Materiały źródłowe

• #1 Bleeding and clotting in hereditary hemorrhagic telangiectasia

  https://www.wjgnet.com/2307-8960/full/v3/i4/330.htm

  Hereditary hemorrhagic telangiectasia (HHT) is a relatively common inherited vascular disorder that was first described in 1864, and is notable for epistaxis, telangiectasia, and arterial venous malformations. […] Patients with HHT are at increased risk for both bleeding and clotting events. […] While the majority of management decisions revolve around bleeding complications, it is not infrequent for these patients to require anticoagulation for thrombosis. […] Any anticoagulation recommendations must take into account the bleeding risks associated with HHT. […] Recent reviews have found that HHT patients can be safely anticoagulated, with the most frequent complication being worsened epistaxis. […] Current recommendations include screening asymptomatic HHT patients with transthoracic contrast echocardiography (TTCE), or chest computed tomography if TTCE is not available.

• #2 Hereditary haemorrhagic telangiectasia: diagnosis, screening and management | Medicine Today

  https://medicinetoday.com.au/mt/2023/may/regular-series/hereditary-haemorrhagic-telangiectasia-diagnosis-screening-and-management

  Hereditary haemorrhagic telangiectasia (HHT) is underdiagnosed, and there is often a delay in diagnosis. […] Thus, it is important for primary care physicians to recognise the presentation of HHT, as prompt diagnosis allows for appropriate screening and preventative treatment in both patients and affected family members. […] Early screening by GPs and referrals to specialists may reduce morbidity and mortality associated with pulmonary or central nervous system (CNS) AVMs, as patients with these complications are assessed and pre-emptively treated at HHT centres. […] One of the most important aspects of HHT care is to screen for potential end-organ involvement. […] Patients with HHT should be made aware of the autosomal dominant transmission of the disease and offered genetic counselling.

• #3 Infection prevention in patients with hereditary hemorrhagic telangiectasia

  https://pmc.ncbi.nlm.nih.gov/articles/PMC6165807/

  After reading the manuscript by Kritharis A et al., with interest we agree that the prevention of future hereditary hemorrhagic telangiectasia (HHT) complications is as important as treating the immediate active issues. For this reason, we would like to add an important part of the management of these patients that Kritharis A et al. did not mention in their manuscript, which is the prevention of severe infections through vaccination. […] Patients with HHT have an increased risk of infection potentially leading to serious infections requiring hospitalization, which according to the series is between 13.6% and 28.6% of the cases. […] As the manufacturers highlight in the technical documentation, the drugs used to prevent bleeding nearly double the frequency of moderate-severe infections, including pneumonia, that occurs in 10% of patients receiving Thalidomide, as well as the reactivation of hepatitis B or varicella zoster viruses that, in post-marketing studies, reported cases of acute liver failure and disseminated zoster. This fact justifies screening and vaccination against hepatitis B virus of patients with HHT without circulating surface antibodies against the virus as well as zoster vaccination of patients aged 50 years or older with varicella zoster antibodies at least once a month before starting treatment. […] For these reasons, we consider that vaccination protocols should be implemented in the management of patients affected by HHT, along with adequate oral hygiene and antibiotic prophylaxis prior to risky dental maneuvers.

• #4 Prevention of serious infections in hereditary hemorrhagic telangiectasia: roles for prophylactic antibiotics, the pulmonary capillaries-but not vaccination

  https://pmc.ncbi.nlm.nih.gov/articles/PMC6355489/

  Rodrguez-Garca et al. recently suggested that vaccination against Haemophilus influenzae, streptococcus pneumonia, and influenza should be considered a potential route to prevent the major deep-seated infections observed in some patients with hereditary hemorrhagic telangiectasia (HHT1). […] Furthermore, we are concerned that a focus on these pathogens risks missing the relevant necessary foci that require a different approach-transient bacteremias, prophylactic antibiotics, and pulmonary capillary clearance functions. […] Prophylactic antibiotics prior to dental and surgical procedures has been a longstanding recommendation for patients with HHT and pulmonary AVMs, and this is one of the five VASCERN HHT Outcome Measures published in 2018. […] Reducing risks of the abscesses and other infections associated with pulmonary AVMs in HHT patients will require a more fundamental understanding of transient dental and surgical bacteremias, with a focus on pulmonary capillary clearance functions.

• #5 Prevention of serious infections in hereditary hemorrhagic telangiectasia: roles for prophylactic antibiotics, the pulmonary capillaries-but not vaccination

  https://pmc.ncbi.nlm.nih.gov/articles/PMC6355489/

  Rodrguez-Garca et al. recently suggested that vaccination against Haemophilus influenzae, streptococcus pneumonia, and influenza should be considered a potential route to prevent the major deep-seated infections observed in some patients with hereditary hemorrhagic telangiectasia (HHT1). […] Furthermore, we are concerned that a focus on these pathogens risks missing the relevant necessary foci that require a different approach-transient bacteremias, prophylactic antibiotics, and pulmonary capillary clearance functions. […] Prophylactic antibiotics prior to dental and surgical procedures has been a longstanding recommendation for patients with HHT and pulmonary AVMs, and this is one of the five VASCERN HHT Outcome Measures published in 2018. […] Reducing risks of the abscesses and other infections associated with pulmonary AVMs in HHT patients will require a more fundamental understanding of transient dental and surgical bacteremias, with a focus on pulmonary capillary clearance functions.

• #6 Hereditary Hemorrhagic Telangiectasia: Diagnosis and Management | AAFP

  https://www.aafp.org/pubs/afp/issues/2010/1001/p785.html

  Transthoracic contrast echocardiography should be used as the initial screening test for pulmonary AVMs. […] Adults with possible or confirmed HHT should receive MRI to screen for cerebrovascular AVMs using a protocol with and without contrast media administration and using sequences that detect blood products to maximize sensitivity. […] Patients with documented pulmonary AVMs (treated or untreated) should be advised to use antibiotic prophylaxis for any procedures that have a risk of bacteremia (e.g., dental); to have air filters on all intravenous lines; and to avoid scuba diving because of the risk of decompression sickness. […] Screening should include contrast echocardiography and MRI of the brain. […] Patients who have AVMs, as well as those who have not yet been screened, should receive antibiotic prophylaxis before dental work or other dirty medical procedures to avoid the possible development of brain infection and abscess formation. […] The current recommendation is to treat any AVM that is at least 3 mm in diameter, although some physicians will treat AVMs as small as 1 mm.

• #7 Hereditary hemorrhagic telangiectasia and pulmonary arteriovenous malformations: clinical aspects – Meier – Cardiovascular Diagnosis and Therapy

  https://cdt.amegroups.org/article/view/18281/html

  Hereditary hemorrhagic telangiectasia (HHT) is a genetic disorder of blood vessel formation resulting in mucocutaneous telangiectasias and visceral arteriovenous malformations. […] Management hinges on timely screening and diagnosis, followed by treatment of amenable PAVMs with transcatheter embolization in conjunction with medical management and prophylactic measures to treat and prevent complications. […] Effective screening is necessary to identify which HHT patients have PAVMs so that their dire clinical consequences can be avoided. Transthoracic contrast echocardiography (TTCE) is the preferred method for PAVM screening because of its high sensitivity and excellent negative predictive value. […] Steps to reduce embolic complications are recommended for all patients with PAVMs and for patients with confirmed or suspected HHT who have not yet been screened for PAVMs. To prevent septic embolic complications, antibiotics are recommended before procedures that may result in bacteremia. […] Prophylactic measures are advised for all patients after positive screening regardless of CT results.

