Antibody deficiencies with normal IgG in adults with Non-cystic fibrosis bronchiectasis or recurrent pneumonia: Cross-sectional study / Deficiencias Predominantemente de Anticuerpos con niveles normales de IgG en adultos con bronquiectasias no fibrosis quística o neumonía recurrente en Colombia

Abstract Background: Inborn errors of immunity, mainly Predominantly Antibody deficiencies with normal IgG levels are unrecognized in adults with lung diseases such as bronchiectasis or recurrent pneumonia. Objective: To determine IgM, IgA, IgG2 subclass deficiencies, and Specific antibody deficiency (anti-pneumococcal polysaccharide antibodies) in adults with non-cystic fibrosis bronchiectasis or recurrent pneumonia. Methods: Cross-sectional study. Consecutive patients with non-cystic fibrosis bronchiectasis or recurrent pneumonia were recruited in Cali, Colombia. IgG, IgA, IgM, and IgE, IgG2subclass and IgG anti-pneumococcal serum levels were measured. Results: Among the 110 participants enrolled, Antibody deficiencies with normal serum IgG levels were found in 11(10%) cases. IgA deficiency (3 cases), IgM deficiency (2 cases) and IgG2 deficiency (2 cases) were the most frequent primary immunodeficiencies. In addition, IgG2+IgA deficiency, Ataxia-telangiectasia, Hyper-IgE syndrome and Specific Antibody Deficiency(anti-polysaccharides) were found in one case each. Conclusions: Predominantly antibody deficiencies with normal IgG levels are an important etiology of non-cystic fibrosis bronchiectasis and recurrent pneumonia in adults.

Resumen Antecedentes: Los Errores Innatos de la Inmunidad principalmente las Deficiencias Predominantemente de anticuerpos con niveles normales de IgG no se conocen en adultos con enfermedades pulmonares como las bronquiectasias o la neumonía recurrente. Objetivo: Determinar las deficiencias de IgM, IgA y de subclase de IgG2 y la Deficiencia Específica de Anticuerpos (anticuerpos antineumocócicos de polisacáridos) en adultos con Bronquiectasias no Fibrosis Quística (BQnoFQ) o neumonía recurrente. Métodos: Estudio observacional prospectivo. Se reclutaron 110 pacientes consecutivos con BQnoFQ o neumonía recurrente en Cali, Colombia. Se midieron los niveles séricos de IgG, IgA, IgM e IgE, subclase IgG2 y anticuerpos anti-neumococo. Resultados: Se encontraron deficiencias de anticuerpos con niveles normales de IgG en el 10% de los sujetos; Cuatro casos con IgG2 baja, incluido 1 caso de deficiencia de IgG2 + IgA, 1 caso de ataxia-telangiectasia, 3 deficiencias de IgA (IgAD), 2 deficiencias selectiva de IgM (IgMD), 1 síndrome de Hiper-IgE (HIES-AR) y 1 deficiencia específica de anticuerpos. Ocho pacientes fueron diagnosticados con enfermedades relacionadas con la hipogammaglobulinemia IgG. Conclusiones: Las deficiencias predominantemente de anticuerpos con niveles normales de IgG son una etiología importante de BQnoFQ y neumonía recurrente en adultos. Los sujetos con bronquiectasias o neumonía recurrente requieren una evaluación exhaustiva de la respuesta inmune humoral y clínica.

