Hasil untuk "Specialties of internal medicine"

Raquel Behar-Lagares, Raquel Behar-Lagares, Ana Virseda-Berdices et al.

IntroductionA significant proportion of COVID-19 Intensive Care Unit (ICU) survivors develop long-term respiratory complications, including pulmonary fibrosis. Telomere attrition, a marker of cellular senescence, has emerged as a potential biomarker for post-COVID-19 sequelae. This study investigated the association between peripheral blood relative telomere length (RTL) and long-term pulmonary outcomes in COVID-19 ICU survivors, with a specific focus on gender-specific differences.MethodsICU-admitted COVID-19 patients were followed for at least one year post-discharge. RTL was quantified from peripheral blood using monochromatic multiplex quantitative PCR (MMqPCR) at hospital admission and one-year post-discharge. Primary outcomes were respiratory symptoms and diffuse parenchymal lung disease (DPLD), assessed via imaging. Data were analyzed using gender-stratified generalized linear models, adjusted for clinical covariates.ResultsAt one year, 43.8% of patients reported respiratory symptoms and 23.9% developed DPLD. A total of 73 ICU survivors were included, with 51 men and 22 women. At one year, 43.8% of patients reported respiratory symptoms and 23.9% developed DPLD. Longitudinal analysis showed significant RTL shortening in both men and women who underwent IMV (p=0.011 and p=0.016, respectively), and in men who needed pronation during their ICU stay (p=0.037). Regarding one-year symptoms, in women, repeated-measures analysis showed an association with persistent respiratory symptoms, particularly in those who needed pronation during their ICU stay [adjusted arithmetic mean ratio (aAMR)=0.73) (95%CI=0.60-0.90); p=0.003]. At follow-up, women who had undergone pronation and had shorter RTL continued to show a higher prevalence of symptoms [aAMR= 0.66 (0.58-0.76); p< 0.001]. In contrast, men with shorter RTL at one-year post-discharge had an association with the presence of DPLD [aAMR = 0.64 (0.50-0.81); p = 0.001]. This association in men was significant particularly among those who required IMV [aAMR= 0.61 (0.49-0.76); p< 0.001] or prone positioning [aAMR= 0.56 (0.44-0.71); p= 0.016]. DiscussionThese findings underscore the role of telomere attrition as a sex-specific biomarker of aging-associated pulmonary vulnerability in the aftermath of critical COVID-19 illness. RTL may serve as a prognostic marker for long-term respiratory sequelae, potentially guiding risk stratification and individualized follow-up strategies in post-ICU COVID-19 survivors.

Tariq Mahmood, Vimal Kumar

Takuya Mizumoto, Yoshihide Nanno, Jun Ishida et al.

ABSTRACT Aim Regarding the resectability of pancreatic adenocarcinoma (PDAC), not only anatomical factors but also biological and conditional factors have come to be considered. This study examined the impact of the Global Leadership Initiative on Malnutrition (GLIM) criteria on prognosis after resection of anatomically resectable PDAC. Methods The medical records of consecutive patients who underwent resection for resectable PDAC between January 1, 2014, and December 31, 2022, were retrospectively reviewed. Patients were classified as normal, moderately, or severely malnourished according to the GLIM criteria. Results In total, 194 patients were included in the analysis. According to the GLIM criteria, 61 (31.4%), 49 (25.2%), and 84 (42.3%) patients were normal, moderately, and severely malnourished, respectively. Patients with malnutrition had shorter overall, recurrence‐free, and disease‐specific survival (OS, RFS, and DSS) than normal patients (OS, normal vs. moderate, p = 0.015; normal vs. severe, p < 0.001; RFS, normal vs. moderate p = 0.012, normal vs. severe, p < 0.001; DSS, normal vs. moderate, p = 0.023; normal vs. severe, p < 0.001). In multivariate analysis regarding OS using all factors, moderate or severe malnutrition according to the GLIM criteria (p = 0.007), performance status (p = 0.086), preoperative diabetes (p = 0.017), tumor diameter ≥ 3 cm (p = 0.002), lymph node metastasis (p < 0.001), and postoperative adjuvant therapy (p = 0.027) were independent prognostic factors. In multivariate analysis using preoperative factors, malnutrition according to the GLIM criteria remained a significant prognostic factor (p = 0.003). Conclusion The GLIM criteria are effective prognostic predictors in patients with resectable PDAC undergoing upfront surgery. Preoperative nutritional assessment using these criteria may contribute to determining treatment plans for resectable PDAC.

