The impact of race/ethnicity on pancreaticoduodenectomy outcomes for pancreatic cancer.

PURPOSE: To investigate the impact of race/ethnicity on surgical outcomes following pancreaticoduodenectomy for pancreatic cancer. METHODS: A retrospective review of patients undergoing pancreaticoduodenectomy for adenocarcinoma in the National Surgical Quality Improvement Program (NSQIP) database from 2014 to 2019. Patient and tumor characteristics and 30-day postoperative outcomes were compared. Multivariable logistic and linear regression models were conducted to investigate the relationship between race/ethnicity and surgical outcomes. RESULTS: Six thousand five hundred and sixty-two patients were included (84.5% White, 7.9% Black, 3% Hispanic, 4.6% Asian). Larger proportions of Blacks had preoperative American Society of Anesthesiologists class 3 or 4. There were no significant differences in tumor characteristics or operative techniques. A smaller proportion of Asians and Hispanics received neoadjuvant chemotherapy and/or radiation than Blacks and Whites. Relative to White, the Black race was independently associated with postoperative sepsis and reoperation. Both Black and Hispanic race/ethnicity were associated with prolonged intubation and delayed gastric emptying, and minorities races/ethnicities were associated with longer length of hospital stay. Relative to White, Hispanic, and Asian race/ethnicity were independently associated with a lower likelihood of neoadjuvant therapy (NAT) receipt. CONCLUSION: In ACS-NSQIP participating hospitals, non-White race/ethnicity was independently associated with adverse outcomes after pancreatic cancer resection. A possible disparity in NAT receipt may exist in Asian and Hispanic patients undergoing surgical resection.

Socioeconomic Disparities in Neoadjuvant Chemotherapy for Early-Stage Breast Cancer.

BACKGROUND: Neoadjuvant chemotherapy (NCT) is often used for patients with early-stage breast cancer. Disparities in the use of NCT based on clinical, demographic, and socioeconomic factors have not been evaluated. METHODS: Data from the National Cancer Database was analyzed for patients with T1-2, N0-1 breast cancer from 2006 to 2015. Univariate and multivariate analysis determined which factors predicted for the receipt of NCT. RESULTS: We found 159 946 eligible patients. Factors associated with receipt of NCT included T2 vs. T1 disease, N1 vs. N0, and treatment at an academic facility. Race itself was not significant; however, a higher level of education amongst Black populations correlated with the receipt of NCT. DISCUSSION: Clinical factors are the greatest determinants for receipt of NCT in early-stage breast cancer. Disparities exist that cannot be explained by race alone; socioeconomic and demographic factors are important. Cancer care should be evaluated in the context of the intersectionality of these health determinants.

When Is It Safe to Proceed With Pancreaticoduodenectomy Without Biliary Decompression?

BACKGROUND: An absolute bilirubin level where preoperative biliary decompression (PBD) is indicated before pancreaticoduodenectomy has been elusive. Our goal was to identify a total bilirubin level whereby biliary decompression provides clear benefit, despite associated expenses and potential complications. MATERIALS AND METHODS: We reviewed a prospectively collected database of patients undergoing pancreaticoduodenectomy at the Vidant Medical Center between 2007 and 2016. Patients were arbitrarily subdivided into 3 groups based on presenting bilirubin level (≤10 mg/dL, 10.1-14.9 mg/dL, and ≥15 mg/dL) to determine the presence of overall complications, severe complications (Clavien-Dindo classification ≥3), prolonged length of stay (>1 SD), readmissions, or mortality. RESULTS: Common bile duct stenting independently predicted a higher incidence of complications in patients presenting with bilirubin ≤10 mg/dL (P = .03) vs. those patients going directly to surgery. No differences were observed for patients with bilirubin between 10.1 mg/dL and 14.9 mg/dL. Biliary decompression in patients with bilirubin ≥15 mg/dL independently predicted fewer overall (73.8% vs. 100%, P = .0082) and less severe complications (14.3% vs. 44.5%, P = .03) and lower readmission rates (15.8% vs. 55.6%, P = .03) vs. those going directly to surgery. Patients not undergoing biliary decompression underwent pancreaticoduodenectomy sooner than those decompressed (4.7 days vs. 17.2 days, P = .01). DISCUSSION: All patients presenting with bilirubin ≥15 mg/dL should undergo PBD, while those with bilirubin ≤10 mg/dL should forego stent placement to avoid stent-related complications. The decision to stent between 10.1 and 14.9 mg/dL should be made on a case-by-case basis keeping in mind timeliness to definitive cancer treatment.

