Somatic Symptoms of Depression Lose Association with Mortality upon Adjustment for Frailty: Analysis from the Fitness Haemodialysis Cohort.

Introduction: The somatic symptom component of depression is associated with increased hospitalisation and mortality and poorer health-related quality of life (HRQOL). However, the relationship of subsets of depression symptoms with frailty and outcomes is not known. This study aimed to (1) explore the relationship between the Clinical Frailty Scale (CFS) and components of depression and (2) their association with mortality, hospitalisation, and HRQOL in haemodialysis recipients. Methods: We conducted a prospective cohort study of prevalent haemodialysis recipients, with deep bio-clinical phenotyping including CFS and PHQ-9 somatic (fatigue, poor appetite, and poor sleep) and cognitive component scores. EuroQol EQ-5D summary index assessed HRQOL at the baseline. Electronic linkage to English national administration datasets ensured robust follow-up data for hospitalisation and mortality events. Findings. Somatic (ß = 0.067; 95% C.I. 0.029 to 0.104; P < 0.001) and cognitive (ß = 0.062; 95% C.I. 0.034 to 0.089; P<0.001) components were associated with increased CFS scores. Both somatic (ß = -0.062; 95% C.I. -0.104 to -0.021; P<0.001) and cognitive (ß = 0.052; 95% C.I. -0.081 to -0.024; P < 0.001) scores were associated with lower HRQOL. Somatic scores lost mortality association on addition of CFS to the multivariable model (HR1.06; 95% C.I. 0.977 to 1.14; P=0.173). Cognitive symptoms were not associated with mortality. Neither the component score was associated with hospitalisation on multivariable analyses. Conclusions: Both somatic and cognitive depression symptoms are associated with frailty and poorer HRQOL in haemodialysis recipients but were not associated with mortality or hospitalisation when adjusted for frailty. The risk profile of depression somatic scores may be related to overlap with symptoms of frailty.

Gender Disparity in Expression of Sarcopenia in Haemodialysis Recipients: Analysis from the FITNESS Cohort.

Background: There has been little exploration of the interplay between sarcopenia and frailty in haemodialysis, particularly regarding gender difference. We aimed to (1) assess whether ultrasound-derived low muscle mass (LMM) and sarcopenia are more common in male or female haemodialysis recipients; (2) assess whether age influences any observed gender difference, and (3) explore the interplay between sarcopenia, frailty, and gender in haemodialysis recipients. Methods: This was an exploratory analysis of a subgroup of adult prevalent (≥3 months) haemodialysis with frailty phenotype (FP) scores. Bilateral anterior thigh thickness (BATT) was obtained according to an established ultrasound protocol. Associations with frailty were explored via both linear and logistic regressions for BATT, LMM, and sarcopenia with a priori covariables, stratified by gender. Results: In total of 223 studies, participants had ultrasound measurements. Males showed greater prevalence of LMM. On adjusted analyses, LMM was associated with lower hand grip strength in males (ß = -4.17; 95% C.I. -7.57 to -0.77; P=0.02), but not females (ß = -1.88; 95% C.I. -5.41 to 1.64; P=0.29). LMM was also associated with slower walking speed in both males (ß = -0.115; 95% C.I. -0.258 to -0.013; P=0.03) and females (ß = -0.152; 95% C.I. -0.300 to -0.005; P=0.04). Sarcopenia was associated with greater odds of frailty on adjusted models in males (OR = 9.86; 95% C.I. 1.8 to 54.0; P=0.01), but not females (OR = 5.16; 95% C.I. 0.22 to 124; P=0.31). Conclusions: The clinical expression and significance of sarcopenia differ substantially between males and females on haemodialysis. Further work is required to elucidate underlying mechanisms and guide tailored treatment.

Cognitive Impairment, Frailty, and Adverse Outcomes Among Prevalent Hemodialysis Recipients: Results From a Large Prospective Cohort Study in the United Kingdom.