Pankaj Garg, Mostafa Ali, Mohammad Alomari et al.

Abstract Cardiac benign metastatic leiomyoma (BML) is a rare cardiac tumor that is usually asymptomatic, frequently misdiagnosed and may result in serious complications, including embolization, heart failure and death. This review highlights the importance of considering cardiac BML in the differential diagnosis of cardiac masses, especially in women with a history of uterine leiomyomas. This review summarizes the current knowledge about cardiac BML, including its demographics, clinical presentation, etio-pathogenesis, diagnosis, and management. The authors discuss the challenges associated with diagnosing cardiac BML and emphasize the importance of a thorough history, physical examination, and imaging studies. They also review the different treatment options for cardiac BML, including surgical resection and role of medical and surgical castration. Early diagnosis and management of cardiac BML is crucial to prevent complications. This review provides valuable insights for clinicians who may encounter this rare condition. By raising awareness of cardiac BML and its management strategies, this review can improve patient care and outcomes.

Cuimei Wei, Yaohui Huang, Ping Xi et al.

Abstract Objective The strong correlation between the ratio of residual cholesterol to high-density lipoprotein cholesterol (RC/HDL-c) and major cardiovascular events has been extensively studied. However, the role of this ratio in diabetic retinopathy (DR) has not been investigated. Hence, this present study aims to examine the association between the RC/HDL-c ratio and DR in patients diagnosed with type 2 diabetes mellitus (T2DM). Methods This study conducted a cross-sectional analysis involving a total of 1942 patients diagnosed with T2DM in two Taiwanese hospitals, spanning from April 2002 to November 2004. The primary objective was to explore the independent association between the RC/HDL-c ratio and the presence of DR, as well as proliferative diabetic retinopathy (PDR), using a binary logistic regression model. To accurately determine the shape of the association between these variables, we utilized a generalized additive model (GAM) and employed smooth curve fitting techniques. The data was downloaded from the website: https://journals.plos.org/plosone . Results Our study comprised participants with an average age of 64.06 ± 11.32 years, with males accounting for 43.05% of the total. Among the patients, 35.12% were found to have DR, while PDR was present in 18.23% of cases. The average RC/HDL-c ratio was calculated as 0.67 ± 0.39. Utilizing a fully adjusted logistic regression model, we investigated the potential association between the TC/HDL-c ratio and both DR and PDR. However, no statistically significant association was observed (DR: OR 1.060; 95% CI 0.707, 1.588; PDR: OR 1.258; 95% CI 0.773, 2.047). Interestingly, we did discover a non-linear association between the RC/HDL-c ratio and DR. Employing a two-piece logistic regression model and a recursive algorithm, we identified an inflection point at 0.460. When the RC/HDL-c ratio fell below 0.460, each 1-unit increase in the ratio was associated with an 11.8-fold increase in the adjusted odds of developing DR (OR = 12.824; 95% CI 3.583, 45.897). Moreover, a non-linear association between the RC/HDL-c ratio and PDR was observed, with an inflection point occurring at 0.90. When the RC/HDL-c ratio was below 0.90, a one-unit increase in the ratio was linked to a 1.46-fold increase in the adjusted odds of PDR (OR = 2.459; 95% CI: 1.245, 4.857). Conclusion This study contributes valuable insights into the intricate association between the RC/HDL-c ratio and both DR and PDR in individuals diagnosed with T2DM. By identifying a non-linear association, our findings enhance the existing knowledge surrounding the link between the RC/HDL-c ratio and the development of DR and PDR.

