Causa inusual de cianosis en el recién nacido: hemoglobina M / Unusual cause of cyanosis in the newborn: hemoglobin M

Resumen La hemoglobina M es un desorden hereditario infrecuente, causante de metahemoglobinemia y, por ende, cianosis. Las manifestaciones clínicas de esta enfermedad son amplias y variadas, por lo que se debe considerar como diagnóstico diferencial en un recién nacido con cianosis sin otra causa aparente. A continuación, se presenta el caso de un recién nacido de 5 horas de vida con cianosis generalizada desde el nacimiento y con saturaciones de oxígeno de entre 60-70%, en el que se descartaron patologías como hipoxia perinatal, patología pulmonar o cardíaca y sepsis y se documentó un nivel de metahemoglobina elevado, reportado en 21,6%, con lo cual se estableció el diagnóstico de metahemoglobinemia. El tratamiento administrado fueron dos dosis de azul de metileno, pero no hubo respuesta clínica. Por este motivo, se realizó electroforesis de hemoglobina, la cual fue compatible con hemoglobina M (Iwate o Kankakee), lo que se confirma su causa congénita de metahemoglobinemia.

Abstract Hemoglobin M is a rare hereditary disorder that causes ethemoglobinemia and therefore cyanosis. The clinical manifestations of this condition differs considerably, so it should be considered as a differential diagnosis in a newborn with cyanosis, with no other apparent cause. The case of a 5 hours old newborn is presented below, with generalized cyanosis from birth with oxygen saturations between 60-70%, in whom, upon ruling out pathologies such as perinatal hypoxia, pulmonary disease, heart disease and sepsis, a high level of methemoglobin is documented, reported in 21,6%; the diagnosis of methemoglobinemia was established. The treatment administered was two doses of methylene blue with no response. For this reason, hemoglobin electrophoresis was performed, which was compatible with Hemoglobin M (Iwate or Kankakee), confirming its congenital cause.

Sulfohemoglobinemia secundaria a zopiclona. Casos clínicos / Sulfhemoglobinemia secondary to the use of zopiclone. Report of two cases

Sulfhemoglobin (SulfHb) is formed by hemoglobin (Hb) oxidation by sulfur compounds. Sulfhemoglobinemia is mainly associated with drugs or intestinal bacterial overgrowth. Patients present with central cyanosis, an abnormal pulse oximetry and normal arterial oxygen partial pressure. These features are shared with methemoglobinemia (MetHb) whose diagnosis requires an arterial co-oximetry. Depending on the device used, SulfHb may produce interference with this technique. We report two females aged 31 and 43 years, consulting at the emergency room with cyanosis. Both had a history of acute and chronic, high dose zopiclone ingestion. Pulse oximetry showed desaturation but with normal arterial oxygen partial pressure. Cardiac and pulmonary diseases were ruled out. Co-oximetry in two different analyzers showed interference or normal MetHb percentages. No other complications ensued, and cyanosis decreased over days. Since MetHb was discarded among other causes of cyanosis in a compatible clinical context, the diagnosis of sulfhemoglobinemia was made. The confirmatory method is not available in Chile. The presence of SulfHb is difficult to diagnose, confirmatory tests are not readily available, and it frequently interferes with arterial co-oximetry. This is attributed to a similar absorbance peak of both pigments in arterial blood. Venous co-oximetry can be useful in this context. SulfHb is a self-limited condition in most cases, however it must be differentiated from methemoglobinemia to avoid inappropriate treatments like methylene blue.

Causas de estridor congénito / Causes of congenital stridor

INTRODUCTION. Airway abnormalities are rare but potentially fatal. Stridor is a res-piratory noise with greater predominance in the inspiratory phase. OBJECTIVE. To evaluate the etiology of stridor, determine its comorbidities and mortality. MATERIALS AND METHODS. Retrospective cross-sectional study. Population of 110 and sample of 33 data from the Medical Records of neonatal or infant patients who presented stri-dor at the Carlos Andrade Marín Specialties Hospital of Quito-Ecuador, from january 2009 to december 2020. RESULTS. The 51,51% (17; 33) of cases were men. The age of the first consultation for stridor was within the first month in 18,00% (6; 33) and 40,00% (13; 33) at 3 months. The most frequent congenital laryngeal patholo-gy was: laryngomalacia 81,82% (27; 33), followed by subglottic stenosis 9,09% (3; 33), bilateral chordal paralysis 6,06% (2; 33) and tracheal stenosis 3,03% (1; 33). The 51,51% (17; 33) presented comorbidities of causes: neurological, pulmonary and genetic among the main ones. Mortality was 18,20% (6; 33) related to the severity of comorbidities, except one secondary to tracheal stenosis. CONCLUSION. Laryn-gomalacia and subglottic stenosis were the predominant pathologies with congenital stridor. The comorbidities that occurred were neurological, pulmonary, genetic and caused mortality within 90 days after diagnosis.

INTRODUCCIÓN. Las anomalías de la vía aérea son poco frecuentes, pero potencialmente mortales. El estridor es un ruido respiratorio con mayor predominio en la fase inspiratoria. OBJETIVO. Evaluar la etiología del estridor, determinar sus comorbilidades y la mortalidad. MATERIALES Y MÉTODOS. Estudio transversal retrospectivo. Población de 110 y muestra de 33 datos de Historias Clínicas de pacientes neonatos o lactantes que presentaron estridor en el Hospital de Especialidades Carlos Andrade Marín de Quito - Ecuador, de enero 2009 a diciembre 2020. RESULTADOS. El 51,51% (17; 33) de casos fueron hombres. La edad de la primera consulta por estridor fue dentro del primer mes en el 18,00% (6; 33) y del 40,00% (13; 33) a los 3 meses. La patología congénita laríngea más frecuente fue: laringomalacia 81,82% (27; 33), seguida de estenosis subglótica 9,09% (3; 33), parálisis cordal bila-teral 6,06% (2; 33) y estenosis traqueal 3,03% (1; 33). El 51,51% (17; 33) presentaron comorbilidades de causas: neurológica, pulmonar y genética entre las principales. La mortalidad fue 18,20% (6; 33) relacionada con la severidad de las comorbilidades, excepto una secundaria a estenosis traqueal. CONCLUSIÓN. La laringomalacia y la estenosis subglótica fueron las patologías que predominaron con estridor congénito. Las comorbilidades que se presentaron fueron neurológica, pulmonar, genética y causaron mortalidad dentro de los 90 días posteriores al diagnóstico.

