Summer Vacations not Only a Memory for Life, but Also a Rare Infection

Introduction: Rhinoscleroma is a rare, chronic, granulomatous disease that most frequently affects the upper respiratory tract, especially the nasal cavity and sometimes extends through the lower respiratory tract. Is associated with Klebsiella rhinoscleromatis, which is endemic in certain geographic regions namely Central America. The pathogenesis and risk factors remain unclear. Clinical case: We report a five years Old Portuguese boy, previously healthy, brought to the Emergency Department with epistaxis, without other accompanying signs or symptoms. The Otorhinolaryngologist (ORL) performed rhinoscopy and identified an intranasal bleeding mass. The MRI revealed an intranasal mass with extension to the ethmoid bone sinus, and performed biopsy. The histopathology was vital, making the diagnosis of Rhinoscleroma. The child had traveled abroad for the first time on vacations a year before to Dominican Republic. The bacteriologic exam identified a Klebsiella spp. sensible to the association of amoxicillin and clavulanic acid. Blood test performed excluded association of immunodeficiency. Since it’s a rare disease genetic study are under course. Monthly evaluation by ORL and pediatrician was performed which documented progressive reduction until total disappearing of the macro and microscopic lesion, and negative bacteriologic exam. Six months of antibiotic therapy were completed without any known secondary effects. The child remained asymptomatic up to the last visit, 3 months following treatment and has shown no evidence of recurrence. Conclusion: Globalization and free transit of people to areas far from origin countries here some rare diseases are endemic brings a new challenge to modern medicine. Sometimes vacations bring more than memories.

Health-Related Quality of Life of Pediatric Intensive Care Survivors

Objetivo: Avaliar a qualidade de vida relacionada à saúde (QVRS)de crianças sobreviventes à alta da terapia intensiva pediátrica. Métodos: Foi realizada uma avaliação prospectiva da QVRS na admissão e após 6 meses em crianças com idade igual ou superior a 6 anos, internadas em três unidades de terapia intensiva pediátricas (UTIPs) terciárias de maio de 2002 a junho de 2004. A QVRS foi avaliada com o questionário Health Utilities Index Mark 3 (HUI3), aplicado a um representante da criança. Resultados: Das 517 admissões elegíveis, 44 crianças faleceram na UTIP (8,5%) e 320 casos foram avaliados na admissão; entre eles, foi possível realizar o seguimento de 252 casos. Não foram encontradas diferenças estatisticamente significativas entre os escores globais do HUI3 antes da admissão e no seguimento [medianas (intervalo interquartil) de 0,86 (0,42-1,00) e 0,83 (0,45-1,00); p = 0,674, respectivamente]. No âmbito individual, 21% das crianças não apresentaram mudanças na QVRS, foi observada melhora em 40% e agravamento em 38% dos casos. Deficiência grave antes da admissão (escore global do HUI3 < 0,70)esteve presente em 36% dos casos, com melhora no seguimento aos 6 meses em 60% deles. Entre aqueles que apresentaram agravamento da QVRS no seguimento, 45% eram vítimas de trauma. Conclusões: Embora a QVRS seja globalmente semelhante nas duas avaliações, foram encontradas várias diferenças no âmbito individual. As crianças com baixa QVRS antes da admissão (deficiência grave) podem se beneficiar da terapia intensiva pediátrica, visto que muitas dessas crianças melhoraram a QVRS, em comparação com seu estado pré-admissão.

Optimal Cut-Off Value for Homeostasis Model Assessment (HOMA) Index of Insulin-Resistance in a Population of Patients Admitted Electively in a Portuguese Cardiology Ward

