Cystic and pseudocystic pulmonary malformations in children: Clinico-pathological correlation.

Pulmonary malformations are rare disorders, with cystic and pseudocystic pulmonary malformations (CPPM) the most frequent, and constitute the first cause of lobectomy in children <1 year of age. Morphological overlap of congenital cystic pulmonary lesions might correspond to a spectrum of lesions in which bronchial atresia is a common etiopathogenetic mechanism. We aimed to report the frequency of CPPM resected in a tertiary-level hospital and to evaluate the degree of agreement between presurgical and anatomopathological diagnoses. We studied 44 surgical pieces with a diagnosis of CPPM received at the Pathology Service from 2009 to 2014, resected from 39 patients, 51.3 % males, with a median age of 16.8 months. Up to 69.2% of the patients had adenomatoid malformation of pulmonary airway (AMPA), with type 2 the most frequent (55.5%). Pulmonary sequestration was present in 15.4% of patients; in two cases the diagnosis was an incidental finding during surgery for the repair of a diaphragmatic hernia. Congenital lobar hyperinflation (CLH) occurred in 7.6% cases. Bronchogenic cyst (BC) was present in 7.6% cases. Presurgical and anatomopathological diagnoses in all patients coincided in 71.8% of cases. Kappa coefficient was 0.56 for global concordance in patients with AMPA, and 0.72, 0.64, 0.37 and 0.33 for CLH, BC, and types 1 and 2 AMPA, respectively. This relatively low interobserver agreement could reflect the low reproducibility of diagnoses used in the current nomenclature. Thus, the new nomenclature must be promoted in order to allow for better reproducibility and greater clinico-pathological concordance. The anatomopathological analysis must include the intentional search for bronchial atresia.

Unsuspected Lung Pathology in Autopsies of Children with Cancer.

BACKGROUND: Although pulmonary involvement is common in patients with cancer, its frequency and nature is seldom reported in the medical literature. OBJECTIVE: To determine the frequency and type of lung pathological conditions revealed by autopsy in children with cancer. METHODS: All reports from autopsies performed in children with cancer from 1989 to 2012 in a pediatric hospital were reviewed. RESULTS: In the analyzed period, 118 autopsies (10.2% of all autopsies) corresponded to children who died with cancer; 76 had complete information and were included in the analysis. Children were seen in the Hematology (41 cases) or the Oncology (35 cases) services. Their median age at decease was 7 years (range, 15 days to 16.1 years) and 46.1% were females. Main diagnoses were acute lymphoblastic (31 patients) or myeloblastic (10 patients) leukemias and tumors of the central nervous system (12 patients). A pathological respiratory condition was diagnosed antemortem in 31 (40.8%) patients, and at autopsy in 62 (81.6%) cases. Omitted diagnoses occurred in 58 (76.3%) children, being pneumonia (24 cases) and pulmonary hemorrhage (23 cases) the most frequent omissions. Nine patients had clinically unsuspected tumor infiltration or metastases. CONCLUSIONS: In these children with cancer, more than 80% of autopsies revealed some lung pathology, mainly of infectious or hemorrhagic nature. Thus, pulmonary involvement should be investigated in all children with cancer in a timely and intentional manner.

[Airway resistance and urinary LTE4 in children with bronchopulmonary dysplasia. Preliminary report]. / Resistencia de las vías aéreas y LTE4 urinario en niños con displasia broncopulmonar. Informe preliminar.

OBJECTIVE: To assess airway resistance values and urinary leukotriene E4 (LTE4) concentrations before and after salbutamol inhalation in children with bronchopulmonary dysplasia (BPD). MATERIAL AND METHODS: Children with BPD were cross-sectionally studied to measure airway resistance by the interrupter technique (Rint), before and after inhaling 200 ig salbutamol, and to quantify urinary leukotriene E4 (LTE4) by immunoassay. RESULTS: Thirty one children with BPD (15 females) aged between 3 months and 9 years were studied. Our results showed that LTE4 did not correlate with Rint values (r = 0.12, p = 0.52) even after adjusting by gender, atopy history, steroid use, and gastroesophageal reflux. Likewise, LTE4 did not correlate with the degree of the airway response to salbutamol (r = -0.13, p = 0.50). A strong inverse association between age and Rint (r = -0.58, p < 0.001) was observed. CONCLUSION: We concluded that urinary LTE, did not correlate with airway resistance or with the response to a bronchodilator drug in children with BPD, suggesting that leukotrienes are not involved in airway obstruction in this disease.

[Clinical competence of emergency clinicians in the management of asthmatic exacerbation in children]. / Competencia clínica de médicos de urgencias en la atención de niñios con exacerbación asmática.