Compromiso vesical en paciente pediátrico con poliarteritis nodosa: primera descripción / Bladder involvement in a paediatric patient with polyarteritis nodosa: First description

RESUMEN La poliarteritis nudosa (PAN) es una vasculitis necrosante, rara en la infancia, caracterizada por el compromiso de vasos pequeños/medianos y de múltiples órganos. Presentamos a una paciente que inició a los 4 arios con síndrome febril prolongado, dolor abdominal crónico, mialgias incapacitantes y compromiso en la piel, quien luego de 2 años de cuadro clínico completa criterios clínicos para PAN. Recibió tratamiento con corticoide sistêmico por vía oral e intravenosa, 6 meses de ciclofosfamida por vía intravenosa y manejo de mantenimiento con inmunosupresores convencionales sin respuesta adecuada, logrando control de la enfermedad únicamente con ciclofosfamida por vía oral y corticoide a largo plazo. Luego de 5 años y de recibir una dosis alta acumulada de ciclofosfamida, inicia con cuadros de hematuria macroscópica. Se evaluaron, entre otras causas, la toxicidad por ciclofosfamida y la actividad de la enfermedad. El estudio incluyó biopsia vesical, con hallazgo de vas-culitis necrosante de paredes vesicales. La vasculitis vesical es raramente reportada en la literatura (3-5 casos en adultos) y en lo consultado no hay reportes en niños. Se describe, en nuestro conocimiento, el primer caso de compromiso vesical asociado a vasculitis sistêmica reportado en la edad pediátrica.

A B S T R A C T Polyarteritis nodosa (PAN) is a necrotising vasculitis, rare in childhood, and characterized by the inflammation of small and medium vessels and multiple organ involvement. The case is presented of a 4 year old girl with prolonged febrile syndrome, chronic abdominal pain, disabling myalgia, and skin involvement. After 2years of symptoms, she met clinical criteria for PAN. She received treatment with oral and intravenous systemic corticosteroids, 6 months of intravenous cyclophosphamide and maintenance with conventional immuno-suppressants without an adequate response. However, she showed clinical improvement with oral cyclophosphamide and long-term corticosteroids. She had several relapses during follow-up visits due to irregular treatment requiring a high cumulative dose of cyclophosphamide. Five years later she presented with macroscopic haematuria, and was assessed for, among other causes, cyclophosphamide toxicity and disease activity. The workup included cystoscopy and bladder biopsy with findingof necrotising vasculitis of bladder wall. Bladder vasculitis is rarely reported in the literature (3-5 cases in adults) and in that consulted there are no reports in children. To our knowledge, this is the first case of bladder involvement associated with systemic vasculitis reported in the paediatric age.

Ophthalmological treatment of early-onset sarcoidosis/Blau syndrome in a Colombian child: A case report.

PURPOSE: To report the ophthalmological approach of a patient with Blau syndrome (BS) in Colombia. OBSERVATIONS: We describe a 9-year-old Colombian boy with sporadic BS due to a de novo nucleotide-binding oligomerization domain containing 2 (NOD2) mutation, who presented with joint and dermatologic symptoms. He was referred to the uveitis service with a single functional eye, due to retinal detachment in the other eye. Despite treatment with corticosteroids, methotrexate, and adalimumab, the patient continued to exhibit progressive disease. CONCLUSION: BS-related uveitis is characterized by severe ocular morbidity. Appropriate interdisciplinary treatment is necessary for the correct identification and management of the disease, considering the inherent difficulty in its diagnosis due to its diverse clinical manifestations. The severity of BS-related uveitis in this report highlights the need for more effective therapies.

Predictive Value of Preoperative Serum CA19-9 on Margin Status.

