Abstract Background: Clinically evident interstitial lung disease (ILD) affects between 10 and 42% of the patients with rheumatoid arthritis (RA). Airway involvement seems to be even more common. Most of the available evidence comes from studies performed in established RA patients. The aim of our study was to know the prevalence of non-diagnosed lung disease (airway and interstitial involvement) in patients with early RA and look for associated factors. Methods: We designed an observational, multicenter, cross-sectional study, and included patients with RA of less than two years since diagnosis. We performed a structured questionnaire, HRCT and lung functional tests looking for lung disease, together with joint disease evaluation. We analyzed which variables were associated with the presence of lung disease on HRCT. Results: We included 83 patients, 83% females. The median (IQR) of time since RA diagnosis was 3 (1–6) months. In the HRCT, 57 patients had airway compromisea (72%), and 6 had interstitial abnormalities (7.5%). The most common altertion found in lung functional tests was a reduced DLCO (14%). The presence of at least one abnormality in the physical exam was associated with lung involvement on HRCT [13 (21.6%) vs 0 (0%); p = 0.026]. Also, patients with lung involvement presented significantly lower values of FVC% and DLCO%, and higher values of RV/TLC. No variable related to joint involvement was found associated with alterations in HRCT. Conclusion: Our study shows that a large proportion of early RA patients has abnormal findings in HRCT. Further studies are required to confirm these findings.

Deglutitive disorders that result from different diseases may cause aspiration episodes into the airways, determining a lung injury whose severity will depend on the material that was aspirated and the exposure time. If the aspirated agent has an oily composition, its aspiration is capable of triggering a pulmonary inflammatory reaction called lipoid pneumonia which after some time may cause irreversible parenchymal fibrosis. The case reported in this study describes a patient with deglutitive disorders and prolonged intake of vaseline as laxative. The large assymetric involvement of the lung parenchyma and the diagnostic methodology used to identify lung macrophages charged with lipids take particular importance. A review of the evidence has been carried out in that regard during the discussion of the case.

Los trastornos deglutorios secundarios a diferentes patologías pueden desencadenar episodios aspirativos hacia la vía aérea, condicionando una injuria pulmonar cuya gravedad dependerá del material aspirado y del tiempo de exposición. Cuando el agente aspirado es de composición oleosa, su aspiración es capaz de desencadenar una reacción inflamatoria pulmonar denominada neumonía lipoidea, pudiendo con el tiempo conducir a una fibrosis parenquimatosa irreversible. En el caso presentado se describe un paciente con trastornos deglutorios e ingesta prolongada de vaselina como laxante. Revisten interés el extenso compromiso asimétrico del parénquima pulmonar y la metodología diagnóstica empleada para identificar los macrófagos pulmonares cargados con lípidos, realizándose una revisión de la evidencia al respecto en la discusión del caso.

The Hughes-Stovin Syndrome (HSS) is a very rare clinical disorder characterized by thrombophlebitis and multiple pulmonary and/or bronchial aneurysms. The exact etiology and pathogenesis of HSS is unknown; possible causes include infections and angiodysplasia. HSS has also been considered as a variant of Behcet's disease (BD). Patients with HSS usually present with cough, dyspnea, fever, chest pain and haemoptysis. Early diagnosis and treatment are important, because patients are at a high risk for devastating pulmonary artery aneurysm rupture. Treatment usually involves immunosuppressive agents.

El síndrome de Hughes-Stovin (SHS) es una enfermedad rara, caracterizada por la presencia de tromboflebitis y aneurismas de las arterias pulmonares y/o bronquiales. Si bien la etiología y patogénesis del SHS se desconoce, se han identificado asociaciones con ciertas infecciones y angiodisplasia. Pertenece al grupo de las vasculitis generalizadas, con un mecanismo patológico muy parecido al de la enfermedad de Behçet (EB), incluso considerándose como una variante de esta última. Las manifestaciones clínicas incluyen tos, disnea, fiebre, toracodinia y hemoptisis. Es fundamental realizar un diagnóstico y tratamiento de manera temprana debido a que estos pacientes presentan un alto riesgo de ruptura de los aneurismas de arteria pulmonar. El tratamiento se basa en el uso de agentes inmunosupresores.