• #8 Hereditary Hemorrhagic Telangiectasia: Diagnosis and Management | AAFP

  https://www.aafp.org/pubs/afp/issues/2010/1001/p785.html

  Hereditary hemorrhagic telangiectasia is an uncommon autosomal dominant disease that occurs in approximately one in 5,000 to 8,000 persons. […] Screening and treatment recommendations have been created in an attempt to limit the morbidity and mortality associated with this disease. Patients with confirmed or suspected hereditary hemorrhagic telangiectasia should be screened for brain and lung arteriovenous malformations using magnetic resonance imaging of the brain and contrast echocardiography. […] Patients with a history of pulmonary arteriovenous malformations or those who have not been screened should use antibiotic prophylaxis before dental treatment, endoscopy, or other procedures that could cause bacteremia because of the risk of paradoxical brain embolism or infection. […] All patients with possible or confirmed HHT should be screened for pulmonary AVMs.

• #9 Hereditary Hemorrhagic Telangiectasia: Diagnosis and Management | AAFP

  https://www.aafp.org/pubs/afp/issues/2010/1001/p785.html

  Transthoracic contrast echocardiography should be used as the initial screening test for pulmonary AVMs. […] Adults with possible or confirmed HHT should receive MRI to screen for cerebrovascular AVMs using a protocol with and without contrast media administration and using sequences that detect blood products to maximize sensitivity. […] Patients with documented pulmonary AVMs (treated or untreated) should be advised to use antibiotic prophylaxis for any procedures that have a risk of bacteremia (e.g., dental); to have air filters on all intravenous lines; and to avoid scuba diving because of the risk of decompression sickness. […] Screening should include contrast echocardiography and MRI of the brain. […] Patients who have AVMs, as well as those who have not yet been screened, should receive antibiotic prophylaxis before dental work or other dirty medical procedures to avoid the possible development of brain infection and abscess formation. […] The current recommendation is to treat any AVM that is at least 3 mm in diameter, although some physicians will treat AVMs as small as 1 mm.

• #10 Hereditary Hemorrhagic Telangiectasia (also known as HHT, Osler-Weber-Rendu syndrome, Osler-Weber-Rendu disease, Osler-Rendu disease, Osler’s disease, Weber-Osler disease, and Rendu-Osler-Weber syndrome) – CDHO

  https://cdho.org/factsheets/hereditary-hemorrhagic-telangiectasia/

  Is antibiotic prophylaxis required? Yes, if there are pulmonary arteriovenous malformations (PAVMs), whether treated or untreated, or if the patient/client has not yet been screened at a specialized HHT centre. (Blood-borne bacteria resulting from invasive procedures can pass through PAVMs, lodge in the brain, and cause brain abscesses.) If the patient/client has been screened for PAVMs and the tests confirm that there are none, then prophylactic antibiotics are not required. […] Is postponing treatment advised? Possibly. If the patient/client with HHT has not had the appropriate diagnostic tests for pulmonary AVMs, ideally dental hygiene treatment should be delayed until testing occurs. […] In order to reduce risk of bleeding from oral telangiectasias, the dental hygienist should advise the patient/client as follows: use a soft-bristled toothbrush, floss only in areas free of telangiectasias, use lip sunscreen to protect against sun exposure, avoid hard, irritating, and hot foods, keep dental prostheses (such as mouth guards) in good condition, ensuring proper fit and avoiding damage to the gingiva and irritation of telangiectasias.

• #11 Hereditary hemorrhagic telangiectasia and pregnancy: potential adverse | IJWH

  https://www.dovepress.com/hereditary-hemorrhagic-telangiectasia-and-pregnancy-potential-adverse–peer-reviewed-fulltext-article-IJWH

  For all pregnant women with HHT, antibiotic prophylaxis during delivery is recommended. Also, avoidance of a prolonged stage of labor in HHT patients in whom brain AVM has not been excluded is advised. […] The majority of pregnancies in patients with HHT have no complications. However, pregnancy is high risk due to the possibility of severe, life threatening complications. Thus, women with HHT should be appropriately counseled on the small but serious risks. In addition, it is important for these patients to be appropriately managed since prior awareness of HHT has been associated with enhanced survival for the mother. […] HHT should be managed per international consensus guidelines. Women with HHT planning pregnancy should be advised that risks are low but serious. However, they should be reassured that close obstetric care and prior awareness of the diagnosis of HHT is usually associated with good pregnancy outcomes.

• #12 Hereditary hemorrhagic telangiectasia, embolization, and Young’s procedure: oral surgical management | Journal of Oral Medicine and Oral Surgery

  https://www.jomos.org/articles/mbcb/full_html/2018/02/mbcb170009/mbcb170009.html

  Hereditary hemorrhagic telangiectasia (HHT) case with history of embolization and Youngs procedure: surgical management. […] The management of patients affected by HHT needs rigorous hemostatic methods and outpatient postoperative monitoring. […] In patients with HHT, two types of risks are present: hemorrhagic risk and infectious risk. […] The hemorrhagic risk: This is linked to capillary fragility. It can be prevented by proper oral hygiene and regular follow-up. In cases where surgery was performed, local methods of hemostasis must be implemented systematically; […] Although no randomized trials have been performed for antibiotic prophylaxis in HHT, it is advisable before any invasive procedure. […] This clinical case reminds us that patients who have HHT require rigorous methods of hemostasis in addition to regular postoperative outpatient follow-up. A periodical dental check-up is recommended to minimize the need for tooth extraction.

• #13 Post-NICE 2008: antibiotic prophylaxis prior to dental procedures for patients with pulmonary arteriovenous malformations (PAVMs) and hereditary haemorrhagic telangiectasia | British Dental Journal

  https://www.nature.com/articles/sj.bdj.2008.978

  Recently published guidance from NICE highlights that antibiotic prophylaxis is no longer required for patients with structural heart disease at risk of infective endocarditis. […] Individuals with pulmonary arteriovenous malformations and hereditary haemorrhagic telangiectasia are at risk of brain abscess from dental bacteraemias. […] For patients with PAVMs and HHT, there is a link between oral bacteria and PAVM-associated brain abscess. Antibiotic prophylaxis has been recommended for them based on the endocarditis paradigm, with the British Society for Antimicrobial Chemotherapy and Dental Formulary Sub-Committee approving a PAVM-specific card in 2000. […] For these reasons we suggest that: Antibiotic prophylaxis should still be given to PAVM/HHT patients. […] All PAVM patients and their families should pursue strategies to optimise dental hygiene (known to reduce the risk of dental bacteraemias of all causes). […] Further research on susceptibility of the HHT population to dental bacteraemias is warranted.

• #14 Prevention of serious infections in hereditary hemorrhagic telangiectasia: roles for prophylactic antibiotics, the pulmonary capillaries-but not vaccination

  https://pmc.ncbi.nlm.nih.gov/articles/PMC6355489/

  Rodrguez-Garca et al. recently suggested that vaccination against Haemophilus influenzae, streptococcus pneumonia, and influenza should be considered a potential route to prevent the major deep-seated infections observed in some patients with hereditary hemorrhagic telangiectasia (HHT1). […] Furthermore, we are concerned that a focus on these pathogens risks missing the relevant necessary foci that require a different approach-transient bacteremias, prophylactic antibiotics, and pulmonary capillary clearance functions. […] Prophylactic antibiotics prior to dental and surgical procedures has been a longstanding recommendation for patients with HHT and pulmonary AVMs, and this is one of the five VASCERN HHT Outcome Measures published in 2018. […] Reducing risks of the abscesses and other infections associated with pulmonary AVMs in HHT patients will require a more fundamental understanding of transient dental and surgical bacteremias, with a focus on pulmonary capillary clearance functions.

• #15

  https://haematologica.org/article/view/8795

  Rodrguez-Garca et al. recently suggested that vaccination against Haemophilus influenzae, streptococcus pneumonia, and influenza should be considered a potential route to prevent the major deep-seated infections observed in some patients with hereditary hemorrhagic telangiectasia (HHT1). […] We agree with Al-Samkari et al., that patients with HHT should have the usual vaccination strategies, as for members of the general population. In most countries, this includes pneumococcal and haemophilus influenza type b which can cause invasive disease and meningitis in any individual but there is no evidence that HHT patients are at any more or less risk of these infections than the general population. They are however at a significantly increased risk of polymicrobial, deep-seated infections and abscesses especially if they have pulmonary AVMs. Reducing risks of the abscesses and other infections associated with pulmonary AVMs in HHT patients will require a more fundamental understanding of transient dental and surgical bacteremias, with a focus on pulmonary capillary clearance functions. It is important that the question of vaccination, which should be a normal part of infection prevention for everyone, does not obscure the need for a better focus on how to prevent these bacteremias from causing life altering, deep-seated infections.

