CD8+ NKs as a potential biomarker of complete response and survival with lenalidomide plus R-GDP in the R2-GDP-GOTEL trial in recurrent/refractory diffuse large B cell lymphoma.

Background: Diffuse large B cell lymphoma (DLBCL) is the most common non-Hodgkin lymphoma worldwide. DLBCL is an aggressive disease that can be cured with upfront standard chemoimmunotherapy schedules. However, in approximately 35-40% of the patients DLBCL relapses, and therefore, especially in this setting, the search for new prognostic and predictive biomarkers is an urgent need. Natural killer (NK) are effector cells characterized by playing an important role in antitumor immunity due to their cytotoxic capacity and a subset of circulating NK that express CD8 have a higher cytotoxic function. In this substudy of the R2-GDP-GOTEL trial, we have evaluated blood CD8+ NK cells as a predictor of treatment response and survival in relapsed/refractory (R/R) DLBCL patients. Methods: 78 patients received the R2-GDP schedule in the phase II trial. Blood samples were analyzed by flow cytometry. Statistical analyses were carried out in order to identify the prognostic potential of CD8+ NKs at baseline in R/R DLBCL patients. Results: Our results showed that the number of circulating CD8+ NKs in R/R DLBCL patients were lower than in healthy donors, and it did not change during and after treatment. Nevertheless, the level of blood CD8+ NKs at baseline was associated with complete responses in patients with R/R DLBCL. In addition, we also demonstrated that CD8+ NKs levels have potential prognostic value in terms of overall survival in R/R DLBCL patients. Conclusion: CD8+ NKs represent a new biomarker with prediction and prognosis potential to be considered in the clinical management of patients with R/R DLBCL. Clinical trial registration: https://www.clinicaltrialsregister.eu/ctr-search/search?query=2014-001620-29 EudraCT, ID:2014-001620-29.

Case report emphasize pearls of duodenal perforation.

INTRODUCTION: Isolated duodenal perforation secondary to trauma is a rare abdominal surgical condition, with a questionable surgical approach depending on the case. PRESENTATION OF CASE: This is a case report of a 27-year-old male patient who presented with a free perforation in the posterior wall of the third portion of the duodenal frame and secondary retropneumoperitoneum without injuring any contiguous organ, after a medium-impact blunt abdominal trauma during a soccer game. DISCUSSION: A laparotomy was performed, followed by duodenorraphy with Connell-Mayo suture and Lambert suture using vascular prolene in two planes. A nasogastric tube was placed up to the jejunum, and a Jackson-Pratt drain was placed in close to the duodenum next to the sutures. During hospitalization was found a positive bacterial culture of the peritoneal fluid hence received antibiotics, without complication. CONCLUSION: It is essential to make a timely diagnosis with its respective individualized surgical approach and it must be managed as an emergency surgical procedure.

Association of the Surgical Technique With the Structural Valve Deterioration of a Bioprosthesis: A Prospective Cohort Study.

The Trifecta aortic valve is a prosthesis with externally mounted leaflets and a stent which may be deformed during implant. Our aim was to know if the use of the holder as a protection device during the knotting has an impact on the incidence of structural valve deterioration (SVD) or endocarditis. Prospective cohort study where all patients who underwent aortic valve replacement with a Trifecta aortic valve between 2013 and 2018 were included. The use of the holder as a protection device was collected in a database. Propensity-score matched methods were used and analyses were based on competing events. Death without SVD or prosthesis replacement not due to SVD was considered competing events. 782 patients were included, 352 pairs after the matching. Rates of SVD at 5 and 8 years were 5.8% (95% CI 3.5-8.7) and 13.6% (95% CI 9.2-18.9) in the group without holder and 2.3% (95% CI 1-4.5) and 7% (95% CI 4.2-10.8) in the group with holder; sHR = 0.49 (95% CI 0.27-0.86; P = 0.015). The risk of endocarditis at 8 years was 4.8% (95% CI 2.8-7.4) in the group without holder and 2.3% (95% CI 1.1-4.3) in the group with holder, sHR = 0.49 (95% CI 0.21-1.15, P = 0.1). The use of holder as a protection device during the knotting of the Trifecta aortic valve is associated with less risk of SVD.