Tao Sun, Jun Liu

BackgroundThis study seeks to investigate the association between the triglyceride-glucose index (TyG), triglyceride glucose index to high-density lipoprotein cholesterol ratio (TyG/HDL-c), and the risk of diabetes in individuals with nonalcoholic fatty liver disease (NAFLD).MethodsThis retrospective study encompassed 457 NAFLD patients from The Central Hospital of Shaoyang, monitored over a three-year period. Missing data were addressed using multiple imputation, and the Synthetic Minority Over-sampling Technique (SMOTE) was employed to balance the dataset. Multicollinearity analysis was conducted to evaluate the collinearity among variables, while principal component analysis was utilized to examine the distribution of variables in both the original and balanced datasets. A multivariate logistic regression model was used to assess the association between TyG, TyG/HDL-c, and the risk of diabetes in NAFLD patients, adjusting for various covariates. Subgroup analysis was performed to identify differences across diverse populations, and restricted cubic splines (RCS) were used to explore potential non-linear relationships. The receiver operating characteristic (ROC) curve examined the diagnostic value of individual and combined indicators in assessing the risk of diabetes in NAFLD patients.ResultsUpon adjustment for all covariates, TyG was found to significantly elevate the risk of diabetes among patients with NAFLD (OR = 1.96, 95% CI: 1.67-2.30, P &lt; 0.001), with a notable non-linear relationship observed (threshold: 2.39, P-nonlinear = 0.002). Similarly, TyG/HDL-c significantly increased diabetes risk (OR = 1.90, 95% CI: 1.60-2.26, P &lt; 0.001), also demonstrating a distinct non-linear association (threshold: 2.20, P-nonlinear &lt; 0.001). Subgroup analyses revealed significant interactions between TyG and TyG/HDL-c across different gender subgroups (P for interaction &lt; 0.05). The ROC curve analysis indicated that the combined use of TyG and TyG/HDL-c provided superior diagnostic performance for assessing diabetes risk in NAFLD patients (Area Under the Curve [AUC]: 0.703, 95% CI: 0.665-0.740), compared to the use of TyG (AUC: 0.694, 95% CI: 0.656-0.732) or TyG/HDL-c (AUC: 0.693, 95% CI: 0.655-0.731) independently.ConclusionBoth TyG and TyG/HDL-c are significantly associated with an increased risk of diabetes in NAFLD patients, exhibiting non-linear relationships. Furthermore, these associations vary significantly across gender subgroups, their combined use enhances risk assessment, supporting their clinical utility in evaluating diabetes risk.

Sarah Damanti, Sarah Damanti, Lorena Citterio et al.

BackgroundObesity and frailty are prevalent geriatric conditions that share some pathophysiological mechanisms and are associated with adverse clinical outcomes. The relationship between frailty, obesity, and polymorphism remains inadequately explored. Single nucleotide polymorphisms (SNPs) offer insights into genetic predispositions that may influence the development of both frailty and obesity.MethodsWe aimed at investigating whether SNPs associated with frailty also play a role in obesity. Data were collected from the FRASNET cross-sectional study, which included community-dwelling older individuals residing in Milan and nearby areas. Participants were recruited through random sampling. They underwent multidimensional geriatric assessments, which included the collection of blood samples for SNP analysis. Frailty was assessed using the frailty index, and body composition was evaluated using bioelectrical impedance analysis and anthropometric measures.ResultsSNPs related to frailty and linked to the renin–angiotensin system (CYP11B2 rs1799998, AGT rs5051, and AGTR1 rs2131127), apoptosis pathways (CASP8 rs6747918), growth hormone signaling (GHR rs6180), inflammation (TLR4 rs5030717, CD33 rs3865444, and FN1 rs7567647), adducin (ADD3 rs3731566), and the 9p21–23 region (rs518054) were found to be associated with various measures of obesity in community-dwelling older adults.ConclusionsFrailty-related SNPs contribute to obesity in community-dwelling older adults. We identified a novel association between adducin SNPs and visceral fat, which has not been previously reported. Detecting genetic predispositions to obesity and frailty early could aid in identifying individuals at risk, facilitating the adoption of preventive interventions. This represents an initial step toward promoting early intervention strategies.

