Abstract Background Intraoperative fluid management is important for the prevention of perioperative morbidity and mortality. Our study aimed to investigate the perioperative feasibility and benefits of Goal-Directed Fluid Management (GDFM) using noninvasive hemodynamic monitoring in gynecologic oncology patients with acute blood loss and severe fluid loss. We assessed the effects of GDFM on hemodynamics, organ perfusion, complications, and mortality outcomes. Methods This randomized prospective study included 104 patients over the age of 18 years, including 56 patients with endometrial cancer and 48 patients with ovarian cancer who had open surgery. The anesthetic approach was standardized for all patients. We compared the perioperative results of the subjects who were randomized into GDFM (n = 51) and Liberal Fluid Management (LFM) (n = 53) groups using a computer program. Results The median perioperative crystalloid replacement (2000 vs. 2700; p < 0.001) and total volume of fluid (2260 vs. 3200; p < 0.001) were lower in the GDFM group compared to the LFM group. The hemodynamic findings and the HCO3 and lactate levels of the GDFM group did not significantly change perioperatively. The heart rate, mean arterial pressure, and HCO3 levels of the LFM group decreased and serum lactate levels increased perioperatively. The hospitalization rate in ICU (7.8% vs. 28.3%; p = 0.010), rate of patients with comorbidity conditions indicated in ICU (2% vs. 17%; p = 0.024), and rate of complications (17.6% vs. 35.8%; p = 0.047) were lower in the GDFM group compared to the LFM group. Conclusion The amount of intraoperatively administered crystalloid solution and complication rates were significantly lower in gynecologic oncologic surgery patients who received GDFM. Besides, hemodynamic findings, and lactate levels of the GDFM group did not change significantly during the perioperative period.

RESUMO Objetivo: Avaliar as características da superfície ocular em crianças com tireoidite de Hashimoto sem oftalmopatia associada à tireoide e comparar os resultados com aqueles de crianças saudáveis. Métodos: Vinte e duas crianças com tireoidite de Hashimoto (Grupo 1) e 20 crianças saudáveis sem qualquer distúrbio ocular e/ou sistêmico (Grupo 2) participaram do estudo. Utilizou-se o questionário Índice da Doença da Superfície Ocular, medida de osmolaridade do filme lacrimal (Tearlab Osmolarity System, San Diego, CA, EUA), teste de Schirmer e tempo de ruptura do filme lacrimal, meibografia e citologia do raspado conjuntival e comparação dos resultados entre os grupos. Resultados: O grupo de estudo incluiu 19 meninas e 3 meninos no Grupo 1 e 12 meninas e 8 meninos no Grupo 2 (p=0,081). A oftalmopatia associada à tireoide não foi identificada em nenhum dos pacientes. A média da osmolaridade do filme lacrimal foi 310,23 ± 11,98 mOsm/l no Grupo 1 e 313,60 ± 15,03 mOsm/l no Grupo 2 (p=0,424). A média do escore do teste de Schirmer foi menor no Grupo 1 (14,91 ± 6,27) do que no Grupo 2 (23,60 ± 5,63) (p=0,001). A média do tempo de ruptura do filme lacrimal foi menor no Grupo 1 (11,78 ± 4,07) em comparação com o Grupo 2 (15,1 ± 1,6) (p=0,013). Além disso, a média da perda de área da glândula meibomiana foi 25,01% ± 10,04% no Grupo 1 e 16,54% ± 6,02% no Grupo 2 (p=0,002). A análise da citologia conjuntival no Grupo 1 revelou alterações de grau 0 em 6 pacientes (27,3%), alterações de grau 1 em 14 pacientes (63,6%) e alterações de grau 2 em 2 pacientes (9,1%), enquanto 18 pacientes (90%) com alteração de grau 0 e 2 pacientes (10%) com alteração de grau 1 (p=0,001) no Grupo 2. Conclusões: O estudo demonstra várias alterações da superfície ocular em crianças com tireoidite de Hashimoto. Esses achados podem indicar uma tendência para olho seco em pacientes pediátricos com tireoidite de Hashimoto, sem evidências clínicas de oftalmopatia associada à tireoide.