El método clínico en la atención a niños con neumonías recurrentes: contradicciones teórico-prácticas

RESUMEN Introducción: Las manifestaciones pulmonares recurrentes en los niños se definen con la frase neumonías recurrentes. La aplicación del método clínico en la atención a pacientes con esas afecciones debe estar encaminada a cumplir su objetivo rector, definido por la identificación y control de la causa subyacente que motiva la recurrencia de las manifestaciones pulmonares. Sin embargo, ese objetivo no siempre se cumple adecuadamente. Objetivo: Describir un grupo de contradicciones en el cuerpo del conocimiento de las neumonías recurrentes en los niños y su influencia en la ejecución del método clínico durante el proceso de atención médica a esos pacientes. Método: A partir de la revisión de la literatura, en una primera etapa se realizó un análisis etimológico y terminológico de la frase "neumonías recurrentes", así como de sus definiciones conceptual y operacional. En una segunda etapa, a partir de las insuficiencias detectadas, se efectuó una valoración crítica de cómo éstas pueden influir en la forma en que los profesionales aplican el método clínico en la atención médica a los niños con esas afecciones. Análisis e integración de la información: Se identificaron contradicciones teórico-prácticas en las definiciones conceptual y operacional de las neumonías recurrentes en niños, las cuales pueden constituir el fundamento de los errores que se producen de manera sistemática durante la recogida de la información y la formulación de hipótesis en el proceso de atención médica en esos pacientes. Consideraciones finales: La aplicación del método clínico en la atención a niños con neumonías recurrentes puede presentar insuficiencias condicionadas por contradicciones teórico-prácticas.

ABSTRACT Introduction: Recurrent pulmonary manifestations in children are defined by the phrase recurrent pneumonias. The application of the clinical method in the care of patients with these conditions must be aimed at fulfilling its guiding objective, defined by the identification and control of the underlying cause that motivates the recurrence of pulmonary manifestations. However, that goal is not always adequately met. Objective: Describe a group of contradictions in the body of knowledge of recurrent pneumonias in children and their influence on the execution of the clinical method during the process of medical care for these patients. Method: Based on the literature review, in a first stage an etymological and terminological analysis of the phrase "recurrent pneumonias" was carried out, as well as its conceptual and operational definitions. In a second stage, based on the insufficiencies detected, a critical assessment was made of how these can influence the way in which professionals apply the clinical method in medical care to children with these conditions. Analysis and integration of information: Theoretical-practical contradictions were identified in the conceptual and operational definitions of recurrent pneumonias in children, which may constitute the basis of the errors that occur systematically during the collection of information and the formulation of hypotheses in the process of medical care in these patients. Final considerations: The use of the clinical method in the care of children with recurrent pneumonia may present insufficiencies conditioned by theoretical-practical contradictions.

Errores innatos de la inmunidad en adultos / Inborn errors of immunity in adults

Introducción: La inmunodeficiencia común variable es un error innato de la inmunidad que tiene su pico de incidencia en la edad adulta. Se caracteriza por una susceptibilidad aumentada a padecer infecciones respiratorias, autoinmunidad y malignidad, secundario a un estado de hipogammaglobulinemia e inmunodisregulación, causado por mutaciones e interacciones genéticas parcialmente comprendidas. El diagnóstico es de exclusión, tiene una gran heterogeneidad clínica y comúnmente es diagnosticado de forma errónea. Objetivo: Describir un caso clínico de un paciente afectado por un error innato de la inmunidad. Caso clínico: Hombre de 35 años que se presenta a la consulta de Medicina Interna - Inmunología refiriendo un cuadro clínico de 3 años de evolución consistente en múltiples episodios de infecciones sino-pulmonares en los últimos meses, presentaba tos productiva, dificultad respiratoria y pérdida de peso no intencional de aproximadamente 8 kg. Conclusiones: La inmunodeficiencia común variable debe considerarse dentro de los diagnósticos diferenciales en todo paciente que presente alguna de sus manifestaciones clínicas, principalmente aquellas relacionadas con infecciones respiratorias a repetición, antecedente que el paciente puede presentar como relevante en sus consultas de primer nivel con medicina general o con especialistas. Su aproximación diagnóstica consiste en la solicitud de niveles séricos de inmunoglobulinas, prueba de laboratorio de fácil acceso para cualquier clínico independiente de su nivel de atención y su tratamiento se fundamenta en la administración periódica de inmunoglobulina humana exógena de forma endovenosa o subcutánea(AU)

