Predictors of acute ischemic cerebral lesions in immune-mediated thrombotic thrombocytopenic purpura and hemolytic uremic syndrome.

BACKGROUND: The immune form of thrombotic thrombocytopenic purpura (iTTP) and the hemolytic and uremic syndrome (HUS) are two major forms of thrombotic microangiopathy (TMA). Their treatment has been recently greatly improved. In this new era, both the prevalence and predictors of cerebral lesions occurring during the acute phase of these severe conditions remain poorly known. AIM: The prevalence and predictors of cerebral lesions appearing during the acute phase of iTTP and Shiga toxin-producing Escherichia coli-HUS or atypical HUS were evaluated in a prospective multicenter study. METHODS: Univariate analysis was performed to report the main differences between patients with iTTP and those with HUS or between patients with acute cerebral lesions and the others. Multivariable logistic regression analysis was used to identify the potential predictors of these lesions. RESULTS: Among 73 TMA cases (mean age 46.9 ± 16 years (range 21-87 years) with iTTP (n = 57) or HUS (n = 16), one-third presented with acute ischemic cerebral lesions on magnetic resonance imagery (MRI); two individuals also had hemorrhagic lesions. One in ten patients had acute ischemic lesions without any neurological symptom. The neurological manifestations did not differ between iTTP and HUS. In multivariable analysis, three factors predicted the occurrence of acute ischemic lesions on cerebral MRI: (1) the presence of old infarcts on cerebral MRI, (2) the level of blood pulse pressure, (3) the diagnosis of iTTP. CONCLUSION: At the acute phase of iTTP or HUS, both symptomatic and covert ischemic lesions are detected in one third of cases on MRI. Diagnosis of iTTP and the presence of old infarcts on MRI are associated with the occurrence of such acute lesions as well as increased blood pulse pressure, that may represent a potential target to further improve the therapeutic management of these conditions.

Guadecitabine vs treatment choice in newly diagnosed acute myeloid leukemia: a global phase 3 randomized study.

This phase 3 study evaluated the efficacy and safety of the new hypomethylating agent guadecitabine (n = 408) vs a preselected treatment choice (TC; n = 407) of azacitidine, decitabine, or low-dose cytarabine in patients with acute myeloid leukemia unfit to receive intensive induction chemotherapy. Half of the patients (50%) had poor Eastern Cooperative Oncology Group Performance Status (2-3). The coprimary end points were complete remission (19% and 17% of patients for guadecitabine and TC, respectively [stratified P = .48]) and overall survival (median survival 7.1 and 8.5 months for guadecitabine and TC, respectively [hazard ratio, 0.97; 95% confidence interval, 0.83-1.14; stratified log-rank P = .73]). One- and 2-year survival estimates were 37% and 18% for guadecitabine and 36% and 14% for TC, respectively. A large proportion of patients (42%) received <4 cycles of treatment in both the arms. In a post hoc analysis of patients who received ≥4 treatment cycles, guadecitabine was associated with longer median survival vs TC (15.6 vs 13.0 months [hazard ratio, 0.78; 95% confidence interval, 0.64-0.96; log-rank P = .02]). There was no significant difference in the proportion of patients with grade ≥3 adverse events (AEs) between guadecitabine (92%) and TC (88%); however, grade ≥3 AEs of febrile neutropenia, neutropenia, and pneumonia were higher with guadecitabine. In conclusion, no significant difference was observed in the efficacy of guadecitabine and TC in the overall population. This trial was registered at www.clinicaltrials.gov as #NCT02348489.