• #16

  https://haematologica.org/article/view/8795

  Rodrguez-Garca et al. recently suggested that vaccination against Haemophilus influenzae, streptococcus pneumonia, and influenza should be considered a potential route to prevent the major deep-seated infections observed in some patients with hereditary hemorrhagic telangiectasia (HHT1). […] We agree with Al-Samkari et al., that patients with HHT should have the usual vaccination strategies, as for members of the general population. In most countries, this includes pneumococcal and haemophilus influenza type b which can cause invasive disease and meningitis in any individual but there is no evidence that HHT patients are at any more or less risk of these infections than the general population. They are however at a significantly increased risk of polymicrobial, deep-seated infections and abscesses especially if they have pulmonary AVMs. Reducing risks of the abscesses and other infections associated with pulmonary AVMs in HHT patients will require a more fundamental understanding of transient dental and surgical bacteremias, with a focus on pulmonary capillary clearance functions. It is important that the question of vaccination, which should be a normal part of infection prevention for everyone, does not obscure the need for a better focus on how to prevent these bacteremias from causing life altering, deep-seated infections.

• #17 Prevention of serious infections in hereditary hemorrhagic telangiectasia: roles for prophylactic antibiotics, the pulmonary capillaries-but not vaccination

  https://pmc.ncbi.nlm.nih.gov/articles/PMC6355489/

  Rodrguez-Garca et al. recently suggested that vaccination against Haemophilus influenzae, streptococcus pneumonia, and influenza should be considered a potential route to prevent the major deep-seated infections observed in some patients with hereditary hemorrhagic telangiectasia (HHT1). […] Furthermore, we are concerned that a focus on these pathogens risks missing the relevant necessary foci that require a different approach-transient bacteremias, prophylactic antibiotics, and pulmonary capillary clearance functions. […] Prophylactic antibiotics prior to dental and surgical procedures has been a longstanding recommendation for patients with HHT and pulmonary AVMs, and this is one of the five VASCERN HHT Outcome Measures published in 2018. […] Reducing risks of the abscesses and other infections associated with pulmonary AVMs in HHT patients will require a more fundamental understanding of transient dental and surgical bacteremias, with a focus on pulmonary capillary clearance functions.

• #18 Infection prevention in patients with hereditary hemorrhagic telangiectasia

  https://pmc.ncbi.nlm.nih.gov/articles/PMC6165807/

  After reading the manuscript by Kritharis A et al., with interest we agree that the prevention of future hereditary hemorrhagic telangiectasia (HHT) complications is as important as treating the immediate active issues. For this reason, we would like to add an important part of the management of these patients that Kritharis A et al. did not mention in their manuscript, which is the prevention of severe infections through vaccination. […] Patients with HHT have an increased risk of infection potentially leading to serious infections requiring hospitalization, which according to the series is between 13.6% and 28.6% of the cases. […] As the manufacturers highlight in the technical documentation, the drugs used to prevent bleeding nearly double the frequency of moderate-severe infections, including pneumonia, that occurs in 10% of patients receiving Thalidomide, as well as the reactivation of hepatitis B or varicella zoster viruses that, in post-marketing studies, reported cases of acute liver failure and disseminated zoster. This fact justifies screening and vaccination against hepatitis B virus of patients with HHT without circulating surface antibodies against the virus as well as zoster vaccination of patients aged 50 years or older with varicella zoster antibodies at least once a month before starting treatment. […] For these reasons, we consider that vaccination protocols should be implemented in the management of patients affected by HHT, along with adequate oral hygiene and antibiotic prophylaxis prior to risky dental maneuvers.

• #19 Osler-Weber-Rendu Disease (Hereditary Hemorrhagic Telangiectasia) Guidelines: Second International Hereditary Hemorrhagic Telangiectasia Guidelines

  https://emedicine.medscape.com/article/2048472-guidelines

  In September 2020, the Second International Hereditary Hemorrhagic Telangiectasia (HHT) Guidelines, aimed at developing evidence-based consensus guidelines for the management and prevention of HHT-related symptoms and complications, were published. The guidelines included the following recommendations. […] To reduce HHT-related epistaxis, use moisturizing topical therapies that humidify the nasal mucosa. […] Consider oral tranexamic acid (TXA) for epistaxis that does not respond to moisturizing topical therapies. […] Consider ablative therapies (eg, laser treatment, radiofrequency ablation [RFA], electrosurgery, and sclerotherapy) for nasal telangiectasias that have not responded to moisturizing topical therapies. […] Consider systemic antiangiogenic agents for epistaxis that has not responded to moisturizing topical therapies, ablative therapies, or TXA.

• #20 Hereditary hemorrhagic telangiectasia – Diagnosis and treatment – Mayo Clinic

  https://www.mayoclinic.org/diseases-conditions/hht/diagnosis-treatment/drc-20351136

  To help prevent HHT nosebleeds, you may want to: […] Not use certain medicines. Your risk of bleeding can be higher from using certain medicines and drugs you get without a prescription. These include aspirin, ibuprofen (Advil, Motrin IB, others), fish oil supplements, ginkgo and St. John’s wort. […] Not eat certain foods. In some people, having blueberries, red wine, dark chocolate or spicy foods can cause HHT nosebleeds. Try keeping a food diary to see if there’s any link between what you eat and how bad your nosebleeds are. […] Keep your nose moist. Use saline sprays, lotions or gels that add moisture to help lower the risk of bleeding. Using a bedside humidifier overnight also is helpful. […] Not do heavy lifting. Bending over and lifting heavy objects can cause nosebleeds.

• #21 Hereditary haemorrhagic telangiectasia: diagnosis, screening and management | Medicine Today

  https://medicinetoday.com.au/mt/2023/may/regular-series/hereditary-haemorrhagic-telangiectasia-diagnosis-screening-and-management

  When a causative mutation has been identified in the proband, predictive testing can be considered in relatives. […] Preimplantation diagnosis is an option for patients with HHT wish to be parents and to ensure the causative mutation is not passed on to offspring. […] There are no standard medical therapies for HHT given the few randomised trials in this field. […] Management can include supportive care, lesion-specific therapy and systemic treatment. […] Supportive measures include iron supplementation (oral and intravenous initially, if not effective) and red blood cell transfusion for bleeding and anaemia. […] Patients with HHT who are reviewed in a multidisciplinary team clinic are provided with education on nasal care, consisting of the use of regular nasal lubricants, such as petroleum jelly, paw paw-containing ointment or moisturising nasal sprays.

• #22 Hereditary hemorrhagic telangiectasia – Diagnosis and treatment – Mayo Clinic

  https://www.mayoclinic.org/diseases-conditions/hht/diagnosis-treatment/drc-20351136

  To help prevent HHT nosebleeds, you may want to: […] Not use certain medicines. Your risk of bleeding can be higher from using certain medicines and drugs you get without a prescription. These include aspirin, ibuprofen (Advil, Motrin IB, others), fish oil supplements, ginkgo and St. John’s wort. […] Not eat certain foods. In some people, having blueberries, red wine, dark chocolate or spicy foods can cause HHT nosebleeds. Try keeping a food diary to see if there’s any link between what you eat and how bad your nosebleeds are. […] Keep your nose moist. Use saline sprays, lotions or gels that add moisture to help lower the risk of bleeding. Using a bedside humidifier overnight also is helpful. […] Not do heavy lifting. Bending over and lifting heavy objects can cause nosebleeds.

