Disseminated visceral Kaposi´s sarcoma in a pediatric bilateral lung transplant recipient.

Cancer is a major adverse outcome of solid organ transplantation, and risks are especially high for malignancies caused by viral infections. HHV-8 is the etiologic agent of Kaposi´s sarcoma (KS). We report a case of visceral KS occurring in a 15-year-old patient after lung transplantation. The evolution was dramatically fast and interestingly, KS lesions were diffusely observed, but not in the skin. The autopsy showed the presence of numerous tumoral lesions in many organs. Microscopically, they all had very similar features, regardless of the organ affected. KS presented without cutaneous involvement. The girl was not tested for HHV-8 prior to transplantation as it was not part of our protocol. The donor was negative. The aim of the report is to alert other teams, especially those working in pediatrics, about this rare but potential complication in the setting of solid organ transplantation.

Primary lung tumors in children: 24 years of experience at a referral center.

PURPOSE: Primary lung tumors are rare during childhood and encompass a wide variety of histological types. Each has a different biology and a different therapeutic approach. The aim of this article is to review the experience of a pediatric referral center with this kind of tumors during the last 24 years. METHODS: A retrospective chart review was performed for patients with diagnosis of primary lung tumor between the years 1990-2014. The variables analyzed were age, sex, course of the disease, symptoms, localization, surgery, histology and outcome. RESULTS: Between 1990 and 2014, 38 patients with primary lung tumors were treated at our institution. Age at presentation was 6.6 ± 5.2 years (r 0.91-16.58) and the female:male relationship was 1.37. Inflammatory myofibroblastic lung tumor (n = 13), carcinoid tumor (n = 6) and pleuropulmonary blastoma (n = 6) were the most frequent histological types. Persistent radiographic abnormality was the most frequent presenting sign (34 %). Global mortality was 15.8 % varying according to histology. CONCLUSION: Although the diagnosis of primary lung tumor is rare, the persistence of a radiographic abnormality in spite of adequate treatment for inflammatory processes forces us to evaluate further. The age of the patient is an important factor in the decision of the diagnostic work-up.

[Cough and hypoxemia as clinical manifestation of pulmonary alveolar proteinosis. Clinical case report]. / Tos e hipoxemia como manifestación clínica de la proteinosis alveolar. Reporte de caso clínico.

Alveolar proteinosis is a rare chronic lung disease, especially in children, characterized by abnormal accumulation of lipoproteins and derived surfactant in the intra-alveolar space that generates a severe reduction of gas exchange. Idiopathic presentation form constitutes over 90% of cases, a phenomenon associated with production of autoimmune antibodies directed at the receptor for granulocyte-macrophage colony-stimulating factor. A case of a girl of 5 years of age treated because of atypical pneumonia with unfavorable evolution due to persistent hypoxemia is presented. The diagnosis is obtained through pathologic examination of lung biopsy by thoracotomy, as treatment is carried out by 17bronchopulmonary bronchoscopy lavages and the patient evidences marked clinical improvement.

La proteinosis alveolar es una enfermedad pulmonar crónica poco frecuente, especialmente en pediatría, caracterizada por la acumulación anormal de lipoproteínas y derivados del surfactante en el espacio intraalveolar, que genera una grave reducción del intercambio gaseoso. La forma de presentación idiopática constituye más del 90% de los casos relacionados con un fenómeno de autoinmunidad, con producción de anticuerpos dirigidos contra el receptor del factor estimulante de colonias de granulocitos y macrófagos. Se presenta un caso clínico de una niña de 4 años de edad tratada por neumonía atípica con evolución desfavorable por hipoxemia persistente. El diagnóstico se obtuvo a través del estudio anatomopatológico de la biopsia pulmonar por toracotomía. Se llevaron a cabo 17 lavados broncopulmonares mediante endoscopía respiratoria y la paciente evidenció franca mejoría clínica.

Congenital cystic adenomatoid malformation: clinical features, pathological concepts and management in 172 cases.