INTRODUCTION: Insulin resistance is the pathophysiological key to explain metabolic syndrome. Although clearly useful, the Homeostasis Model Assessment index (an insulin resistance measurement) hasn't been systematically applied in clinical practice. One of the main reasons is the discrepancy in cut-off values reported in different populations. We sought to evaluate in a Portuguese population the ideal cut-off for Homeostasis Model Assessment index and assess its relationship with metabolic syndrome. MATERIAL AND METHODS: We selected a cohort of individuals admitted electively in a Cardiology ward with a BMI < 25 Kg/m2 and no abnormalities in glucose metabolism (fasting plasma glucose < 100 mg/dL and no diabetes). The 90th percentile of the Homeostasis Model Assessment index distribution was used to obtain the ideal cut-off for insulin resistance. We also selected a validation cohort of 300 individuals (no exclusion criteria applied). RESULTS: From 7 000 individuals, and after the exclusion criteria, there were left 1 784 individuals. The 90th percentile for Homeostasis Model Assessment index was 2.33. In the validation cohort, applying that cut-off, we have 49.3% of individuals with insulin resistance. However, only 69.9% of the metabolic syndrome patients had insulin resistance according to that cut-off. By ROC curve analysis, the ideal cut-off for metabolic syndrome is 2.41. Homeostasis Model Assessment index correlated with BMI (r = 0.371, p < 0.001) and is an independent predictor of the presence of metabolic syndrome (OR 19.4, 95% CI 6.6 - 57.2, p < 0.001). DISCUSSION: Our study showed that in a Portuguese population of patients admitted electively in a Cardiology ward, 2.33 is the Homeostasis Model Assessment index cut-off for insulin resistance and 2.41 for metabolic syndrome. CONCLUSION: Homeostasis Model Assessment index is directly correlated with BMI and is an independent predictor of metabolic syndrome.

Invasive Assessment of the Coronary Microcirculation Using the Index of Microcirculatory Resistance: Description and Validation of an Animal Model

INTRODUCTION: The index of microcirculatory resistance (IMR) enables/provides quantitative, invasive, and real-time assessment of coronary microcirculation status. AIMS: The primary aim of this study was to validate the assessment of IMR in a large animal model, and the secondary aim was to compare two doses of intracoronary papaverine, 5 and 10 mg, for induction of maximal hyperemia and its evolution over time. METHODS: Measurements of IMR were performed in eight pigs. Mean distal pressure (Pd) and mean transit time (Tmn) were measured at rest and at maximal hyperemia induced with intracoronary papaverine, 5 and 10 mg, and after 2, 5, 8 and 10 minutes. Disruption of the microcirculation was achieved by selective injection of 40-μm microspheres via a microcatheter in the left anterior descending artery. RESULTS: In each animal 14 IMR measurements were made. There were no differences between the two doses of papaverine regarding Pd response and IMR values - 11 ± 4.5 U with 5 mg and 10.6 ± 3 U with 10 mg (p=0.612). The evolution of IMR over time was also similar with the two doses, with significant differences from resting values disappearing after five minutes of intracoronary papaverine administration. IMR increased with disrupted microcirculation in all animals (41 ± 16 U, p=0.001). CONCLUSIONS: IMR provides invasive and real-time assessment of coronary microcirculation. Disruption of the microvascular bed is associated with a significant increase in IMR. A 5-mg dose of intracoronary papaverine is as effective as a 10-mg dose in inducing maximal hyperemia. After five minutes of papaverine administration there is no significant difference from resting hemodynamic status.

Mental Health and Quality of Life in Alcoholic Liver Disease Patients After Liver Transplantation: a Prospective Controlled Study

OBJECTIVE: Alcoholic liver disease (ALD) is one of the most important indications for liver transplantation. Discordant conclusions have been found concerning quality of life and mental health after transplantation in this particular group. The aim of this work was to investigate improvements in mental health and quality of life among transplanted patients for ALD. METHODS: We studied 45 consecutive transplant candidates with ALD, attending the outpatient clinics. Among these patients we transplanted 24 with the control candidates remaining in wait for transplantation. RESULTS: There was a significant improvement in all mental health and quality of life dimensions among the transplanted ALD group. We also observed a favorable evolution of coping mechanisms (CM) in this group. CONCLUSION: There is a favorable adjustment of ALD patients after transplantation as shown in CM evolution, which might explain the improved mental health and quality-of-life dimensions.