Serum carbohydrate antigen (CA19-9) is known to correlate with stage, resectability, and prognosis of pancreatic cancer. The goal of pancreaticoduodenectomy is to achieve an R0 resection because worse outcomes are reported in the presence of positive margins. The purpose of this study was to evaluate the predictive utility of CA19-9 for pancreaticoduodenectomy margin status. A retrospective review of patients with pancreatic adenocarcinoma undergoing pancreaticoduodenectomy between October 2007 and November 2018 at our institution was performed. Patient demographics, preoperative CA19-9, and tumor characteristics were analyzed. Univariate and multivariate logistic regression was performed to determine factors associated with positive margins. A total of 184 patients were included. The mean age was 65 years; most patients were male and white. Majority had a positive preoperative CA19-9 (69%). There were nearly twice as many patients with negative as positive margins. Groups had similar demographics and preoperative CA19-9. A greater proportion of patients with negative margins had smaller tumors and early disease. On univariate and multivariate analysis, larger and higher stage tumors had greater odds of positive margins (P < 0.05). There was no significant association between margin status and preoperative CA19-9. Preoperative CA19-9 is not predictive of margin status. These results suggest that although preoperative CA19-9 values are associated with both resectability and prognosis, positive margins may not be a contributing mechanism.

Juvenile polyautoimmunity in a rheumatology setting.

Overt polyautoimmunity (PolyA) corresponds to the presence of more than one well-defined autoimmune disease (AD) manifested clinically in a single patient. The current study aimed to describe the main characteristics of juvenile PolyA in a pediatric rheumatology setting and analyze the chronological aspects, index cases, familial autoimmunity, and clustering pattern. This was a cross-sectional and multicenter study in which 313 children with overt PolyA were included. Patients were systematically interviewed and their medical records reviewed using a questionnaire that sought information about demographic, clinical, immunological, and familial characteristics. A hierarchical cluster analysis was done to determine similarities between autoimmune diseases based on PolyA. PolyA occurred simultaneously in 138 (44%) patients. Multiple autoimmune syndrome was observed in 62 (19.8%) patients. There were 25 index diseases of which, systemic lupus erythematosus (SLE, n = 134, 42.8%), juvenile idiopathic arthritis (JIA, n = 40, 12.7%), Hashimoto's thyroiditis (HT, n = 24, 7.66%), immune thrombocytopenic purpura (ITP n = 20, 6.39%), antiphospholipid syndrome (APS, n = 15, 4.79%), and vitiligo (VIT, n = 15, 4.79%) were the most frequent and represented 79.23% of the total number of patients. Familial autoimmunity influenced PolyA. A high aggregation of autoimmunity was observed (λr = 3.5). Three main clusters were identified, of which SLE and APS were the most similar pair of diseases (based on the Jaccard index) followed by HT and JIA, which were related to ITP and Sjögren's syndrome. The third cluster was composed of localized scleroderma and VIT. Our findings may assist physicians to make an early diagnosis of this frequent condition. Pediatric patients with ADs should be systematically assessed for PolyA.

A simple matrix to predict treatment success and long-term survival among patients undergoing pancreatectomy.

BACKGROUND: A more accurate measure of long-term survival among patients who have undergone a successful resection for pancreatic adenocarcinoma may be computed by accounting for time already survived during the initial treatment window. METHODS: Patients diagnosed with pancreatic adenocarcinoma, from 2004 through 2013, were identified from the American College of Surgeons National Cancer Database (NCDB). A risk-stratification matrix was constructed including age, histopathologic factors and the use of adjuvant therapy, given successful treatment and survival at 3-month following diagnosis. RESULTS: A total of 25,897 patients (50% male, 53% >65 years of age) presented with stage I-III pancreatic cancer. The majority of patients had tumors >2 cm size (82%), grade I/II (65%), lymphatic invasion (LI) (66%), and negative margins (76%). A survival advantage for adjuvant therapy was observed among all patients, independent of their risk-profile. For example, a patient ≤65 years of age, with early stage cancer (size ≤2 cm, grade I/II, -ve LI, -ve margins) who received adjuvant therapy had a 62% probability of being alive beyond three years (95%CI = 59%-66%). In contrast, the survival probability decreased to 53% (95%CI = 59%-66%) without adjuvant therapy. CONCLUSIONS: These results provide surgeons and patients with more accurate information regarding long-term survival, as well as the benefit of opting for adjuvant therapy after successful pancreatic surgery.

Unexpected relevant role of gene mosaicism in patients with primary immunodeficiency diseases.

