Taquipnea persistente como manifestación inicial de hiperplasia de células neuroendocrinas: reporte de un caso clínico / Persistent tachypnea as initial manifestation of hyperplasia of neuroendocrine cells: case report

Se realiza revisión de la literatura y presentación de un caso clínico de Hiperplasia de Células Neuroendocrinas en paciente lactante masculino que inicia su padecimiento a los 3 meses de vida con dificultad respiratoria caracterizada por retracciones subcostales y taquipnea persistente, posterior-mente a los 8 meses de edad se agrega hipoxemia respirando aire ambiente que requiere uso de oxígeno suplementario continuo. Tiene antecedente de tres hospitalizaciones, con diagnóstico de Bronquiolitis y Neumonía atípica, realizándose panel viral respiratorio con reporte negativo. El paciente persiste con sintomatología respiratoria a pesar de tratamientos médicos, por lo que se deriva a neumología pediátrica, unidad de enfermedad pulmonar intersticial del lactante, iniciando protocolo de estudio, se realiza tomografía tórax de alta resolución, que evidencia imágenes en vidrio despulido en lóbulo medio y región lingular, además de atrapamiento aéreo. Se concluye el diagnóstico de Hiperplasia de Células neuroendocrinas con base a la clínica y hallazgos tomográficos. La Hiperplasia de Células Neuroendocrinas es una patología pulmonar intersticial poco frecuente, cuyo diagnóstico es clínico y radiológico, en la minoría de los casos se requiere biopsia pulmonar para confirmación. Puede ser fácilmente confundida con otras enfermedades respiratorias comunes, por lo que es importante sospecharla para realizar un diagnóstico precoz. La mayor parte de los casos evolucionan con declinación de los síntomas, mejorando espontáneamente en los primeros años de vida.

A review of the literature and presentation of a clinical case of Neuroendocrine Cell Hyperplasia in a male infant patient who begins his condition at 3 months of age with respiratory distress characterized by subcostal retractions and persistent tachypnea is presented. After 8 months of age hypoxemia is added requiring continuous oxygen therapy. He has a history of three hospitalizations, with a diagnosis of bronchiolitis and atypical pneumonia, respiratory viral panel has a negative report. The patient persists with respiratory symptoms despite medical treatments, so it is referred to pediatric pulmonology, initiating study protocol for interstitial lung disease of the infant. A high resolution chest tomography is performed, which evidences images in polished glass in the middle lobe and lingular region, in addition to air entrapment. The diagnosis of neuroendocrine cell hyperplasia is concluded based on clinical and tomographic findings. Neuroendocrine Cell Hyperplasia is a rare interstitial pulmonary pathology, whose diagnosis is clinical and radiological. Lung biopsy is required only in the minority of cases for confirming diagnosis. It can be easily confused with other common respiratory diseases, so it is important to suspect it to make an early diagnosis. Most cases evolve with decline in symptoms, improving spontaneously in the first years of life.

Taquipnea persistente de la infancia: hiperplasia de células neuroendocrinas, una revisión / Persistent tachypnea of infancy: neuroendocrine cell hyperplasia, a review

La hiperplasia de células neuroendocrinas de la infancia (HCNEI) constituye una de las enfermedades intersticiales más frecuentes en pediatría. Tanto su etiología como los mecanismos fisiopatológicos involucrados son inciertos. Suele presentarse en pacientes por lo demás sanos, durante los primeros meses de vida con taquipnea, retracciones costales, rales e hipoxemia. En la tomografía axial computada de tórax de alta resolución (TACAR) presenta imágenes características en vidrio esmerilado de distribución central y zonas de atrapamiento aéreo. Para el diagnóstico, además de la clínica y la TACAR, podemos recurrir a la biopsia en casos atípicos. Los hallazgos histológicos reflejan una arquitectura pulmonar normal y un aumento en el número de células neuroendocrinas. El manejo global es con medidas de sostén, ya que no se cuenta con un tratamiento específico. La sintomatología suele mejorar con la edad y el pronóstico es favorable.

Neuroendocrine cell hyperplasia of infancy (NEHI) is one of the most common interstitial lung diseases of childhood. The etiology and pathophysiological mechanisms involved are uncertain. It usually presents in otherwise healthy patients during the first months of life with tachypnea, rib retractions, crackles, and hypoxemia. High-resolution chest computed tomography (HRCT) shows ground-glass opacities of central distribution and areas of air trapping. For diagnosis purposes, in addition to clinical and HRCT features, a lung biopsy is indicated for atypical cases. Histological findings reflect normal architecture and an increased number of neuroendocrine cells. The management consists of supportive and preventive care, since there is no specific treatment. Symptoms usually improve with age and the prognosis is favorable.