When Does Quality of Life Improve After Liver Transplantation? A Longitudinal Prospective Study

OBJECTIVES: We sought to investigate the improvement in quality of life (mental and physical components) at 1 and 6 months after liver transplantation. METHODS: A sample of liver transplant candidates (n = 60), comprising consecutive patients attending outpatient clinics of a liver transplantation central unit (25% of the patients had familial amyloid polyneuropathy [FAP] and the remaining patents had chronic liver diseases), was assessed by means of the Short Form (SF)-36, Portuguese-validated version, a self-rating questionnaire developed by the Medical Outcome Trust, to investigate certain primary aspects of quality of life, at 3 times: before, and at 1 and 6 months after transplantation. RESULTS: We observed a significant improvement in quality of life (both mental and physical components) by 1 month after transplantation. Between the first month and the sixth month after transplantation, there also was an improvement in the quality of life (both mental and physical components), although only the physical components of quality of life was significantly improved. CONCLUSIONS: Our findings suggested that quality of life improved early after liver transplantation (1 month). Between the first and the sixth months, there only was a significant improvement in the physical quality of life.

Psychosocial Determinants of Quality of Life 6 Months After Transplantation: Longitudinal Prospective Study

OBJECTIVES: We sought to investigate the psychosocial determinants of quality of life at 6 months after transplantation. METHODS: A sample of liver transplant candidates (n = 60), composed of consecutive patients (25% with familial amyloid polyneuropathy [FAP]) attending outpatient clinics was assessed in the pretransplant period using the Neo Five Factor Inventory, Hospital Anxiety and depression Scale (HADS), Brief COPE, and SF-36, a quality-of-life, self-rating questionnaire. Six months after transplantation, these patients were assessed by means of the SF-36. RESULTS: Psychosocial predictors where found by means of multiple regression analysis. The physical component of quality of life at 6 months after transplantation was determined based upon coping strategies and physical quality of life in the pretransplant period (this model explained 32% of variance). The mental component at 6 months after transplantation was determined by depression in the pretransplant period and by clinical diagnoses of patients. Because FAP patients show a lower mental component of quality of life, this diagnosis explained 25% of the variance. CONCLUSIONS: Our findings suggested that coping strategies and depression measured in the pretransplant period are important determinants of quality of life at 6 months after liver transplantation.

Impact of Syncope on Quality of Life: Validation of a Measure in Patients Undergoing Tilt Testing

INTRODUCTION AND OBJECTIVES: Recurrent syncope has a significant impact on quality of life. The development of measurement scales to assess this impact that are easy to use in clinical settings is crucial. The objective of the present study is a preliminary validation of the Impact of Syncope on Quality of Life questionnaire for the Portuguese population. METHODS: The instrument underwent a process of translation, validation, analysis of cultural appropriateness and cognitive debriefing. A population of 39 patients with a history of recurrent syncope (>1 year) who underwent tilt testing, aged 52.1 ± 16.4 years (21-83), 43.5% male, most in active employment (n=18) or retired (n=13), constituted a convenience sample. The resulting Portuguese version is similar to the original, with 12 items in a single aggregate score, and underwent statistical validation, with assessment of reliability, validity and stability over time. RESULTS: With regard to reliability, the internal consistency of the scale is 0.9. Assessment of convergent and discriminant validity showed statistically significant results (p<0.01). Regarding stability over time, a test-retest of this instrument at six months after tilt testing with 22 patients of the sample who had not undergone any clinical intervention found no statistically significant changes in quality of life. CONCLUSIONS: The results indicate that this instrument is of value for assessing quality of life in patients with recurrent syncope in Portugal.

Health-Related Quality of Life Impact of a Triple Combination of Olmesartan Medoxomil, Amlodipine Besylate and Hydrochlorotiazide in Subjects with Hypertension

BACKGROUND: A post-hoc analysis was performed on the data from a 54 weeks phase III study (ClinicalTrials.gov identifier: NCT00923091) to measure changes in the health-related quality of life (HRQoL) of 2,690 patients aged ≥18 with moderate-to-severe hypertension who received one of six doses of olmesartan/amlodipine/hydrochlorothiazide (OLM/AML/HCTZ), using the MINICHAL and EQ-5D instruments. METHODS: Descriptive statistics were used to assess blood pressure and HRQoL scores over the study period. Analysis of covariance (ANCOVA) was used to identify those factors that could possibly have influenced HRQoL. Linear regression was used to assess the relationship between changes in blood pressure and HRQoL scores. RESULTS: Patients' baseline MINICHAL mood and somatic domains scores were 5.5 and 2.6. Over the study period HRQoL improved as both MINICHAL scores decreased by 31-33%. Patients' baseline EQ-5D index and VAS scores were 0.9 and 73.4 respectively, increasing by 6% and 12% over the study period. Patients' QALY gain over the 54 weeks study period was estimated to be 0.029 QALYs. The ANCOVA showed that changes in patients' HRQoL was likely to have been influenced by patients' achievement of blood pressure control, the amount of concomitant medication and patients' last used dosage strength of antihypertensive. Linear regression showed that blood pressure improvement may have been associated with improved HRQoL. CONCLUSIONS: This study showed that OLM/AML/HCTZ reduced blood pressure and significantly increased blood pressure control whilst improving patients' HRQoL. Achieving blood pressure control, amount of concomitant medication and dosage strength of antihypertensive impacted on patients' HRQoL.