Rationale & Objective: Frailty and cognitive impairment are common in hemodialysis recipients and have been associated with high mortality. There is considerable heterogeneity in frailty reporting, with little comparison between commonly used frailty tools and little exploration of the interplay between cognition and frailty. The aims were to explore the relationship between frailty scores and cognition and their associations with hospitalization and mortality. Study Design: Prospective cohort study. Setting & Population: Prevalent hemodialysis recipients linked to national datasets for hospitalization and mortality. Predictors: Montreal Cognitive Assessment (MoCA), Frailty Phenotype, Frailty Index (FI), Edmonton Frailty Scale, and Clinical Frailty Scale (CFS) were performed at baseline. Cognitive impairment was defined as MoCA scores of <26, or <21 in dexterity impairment, <18 in visual impairment. Outcomes: Mortality, hospitalization. Analytical Approach: Cox proportional hazards model for mortality, censored for end of follow-up. Negative binomial regression for admission rates, censored for death/end of follow-up. Results: In total, 448 participants were recruited with valid MoCAs and followed up for a median of 685 days. There were 103 (23%) deaths and 1,120 admissions of at least one night. Cognitive impairment was identified in 346 (77.2%) participants. Increasing frailty by all definitions was associated with poorer cognition. Cognition was not associated with mortality (HR, 0.99; 95% CI, 0.95-1.03; P = 0.41) or hospitalization (IRR, 1.01; 95% CI, 0.99-1.04; P = 0.39) on multivariable analyses. There were interactions between MoCA scores and increasing frailty by FI (P = 0.002) and Clinical Frailty Scale (P = 0.005); admissions were highest when both MoCA and frailty scores were high, and when both scores were low. Limitations: As frailty is a dynamic state, a single cross-sectional assessment may not accurately reflect its year-to-year variability. In addition, these findings are in maintenance dialysis and may not be transferable to incident hemodialysis. There were small variations in application of frailty tool criteria from other studies, which may have influenced the results. Conclusions: Cognitive impairment is highly prevalent in this hemodialysis cohort. The interaction between cognition and frailty on rates of admission suggests the MoCA offers value in identifying higher risk hemodialysis populations with both high and low degrees of frailty.

A clinical frailty scale obtained from MDT discussion performs poorly in assessing frailty in haemodialysis recipients.

BACKGROUND: The Clinical Frailty Scale (CFS) is a commonly utilised frailty screening tool that has been associated with hospitalisation and mortality in haemodialysis recipients, but is subject to heterogenous methodologies including subjective clinician opinion. The aims of this study were to (i) examine the accuracy of a subjective, multidisciplinary assessment of CFS at haemodialysis Quality Assurance (QA) meetings (CFS-MDT), compared with a standard CFS score via clinical interview, and (ii) ascertain the associations of these scores with hospitalisation and mortality. METHODS: We performed a prospective cohort study of prevalent haemodialysis recipients linked to national datasets for outcomes including mortality and hospitalisation. Frailty was assessed using the CFS after structured clinical interview. The CFS-MDT was derived from consensus at haemodialysis QA meetings, involving dialysis nurses, dietitians, and nephrologists. RESULTS: 453 participants were followed-up for a median of 685 days (IQR 544-812), during which there were 96 (21.2%) deaths and 1136 hospitalisations shared between 327 (72.1%) participants. Frailty was identified in 246 (54.3%) participants via CFS, but only 120 (26.5%) via CFS-MDT. There was weak correlation (Spearman Rho 0.485, P < 0.001) on raw frailty scores and minimal agreement (Cohen's κ = 0.274, P < 0.001) on categorisation of frail, vulnerable and robust between the CFS and CFS-MDT. Increasing frailty was associated with higher rates of hospitalisation for the CFS (IRR 1.26, 95% C.I. 1.17-1.36, P = 0.016) and CFS-MDT (IRR 1.10, 1.02-1.19, P = 0.02), but only the CFS-MDT was associated with nights spent in hospital (IRR 1.22, 95% C.I. 1.08-1.38, P = 0.001). Both scores were associated with mortality (CFS HR 1.31, 95% C.I. 1.09-1.57, P = 0.004; CFS-MDT HR 1.36, 95% C.I. 1.16-1.59, P < 0.001). CONCLUSIONS: Assessment of CFS is deeply affected by the underlying methodology, with the potential to profoundly affect decision-making. The CFS-MDT appears to be a weak alternative to conventional CFS. Standardisation of CFS use is of paramount importance in clinical and research practice in haemodialysis. TRIAL REGISTRATION: Clinicaltrials.gov : NCT03071107 registered 06/03/2017.

Depression is associated with frailty and lower quality of life in haemodialysis recipients, but not with mortality or hospitalization.

