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1.
BMJ Case Rep ; 14(1)2021 Jan 08.
Artículo en Inglés | MEDLINE | ID: mdl-33419752

RESUMEN

Hereditary haemorrhagic telangiectasia (HHT) also known as Osler-Weber-Rendu syndrome is an autosomal dominant disorder affecting 1 in 8000 individuals. The eponym recognises the 19th-century physicians William Osler, Henri Jules Louis Marie Rendu and Frederick Parkes Weber who each independently described the disease. It is characterised by epistaxis, telangiectasia and visceral arteriovenous malformations. Individuals with HHT have been found to have abnormal plasma concentrations of transforming growth factor beta and vascular endothelial growth factor secondary to mutations in ENG, ACVRL1 and MADH4. Pulmonary artery malformations (PAVMs) are abnormal communications between pulmonary arteries and veins and are found in up to 50% of individuals with HHT. The clinical features suggestive of PAVMs are stigmata of right to left shunting such as dyspnoea, hypoxaemia, cyanosis, cerebral embolism and unexplained haemoptysis or haemothorax. The authors present the case of a 33-year-old woman presenting with progressive dyspnoea during the COVID-19 pandemic. She had a typical presentation of HHT with recurrent epistaxis, telangiectasia and pulmonary arteriovenous malformations. Although rare, PAVM should be considered in individuals presenting to the emergency department with dyspnoea and hypoxaemia. Delayed diagnosis can result in fatal embolic and haemorrhagic complications.


Asunto(s)
Malformaciones Arteriovenosas/diagnóstico , Disnea/fisiopatología , Epistaxis/fisiopatología , Hipoxia/fisiopatología , Telangiectasia Hemorrágica Hereditaria/diagnóstico , Receptores de Activinas Tipo II/genética , Adulto , Antitiroideos/uso terapéutico , Malformaciones Arteriovenosas/fisiopatología , Análisis de los Gases de la Sangre , COVID-19/diagnóstico , Carbimazol/uso terapéutico , Diagnóstico Diferencial , Femenino , Enfermedad de Graves/complicaciones , Enfermedad de Graves/tratamiento farmacológico , Humanos , Trastornos Migrañosos/complicaciones , Arteria Pulmonar/anomalías , Arteria Pulmonar/diagnóstico por imagen , Venas Pulmonares/anomalías , Venas Pulmonares/diagnóstico por imagen , SARS-CoV-2 , Telangiectasia Hemorrágica Hereditaria/complicaciones , Telangiectasia Hemorrágica Hereditaria/fisiopatología , Tomografía Computarizada por Rayos X
2.
Eur Radiol ; 30(8): 4466-4474, 2020 Aug.
Artículo en Inglés | MEDLINE | ID: mdl-32279114

RESUMEN

OBJECTIVES: To investigate MRI features in discriminating chronic invasive fungal rhinosinusitis (CIFRS) from sinonasal squamous cell carcinomas (SNSCC). METHODS: MRI findings of 33 patients with CIFRS and 47 patients with SNSCC were retrospectively reviewed and compared. Multivariate logistic regression analysis was performed to identify significant imaging features in distinguishing between CIFRS and SNSCC. The ROC curves and the AUC were used to evaluate diagnostic performance. RESULTS: There were significant differences in cavernous sinus involvement (p < 0.001), sphenoid sinus involvement (p < 0.001), meningeal involvement (p = 0.024), T2 signal intensity (p = 0.006), and enhancement pattern (p < 0.001) between CIFRS and SNSCC. Multivariate logistic regression analysis identified cavernous sinus involvement (odds ratio [OR] = 0.06, 95% confidence interval [95% CI] = 0.02-0.20) and sphenoid sinus involvement (OR = 0.14, 95% CI = 0.05-0.45) as significant indicators for CIFRS and T2 isointensity to gray matter (OR = 4.44, 95% CI = 1.22-16.22) was a significant indicator for SNSCC. ROC curve analysis showed the AUC from a combination of three imaging features was 0.95 in differentiating CIFRS and SNSCC. CONCLUSIONS: MRI showed significant differences between CIFRS and SNSCC features. In immunocompromised patients, a sinonasal hypointense mass on T2WI with septal enhancement or loss of contrast enhancement, and involvement of cavernous sinus, sphenoid sinus, and meninges strongly suggest CIFRS. KEY POINTS: • Chronic invasive fungal rhinosinusitis (CIFRS) is often difficult to distinguish from sinonasal squamous cell carcinomas (SNSCC) in clinical practice. • Cavernous sinus and sphenoid sinus involvement appear to be significant indicators for CIFRS. T2 isointensity to gray matter appears to be a significant indicator for SNSCC. • Loss of contrast enhancement and septal enhancement can be used to distinguish CIFRS from SNSCC with a high degree of specificity.


