Síndrome hepatopulmonar como causa de hipoxemia en niños con enfermedad hepática / Hepatopulmonary syndrome as a cause of hypoxemia in hepatic diseases in children

Resumen:Introducción: El síndrome hepatopulmonar es una complicación rara caracterizada por enfermedad hepática asociada con dilataciones vasculares intrapulmonares e hipoxemia. La prevalencia reportada en los escasos estudios realizados en niños con cirrosis es del 3-8%. Aunque es una entidad poco frecuente, es de suma importancia que el clínico la reconozca debido a su curso progresivo.Caso clínico: Se presenta el caso de una niña de 8 años de edad con diagnóstico de cirrosis hepática e hipertensión portal, con sintomatología de disnea y cianosis. Al examen físico se encontró desnutrida, con ictericia generalizada, telangiectasias en abdomen, hipocratismo digital severo, acrocianosis, platipnea y ortodeoxia. La gasometría arterial con PaO2 de 59 mmHg. El ecocardiograma con test de suero salino agitado resultó positivo y la gammagrafía de perfusión pulmonar con macroagregados de albúmina reportó cortocircuito derecha a izquierda del 15%, demostrándose así la existencia de shunt intrapulmonar. Se integró el diagnóstico de síndrome hepatopulmonar de severidad grave, y se recomendó trasplante hepático como el único tratamiento eficaz.Conclusiones: En niños con enfermedad hepática que presenten disnea e hipoxemia, y en los que estén en protocolo de trasplante hepático, debe de buscarse intencionadamente el síndrome hepatopulmonar, ya que el pronóstico dependerá del diagnóstico oportuno.

Abstract:Background: Hepatopulmonary syndrome is a rare complication characterized by liver disease associated with hypoxemia and intrapulmonary vascular dilatations. The prevalence reported in the few studies in children with cirrhosis is 3-8%. Although uncommon, it is important for physicians to recognize this condition because of its progressive course.Case report: We report the case of an 8-year-old girl diagnosed with liver cirrhosis and portal hypertension with symptoms of dyspnea and cyanosis. On physical examination the patient was found malnourished with jaundice, telangiectasias in abdomen, severe clubbing, acrocyanosis, platypnea and orthodeoxia; arterial blood gas showed PaO2 of 59 mmHg. Echocardiography with agitated saline test was positive and lung perfusion scan with albumin macroaggregates reported 15% right-to-left short circuit, thus demonstrating the existence of intrapulmonary shunt. Diagnosis of severe hepatopulmonary syndrome was made. Liver transplantation is recommended as the only effective treatment.Conclusions: In children with liver disease presenting dyspnea and hypoxemia and those enrolled in a liver transplant protocol, hepatopulmonary syndrome must be intentionally searched because the prognosis will depend on timely diagnosis.

Tuberculosis perinatal / Perinatal tuberculosis

Introducción: La tuberculosis perinatal es adquirida durante el parto o durante el periodo neonatal temprano. Aunque su incidencia es desconocida, se realizó una búsqueda en Medline y se encontraron 28 casos de tuberculosis perinatal reportados de 1983 a 2011. El diagnóstico de esta enfermedad es importante debido a que los síntomas son inespecíficos y se confunden con otras enfermedades infecciosas. Además, presenta una alta tasa de mortalidad (hasta del 60%), por lo que se requiere de una pronta sospecha diagnóstica por parte del personal médico para evitar un desenlace fatal. Caso clínico: Se describe el caso de un masculino de 3 meses de edad, hijo de madre de 29 años que falleció por choque séptico a los 15 días del parto, que inició padecimiento a los 30 días de vida con tos y dificultad respiratoria. Se realizó el diagnóstico de neumonía de focos múltiples. Presentó deterioro respiratorio, por lo que requirió cambio de antibiótico en dos ocasiones aunque no se observó mejoría. Se recabó el dictamen de la autopsia de la madre que reveló tuberculosis peritoneal. Al paciente se le realizó PCR de aspirado traqueal y de líquido pleural, los cuales fueron positivos para Mycobacterium tuberculosis. Se estableció el diagnóstico de tuberculosis perinatal. No se encontró granuloma hepático. Conclusiones: La infección perinatal debe sospecharse en niños con sepsis o neumonía sin respuesta a antibióticos. En este caso, el antecedente de la madre con tuberculosis orientó al diagnóstico.