Introduction: Common variable immunodeficiency is an inborn error of immunity that has its peak incidence in adulthood. It is characterized by an increased susceptibility to respiratory infections, autoimmunity and malignancy, secondary to a state of hypogammaglobulinemia and immunodysregulation, caused by mutations and partially understood genetic interactions. The diagnosis is one of exclusion, has great clinical heterogeneity and is commonly misinterpreted. Objective: To describe a clinical case of a patient affected by an inborn error of immunity. Methods: Retrospective description of a case report. Conclusions: Common variable immunodeficiency disorder should be considered within the differential diagnoses in every patient who presents any of its clinical manifestations, mainly those related to recurrent respiratory infections, an antecedent that the patient may present as relevant during the first-level consultations with general medicine physicians or with specialists. Its diagnostic approach consists in measuring serum immunoglobulin levels, an easily accessible laboratory test for any clinic physician regardless of their healthcare level, while its treatment is based on the periodic administration of exogenous human immunoglobulin intravenously or subcutaneously(AU)

A rare case of Sjogren-Larsson syndrome with recurrent pneumonia and asthma / 소아과

Sjogren-Larsson syndrome (SLS) is a rare autosomal recessive neurocutaneous disorder with worldwide incidence of 0.4 per 100,000 people. It is characterized by the triad of congenital ichthyosis, spastic diplegia or quadriplegia, and mental retardation. Herein we report a 2-year-old male child with SLS, asthma, and recurrent pneumonia. SLS was confirmed by a molecular genetics study that revealed a deletion mutation in the ALDH3A2 gene. An ALDH3A2 gene mutation results in dysfunction of the microsomal enzyme fatty aldehyde dehydrogenase and impaired metabolism and accumulation of leukotriene B4, which is a key molecule and a pro-inflammatory mediator in developing allergic diseases, especially asthma. An increased level of leukotriene B4 has been reported in SLS patients. As far as we are aware, this is the first report of SLS associated with asthma and recurrent pneumonia. In conclusion, pediatricians should be aware of and evaluate patients with SLS for possible associated asthma and allergic disorders.

Las afecciones pulmonares recurrentes en niños / Recurrent pulmonary illnesses

INTRODUCCIÓN: los estudios sobre las afecciones pulmonares recurrentes en niños son escasos; el mayor porcentaje de ellos se realizó en escenarios fuera de la atención primaria, y los datos acerca de las causas subyacentes y los factores de riesgo para esas afecciones son inconsistentes. OBJETIVO: analizar las causas subyacentes y los posibles factores de riesgo de la recurrencia de afecciones pulmonares en niños. MÉTODOS: se realizó un estudio de casos y controles. 63 niños, previamente identificados por médicos de la atención primaria, fueron investigados a partir de un algoritmo para el diagnóstico clínico en la consulta especializada sobre neumopatías recurrentes y crónicas de la provincia Sancti Spíritus, desde el 1º de enero de 2011 al 30 de abril de 2014. En igual período, 104 niños sin antecedentes de afecciones pulmonares recurrentes, fueron incluidos como controles. Se compararon las características demográficas y clínicas de ambos grupos. RESULTADOS: en el 84,1 % de los niños con afecciones pulmonares recurrentes se identificó alguna causa subyacente; prevalecieron el asma, las inmunodeficiencias, las bronquiectasias y la enfermedad por reflujo gastroesofágico. El antecedente de ser bajo peso al nacer tuvo asociación con la recurrencia de las afecciones pulmonares, mientras que la presencia de tabaquismo intradomiciliario se asoció a la no recurrencia. CONCLUSIONES: un adecuado enfoque diagnóstico de los niños con afecciones pulmonares recurrentes puede ser realizado a partir de la integración de los niveles de atención sanitaria, y debe tener como eje central la búsqueda de las causas subyacentes responsables de la recurrencia de esas afecciones.