Lenalidomide plus R-GDP (R2-GDP) in Relapsed/Refractory Diffuse Large B-Cell Lymphoma: Final Results of the R2-GDP-GOTEL Trial and Immune Biomarker Subanalysis.

PURPOSE: New therapeutic options are needed in relapsed/refractory diffuse large B-cell lymphoma (R/R DLBCL). Lenalidomide-based schedules can reverse rituximab refractoriness in lymphoma. PATIENTS AND METHODS: In the phase II R2-GDP trial, 78 patients unsuitable for autologous stem cell transplant received treatment with the following schedule: lenalidomide 10 mg Days (D)1-14, rituximab 375 mg/m2 D1, cisplatin 60 mg/m2 D1, gemcitabine 750 mg/m2 D1 and D8, and dexamethasone 20 mg D1-3, up to 6 cycles (induction phase), followed by lenalidomide 10 mg (or last lenalidomide dose received) D1-21 every 28 days (maintenance phase). Primary endpoint was overall response rate (ORR). Secondary endpoints included progression-free survival (PFS), overall survival (OS), safety, and monitorization of key circulating immune biomarkers (EU Clinical Trials Register number: EudraCT 2014-001620-29). RESULTS: After a median follow-up of 37 months, ORR was 60.2% [37.1% complete responses (CR) and 23.1% partial responses (PR)]. Median OS was 12 months (47 vs. 6 months in CR vs. no CR); median PFS was 9 months (34 vs. 5 months in CR vs. no CR). In the primary refractory population, ORR was 45.5% (21.2% CR and 24.3% PR). Most common grade 3-4 adverse events were thrombocytopenia (60.2%), neutropenia (60.2%), anemia (26.9%), infections (15.3%), and febrile neutropenia (14.1%). Complete responses were associated with a sharp decrease in circulating myeloid-derived suppressor cells and regulatory T cells. CONCLUSIONS: R2-GDP schedule is feasible and highly active in R/R DLBCL, including the primary refractory population. Immune biomarkers showed differences in responders versus progressors.

Blunt abdominal trauma managed with mesenteric embolization: case report.

Blunt abdominal trauma occurs in 20% of polytraumatized patients, of which approximately 5% may have mesenteric lesions. We present a case of a woman suffering from a blunt abdominal trauma, who underwent computed tomography with evidence of hematoma on the right flank, which was controlled by angioembolization of the superior mesenteric artery. Treatment of mesenteric lesions will be determined according to the hemodynamic status of the patient, since in view of stability, a choice can be made between open surgery and embolization, but in case of instability, laparotomy is recommended.

El trauma abdominal cerrado se presenta en el 20% de los pacientes politraumatizados, de los cuales aproximadamente el 5% pueden llegar a tener lesiones mesentéricas. Presentamos el caso de una mujer que sufre un traumatismo abdominal cerrado a la cual se le realizó tomografía computarizada con evidencia de un hematoma en el flanco derecho, que se logra controlar por angioembolización de la arteria mesentérica superior. El tratamiento de las lesiones mesentéricas se determinará según el estado hemodinámico del paciente, pues ante la estabilidad se puede escoger entre cirugía abierta y embolización, pero en caso de inestabilidad se recomienda la laparotomía.

Portosplenomesenteric thrombosis after laparoscopic gastric sleeve.

BACKGROUND: Bariatric surgery is an effective way to lose weight and the laparoscopic gastric sleeve is one of the techniques currently used. Portosplenomesenteric venous thrombosis is a complication that occurs in patients undergoing this surgical technique. At the moment there are no protocols to reduce the risk of this thrombotic event, so we show our experience and propose an algorithm. CASE REPORT: A total of 620 patients were evaluated, six of whom presented between postoperative days 10 to 20 abdominal pain, nausea and dehydration. Therefore, a double contrast abdominal computed tomography scan was carried out, which demonstrated portomesenteric and portoesplenomesenteric thrombosis, in addition to two patients with signs of intestinal ischemia, which required reoperation. One of the patients died of pulmonary thromboembolism.