BACKGROUND: Postzygotic de novo mutations lead to the phenomenon of gene mosaicism. The 3 main types are called somatic, gonadal, and gonosomal mosaicism, which differ in terms of the body distribution of postzygotic mutations. Mosaicism has been reported occasionally in patients with primary immunodeficiency diseases (PIDs) since the early 1990s, but its real involvement has not been systematically addressed. OBJECTIVE: We sought to investigate the incidence of gene mosaicism in patients with PIDs. METHODS: The amplicon-based deep sequencing method was used in the 3 parts of the study that establish (1) the allele frequency of germline variants (n = 100), (2) the incidence of parental gonosomal mosaicism in families with PIDs with de novo mutations (n = 92), and (3) the incidence of mosaicism in families with PIDs with moderate-to-high suspicion of gene mosaicism (n = 36). Additional investigations evaluated body distribution of postzygotic mutations, their stability over time, and their characteristics. RESULTS: The range of allele frequency (44.1% to 55.6%) was established for germline variants. Those with minor allele frequencies of less than 44.1% were assumed to be postzygotic. Mosaicism was detected in 30 (23.4%) of 128 families with PIDs, with a variable minor allele frequency (0.8% to 40.5%). Parental gonosomal mosaicism was detected in 6 (6.5%) of 92 families with de novo mutations, and a high incidence of mosaicism (63.9%) was detected among families with moderate-to-high suspicion of gene mosaicism. In most analyzed cases mosaicism was found to be both uniformly distributed and stable over time. CONCLUSION: This study represents the largest performed to date to investigate mosaicism in patients with PIDs, revealing that it affects approximately 25% of enrolled families. Our results might have serious consequences regarding treatment and genetic counseling and reinforce the use of next-generation sequencing-based methods in the routine analyses of PIDs.

Pentacyclic Triterpenes from Cecropia telenitida Can Function as Inhibitors of 11ß-Hydroxysteroid Dehydrogenase Type 1.

Plant extracts from the genus Cecropia have been used by Latin-American traditional medicine to treat metabolic disorders and diabetes. Previous results have shown that roots of Cecropia telenitida contain pentacyclic triterpenes and these molecules display a hypoglycemic effect in an insulin-resistant murine model. The pharmacological target of these molecules, however, remains unknown. Several lines of evidence indicate that pentacyclic triterpenes inhibit the 11ß-hydroxysteroid dehydrogenase type 1 enzyme, which highlights the potential use of this type of natural product as phytotherapeutic or botanical dietary supplements. The main goal of the study was the evaluation of the inhibitory effect of Cecropia telenitida molecules on 11ß-hydroxysteroid dehydrogenase type 1 enzyme activity. A pre-fractionated chemical library was obtained from the roots of Cecropia telenitida using several automated chromatography separation steps and a homogeneous time resolved fluorescence assay was used for the bio-guided isolation of inhibiting molecules. The screening of a chemical library consisting of 125 chemical purified fractions obtained from Cecropia telenitida roots identified one fraction displaying 82% inhibition of the formation of cortisol by the 11ß-hydroxysteroid dehydrogenase type 1 enzyme. Furthermore, a molecule displaying IC50 of 0.95 ± 0.09 µM was isolated from this purified fraction and structurally characterized, which confirms that a pentacyclic triterpene scaffold was responsible for the observed inhibition. Our results support the hypothesis that pentacyclic triterpene molecules from Cecropia telenitida can inhibit 11ß-hydroxysteroid dehydrogenase type 1 enzyme activity. These findings highlight the potential ethnopharmacological use of plants from the genus Cecropia for the treatment of metabolic disorders and diabetes.

Evaluación de los criterios de clasificación SLICC en pacientes con lupus eritematoso sistémico juvenil seguidos en una clínica pediátrica de Bogotá, Colombia / Evaluation of the SLICC classification criteria in patients with juvenile systemic lupus erythematosus followed up in a pediatric institution in Bogotá, Colombia

RESUMEN Introducción: El lupus eritematoso sistémico es una enfermedad multisistémica que se acompaña de importante morbilidad. A través de los arios se han publicado diversos criterios de clasificación con un desempeño variable. Recientemente el grupo de Clínicas de Colaboración del Lupus Eritematoso Sistémico (SLICC) propone una versión que ha sido validada en niños y adultos. No se conoce el desempeño de estos criterios en la población juvenil colombiana con lupus eritematoso sistémico. Objetivos: Evaluar la sensibilidad y la especificidad de los criterios SLICC en una población pediátrica con lupus eritematoso sistémico juvenil en la ciudad de Bogotá, Colombia. Materiales y métodos: Evaluación retrospectiva de datos clínicos y paraclínicos durante el primer mes de enfermedad en los pacientes con lupus eritematoso sistémico juvenil y en los pacientes controles valorados en una consulta de reumatología pediátrica. Resultados: N = 110, 55 casos y 55 controles. La edad promedio de inicio en los casos fue de 12,8 años (7-15 años) y en controles 11,1 años (2-15 años). La distribución por sexo en los casos fue de F:M 5,4:1 y en los controles F:M 1,8:1. Se aplicaron los criterios ACR1997 y SLICC a ambos grupos de pacientes. La sensibilidad y la especificidad de los criterios ACR fue del 78 y 96% y de los criterios SLICC fue del 89 y 87%, respectivamente. Conclusiones: En este grupo de pacientes pediátricos la sensibilidad de los criterios SLICC en el primer mes de diagnóstico fue mayor al compararla con los criterios ACR 1997. Esto se acompañó de una menor especificidad. Estos hallazgos concuerdan con estudios previos. © 2018 Asociación Colombiana de Reumatología. Publicado por Elsevier España, S.L.U.