Introduction.In Argentina, trauma is the most common cause of death among children older than 1 year old, has a high morbidity rate, and results in large costs for the health system. Objective.To identify causes of injuries in patients admitted to the hospital due to a trauma, and to analyze the relationship between epidemiological factors and severe trauma. Population and Methods.Prospective study. Children and adolescents aged 0 to 18 years old admitted to the hospital due to unintentional trauma between April 2012 and March 2013 were included. They were divided into two groups based on severity according to the pediatric trauma score (8 or lower) to identify risk factors by means of a logistic regression model. Predictive outcome measures: patients' and parents' demographic characteristics, socioeconomic factors, event data, initial care, course, and risk factors. Patients were stratified into three age groups for the analysis of the type of injury and the anatomic location. Results.Two hundred and thirty-seven patients were included. Traumatic brain injuries were predominant among children younger than 3 years old, while limb fractures were most common among children older than 3 years old. In the bivariate analysis, foreign parents, a state of poverty or destitution, an immediate preventable cause, dangerous heights, and an unsafe heating system were statistically significant outcome measures. Based on multiple regression, outcome measures included were foreign parents, living in a slum area, an immediate preventable cause, and an unsafe heating system. Conclusions.The main cause of trauma was related to falls from heights, and some of the studied socioeconomic factors were associated with a higher risk of trauma. This information may be useful to develop prevention measures.

Introducción. En Argentina, el trauma es la causa más frecuente de muerte en niños mayores de 1 año, con una elevada morbilidad y un alto costo para el sistema de salud. Objetivo. Identificar las causas de las lesiones en los pacientes internados por trauma y analizar la asociación entre los factores epidemiológicos y el trauma grave. Población y métodos. Estudio prospectivo. Se incluyeron los niños de 0-18 años internados por trauma no intencional entre abril de 2012 y marzo de 2013. Se dividieron en dos grupos según el índice de trauma pediátrico grave (8 o menor) para identificar factores de riesgo mediante un modelo de regresión logística. Variables predictivas: características demográficas del paciente y sus padres, factores socioeconómicos, datos del incidente, asistencia inicial, evolución y factores de riesgo. Se estratificaron en tres grupos etarios para el análisis de la topografía de la lesión. Resultados. Se incluyeron 237 pacientes. En menores de 3 años, predominó el traumatismo craneoencefálico y, en mayores de 3 años, las fracturas de miembros. En el análisis bivariado, padres extranjeros, indigencia o pobreza, causa inmediata prevenible, altura peligrosa y calefacción insegura resultaron estadísticamente significativos. Por regresión múltiple, quedaron incluidas las variables padres extranjeros, residencia en asentamiento, causa inmediata prevenible y calefacción insegura. Conclusiones. La principal causa de trauma fue la caída de altura y algunos de los factores socioeconómicos explorados se asociaron a mayor riesgo de trauma. Esto podría ser utilizado para elaborar medidas de prevención.

The alpha-1 antitrypsin (AAT) deficiency is a rare hereditary condition which is rarely diagnosed in the world, including Argentina. Underdiagnosis is mainly due to lack of knowledge of its diagnosis and treatment by many physicians. For this reason, the Argentine Association of Respiratory Medicine convened a group of experts to develop the present guidelines. AAT is a glycoprotein secreted by the liver; it reaches high levels in blood, body tissues and fluids. Its main function is to inhibit the neutrophil elastase and other serum proteases providing 90% of human serine antiprotease activity. The AAT deficiency is produced by mutations of the SERPINA1 gene. Its clinical manifestations are pulmonary emphysema, liver cirrhosis, and less often panniculitis, systemic vasculitis and possibly other conditions. The severe AAT deficiency affects mainly Caucasian individuals. The highest prevalence, ranging from 1 in 2000 to 1 in 5000 population is observed in northern, western and central Europe. In the USA and Canada, the prevalence varies from 1 in 5000 to 1 in 10000 population. It is 5 times less frequent in Latin American countries. It is estimated that in Argentina there may be 18000 cases with severe deficiency of SZ y ZZ genotypes, most of them undiagnosed. It is crucial to suspect the disease in order to measure the serum AAT concentration, and, if the concentrations are low, to confirm the diagnosis with the phenotype or genotype determinations. Case detection allows genetic advice, control of blood-related relatives and in selected cases, replacement therapy.