ANTECEDENTES: La cirugía bariátrica es una forma eficaz de perder peso, y la manga gástrica laparoscópica es una de las técnicas usadas actualmente. La trombosis venosa portoesplenomesénterica es una complicación que se presenta en los pacientes sometidos a esta técnica quirúrgica. En el momento no existen protocolos para disminuir el riesgo de este evento trombótico, por lo cual mostramos nuestra experiencia y proponemos un algoritmo. CASOS CLÍNICOS: Se evaluaron 620 pacientes, de los cuales seis, entre los días 10 y 20 de posoperatorio, presentaron dolor abdominal, náuseas y deshidratación. Se les realizó tomografía computarizada de abdomen con doble contraste, que demostró trombosis portomesentérica y portoesplenomesentérica; además, dos pacientes tuvieron signos de isquemia intestinal y requirieron reintervención quirúrgica. Uno de los pacientes falleció por tromboembolia pulmonar.

Real-World Characteristics and Outcome of Patients Treated With Single-Agent Ibrutinib for Chronic Lymphocytic Leukemia in Spain (IBRORS-LLC Study).

BACKGROUND: Ibrutinib demonstrated remarkable efficacy and favorable tolerability in patients with untreated or relapsed/refractory (R/R) chronic lymphocytic leukemia (CLL), including those with high-risk genetic alterations. The IBRORS-CLL study assessed the characteristics, clinical management and outcome of CLL patients receiving ibrutinib in routine clinical practice in Spain. PATIENTS: Observational, retrospective, multicenter study in CLL patients who started single-agent ibrutinib as first-line treatment or at first or second relapse between January 2016 and January 2019. RESULTS: A total of 269 patients were included (median age: 70.9 years; cardiovascular comorbidity: 55.4%, including hypertension [47.6%] and atrial fibrillation [AF] [7.1%]). Overall, 96.7% and 69% of patients underwent molecular testing for del(17p)/TP53 mutation and IGHV mutation status. High-risk genetic features included unmutated IGHV (79%) and del(17p)/TP53 mutation (first-line: 66.3%; second-line: 23.1%). Overall, 84 (31.2%) patients received ibrutinib as first-line treatment, and it was used as second- and third-line therapy in 121 (45.0%) and 64 (23.8%) patients. The median progression-free survival and overall survival were not reached irrespective of del(17p)/TP53, or unmutated IGHV. Common grade ≥3 adverse events were infections (12.2%) and bleeding (3%). Grade ≥3 AF occurred in 1.5% of patients. CONCLUSION: This real-world study shows that single-agent ibrutinib is an effective therapy for CLL, regardless of age and high-risk molecular features, consistent with clinical trials. Additionally, single-agent ibrutinib was well tolerated, with a low rate of cardiovascular events. This study also emphasized a high molecular testing rate of del(17p)/TP53 mutation and IGHV mutation status in clinical practice according to guideline recommendations.

Hepatoduodenal fistula closure diagnosed and characterized Ecoendoscopically (EUS) and managed by OTSC CLIP OVESCO: A case report.

INTRODUCTION: Gastrointestinal (GI) tract perforations are a significant source of morbidity in clinical practice; therefore, an early diagnosis is fundamental for early management. In management, surgery is the definitive therapy; however, there is evidence of a strong response to conservative measurements. PRESENTATION OF CASE: A 53-year-old man known for a laparoscopic cholecystectomy with difficult access and postoperative complications was admitted to our emergency department due to a five-day clinical history comprising acute abdominal pain and feverish peaks up to 38.4 °C. Diagnosis methods CT and NMR were performed but did not lead to a clear diagnosis. Therefore, a EUS was performed observing an anechoic path that communicates the duodenal wall with a right subhepatic collection that was in contact with the proximal bile duct, thickening its walls. A 5 mm fistulous orifice was found. The hepatoduodenal fistula was close endoscopically with the over-the-scope-clip OVESCO OTSC. Post endoscopic closure course was uneventful. DISCUSSION: Duodenal fistulae are considered one of the most serious complications in gastrointestinal surgery, when conventional diagnosis methods do not permit the clinicians to get either a medical diagnosis or the management; the EUS can. Advances in interventional endoscopic techniques offer an alternative management for the closure of GI fistulae. CONCLUSION: Whenever the presence of an organized fistula is clinically suspected, EUS can be considered a useful tool that allows not only the characterization of the fistulous path and but also the definition of the minimally invasive endoscopic treatment.