ABSTRACT Introduction: Systemic lupus erythematosus with an increase in morbidity. Several classification criteria, with a variable performance, Diagnosis have been published in the last few years. The Systemic Lupus International Collaborating Rheumatology Clinics (SLICC) recently developed a new version that has been validated in children and adults. The performance of these criteria in Colombian juvenile lupus patients is unknown. Objectives: To evaluate the sensitivity and specificity of the SLICC criteria in a group of patients with juvenile systemic lupus erythematosus in Bogotá, Colombia. Materials and methods: Retrospective evaluation of clinical and laboratory data during the first month of disease in patients with juvenile systemic lupus erythematosus and in control patients followed-up in a paediatric rheumatology clinic. Results: The study subjects (n=110), with a mean age of 11.1 years (2-16 years), were divided equally into cases and controls. The gender ratio in cases was F:M 5.4:1 and in controls 1.8:1. The ACR 1997 and SLICC criteria were analysed in both groups of patients. The sensitivity and specificity of ACR criteria were 78% and 96%, respectively, and in SLICC criteria were 89% and 87%, respectively. Conclusions: In this group of paediatric patients, the sensitivity of SLICC criteria during the first month of diagnosis was higher than the ACR 1997 criteria. This was associated with a lower specificity. These results are similar to those of previous studies.

Association of Increasing Frailty with Detrimental Outcomes after Pancreatic Resection.

An association between detrimental outcomes and frailty has been documented; however, the impact specific to pancreatic surgery is unknown. Using NSQIP data, patients were classified as non-, mildly, moderately, or severely frail. A total of 16,028 patients were included in the study; most of the patients were white (78.5%) and underwent pancreaticoduodenectomy (PD) (67%). Complications occurred in 17.6 per cent cases, and the median length of stay (LOS) was 11.89 days. Prolonged LOS and mortality occurred in 9.1 and 2.3 per cent of the cases. In the PD group, most of the patients were mildly frail (40.6%), followed by nonfrail (39.83%), whereas in the distal pancreatectomy (DP) group, the majority were nonfrail (43.82%), followed by mildly frail (39.37%) (P < 0.0001). The 30-day complications, mortality, and LOS were significantly higher in patients undergoing PD compared with DP (19.5 vs 14.3%, 2.8 vs 1.2%, and 13.4 vs 8.7 days, respectively; P < 0.0001). PD conferred a significantly higher risk of death in all frailty groups compared with DP [nonfrail: odds ratio (OR) 1.76, mildly frail: OR 1.03, moderately frail: OR 2.03, P < 0.05], with the exception of severely frail patients. Compared with DP, PD conferred a significant risk of complication in all the frailty groups. Increases in frailty are associated with poorer outcomes after pancreatectomy.

Frailty Predicts Failure to Rescue after Thoracoabdominal Operation.