El déficit de alfa-1 antitripsina (AAT) es una condición hereditaria rara y raramente diagnosticada en todo el mundo, incluida Argentina. El infradiagnóstico es fundamentalmente debido a que muchos médicos desconocen su existencia, diagnóstico y tratamiento. Por ello, la Asociación Argentina de Medicina Respiratoria encomendó a un grupo de expertos la elaboración de la presente normativa. La AAT es una glicoproteína secretada por el hígado, muy abundante en sangre, tejidos y fluidos corporales, cuya función principal consiste en inhibir la elastasa del neutrófilo y otras serin proteasas, confiriendo al suero humano más del 90% de su capacidad antiproteasa. El déficit de AAT deriva de mutaciones del gen de la SERPINA1, y se manifiesta clínicamente por enfisema pulmonar, cirrosis hepática y, con menor frecuencia, por paniculitis, vasculitis sistémicas y posiblemente otras enfermedades. El déficit grave de AAT afecta mayoritariamente a individuos de raza caucasiana y tiene su máxima prevalencia (1:2.000-1:5.000 individuos) en el norte, oeste y centro de Europa. En EEUU y Canadá, la prevalencia es de 1: 5.000-10.000, y es 5 veces menor en países latinoamericanos, incluida Argentina, donde se estima que puede haber unos 18.000 individuos con genotipos deficientes graves SZ y ZZ, la inmensa mayoría sin diagnosticar. Sospechar la enfermedad resulta clave para medir la concentración sérica de AAT y completar el diagnóstico con la determinación del fenotipo o genotipo ante concentraciones bajas. La detección de casos permite la puesta en práctica del consejo genético, el chequeo de familiares consanguíneos y, en casos seleccionados, la aplicación de terapia sustitutiva.

Abdominal pain as an initial symptom of meningococcemia is an infrequent entity, rarely described in literature. We present a case of a 4 year-old, male, previously healthy child with a 24 hour history of fever and abdominal pain. He is admitted in a surgical unit with a diagnosis of acute abdomen for surgical resolution. The clinical course turns unfavorably, and patient presents signs of severe sepsis. Urgent laparotomy is performed, observing little brownish fluid and mesenteric adenitis. He then exhibits palpable purpuric rapidly progressive lesions in lower extremities, progressing to septic shock. Later, Neisseria meningitidis serogroup B is isolated from blood cultures. The aim of this article is drawing attention to a nontypical form of manifestation of meningococcemia, as a delayed diagnosis and treatment has an impact on morbidity and mortality among the pediatric population.

El abdomen agudo como síntoma inicial de meningococemia es una entidad muy poco frecuente y raramente descripta en la bibliografía. Presentamos el caso de un paciente de 4 años de edad, sexo masculino, previamente sano, que consulta por síndrome febril y dolor abdominal de 24 h de evolución. Se interna en unidad clínico-quirúrgica con diagnóstico de abdomen agudo quirúrgico. El paciente evoluciona desfavorablemente y súbitamente presenta signos compatibles con sepsis grave. Se realiza laparotomía de urgencia; se observa escasa cantidad de líquido citrino libre en cavidad con adenitis mesentérica. Desarrolla luego lesiones purpúricas palpables de rápida progresión en miembros inferiores y evolución al shock séptico. Se aísla en hemocultivos periféricos Neisseria meningitidis serogrupo B. El objetivo de esta publicación es exponer y alertar sobre una modalidad de presentación clínica poco frecuente de la meningococemia, pues el retraso en su diagnóstico y tratamiento impactan sobre la morbimortalidad en la población pediátrica.