Circulating myeloid-derived suppressor cells and regulatory T cells as immunological biomarkers in refractory/relapsed diffuse large B-cell lymphoma: translational results from the R2-GDP-GOTEL trial.

BACKGROUND: The search for immunological markers with ability of predicting clinical outcome is a priority in lymphomas, and in cancer in general. It is well known that some immunomodulatory cells, such as myeloid derived suppressor cells (MDSCs) or regulatory T cells (Tregs), are recruited by tumors, jeopardizing antitumor immunosurveillance. In this work, we have studied blood levels of these immunosuppressive cells in patients with relapsed/refractory diffuse large B-cell lymphoma (R/R DLBCL), prior to and along the course of the experimental rituximab, gemcitabine, dexamethasone, and cisplatin (R2-GDP) schedule, as a translational substudy of the R2-GDP-GOTEL trial (EudraCT Number: 2014-001620-29), which included lenalidomide as an immunomodulator. METHODS: Blood samples were taken before treatment, at cycle 3 and end of induction. Samples were analyzed by flow cytometry. Non-parametric tests were used. Mann-Whitney U test was used to compare basal cells distributions, and Wilcoxon test was considered to compare cells distribution at different times. Spearman test was performed to measure the degree of association between cell populations. RESULTS: In this study, MDSC and Treg circulating concentration was found increased in all patients compared with a healthy control group and decreased after treatment only in patients with longest overall survival (>24 months), reaching the levels of the healthy group. Likewise, the number of inhibited T lymphocytes expressing Programmed Death-1 (PD-1) were increased in peripheral blood from patients and decreased on the treatment, whereas activated T lymphocytes increased after therapy in those with better overall survival. CONCLUSIONS: In conclusion, blood concentration of MDSCs and Treg cells may be good prognostic markers for overall survival after 2 years in R/R DLBCL. These results point to a possible role of these elements in the immunosuppression of these patients, as assessed by the circulating activated and inhibited T lymphocytes, and therefore, they may be considered as therapeutic targets in DLBCL.

R-COMP versus R-CHOP as first-line therapy for diffuse large B-cell lymphoma in patients ≥60 years: Results of a randomized phase 2 study from the Spanish GELTAMO group.

The use of non-pegylated liposomal doxorubicin (Myocet® ) in diffuse large B-cell lymphoma (DLBCL) has been investigated in retrospective and single-arm prospective studies. This was a prospective phase 2 trial of DLBCL patients ≥60 years old with left ventricular ejection fraction (LVEF) ≥55% randomized to standard R-CHOP or investigational R-COMP (with Myocet® instead of conventional doxorubicin). The primary end point was to evaluate the differences in subclinical cardiotoxicity, defined as decrease in LVEF to <55% at the end of treatment. Secondary objectives were efficacy, safety, and variations of troponin and N-terminal pro-B-type natriuretic peptide (NT-proBNP) and LVEF along follow-up. Ninety patients were included, 45 in each group. No differences were observed in the percentage of patients with LVEF <55% at end of treatment (11% in R-CHOP arm vs. 7% in R-COMP arm, p = 0.697) or at 4 months (10% vs. 6%, respectively, p = 0.667) and 12 months (8% vs. 7%, respectively, p = 1). However, a higher percentage of R-CHOP compared with R-COMP patients showed increased troponin levels in cycle 6 (100% vs. 63%, p = 0.001) and at 1 month after treatment (88% vs. 56%, respectively, p = 0.015). Cardiovascular adverse events were seen in five R-CHOP patients (nine episodes, four grade ≥3) and in four R-COMP patients (five episodes, all grade 1-2). No significant differences in efficacy were observed. In conclusion, R-COMP is a feasible immunochemotherapy schedule for DLBCL patients ≥60 years, with similar efficacy to R-CHOP. However, the use of non-pegylated doxorubicin instead of conventional doxorubicin was not associated with less early cardiotoxicity, although some reduced cardiac safety signals were observed. Trial registration: ClinicalTrials.gov Identifier: NCT02012088.