ABSTRACT Purpose: We aimed to assess ocular surface characteristics in children with Hashimoto's thyroiditis without thyroid-associated ophthalmopathy and compare the results with those of healthy children. Methods: Twenty-two children with Hashimoto's thyroiditis (Group 1) and 20 healthy children without any ocular and/or systemic disorder (Group 2) were enrolled in the study. Ocular Surface Disease Index questionnaire, tear film osmolarity measurement (TearLab Osmolarity System, San Diego, CA, USA), Schirmer and tear film breakup time tests, meibography, and conjunctival brush cytology were performed and compared the results between the groups. Results: The study group included 19 girls and 3 boys in Group 1 and 12 girls and 8 boys in Group 2 (p=0.081). Thyroid-associated ophthalmopathy was not identified in any of the patients. Mean tear film osmolarity was 310.23 ± 11.98 mOsm/l in Group 1 and 313.60 ± 15.03 mOsm/l in Group 2 (p=0.424). Mean Schirmer test score was lower in Group 1 (14.91 ± 6.27) compared with Group 2 (23.60 ± 5.63) (p=0.001). Mean tear film breakup time was lower in Group 1 (11.78 ± 4.07) compared with Group 2 (15.1 ± 1.6) (p=0.013). Moreover, mean meibomian gland area loss was 25.01% ± 10.04% in Group 1 and 16.54% ± 6.02% in Group 2 (p=0.002). Conjunctival cytologic analysis in Group 1 revealed grade 0 changes in 6 patients (27.3%), grade 1 changes in 14 patients (63.6%), and grade 2 changes in 2 patients (9.1%), whereas 18 patients (90%) had grade 0 changes and 2 patients (10%) had grade 1 changes (p=0.001) in Group 2. Conclusions: The study demonstrates several ocular surface changes in children with Hashimoto's thyroiditis. These findings may indicate a tendency for dry eye in pediatric Hashimoto's thyroiditis patients without clinical evidence of thyroid-associated ophthalmopathy.

Resumo Objetivo: A amiloidose AA é uma complicação rara de condições inflamatórias crônicas. A maior parte dos pacientes com amiloidose AA apresenta nefropatia, que leva à insuficiência renal e à morte. Estudaram-se as características clínicas e a sobrevida em pacientes com amiloidose AA. Métodos: Analisaram-se retrospectivamente 81 pacientes (51 homens, 30 mulheres) com amiloidose AA comprovada por biópsia renal. Os pacientes foram divididos em grupos de desfecho bom e ruim de acordo com os resultados de sobrevida. Resultados: A maior parte dos pacientes (55,6%) tinha proteinúria na faixa nefrótica no momento do diagnóstico. Os distúrbios subjacentes mais frequentes foram a febre familiar do Mediterrâneo (FFM, 21,2%) e a artrite reumatoide (10,6%) no grupo de desfecho bom e a malignidade (20%) no grupo de desfecho ruim. Somente a pressão arterial diastólica no grupo de desfecho bom e o nível de fósforo no grupo de desfecho ruim foram mais elevados. Os níveis séricos de creatinina aumentaram após o tratamento em ambos os grupos, enquanto a proteinúria diminuiu no grupo de desfecho bom. O aumento na creatinina sérica e a diminuição na TFGe do grupo de desfecho ruim foram mais significativos no grupo de desfecho bom. No momento do diagnóstico, 18,5% e 27,2% de todos os pacientes tinham doença renal crônica avançada (estágios 4 e 5, respectivamente). A duração média da sobrevida renal foi de 65 ± 3,54 meses. Entre todos os pacientes, 27,1% iniciaram tratamento de diálise durante o período de seguimento e 7,4% de todos os pacientes foram submetidos a transplante renal. Níveis elevados de pressão arterial sistólica [taxas de risco (HR) 1,03, intervalo de confiança (IC) de 95%: 1 a 1,06, p = 0,036], creatinina sérica (HR 1,25, IC 95%: 1,07 a 1,46, p = 0,006) e excreção urinária de proteínas (HR 1,08, IC 95%: 1,01 a 1,16, p = 0,027) foram preditores de doença renal terminal. A mediana da sobrevida de pacientes com comprometimento de órgãos foi de 50,3 ± 16 meses. Conclusão: O presente estudo indicou que a FFM constituiu uma grande proporção de casos e crescente quantidade de pacientes com amiloidose AA idiopática. Adicionalmente, observou-se que a sobrevida do paciente não foi afetada pelas diferentes causas etiológicas na amiloidose AA.

Abstract Aim Amyloid A amyloidosis is a rare complication of chronic inflammatory conditions. Most patients with amyloid A amyloidosis present with nephropathy and it leads to renal failure and death. We studied clinical characteristics and survival in patients with amyloid A amyloidosis. Methods: A total of 81 patients (51 males, 30 females) with renal biopsy proven amyloid A amyloidosis were analyzed retrospectively. The patients were divided into good and poor outcomes groups according to survival results. Results: Most of the patients (55.6%) had nephrotic range proteinuria at diagnosis. Most frequent underlying disorders were familial Mediterranean fever (21.2%) and rheumatoid arthritis (10.6%) in the good outcome group and malignancy (20%) in the poor outcome group. Only diastolic blood pressure in the good outcome group and phosphorus level in the poor outcome group was higher. Serum creatinine levels increased after treatment in both groups, while proteinuria in the good outcome group decreased. Increase in serum creatinine and decrease in estimated glomerular filtration rate of the poor outcome group were more significant in the good outcome group. At the time of diagnosis 18.5% and 27.2% of all patients had advanced chronic kidney disease (stage 4 and 5, respectively). Median duration of renal survival was 65 ± 3.54 months. Among all patients, 27.1% were started dialysis treatment during the follow-up period and 7.4% of all patients underwent kidney transplantation. Higher levels of systolic blood pressure [hazard ratios 1.03, 95% confidence interval: 1-1.06, p = 0.036], serum creatinine (hazard ratios 1.25, 95% confidence interval: 1.07-1.46, p = 0.006) and urinary protein excretion (hazard ratios 1.08, 95% confidence interval: 1.01-1.16, p = 0.027) were predictors of end-stage renal disease. Median survival of patients with organ involvement was 50.3 ± 16 months. Conclusion Our study indicated that familial Mediterranean fever constituted a large proportion of cases and increased number of patients with idiopathic amyloid A amyloidosis. Additionally, it was observed that patient survival was not affected by different etiological causes in amyloid A amyloidosis.