Hiperplasia de células neuroendocrinas: reporte de caso / Neuroendocrine cell hyperplasia: case report

A clinical case of Neuroendocrine Cell Hyperplasia is presented with a bibliographic review. An infant patient with respiratory distress syndrome, characterized by nasal flaring, retractions, and tachypnea with temporary resolution with the use of bronchodilators. However, the patient requires oxygen. With complementary examinations (negative viral panel twice) and epidemiology it is classified as a viral Bronchiolitis. Without improvement, extrapulmonar pathologies were suspected, discarding hearth disease, epilepsy, pathological gastroesophageal reflux. New tests were performed to rule out other pathologies, including immunological disorders. Those results were normal, so a high-resolution chest tomography was done which allowed the diagnosis of Neuroendocrine Cell Hyperplasia. During the follow up the child had improved and required oxygen until he was two years old. Neuroendocrine Cell Hyperplasia belongs to a huge group of less common interstitial disorders, which diagnosis is clinical and radiological. It can easily be confused with common respiratory disorders. For this reason, it is important to know about this disease to make an early diagnosis. Most of the cases had a gradual (months to years) improvement.

Se presenta un caso clínico de Hiperplasia de Células Neuroendocrinas y la revisión de la literatura. Paciente lactante menor con cuadro de dificultad respiratoria, caracterizado por aleteo nasal, retracciones y taquipnea persistente acompañada de desaturación. Sin adecuada respuesta al uso de broncodilatadores. Por exámenes complementarios, panel viral negativo en dos ocasiones y epidemiología, se le diagnostica una bronquiolitis viral. Por no presentar mejoría se completan estudios, descartándose neumonía atípica, cardiopatía, epilepsia, reflujo gastroesofágico patológico y compromiso inmunológico. El diagnóstico fue determinado en base a la clínica, junto con imágenes en vidrio esmerilado característicos en lóbulo medio y língula. En su seguimiento mejora paulatinamente, requiriendo soporte de oxígeno hasta los dos años. La Hiperplasia de Células Neuroendocrinas es una patología intersticial pulmonar poco frecuente, cuyo diagnóstico es clínico y radiológico. Puede ser fácilmente confundida con desórdenes respiratorios comunes, por lo que es importante sospecharla para realizar un diagnóstico precoz. La mayor parte de los casos evolucionan con declinación de los síntomas, mejorando espontáneamente en meses o en los primeros años de vida.

Trombo serpiginoso em átrio direito: apresentação atípica de tromboembolismo pulmonar. Relato de caso / Serpiginous thrombus in right atrium: atypical presentation of pulmonary thromboembolism. Case report

O tromboembolismo pulmonar é um grave problema de saúde pública devido ao subdiagnóstico e às elevadas morbidade e mortalidade. Quando a embolia pulmonar é maciça com repercussão hemodinâmica importante e a terapia adequada não ocorre nas primeiras horas, a mortalidade é superior a 85%. Na suspeita clínica de tromboembolismo pulmonar, a avaliação ecocardiográfica pode ter papel fundamental na avaliação da mobilidade e da estrutura do ventrículo direito, presença de hipertensão pulmonar e documentação da presença de trombo. A detecção ecocardiográfica de trombo móvel nas câmaras cardíacas direitas permite identificar um grupo de pacientes de alto risco, com mortalidade muito elevada, quando comparada ao tromboembolismo pulmonar em geral. Além da terapia clínica clássica, com heparinas e trombolíticos, as terapêuticas endovascular e cirúrgica devem ser consideradas e podem contribuir para o prognóstico desses pacientes. Relata-se um caso de uma paciente de 33 anos de idade admitida em uma unidade de emergência da no 8o dia de pós-operatório de apendicectomia, com queixas de dor torácica e dispneia de início súbito. Ecocardiograma transtorácico evidenciou presença de trombo serpiginoso solto em átrio direito, que ocluía intermitentemente a valva tricúspide durante o ciclo cardíaco. Diante das características ecocardiográficas atípicas do trombo e da significativa chance de embolização maciça, optou-se por intervenção cirúrgica de emergência.