• #23 Hereditary hemorrhagic telangiectasia – Diagnosis and treatment – Mayo Clinic

  https://www.mayoclinic.org/diseases-conditions/hht/diagnosis-treatment/drc-20351136

  To help prevent HHT nosebleeds, you may want to: […] Not use certain medicines. Your risk of bleeding can be higher from using certain medicines and drugs you get without a prescription. These include aspirin, ibuprofen (Advil, Motrin IB, others), fish oil supplements, ginkgo and St. John’s wort. […] Not eat certain foods. In some people, having blueberries, red wine, dark chocolate or spicy foods can cause HHT nosebleeds. Try keeping a food diary to see if there’s any link between what you eat and how bad your nosebleeds are. […] Keep your nose moist. Use saline sprays, lotions or gels that add moisture to help lower the risk of bleeding. Using a bedside humidifier overnight also is helpful. […] Not do heavy lifting. Bending over and lifting heavy objects can cause nosebleeds.

• #24 Hereditary hemorrhagic telangiectasia – Diagnosis and treatment – Mayo Clinic

  https://www.mayoclinic.org/diseases-conditions/hht/diagnosis-treatment/drc-20351136

  To help prevent HHT nosebleeds, you may want to: […] Not use certain medicines. Your risk of bleeding can be higher from using certain medicines and drugs you get without a prescription. These include aspirin, ibuprofen (Advil, Motrin IB, others), fish oil supplements, ginkgo and St. John’s wort. […] Not eat certain foods. In some people, having blueberries, red wine, dark chocolate or spicy foods can cause HHT nosebleeds. Try keeping a food diary to see if there’s any link between what you eat and how bad your nosebleeds are. […] Keep your nose moist. Use saline sprays, lotions or gels that add moisture to help lower the risk of bleeding. Using a bedside humidifier overnight also is helpful. […] Not do heavy lifting. Bending over and lifting heavy objects can cause nosebleeds.

• #25 Osler-Weber-Rendu Disease (Hereditary Hemorrhagic Telangiectasia) Guidelines: Second International Hereditary Hemorrhagic Telangiectasia Guidelines

  https://emedicine.medscape.com/article/2048472-guidelines

  In September 2020, the Second International Hereditary Hemorrhagic Telangiectasia (HHT) Guidelines, aimed at developing evidence-based consensus guidelines for the management and prevention of HHT-related symptoms and complications, were published. The guidelines included the following recommendations. […] To reduce HHT-related epistaxis, use moisturizing topical therapies that humidify the nasal mucosa. […] Consider oral tranexamic acid (TXA) for epistaxis that does not respond to moisturizing topical therapies. […] Consider ablative therapies (eg, laser treatment, radiofrequency ablation [RFA], electrosurgery, and sclerotherapy) for nasal telangiectasias that have not responded to moisturizing topical therapies. […] Consider systemic antiangiogenic agents for epistaxis that has not responded to moisturizing topical therapies, ablative therapies, or TXA.

• #26 Hereditary haemorrhagic telangiectasia: diagnosis, screening and management | Medicine Today

  https://medicinetoday.com.au/mt/2023/may/regular-series/hereditary-haemorrhagic-telangiectasia-diagnosis-screening-and-management

  If there are no contraindications, some patients can take regular tranexamic acid (at a dose of 1g) to manage symptoms. […] Antibiotic prophylaxis is recommended for dental work and other procedures with a significant risk of bacteraemia in all patients with evidence of a pulmonary shunt. […] Most pregnancies in women with HHT are uneventful. […] Thus, screening for cerebral and pulmonary AVMs should be performed prior to pregnancy. […] Pregnant women with untreated pulmonary or CNS AVMs, as well as those who have not been screened, should be considered to have a high risk of haemorrhagic and neurological complications.

• #27 Osler-Weber-Rendu Disease (Hereditary Hemorrhagic Telangiectasia) Guidelines: Second International Hereditary Hemorrhagic Telangiectasia Guidelines

  https://emedicine.medscape.com/article/2048472-guidelines

  In September 2020, the Second International Hereditary Hemorrhagic Telangiectasia (HHT) Guidelines, aimed at developing evidence-based consensus guidelines for the management and prevention of HHT-related symptoms and complications, were published. The guidelines included the following recommendations. […] To reduce HHT-related epistaxis, use moisturizing topical therapies that humidify the nasal mucosa. […] Consider oral tranexamic acid (TXA) for epistaxis that does not respond to moisturizing topical therapies. […] Consider ablative therapies (eg, laser treatment, radiofrequency ablation [RFA], electrosurgery, and sclerotherapy) for nasal telangiectasias that have not responded to moisturizing topical therapies. […] Consider systemic antiangiogenic agents for epistaxis that has not responded to moisturizing topical therapies, ablative therapies, or TXA.

• #28 Osler-Weber-Rendu Disease (Hereditary Hemorrhagic Telangiectasia) Guidelines: Second International Hereditary Hemorrhagic Telangiectasia Guidelines

  https://emedicine.medscape.com/article/2048472-guidelines

  In September 2020, the Second International Hereditary Hemorrhagic Telangiectasia (HHT) Guidelines, aimed at developing evidence-based consensus guidelines for the management and prevention of HHT-related symptoms and complications, were published. The guidelines included the following recommendations. […] To reduce HHT-related epistaxis, use moisturizing topical therapies that humidify the nasal mucosa. […] Consider oral tranexamic acid (TXA) for epistaxis that does not respond to moisturizing topical therapies. […] Consider ablative therapies (eg, laser treatment, radiofrequency ablation [RFA], electrosurgery, and sclerotherapy) for nasal telangiectasias that have not responded to moisturizing topical therapies. […] Consider systemic antiangiogenic agents for epistaxis that has not responded to moisturizing topical therapies, ablative therapies, or TXA.

• #29 Osler-Weber-Rendu Disease (Hereditary Hemorrhagic Telangiectasia) Guidelines: Second International Hereditary Hemorrhagic Telangiectasia Guidelines

  https://emedicine.medscape.com/article/2048472-guidelines

  Consider septodermoplasty for epistaxis that has not responded sufficiently to moisturizing topical therapies, ablative therapies, or TXA. […] Consider nasal closure for epistaxis that has not responded sufficiently to moisturizing topical therapies, ablative therapies, or TXA. […] Test for iron deficiency and anemia in all adult HHT patients, regardless of symptoms, and in all pediatric HHT patients with recurrent bleeding and/or symptoms of anemia. […] Provide HHT patients with anticoagulation (prophylactic or therapeutic) or antiplatelet therapy when there is an indication, with individualized bleeding risks taken into consideration; bleeding in HHT is not an absolute contraindication for these therapies. […] Where possible, avoid the use of dual antiplatelet therapy (DAPT) and/or a combination of antiplatelet therapy and anticoagulation.

• #30 Osler-Weber-Rendu Disease (Hereditary Hemorrhagic Telangiectasia) Guidelines: Second International Hereditary Hemorrhagic Telangiectasia Guidelines

  https://emedicine.medscape.com/article/2048472-guidelines

  Consider septodermoplasty for epistaxis that has not responded sufficiently to moisturizing topical therapies, ablative therapies, or TXA. […] Consider nasal closure for epistaxis that has not responded sufficiently to moisturizing topical therapies, ablative therapies, or TXA. […] Test for iron deficiency and anemia in all adult HHT patients, regardless of symptoms, and in all pediatric HHT patients with recurrent bleeding and/or symptoms of anemia. […] Provide HHT patients with anticoagulation (prophylactic or therapeutic) or antiplatelet therapy when there is an indication, with individualized bleeding risks taken into consideration; bleeding in HHT is not an absolute contraindication for these therapies. […] Where possible, avoid the use of dual antiplatelet therapy (DAPT) and/or a combination of antiplatelet therapy and anticoagulation.