BACKGROUND: An association between frailty and mortality exists; we hypothesized this is secondary to failure to rescue (F2R). STUDY DESIGN: Data were obtained from the NSQIP (2005 to 2012) for patients undergoing thoracoabdominal operations. Using the Modified Frailty Index, patients were classified as not (0 points), mildly (1 point), moderately (2 points), and severely (≥3) frail. RESULTS: There were 962,913 patients included; a majority were non-frail (52.2%), followed by mildly frail (33.8%). Complications were noted in 15.3%, major complications in 9.5%, mortality in 1.8%, and F2R in 1.3% of patients. On multivariate analysis, increases in frailty were associated with an increase in the risk of major complications (mildly: risk ratio [RR] 1.51; moderately: RR 2.69; and severely frail: RR 5.63 compared with non-frail; p < 0.0001), and death (mildly frail: RR 1.84; moderately frail: RR 4.44; and severely frail: RR 12.4). On univariate analysis, older patients, males, those undergoing small bowel interventions, gastric operations, or other procedures, and the frail were more likely to experience F2R (p < 0.0001). On multivariate analysis, males (RR 1.07), those undergoing small bowel intervention (RR 1.91), gastric operation (RR 1.83), and other procedures (RR 2.43) compared with hernia repair were more likely to experience F2R. As frailty increases F2R increases (mildly frail: RR 1.48; moderately frail: RR 2.41; and severely frail: RR 4.41) (p < 0.0001). Components of the Modified Frailty Index were analyzed separately; measures of impaired functional status were independently associated with increased F2R (RR 2.91; p < 0.0001), and those measuring comorbid medical conditions were not. CONCLUSIONS: Increases in frailty independently predict risk for F2R. The greatest predictors of F2R in the Modified Frailty Index are those associated with disability and not comorbidities.

Manifestaciones mucocutáneas y perfil inmunológico en pacientes con lupus eritematoso sistémico en pediatría / Manifestations of Mucocutaneous and immunological profile in patients with systemic lupus erythematosus in Pediatrics

Introducción: El lupus eritematoso sistémico juvenil (LESJ) es una enfermedad autoinmume cuyas manifestaciones mucocutáneas tienen una expresión clínica variable y su frecuencia varia ampliamente, entre el 10 y el 85%. En la actualidad se incluyen criterios de clasificación de LES al lupus cutáneo agudo, subagudo y crónico, ulceras orales-nasales y la alopecia no cicatrizal.Objetivo: Describir las manifestaciones mucocutáneas y el perfil inmunológico en pacientes con LESJ.

Esclerodermia localizada juvenil: ¿es una enfermedad benigna? / Juvenile localized scleroderma: is it a benign disease?

Resumen Introducción: La esclerodermia localizada juvenil es una enfermedad polimórfica que ocurre con mayor frecuencia en niñas. Se acompaña de morbilidad importante. El compromiso extradérmico es frecuente y se reportan tasas de poliautoinmunidad de hasta 7%. Al momento, se desconocen las características clínicas de los pacientes colombianos con esta enfermedad. Objetivo: Describir las características clínicas, morbilidades y secuelas en pacientes con diagnóstico de esclerodermia localizada juvenil, en múltiples centros de reumatología pediátrica en Colombia. Materiales y métodos: Estudio descriptivo, retrospectivo y multicéntrico. Pacientes con diagnóstico de esclerodermia localizada juvenil con un mínimo de 1 ario de evolución y 6 meses de seguimiento, en 10 centros de reumatología pediátrica mediante revisión de historias clínicas. Resultados: El n = 88. La distribución por género fue: femenino 2,1; masculino 1. Edad promedio al inicio de la enfermedad 7,1 años (0-14). Promedio de duración de la enfermedad al diagnóstico 16,5 meses (1-96). La distribución por subtipos fue morfea circunscrita (32,9%), mixta (31,8%), linear (21,5%, asciende a 55% al incluir formas mixtas con lesiones lineares) generalizada (11,4%) y panesclerótica (2,3%). Se detectaron alteraciones estéticas en el 91%, alteraciones del crecimiento en 41% y compromiso funcional de articulaciones vecinas en 32%. Se presentó compromiso extradérmico en 22,7% y poliautoinmunidad en 12,5%. Conclusiones: La esclerodermia localizada juvenil es una enfermedad polimórfica e impredecible. En la mayoría de los casos el diagnóstico es tardío. La tasa de compromiso extradérmico sugiere que no es una enfermedad limitada a la piel. Un diagnóstico temprano, tratamiento dinámico y seguimiento cercano permiten prevenir y detectar tempranamente complicaciones derivadas de la enfermedad.