INTRODUCTION: the studies conducted on recurrent pulmonary illnesses in children are scanty; the highest percentage of them has been made in settings other than the primary health care and data on the underlying causes and the risk factors for these illnesses are inconsistent. OBJECTIVE: to analyze the underlying causes and the possible risk factors of recurrent pulmonary illnesses in children. METHODS: a case-control study was conducted in which 63 children, previously selected by physicians at the primary health care, were examined on the basis of an algorithm for clinical diagnosis in a specialized service for chronic recurrent pneumopathies. It was carried out in Sancti Spiritus province from January 1st 2011 to April 30th 2014. One hundred and four children without a history of recurrent pulmonary problems were included as controls in the same period of time. The demographic and clinical characteristics of both groups were then compared. RESULTS: in the study group, 84.1 % of children showed some underlying cause for recurrent pulmonary illnesses; asthma, immunodeficiencies, brochiectasy and gastroesophageal reflux disease prevailed. Being a low birthweighed infant was associated to recurrence in pulmonary illnesses whereas smoking at home was associated to non-recurrence. CONCLUSIONS: adequate diagnosis of children with recurrent pulmonary illnesses may be made on the basis of integration of all the health care levels and should be focused on identifying the underlying causes of recurrence.

Malformación congénita de la vía aérea pulmonar / Congenital pulmonary airway malformation

La malformación congénita de la vía aérea pulmonar, anteriormente llamada malformación adenomatoidea quística pulmonar, es una rara anormalidad en el desarrollo de las vías respiratorias terminales. Las lesiones son de distribución y tamaños variables, usualmente unilaterales. El diagnóstico puede realizarse desde el periodo prenatal mediante ecografía gestacional, encontrándose en ocasiones, graves repercusiones fetales como hidrops fetal, el cual es el mayor predictor de muerte. En los recién nacidos la enfermedad se manifiesta con dificultad respiratoria aguda; en los niños y adultos con infecciones pulmonares recurrentes y complicaciones como absceso pulmonar, hemoptisis, neumotórax y manifestaciones extrapulmonares. En pacientes sintomáticos está indicado el tratamiento quirúrgico para prevenir infecciones y la transformación neoplásica; sin embargo, sigue siendo controversial el tratamiento profiláctico frente al tratamiento expectante en pacientes asintomáticos. Se presenta el caso de una paciente femenina de cuatro años de edad con infecciones pulmonares recurrentes; las radiografías de tórax y la tomografía pulmonar sugieren malformación adenomatoidea quística, la cual es confirmada posteriormente por estudio patológico.

Congenital pulmonary airway malformation, previously known as congenital cystic adenomatoid malformation, is a rare developmental anomaly of the terminal respiratory structures. Cysts can vary in size, distribution, and are most commonly unilateral. The diagnosis can be made prenatally using ultrasound scanning; the fetal lesions can be associated with hydrops fetalis, which is the major predictor of death. In newborns, it manifests as acute respiratory distress, in children and adults, manifestations include recurrent lung infections, with complications such as lung abscess, hemoptysis, pneumothorax and extrapulmonary anomalies. Surgical treatment is indicated in symptomatic patients to prevent infections and the potential neoplastic transformation, however prophylactic surgery versus expectant treatment remains controversial in asymptomatic patients. We present a 4 year old female patient with recurrent pulmonary infections whose thoracic x-ray and pulmonary computed axial tomography suggests cystic adenomatoid malformation, subsequently confirmed by pathological study.

Tracheobronchopathia Osteochondroplastica in a 5-year-old Girl.

Tracheobronchopathia osteochondroplastica (TO) is considered an orphan disease with exceptional occurrence in children. We report a 5-year-old female child who was referred to us with chronic cough and recurrent pneumonia. After several investigations, bronchoscopy showed multiple nodules in the tracheobronchial lumen, whose distribution was consistent with TO. The patient was followed for four years, with no change in the pattern of the disease.