Background: Frailty and depression are highly prevalent in haemodialysis recipients, exhibit a reciprocal relationship, and are associated with increased mortality and hospitalization, and lower quality of life. Despite this, there has been little exploration of the relationship between depression and frailty upon patient outcomes. We aimed to explore the relationship between depression and frailty, and their associations with mortality, hospitalization and quality of life. Methods: We performed a prospective cohort study of prevalent haemodialysis recipients linked to national datasets for outcomes including mortality and hospitalization. Depression was assessed using the Patient Health Questionnaire-9 (PHQ-9), frailty using the Clinical Frailty Scale (CFS) and quality of life using the EuroQol 5-Dimension (EQ-5D) Summary Index. Results: A total of 485 prevalent haemodialysis recipients were recruited, with 111 deaths and 1241 hospitalizations during follow-up. CFS was independently associated with mortality [hazard ratio (HR) 1.31; 95% confidence interval (CI) 1.08, 1.59; P = .006], hospitalization [incidence rate ratio (IRR) 1.13; 95% CI 1.03, 1.25; P = .010] and lower quality of life (Coef. -0.401; 95% CI -0.511, -0.292; P < .001). PHQ-9 score was independently associated with lower quality of life (Coef. -0.042; 95% CI -0.063, -0.021; P < .001), but not mortality (HR 1.00; 95% CI 0.96, 1.04; P = .901) or hospitalization (IRR 0.99; 95% CI 0.97, 1.01; P = .351). In an adjusted model including CFS, moderate depression was associated with reduced hospitalization (IRR 0.72; 95% CI 0.56, 0.93; P = .013). Conclusions: With the addition of frailty, depression was associated with lower hospital admissions, but poorer quality of life. The relationship between frailty and depression, and their influence on outcomes is complex, requiring further study.

Ultrasound quadriceps muscle thickness is variably associated with frailty in haemodialysis recipients.

BACKGROUND: Ultrasonographic quantitation of quadriceps muscle mass is increasingly used for assessment of sarcopenia, but its relationship with frailty in haemodialysis recipients is not known. This study explores the relationship between ultrasound-derived bilateral anterior thigh thickness (BATT), sarcopenia, and frailty by common frailty tools (Frailty Phenotype [FP], Frailty Index [FI], Edmonton Frailty [EFS], and Clinical Frailty Scale [CFS]). METHODS: This was an exploratory analysis of a subgroup of adult prevalent (≥3 months) haemodialysis recipients deeply phenotyped for frailty. Ultrasound assessment of BATT was obtained with participants at an angle of ≤45°, with legs outstretched and knees resting at 10°-20°, according to an established protocol. Associations with frailty were explored via both linear and logistic regressions for BATT, Low Muscle Mass (LMM), and sarcopenia with stepwise adjustment for a priori covariables. RESULTS: In total 223 study participants had ultrasound measurements. Frailty ranged from 34% for FP to 58% for FI. BATT was associated with increasing frailty on simple linear regression by all frailty tools, but lost significance on addition of covariables. Upon dichotomising frailty tools into Frail/Not Frail, BATT was associated with frailty by all tools on univariable analyses, but only retained association for EFS on the fully adjusted model (OR 0.97, 95% C.I. 0.94-1.00, P = 0.05). CONCLUSIONS: Ultrasound measures of quadriceps thickness is variably associated with frailty in prevalent haemodialysis recipients, dependent upon the frailty tool used, but not independent of other variables. Further work is required to establish the added value of sarcopenia measurement in frail haemodialysis patients. TRIAL REGISTRATION: Clinicaltrials.gov : NCT03071107 registered 06/03/2017.

Self-reported health change in haemodialysis recipients modulates the effect of frailty upon mortality and hospital admissions: outcomes from a large prospective UK cohort.

BACKGROUND: Frailty among haemodialysis patients is associated with hospitalization and mortality, but high frailty prevalence suggests further discrimination of risk is required. We hypothesized that incorporation of self-reported health with frailty measurement may aid risk stratification. METHODS: Prospective cohort study of 485 prevalent haemodialysis recipients linked to English national datasets. Frailty Phenotype (FP), Frailty Index (FI), Edmonton Frail Scale (EFS), Clinical Frailty Scale (CFS) and self-reported health change were assessed. Mortality was explored using Fine and Gray regression, and admissions by negative binomial regression. RESULTS: Over a median 678 (interquartile range 531-812) days, there were 111 deaths, and 1241 hospitalizations. Increasing frailty was associated with mortality on adjusted analyses for FP [subdistribution hazard ratio (SHR) 1.26, 95% confidence interval (CI) 1.05-1.53, P = .01], FI (SHR 1.21, 95% CI 1.09-1.35, P = .001) and CFS (SHR 1.32, 95% CI 1.11-1.58, P = .002), but not EFS (HR 1.08, 95% CI 0.99-1.18, P = .1). Health change interacted with frailty tools to modify association with mortality; only those who rated their health as the same or worse experienced increased mortality hazard associated with frailty by FP (Pinteraction = .001 and 0.035, respectively), FI (Pinteraction = .002 and .007, respectively) and CFS (Pinteraction = .009 and 0.02, respectively). CFS was the only frailty tool associated with hospitalization (incidence rate ratio 1.12, 95% CI 1.02-1.23, P = .02). CONCLUSIONS: We confirm the high burden of hospitalization and mortality associated with haemodialysis patients regardless of frailty tool utilized and introduce the discriminatory ability of self-reported health to identify the most at-risk frail individuals.