Disturbed Correlation Between Arterial Resistance and Pulsatility in Glaucoma Patients

PURPOSE: (i) To investigate whether pulsatility index (PI) and mean flow velocities (MFV) are altered in glaucoma patients. (ii) To evaluate the significance of PI in retrobulbar autoregulation capacity. METHODS: Patients with primary open-angle glaucoma (POAG; n = 49), normal tension glaucoma (NTG; n = 62) and healthy controls (n = 48) underwent colour Doppler imaging measurements of the retrobulbar vasculature. Kruskal-Wallis test was used to compare variables between the three diagnostic groups. Restricted cubic splines were used to determine nonlinearities between the resistive index (RI) and PI correlations. RESULTS: Mean flow velocities (MFV) were lower in both short posterior ciliary arteries (SCPA) and central retinal arteries (CRA) from the two glaucoma groups (p < 0.04 versus healthy controls). No differences were detected in RI or PI in any arteries of the three diagnostic groups (p > 0.08). In healthy individuals, correlations between RI and PI were linear in all arteries. In both POAG and NTG patients, CRA presented a nonlinear curve with a cutpoint at RI 0.77 (p < 0.001) and 0.61 (p = 0.03), respectively, above which the slope increased nearly five- and tenfold (POAG: 1.96 to 10.06; NTG: -0.46-4.06), respectively. A nonlinear correlation in the ophthalmic artery was only observed in NTG patients, with a cutpoint at RI 0.82 (p < 0.001), above which the slope increased from 3.47 to 14.03. CONCLUSIONS: Glaucoma patients do not present the linear relationships between RI and PI observed in healthy individuals. Their nonlinear relations may be indicative of an altered autoregulation and suggest a possible threshold RI could be determined above which autoregulatory disturbances become more relevant.

Influenza A(H1N1)pdm09 Resistance and Cross-Decreased Susceptibility to Oseltamivir and Zanamivir Antiviral Drugs

Neuraminidase inhibitors (NAIs) oseltamivir and zanamivir are currently the only effective antiviral drugs available worldwide for the management of influenza. The potential development of resistance is continually threatening their use, rationalizing and highlighting the need for a close and sustained evaluation of virus susceptibility. This study aimed to analyze and characterize the phenotypic and genotypic NAIs susceptibility profiles of A(H1N1)pdm09 viruses circulating in Portugal from 2009 to 2010/2011. A total of 144 cases of A(H1N1)pdm09 virus infection from community and hospitalized patients were studied, including three suspected cases of clinical resistance to oseltamivir. Oseltamivir resistance was confirmed for two of the suspected cases. Neuraminidase (NA) H275Y resistant marker was found in viruses from both cases but for one it was only present in 26.2% of virus population, raising questions about the minimal percentage of resistant virus that should be considered relevant. Cross-decreased susceptibility to oseltamivir and zanamivir (2-4 IC50 fold-change) was detected on viruses from two potentially linked community patients from 2009. Both viruses harbored the NA I223V mutation. NA Y155H mutation was found in 18 statistical non-outlier viruses from 2009, having no impact on virus susceptibility. The mutations at NA N369K and V241I may have contributed to the significantly higher baseline IC50 value obtained to oseltamivir for 2010/2011 viruses, compared to viruses from the pandemic period. These results may contribute to a better understanding of the relationship between phenotype and genotype, which is currently challenging, and to the global assessment of A(H1N1)pdm09 virus susceptibility profile and baseline level to NAIs.