Caracterización de estenosis traqueal por intubación prolongada en pacientes adultos / Characterization of tracheal stenosis by prolonged Intubation in adult patients

La estenosis traqueal es la disminución del calibre de la luz laríngea y traqueal como resultado de la maduración de tejido cicatrizal por lesión isquémica que el balón del tubo endotraqueal produce sobre las mucosas de la pared laringo traqueal cuando es insuflada por encima de la presión capilar (20-30 mm Hg) por un periodo incluso corto. La Asociación Americana de Cuidados Respiratorios recomienda que se utilice intubación para aquellos pacientes que ameriten ventilación mecánica por 7-10 días o menos y traqueostomía para aquellos pacientes que necesitan ventilación por más tiempo. Objetivo: Caracterizar la estenosis traqueal por intubación prolongada. Metodología: Se realizó un estudio descriptivo, retrospectivo que incluyó pacientes adultos con diagnóstico de estenosis traqueal por intubación mayor de 7 días en el Hospital General San Juan de Dios durante enero 2016 a diciembre 2019. Se evaluaron los datos epidemiológicos, clínicos, diagnóstico y terapéuticos en los registros clínicos de los servicios de cirugía torácica, otorrinolaringología y neumología. Resultados: Se evaluaron 52 pacientes adultos con intubación traqueal prolongada que desarrollaron estenosis traqueal. La mayoría son hombres jóvenes con mediana de intubación de dos semanas, la indicación de intubación más frecuente fue por trauma craneoencefálico severo. La forma de diagnóstico más frecuente fue clínico seguido por radiografía y tomografía teniendo en su mayoría estenosis tipo I y II. La mayoría de los pacientes con estenosis traqueal son tratados de manera quirúrgica, comúnmente con traqueostomía, dos semanas después del primer día de intubación. La única variable asociada al tipo de tratamiento fue que se le realizara al paciente una traqueotomía, la cual fue la forma de tratamiento quirúrgico de la mayoría de los pacientes para la corrección de la estrechez traqueal (p=0.01). Conclusiones: el tiempo de intubación endotraqueal es determinante para el desarrollo de la estenosis traqueal. En este estudio se documentaron 52 pacientes que recibieron intubación traqueal prolongada y desarrollaron estenosis traqueal tras una mediana de intubación de dos semanas, lo cual deberá hacernos reflexionar sobre las prácticas y guías para implementar la realización de traqueostomías tempranas en pacientes ventilados después de 7 días (AU)

Tracheal stenosis is the decrease in the caliber of the laryngeal and tracheal lumen as a result of the maturation of scar tissue due to ischemic injury that the balloon of the endotracheal tube produces on the mucosa of the laryngo-tracheal wall when it is insufflated above capillary pressure (20-30 mm Hg) for an even short period. The American Association for Respiratory Care recommends that intubation be used for those patients who require mechanical ventilation for 7-10 days or less and tracheostomy for those patients who require ventilation for longer. Objective: To characterize tracheal stenosis due to prolonged intubation. Methodology: A descriptive, retrospective study was carried out that included adult patients with a diagnosis of tracheal stenosis due to intubation greater than 7 days at the San Juan de Dios General Hospital from january 2016 to december 2019. Epidemiological, clinical, diagnostic and therapeutic data were evaluated in the clinical records of the thoracic surgery, otorhinolaryngology and pulmonology services. Results: 52 adult patients with prolonged tracheal intubation who developed tracheal stenosis were evaluated. Most are young men with a median intubation of two weeks, the most frequent indication for intubation was for severe head trauma. The most frequent form of diagnosis was clinical followed by radiography and tomography, mostly type I and II stenosis. Most patients with tracheal stenosis are treated surgically, commonly with a tracheostomy, two weeks after the first day of intubation. The only variable associated with the type of treatment was that the patient underwent a tracheostomy, which was the form of surgical treatment for most patients to correct the tracheal narrowing (p = 0.01). Conclusions: endotracheal intubation time is decisive for the development of tracheal stenosis. In this study, 52 patients who received prolonged tracheal intubation and developed tracheal stenosis after a median intubation of two weeks were documented, which should make us reflect on the practices and guidelines for implementing early tracheostomies in patients ventilated after 7 days

Pólipo piloso nasofaríngeo en un recién nacido con obstrucción intermitente de la vía aérea. Presentación de un caso / Nasopharyngeal hairy polyp in a newborn with intermittent airway obstruction. Case report

Los pólipos pilosos nasofaríngeos son tumores benignos poco frecuentes. Se presenta el caso de esta patología en una paciente recién nacida, quien presentó cianosis y dificultad respiratoria por obstrucción de la vía aérea superior, durante las primeras 24 horas de vida. La paciente requirió maniobras de reanimación e intubación endotraqueal. Estudios diagnósticos confirmaron la presencia de una masa en la pared lateral de la faringe. Se realizó la extirpación quirúrgica exitosa con evolución satisfactoria de la paciente

