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Recessive dystrophic epidermolysis bullosa (RDEB) manifests with blistering and erosions of the skin and mucous membranes due to mutations in COL7A1. The repetitive wound healing processes lead to extensive cutaneous scarring. The scarring is driven by inflammatory processes, particularly the TGF-ß signaling pathways, resulting in excess synthesis and deposition of the extracellular matrix, especially collagen. There is currently no effective or specific treatment for RDEB. Losartan, an angiotensin II type 1 receptor antagonist, is an inhibitor of TGF-ß activity. Previous preclinical studies with hypomorphic Col7a1 mice recapitulating features of RDEB have suggested that losartan may improve the clinical features of RDEB. In this case series, we assessed the effects of losartan on the clinical and histopathologic features in seven patients with RDEB; three females and four males; aged 18.1 ± 9.1 years. The diagnosis was based on characteristic clinical features and the presence of biallelic loss-of-function mutations in COL7A1. Daily oral administration of losartan (0.7 mg/kg) for six weeks resulted in subjective improvement of the clinical features, as judged by the treating physicians and the patients, and the severity of the disease objectively improved based on Birmingham Epidermolysis Bullosa Severity (BEBS) score (30.1 ± 12.8 versus 23.3 ± 10.4, before and after treatment, p = 0.018), accompanied by improvement of quality of life, as determined by the EB-QoL questionnaire (24.0 ± 8.1 versus 17.7 ± 5.5, p = 0.018). Histopathology of the selected lesions revealed after treatment increased number of mast cells, and enhanced microvasculature in the mid and lower dermis. The width of collagen bundles in dermis was suggested to be decreased in four samples and changed from dense to loose in appearance. In summary, this case series reports beneficial effects of losartan on RDEB as a potentially novel treatment.
Assuntos
Epidermólise Bolhosa Distrófica , Animais , Cicatriz/patologia , Colágeno , Colágeno Tipo VII/genética , Epidermólise Bolhosa Distrófica/diagnóstico , Epidermólise Bolhosa Distrófica/tratamento farmacológico , Epidermólise Bolhosa Distrófica/genética , Feminino , Losartan/uso terapêutico , Masculino , Camundongos , Qualidade de Vida , Fator de Crescimento Transformador betaRESUMO
BACKGROUND: Heart failure is a growing cardiovascular disease burden with high mortality rates, primarily attributed to myocardial ischemia. Previous studies have shown promising effects of Photobiomodulation in heart failure treatment. In this study, we aimed to investigate the effect of Photobiomodulation on myocardial function of patients with advanced ischemic heart failure. METHOD: A non-randomized case series study involving 10 advanced heart failure patients was conducted. Patients received 15 sessions of transcutaneous and intravenous Photobiomodulation therapy using low-level red (658 nm) and infrared lasers (810 nm). All participants were evaluated by six-minute walk tests, dyspnea function classes, and echocardiography. 3 months after the sessions, the mentioned tests were reevaluated RESULT: The study included ten cardiomyopathic patients, 90 % male and the mean age was 63.20±6.01 years. The six-minute walk test change and shortness of breath function class change has 0.852 (p-value=0.006) correlation and the correlation between the Six-minute walk test change and the Systolic ejection fraction rate change, was 0.73 (p-value=0.025). CONCLUSION: Based on the results of the study, it can be concluded that interventions in cardiomyopathic patients have shown promising improvements in certain cardiac function parameters. Specifically, the significant enhancement in the six-minute walk test post-intervention (p = 0.013) suggests a positive impact on functional capacity. Although the increase in systolic ejection fraction rate was not statistically significant (p = 0.197), the correlations identified provide valuable insights into the interplay between variables such as shortness of breath function class and the six-minute walk test. These findings underscore the complexity of managing cardiomyopathy and highlight the importance of further research to elucidate the relationships between different clinical parameters and patient outcomes in this population.