• #31 Post-NICE 2008: antibiotic prophylaxis prior to dental procedures for patients with pulmonary arteriovenous malformations (PAVMs) and hereditary haemorrhagic telangiectasia | British Dental Journal

  https://www.nature.com/articles/sj.bdj.2008.978

  Recently published guidance from NICE highlights that antibiotic prophylaxis is no longer required for patients with structural heart disease at risk of infective endocarditis. […] Individuals with pulmonary arteriovenous malformations and hereditary haemorrhagic telangiectasia are at risk of brain abscess from dental bacteraemias. […] For patients with PAVMs and HHT, there is a link between oral bacteria and PAVM-associated brain abscess. Antibiotic prophylaxis has been recommended for them based on the endocarditis paradigm, with the British Society for Antimicrobial Chemotherapy and Dental Formulary Sub-Committee approving a PAVM-specific card in 2000. […] For these reasons we suggest that: Antibiotic prophylaxis should still be given to PAVM/HHT patients. […] All PAVM patients and their families should pursue strategies to optimise dental hygiene (known to reduce the risk of dental bacteraemias of all causes). […] Further research on susceptibility of the HHT population to dental bacteraemias is warranted.

• #32 Hereditary Hemorrhagic Telangiectasia (also known as HHT, Osler-Weber-Rendu syndrome, Osler-Weber-Rendu disease, Osler-Rendu disease, Osler’s disease, Weber-Osler disease, and Rendu-Osler-Weber syndrome) – CDHO

  https://cdho.org/factsheets/hereditary-hemorrhagic-telangiectasia/

  Is antibiotic prophylaxis required? Yes, if there are pulmonary arteriovenous malformations (PAVMs), whether treated or untreated, or if the patient/client has not yet been screened at a specialized HHT centre. (Blood-borne bacteria resulting from invasive procedures can pass through PAVMs, lodge in the brain, and cause brain abscesses.) If the patient/client has been screened for PAVMs and the tests confirm that there are none, then prophylactic antibiotics are not required. […] Is postponing treatment advised? Possibly. If the patient/client with HHT has not had the appropriate diagnostic tests for pulmonary AVMs, ideally dental hygiene treatment should be delayed until testing occurs. […] In order to reduce risk of bleeding from oral telangiectasias, the dental hygienist should advise the patient/client as follows: use a soft-bristled toothbrush, floss only in areas free of telangiectasias, use lip sunscreen to protect against sun exposure, avoid hard, irritating, and hot foods, keep dental prostheses (such as mouth guards) in good condition, ensuring proper fit and avoiding damage to the gingiva and irritation of telangiectasias.

• #33 Hereditary Hemorrhagic Telangiectasia (HHT) > Fact Sheets > Yale Medicine

  https://www.yalemedicine.org/conditions/hht

  Because this condition doesnt cause any warning symptoms and leaves people vulnerable to stroke and life-threatening infections proper diagnosis, treatment, and monitoring is important. […] Patients with suspected HHT, such was those with family members having this condition, says Jeffrey Pollak, MD, interventional radiologist and medical director of the Yale Medicine HHT and Vascular Malformations Program, should be screened for this condition and specific sites of involvement, especially since sudden life-altering events that might occur even in the absence of any preceding symptoms can potentially be prevented if HHT is known and treated in advance. […] Early diagnosis, treatment, and follow up can help people avoid these complications. […] Once a family member is diagnosed, its recommended that other family members seek genetic counseling and screening.

• #34 Hereditary hemorrhagic telangiectasia and pulmonary arteriovenous malformations: clinical aspects – Meier – Cardiovascular Diagnosis and Therapy

  https://cdt.amegroups.org/article/view/18281/html

  Hereditary hemorrhagic telangiectasia (HHT) is a genetic disorder of blood vessel formation resulting in mucocutaneous telangiectasias and visceral arteriovenous malformations. […] Management hinges on timely screening and diagnosis, followed by treatment of amenable PAVMs with transcatheter embolization in conjunction with medical management and prophylactic measures to treat and prevent complications. […] Effective screening is necessary to identify which HHT patients have PAVMs so that their dire clinical consequences can be avoided. Transthoracic contrast echocardiography (TTCE) is the preferred method for PAVM screening because of its high sensitivity and excellent negative predictive value. […] Steps to reduce embolic complications are recommended for all patients with PAVMs and for patients with confirmed or suspected HHT who have not yet been screened for PAVMs. To prevent septic embolic complications, antibiotics are recommended before procedures that may result in bacteremia. […] Prophylactic measures are advised for all patients after positive screening regardless of CT results.

• #35 Hereditary Hemorrhagic Telangiectasia: Diagnosis and Management | AAFP

  https://www.aafp.org/pubs/afp/issues/2010/1001/p785.html

  Transthoracic contrast echocardiography should be used as the initial screening test for pulmonary AVMs. […] Adults with possible or confirmed HHT should receive MRI to screen for cerebrovascular AVMs using a protocol with and without contrast media administration and using sequences that detect blood products to maximize sensitivity. […] Patients with documented pulmonary AVMs (treated or untreated) should be advised to use antibiotic prophylaxis for any procedures that have a risk of bacteremia (e.g., dental); to have air filters on all intravenous lines; and to avoid scuba diving because of the risk of decompression sickness. […] Screening should include contrast echocardiography and MRI of the brain. […] Patients who have AVMs, as well as those who have not yet been screened, should receive antibiotic prophylaxis before dental work or other dirty medical procedures to avoid the possible development of brain infection and abscess formation. […] The current recommendation is to treat any AVM that is at least 3 mm in diameter, although some physicians will treat AVMs as small as 1 mm.

• #36 Hereditary Hemorrhagic Telangiectasia: Diagnosis and Management | AAFP

  https://www.aafp.org/pubs/afp/issues/2010/1001/p785.html

  Transthoracic contrast echocardiography should be used as the initial screening test for pulmonary AVMs. […] Adults with possible or confirmed HHT should receive MRI to screen for cerebrovascular AVMs using a protocol with and without contrast media administration and using sequences that detect blood products to maximize sensitivity. […] Patients with documented pulmonary AVMs (treated or untreated) should be advised to use antibiotic prophylaxis for any procedures that have a risk of bacteremia (e.g., dental); to have air filters on all intravenous lines; and to avoid scuba diving because of the risk of decompression sickness. […] Screening should include contrast echocardiography and MRI of the brain. […] Patients who have AVMs, as well as those who have not yet been screened, should receive antibiotic prophylaxis before dental work or other dirty medical procedures to avoid the possible development of brain infection and abscess formation. […] The current recommendation is to treat any AVM that is at least 3 mm in diameter, although some physicians will treat AVMs as small as 1 mm.

• #37 Osler-Weber-Rendu Disease (Hereditary Hemorrhagic Telangiectasia) Guidelines: Second International Hereditary Hemorrhagic Telangiectasia Guidelines

  https://emedicine.medscape.com/article/2048472-guidelines

  Offer screening for liver vascular malformations (VMs) to adults with definite or suspected HHT. […] Provide intensive first-line management only for patients with complicated and/or symptomatic liver VMs, and tailored such management to the type of liver VM complication(s). […] It is recommended that HHT patients with high-output cardiac failure and pulmonary hypertension be comanaged by the HHT Center of Excellence and an HHT cardiologist or a pulmonary hypertension specialty clinic. […] Consider IV bevacizumab for patients with symptomatic high-output cardiac failure due to liver VMs who have not responded sufficiently to first-line management. […] Refer patients with symptomatic complications of liver VMs (eg, refractory high-output cardiac failure, biliary ischemia, or complicated portal hypertension) for consideration of liver transplantation.