Survival outcomes of patients with extranodal natural-killer T-cell lymphoma: a prospective cohort study from the international T-cell Project.

BACKGROUND: Extranodal natural killer (NK) T-cell lymphoma (ENKTL) is a unique clinicopathological entity, typically associated with poor survival outcomes. Most published data have come from east Asian study groups, with little information available from international cohorts. The effects of treatment advances on routine clinical practice across continental territories has not been clear. We aimed to improve understanding of the clinical characteristics and outcomes of patients with ENKTL. METHODS: We did a substudy of patients with ENKTL from the T-cell Project, a global prospective cohort study. The T-cell Project registered consecutively diagnosed adults (>18 years) with newly diagnosed, untreated mature T-cell or NK lymphomas (WHO 2001 or 2008 classifications) from 74 centres in 13 countries (in Asia, Europe, North America, and South America). In total, 1695 patients with mature T-cell or NK lymphomas were enrolled between Oct 12, 2006 and Feb 28, 2018 in the T-cell Project. The first patient with ENKTL was enrolled on Feb 15, 2007, and the last on May 26, 2017. Data on baseline characteristics, first-line treatment, treatment response, and survival outcomes were recorded in a central database (locked March 30, 2019). The primary outcome was 5-year overall survival. The T-cell Project is registered on ClinicalTrials.gov, NCT01142674. FINDINGS: 166 patients were diagnosed with ENKTL, comprising 11% of 1553 eligible registered cases and distributed across 40 participating centres in four continents. At a median follow-up of 44 months (IQR 20-61), overall survival at 5 years was 54% (95% CI 44-63) in patients with nasal disease (n=98) and 34% (27-46) in patients with extranasal disease (n=68). INTERPRETATION: To our knowledge, this study presents the largest international cohort of patients with ENKTL. We describe a clinically significant improvement in the survival of patients with ENKTL treated in routine clinical practice over the past decade, likely to be attributable to the increasing use of treatment protocols specific for ENKTL. FUNDING: The Fondazione Cassa di Risparmio di Modena, the Associazione Angela Serra per la Ricerca sul Cancro, the Fondazione Italiana Linfomi, Allos Therapeutics, Spectrum Pharmaceuticals, Associazione Italiana per la Ricerca sul Cancro, and the National Cancer Institute at the National Institutes of Health.

Effects of Chronic Administration of Capsaicin on Biomarkers of Kidney Injury in Male Wistar Rats with Experimental Diabetes.

Capsaicin is an agonist of the transient receptor potential vanilloid type 1 (TRPV1) channel, which has been related to the pathophysiology of kidney disease secondary to diabetes. This study aimed to evaluate the chronic effect of capsaicin administration on biomarkers of kidney injury in an experimental rat model of diabetes. Male Wistar rats were assigned to four groups: (1) healthy controls without diabetes (CON), (2) healthy controls plus capsaicin at 1 mg/kg/day (CON + CAPS), (3) experimental diabetes without capsaicin (DM), and (4) experimental diabetes plus capsaicin at 1 mg/kg/day (DM + CAPS). For each group, 24-h urine samples were collected to determine diuresis, albumin, cystatin C, ß2 microglobulin, epidermal growth factor (EGF), alpha (1)-acid glycoprotein, and neutrophil gelatinase-associated lipocalin (NAG-L). Blood samples were drawn to measure fasting glucose. After 8 weeks, the CON + CAPS and DM + CAPS groups showed increased diuresis compared to the CON and DM groups, but the difference was significant only in the DM + CAPS group. The two-way ANOVA only showed a statistically significant effect of CAPS on the urinary EGF levels, as well as a tendency to have a significant effect in the urinary NAG-L levels. The EGF levels decreased in both CAPS-treated groups, but the change was only significant in the CON + CAPS group vs. CON group; and the NAG-L levels were lower in both CAPS-treated groups. These results show that capsaicin had a diuretic effect in healthy and diabetic rats; additionally, it increased the urinary EGF levels and tended to decrease the urinary NAG-L levels.

