RESUMEN
BACKGROUND: Hereditary Hemorrhagic Telangiectasia (HHT) is a vascular autosomically inherited rare disease. Epistaxis (nose bleeds) is the most common symptom in HHT, leading to anemia and affecting the patient's quality of life. In addition to epistaxis, gastrointestinal bleeding (GI), more often at older ages, may lead to severe anemia and the need for blood transfusions. Thus, finding drugs to control both types of bleeding is a primary necessity in HHT. METHODS: A cross-sectional observational study was conducted in a series of 11 HHT patients treated with low tacrolimus doses (0.5-2 mg/day) on an off-label prescription basis. Patients showed refractory bleeding to previous treatments. The epistaxis severity score (ESS) and hemoglobin levels were the parameters used to evaluate the impact of tacrolimus. The occurrence of side effects was also recorded. RESULTS: Tacrolimus was well tolerated in all of the patients except 2 (who stopped the treatment). The remaining patients tolerated the treatment, with a general improvement in their health condition. Epistaxis was significantly reduced when comparing the ESS before and after the treatment. Hemoglobin levels significantly increased, overcoming the anemia, during the course of the treatment. CONCLUSION: Tacrolimus at low doses should be considered as a promising treatment for epistaxis and gastrointestinal bleeding in HHT.
RESUMEN
Epistaxis is the most prevalent clinical symptom in Hereditary Haemorrhagic Telangiectasia (HHT), causing anaemia and decreasing the quality of life (QOL). Since 2013, in Hospital Universitario Fundación Alcorcón, more than 150 HHT patients have been treated by nose sclerotherapy on demand. This study shows the results of 105 patients treated with sclerotherapy between 2017 and 2019. HHT-ESS (epistaxis severity score) was used to measure the severity and frequency of epistaxis. QOL was determined before and after treatment by EuroQol-5D (EQ-5D) and the visual analogue scale (VAS) on the health condition. According to HHT-ESS before treatment, 22 patients presented mild, 35 moderate, and 47 severe epistaxes. Sclerotherapy significantly decreased the frequency and severity of epistaxis, with a significant drop of HHT-ESS in 4.6 points, from 6.23 ± 2.3 to 1.64 ± 1.6. Furthermore, the QOL significantly improved, the EQ-5D scale raised from 0.7 ± 0.26 pre- to 0.92 ± 0.16 post-treatment (p < 0.05). Additionally, VAS mean value showed a significant increase from 4.38 ± 2.4 to 8.35 ± 1.2. The QOL improvement was correlated with the ESS decrease. In conclusion, this study shows that on-demand sclerotherapy at the office significantly reduces HHT epistaxis as well as improved the patients' QOL.
RESUMEN
At the moment of writing this communication, the health crisis derived from the COVID-19 pandemic has affected more than 120 million cases, with 40 million corresponding to Europe. In total, the number of deaths is almost 3 million, but continuously rising. Although COVID-19 is primarily a respiratory disease, SARS-CoV-2 infects also endothelial cells in the pulmonary capillaries. This affects the integrity of the endothelium and increases vascular permeability. In addition, there are serious indirect consequences, like disruption of endothelial cells' junctions leading to micro-bleeds and uncontrolled blood clotting. The impact of COVID-19 in people with rare chronic cardiovascular diseases is unknown so far, and interesting to assess, because the virus may cause additional complications in these patients. The aim of the present work was to study the COVID-19 infection among the patients with Hereditary Hemorrhagic Telangiectasia (HHT). A retrospective study was carried out in a 138 HHT patients' sample attending an Ear Nose and Throat (ENT) reference consult. The evaluation of the COVID-19 infection in them reveals milder symptoms; among the 25 HHT patients who were infected, only 3 cases were hospitalized, and none of them required ICU or ventilation assistance. The results are discussed in the light of macrophage immune response.
RESUMEN
OBJECTIVE: To describe the long-term efficacy of transtympanic steroids (TTS) using methyl-prednisolone in the treatment of Ménière's disease (MD). DESIGN: Descriptive prospective study. MAIN OUTCOME MEASURES: Pure-tone average (PTA) corresponding to the conversational frequencies on the audiogram (0.5, 1, 2, and 3 kHz), visual analog scale on tinnitus annoyance, and number of vertigo spells 24 months after treatment. RESULTS: Thirty-four MD patients referred to a tertiary center were treated with TTS. All patients were diagnosed as probable or definitive MD (following American Academy of Otolaryngology-Head and Neck Surgery 1995 criteria) and treated by TTS (3 consecutive doses). Data from 32 patients were achieved after 12 months. Forty-eight percent of the patients reduced the PTA in 10 or more decibels, average improvement was 8.6 dB compared with initial PTA (p = 0.004). Tinnitus relief was achieved by 81.5% of the patients. Number of vertigo spells was reduced from 4.3 to 0.3 after 12 months (p = 0.002); 81% of the patients were free of vertigo spells, and 92.6% had 1 or less spells of vertigo. Data from 29 patients were achieved after 24 months. A reduction of PTA in 10 or more decibels was shown by the 33.3% of the sample, and PTA improved in 3.3 dB compared with initial PTA (nonsignificant). Tinnitus relief was achieved in 78% of the patients. Number of vertigo spells was reduced from 4.3 to 0.5 (p = 0.033). Seventy-eight percent of the cases were free of vertigo, and 96% had none or 1 spell. Because of an increase in any of the symptoms, 12 patients (35.2%) required retreatment with 1 or 2 series of TTS (1-3 doses) along the 2-year period. Two patients of the sample (6.25%) required transtympanic gentamicin for vertigo control due to lack of benefit with TTS (14 and 18 mo since TTS). CONCLUSION: Transtympanic steroids in this cohort were associated with good preservation of hearing. Tinnitus control is achieved in more than 70% of the patients, and number of vertigo spells can be dramatically reduced in more than 90% of the patients after a 24-month follow-up.