OBJECTIVE: Congenital cystic adenomatoid malformation (CCAM) is the most common surgically resected pulmonary malformation in children. This retrospective study was undertaken to present the experience of 172 CCAM cases in a pediatric hospital. METHODS: Published series with a small number of patients reports details of lesions, progress and management. As this study addresses clinical characteristics, progress and surgical procedures in 172 children with CCAM diagnosis, the population includes cases treated and followed up in a pediatric hospital throughout 25 years (1986-2011). RESULTS: Mean age at diagnosis was 48 months (r = 0.03-213), 52% (n = 90) were male. The most common presenting symptoms were respiratory distress in children under 6 months of age (40%) and recurrent pneumonia in older ones (75%; p = 0.001). Lobectomy was the procedure of choice in the majority. All histological types were found: 1 (70%), 2 (24%), 4 (4%), and 0 and 3 (n = 1). A mixed pattern was observed in nine patients. Associated anomalies were found in 47% of children. The most frequent was sequestration (71%), mostly present in CCAM type 2 (p = 0.001). Severe anomalies were mostly related to type 2 (p = 0.008). A pleuropulmonary blastoma and a bronchioloalveolar carcinoma were also observed. Mortality was 5% (n = 9). Risk factors for mortality were respiratory failure (OR = 25.7 [95%CI 3.2-221]; p = 0.03), sepsis (OR = 9.9 [95%CI 8.2-12]; p = 0.002), respiratory assistance requirements (OR = 9.5 [95%CI 2.3-37]; p = 0.04), and severe associated comorbidities (OR = 3.3 [95%CI 1.2-22]; p = 0.008). CONCLUSIONS: Related anomalies were observed in almost half of the population. Due to the possibility of recurrent infection or development of malignancies, surgical resection should be considered when CCAM is diagnosed. Surgical outcome is favorable with manageable complications.

Inflammatory myofibroblastic tumor of the lung in children: anaplastic lymphoma kinase (ALK) expression and clinico-pathological correlation.

The inflammatory myofibroblastic tumor (IMT) is a rare neoplastic lesion with a high incidence in children and young people, and may arise in lungs, soft tissue, or viscera. It is recognized as a borderline tumor with the possibility to recur, undergo malignant transformation, and metastasize. IMT is composed of fascicles of bland myofibroblastic cells admixed with an inflammatory infiltrate consisting of lymphocytes, plasma cells, and eosinophils. We reviewed pulmonary IMT diagnosed at Garrahan Hospital in Buenos Aires, Argentina, during 12 years and examined the clinical, laboratory, and pathological features as well as molecular genetics. Eight pediatric cases were evaluated with a male-to-female ratio of 5:3 and a median age of 6 years at diagnosis. The most common lung localization was the upper lobe. All cases underwent surgical excision and no local recurrences were found. Five out of eight patients, including two cases with metastatic/multifocal lesions in the central nervous system (CNS), are alive and disease free after a median follow-up of 30 months. Anaplastic lymphoma kinase (ALK) expression was negative in all pulmonary samples by immunohistochemistry (IHC), however, rearrangement for ALK locus by fluorescence in situ hybridization was found in one lung and in two CNS samples. These findings may reflect higher sensitivity of the molecular biologic procedure compare to traditional IHC practice. In our pediatric experience, 25% of patients with lung IMT developed CNS lesions; therefore we consider that CNS screening in these patients should be considered, at diagnosis and later during follow up.

Acute lower respiratory tract infections by adenovirus in children: histopathologic findings in 18 fatal cases.

Adenoviruses (Ads) are the 2nd-most common cause of acute lower respiratory tract infections of viral etiology in immunocompetent children who require hospitalization in Argentina. A new pathogenic genomic variant, Ad7h, has been identified and was associated with severe sequelae and a high mortality rate. A retrospective observational study was conducted in hospitalized children with acute lower respiratory tract infections between March 1988 and May 2005. Nasopharyngeal or tracheal aspirates, bacteriological analysis on blood samples and pleural fluids, and histopathologic studies on autopsies were performed. A total of 415 children were included in the study. Sixty-two patients died in the acute stage of illness (1-8 weeks), and postmortem examination was performed on 18 of them. Mean age was 8.25 months. The male to female ratio was 1.3∶1. The Ad was genome typed in 9% of all children, of whom 29 corresponded to the Ad7h variant. This genome type was diagnosed in 7 of the autopsies performed. Necrotizing bronchitis and bronchiolitis with abundant typical adenoviral inclusion nuclear cells and diffuse alveolar damage were the most important findings. Superimposed bacterial infection occurred in 11 cases, and cytomegalovirus was found in 1. In our population, Ad respiratory infection was found to have a severe course with sequelae or death. No differences were observed between lesions caused by other previously described genome types and those found in cases in which Ad7h was identified.