Nasopharyngeal hairy polyps are rare benign tumors. We present a newborn case with a hairy polyp mass causing cyanosis and respiratory distress due to obstruction of the upper airway during the first 24 hours of life. The patient required resuscitation and endotracheal intubation. Diagnostic studies confirmed the presence of a mass in the lateral pharyngeal wall. Surgical treatment and removal of the mass was performed with satisfactory evolution of the patient

Metahemoglobinemia secundaria a inhalación de "poppers": caso clínico y revisión de la literatura / Methemoglobinemia secondary to amyl nitrite inhalation. A case report and literature review

El nitrito de amilo, conocida como "poppers", se ha masificado como droga recreacional en parte por sus efectos con objetivos sexuales. Su consumo se asocia a complicaciones psiquiátricas y médicas. Reportamos el caso de un paciente que se presenta con metahemog-lobinemia moderada secundaria a la inhalación de nitrito de amilo asociado a alcohol. Al ingreso presenta cianosis peribucal y en extremidades, disociación entre oximetría de pulso y presión parcial de oxígeno en gases arteriales, además de metahemoglobinemia 29,9%. Se descartan otras intoxicaciones y causas primarias de metahemoglobinemia. Se maneja con oxigenoterapia, hidratación y ácido ascórbico, presentando una evolución favorable. Presentamos el primer caso en Chile de metahemoglobinemia secundaria al consumo de nitrito de amilo con fines recreativos.

Amyl nitrite, known as "poppers", has become popular as a recreational drug for sexual purposes. Its consumption is associated with psychiatric and medical complications. We report the case of a patient presenting with moderate methemoglobinemia secondary to amyl nitrite inhalation associated with alcohol. At admission, perioral and extremities cyanosis, a dissociation between pulse oximetry and partial pressure of oxygen in arterial gases, and methemoglobinemia 29.9% were present. Other intoxications and primary causes of methemoglobinemia were ruled out. The patient receives oxygen therapy, hydration, and ascorbic acid, presenting a favorable evolution. We report the first Chilean's case of methemoglobinemia secondary to amyl nitrite consumption for recreational purposes

Anesthetic management for patient with severe cyanosis following bioprosthetic valve stenosis / Manejo anestésico de paciente com cianose grave após estenose de valva bioprotética

Abstract We presented a 39-year-old female patient with life-threatening hypoxemia after tricuspid valve replacement because of Ebstein's anomaly. And the severe cyanosis is due to bioprosthetic valve stenosis and atrial septal defect. Anesthetic management of a patient with severe obstructive prosthetic valve dysfunction can be challenging. Similar considerations should be given to patients with Ebstein's anomaly to maintain the pressure equalized between the right and left atrial. Transesophageal echocardiography and cerebral oxygen saturation provided real time information in perioperative care.

Resumo Apresentamos o caso de uma paciente de 39 anos, com hipoxemia em risco de vida após a substituição da valva tricúspide devido à anomalia de Ebstein e cianose grave devido à estenose de valva bioprotética e comunicação interatrial. O manejo anestésico de um paciente com disfunção obstrutiva grave de prótese valvar pode ser um desafio. Os pacientes com anomalia de Ebstein também precisam de atenção especial para manter a pressão equalizada entre o átrio direito e o esquerdo. A ecocardiografia transesofágica e a saturação cerebral de oxigênio forneceram informações em tempo real nos cuidados perioperatórios.

Hemodynamic, metabolic and ventilatory responses to exercise in adults with congenital heart disease / Respostas hemodinâmicas, metabólicas e ventilatórias ao exercício em adultos com cardiopatia congênita

Background: Congenital heart disease in adults shares some features with heart failure (HF), including exercise intolerance, ventilatory inefficiency, inflammatory and neurohormonal activation, cardiac arrhythmias and myocardial fibrosis. Over the last years, cardiopulmonary exercise test has gained importance in the diagnostic and prognostic evaluation of congenital heart diseases, as has already occurred in HF. Objective: To describe the behavior of hemodynamic, metabolic and ventilatory parameters in response to exercise in adults with congenital heart disease. Methods: Observational cross-sectional study evaluating 31 adults with congenital acyanotic or cyanotic heart disease, treated clinically, surgically or percutaneously, referred for cardiopulmonary exercise test. A descriptive analysis of the data was performed. Results: Patients aged 35.7 ± 14.2 years were included. Oxygen consumption (VO2) was 44.86 ± 18.01% of predicted at peak exercise and 36.92 ± 12.93% of predicted maximal VO2 at anaerobic threshold. We found an oxygen uptake efficiency slope (OUES) of 1.49 ± 0.89 (61.43 ± 26.63% of predicted), oxygen pulse of 58.90 ± 22.24% and increment in systolic arterial pressure during exercise was 31.42 ± 21.60 mmHg. Conclusion: Adults with congenital heart disease had similar responses to heart failure patients during exercise ­ reduced aerobic capacity, ventilatory inefficiency for oxygen consumption and limited inotropic response to exercise, characterized by reduced oxygen pulse and small increase in systolic arterial pressure

Metahemoglobinemia inducida por dapsona: presentación de un caso pediátrico / Methemoglobinemia due to Dapsone: a pediatric case report

La metahemoglobinemia es una patología caracterizada por la presencia de altas concentraciones de metahemoglobina en sangre. Esta es una forma oxidada de la hemoglobina, muy afín al oxígeno, que es incapaz de cederlo a los tejidos. Es una entidad poco frecuente, con baja sospecha diagnóstica. Aunque puede ser congénita en recién nacidos con cianosis, es más frecuente la adquirida por fármacos y tóxicos. En la Argentina, no se conoce la incidencia real de esta patología. El objetivo es comunicar un caso de metahemoglobinemia en una paciente pediátrica que ingresó al Hospital Magdalena V. de Martínez con cianosis en la cara y las extremidades, en mal estado general, con el antecedente de ingesta de varios comprimidos de dapsona, y se constató concentración sérica de metahemoglobina del 35%. El tratamiento consistió en la administración endovenosa de azul de metileno. Su evolución fue favorable.