Background: Perinatal tuberculosis is acquired during birth or during the early neonatal period. Although its incidence is unknown, a search was conducted in Medline and 28 cases were found of perinatal tuberculosis reported from 1983 to 2011. Diagnosis of this disease is important due to having nonspecific symptoms that are mistaken for other infectious diseases. The disease has a high mortality rate (60%); therefore, it requires prompt diagnostic suspicion by the medical staff to prevent a fatal outcome. Case report: We describe the case of a 3-month-old male whose 29-year-old mother died of septic shock at 15 days of delivery. The infant's condition began within 30 days of age with cough and difficulty breathing with a diagnosis of multiple foci pneumonia. The infant presented respiratory impairment, meriting change of antibiotics twice, without improvement. The autopsy report of the mother revealed peritoneal tuberculosis. PCR was carried out using tracheal aspirate and pleural fluid of the patient, which were positive for Mycobacterium tuberculosis. Perinatal diagnosis of tuberculosis was established. No hepatic granuloma was found. Conclusions: Perinatal infection should be suspected in children with sepsis and/or pneumonia unresponsive to antibiotics. In this care, the history of tuberculosis in the mother should have oriented the diagnosis.

Aplasia pulmonar: a propósito de dos casos / Pulmonary aplasia: report of two cases

Introducción: La aplasia pulmonar es un raro trastorno del desarrollo pulmonar. Se caracteriza por la presencia de un bronquio principal rudimentario en ausencia de pulmón y arteria pulmonar. Generalmente se asocia con otros defectos congénitos, y debe sospecharse ante la opacidad del hemitórax. Casos clínicos: Se describen dos casos de pacientes con aplasia pulmonar diagnosticados en el Hospital Infantil de México Federico Gómez en los últimos 5 años. La primera paciente presentó dificultad respiratoria desde el nacimiento. Se observó radiopacidad total del hemitórax izquierdo, y se completó el diagnóstico de aplasia pulmonar con gammagrafía pulmonar y broncoscopia. La evolución ha sido insidiosa, con sintomatología respiratoria crónica, dependencia de oxígeno y neumonías recurrentes. La segunda paciente, de 5 años de edad, permaneció asintomática durante los primeros 2 años de vida. Fue hospitalizada por gastroenteritis infecciosa, y la radiografía sugirió hernia diafragmática derecha. Se intervino y se encontró eventración diafragmática derecha y ausencia de pulmón ipsilateral. Se completó el diagnóstico con broncoscopia que mostró bronquio principal derecho con saco ciego terminal. Conclusiones: La aplasia pulmonar es una entidad infrecuente. Debido a la variabilidad en la presentación clínica debe tenerse un alto índice de sospecha ante el hallazgo de la radiopacidad total del hemitórax. Los métodos diagnósticos que se utilizan son radiografía, tomografía y gammagrafía. Para confirmar el diagnóstico se requiere realizar broncoscopia. La escisión del muñón y la traslocación diafragmática se han descrito como opciones quirúrgicas de tratamiento.

Background: Pulmonary aplasia is a rare disorder of lung development characterized by the presence of a rudimentary main bronchus in the absence of lung and pulmonary artery. It is generally associated with other congenital defects and must be suspected in the presence of a total radiopaque hemithorax. Case reports: We describe two cases of pulmonary aplasia diagnosed in the Hospital Infantil de México "Federico Gómez" in the last 5 years. The first case was a female who presented respiratory distress from birth with a radiopaque left hemithorax in which the diagnosis of pulmonary aplasia was completed with bronchoscopy and lung scan. Her evolution has been insidious, characterized by chronic respiratory symptoms, oxygen dependence and pneumonias. The second case is a 5 year old female, who remained asymptomatic untill the age of two years when she was hospitalized for gastroeteritis. She underwet chest X-rays with findings suspicious of right diaphragmatic hernia. She was then transferred to our hospital. She underwent surgery at which time diaphragmatic eventration and no ipsilateral lung were found. The diagnosis was completed with a blind bottom main right bronchus in bronchoscopy. Conclusions: Pulmonary aplasia is an uncommon pathology. Due to great variability in clinical presentation, there must be a high index of suspicion in the presence of a fully radiopaque hemithorax. Among the diagnostic methods, X-rays, tomography and lung scan are useful. Bronchoscopy is required for diagnostic confirmation. Surgical removal of the stump and translocation of the diaphragm have been proposed as surgical options.

[Perinatal tuberculosis]. / Tuberculosis perinatal.