Congenital Bronchoesophageal Fistula with Imperforate Anus and Atrial Septal Defect in a 3-Year-Old Child / 소아알레르기및호흡기학회지

Congenital bronchoesophageal fistula (BEF), congenital communication between esophagus and bronchus, is a rare malformation. Benign BEF shows only nonspecific symptoms that may delay a correct diagnosis and proper treatment. This is a case of a 3-year-old boy presenting congenital BEF with imperforate anus and atrial septal defect. He had recurrent pneumonia in the right lower lobe since he was 5 months old. A barium swallow examination showed a communicating fistula between the lower esophagus and the right lower lobe bronchus. Computed tomography scan supported to delineate the course of the fistula. He underwent resection surgery of the fistula and lobectomy of the right lower lobe. He recovered without any postoperative complications.

Malformación adenomatoidea quística pulmonar: importancia del diagnóstico prenatal / Cystic adenomatoid malformation of the lung: importance of prenatal diagnosis

La malformación adenomatoidea quística pulmonar (MAQP)es una anomalía de la vía aérea pulmonar poco frecuente cuyodiagnóstico suele realizarse en el período prenatal medianteecografía. Durante el embarazo, debe realizarse seguimiento ecográfico para valorar el desarrollo pulmonar. Presentamos el caso clínico de una paciente de 4 años con diagnóstico prenatal de MAQP, no confirmado mediante radiografía de tóraxrealizada al nacimiento, lo cual retrasó el diagnóstico definitivo; fue intervenida con 4 años de edad tras haber presentado varias neumonías a repetición. Una radiografía de tórax normal realizada al nacimiento no descarta la presencia de estamalformación, por lo que es necesario realizar una tomografía computarizada a las 4 semanas del nacimiento para confirmar o descartar la MAQP. Una vez diagnosticada, el tratamiento quirúrgico debe ser precoz para evitar complicaciones.

Cystic adenomatoid malformation of the lung is a rare malformation of the lung airway which often performed diagnosed in the prenatal period by ultrasound. Ultrasound monitoring should be performed during pregnancy to assess lung development. We report the case of a 4-year-old patient with prenatal diagnosis of cystic adenomatoid malformation of the lung, not confirmed by chest radiograph at birth. The patient underwent surgery at 4 years of age after diagnosis was made for presenting recurrent pneumonia. A normal chest radiograph at birth does not exclude this malformation and a computerized tomography at 4 weeks of birth must be done to confirm or rule out this anomaly. Once the diagnosis is made, surgical treatment should be prompted to avoid complications.

Hallazgos endoscópicos en niños con neumonía recurrente / Endoscopic findings in children with recurrent pneumonia

Introducción: La neumonía recurrente (NR) es una condición respiratoria frecuente de consulta en los centros de referencia. Numerosas son las causas posibles involucradas en su patogenia; sin embargo, existe escasa información en cuanto a los hallazgos endoscópicos más frecuentes en niños con esta condición. Material y Métodos: Se revisaron retrospectivamente todas las fibrobroncoscopías (FB) practicadas en el Hospital Clínico de la Pontificia Universidad Católica durante el período marzo 1993-febrero 2004. Resultados: Un total de 65 niños con NR (edad promedio: 42 meses, 52% hombres) fueron admitidos para evaluación endoscópica (91% unifocal). Las condiciones médicas de base asociadas más frecuentes fueron: Síndrome Bronquial Obstructivo Recurrente (27), reflujo gastroesofágico (11), inmunodeficiencias (10), asma (8) y daño neurológico (6). Catorce pacientes presentaron más de un hallazgo endoscópico, siendo los más frecuentes: Broncomalacia (36%), bronquios supernumerarios (36%), estenosis bronquial (33%) y bronquio traqueal (10%). Los hallazgos fueron más frecuentes en el lado derecho (63% vs 17%) siendo ésta diferencia significativa (p < 0,01). Veinticuatro niños tenían una evaluación endoscópica completamente normal. El análisis de regresión mostró una estrecha relación entre el sexo masculino y la presencia de una vía aérea anormal (p = 0,008). Finalmente, no se logró establecer relación entre el número de defectos endoscópicos y el número o la localización de las neumonías (p = 0,67 y 0,64 respectivamente). Conclusiones: Se concluye que los niños con NR preferentemente unifocal, presentan una alta incidencia de hallazgos endoscópicos que pueden corresponder a variantes anatómicas normales o patológicas de la vía aérea según la presencia de manifestaciones clínicas.