Methemoglobinemia is a condition characterized by a high blood concentration of methemoglobin. Methemoglobinemia is a disorder that occurs when hemoglobin in the blood is oxidized to form methemoglobin, rendering it unable to transport oxygen. Although it can be congenital in cyanotic newborn, it is more often an adverse medication effect. The aim is to report a pediatric methemoglobinemia case, assisted in Magdalena V. de Martínez Hospital, with cyanosis in face and limb, in poor condition, that consumed dapsone accidentally. Her methemoglobin concentration was 35%. Intravenous methylene blue was administered with favorable outcome.

Cianosis en un paciente de 14 años: Presentación de un caso de metahemoglobinemia / Cyanosis in 14-year-old patient: Methemoglobinemia: case report

La cianosis es la coloración azulada de la piel y las mucosas debida al aumento de la concentración de hemoglobina reducida en los capilares o a la presencia de metahemoglobina en concentraciones mayores de las normales. Es importante pensar en metahemoglobinemia como diagnóstico diferencial frente a un paciente con cianosis que no responde a la administración de oxígeno cuando no existen causas cardiorrespiratorias que la justifiquen, dado que requiere de otros métodos diagnósticos y de tratamiento específico. Se presenta el caso de un paciente adolescente de 14 años de edad con cianosis secundaria a metahemoglobinemia de probable etiología congénita. Se discuten las causas, forma de presentación, diagnóstico y tratamiento.

The bluish coloration of skin and mucous membranes, called as cyanosis, could be explained by high reduced hemoglobin in the capillaries, or the presence of elevated methemoglobin concentration. It is important to think of methemoglobinemia as a differential diagnosis in a cyanotic patient who does not respond to oxygen administration once cardiorespiratory causes are discarded; since it requires other diagnostic methods and specific treatment. We described a case of cyanosis in a fourteen-year-old adolescent with probable congenital methemoglobinemia. We discussed their probable causes, clinic presentation, diagnosis and treatment.

Malformación de Abernethy: causa inusual de cianosis central en pediatría / Abernethy malformation: unusual cause of central cyanosis in pediatrics

El shunt porto sistémico congénito es una causa rara de hipoxemia y una patología muy poco frecuente con complicaciones severas si no es tratada. Fue descrito por primera vez por John Abernethy en 1793. Existen dos tipos: tipo I (shunt termino lateral) en el que hay ausencia total del flujo portal intrahepático y tipo II (shunt latero lateral) con flujo portal parcialmente conservado. Se presenta el caso de una niña de 6 años de edad con antecedente de hipoxemia crónica desde los 4 años y medio de vida, acompañado de disnea progresiva, quien fue referida a la unidad de neumología pediátrica con diagnóstico de cianosis central. Entre los estudios diagnósticos considerados se realizó ecografía doppler del sistema venoso portal, evidenciándose ausencia de vena porta principal; además se realizó angiotomografía del sistema arterio-venoso portal y mesentérico, confirmándose la agenesia de vena porta. Se completó el estudio con una porto-esplenografía que confirmó el diagnóstico de malformación de Abernethy tipo I b. La malformación de Abernethy tipo I es más frecuente en el sexo femenino, tiene varias formas de presentación y el tratamiento es el trasplante hepático. En la malformación de Abernethy tipo II la circulación portal es variable y tiene mejor pronóstico que la de tipo I. La disnea al ejercicio y la cianosis central es una forma de presentación que debemos tener en cuenta en el diagnóstico diferencial de la patología cardiorrespiratoria en la edad pediátrica.

The congenital portosystemic shunt is an uncommon disease with severe complications if not treated. This rare cause of hypoxemia was first described by John Abernethy in 1973. There are two types: type I (termino-lateral shunt), in which there is total absence of the intrahepatic portal flow, and type II (latero-lateral shunt), in which the portal flow is partially preserved. We present the case of a 6-year-old girl with chronic hypoxemia history since 4 and a half years of age, showing progressive dyspnea, who was referred to the Pediatric Pulmonary Division with the diagnosis of central cyanosis. An Echo-Doppler in the portal venous system was performed, reporting agenesis of the principal portal vein. This finding was corroborated by an angiography of the portal and mesenteric arteriovenous system. The study was completed with a portosplenography, which confirmed the diagnosis of type Ib Abernethy malformation. The type I Abernethy malformation is more common in females, shows up in different ways and is treated with liver transplantation. On the other hand, type II Abernethy malformation shows a variable portal circulation and has a better prognosis than type I. Dyspnea when exercising and central cyanosis should be considered to make a differential diagnosis of cardiorespiratory disease at a pediatric age.