Desiree Louise Draeger, Oliver W. Hakenberg

BackgroundTriple-drug cisplatin- and taxane-based chemotherapy is the standard treatment for metastatic penile squamous cell cancer (PeSCC), with a moderate response rate of 30% to 38%. Relapse after first-line chemotherapy has a poor prognosis and there is no established second-line treatment. Mitomycin C (MMC) is used as an effective chemotherapy in squamous cell carcinoma of other localities. We therefore used MMC as a single agent for the second-line treatment for patients with advanced PeSCC.MethodsNine patients [median age 63 years (range 31 years–81 years)], who, after inguinal and pelvic lymphadenectomy and progression after first-line chemotherapy, received second-line treatment with 20 mg of MMC administered intravenously and weekly, were included in this study. The median number of cycles of MMC was 6 (range 2–12 cycles) and the median cumulative dose was 120 mg absolute (range 40 mg absolute–240 mg absolute). The patients’ toxicity and treatment responses were evaluated, with the latter evaluated using 18F-FDG-PET/CT.ResultsCommon Terminology Criteria for Adverse Events (CTCAE) grades 3 or 4 thrombocytopenia and grades 2 or 3 leukopenia occurred in all patients, as did anemia. In seven patients, the application interval had to be extended due to thrombocytopenia. Stable disease was achieved in two patients, and all others progressed under treatment. Seven patients died of the disease, with most patients dying 6 months after starting MMC therapy. Of the two patients who responded with disease stabilization, one died of progressive disease 14 months after MMC treatment. The other responding patient has been stable for over 1 year and is still receiving treatment, which he tolerates well, and has a good quality of life.ConclusionMMC has only moderate efficacy as a second-line treatment in patients with metastatic PeSCC. With MMC treatment, hematological toxicity is marked.

Mohammed A. Talle, Anton F. Doubell, Pieter-Paul S. Robbertse et al.

(1) Background: Altered cardiac morphology and function are associated with increased risks of adverse cardiac events in hypertension. Our study aimed to assess left ventricular (LV) morphology, geometry, and function using cardiovascular magnetic resonance (CMR) imaging in patients with hypertensive crisis. (2) Methods: Patients with hypertensive crisis underwent CMR imaging at 1.5 Tesla to assess cardiac volume, mass, function, and contrasted study. Left ventricular (LV) function and geometry were defined according to the guideline recommendations. Late gadolinium enhancement (LGE) was qualitatively assessed and classified into ischemic and nonischemic patterns. Predictors of LGE was determined using regression analysis. (3) Results: Eighty-two patients with hypertensive crisis (aged 48.5 ± 13.4 years, and 57% males) underwent CMR imaging. Of these patients, seventy-eight percent were hypertensive emergency and twenty-two percent were urgency. Diastolic blood pressure was higher under hypertensive emergency (<i>p</i> = 0.032). Seventy-nine percent (92% of emergency vs. 59% of urgency, respectively; <i>p</i> = 0.003) had left ventricular hypertrophy (LVH). The most prevalent LV geometry was concentric hypertrophy (52%). Asymmetric LVH occurred in 13 (22%) of the participants after excluding ischemic LGE. Impaired systolic function occurred in 46% of patients, and predominantly involved hypertensive emergency. Nonischemic LGE occurred in 75% of contrasted studies (67.2% in emergency versus 44.4% in urgency, respectively; <i>p</i> < 0.001). Creatinine and LV mass were independently associated with nonischemic LGE. (5) Conclusion: LVH, altered geometry, asymmetric LVH, impaired LV systolic function, and LGE are common under hypertensive crisis. LVH and LGE more commonly occurred under hypertensive emergency. Longitudinal studies are required to determine the prognostic implications of asymmetric LVH and LGE in hypertensive crisis.

Wilmer Stalin Sanango Reinoso, Jorge Oswaldo Herrera Ordoñez , Soraya Puertas Azanza et al.

Introducción: El control de la tensión arterial (TA), es un objetivo fundamental, en los pacientes con enfermedad renal crónica estadio 5D, ya que la hipertensión y la hipotensión causan efectos adversos en la calidad de vida del paciente y predisponen al aumento del riesgo cardiovascular. El objetivo del presente estudio fue realizar un análisis económico de la prescripción de antihipertensivos en un grupo de pacientes en terapia sustitutuva de la función renal. Métodos: Se trata de un estudio transversal, realizado en el servicio de hemodiálisis del hospital “José Carrasco Arteaga”. Las variables fueron: tipo de diálisis, sexo, uso de antihipertensivos, tipo de antihipertensivos y costo. La muestra fue no probabilística. Resultados: Se analizan 174 pacientes, 73 (41.95%) en programa de hemodiálisis (HD), 80 (45.98%) en programa de diálisis peritoneal manual (DP) y 21 (12.07%) en diálisis peritoneal automatizada (DPA). Fueron 74% de hombres en HD, 39 casos (48.7%) en DP, 12 casos (57.14%) en DPA. El uso de antihipertensivos en HD fue del 84%, en DP fue del 86% y en DPA fue del 86%. El costo de antihipertensivos fue en  HD 12.5 ± 8.7 USD, en DP 15.7 ± 22.4 y en DPCA  16.4 ± 18.6 USD P>0.05. Conclusión: el uso de antipertensivos en este grupo de estudio fue en mas del 84% en las diferentes modalidades de diálisis, sin diferencia estadítica en el análisis de costo.