Introduction: Recurrent pneumonia (RP) is a respiratory condition which is a regular consult at reference centers. The probable causes involved in its pathogenesis are numerous; however, insufficient data is available on the endoscopic findings in children. Materials and Methods: Retrospective review of all fiberbronchoscopies (FB) carried out in the Hospital Clínico de la Pontificia Universidad Católica between March 1993 and February 2004. Results: A total of 65 children with RP (mean age: 42 months, 52% male) were admitted for endoscopic evaluation (91% unifocal). The most frequent associated basal medical conditions were: Recurrent Bronchial Obstructive Syndrome (27), gastroesophageal reflux (11), immunodeficiencies (10), asthma (8) and neurologic damage (6). Fourteen patients presented more than one endoscopic finding, the most frequent being: Bronchomalacia (36%), supernumerary bronchi (36%), bronchial stenosis (33%) and tracheal bronchus (10%). The findings were more frequent in the right lung (63% vs. 17%), this difference being statistically significant (p < 0,01). Twenty-four children had a completely normal endoscopic evaluation. Regression analysis demonstrated a close relationship between male sex and the presence of an abnormal airway (p = 0,008). Lastly, a relationship could not be established between the number of endoscopic defects and the number or location of pneumonias (p = 0,67 and 0,64, respectively). Conclusions: It is concluded that children with RP, primarily unifocal, present a high incidence of endoscopic findings which may correspond to normal or pathologic anatomic variants based on the clinical manifestations.

The Significance of Serologic Allergy Tests in Children with Recurrent Pneumonia / 소아과

PURPOSE: Pneumonia is one of the most common respiratory diseases in hospitalized children, and often recurs. It has been reported that asthma is one of the significant contributing factors to recurrent pneumonia. On the basis of similarities between asthma and recurrent pneumonia, we intended to evaluate the influence of allergy on recurrent pneumonia. METHODS: Seventy one children with recurrent pneumonia were defined as recurrent group(RG) and twenty one children with first episodes of pneumonia were defined as control group(CG). WBC, total eosinophil count, Eosinophilic Cationic Protein(ECP), serum total IgE and specific IgE for eleven common allergens using the CAP system were measured and compared. Recurrent pneumonia was defined as at least two pneumonia episodes in a year, or at least three episodes from birth to the time of our study. Children with previous allergic disorders were excluded. RESULTS: There were no statistical differences in WBC, total IgE, and ECP between the two groups. Total eosinophil count in RG was significantly higher than CG(P=0.021), significantly correlating with ECP(r=0.755, P<0.05) and total IgE(r=0.354, P<0.05). When the cut-off value was 0.35 kU/L in CAP test, positive prevalence of CAP in RG was significantly higher than CG(P=0.009). Also, the positive prevalence of house dust mites group, especially Dermatophagoides farinae(D.f.), was significantly higher in RG(P=0.019, 0.025, respectively). When the cut-off value was 0.7 kU/L in CAP test, only a positive prevalence of house dust mites group was significantly higher in RG than CG (P=0.042). The quantitative levels of specific IgE for Dermatophagoides pteronyssinus(D.p.) and D.f. were very high in RG(P=0.018, 0.028, respectively), significantly correlating with each other(r=0.639, P<0.05). CONCLUSION: Allergic sensitization, especially to house dust mites, is one important contributable factor in recurrent pneumonia in children. Further study, such as bronchial hyperresponsiveness, is needed in children who were sensitized by D.p. or D.f..