Análisis factorial exploratorio de una escala de gravedad de obstrucción bronquial evaluada en menores de tres años, Chile, 2015 / Exploratory factor analysis of a score for grading the severity of bronchial obstruction evaluated in children younger than 3 years old, Chile, 2015

Introducción. La escala de Tal modificada es utilizada en Chile para determinar la gravedad de la obstrucción bronquial. Existen informes de su validez; sin embargo, carece de antecedentes del análisis de su estructura como escala para cuantificar de forma significativa la gravedad de la obstrucción bronquial. Objetivo. Identificar el patrón de relación entre los ítems de la escala y la dimensión gravedad de la obstrucción bronquial en una muestra de menores de tres años que consultan en dos centros de salud de la provincia de Concepción, Chile. Método. Análisis factorial exploratorio. Selección no aleatoria y voluntaria de participantes, menores de 36 meses, agosto-noviembre de 2015. Evaluados de manera independiente por médico y kinesiólogo, en tiempo seguido. Se aplican medidas de tendencia central, análisis factorial exploratorio, por subgrupo de menores y mayores de 6 meses, y consistencia interna. Resultados. 204 mediciones a 102 menores. Con una varianza explicada de 39%, los ítems se dirigen a dos conceptos diferentes, un factor que agrupa frecuencia respiratoria, sibilancia y retracción, y otro que solo lo determina cianosis, con alfa de Cronbach de 0, 5. Al extraer cianosis del análisis, muestra matriz monofactorial con 38% de varianza total explicada y consistencia interna de 0, 62. Conclusiones. Por medio de sus ítems, la estructura de la escala no se ajusta adecuadamente para esta muestra. Cianosis se muestra como un ítem disociado en la estructura. Al ajustar el modelo a solo tres ítems, la estabilidad del alfa como medida de consistencia interna aumenta, pero a un valor cuestionable, lo que, sin duda, variará al ser aplicada en otra muestra.

Introduction. The modified Tal's score has been used in Chile to determine the severity of bronchial obstruction. Its validity has been reported already; however, its structure as a scale to significantly quantify the severity of bronchial obstruction has not been analyzed. Objective. To identify the relationship pattern among the score's items and the severity of bronchial obstruction in a sample of children younger than 3 years seen in two health care centers in the province of Concepción, Chile. Method. Exploratory factor analysis. Nonrandom, voluntary selection of participants youngerthan36months, August-November 2015. Participants were assessed independently and subsequently by a physician and a physical therapist. The following measurements were applied: central tendency, exploratory factor analysis, subgroups of participants younger and older than 6 months, and internal consistency. Results. A total of 204 measurements were done in 102 children. With a 39% explained variance, items incline towards to two different concepts: one factor groups respiratory rate, wheezing, and retractions, and the other determines only cyanosis, with a Cronbach's alpha of 0.5. Once cyanosis is removed from the analysis, the original one-factor matrix sample shows a 38% total explained variance and a 0.62 internal consistency. Conclusions. Based on its items, the score's structure does not fit adequately to this sample. Cyanosis appears as a dissociated item within the structure. Once the model is adjusted to only 3 items, the stability of Chronbach's alpha as a measure of internal consistency increases but at an objectionable value, which will undoubtedly vary once it is applied to a different sample.

Metahemoglobinemia relacionada con ingesta de puré de acelgas / Methemoglobinemia due to ingestion of chard puree

La metahemoglobinemia es un síndrome clínico dado por la presencia de una forma aberrante de hemoglobina, ocasionada por diversos agentes oxidantes. Se describe un caso clínico de metahemoglobinemia severa asociada a la ingesta de puré de acelgas con alto contenido en nitratos y nitritos. Paciente de un año, con antecedentes de comunicación interauricular (CIA), que presentó un cambio en coloración de la piel 7 h antes, en forma progresiva, acompañado de vómitos. Ingresó al Departamento de Emergencia con cianosis generalizada que no mejoró con oxigenoterapia, taquicardia y tendencia a hipotensión arterial. En cuidados intensivos se realizó ecocardiograma que evidenció CIA sin repercusión hemodinámica. Metahemoglobinemia 37%. Se realizó dosis de azul de metileno al 1% por vía intravenosa, con franca mejoría clínica a la hora de la administración del antídoto y descenso de niveles de metahemoglobina. Alta médica a las 36 horas del ingreso. Existía una relación cronológica entre la exposición a nitratos por ingesta de un puré de acelgas y la aparición del cuadro. Los niveles de nitratos hallados en dicho alimento fueron muy elevados considerando estándares internacionales, lo que sumado a una inadecuada conservación del alimento cocido los días previos, permitió confirmar el planteo etiológico realizado. Resulta importante sospechar esta entidad patológica poco frecuente frente a cianosis que no mejora con oxígeno, y prevenir cuadros similares al descrito mediante una adecuada manipulación y conservación de las verduras con alto contenido en nitratos.

Methemoglobinemia is a clinical syndrome due to the presence of an aberrant form of hemoglobin, caused by various oxidizing agents. The study reports a case of severe methemoglobinemia associated with the ingestion of chard puree with high levels of nitrates and nitrites. A 1-year-old patient with a history of atrial septal defect (ASD), who progressively showed change of skin color 7 hours earlier, accompanied by vomiting. She was admitted to the Emergency Department with generalized cyanosis not improving with oxygen therapy, tachycardia and tendency to hypotension. In the intensive care unit, an echocardiogram showed ASD without hemodynamic complications. Methemoglobinemia 37%. A 1% methylene blue dose was administered intravenously, with clinical improvement one hour after antidote administration and decrease in methemoglobin levels. Medical discharge at 36 hours of admission. There was a chronological relationship between nitrates exposure by ingestion of chard puree and the clinical onset of methemoglobinemia. The toxic cause was confirmed after high nitrates levels were found in this vegetable considering international standards, and an inadequate preservation of the cooked chard on previous days. It is important to suspect this rare pathological entity when cyanosis fails to improve with oxygen, and to prevent poisonings similar to those described by an adequate manipulation and preservation of vegetables with high nitrate levels.