BACKGROUND: Perinatal tuberculosis is acquired during birth or during the early neonatal period. Although its incidence is unknown, a search was conducted in Medline and 28 cases were found of perinatal tuberculosis reported from 1983 to 2011. Diagnosis of this disease is important due to having nonspecific symptoms that are mistaken for other infectious diseases. The disease has a high mortality rate (60%); therefore, it requires prompt diagnostic suspicion by the medical staff to prevent a fatal outcome. CASE REPORT: We describe the case of a 3-month-old male whose 29-year-old mother died of septic shock at 15 days of delivery. The infant's condition began within 30 days of age with cough and difficulty breathing with a diagnosis of multiple foci pneumonia. The infant presented respiratory impairment, meriting change of antibiotics twice, without improvement. The autopsy report of the mother revealed peritoneal tuberculosis. PCR was carried out using tracheal aspirate and pleural fluid of the patient, which were positive for Mycobacterium tuberculosis. Perinatal diagnosis of tuberculosis was established. No hepatic granuloma was found. CONCLUSIONS: Perinatal infection should be suspected in children with sepsis and/or pneumonia unresponsive to antibiotics. In this care, the history of tuberculosis in the mother should have oriented the diagnosis.

[Pulmonary aplasia: report of two cases]. / Aplasia pulmonar: a propósito de dos casos.

BACKGROUND: Pulmonary aplasia is a rare disorder of lung development characterized by the presence of a rudimentary main bronchus in the absence of lung and pulmonary artery. It is generally associated with other congenital defects and must be suspected in the presence of a total radiopaque hemithorax. CASE REPORTS: We describe two cases of pulmonary aplasia diagnosed in the Hospital Infantil de México "Federico Gómez" in the last 5 years. The first case was a female who presented respiratory distress from birth with a radiopaque left hemithorax in which the diagnosis of pulmonary aplasia was completed with bronchoscopy and lung scan. Her evolution has been insidious, characterized by chronic respiratory symptoms, oxygen dependence and pneumonias. The second case is a 5 year old female, who remained asymptomatic untill the age of two years when she was hospitalized for gastroeteritis. She underwet chest X-rays with findings suspicious of right diaphragmatic hernia. She was then transferred to our hospital. She underwent surgery at which time diaphragmatic eventration and no ipsilateral lung were found. The diagnosis was completed with a blind bottom main right bronchus in bronchoscopy. CONCLUSIONS: Pulmonary aplasia is an uncommon pathology. Due to great variability in clinical presentation, there must be a high index of suspicion in the presence of a fully radiopaque hemithorax. Among the diagnostic methods, X-rays, tomography and lung scan are useful. Bronchoscopy is required for diagnostic confirmation. Surgical removal of the stump and translocation of the diaphragm have been proposed as surgical options.

[Hepatopulmonary syndrome as a cause of hypoxemia in hepatic diseases in children]. / Síndrome hepatopulmonar como causa de hipoxemia en niños con enfermedad hepática.

BACKGROUND: Hepatopulmonary syndrome is a rare complication characterized by liver disease associated with hypoxemia and intrapulmonary vascular dilatations. The prevalence reported in the few studies in children with cirrhosis is 3-8%. Although uncommon, it is important for physicians to recognize this condition because of its progressive course. CASE REPORT: We report the case of an 8-year-old girl diagnosed with liver cirrhosis and portal hypertension with symptoms of dyspnea and cyanosis. On physical examination the patient was found malnourished with jaundice, telangiectasias in abdomen, severe clubbing, acrocyanosis, platypnea and orthodeoxia; arterial blood gas showed PaO2 of 59mmHg. Echocardiography with agitated saline test was positive and lung perfusion scan with albumin macroaggregates reported 15% right-to-left short circuit, thus demonstrating the existence of intrapulmonary shunt. Diagnosis of severe hepatopulmonary syndrome was made. Liver transplantation is recommended as the only effective treatment. CONCLUSIONS: In children with liver disease presenting dyspnea and hypoxemia and those enrolled in a liver transplant protocol, hepatopulmonary syndrome must be intentionally searched because the prognosis will depend on timely diagnosis.

Síndrome de la cimitarra: serie de casos / Scimitar syndrome: a case series

Introducción: El síndrome de la cimitarra es una compleja anomalía congénita del desarrollo pulmonar, infrecuente y de múltiples variables. Se denomina así por su semejanza radiológica con la clásica espada torcida. Su característica definitoria es el drenaje pulmonar anómalo. Se asocia con diversas malformaciones cardiotorácicas y un amplio espectro de manifestaciones clínicas. Serie de casos: Se reporta una serie proveniente de la base de datos del Hospital Infantil de México Federico Gómez, revisión del periodo 2009-2013. Se encontraron nueve pacientes con diagnóstico de síndrome de la cimitarra. Se recabaron del expediente médico las características demográficas, estado clínico y parámetros hemodinámicos reportados. Conclusiones: En la presente serie llaman la atención ciertas diferencias entre el grupo de estudio y lo reportado en la literatura internacional. Se encontró que la mayoría de los pacientes fueron del sexo femenino, diagnosticados entre el primero y el vigésimo mes de vida. En el momento del estudio estaban asintomáticos; la mitad contaba con antecedentes de enfermedad respiratoria y el total con hipertensión pulmonar. La tercera parte del grupo requirió manejo quirúrgico.