Clinical Investigation of Recurrent Pneumonia in Adults: Analysis of Patients From Hallym University Medical Center / 결핵및호흡기질환

BACKGROUND: Recurrent pneumonia in adults is not uncommon. However, there is no domestic data about recurrent pneumonia in adults. Therefore, we investigated the associated diseases and clinical findings of recurrent pneumonia in adults. METHODS: Among 5513 patients who were treated in five teaching hospitals of Hallym medical center?over a 5-year period, we retrospectively reviewed the medical records of the 58 who were compatible with diagnostic criteria of recurrent pneumonia. RESULTS: The number of patients with recurrent pneumonia was 58 (1.05%, 58/5513) during the 5 years. Thirty- seven patients were male and 21 were female. Mean age was 66.4 (+/-14.9) years. Median interval between each pneumonic episode was 18.5 months. Associated diseases were 25 cases of respiratory diseases, 13 of heart diseases, 13 of diabetes mellitus, 7 of lung malignancies, 11 of malignancies other than lung, 7 of neurologic disease, and 8 of miscellaneous diseases. Three cases had no underlying illness. Of the 8 cases with 2 or more times of recurrence, 4 were associated with respiratory diseases, 2 with aspiration pneumonia due to neurologic diseases, 1 with heart disease and 1 with no underlying illness. Recurrent pneumonic episodes affecting the same location were 30 of the total recurrent pneumonic episodes (30/67, 47.8%) and common associated diseases were respiratory diseases including lung malignancies. The etiology of recurrent pneumonia was Streptococcus pneumoniae, methicillin- resistant Staphylococcus aureus, Pseudomonas aeruginosa, Klebsiella pneumoniae, atypical organisms, etc. CONCLUSION: Recurrent pneumonia in adults had a low incidence rate compared with children, but most cases had associated illness. Respiratory diseases including lung cancer were the most common associated illness of recurrent pneumonia.

A Case of Alport Syndrome Associated with Diffuse Esophageal Leiomyomatosis

We report a case of Alport syndrome associated with esophageal leiomyomatosis, presenting as recurrent pneumonia. A 5-year old girl who had a history of cataract visited the out patient clinic with a complaint of recurrent wheezing and respiratory difficulty which had started five months previously. Chest magnetic resonance image(MRI) and esophagography, checked on the suspicion of achalasia, revealed esophageal leiomyomatosis and renal biopsy revealed Alport syndrome. In the pediatric population, this tumor is a rare cause of dysphagia and is often misdiagnosed as an esophageal motility disorder. Although a number of Alport syndrome associated with leiomyomatosis were reported in the literature, this is a second case report presented with recurrent pneumonia in Korea.

Pneumonia de repetição / Recurrent pneumonia

A pneumonia recorrente definida, como 3 ou mais episódios de pneumonia em 1 ano, deve melhor ser discutida entre os pediatras. O achado radiológico de normalidade entre os episódios agudos deve ser bem caracterizado. O autor discute quais os pacientes que realmente necessitam de avaliação clínica e investigação diagnóstica complementar pelo especialista.

The recurrent pneumonia episodes (three or more times a year), must be better dicussed among pediatricians. We emphasize the importance of finding normal chest roentgenograms between acute episodes; so thatit will be adequately charaterized. Therefore the identification of the patiens that really need subsequent clinical evalution and complementary diagnosis investigation by the specialist will be better select.

A Case of Tracheal Bronchus / 소아알레르기및호흡기학회지

Tracheal bronchus is an aberrant bronchus that arises most often from right tracheal wall above the carina. It is a rare congenital anomaly, which is usually asymptomatic but occasionally associated with recurrent pneumonia, chronic bronchitis and bronchiectasis. Anomalies found in association with tracheal bronchi include respiratory(tracheal hypoplasia, tracheal stenosis, cystic lung lesion), gastrointestinal and musculoskeletal systems. Tracheal bronchus has been diagnosed by conventional tomography, bronchography and bronchoscopy in the past. Technical advances have greatly enhanced the utility of this diagnostic modality. So chest CT, even three-dimensional reconstruction, is of particular importance in the evaluation of mediastinal, pleural and lung parenchymal lesions. We experienced a case of duodenal atresia and tracheal bronchus in 6-month-old child who suffered from recurrent pneumonia and dyspnea. So we report with a brief review and its related literatures.