Asunto(s)
Diagnóstico Diferencial , Infecciones Fúngicas Invasoras/diagnóstico por imagen , Neoplasias de los Senos Paranasales/diagnóstico por imagen , Rinitis/diagnóstico por imagen , Sinusitis/diagnóstico por imagen , Carcinoma de Células Escamosas de Cabeza y Cuello/diagnóstico por imagen , Adulto , Anciano , Aspergilosis/diagnóstico por imagen , Aspergilosis/inmunología , Aspergilosis/fisiopatología , Seno Cavernoso/diagnóstico por imagen , Enfermedad Crónica , Epistaxis/fisiopatología , Dolor Facial/fisiopatología , Femenino , Cefalea/fisiopatología , Humanos , Huésped Inmunocomprometido , Infecciones Fúngicas Invasoras/inmunología , Infecciones Fúngicas Invasoras/fisiopatología , Modelos Logísticos , Imagen por Resonancia Magnética , Masculino , Meninges/diagnóstico por imagen , Persona de Mediana Edad , Mucormicosis/diagnóstico por imagen , Mucormicosis/inmunología , Mucormicosis/fisiopatología , Análisis Multivariante , Obstrucción Nasal/fisiopatología , Neoplasias Nasales/diagnóstico por imagen , Neoplasias Nasales/fisiopatología , Neoplasias de los Senos Paranasales/fisiopatología , Estudios Retrospectivos , Rinitis/inmunología , Rinitis/fisiopatología , Rinorrea/fisiopatología , Sinusitis/inmunología , Sinusitis/fisiopatología , Seno Esfenoidal/diagnóstico por imagen , Carcinoma de Células Escamosas de Cabeza y Cuello/fisiopatología , Trastornos de la Visión/fisiopatología
3.
Zh Nevrol Psikhiatr Im S S Korsakova ; 120(3. Vyp. 2): 13-15, 2020.
Artículo en Ruso | MEDLINE | ID: mdl-32307424

RESUMEN

The aspects of disruption of venous blood outflow from face region into skull are well-known; they can occur at any age - from neonatal to old age, however, the possible compensation mechanisms for such disorders are usually difficult to identify. A description is given of a unique case of unilateral epistaxis and a homolateral (right-sided) headache that developed much later. Examination revealed hypoplasia of the left sigmoid sinus and blood overflow of the right sigmoid sinus. After prescription of betahistine in a dose of 24 mg before bedtime and after awakening the headache and nosebleeds disappeared on the second day. The effect was persistent. The case demonstrates previously unknown compensation mechanisms for venous outflow - a retrograde outflow from the skull to the face region.


Asunto(s)
Cara/irrigación sanguínea , Cráneo/irrigación sanguínea , Venas/fisiología , Betahistina/uso terapéutico , Senos Craneales/patología , Senos Craneales/fisiopatología , Epistaxis/tratamiento farmacológico , Epistaxis/fisiopatología , Cara/patología , Cefalea/tratamiento farmacológico , Cefalea/patología , Cefalea/fisiopatología , Humanos , Cráneo/patología
4.
Rheumatol Int ; 40(2): 303-311, 2020 Feb.
Artículo en Inglés | MEDLINE | ID: mdl-31385079

RESUMEN

The different sets of criteria for diagnosis or classification of anti-neutrophil cytoplasmic antibody-associated vasculitis (AAV) lead to numerous overlapping and reclassified diagnoses in clinical practice. We designed this study to assess the difficulties in classifying patients with AAV. As a secondary objective, different variables were tested to predict prognosis. We conducted a retrospective chart review in a Western Spain multicentre survey. A total of 115 adult patients diagnosed with AAV from 2002 to 2013 and followed for at least 3 years were included. They were classified according to (1) Chapel Hill Consensus Conference (CHCC), (2) European Medicines Agency algorithm and (3) French Vasculitis Study Group/European Vasculitis Society phenotypes. Fifty-three patients (46%) had neither distinctive histopathological data of a single AAV definition nor any surrogate markers for granulomatous inflammation and thus did not fulfill any diagnostic criteria. Ocular, ear, nose, throat, skin, and lung involvement were more frequent with proteinase 3 (PR3) antibodies, whereas peripheral neuropathy was more frequent with myeloperoxidase (MPO) antibodies. When the disease was severe at diagnosis, the HR for mortality was 10.44. When induction treatment was not given in accordance with the guidelines, the HR for mortality was 4.00. For maintenance treatment, the HR was 5.49 for mortality and 2.48 for relapse. AAV classification is difficult because many patients had neither specific clinical data nor distinctive histological features of a single CHCC definition. A structured clinical assessment of patient severity is the best tool to guide the management of AAV.


Asunto(s)
Vasculitis Asociada a Anticuerpos Citoplasmáticos Antineutrófilos/clasificación , Vasculitis Asociada a Anticuerpos Citoplasmáticos Antineutrófilos/patología , Vasculitis Asociada a Anticuerpos Citoplasmáticos Antineutrófilos/fisiopatología , Mortalidad , Anciano , Vasculitis Asociada a Anticuerpos Citoplasmáticos Antineutrófilos/inmunología , Anticuerpos Anticitoplasma de Neutrófilos/inmunología , Síndrome de Churg-Strauss/clasificación , Síndrome de Churg-Strauss/inmunología , Síndrome de Churg-Strauss/patología , Síndrome de Churg-Strauss/fisiopatología , Epistaxis/inmunología , Epistaxis/patología , Epistaxis/fisiopatología , Oftalmopatías/inmunología , Oftalmopatías/patología , Oftalmopatías/fisiopatología , Femenino , Enfermedades Gastrointestinales/inmunología , Enfermedades Gastrointestinales/patología , Enfermedades Gastrointestinales/fisiopatología , Granulomatosis con Poliangitis/clasificación , Granulomatosis con Poliangitis/inmunología , Granulomatosis con Poliangitis/patología , Granulomatosis con Poliangitis/fisiopatología , Humanos , Hipertensión/inmunología , Hipertensión/patología , Hipertensión/fisiopatología , Enfermedades Renales/inmunología , Enfermedades Renales/patología , Enfermedades Renales/fisiopatología , Fallo Renal Crónico/fisiopatología , Enfermedades Pulmonares/inmunología , Enfermedades Pulmonares/patología , Enfermedades Pulmonares/fisiopatología , Masculino , Poliangitis Microscópica/clasificación , Poliangitis Microscópica/inmunología , Poliangitis Microscópica/patología , Poliangitis Microscópica/fisiopatología , Persona de Mediana Edad , Mieloblastina/inmunología , Enfermedades del Sistema Nervioso Periférico/inmunología , Enfermedades del Sistema Nervioso Periférico/patología , Enfermedades del Sistema Nervioso Periférico/fisiopatología , Peroxidasa/inmunología , Prevención Primaria , Pronóstico , Modelos de Riesgos Proporcionales , Recurrencia , Estudios Retrospectivos , Índice de Severidad de la Enfermedad , Sinusitis/inmunología
5.
Pediatr Hematol Oncol ; 36(5): 267-276, 2019 Aug.
Artículo en Inglés | MEDLINE | ID: mdl-31339399