Pulmonary thromboembolism is a serious public health problem due to misdiagnosis and high morbidity and mortality. When pulmonary embolism is massive with important hemodynamic repercussion, and the appropriate therapy does not take place in the early hours, mortality is higher than 85%. If there is clinical suspicion of pulmonary thromboembolism, an echocardiographic evaluation may have a key role in the evaluation of mobility and structure of the right ventricle, presence of pulmonary hypertension, and documentation of the presence of thrombus. Echocardiographic detection of mobile thrombus in right cardiac chambers allows the identification of a group of high-risk patients with very high mortality when compared to pulmonary thromboembolism in general . In addition to the classical clinical therapy with heparins and thrombolytics, endovascular and surgical therapy should be considered and may contribute to these patients' prognosis. A case is reported of a 33-year-old female patient admitted to an Emergency Unit at 8th postoperative day (POD) of appendectomy, with complaints of chest pain and dyspnea of sudden onset. Transthoracic echocardiography showed the presence of a floating serpiginous thrombus in the right atrium, which intermittently occluded the tricuspid valve during the cardiac cycle. Due to the atypical echocardiographic features of the thrombus, and significant chance of massive embolization, an emergency surgery was chosen.

Mecanismos fisiopatológicos de taquipnea / Physiopathological mechanisms of tachypnea

Tachypnea is a common symptom in respiratory diseases, generally triggered for metabolic compensation purposes. Its presence results from integrated complex mechanisms, both physiological and pathological; for a good clinical approach, it is indispensable to know these mechanisms.

La taquipnea es la manifestación más común de las enfermedades respiratorias y obedece generalmente a una respuesta de compensación metabólica. Su presencia resulta de mecanismos de integración complejos tanto fisiológicos como patológicos que es necesario conocer para el mejor abordaje clínico de un paciente.

Síndrome de hiperatividade simpática paroxística causada por síndrome da embolia gordurosa / Paroxysmal sympathetic hyperactivity syndrome caused by fat embolism syndrome

RESUMO A hiperatividade simpática paroxística representa uma complicação incomum, com potencial risco à vida, de lesões cerebrais graves, mais comumente de origem traumática. Seu diagnóstico clínico se baseia na manifestação recorrente de taquicardia, hipertensão, diaforese, taquipneia e, às vezes, febre, além de posturas distônicas. Os episódios podem ser induzidos por estímulos ou ocorrer de forma espontânea. É comum que ocorra subdiagnóstico desta síndrome, e o retardamento de seu reconhecimento pode aumentar a morbidade e a incapacidade em longo prazo. Evitar os desencadeantes e a farmacoterapia podem ter muito sucesso no controle desta complicação. A síndrome da embolia gordurosa é uma complicação rara, mas grave, das fraturas de ossos longos. Sinais neurológicos, petéquias hemorrágicas e insuficiência respiratória aguda são as características que constituem seu quadro clínico. O termo "embolia gordurosa cerebral" é estabelecido quando predomina o envolvimento neurológico. O diagnóstico é clínico, porém achados específicos de neuroimagem podem confirmá-lo. As manifestações neurológicas incluem diferentes graus de alteração da consciência, défices focais ou convulsões. Seu tratamento é de suporte, porém são possíveis desfechos favoráveis, mesmo nos casos com apresentação grave. Relatamos dois casos de hiperatividade simpática paroxística após embolia gordurosa cerebral, uma associação muito incomum.

ABSTRACT Paroxysmal sympathetic hyperactivity represents an uncommon and potentially life-threatening complication of severe brain injuries, which are most commonly traumatic. This syndrome is a clinical diagnosis based on the recurrent occurrence of tachycardia, hypertension, diaphoresis, tachypnea, and occasionally high fever and dystonic postures. The episodes may be induced by stimulation or may occur spontaneously. Underdiagnosis is common, and delayed recognition may increase morbidity and long-term disability. Trigger avoidance and pharmacological therapy can be very successful in controlling this complication. Fat embolism syndrome is a rare but serious complication of long bone fractures. Neurologic signs, petechial hemorrhages and acute respiratory failure constitute the characteristic presenting triad. The term cerebral fat embolism is used when the neurological involvement predominates. The diagnosis is clinical, but specific neuroimaging findings can be supportive. The neurologic manifestations include different degrees of alteration of consciousness, focal deficits or seizures. Management is supportive, but good outcomes are possible even in cases with very severe presentation. We report two cases of paroxysmal sympathetic hyperactivity after cerebral fat embolism, which is a very uncommon association.