Extracorporeal membrane oxygenation system as a bridge to reparative surgery in ventricular septal defect complicating acute inferoposterior myocardial infarction.

Post-infarction ventricular septal defect (VSD) is a rare but potentially lethal complication of acute myocardial infarction. Medical management is usually futile, so definitive surgery remains the treatment of choice but the risk surgery is very high and the optimal timing for surgery is still under debate. A 55-year-old man with no previous medical history attended the emergency-room for 12 h evolution of oppressive chest pain and strong anginal pain 7 days ago. On physical examination, blood pressure was 96/70 mmHg, pansystolic murmur over left sternal border without pulmonary crackles. An electrocardiogram revealed sinus rhythm 110 bpm, elevation ST and Q in inferior-posterior leads. Transthoracic echocardiogram showed inferoposterior akinesia, posterior-basal septal rupture (2 cm × 2 cm) with left-right shunt. Suspecting VSD in inferior-posterior acute myocardial infarction evolved, we performed emergency coronarography with 3-vessels disease and complete subacute occlusion of the mid segment of the right coronary artery. Left ventriculography demonstrated shunting of contrast from the left ventricule to the right ventricule. He was rejected for heart transplantation because of his age. Considering the high surgical risk to early surgery and his hemodynamic and clinical stability, delayed surgical treatment is decided, and 4 days after admission the patient suffered hemodynamic instability so venoarterial extracorporeal membrane oxygenation system (ECMO) is implanted as a bridge to reparative surgery. The 9th day after admission double bypass, interventricular defect repair with pericardial two-patch exclusion technique, and ECMO decannulation were performed. The patient's postoperative course was free of complications and was discharged 10 days post VSD repair surgery. Follow-up 3-month later revealed the patient to be in good functional status and good image outcome with intact interventricular septal patch without shunt. ECMO as a bridge to reparative surgery in postinfarction VSD is an adequate option to stabilize patients until surgery.

Predictive risk models for proximal aortic surgery.

Predictive risk models help improve decision making, information to our patients and quality control comparing results between surgeons and between institutions. The use of these models promotes competitiveness and led to increasingly better results. All these virtues are of utmost importance when the surgical operation entails high-risk. Although proximal aortic surgery is less frequent than other cardiac surgery operations, this procedure itself is more challenging and technically demanding than other common cardiac surgery techniques. The aim of this study is to review the current status of predictive risk models for patients who undergo proximal aortic surgery, which means aortic root replacement, supracoronary ascending aortic replacement or aortic arch surgery.

Malate-dependent Fe accumulation is a critical checkpoint in the root developmental response to low phosphate.

Low phosphate (Pi) availability constrains plant development and seed production in both natural and agricultural ecosystems. When Pi is scarce, modifications of root system architecture (RSA) enhance the soil exploration ability of the plant and lead to an increase in Pi uptake. In Arabidopsis, an iron-dependent mechanism reprograms primary root growth in response to low Pi availability. This program is activated upon contact of the root tip with low-Pi media and induces premature cell differentiation and the arrest of mitotic activity in the root apical meristem, resulting in a short-root phenotype. However, the mechanisms that regulate the primary root response to Pi-limiting conditions remain largely unknown. Here we report on the isolation and characterization of two low-Pi insensitive mutants (lpi5 and lpi6), which have a long-root phenotype when grown in low-Pi media. Cellular, genomic, and transcriptomic analysis of low-Pi insensitive mutants revealed that the genes previously shown to underlie Arabidopsis Al tolerance via root malate exudation, known as SENSITIVE TO PROTON RHIZOTOXICITY (STOP1) and ALUMINUM ACTIVATED MALATE TRANSPORTER 1 (ALMT1), represent a critical checkpoint in the root developmental response to Pi starvation in Arabidopsis thaliana Our results also show that exogenous malate can rescue the long-root phenotype of lpi5 and lpi6 Malate exudation is required for the accumulation of Fe in the apoplast of meristematic cells, triggering the differentiation of meristematic cells in response to Pi deprivation.