RESUMEN

Background: Sickle cell anaemia (SCA) is the most common genetic diseases in the Democratic Republic of Congo (DRC). It is estimated 30,000 to 40,000 neonates with SCA are born annually. Despite this high incidence rate, and the severity of the Bantu haplotype found in Congolese patients, major clinical characteristics remain poorly defined. The objective of this study was to assess the clinical and haematological profile of the SCA in patients less than 24 years of age. Methods: A cross-sectional study was conducted in Kinshasa, the large city of the DRC. Patients were consecutively selected in three health institutions. Results: The study includes 256 sickle cell patients. The mean age of 8.4 (SD = 4.9) years. The Hand-foot syndrome was most common (52.7%) first presentation revealing the disease in our series. The most prevalent crises found in our series were vaso-occlusive crises (VOC) in 170 cases (66.4%) and severe hemolysis in 136 cases (53.1%). Splenic sequestration was noted in 19 cases (7.4%). The age at the first pain crisis was 18.2±15.2 months-of-age and the age at the first transfusions was 29.2±27.6 months-of -age. The most common signs associated with sickle cell disease in our series were hepatomegaly (53.9%), splenomegaly (41.7%), and adenotonsillar hypertrophy (34.8%). Epistaxis was reported in 9.4%. Conclusion: The clinical course of patients in DRC was comparable to reports from Western countries, with the notable exception of epistaxis which was significantly higher in patients in the DRC.


Asunto(s)
Anemia de Células Falciformes , Epistaxis , Adolescente , Anemia de Células Falciformes/epidemiología , Anemia de Células Falciformes/fisiopatología , Anemia de Células Falciformes/terapia , Niño , Preescolar , Congo/epidemiología , Estudios Transversales , República Democrática del Congo , Epistaxis/epidemiología , Epistaxis/fisiopatología , Epistaxis/terapia , Femenino , Humanos , Lactante , Masculino , Prevalencia
6.
Ann Hepatol ; 18(4): 613-619, 2019.
Artículo en Inglés | MEDLINE | ID: mdl-31122880

RESUMEN

INTRODUCTION AND OBJECTIVES: Niemann-Pick disease type A (NPD-A) and B (NPD-B) are lysosomal storage diseases with a birth prevalence of 0.4-0.6/100,000. They are caused by a deficiency in acid sphingomyelinase, an enzyme encoded by SMPD1. We analyzed the phenotype and genotype of four unrelated Mexican patients, one with NPD-A and three with NPD-B. PATIENTS AND METHODS: Four female patients between 1 and 7 years of age were diagnosed with NPD-A or NPD-B by hepatosplenomegaly, among other clinical characteristics, and by determining the level of acid sphingomyelinase enzymatic activity and sequencing of the SMPD1 gene. Additionally, a 775bp amplicon of SMPD1 (from 11:6393835_6394609, including exons 5 and 6) was analyzed by capillary sequencing in a control group of 50 unrelated healthy Mexican Mestizos. RESULTS: An infrequent variant (c.1343A>G p.Tyr448Cys) was observed in two patients. One is the first NPD-A homozygous patient reported with this variant and the other a compound heterozygous NPD-B patient with the c.1829_1831delGCC p.Arg610del variant. Another compound heterozygous patient had the c.1547A>G p.His516Arg variant (not previously described in affected individuals) along with the c.1805G>A p.Arg602His variant. A new c.1263+8C>T pathogenic variant was encountered in a homozygous state in a NPD-B patient. Among the healthy control individuals there was a heterozygous carrier for the c.1550A>T (rs142787001) pathogenic variant, but none with the known pathogenic variants in the 11:6393835_6394609 region of SMPD1. CONCLUSIONS: The present study provides further NPD-A or B phenotype-genotype correlations. We detected a heterozygous carrier with a pathogenic variant in 1/50 healthy Mexican mestizos.


Asunto(s)
Enfermedad de Niemann-Pick Tipo A/genética , Enfermedad de Niemann-Pick Tipo B/genética , Esfingomielina Fosfodiesterasa/genética , Adolescente , Adulto , Niño , Preescolar , Epistaxis/fisiopatología , Femenino , Tamización de Portadores Genéticos , Genotipo , Trastornos del Crecimiento/fisiopatología , Voluntarios Sanos , Hepatomegalia/fisiopatología , Heterocigoto , Humanos , Lactante , Hígado/patología , Hígado/ultraestructura , México , Enfermedad de Niemann-Pick Tipo A/metabolismo , Enfermedad de Niemann-Pick Tipo A/patología , Enfermedad de Niemann-Pick Tipo A/fisiopatología , Enfermedad de Niemann-Pick Tipo B/metabolismo , Enfermedad de Niemann-Pick Tipo B/patología , Enfermedad de Niemann-Pick Tipo B/fisiopatología , Fenotipo , Esfingomielina Fosfodiesterasa/metabolismo , Esplenomegalia/fisiopatología , Adulto Joven
7.
Eur Arch Otorhinolaryngol ; 276(6): 1693-1699, 2019 Jun.
Artículo en Inglés | MEDLINE | ID: mdl-30888493