Fenómeno de Raynaud asociado a ácido nítrico: reporte de un caso / Raynaud's phenomenon associated with nitric acid: Case report

RESUMEN El fenómeno de Raynaud es un trastorno clínico, caracterizado por ataques episódicos de vasoespasmo de arterias y arteriolas periféricas, causando así isquemia tisular de porciones distales del cuerpo, como manos y pies, produciendo cambios en la coloración de la piel como: palidez, cianosis e hiperemia. Se ha asociado la aparición del fenómeno de Raynaud en personas que manipulan solventes orgánicos o herramientas vibratorias por un largo período, incluso se ha descrito que los solventes pueden ser gatilladores de algunas enfermedades inmunológicas como la esclerodermia, sin embargo, hasta donde conocemos no existen reportes de la exposición de compuestos químicos utilizados en la metalurgia, como el ácido nítrico y el desarrollo de enfermedades de espectro esclerodérmico. En el presente artículo se presenta un caso clínico relacionado con la aparición de fenómeno de Raynaud frente a la exposición a compuestos químicos utilizados en la metalurgia, en especial al ácido nítrico.

ABSTRACT The Raynaud phenomenon is a clinical disorder, characterized by episodic attacks of vasospasm of peripheral arteries and arterioles, causing tissue ischemia of distal portions of the body, such as hands and feet, causing changes in skin color such as pallor, cyanosis and hyperemia. The occurrence of the Raynaud phenomenon in people who handle organic sol vents or vibratory tools has been associated for a long time, and it has even been described that the solvents may be triggering some immune diseases such as scleroderma, however, as far as we know there are reports of exposure of chemical compounds used in metallurgy, such as nitric acid and the development of scleroderma-spectrum diseases. This article presents a clinical case related to the appearance of Raynaud s phenomenon against exposure to chemical compounds used in metallurgy, especially nitric acid.

Drenaje pulmonar anómalo total con drenaje de pulmonares a vena porta / Total anomalous pulmonary drainage with drainage

Las anomalías de drenajes de las venas pulmonares se deben a una alteración precoz en el desarrollo embrionario de las venas pulmonares (derechas o izquierdas) mientras están presentes aún conexiones con venas sistémicas (cardinal, umbilicovitelinas). La conexión anómala total de las venas pulmonares constituye entre 0.4 y 2.0% de las cardiopatías congénitas: ocurre en 6.8 de cada 100,000 individuos. Se diagnostica en 68% de los pacientes en etapa neonatal, lo que refleja la gravedad de esta cardiopatía. La variedad de conexión anómala total de las venas pulmonares infracardiaca representa entre 15 y 26% de todas sus variedades.

The anomalies of pulmonary venous drainage due to alteration in early embryonic development of the pulmonary veins (right or left) while still connections are present with systemic veins (cardinal, umbilicovitelins). These malformations represent 0.4 to 2.0 % of all congenital heart diseases, with an incidence of 6.8 patients in every 100,000 births. Sixty eight percent of the cases are diagnosed in newborns, which indicates that most patients are symptomatic early in life. The infracardiac TAPVC represent 15 to 25% of all TAPVC.

Metahemoglobinemia adquirida inducida por fármacos antipalúdicos / Acquired methemoglobinemia induced by antymalaric drugs

Clásicamente se describe cianosis como niveles de hemoglobina desoxigenada en sangre periférica por encima de 5g/dl; sin embargo se ha dejado a un lado, que dicha expresión clínica no sólo responde a esta condición, sino también a la acumulación distal de pigmentos anómalos producto de la alteración en la morfología habitual de la molécula de hemglobina o el estado químico de ésta. Por tal motivo, se presenta un interesante caso clínico en paciente masculino de 35 años de edad, quien consulta por cuadro febril agudo y diagnóstico de Paludismo por Plasmodium vivax, iniciando tratamiento antipalúdico. Posteriormente presenta, en ausencia de fiebre, cianosis peribucal y acral acompañada de disminución de la saturación a 83% por oximetría de pulso. El diagnóstico se basó en la evidencia clínica y la oximetría de pulso, el manejo terapéutico consistió en la administración de oxígeno húmedo y bolos de azul de metileno, notando franca mejoría clínica(AU)

Methemoglobinemia has traditionally been described in multiple studies. Cyanosis, and deoxygenated hemoglobin levels in peripheral blood above 5g / dL, not only responds to this condition, but also to the distal accumulation of anomalous pigments as products of an alteration in the normal morphology of the haemoglobin molecule or chemical state of this. An interesting case report is presented in a male, who after the diagnosis of Plasmodium Vivax Malaria and malaria treatment presenteded perioral the and distal cyanosis, as well as desaturation of 83% by oximetry. The diagnosis was based on clinical evidence and pulse oximetry, and therapeutic approach was based on the administration of wet oxygen and methylene blue bowling, showing marked clinical improvement(AU)

Factores de riesgo asociados a complicaciones por Bordetella pertussis en la Fundación Hospital de la Misericordia, 2009-2013 / Risk factors associated with complications of Bordetella pertussis at the Fundacion Hospital de la Misericordia, 2009-2013

Objetivo: Caracterizar factores de riesgo asociados a complicaciones en los pacientes con tosferina en 2009-2013. Materiales y métodos: Estudio de casos y controles. Identificación de factores de riesgo asociados a complicaciones en pacientes con Bordetella pertussis , confirmados con reacción en cadena de la polimerasa. Resultados: Se caracterizó a 96 pacientes. Un total de 76% eran menores de 6 meses, media de 4,56 ± 6,8 meses. El 58,3% eran niños y el 41,5%, niñas. Un 51% tuvo alguna complicación. Al 58% se les hospitalizó en cuidado crítico. Un 9,4% tuvo sepsis y el 14,6% requirió ventilación asistida. Un 4,2% fallecieron. La media de estancia en cuidado crítico fue de 8,75 días ( ± 85) y hospitalaria de 13,92 días ( ± 12,81). Se identificaron los siguientes factores de riesgo asociados a complicación: noción de contagio (OR: 2,20), ausencia de vacunación (OR: 3,31). Clínica: cianosis (OR: 4,07), deshidratación (OR: 2,72), taquicardia (OR: 2,64), taquipnea (OR: 3,94), desaturación (OR: 3,34). Paraclínica: coinfección bacteriana (OR: 7,66), linfocitosis (OR: 3,18), trombocitosis (OR: 3,038). Conclusión: Los antecedentes, clínica y paraclínicos pueden definir pacientes con infección por Bordetella pertussis en riesgo de complicaciones e ingreso en cuidado crítico.