Ameloblastoma metastásico / Metastatic ameloblastoma

77REPERT MED CIR. 2023;32(1):77-80Mario Alexander Melo MD*María Camila PiñerosbAna María TorresbFabián Parra F. MDcRafael Baracaldo A. MDda Patología. Grupo de Patología Oncológica, Instituto Nacional de Cancerología, Universidad El Bosque. Bogotá DC, Colombia. b Odontólogía, Patología Oral y Medios Diagnósticos, Universidad El Bosque, Bogotá DC, Colombia. c Radiología e Imágenes Diagnósticas, Fundación Universitaria Sanitas, Bogotá DC, Colombia. d Patología, Fundación Universitaria de Ciencias de la Salud. Bogotá DC, Colombia.Introducción: el ameloblastoma es un tumor epitelial benigno de origen odontogénico, de crecimiento progresivo, caracterizado por expansión ósea y tendencia a la recurrencia local si no se trata en forma adecuada. De acuerdo con las características clínicas y radiográficas se clasifica en ameloblastoma uniquístico, periférico/extraóseo y el metastásico. Presentación del caso: el presente manuscrito se quiere reseñar el caso clínico de un ameloblastoma metastásico, el cual es capaz de producir siembras a pesar de su apariencia histológica benigna. Discusión y conclusiones: las variadas formas clínicas y radiográficas hacen que el ameloblastoma metastásico requiera, desde su diagnóstico inicial, un estudio detallado ya que es importante entender el concepto de esta patología como lesión clínica que realiza metástasis a distancia, pero su comportamiento histológico es benigno

Introduction: ameloblastoma is a benign epithelial odontogenic tumor that progresses slowly and is characterized by bone expansion and tendency for local recurrence if not treated properly. According to the clinical and radiographic characteristics, it is classified as unicystic, peripheral/extraosseous and metastatic ameloblastoma. Case presentation: the present manuscript aims to review the clinical case of a metastatic ameloblastoma, which can metastasize in spite of a benign histological appearance. Discussion and conclusions: the various clinical and radiographic forms of metastatic ameloblastoma require, from its initial diagnosis, a detailed study, since it is important to understand the concept of this clinico-pathological entity as a lesion with distant metastases but benign histological features

Colecistectomía laparoscópica: técnica por video e-learning

Introducción: La Colecistectomía laparoscópica es una de las cirugías más frecuentes en nuestro país. Diversas dificultades han limitado una buena formación al respecto. Contar con un apoyo educativo capaz de transmitir la experiencia quirúrgica que facilite su aprendizaje, resulta imprescindible. Objetivo: Describir la técnica quirúrgica de una colecistectomía laparoscópica estándar incorporando elementos propios de la decisión quirúrgica, apoyado en tecnología e-learning. Materiales y Método: Estudio descriptivo. Se confeccionó material audiovisual de una colecistectomía laparoscópica, editado con apoyo de dibujo y animación 3D, e incorporando elementos técnicos propios del acto quirúrgico. Finalmente se redactó el texto de la técnica y se enlazó a través de códigos QR a capsulas del material audiovisual confeccionado. Resultados: Cinco pasos descriptivos claves de la colecistectomía laparoscópica apoyados con enlaces directos, tanto a través de un link asociado al texto como a través de una imagen QR anexa, a capsulas audiovisuales con información técnica y estratégica propia del ejercicio quirúrgico intraoperatorio. Conclusión: Técnica quirúrgica de una colecistectomía laparoscópica expuesta paso a paso apoyado con material audiovisual de una forma dinámica e innovadora, basado en nuevas tecnologías facilitadoras del aprendizaje.

Background: The laparoscopic cholecystectomy's technique has a great relevance in training programs. Their teaching requires a methodology that incorporates technical details that are lost with just reading or watching the procedure. Aim: This study presents the description of the surgical steps associated to an anatomical e-learning support that includes strategic and technical elements. Materials and Method: A standard laparoscopic cholecystectomy was used to make an educational video highlighting the critical aspect and concepts of its execution. The video incorporated drawings, painting and animations that fa- cilitate understanding. It was split and linked to Qr codes. Results: The detailed description of the steps of a cholecystectomy, such as a proper exposure of the surgical field, the dissection of the hepatocystic triangle, the safety view or gallbladder release, was associated with a Qr link. Conclusión: A step-by-step laparoscopic cholecystectomy in a dynamic and innovative way with an audiovisual support that facilitate learning.

Transoral robotic surgery for parapharyngeal carcinoma ex-pleomorphic adenoma: A case report.