Predisposing Factors of Recurrent Pneumonia in Children / 소아알레르기및호흡기학회지

PURPOSE: While the recurrent pneumonia is a relatively common problem, there are very few studies or reports in the literature on the subject. This study was designed to provide with a systematic approach to evaluation of the patient who presented with recurrent pneumonia. METHODS: We reviewed clinical records of 12,137 patients who had been admitted to Yonsei University Severance Hospital with pneumonia for the period of 10 years from January 1986 to December 1995. Recurrent pneumonia is defined as pneumonia with at least 2 episodes in a year or with at least 3 episodes for the period of 10 years mentioned above. We investigated incidence of recurrent pneumonia, age distribution, number of recurrent episodes. Then we reviewed the interval period of episodes, location of lesions in chest X-ray, associated etiology in 200 patients with recurrent pneumonia who were selected randomly. RESULTS: Among the patients with pneumonia, 840 patients(6.9%) had recurrent pneumoia. The patients with two episodes of pneumonia were in the highest in incidence(66.9%). The intervals between episodes were usually 1 to 3 month(45.2%). The lesions involved most frequently in both lung fields(61%) on simple chest X-ray. Predisposing factors were widely varied. They were in orders of frequency respiratory disease(43.0%), neuromuscular disease(9.5%), anatomic problem(6.5%), immunologic disease(3.5%) and gastrointestinal disease(1.5%). The most frequent predisposing factor among the respiratory diseases was bronchio;itis(24.0%) and asthma(14.0%). CONCLUSION: The key to optimal treatment lies in the prompt detection of the predisposing factors. We emphasize that patients present recurrent pneumonia should be carefully evaluated for possibility of underlying bronchiolitis in children under age two years and asthma in older children.

A Case of Bronchiolar Papilloma / 결핵

A 59-year-old woman was admitted to the hospital because of intermittent fever and right side chest pain. She has a same episode eight months before this entry. Chest CT scan demonstrated ill-defined parenchymal consolidation containing dilated bronchi of right lower lung field, but no endobronchial mass in the bronchial trees. Fiberoptic bronchoscopy seeking the cause of recurrent pneumonia revealed a small, round mass nearly completely obstructing the lumen of basal segmental bronchus of right lower lobe. The diagnosis of bronchiolar papilloma was made from the biopsy specimens of the bronchoscopic examination. The patient was treated with right lower lobectomy because of irreversible secondary changes below the obstructed bronchus. This thoracotomic excision resulted in complete relief of symptoms and the postoperative course was uneventful for 12 months. Here we report a extremely rare tumor with a brief review of literatures.

A Case of Bronchiolar Papilloma / 결핵

A 59-year-old woman was admitted to the hospital because of intermittent fever and right side chest pain. She has a same episode eight months before this entry. Chest CT scan demonstrated ill-defined parenchymal consolidation containing dilated bronchi of right lower lung field, but no endobronchial mass in the bronchial trees. Fiberoptic bronchoscopy seeking the cause of recurrent pneumonia revealed a small, round mass nearly completely obstructing the lumen of basal segmental bronchus of right lower lobe. The diagnosis of bronchiolar papilloma was made from the biopsy specimens of the bronchoscopic examination. The patient was treated with right lower lobectomy because of irreversible secondary changes below the obstructed bronchus. This thoracotomic excision resulted in complete relief of symptoms and the postoperative course was uneventful for 12 months. Here we report a extremely rare tumor with a brief review of literatures.

A case of chronic recurrent obstructive bronchitis associated with gastro-esophageal reflux

No abstract available.