Ricardo Fontes-Carvalho, Gláucia Maria Moraes de Oliveira, Pedro Gonçalves-Teixeira et al.

Tiago Sepúlveda Santos, Rita Marçal, Oana Moldovan et al.

Type IV Ehlers-Danlos syndrome (vascular) is a rare connective tissue disease caused by COL3A1 gene mutation on type III collagen. Clinical presentation is related to vascular fragility and risk of rupture of the arterial wall. Definite diagnosis is given by genetic study and the approach to these patients requires a multidisciplinary team and effective blood pressure control. There is currently only one medication with potential benefit in prevention of cardiovascular events: celiprolol. This article describes the case of a 41-year-old female patient, diagnosed with vascular Ehlers-Danlos syndrome after multiple major cardiovascular events: aortic, coronary and carotid dissections and venous and arterial thrombosis. These required multiple surgical interventions and long-term admission in intensive care units leading to complete functional recovery. This case report seeks to stress the need for an early diagnosis to prevent the severe cardiovascular complications of this rare syndrome. Resumo: A síndrome de Ehlers-Danlos tipo IV (vascular) é uma doença rara do tecido conjuntivo, caracterizada por uma mutação no gene COL3A1 do colagénio tipo III. A apresentação clínica cursa com fragilidade vascular e risco de rutura da parede arterial em idades jovens. O diagnóstico definitivo é estabelecido pelo estudo genético e a abordagem destes doentes assenta numa equipa multidisciplinar e num eficaz controlo da pressão arterial, existindo atualmente um único fármaco com aparente benefício na prevenção de eventos vasculares, o celiprolol. Este artigo descreve o caso de uma doente, cujo diagnóstico de síndrome de Ehlers-Danlos vascular foi feito aos 41 anos, na sequência múltiplos eventos cardiovasculares major, a saber: dissecção aórtica, coronária, carotídea, tromboses arteriais e venosas, que implicaram múltiplas intervenções cirúrgicas e internamento prolongado em Unidade de Cuidados Intensivos, com total recuperação funcional. Pretende-se, com este caso, salientar a gravidade das manifestações cardiovasculares desta síndrome e a necessidade do diagnóstico precoce, com o objectivo de prevenção das mesmas.

Xiaoxue Xie, Caiping Chen, Wenting Chen et al.

B7 family members and their receptors play key roles in regulating T cell responses, and constitute very attractive targets for developing immunotherapeutic drugs. V-Set and Immunoglobulin domain containing 3 (VSIG3), a ligand for the novel B7 family immune checkpoint V-domain immunoglobulin suppressor of T cell activation (VISTA), can significantly inhibit T cell functions. Inhibitors targeting the VISTA/VSIG3 pathway are of great significance in tumor immunology. Here, we show the crystal structure of the extracellular domain (ECD) of the human VSIG3 protein at 2.64 angstrom resolution, and we produce recombinant human VSIG-3 ECD in both CHO cells and E. coli. Furthermore, we demonstrated the interaction of VISTA and VSIG3 by coimmunoprecipitation (Co-IP). Based on protein-protein docking for VISTA and VSIG3, we report a small molecule inhibitor of VSIG3 K284-3046 and evaluate its biological activities in vitro. This study was the first to reveal the crystal structure of VSIG3, and provides the structural basis for designing antibodies or compounds for the unique VSIG3/VISTA coinhibitory pathway in the treatment of cancers, autoimmune diseases and may be beneficial of designing vaccines.