RESUMEN

PURPOSE: To investigate the clinical profile and outcomes of different treatment strategies in patients hospitalized for spontaneous severe epistaxis. METHODS: This is a retrospective descriptive study of a case series of patients hospitalized for epistaxis in the University Hospital of Ghent between 2005 and 2012. RESULTS: 124 patients with, respectively, 132 episodes were included. 64% were male. The mean age was 65 years. 73% had comorbidities of which arterial hypertension was the most common. 61% were taking one or more antithrombotics and in 25.7% a recent change in the medication schedule took place. 47% of the episodes necessitated a vascular intervention. The most performed surgery was endoscopic sphenopalatine artery ligation. The 1-year success rate of conservative treatment was 47% and of vascular intervention 81%. No significant difference between the recurrence rates and need for vascular intervention of the different comorbidities and medications was detected using Pearson chi-squared and Fisher's exact testing. The overall 5-year survival rate was 83.6%. CONCLUSIONS: The typical pattern of a patient presenting with severe epistaxis was a patient in the sixth decade, male, suffering from comorbidities and taking one or more antithrombotic agents. Based on the above-mentioned success rates of the different treatment options, we think all centres treating epistaxis should apply a well-defined protocol to guide the decision when to proceed with surgery. Furthermore, prospective research needs to precisely investigate the role played by comorbidities and their treatment in the occurrence of epistaxis and to test the effectiveness of proposed algorithms.


Asunto(s)
Embolización Terapéutica/métodos , Endoscopía/métodos , Epistaxis , Ligadura/métodos , Anciano , Bélgica/epidemiología , Comorbilidad , Epistaxis/diagnóstico , Epistaxis/epidemiología , Epistaxis/fisiopatología , Epistaxis/terapia , Femenino , Hospitales Universitarios/estadística & datos numéricos , Humanos , Masculino , Persona de Mediana Edad , Nariz/irrigación sanguínea , Evaluación de Procesos y Resultados en Atención de Salud , Recurrencia , Estudios Retrospectivos , Factores de Riesgo
8.
Orphanet J Rare Dis ; 14(1): 28, 2019 02 04.
Artículo en Inglés | MEDLINE | ID: mdl-30717761

RESUMEN

BACKGROUND: Hereditary hemorrhagic telangiectasia (HHT) is a multisystemic inherited vascular dysplasia that leads to nosebleeds and visceral arteriovenous malformations (AVMs). Anti-angiogenic drugs thalidomide and bevacizumab have been increasingly used off-label with variable results. The HHT working group within the ERN for Rare Multisystemic Vascular Diseases (VASCERN), developed a questionnaire-based retrospective capture of adverse events (AEs) classified using the Common Terminology Criteria for Adverse Events. RESULTS: Sixty-nine HHT patients received bevacizumab, 37 (50.6%) for high output cardiac failure/hepatic AVMs, and 32 (49.4%) for bleeding; the 69 patients received bevacizumab for a mean of 11 months for a total of 63.8 person/years treatment. 67 received thalidomide, all for epistaxis and/or gastrointestinal bleeding; they received thalidomide for a mean of 13.4 months/patient for a total of 75 person/years treatment. AEs were reported in 58 patients, 33 with bevacizumab, 37 with thalidomide. 32 grade 1-3 AEs related to bevacizumab were reported with an average incidence rate of 50 per 100 person-years. 34 grade 1-3 AEs related to thalidomide were reported with an average incidence rate of 45.3 per 100 person-years. Bevacizumab AEs were more common in females (27 AEs in 46 women) than males (6 in 23, p < 0.001). Thalidomide AEs occurred at more similar rates in males (25 AEs in 41 men, 60.9%) and females (12 in 26 (46.2%), but were more common in ENG patients (17 in 17) than in ACVRL1 (14 in 34, p < 0.0001). For bevacizumab, the most common reports were of joint pains (7/69, 10%), headache (3/69, 4.4%) and proteinuria (2/69, 3%), and for thalidomide, peripheral neuropathy (12/67, 18%); drowsiness (8/67, 12%); and dizziness (6/67, 9%). Fatal adverse events were more common in males (p = 0.009), and in patients with ENG pathogenic variants (p = 0.012). One fatal AE was possibly related to bevacizumab (average incidence rate: 1.5 per 100 person-years); 3 fatal AEs were possibly related to thalidomide (average incidence rate: 4 per 100 person-years). CONCLUSIONS: With potential increase in use of Bevacizumab and Thalidomide in HHT patients, data presented support appropriate weighing of the toxicities which can arise in HHT settings and the practice recommendations for their prevention and management.