Precisión de la taquipnea y las retracciones subcostales como signos clínicos para diagnóstico de neumonía adquirida en la comunidad en niños: revisión sistemática y metaanálisis / Identification of tachypnea and subcostal retractions as clinical signs for the diagnosis of community-acquired pneumonia among children: systematic review and meta-analysis

Objetivos. Evaluar si la taquipnea y las retracciones subcostales son predictoras eficientes para el diagnóstico de neumonía adquirida en la comunidad (NAC) en niños. Materiales y métodos. Se revisaron las bases de datos: PubMed, LILACS, The African Journal Database y The Cochrane Central Library. Se incluyeron estudios originales que evaluaron el rendimiento diagnóstico de los criterios clínicos de taquipnea o retracciones subcostales, definidos según los criterios de la Organización Mundial de la Salud (OMS) para el diagnóstico de NAC en niños de hasta cinco años de edad con tos y fiebre. Se estimaron las razones de probabilidades (LR), el odds ratio diagnostico (DOR) y sus respectivos intervalos de confianza al 95% (IC95%) para cada prueba clínica evaluada. Resultados. Se encontraron 975 estudios, incluyendo ocho en la revisión. Se enrolaron 4740 pacientes y analizaron 3584 (75%), de los cuales 916 (19%) tuvieron el diagnóstico de NAC. Al combinar los datos, la taquipnea obtuvo una LR positiva de 3,16, (IC95% 2,11-4,73) y una LR negativa de 0,36 (IC95% 0,23-0,57). El DOR fue de 10,63 (IC95% 4,4-25,66, I2=93%). Al realizar el análisis para retracciones subcostales se obtuvo un LR positivo de 2,49 (IC95% 1,41-4,37) y un LR negativo de 0,59 (IC95% 0,4-0,87). El DOR fue de 5,32 (IC95% 1,88-15,05, I2=89%). Conclusiones. Se puede tomar en cuenta la presencia o ausencia de taquipnea y retracciones subcostales en el diagnóstico de NAC, cabe considerar la incertidumbre relativa en su poder diagnóstico y los LR relativamente modestos. La confianza en estos resultados es baja por la inadecuada calidad de la evidencia en este tema.

Objectives. Determine whether tachypnea and subcostal retractions can be efficient predictors for the diagnosis of Community-Acquired Pneumonia (CAP) among children. Materials and methods. These were the databases used: PubMed, LILACS, The African Journal Database and The Cochrane Central Library. Original studies were included which assessed the diagnostic performance of the clinical criteria for tachypnea or subcostal retraction defined in accordance with the criteria of the World Health Organization (OMS) for CAP diagnosis in children ≤ 5 with cough and fever. The likelihood ratio (LR), the diagnosis odds ratio (DOR), and their respective confidence intervals at 95% (IC95%) were estimated for each clinical test. Results. 975 studies were found, eight were included in the review. 4740 patients were enrolled and 3584 (75%) were analyzed, 916 (19%) of which had a CAP diagnosis. When data were combined, tachypnea had a positive LR of 3.16, (95% CI 2.11-4.73) and a negative LR of 0.36 (95% CI 0.23-0.57). The DOR was 10.63 (95% CI 4.4-25.66, I2=93%). When subcostal retractions were analyzed, a positive LR of 2.49 (95%CI 1.41-4.37) and a negative LR of 0.59 (95%CI 0.4-0.87) were obtained. The DOR was 5.32 (95%CI 1.88-15.05, I2=89%). Conclusions. The presence or absence of tachypnea and subcostal retractions can be used in CAP diagnosis; it is worth considering the relative uncertainty in its diagnostic power and relatively modest LR. The confidence of these results is low due to the inadequate quality of the related evidence.

Recurrent Plastic Bronchitis in a Child with 2009 Influenza A (H1N1) and Influenza B Virus Infection

Plastic bronchitis is an uncommon disorder characterized by the formation of bronchial casts. It is associated with congenital heart disease or pulmonary disease. In children with underlying conditions such as allergy or asthma, influenza can cause severe plastic bronchitis resulting in respiratory failure. A review of the literature showed nine cases of plastic bronchitis with H1N1 including this case. We report a case of a child with recurrent plastic bronchitis with eosinophilic cast associated with influenza B infection, who had recovered from plastic bronchitis associated with an influenza A (H1N1) virus infection 5 months previously. To the best of our knowledge, this is the first case of recurrent plastic bronchitis related to influenza viral infection. If patients with influenza virus infection manifest acute respiratory distress with total lung atelectasis, clinicians should consider plastic bronchitis and early bronchoscopy should be intervened. In addition, management for underlying disease may prevent from recurrence of plastic bronchitis.