BACKGROUND: Carcinoma ex-pleomorphic adenoma (Ca ex-PA) comprises 0.5% of head and neck neoplasms. Transoral robotic surgery (TORS) is an approach being used to treat a variety of benign and malignant head and neck neoplasms. Recently, this technique has gained popularity as an alternative for parapharyngeal space (PPS) tumor resection. To our knowledge, this is the first case of Ca ex-PA managed successfully by TORS of the PPS. CASE: Fifty-nine-year-old male with incidental mass in PPS, initial diagnosis of pleomorphic adenoma, who underwent transoral robotic resection. The histopathology diagnosis with minimally invasive Ca ex-PA findings and malignant component of high-grade epithelial/myoepithelial carcinoma and salivary duct carcinoma. Patient discharged on the fifth post-operative day without complications. CONCLUSION: Based on our findings, TORS may be a safe procedure to remove selected Ca ex-PA from the PPS; however, further research is needed.

Immune-mediated thrombotic thrombocytopenic purpura prognosis is affected by blood pressure.

Background: The prevalence, prognostic role, and diagnostic value of blood pressure in immune-mediated thrombotic thrombocytopenic purpura (iTTP) and other thrombotic microangiopathies (TMAs) remain unclear. Methods: Using a national cohort of iTTP (n = 368), Shigatoxin-induced hemolytic uremic syndrome (n = 86), atypical hemolytic uremic syndrome (n = 84), and hypertension-related thrombotic microangiopathy (n = 25), we sought to compare the cohort's blood pressure profile to assess its impact on prognosis and diagnostic performances. Results: Patients with iTTP had lower blood pressure than patients with other TMAs, systolic (130 [interquartile range (IQR) 118-143] vs 161 [IQR 142-180] mmHg) and diastolic (76 [IQR 69-83] vs 92 [IQR 79-105] mmHg, both p < 0.001). The best threshold for iTTP diagnosis corresponded to a systolic blood pressure <150 mmHg. iTTP patients presenting with hypertension had a significantly poorer survival (hazard ratio 1.80, 95% confidence interval 1.07-3.04), and this effect remained significant after multivariable adjustment (hazard ratio = 1.14, 95% confidence interval 1.00-1.30). Addition of a blood pressure criterion modestly improved the French clinical score to predict a severe A disintegrin and metalloprotease with thrombospondin type 1 deficiency in patients with an intermediate score (i.e., either platelet count <30 × 109/L or serum creatinine <200 µM). Conclusions: Elevated blood pressure at admission affects the prognosis of iTTP patients and may help discriminate them from other TMA patients. Particular attention should be paid to blood pressure and its management in these patients.

Androgen receptor expression and prognosis in Hispanic/Latino women with triple negative breast cancer.

INTRODUCTION AND OBJECTIVE: Triple-negative breast cancer (TNBC) is an aggressive subtype that has no available molecular targets. It occurs most often in premenopausal African-American and Hispanic/Latino women. In Colombia, its prevalence has been reported to be 20.6%. Androgen receptor (AR) belongs to the steroid nuclear receptor family and has been recently considered a potential biomarker for breast cancer. Considering the high prevalence of TNBC in Colombian women and the lack of knowledge of AR expression, our aim was to determine the frequency of AR expression and its association with pathological variables. MATERIALS AND METHODS: 149 women diagnosed with TNBC between 2011 and 2014 were included. Clinical and pathological data were obtained from medical and pathology reports. Information on hormone receptor status, Ki67 expression and HER2 was reevaluated by a pathologist. AR expression was considered positive when it exceeded 1% of nuclear staining in tumor cells. RESULTS: AR expression was detected in 41.6% of the samples. Although we did not find statistically significant differences in clinic-pathological variables according to AR expression, patients with AR expression over 50% were younger (53.92 years vs. 60.78 years, p=0.027) and presented higher Ki67 expression (64.06% vs. 47.32%, p=0.05), compared to patients with a low AR expression. The median overall survival in our sample of TNBC patients was 2.45 years. CONCLUSIONS: The expression of AR in our sample was similar to the expression in populations of European descent. We found statistically significant differences in age at diagnosis and Ki67 expression according to AR expression.