Asunto(s)
Bevacizumab/efectos adversos , Telangiectasia Hemorrágica Hereditaria/fisiopatología , Talidomida/efectos adversos , Adolescente , Adulto , Bevacizumab/uso terapéutico , Epistaxis/tratamiento farmacológico , Epistaxis/metabolismo , Epistaxis/fisiopatología , Femenino , Hemorragia/tratamiento farmacológico , Hemorragia/metabolismo , Hemorragia/fisiopatología , Humanos , Masculino , Estudios Retrospectivos , Encuestas y Cuestionarios , Telangiectasia Hemorrágica Hereditaria/tratamiento farmacológico , Telangiectasia Hemorrágica Hereditaria/metabolismo , Talidomida/uso terapéutico , Adulto Joven
9.
Otolaryngol Head Neck Surg ; 160(1): 22-35, 2019 01.
Artículo en Inglés | MEDLINE | ID: mdl-30200816

RESUMEN

OBJECTIVES: The aim of this study (PROSPERO ID: CRD42017081952) was to evaluate medical treatment for epistaxis from hereditary hemorrhagic telangiectasia (HHT). DATA SOURCES: PubMed, Embase, Scopus, and Cochrane Library databases were interrogated from their inceptions to November 2017. REVIEW METHODS: Randomized clinical trials comparing medical treatment with placebo for epistaxis of HHT were included. We used a random-effects model to synthesize overall effects. Heterogeneity was evaluated with the I2 statistic. RESULTS: Eight studies were identified after systematic searching. The use of bevacizumab (BV), tranexamic acid, and estrogen, regardless of the route of administration, had no significant influence on frequency of episodes. Tamoxifen was superior to placebo in both frequency and severity of epistaxis. For duration of epistaxis, nasal spray BV, oral or nasal spray tranexamic acid, and nasal spray estrogen had no significant differences versus placebo, but patients receiving submucosal BV showed lower duration of epistaxis (mean difference: -219.00 min/mo, 95% CI: -271.90 to -166.10). Medical treatment for HHT had no significant changes of mean hemoglobin concentration (pooled mean difference: -0.23 mg/dL, 95% CI: -0.65 to 0.20, I2 = 0%) or quality of life (pooled standardized mean difference: 0.07, 95% CI: -0.16 to 0.30, I2 = 0%). CONCLUSIONS: Only limited evidence provides a benefit on frequency of epistaxis by treatment with tamoxifen and duration of epistaxis by treatment with submucosal BV among patients with HHT. Mean hemoglobin concentration and quality of life were not influenced by medical treatment.


Asunto(s)
Inhibidores de la Angiogénesis/administración & dosificación , Bevacizumab/administración & dosificación , Epistaxis/tratamiento farmacológico , Telangiectasia Hemorrágica Hereditaria/tratamiento farmacológico , Ácido Tranexámico/administración & dosificación , Epistaxis/etiología , Epistaxis/fisiopatología , Femenino , Humanos , Masculino , Pronóstico , Calidad de Vida , Ensayos Clínicos Controlados Aleatorios como Asunto , Medición de Riesgo , Índice de Severidad de la Enfermedad , Telangiectasia Hemorrágica Hereditaria/complicaciones , Telangiectasia Hemorrágica Hereditaria/diagnóstico , Resultado del Tratamiento
10.
Am Fam Physician ; 98(4): 240-245, 2018 08 15.
Artículo en Inglés | MEDLINE | ID: mdl-30215971

RESUMEN

Epistaxis is a common emergency encountered by primary care physicians. Up to 60% of the general population experience epistaxis, and 6% seek medical attention for it. More than 90% of cases arise from the anterior nasal circulation, and most treatments can be easily performed in the outpatient setting. Evaluation of a patient presenting with epistaxis should begin with assessment of vital signs, mental status, and airway patency. When examining the nose, a nasal speculum and a good light source, such as a headlamp, can be useful. Compressive therapy is the first step to controlling anterior epistaxis. Oxymetazoline nasal spray or application of cotton soaked in oxymetazoline or epinephrine 1: 1,000 may be useful adjuncts to compressive therapy. Directive nasal cautery, most commonly using silver nitrate, can be used to control localized continued bleeding or prominent vessels that are the suspected bleeding source. Finally, topical therapy and nasal packing can be used if other methods are unsuccessful. Compared with anterior epistaxis, posterior epistaxis is more likely to require hospitalization and twice as likely to need nasal packing. Posterior nasal packing is often associated with pain and a risk of aspiration if it is dislodged. After stabilization, patients with posterior packing often require referral to otolaryngology or the emergency department for definitive treatments.


Asunto(s)
Atención Ambulatoria/métodos , Epistaxis , Nariz , Manejo de Atención al Paciente/métodos , Epistaxis/diagnóstico , Epistaxis/fisiopatología , Epistaxis/terapia , Humanos , Nariz/anatomía & histología , Nariz/irrigación sanguínea
11.
Clin Exp Rheumatol ; 36 Suppl 111(2): 78-84, 2018.
Artículo en Inglés | MEDLINE | ID: mdl-29799391

RESUMEN

OBJECTIVES: Granulomatosis with polyangiitis is a rare autoimmune disease of the group of antineutrophil cytoplasmic antibody (ANCA)-associated vasculitides. Involvement of the ear, nose and throat (ENT)-region is only described in a few case series and case reports. The objective of this study is to systematically characterise the ENT-involvement in a large series of GPA patients. METHODS: GPA patients examined in the Department of Otorhinolayngology of the Christian-Albrechts-University of Kiel between 1990 and 2012 were included. Diagnosis was based on histological, serological and clinical parameters. GPA patients were examined in a standardised way based on the Ear Nose and Throat Activity Score (ENTAS) or its precursor. Medical history, ENT examination, diagnostic findings (ear, nose) and cranial radiology were documented cumulatively. RESULTS: A total of 230 GPA patients were included in this study. Over 95% of them showed ENT-involvement. 59% of the patients showed nasal obstructions, 57% a loss of smell. A hearing loss was diagnosed in 23% of the patients, 50% involvement in MR or CT scans and 15% showed laryngeal involvement. CONCLUSIONS: The data of the largest monocentric study presented here demonstrate a frequent ENT-involvement in GPA patients. Rhinological and ontological manifestations are most common.