• #38 Hereditary Hemorrhagic Telangiectasia: Diagnosis and Management | AAFP

  https://www.aafp.org/pubs/afp/issues/2010/1001/p785.html

  Transthoracic contrast echocardiography should be used as the initial screening test for pulmonary AVMs. […] Adults with possible or confirmed HHT should receive MRI to screen for cerebrovascular AVMs using a protocol with and without contrast media administration and using sequences that detect blood products to maximize sensitivity. […] Patients with documented pulmonary AVMs (treated or untreated) should be advised to use antibiotic prophylaxis for any procedures that have a risk of bacteremia (e.g., dental); to have air filters on all intravenous lines; and to avoid scuba diving because of the risk of decompression sickness. […] Screening should include contrast echocardiography and MRI of the brain. […] Patients who have AVMs, as well as those who have not yet been screened, should receive antibiotic prophylaxis before dental work or other dirty medical procedures to avoid the possible development of brain infection and abscess formation. […] The current recommendation is to treat any AVM that is at least 3 mm in diameter, although some physicians will treat AVMs as small as 1 mm.

• #39 Osler-Weber-Rendu Disease (Hereditary Hemorrhagic Telangiectasia) Guidelines: Second International Hereditary Hemorrhagic Telangiectasia Guidelines

  https://emedicine.medscape.com/article/2048472-guidelines

  Consider septodermoplasty for epistaxis that has not responded sufficiently to moisturizing topical therapies, ablative therapies, or TXA. […] Consider nasal closure for epistaxis that has not responded sufficiently to moisturizing topical therapies, ablative therapies, or TXA. […] Test for iron deficiency and anemia in all adult HHT patients, regardless of symptoms, and in all pediatric HHT patients with recurrent bleeding and/or symptoms of anemia. […] Provide HHT patients with anticoagulation (prophylactic or therapeutic) or antiplatelet therapy when there is an indication, with individualized bleeding risks taken into consideration; bleeding in HHT is not an absolute contraindication for these therapies. […] Where possible, avoid the use of dual antiplatelet therapy (DAPT) and/or a combination of antiplatelet therapy and anticoagulation.

• #40 Osler-Weber-Rendu syndrome Information | Mount Sinai – New York

  https://www.mountsinai.org/health-library/diseases-conditions/osler-weber-rendu-syndrome

  Genetic counseling is recommended for couples who want to have children and who have a family history of HHT. […] If you have this condition, medical treatments can prevent certain types of strokes and heart failure.

• #41 Hereditary Hemorrhagic Telangiectasia (HHT) (Osler-Weber-Rendu) – MD Searchlight

  https://mdsearchlight.com/genetic-disorders/hereditary-hemorrhagic-telangiectasia-hht-osler-weber-rendu/

  People diagnosed with HHT (Hereditary Hemorrhagic Telangiectasia) need to understand the potential impact of this diagnosis. As HHT is an inherited disease, meaning its passed down from parents to their children, its crucial to also check other family members for signs of this condition. Additionally, its recommended to receive genetic counseling before starting a family. Discovering if you have HHT before symptoms appear allows for quicker testing and treatment, and it can also help identify other family members who may be at risk.

• #42 Hereditary haemorrhagic telangiectasia: diagnosis, screening and management | Medicine Today

  https://medicinetoday.com.au/mt/2023/may/regular-series/hereditary-haemorrhagic-telangiectasia-diagnosis-screening-and-management

  When a causative mutation has been identified in the proband, predictive testing can be considered in relatives. […] Preimplantation diagnosis is an option for patients with HHT wish to be parents and to ensure the causative mutation is not passed on to offspring. […] There are no standard medical therapies for HHT given the few randomised trials in this field. […] Management can include supportive care, lesion-specific therapy and systemic treatment. […] Supportive measures include iron supplementation (oral and intravenous initially, if not effective) and red blood cell transfusion for bleeding and anaemia. […] Patients with HHT who are reviewed in a multidisciplinary team clinic are provided with education on nasal care, consisting of the use of regular nasal lubricants, such as petroleum jelly, paw paw-containing ointment or moisturising nasal sprays.

• #43 Hereditary hemorrhagic telangiectasia and pregnancy: potential adverse | IJWH

  https://www.dovepress.com/hereditary-hemorrhagic-telangiectasia-and-pregnancy-potential-adverse–peer-reviewed-fulltext-article-IJWH

  Pregnancy in patients with HHT is considered high risk. This is due to the possibility of life threatening complications. However, most pregnancies proceed without issue. […] Women with HHT appear to be most vulnerable to complications in the second and third trimesters due to physiologic adaptations which occur in pregnancy. During the latter trimesters, peripheral vascular resistance decreases. In addition, cardiac output increases nearly 50% by the end of the second trimester. These physiologic changes may exacerbate existing shunting lesions. For example, high output heart failure secondary to hepatic AVMs could occur. […] All women with HHT considering pregnancy should be screened for pulmonary AVMs with a thoracic computerized tomography scan to reduce complications such as stroke and hemorrhage; if pulmonary AVMs are found, these women should be treated prior to conception. However, if women are already pregnant and found to have asymptomatic pulmonary AVMs, they should not be treated during pregnancy.

• #44 Hereditary hemorrhagic telangiectasia and pregnancy: potential adverse | IJWH

  https://www.dovepress.com/hereditary-hemorrhagic-telangiectasia-and-pregnancy-potential-adverse–peer-reviewed-fulltext-article-IJWH

  Pregnancy in patients with HHT is considered high risk. This is due to the possibility of life threatening complications. However, most pregnancies proceed without issue. […] Women with HHT appear to be most vulnerable to complications in the second and third trimesters due to physiologic adaptations which occur in pregnancy. During the latter trimesters, peripheral vascular resistance decreases. In addition, cardiac output increases nearly 50% by the end of the second trimester. These physiologic changes may exacerbate existing shunting lesions. For example, high output heart failure secondary to hepatic AVMs could occur. […] All women with HHT considering pregnancy should be screened for pulmonary AVMs with a thoracic computerized tomography scan to reduce complications such as stroke and hemorrhage; if pulmonary AVMs are found, these women should be treated prior to conception. However, if women are already pregnant and found to have asymptomatic pulmonary AVMs, they should not be treated during pregnancy.

• #45 Hereditary hemorrhagic telangiectasia and pregnancy: potential adverse | IJWH

  https://www.dovepress.com/hereditary-hemorrhagic-telangiectasia-and-pregnancy-potential-adverse–peer-reviewed-fulltext-article-IJWH

  Pregnancy in patients with HHT is considered high risk. This is due to the possibility of life threatening complications. However, most pregnancies proceed without issue. […] Women with HHT appear to be most vulnerable to complications in the second and third trimesters due to physiologic adaptations which occur in pregnancy. During the latter trimesters, peripheral vascular resistance decreases. In addition, cardiac output increases nearly 50% by the end of the second trimester. These physiologic changes may exacerbate existing shunting lesions. For example, high output heart failure secondary to hepatic AVMs could occur. […] All women with HHT considering pregnancy should be screened for pulmonary AVMs with a thoracic computerized tomography scan to reduce complications such as stroke and hemorrhage; if pulmonary AVMs are found, these women should be treated prior to conception. However, if women are already pregnant and found to have asymptomatic pulmonary AVMs, they should not be treated during pregnancy.

• #46 Hereditary hemorrhagic telangiectasia and pregnancy: potential adverse | IJWH

  https://www.dovepress.com/hereditary-hemorrhagic-telangiectasia-and-pregnancy-potential-adverse–peer-reviewed-fulltext-article-IJWH

  Pregnancy in patients with HHT is considered high risk. This is due to the possibility of life threatening complications. However, most pregnancies proceed without issue. […] Women with HHT appear to be most vulnerable to complications in the second and third trimesters due to physiologic adaptations which occur in pregnancy. During the latter trimesters, peripheral vascular resistance decreases. In addition, cardiac output increases nearly 50% by the end of the second trimester. These physiologic changes may exacerbate existing shunting lesions. For example, high output heart failure secondary to hepatic AVMs could occur. […] All women with HHT considering pregnancy should be screened for pulmonary AVMs with a thoracic computerized tomography scan to reduce complications such as stroke and hemorrhage; if pulmonary AVMs are found, these women should be treated prior to conception. However, if women are already pregnant and found to have asymptomatic pulmonary AVMs, they should not be treated during pregnancy.