Estenosis subglótica como manifestación inicial de granulomatosis de Wegener en una adolescente: Caso clínico / Subglottic stenosis as the initial manifestation of Wegener's granulomatosis in a teenager: Case report

La granulomatosis de Wegener es una vasculitis granulomatosa necrotizante de origen autoinmune que afecta, principalmente, a la vía aérea superior e inferior y los riñones. Es muy rara en los niños y adolescentes. Cuando se inicia a edad temprana, se asocia, con frecuencia, a estenosis subglótica. La estenosis subglótica es una manifestación potencialmente fatal de la granulomatosis de Wegener. Su diagnóstico requiere un alto grado de sospecha porque puede desarrollarse en ausencia de otros signos de actividad y, en ocasiones, es la manifestación inicial de la enfermedad. Se confirma mediante la visualización endoscópica de la lesión. El tratamiento es complejo; requiere, a menudo, de repetidas intervenciones, debido a reestenosis. Presentamos a una paciente de 13 años de edad con estenosis subglótica secundaria a granulomatosis de Wegener. Describimos las manifestaciones clínicas, el diagnóstico y el tratamiento de esta rara causa de dificultad respiratoria en la población pediátrica.

Wegener's granulomatosis is a necrotizing granulomatous vasculitis of autoimmune origin that primarily affects the upper and lower airways and kidneys. It is very rare in children and adolescents. When started at a young age it is often associated with subglottic stenosis. Subglottic stenosis is a potentially fatal manifestation of Wegener's granulomatosis. Its diagnosis requires a high index of suspicion since it might develop in the absence of other signs of activity. Occasionally, subglottic stenosis may present as the initial manifestation of the disease. Diagnosis is confirmed by endoscopic visualization of the lesion. The treatment is complex, and it often requires repeated interventions due to restenosis. We present a 13-year-old patient with subglottic stenosis secondary to Wegener's granulomatosis. We describe the clinical manifestations, diagnosis and treatment of this rare cause of respiratory distress in the pediatric population.

[Subglottic stenosis as the initial manifestation of Wegener's granulomatosis in a teenager. Case report]. / Estenosis subglótica como manifestación inicial de granulomatosis de Wegener en una adolescente. Caso clínico.

Wegener's granulomatosis is a necrotizing granulomatous vasculitis of autoimmune origin that primarily affects the upper and lower airways and kidneys. It is very rare in children and adolescents. When started at a young age it is often associated with subglottic stenosis. Subglottic stenosis is a potentially fatal manifestation of Wegener's granulomatosis. Its diagnosis requires a high index of suspicion since it might develop in the absence of other signs of activity. Occasionally, subglottic stenosis may present as the initial manifestation of the disease. Diagnosis is confirmed by endoscopic visualization of the lesion. The treatment is complex, and it often requires repeated interventions due to restenosis. We present a 13-year-old patient with subglottic stenosis secondary to Wegener's granulomatosis. We describe the clinical manifestations, diagnosis and treatment of this rare cause of respiratory distress in the pediatric population.

La granulomatosis de Wegener es una vasculitis granulomatosa necrotizante de origen autoinmune que afecta, principalmente, a la vía aérea superior e inferior y los riñones. Es muy rara en los niños y adolescentes. Cuando se inicia a edad temprana, se asocia, con frecuencia, a estenosis subglótica. La estenosis subglótica es una manifestación potencialmente fatal de la granulomatosis de Wegener. Su diagnóstico requiere un alto grado de sospecha porque puede desarrollarse en ausencia de otros signos de actividad y, en ocasiones, es la manifestación inicial de la enfermedad. Se confirma mediante la visualización endoscópica de la lesión. El tratamiento es complejo; requiere, a menudo, de repetidas intervenciones, debido a reestenosis. Presentamos a una paciente de 13 años de edad con estenosis subglótica secundaria a granulomatosis de Wegener. Describimos las manifestaciones clínicas, el diagnóstico y el tratamiento de esta rara causa de dificultad respiratoria en la población pediátrica.