Polineuropatía por amiloidosis por transtiretina de inicio tardío. Caso clínico / Late-onset hereditary transthyretin amyloidosis with polyneuropathy. Report of one case

Hereditary transthyretin amyloidosis is a multisystemic autosomal dominant genetic disorder characterized by progressive distal sensory-motor polyneuropathy or restrictive cardiomyopathy, secondary to amyloid deposits. Its pathogenesis lies in the TTR gene mutation, and the Val50Met mutation is the most frequent. Patients have significant differences in the onset and severity of clinical presentation according to their country of origin. The diagnosis of this pathology is complex, even more in countries where it is not considered endemic. However, early suspicion and management are essential to improve survival and avoid unnecessary diagnostic and therapeutic strategies. We report a 69-year-old woman who presented a sensory-motor polyneuropathy, predominantly sensory, associated with distal neuropathic pain and bilateral vitritis. The history of her Italian father with polyneuropathy of unspecified etiology stood out. A vitreous biopsy identified amyloid substance deposits (congo red positive). These were also confirmed on a superficial peroneal nerve biopsy. During the etiological study of her polyneuropathy, an increased Kappa/Lambda index of 2.55 mg/L stood out. Therefore, light chain amyloidosis was suspected, and chemotherapy treatment was indicated without favorable response. After 10 years of progressive neurological and ophthalmological involvement, a genetic study confirmed the first case of late-onset hereditary transthyretin amyloidosis Val50Met with polyneuropathy in Chile.

Correlations, agreement and utility of frailty instruments in prevalent haemodialysis patients: baseline cohort data from the FITNESS study.

BACKGROUND: Frailty is associated with poor outcomes for haemodialysis patients, but its prevalence is uncertain due to heterogeneous definitions. The aim of this study was to compare and contrast prevalence and features of commonly used frailty instruments in a British haemodialysis cohort. METHODS: The FITNESS (Frailty Intervention Trial iN End-Stage patientS on haemodialysis) study recruited adults aged ≥18 years after informed consent, with ≥3 months haemodialysis exposure and no hospital admission within 4 weeks unless for dialysis access. Study participants were clinically phenotyped with frailty instruments including the Frailty Index (FI), Frailty Phenotype (FP), Edmonton Frailty Scale (EFS) and Clinical Frailty Scale (CFS), alongside comprehensive baseline data collection of biochemical, clinical and social characteristics. RESULTS: Between 12 January 2018 and 18 April 2019, 485 haemodialysis patients were recruited. Baseline demographics were median age 63 years, male sex 58.6% and non-White ethnicity 42.1%. Prevalence of frailty was high; 41.9% of participants were frail by FP, 63.3% by FI, 50.2% by EFS and 53.8% by CFS. Female gender was associated with increased frailty, with no independent association observed with age or ethnicity. While correlation between frailty instruments was strong, intraclass correlation coefficient for frailty agreement was 0.628 (95% confidence interval 0.585-0.669) and only weak agreement between instrument pairs. CONCLUSION: Frailty is highly prevalent among haemodialysis patients regardless of criteria used. However, our data suggest caution when interpreting heterogenous definitions of frailty for haemodialysis patients as they are not interchangeable. Consensus agreement on the optimal frailty definition for haemodialysis patients must balance ease of use with predictive ability for adverse outcomes before determining clinical application.

[Late-onset hereditary transthyretin amyloidosis with polyneuropathy. Report of one case]. / Polineuropatía por amiloidosis por transtiretina de inicio tardío. Caso clínico.