Asunto(s)
Granulomatosis con Poliangitis/fisiopatología , Enfermedades Otorrinolaringológicas/fisiopatología , Adolescente , Adulto , Anciano , Anciano de 80 o más Años , Audiometría de Tonos Puros , Mareo/etiología , Mareo/fisiopatología , Disfonía/etiología , Disfonía/fisiopatología , Dolor de Oído/etiología , Dolor de Oído/fisiopatología , Epistaxis/epidemiología , Epistaxis/fisiopatología , Femenino , Granulomatosis con Poliangitis/complicaciones , Granulomatosis con Poliangitis/diagnóstico por imagen , Pérdida Auditiva/etiología , Pérdida Auditiva/fisiopatología , Pruebas Auditivas , Humanos , Laringoscopía , Imagen por Resonancia Magnética , Masculino , Persona de Mediana Edad , Trastornos del Olfato/etiología , Trastornos del Olfato/fisiopatología , Enfermedades Otorrinolaringológicas/diagnóstico por imagen , Enfermedades Otorrinolaringológicas/etiología , Otoscopía , Rinitis/etiología , Rinitis/fisiopatología , Rinomanometría , Acúfeno/etiología , Acúfeno/fisiopatología , Adulto Joven
13.
Folia Morphol (Warsz) ; 77(2): 345-355, 2018.
Artículo en Inglés | MEDLINE | ID: mdl-29131280

RESUMEN

BACKGROUND: Epistaxis is a frequent problem otorhinolaryngologists faces of in their practice. The variations of the sphenopalatine foramen (SPF) and consequ-ently the artery may be one of the major sources of such bleeding. The present work aimed to localise the site of SPF and also to illustrate its different shapes, number and any other variation. MATERIALS AND METHODS: In the current study, 20 adult skulls of both sexes with total 40 half skulls were used in addition to 20 heads of adult living subjects of both sexes aged between 30 and 60 years examined with multislice helical com-puted tomography with 3-dimensional reconstruction of SPF. Then, examination of another ten dried skulls with endoscope was performed. RESULTS: The number of the SPF is varied being single in nearly 80% and multiple in 20% of examined cases. The shape of the foramen also is varied; regular in 67.5% and irregular in 32.5% of all cases. The site of the foramen on the lateral nasal wall is placed in the superior meatus in most of examined skulls (62.5%) while in the rest (37.5%) they are found in the superior meatus and extending to the middle one. CONCLUSIONS: There are variation of the number, shape and site of the SPF, and consequently of the branches of the sphenopalatine artery, and this may explain the surgical failure in management of severe epistaxis. The data obtained from the current work support the predication of more than one sphenopalatine ar-teries and gives ample knowledge on the endoscopic study of the lateral nasal wall and consequently the surgical treatment of severe epistaxis. (Folia Morphol 2018; 77, 2: 345-355).


Asunto(s)
Arterias , Endoscopía , Tomografía Computarizada Multidetector , Cavidad Nasal , Adulto , Arterias/diagnóstico por imagen , Arterias/fisiopatología , Epistaxis/diagnóstico por imagen , Epistaxis/fisiopatología , Femenino , Humanos , Masculino , Persona de Mediana Edad , Cavidad Nasal/diagnóstico por imagen , Cavidad Nasal/fisiopatología
14.
Curr Opin Otolaryngol Head Neck Surg ; 25(1): 64-68, 2017 Feb.
Artículo en Inglés | MEDLINE | ID: mdl-27841771

RESUMEN

PURPOSE OF REVIEW: Hereditary hemorrhagic telangiectasia (HHT), or Osler Weber-Rendu disease, is a rare inherited disorder of fibrovascular tissue affecting various organs. Epistaxis is the most common symptom of HHT but as the disease affects multiple organs, a multisystem and multidisciplinary approach to management is required. The purpose of this article is to provide an overview of the multidisciplinary approach to HHT for the otolaryngologist and to discuss the current pharmacologic and procedural treatment options available for HHT-related epistaxis. RECENT FINDINGS: Multidisciplinary expert guidelines have better defined what screening tests are advised for the multisystem evaluation of the HHT patient. New pharmacologic therapies including bevacizumab (Avastin) used submucosally or topically have shown promise as in-office treatment modalities. Sclerotherapy of telangiectasia, including in-office applications, has recently proven safe and effective. SUMMARY: HHT remains a difficult disease to treat. Being aware of the common organ systems involved by the disease will help the practicing otolaryngologist to ensure the patient receives appropriate multidisciplinary care. For HHT-related epistaxis, new medical and surgical options allow for a wider range of treatments than were previously available.