• #47 Hereditary hemorrhagic telangiectasia and pregnancy: potential adverse | IJWH

  https://www.dovepress.com/hereditary-hemorrhagic-telangiectasia-and-pregnancy-potential-adverse–peer-reviewed-fulltext-article-IJWH

  For all pregnant women with HHT, antibiotic prophylaxis during delivery is recommended. Also, avoidance of a prolonged stage of labor in HHT patients in whom brain AVM has not been excluded is advised. […] The majority of pregnancies in patients with HHT have no complications. However, pregnancy is high risk due to the possibility of severe, life threatening complications. Thus, women with HHT should be appropriately counseled on the small but serious risks. In addition, it is important for these patients to be appropriately managed since prior awareness of HHT has been associated with enhanced survival for the mother. […] HHT should be managed per international consensus guidelines. Women with HHT planning pregnancy should be advised that risks are low but serious. However, they should be reassured that close obstetric care and prior awareness of the diagnosis of HHT is usually associated with good pregnancy outcomes.

• #48 Hereditary hemorrhagic telangiectasia and pregnancy: potential adverse | IJWH

  https://www.dovepress.com/hereditary-hemorrhagic-telangiectasia-and-pregnancy-potential-adverse–peer-reviewed-fulltext-article-IJWH

  For all pregnant women with HHT, antibiotic prophylaxis during delivery is recommended. Also, avoidance of a prolonged stage of labor in HHT patients in whom brain AVM has not been excluded is advised. […] The majority of pregnancies in patients with HHT have no complications. However, pregnancy is high risk due to the possibility of severe, life threatening complications. Thus, women with HHT should be appropriately counseled on the small but serious risks. In addition, it is important for these patients to be appropriately managed since prior awareness of HHT has been associated with enhanced survival for the mother. […] HHT should be managed per international consensus guidelines. Women with HHT planning pregnancy should be advised that risks are low but serious. However, they should be reassured that close obstetric care and prior awareness of the diagnosis of HHT is usually associated with good pregnancy outcomes.

• #49 Bleeding and clotting in hereditary hemorrhagic telangiectasia

  https://www.wjgnet.com/2307-8960/full/v3/i4/330.htm

  Despite the presence of an overwhelming bleeding propensity, HHT patients also suffer from thrombotic complications. […] A recent survey found that 153 of 379 (40.4%) patients with HHT who received antiplatelet or anticoagulant therapy reported no change in epistaxis. […] This survey supports the reasoning that HHT patients who have a strong indication for antiplatelet or anticoagulant use, should not have these agents withheld. […] Due to the inherent bleeding risk in HHT patients, any approach to anticoagulation that may decrease the risk of bleeding would be prudent. […] In light of this, there is an important role for the use of factor IIa and Xa inhibitors in HHT patients requiring acute anticoagulation. […] For long-term anticoagulation to prevent VTE recurrence, the agents associated with the lowest risk of bleeding relative to placebo are low-dose warfarin, low-dose aspirin, and apixaban.

• #50 Osler-Weber-Rendu Disease (Hereditary Hemorrhagic Telangiectasia) Guidelines: Second International Hereditary Hemorrhagic Telangiectasia Guidelines

  https://emedicine.medscape.com/article/2048472-guidelines

  Consider septodermoplasty for epistaxis that has not responded sufficiently to moisturizing topical therapies, ablative therapies, or TXA. […] Consider nasal closure for epistaxis that has not responded sufficiently to moisturizing topical therapies, ablative therapies, or TXA. […] Test for iron deficiency and anemia in all adult HHT patients, regardless of symptoms, and in all pediatric HHT patients with recurrent bleeding and/or symptoms of anemia. […] Provide HHT patients with anticoagulation (prophylactic or therapeutic) or antiplatelet therapy when there is an indication, with individualized bleeding risks taken into consideration; bleeding in HHT is not an absolute contraindication for these therapies. […] Where possible, avoid the use of dual antiplatelet therapy (DAPT) and/or a combination of antiplatelet therapy and anticoagulation.

• #51 Osler-Weber-Rendu Disease (Hereditary Hemorrhagic Telangiectasia) Guidelines: Second International Hereditary Hemorrhagic Telangiectasia Guidelines

  https://emedicine.medscape.com/article/2048472-guidelines

  Consider septodermoplasty for epistaxis that has not responded sufficiently to moisturizing topical therapies, ablative therapies, or TXA. […] Consider nasal closure for epistaxis that has not responded sufficiently to moisturizing topical therapies, ablative therapies, or TXA. […] Test for iron deficiency and anemia in all adult HHT patients, regardless of symptoms, and in all pediatric HHT patients with recurrent bleeding and/or symptoms of anemia. […] Provide HHT patients with anticoagulation (prophylactic or therapeutic) or antiplatelet therapy when there is an indication, with individualized bleeding risks taken into consideration; bleeding in HHT is not an absolute contraindication for these therapies. […] Where possible, avoid the use of dual antiplatelet therapy (DAPT) and/or a combination of antiplatelet therapy and anticoagulation.

• #52 Osler-Weber-Rendu Disease (Hereditary Hemorrhagic Telangiectasia) Guidelines: Second International Hereditary Hemorrhagic Telangiectasia Guidelines

  https://emedicine.medscape.com/article/2048472-guidelines

  Consider septodermoplasty for epistaxis that has not responded sufficiently to moisturizing topical therapies, ablative therapies, or TXA. […] Consider nasal closure for epistaxis that has not responded sufficiently to moisturizing topical therapies, ablative therapies, or TXA. […] Test for iron deficiency and anemia in all adult HHT patients, regardless of symptoms, and in all pediatric HHT patients with recurrent bleeding and/or symptoms of anemia. […] Provide HHT patients with anticoagulation (prophylactic or therapeutic) or antiplatelet therapy when there is an indication, with individualized bleeding risks taken into consideration; bleeding in HHT is not an absolute contraindication for these therapies. […] Where possible, avoid the use of dual antiplatelet therapy (DAPT) and/or a combination of antiplatelet therapy and anticoagulation.

• #53 Safety of direct oral anticoagulants in patients with hereditary hemorrhagic telangiectasia | Orphanet Journal of Rare Diseases | Full Text

  https://ojrd.biomedcentral.com/articles/10.1186/s13023-019-1179-1

  Currently, conventional heparin and warfarin remain first choice anticoagulants in HHT. […] If newer anticoagulants are considered, although study numbers are small, at this stage Apixaban appears to be associated with lesser bleeding risk than Rivaroxaban. […] Expert HHT Centres recommend that HHT patients should receive prophylactic anticoagulation at high risk times, as for the general population. […] Trials of full anticoagulation are also generally recommended by expert Centres, noting that additional ENT treatments may be required to enable patients to tolerate anticoagulation. […] The oral anticoagulant of choice in HHT is warfarin, due to its tolerance data and a reversal agent that is widely available; […] Of the new direct oral anticoagulants, Apixaban appears to be associated with lesser bleeding complications/risk than Rivaroxaban; […] If an HHT patient suffers excessive nosebleeds with one particular anticoagulant, they may successfully switch to an alternate agent, though it is not currently possible to predict which agent will best suit an individual.

• #54 Safety of direct oral anticoagulants in patients with hereditary hemorrhagic telangiectasia | Orphanet Journal of Rare Diseases | Full Text

  https://ojrd.biomedcentral.com/articles/10.1186/s13023-019-1179-1

  Currently, conventional heparin and warfarin remain first choice anticoagulants in HHT. […] If newer anticoagulants are considered, although study numbers are small, at this stage Apixaban appears to be associated with lesser bleeding risk than Rivaroxaban. […] Expert HHT Centres recommend that HHT patients should receive prophylactic anticoagulation at high risk times, as for the general population. […] Trials of full anticoagulation are also generally recommended by expert Centres, noting that additional ENT treatments may be required to enable patients to tolerate anticoagulation. […] The oral anticoagulant of choice in HHT is warfarin, due to its tolerance data and a reversal agent that is widely available; […] Of the new direct oral anticoagulants, Apixaban appears to be associated with lesser bleeding complications/risk than Rivaroxaban; […] If an HHT patient suffers excessive nosebleeds with one particular anticoagulant, they may successfully switch to an alternate agent, though it is not currently possible to predict which agent will best suit an individual.