Hereditary transthyretin amyloidosis is a multisystemic autosomal dominant genetic disorder characterized by progressive distal sensory-motor polyneuropathy or restrictive cardiomyopathy, secondary to amyloid deposits. Its pathogenesis lies in the TTR gene mutation, and the Val50Met mutation is the most frequent. Patients have significant differences in the onset and severity of clinical presentation according to their country of origin. The diagnosis of this pathology is complex, even more in countries where it is not considered endemic. However, early suspicion and management are essential to improve survival and avoid unnecessary diagnostic and therapeutic strategies. We report a 69-year-old woman who presented a sensory-motor polyneuropathy, predominantly sensory, associated with distal neuropathic pain and bilateral vitritis. The history of her Italian father with polyneuropathy of unspecified etiology stood out. A vitreous biopsy identified amyloid substance deposits (congo red positive). These were also confirmed on a superficial peroneal nerve biopsy. During the etiological study of her polyneuropathy, an increased Kappa/Lambda index of 2.55 mg/L stood out. Therefore, light chain amyloidosis was suspected, and chemotherapy treatment was indicated without favorable response. After 10 years of progressive neurological and ophthalmological involvement, a genetic study confirmed the first case of late-onset hereditary transthyretin amyloidosis Val50Met with polyneuropathy in Chile.

Epidemiological and clinical characteristics and outcome of monoclonal gammopathy of renal significance-related lesions in Latin America.

BACKGROUND: Monoclonal gammopathy of renal significance (MGRS)-related lesions are infrequent entities. There are no publications on these disorders in Latin America (LA). The aim of this study was to describe epidemiological and clinical characteristics of these patients in LA. METHODS: We performed a multicentre retrospective study. Patients with diagnosis of MGRS between 2012 and 2018 were included. Epidemiological and clinical data were collected from clinical records. RESULTS: Twenty-seven patients from Chile, Argentina, Ecuador and Uruguay were included. Half debuted with a nephrotic syndrome, and 32% required dialysis. Proliferative glomerulonephritis with monoclonal immunoglobulin deposits was found in 33%, amyloidosis in 26% and monoclonal immunoglobulin deposition disease also in 26%. The immunoglobulin most frequently found in renal biopsies was IgG kappa. In 67% a paraprotein was found. Twenty patients received an anti-plasma cell regimen, and 3 a rituximab-based regimen (IgM-MGRS). Renal response (RR) was achieved in 56%. Early treatment (≤3 months) was associated with higher RR (75% vs 43%). Three patients relapsed within 21.5 months, and 3 progressed: 1 to multiple myeloma, 1 to systemic amyloidosis and another to systemic light-chain deposition disease. Two patients died, both due to infection during induction treatment. CONCLUSION: There was a higher than expected frequency of patients requiring dialysis. The most common MGRS-related lesion was PGNMD. Early treatment was associated with better response. As a rare disease, increasing awareness and promoting early diagnosis are necessary in LA to improve outcomes. SUMMARY AT A GLANCE A collection of 27 cases of MGRS from Latin America with information on epidemiology, clinical characteristics, treatment and outcome of patients diagnosed of MGRS-related renal lesions.

Prognostic impact of renal failure recovery in patients with newly diagnosed multiple mieloma / Impacto pronóstico de remisión renal en pacientes con mieloma múltiple de reciente diagnóstico

ABSTRACT Background Renal failure (RF) is a common complication in patients with newly diagnosed multiple myeloma (NDMM). Aim To evaluate the frequency of RF in NDMM patients, and the prognostic impact of its reversibility. Material and Methods A retrospective study evaluating demographic and clinical characteristics of 154 consecutive patients with NDMM was carried out. Estimated glomerular filtration rate (eGFR) was calculated at the beginning and at the end of the induction therapy. In addition, we evaluated renal responses (RR) according to the International Myeloma Working Group (IMWG) criteria. The induction regimen was based on thalidomide in all cases. Results Fifty-three patients had RF (34.4%). Complete renal response (RR) was achieved in 51%. Three years overall survival in patients without RF, with RF and complete RR, and patients with RF and any other RR, was 66, 47 and 13%, respectively. Median survival was 53, 27 and 6 months, respectively (p < 0.01). In the multivariate analysis, RF and hypercalcemia were independent predictors of a worse outcome. Conclusions Achieving a complete RR in patients with NDMM, is associated with a better survival.