Androgen receptor expression and prognosis in Hispanic/Latino women with triple negative breast cancer

Introduction and objective: Triple-negative breast cancer (TNBC) is an aggressive subtype that has no available molecular targets. It occurs most often in premenopausal African-American and Hispanic/Latino women. In Colombia, its prevalence has been reported to be 20.6%. Androgen receptor (AR) belongs to the steroid nuclear receptor family and has been recently considered a potential biomarker for breast cancer. Considering the high prevalence of TNBC in Colombian women and the lack of knowledge of AR expression, our aim was to determine the frequency of AR expression and its association with pathological variables. Materials and methods: 149 women diagnosed with TNBC between 2011 and 2014 were included. Clinical and pathological data were obtained from medical and pathology reports. Information on hormone receptor status, Ki67 expression and HER2 was reevaluated by a pathologist. AR expression was considered positive when it exceeded 1% of nuclear staining in tumor cells. Results: AR expression was detected in 41.6% of the samples. Although we did not find statistically significant differences in clinic-pathological variables according to AR expression, patients with AR expression over 50% were younger (53.92 years vs. 60.78 years, p=0.027) and presented higher Ki67 expression (64.06% vs. 47.32%, p=0.05), compared to patients with a low AR expression. The median overall survival in our sample of TNBC patients was 2.45 years. Conclusions: The expression of AR in our sample was similar to the expression in populations of European descent. We found statistically significant differences in age at diagnosis and Ki67 expression according to AR expression.(AU)

Introducción y objetivo: El carcinoma de mama triple negativo (CMTN) es un subtipo de carcinoma con comportamiento agresivo y sin dianas moleculares disponibles. Se presenta en mujeres premenopáusicas afroamericanas y latinas. En Colombia, la prevalencia informada es del 20.6%. Los receptores de andrógenos (RA) pertenecen a una familia de receptores nucleares y son considerados como potencial biomarcador. Debido a la alta prevalencia de CMTN en mujeres colombianas y la ausencia de datos sobre los RA, el objetivo del trabajo fue determinar la frecuencia de RA y su asociación con otras variables histopatológicas. Materiales y métodos: El estudio incluyó a 149 mujeres diagnosticadas con CMTN entre 2011 y 2014. Las variables clínicas y patológicas fueron extraídas de la historia clínica. Los receptores hormonales, índice Ki67 y Her2 fueron reevaluados por un patólogo. Los RA fueron considerados positivos cuando más del 1% de los núcleos tumorales estaban teñidos. Resultados: La expresión de RA fue detectada en el 41,6% de las muestras. No se encontraron diferencias significativas en variables patológicas de acuerdo a la expresión de RA. Sin embargo, se observó que pacientes con RA positivos mayor del 50%, eran más jóvenes (53,92 vs. 60,78 años; p=0,027) y presentaban Ki67 más elevados (64,06 vs. 47,32%; p=0,05), comparadas con pacientes de baja expresión de RA. La sobrevida global de las pacientes fue de 2,45 años. Conclusiones: La expresión de RA en la muestra fue similar a la de poblaciones de descendencia europea. Se encontraron diferencias significativas en la edad al momento del diagnóstico y en el índice Ki67, estratificadas por expresión de RA.(AU)

Hidradenoma papilífero de la vulva con componente de carcinoma in situ: reporte de caso y revisión de la bibliografía / Papilliferous hidradenoma of the vulva with carcinoma in situ component: case report and literature review

Resumen ANTECEDENTES: El cáncer de vulva es relativamente raro, representa menos del 1% de los tumores malignos de la mujer; su incidencia aumenta con la edad. La variedad más frecuente es el carcinoma escamoso (80 al 90%), seguido del melanoma. En este reporte se revisa un carcinoma de origen glandular, como el hidradenoma papilífero del tipo glándula mamaria (mammary-like) de la vulva. CASO CLÍNICO: Paciente de 50 años, con una lesión papular en la vulva de dos años de evolución, con crecimiento lento y progresivo, ocasional sensación de masa y dolor, con colposcopia negativa, sin antecedentes de patología mamaria y con una biopsia previa que reportó hidradenoma papilífero vulvar. Se trató con resección completa de la lesión, con anestesia regional, con bordes libres, no se identificó algún componente infiltrante. En la actualidad permanece sin evidencia de recaída ni requerimiento de tratamientos adicionales durante el seguimiento. CONCLUSIÓN: El hidradenoma papilífero es una lesión benigna, poco frecuente, relacionada con las glándulas anogenitales de tipo mammary-like, con buen pronóstico. El tratamiento recomendado es la escisión quirúrgica, que casi siempre es curativa.