Objective: To characterize the risk factors associated with complications in patients infected with Bordetella pertussis , 2009-2013. Material and methods: Case-control study. Identification of risk factors associated with complications in patients with Bordetella pertussis , confirmed by polymerase chain reaction. Results: A total of 96 patients were characterized. 76% were younger than 6 months, mean 4.56 ± 6.8 months; there were 58.3% boys and 41.5% girls. A total of 51% had complications. Some 58% were hospitalized in critical care. Sepsis occurred in 9.4% patients, and 14.6% required assisted ventilation. A total of 4.2% died. The mean critical care stay was 8.75 days ( ± 85) and the mean hospital stay was 13.92 ± 12.81 days. The following risk factors associated with complications were identified: notion of contagion (OR: 2.20), no vaccination (OR: 3.31). Clinical: (OR: 4.07) cyanosis, dehydration (OR: 2.72), tachycardia (OR: 2.64), tachypnea (OR: 3.94), desaturation (OR: 3.34). Paraclinical: Bacterial co-infection (OR: 7.66), lymphocytosis (OR: 3.18), thrombocytosis (OR: 3.038). Conclusion: Background, clinical and paraclinical factors can identify patients with B. pertussis infection at risk for complications and admission to the critical care unit.

Cardiopatias congênitas em adultos: um problema em ascensão / Congenital heart disease in adults: an increasing problem

A população de adolescentes e adultos com cardiopatia congênita é crescente graças às grandes inovações tecnológicas que permitiram avanços nos métodos diagnósticos, melhor conhecimento das cardiopatias congênitas, melhoria do tratamento clínico, surgimento da terapêutica intervencionista percutânea, aperfeiçoamento dos cuidados intensivose dos resultados cirúrgicos, aumentando assim a sobrevida destes pacientes. Algumas cirurgias apresentam resultados excelentes, enquanto outras, usualmente em portadores de cardiopatia congênita complexa, podem evoluir com lesões residuais importantes que ainda necessitarão de tratamento clínico e/ou cirúrgico ou pela hemodinâmica durante o seguimento. Entre outro grupo de pacientes, estão os pacientes não operados, que são diagnosticados ou encaminhados aos serviços especializados tardiamente. Alguns destes apresentam hipertensão pulmonar importante, ou já desenvolveram a síndrome de Eisenmenger e, portanto são inoperáveis, enquanto outros que se apresentam com cardiopatia como a comunicação interatrial, que costuma ser pouco sintomática nas primeiras duas décadas de vida, ou cardiopatias complexas balanceadas por algum grau de estenose pulmonar, que ainda poderão ser operados, mas sem a certeza do benefício da correção, pois estão predispostos a apresentar arritmia cardíaca e disfunção ventricular. Para que se possa oferecer a melhor abordagem, seja ela qual for, para esse grupo de pacientes, devemos continuar a buscar a formação de centros especializados para o atendimento aos adolescentes e adultos com cardiopatia congênita tratada ou não na infância, que cursam com lesões residuais ou hipertensão pulmonar. Além do aspecto técnico, não se pode desconsiderar a qualidade de vida desses pacientes, incluindo aspectos psicossociais e cognitivos.

The adolescent and adult population with congenital heart disease is growing, due to major technological innovations that have led to advances in diagnostic methods, better knowledge of congenital heart disease, improvements in clinical treatment, the emergence of percutaneous interventionist treatment, and the perfection of intensive care and surgical outcomes, all of which have increased the survival of these patients. Some surgical procedures present excellent results, while others, usually in individuals with complex congenitalheart disease, can develop with significant residual lesions that also require clinical and/or surgical treatment, or due to the hemodynamics during follow-up. Among another group ofpatients are those not operated on, who are diagnosed or referred to the specialized services too late. Some of these present significant pulmonary hypertension, or have alreadydeveloped Eisenmenger’s syndrome, and are therefore inoperable, while others presente heart disease such as inter-atrial communication, which generally has few symptoms in the first two decades of life, or complex heart disease balanced by some degree of pulmonarystenosis, who can still be operated on but without certainty of the benefit of correction, as they are predisposed to present cardiac arrhythmia and ventricular dysfunction. In order tooffer the best approach for this group of patients, whatever that may be, we must continue to strive for the formation of specialized centers for the care of adolescents and adults withcongenital heart disease, whether treated or not in infancy, and who also have residual lesions or pulmonary hypotension. In addition to the technical aspect, we must also consider thequality of life of these patients, including the psychosocial and cognitive aspects.