Antecedentes La falla renal (FR) es una complicación frecuente en pacientes con mieloma múltiple (MM). Objetivo Evaluar la frecuencia de FR en pacientes con reciente diagnóstico de MM y determinar la importancia pronóstica de su reversibilidad. Material y Métodos Se realizó un estudio retrospectivo de 154 pacientes consecutivos con MM. La función renal se evaluó mediante la tasa estimada de filtración glomerular al inicio y final de la terapia de inducción. Además, evaluamos las respuestas renales (RR) de acuerdo con los criterios del International Myeloma Working Group (IMWG). El régimen de inducción se basó en talidomida en todos los casos. Resultados Cincuenta y tres pacientes presentaron FR (34,4%) al diagnóstico. La RR completa se logró en 51%. La sobrevida global (SG) a 3 años en pacientes sin FR, con FR y RR completa, y pacientes con FR y cualquier otra RR, fue de 66, 47 y 13%, respectivamente. La SG media fue de 53, 27 y 6 meses (p < 0,01), respectivamente. En el análisis multivariado, la FR y la hipercalcemia fueron factores independientes de menor sobrevida. Conclusiones Lograr una RR completa en pacientes con MM recién diagnosticado se asocia con una mejor sobrevida.

AL amyloidosis in the Chilean public health system: a pending debt: multicenter study of the Chilean Monoclonal Gammopathies Cooperative Group / Amiloidosis AL en el sistema público de Chile: una deuda pendiente: estudio multicéntrico

ABSTRACT Background: Immunoglobulin light chain (AL) amyloidosis is a rare and underdiagnosed entity. Aim: To characterize patients with AL amyloidosis in Chilean public health centers. Material and Methods: We conducted a retrospective, multicenter study. Public centers of the Chilean Monoclonal Gammopathies Cooperative Group were asked to search for patients with AL amyloidosis in their databases. Epidemiological, clinical and laboratory characteristics were evaluated. Results: Forty-two patients aged 22 to 84 years were found. Twenty four percent had localized AL amyloidosis; 64% had a lambda light chain clone; 47% were associated with multiple myeloma and 9% with non-Hodgkin lymphoma. The most commonly involved organ was the kidney (76%). Serum free light chains were measured in 31% and an echocardiogram was performed in 74% of patients. Seventeen percent of patients received only palliative care, 17% were treated with bortezomib, 21% with thalidomide, and 40% with melphalan. No patient was transplanted. The mean overall survival (OS) of the group was 19 months. The 5-year OS was 28%. Conclusions: It is important to obtain these realistic, national data to initiate strategies to improve early diagnosis and proper management of this disease.

La amiloidosis AL es una entidad poco frecuente y subdiagnosticada. Mientras todo el mundo discute sobre las nuevas herramientas diagnósticas y terapéuticas, en Chile y en América Latina en general, estamos lejos de esa realidad. El objetivo del presente estudio fue caracterizar a los pacientes con amiloidosis AL en centros del sistema público de nuestro país. Se realizó un estudio retrospectivo, multicéntrico, descriptivo. Los centros públicos del grupo cooperativo hematológico chileno buscaron en sus bases de datos pacientes diagnosticados con amiloidosis AL. Se evaluaron las características epidemiológicas, clínicas y de laboratorio. La edad media fue de 65 años. A 24% de los pacientes se les diagnosticó amiloidosis AL localizada; 64% tuvo paraproteína con cadena ligera lambda; 47% se asoció con mieloma múltiple y 9% con linfoma no Hodgkin. El órgano afectado con mayor frecuencia fue el riñón (76%). Las cadenas ligeras libres de suero se realizaron en 31% y ecocardiograma en 74%. El 17% recibió solo cuidados paliativos, 17% recibió tratamiento con bortezomib, 21% con talidomida y 40% con melfalán. Ningún paciente fue trasplantado. La media de sobrevida global (SG) del grupo fue de 19 meses. La SG a 5 años fue de 28%. Es importante reportar estos resultados nacionales para iniciar estrategias que mejoren tanto el diagnóstico temprano como el tratamiento de esta patología. Por lo tanto, mejorar la sospecha diagnóstica es crucial.

Prognostic impact of renal failure recovery in patients with newly diagnosed multiple mieloma.

Background Renal failure (RF) is a common complication in patients with newly diagnosed multiple myeloma (NDMM). Aim To evaluate the frequency of RF in NDMM patients, and the prognostic impact of its reversibility. Material and Methods A retrospective study evaluating demographic and clinical characteristics of 154 consecutive patients with NDMM was carried out. Estimated glomerular filtration rate (eGFR) was calculated at the beginning and at the end of the induction therapy. In addition, we evaluated renal responses (RR) according to the International Myeloma Working Group (IMWG) criteria. The induction regimen was based on thalidomide in all cases. Results Fifty-three patients had RF (34.4%). Complete renal response (RR) was achieved in 51%. Three years overall survival in patients without RF, with RF and complete RR, and patients with RF and any other RR, was 66, 47 and 13%, respectively. Median survival was 53, 27 and 6 months, respectively (p < 0.01). In the multivariate analysis, RF and hypercalcemia were independent predictors of a worse outcome. Conclusions Achieving a complete RR in patients with NDMM, is associated with a better survival.