Johana Maria Guevara-Morales, Olga Yaneth Echeverri-Peña

Background and aims: Cystinosis is an inborn error of metabolism, clinically characterised by severe renal involvement and development of corneal cystine deposits, especially in the adult form of the disease. Cystinosis is a treatable condition. Therefore, an early diagnosis is necessary to start therapy. For biochemical confirmation of the condition it is necessary to quantify intracellular cystine concentrations. For this, different methods have been described with variations in cell isolation strategies and the amino acid quantification techniques used. In order to improve confirmatory biochemical diagnosis in our setting, a protocol for intraleukocitary cystine quantification was established. Methods: A high performance liquid chromatography based method for cystine quantification in polymorphonuclear cells was implemented. Evaluation of the best anticoagulant to use and temperature stability of the sample at 4 °C were performed. In addition, we established reference values for our population. Results: It was determined that intraleukocitary cystine quantification must be performed in blood samples containing acid-citrate-dextrose (ACD) as anticoagulant. Samples must be processed immediately due to their poor stability even when refrigerated. Based on the results from 50 healthy individuals, the cut-off point established for our population was 0.34 nmol1/2/cystine/mg. Conclusion: The adaptation performed to the cystine quantification method here presented the highest control population that has been reported in the literature so far. Our results highlight the need for making available a cystine quantification method locally and confirm the convenience for each laboratory to establish its own reference values to provide greater reliability for interpreting results. Resumen: Antecedentes y objetivos: La cistinosis es un error innato del metabolismo cuyas características clínicas incluyen compromiso renal severo, formación de cristales de cistina en la córnea, especialmente en la presentación adulta de la enfermedad. Es una enfermedad tratable por lo cual establecer el diagnostico de forma oportuna es fundamental para iniciar terapia. Para la confirmación bioquímica de la enfermedad se requiere determinar las concentraciones intracelulares de cistina, para lo cual se han reportado diferentes métodos tanto para el aislamiento de las células como las técnicas de cuantificación del aminoácido. Con el objetivo de mejorar el diagnóstico bioquímico confirmatorio en nuestro medio establecimos un protocolo de cuantificación intraleucocitaria de cistina. Métodos: Se realizó implementación de un método de cuantificación de cistina en polimorfonucleares por cromatografía líquida de alta resolución, evaluando el mejor anticoagulante a utilizar, la estabilidad de la muestra a 4 °C y estableciendo valores de referencia para nuestra población. Resultados: Se determinó que la muestra para cuantificación intraleucocitaria de cistina debe ser anticoagulada mediante adición de ácido Cítrico-Dextrosa (ACD) como anticoagulante. La muestra debe ser procesada inmediatamente dada su baja estabilidad incluso en refrigeración. Con 50 individuos sanos se estableció como punto de corte para nuestra población 0.34 nmol1/2cistina/mg. Conclusión: La adaptación realizada del método de cuantificación de cistina utiliza el número más alto de muestras control hasta ahora reportado en la literatura. Nuestros resultados dan cuenta de la necesidad de implementar el método a nivel local y reafirman la conveniencia de que cada laboratorio establezca sus propios valores de referencia para proporcionar una mayor confiabilidad a la hora de interpretar los resultados. Keywords: Cystinosis, Aminoacidopathy, Inborn error of metabolism, Intraleukocitary cystine, Fanconi syndrome., Palabras clave: Cistinosis, Aminoacidopatia, Error innato del metabolismo, Cistina intraleucocitaria, Síndrome de fanconi

Shigeru Fujisaki, Motoi Takashina, Ken-ichi Sakurai et al.

Abstract Background Hilar biliary stricture caused by isolated fungal infections in immunocompetent patients are considered to be extremely rare and difficult to the diagnose from the outset. Case presentation We report a unique case of granulomatous cholangitis based on isolated biliary fungal infection manifesting as obstructive jaundice and mimicking hilar cholangiocarcinoma in an immunocompetent woman. A 67-year-old Japanese woman was referred to our hospital for obstructive jaundice. She had been followed up for hypochondroplasia by the referring physician. Her total bilirubin level was 5.4 mg/dL. Viral hepatitis screening was found to be negative, and serum IgG4 was within normal limits; however, her CA19-9 level was high. Abdominal computed tomography revealed dilatation of the intrahepatic bile ducts. Abdominal echogram detected a solid mass in the hilar bile duct. Her magnetic resonance cholangiopancreatography has also revealed an abrupt stenosis of the primary biliary confluence with upstream dilatation of the intrahepatic bile ducts. Endoscopic nasobiliary drainage was then performed to improve the obstructive jaundice. Although biliary cytology did not reveal malignant findings, the bile duct in the hilum showed severe stenosis, and hilar cholangiocarcinoma could not be completely excluded. The patient had a developmental disorder based on chondrodystrophy. To avoid excessive surgical stress, such as hepatic lobectomy, we performed resection of the extrahepatic bile duct and Roux-en-Y hepaticojejunostomy reconstruction. Intraoperative frozen sections of the resection margins were determined to be negative for tumor. The resected specimen showed multiple strictures inside the common bile duct, numerous calculi in the lumen, and little free space. The final pathological diagnosis was granulomatous cholangitis due to fungal infection. The patient’s postoperative course was deemed uneventful. She was discharged from our hospital 23 days after surgery without antifungal treatment. Conclusions For a unique case of granulomatous cholangitis based on isolated biliary fungal infection mimicking hilar cholangiocarcinoma, we were able to avoid excessive invasion and performed appropriate surgical management.