Background: Scimitar syndrome is a rare and complex congenital anomaly of the lung with multiple variables and is named for its resemblance to the classical radiological crooked sword. Its defining feature is the anomalous pulmonary drainage. It is associated with various cardiothoracic malformations and a wide spectrum of clinical manifestations. Case series: Nine patients diagnosed with scimitar syndrome found in the database of Hospital Infantil de México between 2009 and 2013 were reviewed. Demographic records, clinical status and hemodynamic parameters reported were collected. Conclusions: This case series called attention to certain differences between our group of patients and those reported in the international literature. Patients were predominantly female and were diagnosed between 1 and 20 months of life. All were asymptomatic at the time of the study. Half of the patients had a history of respiratory disease and all patients had with pulmonary hypertension. Surgical management was required in on-third of the patient group.

Bronquiolitis folicular linfoidea posterior a infección por adenovirus / Lymphoid follicular bronchiolitis post-adenovirus infection

Introducción: La bronquiolitis folicular es una lesión pulmonar rara. Consiste en la presencia de abundantes folículos linfoides hiperplásicos con centros germinales reactivos, distribuidos a lo largo de los bronquiolos y compresión de la vía aérea pequeña intratorácica. Existen pocos informes de bronquiolitis folicular en la población pediátrica. Los datos que se conocen de esta enfermedad para esta población han sido extrapolados de estudios realizados en pacientes adultos. El tratamiento se basa en esteroides y, en general, el pronóstico es bueno. Caso clínico: Describimos el caso de una niña de 5 años de edad, con antecedente de neumonía grave a los 3 años, con adenovirus en el panel viral. Posteriormente, presentó sibilancias recurrentes y tos crónica. En la tomografía axial de tórax de alta resolución se observó un patrón en vidrio despulido, bronquiectasias, atrapamiento aéreo y atelectasia basal derecha, por lo que se realizó una biopsia de pulmón, la cual mostró bronquiolitis folicular linfoidea. Conclusiones: La bronquiolitis folicular linfoidea es una entidad poco frecuente, que requiere ser sospechada en pacientes con antecedentes de infección por adenovirus. Presenta cuadro clínico de hiperreactividad bronquial y alteraciones radiológicas con patrón en vidrio despulido, bronquiectasias y atrapamiento aéreo. La biopsia pulmonar por toracotomía es la clave para establecer el diagnóstico y el pronóstico.

Background: Follicular bronchiolitis, a rare lung injury, is characterized by abundant hyperplastic lymphoid follicles with reactive germinal centers distributed along the bronchioles with compression of the lower intrathoracic airway. In the literature there are few reports of follicular bronchiolitis in the pediatric population. Data obtained from this disease have been extracted from studies in adult patients. Treatment is based on steroids with a good prognosis. Case report: We describe the case of a 5-year-old female with a history of severe pneumonia at 3 years of age, isolating adenovirus in the viral panel. Subsequently, she had recurrent wheezing and chronic cough. High resolution thoracic computed tomography (CT) showed ground glass pattern, bronchiectasis, air trapping and right basal atelectasis. Lung biopsy was performed and reported lymphoid follicular bronchiolitis. Conclusions: Lymphoid follicular bronchiolitis is a rare entity that requires a high level of suspicion in patients with a history of adenovirus infection, clinical symptoms of bronchial hyperreactivity and radiological changes in ground glass pattern, bronchiectasis and air trapping. Lung biopsy by thoracotomy is the key for diagnosis and prognosis.

[Scimitar syndrome: a case series]. / Síndrome de la cimitarra: serie de casos.

BACKGROUND: Scimitar syndrome is a rare and complex congenital anomaly of the lung with multiple variables and is named for its resemblance to the classical radiological crooked sword. Its defining feature is the anomalous pulmonary drainage. It is associated with various cardiothoracic malformations and a wide spectrum of clinical manifestations. CASE SERIES: Nine patients diagnosed with scimitar syndrome found in the database of Hospital Infantil de México between 2009 and 2013 were reviewed. Demographic records, clinical status and hemodynamic parameters reported were collected. CONCLUSIONS: This case series called attention to certain differences between our group of patients and those reported in the international literature. Patients were predominantly female and were diagnosed between 1 and 20 months of life. All were asymptomatic at the time of the study. Half of the patients had a history of respiratory disease and all patients had with pulmonary hypertension. Surgical management was required in on-third of the patient group.