AL amyloidosis in the Chilean public health system: a pending debt. Multicenter study of the Chilean Monoclonal Gammopathies Cooperative Group.

BACKGROUND: Immunoglobulin light chain (AL) amyloidosis is a rare and underdiagnosed entity. AIM: To characterize patients with AL amyloidosis in Chilean public health centers. MATERIAL AND METHODS: We conducted a retrospective, multicenter study. Public centers of the Chilean Monoclonal Gammopathies Cooperative Group were asked to search for patients with AL amyloidosis in their databases. Epidemiological, clinical and laboratory characteristics were evaluated. RESULTS: Forty-two patients aged 22 to 84 years were found. Twenty four percent had localized AL amyloidosis; 64% had a lambda light chain clone; 47% were associated with multiple myeloma and 9% with non-Hodgkin lymphoma. The most commonly involved organ was the kidney (76%). Serum free light chains were measured in 31% and an echocardiogram was performed in 74% of patients. Seventeen percent of patients received only palliative care, 17% were treated with bortezomib, 21% with thalidomide, and 40% with melphalan. No patient was transplanted. The mean overall survival (OS) of the group was 19 months. The 5-year OS was 28%. CONCLUSIONS: It is important to obtain these realistic, national data to initiate strategies to improve early diagnosis and proper management of this disease.

[IgG4 associated nephritis and recurrent hemoptysis: Case report]. / Hemoptisis recurrente asociada a falla renal y hematuria: otra de las mil caras de la enfermedad relacionada a IgG4.

IgG4 disease is a multi-systemic condition involving pancreas, salivary glands and lymph nodes. Less frequently, it causes interstitial nephritis and involves the lungs. We report a 58 years old male with a four years history of hemoptysis and renal dysfunction characterized by hematuria and proteinuria, responsive to steroidal therapy. The renal biopsy established the diagnosis of IgG4 associated interstitial nephritis. Lung involvement was considered secondary to the same systemic disease.

Hemoptisis recurrente asociada a falla renal y hematuria: otra de las mil caras de la enfermedad relacionada a IgG4 / IgG4 associated nephritis and recurrent hemoptysis: case report

IgG4 disease is a multi-systemic condition involving pancreas, salivary glands and lymph nodes. Less frequently, it causes interstitial nephritis and involves the lungs. We report a 58 years old male with a four years history of hemoptysis and renal dysfunction characterized by hematuria and proteinuria, responsive to steroidal therapy. The renal biopsy established the diagnosis of IgG4 associated interstitial nephritis. Lung involvement was considered secondary to the same systemic disease.

Infección oculta por el virus de la hepatitis B en pacientes sometidos a trasplante hepático / Occult Hepatitis B Virus Infection in Liver Transplant Patients

Introducción: la infección oculta por el virus de la hepatitis B (VHB) se caracteriza por la presencia del genoma viral en suero y/o tejido hepático de individuos negativos para el antígeno de superficie HBsAg. La infección oculta se ha asociado con el desarrollo de cirrosis y carcinoma hepatocelular. Objetivo: identificar casos de infección oculta por el VHB en pacientes con diagnóstico de cirrosis y carcinoma hepatocelular, sometidos a trasplante hepático. Materiales y métodos: entre febrero de 2013 y marzo de 2014 fueron obtenidas muestras de explante hepático provenientes de pacientes con diagnóstico de cirrosis y/o carcinoma hepatocelular. Se detectó el genoma del VHB mediante amplificación de tres regiones del genoma viral (S, Core y X). Las muestras positivas se confirmaron por reacción en cadena de la polimerasa (PCR) en tiempo real para la región S. Resultados: se analizaron 15 muestras de tejido hepático, y en dos (13,3%) se detectó el genoma del VHB mediante PCR anidada para la región S y por PCR semianidada para la región X, resultado confirmado por PCR en tiempo real. Estas muestras provenían de pacientes negativos para los marcadores serológicos de infección por el VHB, anti-HBc y anti-HBs. Conclusión: la frecuencia de infección oculta reportada en este estudio es similar a lo reportado en Brasil en muestras de biopsias obtenidas de pacientes con hepatitis crónica. Estudios adicionales son necesarios para estimar la frecuencia de infección oculta por VHB (OBI) en pacientes con hepatopatías terminales en Colombia