Asunto(s)
Multimorbilidad , Telangiectasia Hemorrágica Hereditaria/genética , Telangiectasia Hemorrágica Hereditaria/terapia , Terapia Combinada/métodos , Atención Integral de Salud/métodos , Manejo de la Enfermedad , Progresión de la Enfermedad , Epistaxis/fisiopatología , Epistaxis/terapia , Femenino , Humanos , Malformaciones Arteriovenosas Intracraneales/fisiopatología , Malformaciones Arteriovenosas Intracraneales/terapia , Coagulación con Láser/métodos , Enfermedades Pulmonares/fisiopatología , Enfermedades Pulmonares/terapia , Masculino , Pronóstico , Enfermedades Raras , Medición de Riesgo , Telangiectasia Hemorrágica Hereditaria/fisiopatología , Resultado del Tratamiento
15.
Emerg Med J ; 34(8): 543-548, 2017 Aug.
Artículo en Inglés | MEDLINE | ID: mdl-27542804

RESUMEN

OBJECTIVE: The aim of this review is to determine an efficient and safe primary strategy care for paediatric epistaxis. DATA SOURCES: We searched PubMed and Cochrane databases for studies referenced with key words 'epistaxis AND childhood'. This search yielded 32 research articles about primary care in childhood epistaxis (from 1989 to 2015). Bibliographic references found in these articles were also examined to identify pertinent literature. We compared our results to the specific management of adult epistaxis classically described in the literature. RESULTS: Epistaxis is one of the most common reasons for referral of children to a hospital ENT outpatient department. The bleeding usually originates from the anterior septum, as opposed to adults. Crusting, digital trauma, foreign bodies and nasal colonisation with Staphylococcus aureus have been suggested as specific nosebleed factors in children. Rare aetiologies as juvenile nasopharyngeal angiofibroma appear later during adolescence. There are different modes of management of mild epistaxis, which begin with clearing out blood clots and bidigital compression. An intranasal topical local anaesthetic and decongestant can be used over 6 years of age. In case of active bleeding, chemical cauterisation is preferred to anterior packing and electric cauterisation but is only feasible if the bleeding site is clearly visible. In case of non-active bleeding in children, and in those with recurrent idiopathic epistaxis, antiseptic cream is easy to apply and can avoid 'acrobatic' cauterisation liable to cause further nasal cavity trauma. CONCLUSIONS: Aetiologies and treatment vary with patient age and the existence or not of active bleeding at the time of the examination. Local treatments are usually easy to perform, but physicians have to ponder their indications depending on the possible complications in order to inform parents and to know paediatric epistaxis specificities.


Asunto(s)
Epistaxis/terapia , Pediatría/métodos , Adolescente , Anestésicos/farmacología , Anestésicos/uso terapéutico , Cauterización/instrumentación , Cauterización/métodos , Niño , Preescolar , Electrocoagulación/instrumentación , Electrocoagulación/métodos , Servicio de Urgencia en Hospital/organización & administración , Servicio de Urgencia en Hospital/estadística & datos numéricos , Epistaxis/fisiopatología , Femenino , Humanos , Lidocaína/efectos adversos , Lidocaína/farmacología , Lidocaína/uso terapéutico , Masculino , Pediatría/estadística & datos numéricos , Nitrato de Plata/efectos adversos , Nitrato de Plata/uso terapéutico
16.
Am J Health Syst Pharm ; 73(21): 1755-1759, 2016 Nov 01.
Artículo en Inglés | MEDLINE | ID: mdl-27769971

RESUMEN

PURPOSE: The role of topical tranexamic acid in the management of anterior epistaxis in adult patients in the emergency department (ED) is examined. SUMMARY: The use of alternative agents for the treatment of epistaxis before the use of nasal packing may be reasonable due to patient discomfort, potential complications, and the need for follow-up with a healthcare provider for packing removal. One such agent is tranexamic acid. Two published studies evaluated the off-label use of topical tranexamic acid for the treatment of epistaxis. The first trial compared the efficacy of a topical gel containing 10% tranexamic acid with a placebo gel containing glycerin for the treatment of epistaxis. The percentage of patients whose bleeding ceased within 30 minutes of the intervention did not significantly differ between the tranexamic acid and placebo groups (p = 0.16). The second trial compared the efficacy of cotton pledgets soaked in the i.v. formulation of tranexamic acid inserted into the bleeding naris with standard nasal packing therapy. Bleeding cessation occurred within 10 minutes in 71% of the tranexamic acid group versus 31.2% of the standard treatment group (odds ratio, 2.28; 95% confidence interval, 1.68-3.09; p < 0.001). Additional information is necessary to fully evaluate the role of topical tranexamic acid in treatment algorithms; however, the use of topical tranexamic acid may be beneficial in select populations. CONCLUSION: Topical tranexamic acid may have a role in the treatment of anterior epistaxis in select ED patients, though additional studies are needed to confirm its role in treatment algorithms.


Asunto(s)
Antifibrinolíticos/administración & dosificación , Manejo de la Enfermedad , Servicio de Urgencia en Hospital/tendencias , Epistaxis/tratamiento farmacológico , Ácido Tranexámico/administración & dosificación , Administración Tópica , Adulto , Epistaxis/diagnóstico , Epistaxis/fisiopatología , Humanos
17.
Clin Chest Med ; 37(3): 505-11, 2016 09.
Artículo en Inglés | MEDLINE | ID: mdl-27514596

RESUMEN

Hermansky-Pudlak syndrome (HPS) is an autosomal recessive disorder that is associated with oculocutaneous albinism, bleeding diatheses, granulomatous colitis, and highly penetrant pulmonary fibrosis in some subtypes, including HPS-1, HPS-2, and HPS-4. HPS pulmonary fibrosis shows many of the clinical, radiologic, and histologic features found in idiopathic pulmonary fibrosis, but occurs at a younger age. Despite knowledge of the underlying genetic defects, there are currently no definitive therapeutic or preventive approaches for HPS pulmonary fibrosis other than lung transplant.


