2 rats served as controls for the safety of edaravone. Eight rats each received 10 subcutaneous injections (s.c.) of tobramycin (160 mg/kg b.w.) once daily and saline injection intraperitoneally for 2 weeks. Eleven rats were given 10 s.c. tobramycin injections simultaneously with an intraperitoneal injection of edaravone (3 mg/kg b.w.). In part two, tobramycin was injected in 13 rats (as above). Five of these received two edaravone injections 7 days later and four rats similarly 14 days later. Auditory brainstem response (ABR) was used to assess hearing. RESULTS: All rats treated only with tobramycin showed a deterioration of hearing. None of the rats given simultaneous treatment with tobramycin and edaravone demonstrated hearing loss. A 7 day delay in edaravone injection still prevented hearing loss, but a 14 day delay had only a temporary prophylactic effect.