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Terapia com Luz de Baixa Intensidade , Isquemia Miocárdica , Humanos , Masculino , Terapia com Luz de Baixa Intensidade/métodos , Pessoa de Meia-Idade , Feminino , Isquemia Miocárdica/fisiopatologia , Idoso , Cardiomiopatias/fisiopatologia , Insuficiência Cardíaca/fisiopatologia , Resultado do TratamentoRESUMO
BACKGROUND AND AIMS: A better identification of the role of vitamin D in the thrombotic process of acute coronary syndrome (ACS) will help increase the therapeutic options for this important clinical condition. There is little published evidence that 25-hydroxy vitamin D (25(OH)D) serum levels can associate with platelet function and risk of thrombosis. MATERIALS AND METHODS: This prospective study was conducted on 200 patients with a diagnosis of ACS, including patients with unstable angina (UA), non-ST segment elevation myocardial infarction (NSTEMI), and ST segment elevation myocardial infarction (STEMI). In addition to demographics and angiographic data, serum concentrations of 25(OH)D and MPV were recorded in all patients. RESULTS: The types of ACS were STEMI (35%), NSTEMI (25%), and UA (40%). The concentrations of 25(OH)D in patients with UA, NSTEMI, and STEMI were 23.53 ± 13.26, 19.25 ± 8.09 and 14.60 ± 8.24 ng/mL respectively (P < 0.001), and the values of MPV were 9.83 ± 1.35, 10.30 ± 1.21, and 11.56 ± 1.38 fL, respectively (P < 0.001). There was a negative correlation between 25(OH)D and MPV (R = -0.320, P < 0.0001). ROC analysis illustrated a moderate predictive value (AUC 0.706; 95% CI, 0.63-0.72) in identifying the discrimination threshold of MPV (≥ 9.90 fL) for vitamin D deficiency (<20 ng/mL). CONCLUSION: The current study shows an inverse association between MPV and vitamin D levels in ACS patients, especially in the subgroup of STEMI. These findings propose the effect of vitamin D on platelet size and function, suggesting its role in thrombosis and hemostasis, which might explain the link between vitamin D deficiency and cardiovascular diseases.
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Background: Considering the role of higher blood urea nitrogen and lower serum albumin (SA) levels in deceased coronavirus disease 2019 (COVID-19) patients, an increased blood urea nitrogen to SA (B/A) ratio may help to determine those at higher risk of critical illness. This study aimed to evaluate the correlation of the B/A ratio with severity and 30-day mortality in COVID-19 patients. Methods: A total of 433 adult patients with COVID-19 were enrolled. The laboratory markers were measured on admission. Disease severity was categorized into mild disease, severe pneumonia, acute respiratory distress syndrome (ARDS), sepsis, and septic shock. The mortality was followed for 30 days after admission. χ 2 test, Fisher's exact test, and Mann-Whitney U test were performed, as appropriate. Also, logistic regression and the receiver operating characteristic (ROC) curve for the B/A ratio are included. Results: Thirty-day mortality rate was 27.25%. The frequency of mild, severe pneumonia, ARDS, sepsis, and septic shock was 30.72%, 36.95%, 24.02%, 6.00%, and 2.31%, respectively. B/A ratio and SA levels were statistically different between alive and deceased patients. The mean B/A ratio was different among classified disease severities, except for mild disease. Logistic regression revealed the B/A ratio as an independent risk factor for sepsis after adjusting for age and sex. ROC analysis showed B/A ratio had an area under the curve (AUC) of 0.733 for mortality at the cutpoint of 4.944. AUC for sepsis was 0.617 which was greater than other disease severities. Conclusion: The results showed that B/A ratio and SA levels are associated with mortality of COVID-19 patients. A higher B/A ratio is, additionally, associated with COVID-19 severity, except in mild cases and it can act as an independent risk factor in sepsis. However, a greater B/A ratio is not a significant predictor of COVID-19 severity, but it can predict mortality. Therefore, we suggest this marker for clinical assessment of patients with severe COVID-19.
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Marijuana is a widely used illicit substance among young adults and its abuse has been reported worldwide. Marijuana is a rare trigger of acute myocardial infarction and acute pancreatitis. We present a 25-year-old man with acute pancreatitis subsequently complicated by acute ST-elevation myocardial infarction (STEMI), which was associated with marijuana abuse. This case highlights the need and importance of awareness among public about this rare but potentially lethal adverse effect. Also, it draws attention when clinicians confront patients with history of substance abuse, they should be alert to the possibility of concurrent occurrence of serious medical conditions that may be adverse effects of substance use. Acute pancreatitis with concurrent acute STEMI is a rare situation but is a challenge for many emergency physicians, and it can lead to trouble outcomes if it not be quickly diagnosed and properly managed. We demonstrate successful management in this complicated patient with primary angioplasty.