Abstract BACKGROUND: Vulvar cancer is relatively rare, representing less than 1% of malignant tumors in women; its incidence increases with age. The most frequent variety is squamous cell carcinoma (80 to 90%), followed by melanoma. In this report we review a carcinoma of glandular origin, such as papilliferous hydradenoma of the vulva of the mammary gland (mammary-like) type. CLINICAL CASE: We present a 50-year-old patient with 2 years evolution of a papular lesion on the vulva with slow and progressive growth, intermitent sensation of mass and pain, with negative colposcopy, no history of breast pathology and with a previous biopsy that reported vulvar papilliferous hydradenoma. She was treated with complete resection of the lesion under regional anesthesia, with free margins, without identifying an infiltrating component and currently without evidence of relapse or requirement of additional treatments. CONCLUSION: Papilliferous hidradenoma is a rare benign lesion related to the mammary-like anogenital glands, with a good prognosis and its recommended treatment is surgical excision, which is generally curative.

Molecular classification and prognosis in younger adults with acute myeloid leukemia and intermediate-risk cytogenetics treated or not by gemtuzumab ozogamycin: Final results of the GOELAMS/FILO acute myeloid leukemia 2006-intermediate-risk trial.

In this randomized phase 3 study, the FILO group tested whether the addition of 6 mg/m2 of gemtuzumab ozogamycin (GO) to standard chemotherapy could improve outcome of younger patients with de novo acute myeloid leukemia (AML) and intermediate-risk cytogenetics. GO arm was prematurely closed after 254 inclusions because of toxicity. A similar complete remission rate was observed in both arms. Neither event-free survival nor overall survival were improved by GO in younger AML patients (<60 years) ineligible for allogeneic stem-cell transplantation. (P = .086; P = .149, respectively). Using unsupervised hierarchical clustering based on mutational analysis of seven genes (NPM1, FLT3-ITD, CEBPA, DNMT3A, IDH1, IDH2, and ASXL1), six clusters of patients with significant different outcome were identified. Five clusters were based on FLT3-ITD, NPM1, and CEBPA mutations as well as epigenetic modifiers (DNMT3A, IDH1/2, ASXL1), whereas the last cluster, representing 25% of patients, had no mutation and intermediate risk. One cluster isolated FLT3-ITD mutations with higher allelic ratio and a very poor outcome. The addition of GO had no impact in these molecular clusters. Although not conclusive for GO impact in AML patients <60 years, this study provides a molecular classification that distinguishes six AML clusters influencing prognosis in younger AML patients with intermediate-risk cytogenetic.

Conventional chemotherapy for acute myeloid leukemia in older adults: Impact on nutritional, cognitive, and functional status.

OBJECTIVES: The impact of conventional treatment for acute myeloid leukemia (AML) on the nutritional, cognitive, and functional status of elderly patients is seldom studied. This assessment was performed in the context of the LAMSA 2007 trial. METHODS: The trial enrolled 424 patients with de novo AML. Among them, 316 benefited from geriatric assessment (GA) including nutritional, cognitive, and functional status and were scored according to Eastern Cooperative Oncology Group (ECOG) and sorror for the prediction of treatment toxicity, morbidity, and mortality. Patients were investigated at diagnosis for three times during follow-up. RESULTS: This study showed that AML and its treatment have no impact on cognitive (P = .554) nor functional status (P = .842 for Activity of Daily Living and P = .087 for Instrumental Activities of Daily Living). The nutritional status improved over time (P = .041). None of these three parameters at baseline, associated or not with ECOG and sorror scores, impacted survivals or toxicities. CONCLUSIONS: The cognitive, functional, and nutritional status had no impact in this cohort of fit elderly AML patients without unfavorable cytogenetics. The GA tools used provided no additional information compared with ECOG and sorror scores, to predict toxicity, morbidity, or mortality due to intensive chemotherapy.

Alveolar Soft Part Sarcoma of the Tongue in an Adolescent.