Abstract Introduction: Juvenile localized scleroderma is a polymorphic disease. It is more prevalent in girls and has a significant morbidity. Extra-cutaneous involvement is common, and polyautoimmunity can reach 7%. The clinical characteristics of this disease in Colombian patients are currently unknown. Objective: To describe the clinical characteristics, morbidity and outcomes in patients with juvenile localized scleroderma in different paediatric rheumatology clinics in Colombia. Materials and methods: A descriptive, retrospective, and multicentre study was conducted on patients with juvenile localized scleroderma with a minimum of 1 year of disease onset, and 6 months of follow-up in 10 paediatric rheumatology clinics. Results: The study included 88 patients, with a gender distribution of female 2.1: male 1. Mean age at disease onset was 7.1 years (0-14). Mean disease duration at diagnosis was 16.5 months (1-96). Sub-type distribution was, circumscribed (32.9%), mixed (31.8%), and linear (21.5%, that increased to 55% if linear lesions of the mixed subtype are included), generalised (11.4%), and pan-sclerotic morphea (2.3%). Aesthetic compromise was detected in 91%, with growth disturbances in 41%, and joint functional compromise in 32%. Extra-cutaneous involvement occurred in 22.7% and polyautoimmunity in 12.5%. Conclusions: Juvenile localized scleroderma is a polymorphic and unpredictable disease. It diagnosed late in most of the cases. Extra-cutaneous involvement suggests that is not a disease limited to skin. An early diagnosis, a dynamic treatment and a close follow-up helps to prevent, and detect, complications arising from the disease.

Chest Tube Removal in Simple Pneumothorax: Does Water-Seal Duration Matter?

Timing of chest tube (CT) removal after transition from suction to water-seal (WS) varies when treating traumatic simple pneumothoraces (PTXs). Longer periods of WS may identify slow-occurring PTXs reducing CT replacement, whereas shorter periods may expedite patient disposition and have associated cost savings. Prior studies support the need for an interval of WS. We compare durations of WS, looking at rates of CT reinsertion. A 10-year retrospective review on trauma patients with a simple PTX requiring a CT was performed. WS duration of 1 to 8 hours (short - SG) versus 18 to 36 hours (long - LG) were compared. Univariate analysis and multivariate logistic regression were used. Of the 2000 patient charts reviewed, 209 met the criteria, with 43 in the SG and 166 in the LG. Patient demographics and mechanism of injury were similar. There was no difference in CT replacement [6.9% (SG) vs 4.8% (LG), P 0.59]. Logistic regression revealed an increase in CT replacement if the patient ever had positive pressure ventilation (OR 4.1, CI 1.1-17, P 0.04) and if returned to suction from WS (OR 6.3, CI 1.2-28, P 0.03). Short intervals of WS do not increase CT reinsertion while decreasing the total time and morbidity associated with CT.

Primary Site Predicts Grade for Gastroenteropancreatic Neuroendocrine Tumors.

Gastroenteropancreatic neuroendocrine tumors (GEP-NETs) are rare and abstruse neoplasms with increasing incidence and clinical relevance. The National Cancer Data Base was examined to identify GEP-NET cases from 2004 to 2013. In total, 39,454 patients diagnosed with GEP-NET were identified. Median age was 61 years. Majority was female (50.13%), white (79.49%), and had low-grade neoplasms (84.39%). On univariate analysis, age, sex, race, primary site, tumor size, and regional lymph node involvement were associated with tumor grade (P < 0.0001). On multivariate analysis, older age [odds ratio (OR) = 9.57], gender (male, OR = 1.29), and race continued to be associated with high-grade neoplasms. The primary site also remained a significant predictor of tumor grade. High-grade neoplasms were more likely to arise from the esophagus (OR = 317.75), hepatobiliary system (OR = 23.15), colorectum (OR = 14.37), ampulla of Vater (OR = 11.61), and stomach (OR = 7.84) compared with the appendix (OR = 5.41), pancreas (OR = 5.31), and small bowel (referent). The tumor grade for GEP-NETs is highly dependent on the primary site, suggesting different sites may be biologically distinct diseases. A personalized approach to GEP-NET treatment, tailored to the site of origin, is imperative.

REBOA for the IVC? Resuscitative balloon occlusion of the inferior vena cava (REBOVC) to abate massive hemorrhage in retrohepatic vena cava injuries.