Antibiotic treatment schemes for very severe community-acquired pneumonia in children: a randomized clinical study / Esquemas de tratamiento antibiótico para la neumonía extrahospitalaria muy grave en niños: estudio clínico aleatorizado

OBJECTIVE: To compare clinical response to initial empiric treatment with oxacillin plus ceftriaxone and amoxicillin plus clavulanic acid in hospitalized children diagnosed with very severe community-acquired pneumonia (CAP). METHODS: A prospective randomized clinical study was conducted among children 2 months to 5 years old with a diagnosis of very severe CAP in the pediatric ward of São Paulo State University Hospital in Botucatu, São Paulo, Brazil, from April 2007 to May 2008. Patients were randomly divided into two groups by type of treatment: an oxacillin/ceftriaxone group (OCG, n = 48) and an amoxicillin/clavulanic acid group (ACG, n = 56). Analyzed outcomes were: time to clinical improvement (fever and tachypnea), time on oxygen therapy, length of stay in hospital, need to widen antimicrobial spectrum, and complications (including pleural effusion). RESULTS: The two groups did not differ statistically for age, sex, symptom duration before admission, or previous antibiotic treatment. Time to improve tachypnea was less among ACG patients than OCG patients (4.8 ± 2.2 versus 5.8 ± 2.4 days respectively; P = 0.028), as was length of hospital stay (11.0 ± 6.2 versus 14.4 ± 4.5 days respectively; P = 0.002). There were no statistically significant differences between the two groups for fever improvement time, time on oxygen therapy, need to widen antimicrobial spectrum, or frequency of pleural effusion. CONCLUSIONS: Both treatment plans are effective in treating very severe CAP in 2-month-to 5-year-old hospitalized children. The only analyzed outcome that favored amoxicillin/clavulanic acid treatment was time required to improve tachypnea.

OBJETIVO: Comparar la respuesta clínica al tratamiento empírico inicial con oxacilina más ceftriaxona frente a amoxicilina más ácido clavulánico en niños hospitalizados con diagnóstico de neumonía extrahospitalaria muy grave. MÉTODOS: Se llevó a cabo un estudio clínico prospectivo aleatorizado en niños de 2 meses a 5 años de edad con diagnóstico de neumonía extrahospitalaria muy grave en la sala de pediatría del Hospital Universitario del Estado de São Paulo en Botucatu, São Paulo, Brasil, entre abril del 2007 y mayo del 2008. Los pacientes se dividieron aleatoriamente en dos grupos según el tratamiento administrado: un grupo recibió oxacilina/ceftriaxona (n = 48) y otro amoxicilina/ácido clavulánico (n = 56). Los criterios de valoración analizados fueron el tiempo hasta la mejoría clínica (de la fiebre y la taquipnea), el tiempo de administración de oxigenoterapia, la duración de la internación, la necesidad de ampliar el espectro antibiótico y las complicaciones (como el derrame pleural). RESULTADOS: Los dos grupos no presentaban diferencias estadísticas con respecto a la edad, el sexo, la duración de los síntomas antes de la internación o el tratamiento previo con antibióticos. El tiempo hasta la mejoría de la taquipnea fue menor en los pacientes tratados con amoxicilina/ácido clavulánico que en los que recibieron oxacilina/ceftriaxona (4,8 ± 2,2 días frente a 5,8 ±2,4 días, respectivamente; P = 0,028), y también fue menor la duración de la internación (11,0 ± 6,2 días frente a 14,4 ± 4,5 días, respectivamente; P = 0,002). No hubo diferencias estadísticamente significativas entre los dos grupos en relación con el tiempo hasta la mejoría de la fiebre, el tiempo de administración de oxigenoterapia, la necesidad de ampliar el espectro antibiótico ni la frecuencia de derrame pleural. CONCLUSIONES: Ambos esquemas de tratamiento son eficaces para tratar la neumonía extrahospitalaria muy grave en niños de 2 meses a 5 años de edad hospitalizados. El único criterio de valoración analizado que favoreció el tratamiento con amoxicilina/ ácido clavulánico fue el tiempo hasta la mejoría de la taquipnea.