• #55 Hereditary hemorrhagic telangiectasia, embolization, and Young’s procedure: oral surgical management | Journal of Oral Medicine and Oral Surgery

  https://www.jomos.org/articles/mbcb/full_html/2018/02/mbcb170009/mbcb170009.html

  Hereditary hemorrhagic telangiectasia (HHT) case with history of embolization and Youngs procedure: surgical management. […] The management of patients affected by HHT needs rigorous hemostatic methods and outpatient postoperative monitoring. […] In patients with HHT, two types of risks are present: hemorrhagic risk and infectious risk. […] The hemorrhagic risk: This is linked to capillary fragility. It can be prevented by proper oral hygiene and regular follow-up. In cases where surgery was performed, local methods of hemostasis must be implemented systematically; […] Although no randomized trials have been performed for antibiotic prophylaxis in HHT, it is advisable before any invasive procedure. […] This clinical case reminds us that patients who have HHT require rigorous methods of hemostasis in addition to regular postoperative outpatient follow-up. A periodical dental check-up is recommended to minimize the need for tooth extraction.

• #56 Hereditary Hemorrhagic Telangiectasia (also known as HHT, Osler-Weber-Rendu syndrome, Osler-Weber-Rendu disease, Osler-Rendu disease, Osler’s disease, Weber-Osler disease, and Rendu-Osler-Weber syndrome) – CDHO

  https://cdho.org/factsheets/hereditary-hemorrhagic-telangiectasia/

  Is medical consult advised? Yes, liaison with the patient/clients HHT specialist is advisable regarding the type, severity, and management of the patient/clients HHT before undertaking dental hygiene treatment for the first time. (This includes bleeding risk, if any, for fitting a mouth guard and taking an impression.) Medical consult is also warranted for suspicious, but as yet undiagnosed, bleeding disorder, and if known disease is poorly controlled. […] Is the initiation of invasive dental hygiene procedures contra-indicated? Yes. This is a bleeding disorder that may affect appropriateness or safety, and scaling and root planing, including curetting of surrounding tissue, are contraindicated until the patient/client is medically cleared. In some cases, antibiotic prophylaxis is also required.

• #57 Hereditary Hemorrhagic Telangiectasia (also known as HHT, Osler-Weber-Rendu syndrome, Osler-Weber-Rendu disease, Osler-Rendu disease, Osler’s disease, Weber-Osler disease, and Rendu-Osler-Weber syndrome) – CDHO

  https://cdho.org/factsheets/hereditary-hemorrhagic-telangiectasia/

  Is medical consult advised? Yes, liaison with the patient/clients HHT specialist is advisable regarding the type, severity, and management of the patient/clients HHT before undertaking dental hygiene treatment for the first time. (This includes bleeding risk, if any, for fitting a mouth guard and taking an impression.) Medical consult is also warranted for suspicious, but as yet undiagnosed, bleeding disorder, and if known disease is poorly controlled. […] Is the initiation of invasive dental hygiene procedures contra-indicated? Yes. This is a bleeding disorder that may affect appropriateness or safety, and scaling and root planing, including curetting of surrounding tissue, are contraindicated until the patient/client is medically cleared. In some cases, antibiotic prophylaxis is also required.

• #58 Hereditary Hemorrhagic Telangiectasia (also known as HHT, Osler-Weber-Rendu syndrome, Osler-Weber-Rendu disease, Osler-Rendu disease, Osler’s disease, Weber-Osler disease, and Rendu-Osler-Weber syndrome) – CDHO

  https://cdho.org/factsheets/hereditary-hemorrhagic-telangiectasia/

  Is antibiotic prophylaxis required? Yes, if there are pulmonary arteriovenous malformations (PAVMs), whether treated or untreated, or if the patient/client has not yet been screened at a specialized HHT centre. (Blood-borne bacteria resulting from invasive procedures can pass through PAVMs, lodge in the brain, and cause brain abscesses.) If the patient/client has been screened for PAVMs and the tests confirm that there are none, then prophylactic antibiotics are not required. […] Is postponing treatment advised? Possibly. If the patient/client with HHT has not had the appropriate diagnostic tests for pulmonary AVMs, ideally dental hygiene treatment should be delayed until testing occurs. […] In order to reduce risk of bleeding from oral telangiectasias, the dental hygienist should advise the patient/client as follows: use a soft-bristled toothbrush, floss only in areas free of telangiectasias, use lip sunscreen to protect against sun exposure, avoid hard, irritating, and hot foods, keep dental prostheses (such as mouth guards) in good condition, ensuring proper fit and avoiding damage to the gingiva and irritation of telangiectasias.

• #59 Hereditary hemorrhagic telangiectasia, embolization, and Young’s procedure: oral surgical management | Journal of Oral Medicine and Oral Surgery

  https://www.jomos.org/articles/mbcb/full_html/2018/02/mbcb170009/mbcb170009.html

  Hereditary hemorrhagic telangiectasia (HHT) case with history of embolization and Youngs procedure: surgical management. […] The management of patients affected by HHT needs rigorous hemostatic methods and outpatient postoperative monitoring. […] In patients with HHT, two types of risks are present: hemorrhagic risk and infectious risk. […] The hemorrhagic risk: This is linked to capillary fragility. It can be prevented by proper oral hygiene and regular follow-up. In cases where surgery was performed, local methods of hemostasis must be implemented systematically; […] Although no randomized trials have been performed for antibiotic prophylaxis in HHT, it is advisable before any invasive procedure. […] This clinical case reminds us that patients who have HHT require rigorous methods of hemostasis in addition to regular postoperative outpatient follow-up. A periodical dental check-up is recommended to minimize the need for tooth extraction.

• #60 Hereditary Hemorrhagic Telangiectasia (HHT) > Fact Sheets > Yale Medicine

  https://www.yalemedicine.org/conditions/hht

  Because this condition doesnt cause any warning symptoms and leaves people vulnerable to stroke and life-threatening infections proper diagnosis, treatment, and monitoring is important. […] Patients with suspected HHT, such was those with family members having this condition, says Jeffrey Pollak, MD, interventional radiologist and medical director of the Yale Medicine HHT and Vascular Malformations Program, should be screened for this condition and specific sites of involvement, especially since sudden life-altering events that might occur even in the absence of any preceding symptoms can potentially be prevented if HHT is known and treated in advance. […] Early diagnosis, treatment, and follow up can help people avoid these complications. […] Once a family member is diagnosed, its recommended that other family members seek genetic counseling and screening.

• #61 Hereditary Hemorrhagic Telangiectasia (HHT) > Fact Sheets > Yale Medicine

  https://www.yalemedicine.org/conditions/hht

  Even after treatment, patients should be monitored for the rest of their lives, as certain manifestations may worsen over time and some may not develop until later in life. […] While there is no cure for HHT, Dr. Pollak says, together at Yale Medicine, we are working to prevent needless tragedies by diagnosing the disorder early and providing excellent treatment to patients and their families with continued monitoring.

• #62 Hereditary Hemorrhagic Telangiectasia (HHT) > Fact Sheets > Yale Medicine

  https://www.yalemedicine.org/conditions/hht

  Even after treatment, patients should be monitored for the rest of their lives, as certain manifestations may worsen over time and some may not develop until later in life. […] While there is no cure for HHT, Dr. Pollak says, together at Yale Medicine, we are working to prevent needless tragedies by diagnosing the disorder early and providing excellent treatment to patients and their families with continued monitoring.

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