Introduction: Occult hepatitis B virus infection is characterized by the presence of the viral genome in serum and/or liver tissue from individuals who test negative for the HBsAg surface antigen. Occult infection has been associated with the development of cirrhosis and hepatocellular carcinoma. Objective: The objective of this study was to identify cases of occult hepatitis B virus infection in patients with cirrhosis and/or hepatocellular carcinoma undergoing liver transplantation. Materials and methods: Between February 2013 and March 2014 hepatic explant samples were obtained from patients with cirrhosis and/or hepatocellular carcinoma. The hepatitis B virus genome was detected by amplification of three regions of the viral genome (S, Core and X). Positive samples were confirmed by real-time PCR for the S region. Results: Fifteen hepatic tissue samples were analyzed. The genome of the hepatitis B virus was detected in two (13.3%) samples by nested PCR for the S region and by semi-nested PCR for region X. The results were confirmed by real-time PCR. These samples came from patients who had tested negative for anti-HBc and anti-HBs serological markers for hepatitis B virus infection. Conclusion: The frequency of occult infection reported in this study is similar to that reported in Brazil in biopsy specimens obtained from patients with chronic hepatitis. Additional studies are needed to estimate the frequency of occult hepatitis B in patients with end-stage liver disease in Colombia

Hepatitis E Virus Genotype 3 in Colombia: Survey in Patients with Clinical Diagnosis of Viral Hepatitis.

BACKGROUND: Hepatitis E virus is a major cause of outbreaks as well as sporadic hepatitis cases worldwide. The epidemiology of this enterically transmitted infection differs between developing and developed countries. The aims of this study were to describe HEV infection in Colombian patients and to characterize the genotype. METHODS: A prospective study was carried out on 40 patients aged over 15 with a clinical diagnosis of viral hepatitis, recruited from five primary health units in the city of Medellin, Colombia. Fecal samples obtained from the 40 consecutives cases were analyzed for HEV RNA using nested reverse transcription PCR for both ORF1 and ORF2-3. The amplicons were sequenced for phylogenetic analyses. RESULTS: Nine (22.5%) cases of HEV infection were identified in the study population. Three HEV strains obtained from patients were classified as genotype 3. No significant association was found between cases of Hepatitis E and the variables water drinking source, garbage collection system and contact with pigs. CONCLUSIONS: This is the first prospective study of hepatitis E in Colombian patients. The circulation of the genotype 3 in this population is predictable considering the reports of the region and the identification of this genotype from pigs in the state of Antioquia, of which Medellin is the capital. Further studies are necessary to establish whether zoonotic transmission of HEV is important in Colombia.

Hepatitis D virus and hepatitis B virus infection in Amerindian communities of the Amazonas state, Colombia.

BACKGROUND: In Colombia, cases of Hepatitis D virus (HDV) infection have been officially described since 1985 mainly in Amerindian population from Sierra Nevada de Santa Marta (North Caribbean Coast), Uraba (North West), and Amazon (South East). The last official report of a clinical case of HDV infection in Colombia was registered in 2005. OBJECTIVES: The aims of this study were to identify cases of HDV and/or Hepatitis B virus (HBV) infection in asymptomatic Amerindians from Amazonas state, South East Colombia, and to describe the circulating viral genotypes in this population. STUDY DESIGN: The study population was recruited in 19 Amerindian communities in the Amazonas state. Individuals over 18 years old were screened by rapid test for Hepatitis B surface Antigen (HBsAg). Blood samples obtained from individuals positives for HBsAg in the rapid-test assay were analyzed for HBsAg, anti-HBc, anti-HDV IgM/IgG by ELISA. The detection of HBV DNA and HDV RNA was performed by PCR amplification. The viral genotype was determined by sequencing and phylogenetic analysis. RESULTS: A total of 23/861 individuals were positive for HBsAg detection by rapid test. Serological and/or molecular markers of HDV infection were demonstrated in 43.5 % (10/23) of samples from Amerindians. The phylogenetic analysis demonstrated the exclusive circulation of HBV subgenotype F1b of and HDV 3 in this population. CONCLUSIONS: A high frequency of HBV/HDV infection was found in Amerindian population from Amazonas State, Colombia (43.5 %, 10/23). Nine cases were identified in a population of 861 asymptomatic Amerindian individuals; one symptomatic case (with diagnosis of end-stage hepatic disease) was also identified in the study. The circulation of HDV 3 and HBV subgenotype F1b suggests a constant flow of these viral genotypes as a result of the interaction of the Amerindian populations from Amazon basin. Further studies are necessary to confirm whether HBV subgenotype F1b is the prevalent in the population from South East region in Colombia.