Resurgence of pertussis at the age of vaccination: clinical, epidemiological, and molecular aspects / Ressurgimento da coqueluche na era vacinal: aspectos clínicos, epidemiológicos e moleculares

OBJECTIVE: Report the incidence, epidemiology, clinical features, death, and vaccination status of patients with whooping cough and perform genotypic characterization of isolates of B. pertussis identified in the state of Paraná, during January 2007 to December 2013.METHODS: Cross-sectional study including 1,209 patients with pertussis. Data were obtained through the Notifiable Diseases Information System (Sistema de Informação de Agravos de Notificação - SINAN) and molecular epidemiology was performed by repetitive sequence-based polymerase chain reaction (rep-PCR; DiversiLab(r), bioMerieux, France).RESULTS: The incidence of pertussis in the state of Paraná increased sharply from 0.15-0.76 per 100,000 habitants between 2007-2010 to 1.7-4.28 per 100,000 between 2011-2013. Patients with less than 1 year of age were more stricken (67.5%). Fifty-nine children (5%) developed pertussis even after receiving three doses and two diphtheria-tetanus-pertussis (DTP) boosters vaccine. The most common complications were pneumonia (14.5%), otitis (0.9%), and encephalopathy (0.7%). Isolates of B. pertussis were grouped into two groups (G1 and G2) and eight distinct patterns (G1: P1-P5 and G2: P6-P8).CONCLUSION: The resurgence of pertussis should stimulate new research to develop vaccines with greater capacity of protection against current clones and also encourage implementation of new strategies for vaccination in order to reduce the risk of disease in infants.

OBJETIVO: Relatar a incidência, os aspectos epidemiológicos, clínicos, a morte e a vacinação de pacientes com coqueluche e fazer a caracterização genotípica de isolados de Bordetella pertussisidentificados no Estado do Paraná, de janeiro de 2007 a dezembro de 2013.MÉTODOS: Estudo transversal, incluindo 1.209 pacientes com coqueluche. Os dados foram obtidos no Sistema de Informação de Agravos de Notificação (Sinan) e a epidemiologia molecular foi feita por PCR baseada em sequências repetitivas (rep-PCR; DiversiLab(r), bioMerieux, France).RESULTADOS: A incidência de coqueluche no Estado do Paraná aumentou acentuadamente de 0,15-0,76 por 100.000 habitantes entre 2007-2010 para 1,7-4,28 por 100.000 habitantes entre 2011-2013. Os pacientes com menos de um ano foram os mais afetados (67,5%); 59 crianças (5%) desenvolveram coqueluche mesmo depois de receber três doses da vacina e dois reforços com a vacina tríplice DTP. As complicações mais comuns foram pneumonia (14,5%), otite (0,9%) e encefalopatia (0,7%). Isolados de B. pertussis foram agrupados em dois grupos (G1 e G2) e oito padrões distintos (G1: P1-P5 e G2: P6-P8).CONCLUSÃO: O ressurgimento da coqueluche vem para sugerir novas pesquisas com o objetivo se desenvolverem vacinas com maior capacidade de proteção contra os clones atuais e também implantar novas estratégias de vacinação, a fim de reduzir o risco de doenças em lactentes.

Vía aérea difícil, caso clínico / Difficult airway: clinical case

Introducción: la American Society of Anesthesiologists definió la vía aérea difícil como la situación clínica en la que un anestesiólogo con entrenamiento convencional experimenta dificultad para ventilar con máscara facial, dificultad para la intubación endotraqueal o ambas. Ventilación difícil con máscara es 1) la imposibilidad para un anestesiólogo que no cuenta con ayuda, de mantener la saturación de oxígeno (SpO2) > 90% al administrar oxígeno al 100% y ventilar con presión positiva mediante máscara facial a un paciente cuya SpO2 era del 90% antes de la intervención anestésica o 2) la imposibilidad para un anestesiólogo que no cuenta con ayuda, de evitar o revertir los signos de ventilación inadecuada durante la ventilación con presión positiva mediante máscara facial. Estos signos incluyen: cianosis, ausencia de movimientos del tórax, signos auscultatorios de obstrucción severa de la vía aérea, distensión gástrica por entrada de gas y cambios hemodinámicos secundarios a hipoxemia e hipercapnia. Laringoscopia difícil: imposibilidad para visualizar alguna parte de la glotis durante la laringoscopia convencional. Intubación difícil: situación en la que se requieren más de tres intentos o más de diez minutos para la inserción adecuada del tubo endotraqueal mediante laringoscopia convencional. Materiales y métodos: es una revisión de caso clínico y revisión bibliográfica. Resultados: el presente caso ilustra una de las situaciones de vía aérea difícil prevista producidas por patología de vía aérea y mediastino anterior como son las masas tiroideas. Presentación poco frecuente y que implica un manejo anestésico complejo. Conclusiones: podemos concluir que el manejo de una vía aérea difícil anticipada o no es un reto para el anestesiólogo y requiere el entrenamiento y actualización constante del mismo.

Introduction: the ASA define difficult airway as the clinical situation in which a conventionally trained anesthesiologist experiences difficulty with face mask ventilation, endotracheal intubation difficulty , or both. Difficult mask ventilation is 1) the impossibility for an anesthesiologist who has no help to maintain oxygen saturation (SpO2) > 90 % when using 100% oxygen and positive pressure ventilation by face mask to a patient whose SpO2 was 90 % before anesthetic intervention or 2) the impossibility for an anesthesiologist who has no help prevent or reverse signs of inadequate ventilation during positive pressure ventilation by face mask . These signs include cyanosis, absence of chest movement, auscultation signs of severe airway obstruction, gastric distension by gas inlet and hypoxemia and hypercapnia secondary to hemodynamic changes. Difficult laryngoscopy: unable to display some part of the glottis during conventional laryngoscopy. Difficult intubation: a situation in which more than three attempts or more than ten minutes for the proper insertion of the endotracheal tube with conventional laryngoscopy is required.1 Materials and methods: this is a clinical case review and a literature review. Results: this case illustrates one of the difficult airway situations envisaged produced by airway pathology and anterior mediastinal masses such as thyroid. Rare presentation and involves a complex anesthetic management. Conclusions: we can conclude that the difficult airway management anticipated or not, is a challenge for the anesthesiologist and requires constant training and update.