Alveolar soft part sarcoma is a rare malignant soft tissue neoplasm of uncertain histogenesis and aggressive clinical behavior. Alveolar soft part sarcoma arises in the head and neck in 27% of cases, with 25% of head and neck cases occurring in the tongue. Herein the case of a pediatric patient diagnosed with alveolar soft tissue sarcoma of the tongue, who received surgical treatment with total resection of the lesion and chemotherapy without radiotherapy, is presented. To date, the patient is in remission of the disease.

Situación actual del trasplante hepático pediátrico por hepatopatías crónicas en Chile: Inequidades asociadas a la priorización por sistema MELD/PELD

Background: The Chilean allocation system for liver transplantation (LT) uses the MELD/PELD score to prioritize candidates on the waiting list. Aim: To assess if the Chilean allocation system for LT is equitable for pediatric candidates compared to their adult counterparts. Material and Methods: We used the Public Health Institute's registry between October 2011 and December 2017. We analyzed candidates with chronic hepatic diseases listed for LT. The primary outcome was the cadaveric liver transplantation (CLT) rate. Secondary outcomes were death or disease progression in the waiting list and living donor liver transplant (LDLT) rate. Results: We analyzed 122 pediatric and 735 adult candidates. Forty one percent of pediatric candidates obtained a CLT compared to 48% of adults (p = NS). Among patients aged under two years of age, the access to CLT on the waiting list there was 28% of CLT, compared to 48% in adults (p = 0.001). Fifty-seven percent of candidates aged under two years were listed for cholestatic diseases, obtaining a CLT in 18% and requiring a LDLT in 49%. The median time in the waiting list for CLT was 5.9 months in pediatric candidates and 5.1 in adults, while the median time to death in the waiting list was 2.8 and 5.6 months, respectively. The mortality rate at one year in candidates under two years old was 38.1% compared to 32.5% in adults. Conclusions: Pediatric candidates with chronic liver diseases, especially under two years of age, have greater access difficulties to CLT than adults. Half of the pediatric candidates die on the waiting list before three months. The mortality among candidates under two years of age in the waiting list is excessively high.

[A proposal of the Chilean Academy of Medicine to improve organ procurement and transplantation in Chile]. / Donación y trasplante de órganos: propuesta desde la Academia Chilena de Medicina.

The Chilean Academy of Medicine designated a group of specialists to evaluate the practice and to propose reforms for organ donation and transplantation, due to the general insufficiencies at the national level with these procedures. In the last six years the mean number of organ transplants in Chile was 340 cases per year while effective cadaveric donors ranged between 6 and 10 per million inhabitants. These averages remained stable during this period and are among the lowest in the region. Our analysis attributed these deficient results mainly to low organ donation and inefficient procurement due to lack of compliance with protocols and little accountability. The committee proposes several measures for improvement. These are a systematic and obligatory report of potential organ donors by all emergency and critical care centers, frequent evaluation of results, empowering of health authorities to correct insufficiencies in organ procurement, education programs for primary, secondary, technical and university students to improve their knowledge about the social significance and solidarity required for transplantation policies and specialized updated training of all health professionals involved. Organ donation and transplantation must be based on clear and fair ethical considerations in order to be accepted by the general public.

Analysis of a cohort of 279 patients with hairy-cell leukemia (HCL): 10 years of follow-up.

In total, 279 patients with hairy-cell leukemia (HCL) were analyzed, with a median follow-up of 10 years. Data were collected up to June 2018. We analyzed responses to treatment, relapses, survival, and the occurrence of second malignancies during follow-up. The median age was 59 years. In total, 208 patients (75%) were treated with purine analogs (PNAs), either cladribine (159) or pentosatin (49), as the first-line therapy. After a median follow-up of 127 months, the median overall survival was 27 years, and the median relapse-free survival (RFS) was 11 years. The cumulative 10-year relapse incidence was 39%. In patients receiving second-line therapy, the median RFS was 7 years. For the second-line therapy, using the same or another PNA was equivalent. We identified 68 second malignancies in 59 patients: 49 solid cancers and 19 hematological malignancies. The 10-year cumulative incidences of cancers, solid tumors, and hematological malignancies were 15%, 11%, and 5.0%, respectively, and the standardized incidence ratios were 2.22, 1.81, and 6.67, respectively. In multivariate analysis, PNA was not a risk factor for second malignancies. HCL patients have a good long-term prognosis. PNAs are the first-line treatment. HCL patients require long-term follow-up because of their relatively increased risk of second malignancies.