Asunto(s)
Malformaciones Arteriovenosas/fisiopatología , Trastornos de la Coagulación Sanguínea/fisiopatología , Síndrome de Hermanski-Pudlak/fisiopatología , Hipertensión Pulmonar/fisiopatología , Malformaciones Arteriovenosas Intracraneales/fisiopatología , Fibrosis Pulmonar/fisiopatología , Albinismo/complicaciones , Albinismo/fisiopatología , Albinismo Oculocutáneo/etiología , Albinismo Oculocutáneo/fisiopatología , Malformaciones Arteriovenosas/etiología , Trastornos de la Coagulación Sanguínea/etiología , Enfermedad de Crohn/etiología , Enfermedad de Crohn/fisiopatología , Epistaxis/etiología , Epistaxis/fisiopatología , Enfermedades Gastrointestinales/etiología , Enfermedades Gastrointestinales/fisiopatología , Trastornos Hemorrágicos/complicaciones , Trastornos Hemorrágicos/fisiopatología , Síndrome de Hermanski-Pudlak/complicaciones , Humanos , Hipertensión Pulmonar/etiología , Malformaciones Arteriovenosas Intracraneales/etiología , Hepatopatías/etiología , Hepatopatías/fisiopatología , Arteria Pulmonar/anomalías , Fibrosis Pulmonar/etiología , Venas Pulmonares/anomalías , Telangiectasia/etiología , Telangiectasia/fisiopatología
18.
Artículo en Chino | MEDLINE | ID: mdl-26695977

RESUMEN

OBJECTIVE: To investigate the clinical features and management of intractable deep-seated epistaxis. METHODS: Clinical data were analyzed retrospectively in 195 patients with intractable deep-seated epistaxis treated in Affiliated Hospital of Qingdao College between June 2008 and June 2014. The analyses included common risk factors, bleeding sites, treatments and therapeutic effects. SPSS 17.0 software was used to analyze the data. RESULTS: There were 162 males and 33 females, aged from 15 to 88 years old (mean 52 ± 16 years). One hundred and eighty-seven cases (95.9%) were unilateral and 8 cases (4.1%) were bilateral. One hundred and twenty-six cases (64.6%) had the history of hypertension or blood pressure higher than normal on admission. Eight patients were cured by nasal packing and appropriate drugs, and 187 patients were treated by endoscopy and electrocoagulation. The numbers of treatment needed to stop bleeding were 1 time in 184 cases (98.4%), 2 times in 3 cases (1.6%). Thirteen cases did not have certain bleeding point, while the other 174 had certain one or more bleeding sites. The rewarded bleeding sites were 222, which were found in the following different sites: posterior inferior nasal meatus (33.3%, 74 /222), olfactory cleft of nasal septum (26.6%, 59/222), the root of the middle turbinate (14.0%, 31/222), and so on. Hypertension and diabetes were risk factors (OR value was 3.411, 7.142, both P < 0.05). CONCLUSIONS: Intractable deep-seated epistaxis are mainly found in males and are almost unilatera1. Hypertension and diabetes are the common risk factor. The most effective and safe treatment for intractable deep-seated epistaxis is electrocoagulation under nasal endoscopy.


Asunto(s)
Epistaxis/fisiopatología , Cavidad Nasal/fisiopatología , Adolescente , Adulto , Anciano , Anciano de 80 o más Años , Diabetes Mellitus , Electrocoagulación , Endoscopía , Epistaxis/diagnóstico , Epistaxis/terapia , Femenino , Humanos , Hipertensión , Masculino , Persona de Mediana Edad , Tabique Nasal/patología , Senos Paranasales/patología , Estudios Retrospectivos , Factores de Riesgo , Cornetes Nasales/patología , Adulto Joven
19.
J La State Med Soc ; 167(1): 11-6, 2015.
Artículo en Inglés | MEDLINE | ID: mdl-25978749

RESUMEN

STUDY OBJECTIVE: To determine if hypopharyngeal surgery for obstructive sleep apnea is associated with significant morbidity in the early post-operative period. METHODS: Patients with a diagnosis of obstructive sleep apnea who underwent hypopharyngeal surgery at a tertiary care facility between November 2012 and September 2013 were included in this study. Surgical outcomes were assessed from medical records review and a 14 question telephone survey. Results: Twenty-two patients underwent hypopharyngeal surgery for obstructive sleep apnea (OSA). No patient experienced intra-operative complications, post-operative O2 desaturation <90%, prolonged admission for inadequate pain control, pulmonary edema, or airway compromise requiring re-intubation. Post-operative complications included one episode of nasal hemorrhage, one infection requiring hospitalization, and one episode of dehydration treated with IV fluids. 25% of patients experienced some degree of post-operative dysphonia, and 87.5% of patients experienced post-operative dysphagia. The average rating for post-operative pharyngeal pain was 3.5 of 10 by week 3 and 1.75 of 10 by week 4. Most patients described decreased snoring (93.75%), improved feeling of overall health (75%), and increased daytime energy (62.5%). All patients undergoing hypopharyngeal airway surgery were discharged within 23 hours. CONCLUSION: Hypopharyngeal surgery is a safe and well tolerated procedure for the treatment of OSA. Our findings suggest that hypopharyngeal surgery may be performed on an outpatient basis.


Asunto(s)
Hipofaringe/cirugía , Apnea Obstructiva del Sueño , Adulto , Anciano , Epistaxis/etiología , Epistaxis/fisiopatología , Femenino , Humanos , Masculino , Persona de Mediana Edad , Dolor Postoperatorio/etiología , Dolor Postoperatorio/fisiopatología , Periodo Posoperatorio , Edema Pulmonar/etiología , Edema Pulmonar/fisiopatología , Apnea Obstructiva del Sueño/fisiopatología , Apnea Obstructiva del Sueño/cirugía
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