Olga Lamacchia, Francesca Viazzi, Paola Fioretto et al.

Abstract Background We described, in a large sample of patients with type 1 diabetes mellitus (T1DM) and GFR ≤ 60 mL/min/1.73 m2 (with or without albuminuria), the differences in the clinical features associated with the two different chronic kidney disease phenotypes and we investigated, in a subset of patients, the modulating role of albuminuria on kidney disease progression. Methods Clinical data of 1395 patients with T1DM were extracted from electronic medical records. Results Albuminuria was detected in 676 (48.5%) patients, with the remaining 719 (51.5%) patients having normoalbuminuric renal impairment. Those with albuminuria showed an evident worse cardiovascular risk profile as compared to patients with normoalbuminuria. A subgroup of 582 patients was followed up over a 4-year period. One hundred and twenty five patients (21.5%) showed a loss of eGFR > 30%. The proportion of patients reaching the renal outcome was significantly higher among those with baseline albuminuria as compared to patients with normoalbuminuria (P < 0.0001). At the multivariate logistic analysis microalbuminuria, macroalbuminuria and proliferative retinopathy were the only parameters independently associated to eGFR reduction. Conclusions The proportion of T1DM patients with normoalbuminuria renal impairment is high (about 50%). These patients have a slower eGFR decline as compared to that observed in patients with albuminuria renal impairment.

S. Yu. Martsevich, Yu. V. Lukina, N. P. Kutishenko et al.

Aim. To assess quality of life (QoL) and its dynamics during nicorandil treatment in patients with stable ischemic heart disease, to study the relationship of treatment adherence and QoL indicators when treated with nicorandil.Material and methods. Observational program NIKEA included 590 patients with angina pectoris. Seattle Angina Questionnaire (SAQ) was used to assess QoL. Patients completed SAQ at the visit of inclusion (V0) and at the visit after 3 months of observation (V3). Potential and actual adherence to therapy was assessed by medical interviews at visits V0, V1 (1 month of observation) and V3. During the visit V0 417 people (from 590 ones enrolled into the study) completed SAQ (71% response); after 3 months (V3) SAQ was filled in by 454 of 552 people who came to this visit (82% response). According to the results of medical interviews, potential adherence (visit V0) was determined in all 590 patients, actual adherence to nicorandil (visits V1 and V3) was assessed in 552 patients who came to these visits. In accordance with the degree of adherence, all patients were divided into 3 groups: (1) adherent to treatment (taking nicorandil for the first three months), (2) non-adherent (who refused to take nicorandil), and (3) partially non-adherent (who started nicorandil, but for various reasons stopped taking the drug).Results. In all patients, regardless of their adherence to the recommended drug, there was an increase in QoL according to all five scales of the SAQ after 3 months of follow-up. Statistically significant positive dynamics of all SAQ indicators was found only in adherent patients (p&lt;0.0001 for all aspects). Patients, who showed good adherence to nicorandil at V1, had more severe angina at the beginning of treatment (according to “Angina Stability” and “Angina Frequency” indicators). These patients also had lower QoL “Disease Perception” score and more confidence in the doctor (“Treatment Satisfaction” score) than non-adherent patients (p&lt;0.05). In non-adherent patients a mild degree of angina was determined 2 times more often according to “Angina Frequency” indicator (p=0.03).Conclusion. The results of the study confirm the interrelation and mutual influence of the QoL indicators and treatment adherence to nicorandil. Effective treatment with nicorandil in patients with lower health-related QoL indicators could increase treatment adherence. On the other hand, the mild degree of angina, the low level of confidence in the treating doctor, the side effects of the new drug reduce medication adherence of patients, which, as a result, negatively affects the QoL of these patients

Brittney A. Frankel, Tara F. Bishop

M. Tanriover, Shirley Rigby, L. Harry van Hulsteijn et al.

Elizabeth R. Trowbridge, C. Bartels, S. Koslov et al.