BACKGROUND: The use of resuscitative endovascular balloon occlusion as a maneuver for occlusion of the aorta is well described. This technique has life-saving potential in other cases of traumatic hemorrhage. Retrohepatic inferior vena cava (IVC) injuries have a high rate of mortality, in part, due to the difficulty in achieving total vascular isolation. The purpose of this study was to investigate the ability of resuscitative balloon occlusion of the IVC to control suprahepatic IVC hemorrhage in a swine model of trauma. METHODS: Thirteen swine were randomly assigned to control (seven animals) versus intervention (six animals). In both groups, an injury was created to the IVC. Hepatic inflow control was obtained via clamping of the hepatoduodenal ligament and infrahepatic IVC. In the intervention group, suprahepatic IVC control was obtained via a resuscitative balloon occlusion of the IVC placed through the femoral vein. In the control group, no suprahepatic IVC control was established. Vital signs, arterial blood gases, and lactate were monitored until death. Primary end points were blood loss and time to death. Lactate, pH, and vital signs were secondary end points. Groups were compared using the χ and the Student t test with significance at p < 0.05. RESULTS: Intervention group's time to death was significantly prolonged: 59.3 ± 1.6 versus 33.4 ± 12.0 minutes (p = 0.001); and total blood loss was significantly reduced: 333 ± 122 vs 1,701 ± 358 mL (p = 0.001). In the intervention group, five of the six swine (83.3%) were alive at 1 hour compared to zero of seven (0%) in the control group (p = 0.002). There was a trend toward worsening acidosis, hypothermia, elevated lactate, and hemodynamic instability in the control group. CONCLUSIONS: Resuscitative balloon occlusion of the IVC demonstrates superior hemorrhage control and prolonged time to death in a swine model of liver hemorrhage. This technique may be considered as an adjunct to total hepatic vascular isolation in severe liver hemorrhage and could provide additional time needed for definitive repair. LEVEL OF EVIDENCE: Therapeutic study, level II.

Novel nomogram combining depth of invasion and size can accurately predict the risk for regional nodal metastases for appendiceal neuroendocrine tumors (A-NET).

INTRODUCTION: The need for regional lymphadenectomy for treating appendiceal neuroendocrine tumors (A-NET) is determined by the risk of nodal metastasis. Current guidelines for A-NET are solely based on tumor size. Methods Patients with A-NET from 1988 to 2012 were identified from the SEER registry. The depth of invasion was defined as limited to the lamina propria (LP), invading the muscularis propria (MP), and through the serosa (TS). RESULTS: A total of 418 patients were included; the majority were female, white, and node-negative. On univariate and multivariable, the risk of nodal metastasis was associated with age, size, depth of invasion, and extent of surgery. The model predicted the likelihood of nodal metastasis, with an area under the curve of 0.89. On survival analysis, age and tumor size predicted the survival in A-NET. In a Cox regression model, they continued to predict survival. These data were utilized to create a nomogram to predict the risk of nodal metastases. CONCLUSION: This nomogram, accurately predicts the risk of regional nodal metastases in A-NET. In addition to providing valuable information on risk for regional nodal metastases, the depth of invasion is also predictive of survival for A-NET.

Perioperative chemotherapy versus postoperative chemoradiotherapy in patients with resectable gastric/gastroesophageal junction adenocarcinomas: A survival analysis of 5058 patients.

BACKGROUND: Both perioperative chemotherapy (PECT) and postoperative chemoradiotherapy (POCRT) have a significant survival advantage over surgery alone for the treatment of patients with gastric cancer. However, to the best of our knowledge, these regimens have not been compared in a randomized clinical trial. The purpose of the current observational study was to compare overall survival among patients receiving PECT versus POCRT for the treatment of gastric/gastroesophageal junction (GEJ) adenocarcinomas. METHODS: Patients with resected clinical American Joint Committee on Cancer TNM stage II or III adenocarcinomas of the stomach or GEJ from 2004 through 2013 were identified utilizing the National Cancer Data Base. Hazard ratios (HRs), 95% confidence intervals, and P values were computed using a Cox proportional hazards procedure. Multivariable models were adjusted for treatment regimen, age, race, ethnicity, tumor size, TNM stage, Charlson comorbidity index, and tumor grade. RESULTS: Patients receiving PECT had a 72% survival advantage compared with those treated with POCRT (5058 patients; HR, 0.58 [adjusted P<.0001]). The 5-year actuarial survival rate for PECT was 44% compared with 38% for POCRT. A statistically significant survival advantage for PECT also was observed when the analysis was stratified by clinical stage of disease (stage II [3192 patients]: adjusted HR, 0.79 [P = .041]; and stage III [1866 patients]: adjusted HR, 0.49 [P<.0001]). This benefit was greatest among patients with lymph node-positive disease who converted to lymph node-negative status with PECT. CONCLUSIONS: In this large series of patients with stage II/III resected gastric/GEJ adenocarcinomas from >1500 American College of Surgeons Commission on Cancer-accredited facilities, patients receiving PECT were shown to survive longer than those receiving POCRT. Cancer 2017;123:2909-17. © 2017 American Cancer Society.