Tos e hipoxemia como manifestación clínica de la proteinosis alveolar: Reporte de caso clínico / Cough and hypoxemia as clinical manifestation of pulmonary alveolar proteinosis: Clinical case report

La proteinosis alveolar es una enfermedad pulmonar crónica poco frecuente, especialmente en pediatría, caracterizada por la acumulación anormal de lipoproteínas y derivados del surfactante en el espacio intraalveolar, que genera una grave reducción del intercambio gaseoso. La forma de presentación idiopática constituye más del 90% de los casos relacionados con un fenómeno de autoinmunidad, con producción de anticuerpos dirigidos contra el receptor del factor estimulante de colonias de granulocitos y macrófagos. Se presenta un caso clínico de una niña de 4 años de edad tratada por neumonía atípica con evolución desfavorable por hipoxemia persistente. El diagnóstico se obtuvo a través del estudio anatomopatológico de la biopsia pulmonar por toracotomía. Se llevaron a cabo 17 lavados broncopulmonares mediante endoscopía respiratoria y la paciente evidenció franca mejoría clínica.

Alveolar proteinosis is a rare chronic lung disease, especially in children, characterized by abnormal accumulation of lipoproteins and derived surfactant in the intra-alveolar space that generates a severe reduction of gas exchange. Idiopathic presentation form constitutes over 90% of cases, a phenomenon associated with production of autoimmune antibodies directed at the receptor for granulocyte-macrophage colony-stimulating factor. A case of a girl of 5 years of age treated because of atypical pneumonia with unfavorable evolution due to persistent hypoxemia is presented. The diagnosis is obtained through pathologic examination of lung biopsy by thoracotomy, as treatment is carried out by 17bronchopulmonary bronchoscopy lavages and the patient evidences marked clinical improvement.

Malformação adenomatoide cística congênita: características clínicas, conceitos patológicos e tratamento em 172 casos / Congenital cystic adenomatoid malformation: clinical features, pathological concepts and management in 172 cases

OBJETIVO: A malformação adenomatoide cística congênita (MACC) é a malformação pulmonar ressecada cirurgicamente mais comum em crianças. Este estudo retrospectivo foi realizado para apresentar a experiência de 172 casos de MACC em um hospital pediátrico. MÉTODOS: Séries publicadas com um pequeno número de pacientes relatam detalhes de lesões, evolução e tratamento. Como este estudo lida com características clínicas, evolução e procedimentos cirúrgicos em 172 crianças com diagnóstico de MACC, a população inclui casos tratados e acompanhados em um hospital pediátrico ao longo de 25 anos (1986-2011). RESULTADOS: A idade média ao diagnóstico foi de 48 meses (r = 0,03-213), 52% (n = 90) eram do sexo masculino. Os sintomas de apresentação mais comuns foram dificuldade respiratória em crianças com menos de 6 meses de idade (40%) e pneumonia recorrente nas que tinham mais idade (75%; p = 0.001). Lobectomia foi o procedimento de escolha na maioria dos casos. Todos os tipos histológicos foram encontrados: 1 (70%), 2 (24%), 4 (4%) e 0 e 3 (n = 1). Foi observado um padrão misto em nove pacientes. Foram encontradas anomalias associadas em 47% das crianças. A mais frequente foi sequestro (71%), mais presente na MACC tipo 2 (p = 0,001). As anomalias mais graves se relacionaram principalmente com o tipo 2 (p = 0,008). Também foram observados um blastoma pleuropulmonar e um carcinoma broncoalveolar. A mortalidade foi de 5% (n = 9). Os fatores de risco para mortalidade foram falência respiratória (OR = 25,7 [IC95% 3,2-221]; p = 0,03), sepse (OR = 9,9 [IC95% 8,2-12]; p = 0,002), necessidade de assistência respiratória (OR = 9,5 [IC95% 2,3-37]; p = 0,04) e diversas comorbidades associadas (OR = 3,3 [IC95% 1,2-22]; p = 0,008). CONCLUSÕES: Foram observadas anomalias relacionadas em quase metade da população. Devido à possibilidade de infecção recorrente ou desenvolvimento de neoplasias, deve-se considerar a ressecção cirúrgica quando MACC for diagnosticada. O desfecho cirúrgico é favorável e apresenta complicações tratáveis.