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Cannabis , Infarto do Miocárdio , Pancreatite , Doença Aguda , Adulto , Cannabis/efeitos adversos , Eletrocardiografia , Humanos , Masculino , Infarto do Miocárdio/induzido quimicamente , Pancreatite/induzido quimicamente , Pancreatite/complicaçõesRESUMO
COVID-19 is a novel infectious disease, which has challenged people all around the world. As of today, healthcare practitioners and researchers have made great effort to understand the characteristics and clinical presentations of the disease; however, the existing literature is still incomplete in this regard. A growing body of evidence indicates that coagulopathies and thromboembolic events are of utmost importance in COVID-19 patients and are related to poor prognosis. Here, we report three ICU admitted cases of COVID-19, in which massive pulmonary thromboembolism (PTE) occurred a few days after disease onset. Unfortunately, one of the patients did not survive and two were treated; one with thrombectomy and other with antithrombotic agents. It seems that severe cases of COVID-19 are at risk for developing PTE and in-charge physicians should be prepared and plan for anticoagulant prophylaxis using low-molecular-weight heparin (LMWH).
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One of the major reasons for mortality throughout the world is cardiovascular diseases. Therefore, bio-markers of cardiovascular disease are of high importance to diagnose and manage procedure. Detecting biomarkers provided a promising procedure in developing bio-sensors. Fast, selective, portable, accurate, inexpensive, and sensitive biomarker sensing instruments will be necessary for detecting and predicting diseases. One of the cardiac biomarkers may be ordered as C-reactive proteins, lipoprotein-linked phospho-lipase, troponin I or T, myoglobin, interleukin-6, interleukin-1, tumor necrosis factor alpha, LDL and myeloperoxidase. The biomarkers are applied to anticipate cardio-vascular illnesses. Initial diagnoses of these diseases are possible by several techniques; however, they are laborious and need costly apparatus. Current researches designed various bio-sensors for resolving the respective issues. Electrochemical instruments and the proposed bio-sensors are preferred over other methods due to its inexpensiveness, mobility, reliability, repeatability. The present review comprehensively dealt with detecting biomarkers of cardiovascular disease through electro-chemical techniques.
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Técnicas Biossensoriais , Doenças Cardiovasculares , Biomarcadores , Técnicas Eletroquímicas , Humanos , Reprodutibilidade dos TestesRESUMO
Buprenorphine is used to treat opioid addiction. The spectrum of complications of oral buprenorphine is not well known. We describe two cases and based on a hypothesis is proposed which suggests that the use of oral buprenorphine could be relevant to the increase of thrombotic risk.
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A female with massive PTE and absolute contraindication for thrombolytic did not meet guidelines due to unavailability of catheter or surgical embolectomy and giving thrombolytic as a last resort to save a life. In such cases, physicians should consider to act outside of the guidelines to save a life.
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In massive pulmonary embolism (PE), fibrinolytic therapy is a potential lifesaving treatment; therefore, if other treatments are not available, the physicians encounter this question: can we accept the risk of complications from fibrinolytic therapy, especially intracranial hemorrhage, in the presence of absolute contraindication, in order to save the patient's life? Here, we describe three cases of massive PE with absolute contraindication for fibrinolytic therapy who presented to emergency department following dyspnea. Since, surgical or catheter embolectomy were not available and patients were very high risk for transferring to another hospital, systemic fibrinolytic was administered. The patients improved clinically and were discharged from hospital. It seems that, if no other acceptable treatments are available, physicians could consider fibrinolytic therapy, even at the presence of contraindication, to save the patient's life.
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PURPOSE: Anabolic androgenic steroids (AAS) abuse for improving physical appearance and performance in body builders is common and has been considered responsible for serious cardiovascular effects. Due to disagreement about cardiovascular side effects of these drugs in published articles, this case control study was designed to evaluate the echocardiographic findings in body builder athletes who are current and chronic abusers of these drugs. METHODS: Body builder athletes with continuous practice for the preceding two years and were training at least twice weekly were selected and divided into AAS abuser and non user and compared with age and BMI matched non athletic healthy volunteers (15 cases in each group). RESULTS: There was no significant difference in left ventricular size or function either systolic or diastolic in comparison to cases and control groups. The only difference was in diastolic size of septum and free wall but observed differences were only significant (P = 0.05) between first (athletic with AAS abuser) and third group (non athletic and nonuser). The difference between the above-mentioned indexes were not significant between two groups of athletes. CONCLUSION: Observed differences in diastolic size of septum and free wall is in favor of that long term abuse of anabolic steroid results in accentuation of physiologic hypertrophy due to long term sport most probably due to higher rate pressure product. Furthermore long term abuse and supra pharmacologic doses do not have significant effect in size and left ventricular function.