Donación y trasplante de órganos: propuesta desde la Academia Chilena de Medicina / A proposal of the Chilean Academy of Medicine to improve organ procurement and transplantation in Chile

The Chilean Academy of Medicine designated a group of specialists to evaluate the practice and to propose reforms for organ donation and transplantation, due to the general insufficiencies at the national level with these procedures. In the last six years the mean number of organ transplants in Chile was 340 cases per year while effective cadaveric donors ranged between 6 and 10 per million inhabitants. These averages remained stable during this period and are among the lowest in the region. Our analysis attributed these deficient results mainly to low organ donation and inefficient procurement due to lack of compliance with protocols and little accountability. The committee proposes several measures for improvement. These are a systematic and obligatory report of potential organ donors by all emergency and critical care centers, frequent evaluation of results, empowering of health authorities to correct insufficiencies in organ procurement, education programs for primary, secondary, technical and university students to improve their knowledge about the social significance and solidarity required for transplantation policies and specialized updated training of all health professionals involved. Organ donation and transplantation must be based on clear and fair ethical considerations in order to be accepted by the general public.

Good Profile of Efficacy/Tolerance of Bortezomib or Idelalisib in Waldenström Macroglobulinemia Associated with Acquired Von Willebrand Syndrome.

Acquired von Willebrand syndrome (AVWS) in the setting of Waldenström macroglobulinemia (WM) is a challenging condition. No real standard of care is recommended for these patients, although the therapeutic strategy should include a rapid approach to the emergency bleeding events and to the underlying malignant lymphoid disorder. We report here our experience treating three elderly patients with these concomitant hematologic entities. The use of a bortezomib-based chemotherapy regimen showed a good profile of tolerance and efficacy even in a long-term follow-up period. These patients were treated for several years before switching their therapy to idelalisib, a targeted oral therapy that inhibits phosphatidylinositol 3-kinase isoform-delta (PI3KD), which is part of the signaling pathway downstream B-cell receptor. This approach was well tolerated and efficacious, although some adverse effects were observed, particularly at hepatic levels, but were all reversible. The same profile of tolerance/efficacy was observed in one very old patient who received idelalisib as a first-line therapy. We think that bortezomib-based therapy could be considered in refractory patients with AVWS associated with WM.

[Current situation of pediatric liver transplantation in Chile. Inequities associated with the MELD/PELD prioritization system]. / Situación actual del trasplante hepático pediátrico por hepatopatías crónicas en Chile: Inequidades asociadas a la priorización por sistema MELD/PELD.

BACKGROUND: The Chilean allocation system for liver transplantation (LT) uses the MELD/PELD score to prioritize candidates on the waiting list. AIM: To assess if the Chilean allocation system for LT is equitable for pediatric candidates compared to their adult counterparts. MATERIAL AND METHODS: We used the Public Health Institute's registry between October 2011 and December 2017. We analyzed candidates with chronic hepatic diseases listed for LT. The primary outcome was the cadaveric liver transplantation (CLT) rate. Secondary outcomes were death or disease progression in the waiting list and living donor liver transplant (LDLT) rate. RESULTS: We analyzed 122 pediatric and 735 adult candidates. Forty one percent of pediatric candidates obtained a CLT compared to 48% of adults (p = NS). Among patients aged under two years of age, the access to CLT on the waiting list there was 28% of CLT, compared to 48% in adults (p = 0.001). Fifty-seven percent of candidates aged under two years were listed for cholestatic diseases, obtaining a CLT in 18% and requiring a LDLT in 49%. The median time in the waiting list for CLT was 5.9 months in pediatric candidates and 5.1 in adults, while the median time to death in the waiting list was 2.8 and 5.6 months, respectively. The mortality rate at one year in candidates under two years old was 38.1% compared to 32.5% in adults. CONCLUSIONS: Pediatric candidates with chronic liver diseases, especially under two years of age, have greater access difficulties to CLT than adults. Half of the pediatric candidates die on the waiting list before three months. The mortality among candidates under two years of age in the waiting list is excessively high.