UNLABELLED: In spite of its high prevalence, asthma continues to be undertreated worldwide, and a physician's deficient knowledge seems to be the main reason. OBJECTIVES: To measure the degree of physician's knowledge about classification and treatment of asthma exacerbations in children, and to identify physician's factors that might be involved. METHODS: A prospective, observational, cross-sectional and analytical study was carried out. A 13 item questionnaire to evaluate the knowledge about asthma exacerbations was constructed and validated. This instrument was applied to clinicians working at emergency rooms in four secondary-level hospitals. RESULTS: Forty doctors answered the questionnaire, with a median of 38.5% correct answers. Age, time-span working as specialist, years working at emergency rooms, number of courses attended, number of papers on asthma read, and number of asthma patients seen in a weekly basis did not influence the percentage of correct answers. Variables influencing the degree of knowledge were: being pediatrician (38.5% correct answers vs 23.1% in other specialties, p = 0.035) and working at night (50.0 vs 30.8% in other shift p = 0.031). A multiple lineal regression analysis corroborated the influence of these last two variables on the percentage of correct answers. CONCLUSIONS: The questionnaire proved to be a useful tool that fulfilled expected goals. Percentage of correct answers was low among participating physicians, which indicated the need of educative intervention maneuvers to improve medical management of asthma exacerbation.

Day-night fluctuation of pulse oximetry: an exploratory study in pediatric inpatients.

BACKGROUND: Pulse oximetry is a simple and non-invasive procedure widely used nowadays in the clinical practice. However, it is unclear if SpO2 values are constant throughout the 24 hours of the day or have periodic fluctuations. In the present study we evaluated if progressive day-night variations of SpO, values occur in children. MATERIAL AND METHODS: Pulse oximetry (Nonin 2500) was carried out approximately every 2 hours during a 24-hours period in pediatric patients hospitalized due to different diseases but without acute or chronic respiratory diseases. Measurements were analyzed through the cosinor method (sinusoidal curve fitting). RESULTS: A total of 131 patients (23 days to 16 years old) were studied. A sinusoidal fitting of the SpO2 values was accomplished in 84.7% of children. According to these curves, maximal SpO2 values occurred in the late afternoon [4:53 PM (3:49-5:32 PM), median (quartile 1-quartile 3)], while minimal values appeared in the first hours of the day [3:06 AM (2:12-4:08 AM)]. This pattern was the same in sleeping or awake children. More than half of these sinusoidal curves had a period near to 24 hours (between 20 and 28 hours). An additional finding was that maximal and minimal SpO2 values diminished with age (approximately 0.15 and approximately 0.13% SpO2 per year, respectively). In children less than six years old 5th percentile of SpO2 values were 93.8% in the late afternoon and 89.8% in the early hours of the day, while corresponding figures for older children were 91.0% and 88.5%, respectively. CONCLUSIONS: Our results suggested that, regardless of the sleep influence, in most children the SpO2 follows a progressive fluctuation during a 24-hours cycle, a pattern which is suggestive of a circadian rhythm. A prospective study in healthy children is warranted.

Historia natural de la atelectasia pulmonar experimental en perros a tórax cerrado / Natural history of experimental pulmonary atelectasis in dogs with closed thorax

Se estudió el comportamiento del cortocircuito venoarterial (Qs/Qt) en una preparaçäo canina de atelectasia lobar a tórax cerrado. A 10 perros se les ocluyó el bronquio del lóbulo inferior derecho através de broncoscopía rígida, utilizando el globo de una sonda de Foley. A los 15 minutos postatelectasia el QS/Qt llegó a su máximo incremento de 8.2 ñ 3.6% a 29.7 ñ 11.7% (p < 0.05) y la PaO2 a su mayor disminución de 357 ñ 49 mm Hg a 100 ñ 43 mmHg (p < 0.05). A partir de los 45 minutos el Qs/Qt disminuyó y la PaO2 aumentó paulatinamente. Este cambio fué significativo a los 135 minutos (p < 0.05). La estabilidad gasomética y hemodinámica se apreció hasta 3 horas postatelectasia. Dado que los cambios en Qs/Qt y la Pa O2 no fueron secundarios a las modificaciones que ocurreran en gasto cardíaco, se concluye que la historia natural observada de la preparación obedece a vasoconstricción pulmonar hipóxica

Hipercapnea inducida por acetazolamida, su efecto en la circulación pulmonar: estudio experimental / Acetazolamide induced hipercapnea, its effect on the pulmonary circulation: experimental study

Se diseño un modelo de hipercapnea, sin hipoxemia ni acidosis y se evaluaron los efectos del CO2 en la circulación arterial pulmonar. Se estudiaron 6 perros a los que se administró dosis única de acetazolamida (120mg/Kg) para inhibir la acción de la anhidrasa carbónica. Con esta dosis se logró aumentar la PaCO2 de 27.mmHg a 41.mmHg y la PvCO2 de 31.mmHg a 46.mmHg (p<0.01). A lo largo de tres horas la PaCO2 y la PvO2 permanecieron estables y no ocurrió hipoxemia. El pH arterial y el venoso se mantuvieron en promedio en 7.35 ñ 02 y 7.32 ñ.03 respectivamente. Hemodinâmicamente hubo aumento de las resistencias pulmonares totales de 312 ñ 156 a 435 ñ 173 d.s.cm >- (p<0.01) y la presión media de la arteria pulmonar no se modificó. Estos hallazgos aunados a la disminución del índice latido de 20.7 ñ 8.3 a 13.2 ñ 4.8 ml/lat. (p<0.5), sin haber ocurrido cambios en la presión capilar pulmonar, ni en la presión diastólica final del ventrículo derecho nos sugieren posible vasoconstricción pulmonar, la que se atribuye a incremento de la PaCO2 y la PvCO2, en ausencia de otros factores vasoactivos o pasivos