Abstract: Background: Hereditary angioedema is a rare autosomal dominantly inherited immunodeficiency disorder characterized by potentially life-threatening angioedema attacks. Objective: We aimed to investigate the clinical and genetic features of a family with angioedema attacks. Methods: The medical history, clinical features and C1-INH gene mutation of a Turkish family were investigated and outcomes of long-term treatments were described. Results: Five members had experienced recurrent swellings on the face and extremities triggered by trauma. They were all misdiagnosed as familial Mediterranean fever (FMF) depending on frequent abdominal pain and were on colchicine therapy for a long time. They had low C4 and C1-INH protein concentrations and functions. A mutation (c.1247T>A) in C1-INH gene was detected. They were diagnosed as having hereditary angioedema with C1-INH deficiency (C1-INH hereditary angioedema) for the first time. Three of them benefited from danazol treatment without any significant adverse events and one received weekly C1 esterase replacement treatment instead of danazol since she had a medical history of thromboembolic stroke. Study limitations: Small sample size of participants. Conclusion: Patients with C1-INH hereditary angioedema may be misdiagnosed as having familial Mediterranean fever in regions where the disorder is endemic. Medical history, suspicion of hereditary angioedema and laboratory evaluations of patients and their family members lead the correct diagnoses of hereditary angioedema. Danazol and C1 replacement treatments provide significant reduction in hereditary angioedema attacks.

OBJETIVO: Com este estudo pretendeu-se levar a cabo um ensaio clínico que permitisse investigar as diferenças entre a dispersão da onda P (DOP), as propriedades elásticas da aorta e os resultados da ecocardiografia transtorácica em futebolistas profissionais ativos e reformados, face a grupos de controle, de modo a avaliar a influência da atividade desportiva regular na distensibilidade aórtica e o seu potencial efeito na electrofisiologia atrial. MÉTODOS: Para este estudo foram recrutados 42 jovens futebolistas profissionais com um histórico de treino de vários anos. O grupo-controle foi constituído por 27 homens saudáveis e sedentários. No grupo composto por indivíduos mais velhos, foram incluídos 23 futebolistas profissionais já aposentados, saudáveis e com mais de 50 anos de idade e, como grupo-controle, 18 indivíduos com mais de 50 anos de idade que nunca fizeram qualquer tipo de exercício físico regular quando eram mais novos. RESULTADOS: A frequência cardíaca e fração de ejeção eram menores nos jovens futebolistas profissionais. Não se verificaram diferenças significativas nos parâmetros de elasticidade da aorta e a dispersão da onda P entre os jovens futebolistas profissionais e o grupo-controle. Mas, já no caso do grupo dos indivíduos mais velhos com uma atividade desportiva regular, a diferença significativa na dimensão ventricular esquerda, espessura das paredes e funções sistólicas detectada no grupo jovem e ativo desapareceu, enquanto o aumento no diâmetro atrial esquerdo tornou-se expressivo. CONCLUSÃO: Demonstrou-se com este estudo o efeito potencial das propriedades elásticas da aorta, que se alteram com a idade, na electrofisiologia atrial por meio do aumento da dispersão da onda P.

OBJECTIVE: The purpose of this study was to investigate the differences between P wave dispersion, aortic elastic properties and transthoracic echocardiographic findings in the young and old football players compared to control groups in order to asses the influence of regular sportive activity on aortic distensibility and its potential effect on atrial electrophysiology. METHODS : We recruited 42 young football players with a training history of many years. The control group was formed by 27 healthy sedentary men. Twenty-three healthy retired football players of a professional football club aged over 50 years were included in the study as old group and 18 subjects over 50 year old who did not perform regular exercise when they were young were included in the control group of old subjects. RESULTS: The heart rate and ejection fraction were decreased in the young football players. There were no significant differences in the aortic elastic parameters and P wave dispersion between young football players and control group. But in old subjects with sustained participation in regular sportive activity, the significant difference of left ventricular dimension, wall thickness and systolic functions detected in the young group disappeared while increase in the left atrial diameter became significant. CONCLUSION: Potential effect of aortic elastic properties which changes with age, on atrial electrophysiology through increasing P wave dispersion was shown.