OBJECTIVE: Congenital cystic adenomatoid malformation (CCAM) is the most common surgically resected pulmonary malformation in children. This retrospective study was undertaken to present the experience of 172 CCAM cases in a pediatric hospital. METHODS: Published series with a small number of patients reports details of lesions, progress and management. As this study addresses clinical characteristics, progress and surgical procedures in 172 children with CCAM diagnosis, the population includes cases treated and followed up in a pediatric hospital throughout 25 years (1986-2011). RESULTS: Mean age at diagnosis was 48 months (r = 0.03-213), 52% (n = 90) were male. The most common presenting symptoms were respiratory distress in children under 6 months of age (40%) and recurrent pneumonia in older ones (75%; p = 0.001). Lobectomy was the procedure of choice in the majority. All histological types were found: 1 (70%), 2 (24%), 4 (4%), and 0 and 3 (n = 1). A mixed pattern was observed in nine patients. Associated anomalies were found in 47% of children. The most frequent was sequestration (71%), mostly present in CCAM type 2 (p = 0.001). Severe anomalies were mostly related to type 2 (p = 0.008). A pleuropulmonary blastoma and a bronchioloalveolar carcinoma were also observed. Mortality was 5% (n = 9). Risk factors for mortality were respiratory failure (OR = 25.7 [95%CI 3.2-221]; p = 0.03), sepsis (OR = 9.9 [95%CI 8.2-12]; p = 0.002), respiratory assistance requirements (OR = 9.5 [95%CI 2.3-37]; p = 0.04), and severe associated comorbidities (OR = 3.3 [95%CI 1.2-22]; p = 0.008). CONCLUSIONS: Related anomalies were observed in almost half of the population. Due to the possibility of recurrent infection or development of malignancies, surgical resection should be considered when CCAM is diagnosed. Surgical outcome is favorable with manageable complications.

Mortalidad postneonatal por infecciones respiratorias bajas. Antecedentes adversos de la salud y fallas del proceso de atención / Effect of certain adverse conditions of children's status and medical care on postneonatal mortality due to lower respiratory trac infections

Se estudiarion dieciocho lactantes menores de un añofallecidos por una infección respiratoria baja (IRB): neumonitis, neumonía o bronconeumonía (grupo de estudio) y un grupo control (n=18) con igual diagnóstico y sobrevivientes, apareados por edad y sexo, internados en el Hospital de Pediatría Garrahan entre 1987 y 1989. Se comprobó que la mayoría de los casos provenía de áreas con alto porcentaje (>30 por ciento) de familias con necesidades básicas insatisfechas (NBI). Hubo, en ambos grupos, elevada frecuencia de antecedentes adversos en la salud materno-infantil: embarazo no controlado (n=6), desnutrición (n=19), vacunación incompleta (n=15) e internaciones previas por infecciones respiratorias (n=9) siendo la frecuencia de las tres últimas variables desfavorables significativamente mayor en el grupo de estudio. En la mayoría de los casos (n=24) la evoluci'on ambulatoria de la IRB fue de 3 o más días; todos presentaron durante esta etapa dificultad respiratoria, pero sólo en el grupo fallecido tos cianozante (n=7) y apneas (n=4). A pesar de estos signos, la mayoría de los pacientes (n=29) fue atendida ambulatoriamente. Los pacientes fallecidos presentaron mayor gravedad al ingreso, expresada por una mayor frecuencia de hipercapnia (n=16), estadísticamente significativa. En la anatomía patológica predominó la bronconeumonía bacteriana inespecífica, asociada, en algunos casos, a sepsis y neumonitis viral. Estos resultados demuestran que en la población internada por IRB existe una elevada frecuencia de antecedentes adversos, siendo éstos más frecuentes y severos en el grupo fallecido. Asimismo se comprobó insuficiente atención de la enfermedad actual, no relacionada en sus acciones con los factores de riesgo ni con la severidad de los signos presentes. Los resultados señalan la necesidad de generar acciones preventivas y curativas que reorganicen y mejoren el sistema de atención materno-infantil a fin de disminuír la mortalidad infantil debida a IRB.

Enfermedad pulmonar en 238 casos de muerte inesperada del lactante / Pulmonary disease in 238 cases of sudden unexpected death in infancy

Se revisaron todas las láminas correspondientes a los pulmones de las autopsias incluidas en el programa de estudio de las muertes inesperadas de los lactantes realizadas en Montevideo entre el 1 de octubre de 1998 y el 1 de octubre de 2001, en el Centro Hospitalario Pereira Rossell. Esta revisión se hizo sin conocimiento del diagnóstico primario realizado, de los datos de la historia ni de las circunstancias que rodearon a la muerte. Hubo concordancia con el diagnóstico inicial de categoría muerte explicable, autopsia negativa y zona gris en 229 de 238 casos (96,2 por ciento). Con el diagnóstico revisado la ME pasó de 145 a 143 casos (60,9 por ciento a 60 por ciento). La ZG pasó de 43 a 46 casos (de 18 por ciento a 19,3 por ciento). La AN de 50 a 49 casos (de 21 por ciento a 20,5 por ciento).

Malformación adenomatoide quística: 89 casos de Argentina y Uruguay / Cystic adenomatoid malformation: 89 cases from Argentina and Uruguay

Se presenta una revisión de 89 casos de malformación adenomatoide quística (MAQ) del pulmón en niños. Los casos fueron reunidos entre el Hospital J.P. Garrahan de Buenos Aires y el Hospital Pereira Rossell de Montevideo. Hubo un predominio de MAQ tipo I. Un patrón mixto se observó en 10 casos. Se constató patología asociada en 37 por ciento de los pacientes; en 79 por ciento fue pulmonar y en 27 por ciento extrapulmonar. En un paciente se observó tumor maligno pulmonar originado en una MAQ lo que subraya la necesidad de tratamiento quirúrgico. La asociación con infección en la mayoría de nuestros casos con vaso arterial sistémico, es una fuerte evidencia a favor de una patogenia adquirida. La infección por Aspergillus y la asociación con quiste broncogénico en MAQ I son hallazgos importantes en nuestra serie

Sarcoidosis en la infancia / Childhood Sarcoidosis

Presentamos un varón de 4 años 9 meses de edad afectado de Sarcoidosis.El diagnóstico fue confirmado mediante el estudio histopatológico del pulmón,el cual fue indicado por tratarse de una enferemedad intersticial progresiva de etiología desconocida.Finalmente señalamos la favorable evolución clinicoanalítica y radiológica tras el tratamiento con corticoides

Secuestros pulmonares / Pulmonary sequestration

Se presentan 22 casos de secuestros pulmonares observados en pacientes menores de 13 años en el Hospital de Niños de Buenos Aires "Dr. Ricardo Gutiérrez". Se analizan edad de presentación, signos clínicos y radiológicos, malformaciones asociadas, localización, características de la irrigación sistémica, terapéutica, quirúrgica y lesiones anatomopatológicas encontradas en secuestros intra y extralobares. Destacamos especialmente la diferente edad de presentación y sintomatología en ambos grupos, así como la elevada incidencia de malformación adenomatoidea quística (MAQ) como hallazgo anatomopatológico. En los secuestros extralobares la mayoría de los niños presentó un síndrome de dificultad respiratoria durante el primer mes de vida. Este fue causado por las malformaciones asociadas (hernia o eventración diafragmática) o por ocupación torácica con desplazamiento mediastinal por la masa sólida de las formas pseudotumorales de los secuestros. Los intralobares se observaron predominantemente entre los 6 y 24 meses de vida o luego de los 5 años, poniéndose de manifiesto por infección respiratoria crónica o recidivante. Comparamos nuestros hallazgos con 106 casos publicados en la literatura mundial. Se discuten las diferentes teorías acerca de la etiopatogenia de estas anomalías y en virtud de lo observado en nuestros pacientes proponemos causas etiológicas diferentes para los secuestros intra y extralobares

Bronquiectasias en la infancia / Bronchiectasis in childhood

Las bronquiectasias son dilataciones permanentes de las vías aéreas segmentarias y subsegmentarias. En la mayoría de los pacientes, las infecciones necrotizantes del pulmón, únicas o recurrentes, son las principales responsables del daño inicial. Los objetivos de este trabajo fueron identificar las entidades causales y caracterizar los hallazgos clínicos, radiológicos y tomográficos que posibilitan el diagnóstico. En el período desde agosto de 1987 a diciembre de 2001 se examinaron las historias clínicas de 687 pacientes con diagnóstico de bronquiectasia atendidos en el Hospital de Pediatría Dr. J.P. Garrahan de Buenos Aires, Argentina. Las causas de bronquiectasia encontradas con mayor frecuencia fueron: posinfección respiratoria aguda baja inespecífica (n=403), fibrosis quística (n=170) e inmunodeficiencias primarias (n=73). Conclusiones: los signos y síntomas clínicos y los hallazgos radiológicos y tomográficos permitieron el diagnóstico de bronquiectasias. En esta serie, la infección respiratoria aguda baja inespecífica fue la causa más frecuente de bronquiectasias, siendo muy precoz su edad de comienzo. En este grupo hubo un alto porcentaje de niños con lesiones extensas y severas que desarrollaron enfermedad pulmonar grave con insuficiencia respiratoria crónica en etapas tempranas de la vida