[Pulmonary lobectomy in children: the sooner the better?] / Lobectomía pulmonar en niños: ¿cuanto antes mejor?

AIM OF THE STUDY: Recommendation of early pulmonary resection in asymptomatic congenital pulmonary airway malformations (CPAMs) is based on the presumed compensatory lung growth during the first months of life. Our aim is to analyze the long-term pulmonary function after lobectomy before and after one year of age using spirometry. METHODS: We performed a retrospective review of children who underwent pulmonary lobectomy for CPAM between 2001 and 2016. Patients who were old enough (>5 years) to carry out a spirometry were included in the study and were divided into 2 groups (surgery before or after 12 months of age). Pulmonary function testing values were considered normal if they were >80% of predicted. MAIN RESULTS: Forty-seven patients underwent pulmonary lobectomy for CPAM, 23 of them met the inclusion criteria and prospectively performed a spirometry. Among them, 7 had surgery before and 16 after one year of age (0.1 vs. 2); being both groups comparable in terms of sex, type of CPAM and surgical approach. Time from surgery until pulmonary function testing was longer in patients who had surgery before one year of age (9.1 vs. 4.6 years, p = 0.003). After correcting results by time from surgery until spirometry, a better FEV1/FVC was found in patients who had surgery after one year of age (90% vs. 77%, p = 0.043). CONCLUSION: Although spirometry may be influenced by many other variables, these preliminary results do not support the current recommendation of performing early lobectomy in CPAMs. Further studies are required in order to resolve the best age to perform pulmonary lobectomy.

INTRODUCCION/OBJETIVO: La resección temprana de las malformaciones pulmonares asintomáticas ofrece el beneficio teórico de optimizar el crecimiento pulmonar compensatorio durante la infancia. El objetivo de este estudio es determinar si la lobectomía antes del año de vida se asocia con mejor función pulmonar a largo plazo. MATERIALES Y METODOS: Revisión de pacientes en los que se realizó lobectomía pulmonar desde 2001, incluyendo los que tenían edad suficiente para realizar una espirometría (>5 años). Fueron divididos en dos grupos: lobectomía antes o después de 12 meses de vida. Los parámetros espirométricos se consideraron normales cuando eran mayores del 80% esperado. RESULTADOS: Veintitrés de los 47 pacientes intervenidos cumplieron los criterios de inclusión. Siete fueron intervenidos antes (0,1 ± 0,4 años) y 16 después del año de vida (2 ± 3,6 años), siendo ambos grupos comparables en sexo, tipo de malformación y abordaje quirúrgico. El tiempo de seguimiento desde la cirugía hasta que se realizó la espirometría fue mayor en los pacientes intervenidos antes del año de vida (9,1 vs. 4,6 años, p = 0,003). Tras corregir los resultados por el tiempo de seguimiento, se objetivó un mejor cociente FEV1/FVC en los pacientes intervenidos después del año de vida (90% vs. 77%, p = 0,043). CONCLUSION: Aunque la espirometría puede estar influenciada por otras variables, los datos de nuestro estudio no apoyan la recomendación actual de realizar resección temprana en las malformaciones pulmonares congénitas asintomáticas. Se necesitan estudios prospectivos con mayor número de pacientes para determinar la mejor edad para realizar la lobectomía.

[Long-term results of the early endoscopic treatment of acquired tracheal-subglottic stenosis: 10 years of experience]. / Resultados a largo plazo del tratamiento endoscópico precoz de las estenosis subglóticas y traqueales adquiridas: 10 años de experiencia.

INTRODUCTION: Acquired stenosis of the airway is a common complication after endotracheal intubation. Endoscopic dilation has been accepted as the treatment of choice in cases detected precociously. Our goal is to know the current status of the patients treated in our hospital with endoscopic dilation in the last 10 years. MATERIAL AND METHODS: Retrospective cohort study of patients with subglottic and tracheal acquired stenosis (STAS) early treated endoscopically with balloon dilation at our center in the last 10 years. Bronchoscopy control at 2 weeks, a month, 3 and 6 months post-dilation were performed and later on depending on the symptoms. RESULTS: 32 patient were treated in the period considered. The median age was 4.5 (3-120) months. There were necessary 2.5 (1-5) dilations per patient. All cases were extubated in the operating room or in the following 24 hours. There were no complications during the procedure. Follow-up time was 6 (1-10) years. Only 1 of the 32 patients have had recurrence of stenosis 2 years after, it was secondary to reintubations due to new surgical interventions; which it was dilated successfully. CONCLUSIONS: Early endoscopic dilation in the acquired airway stenosis is a safe and effective long-term procedure. The results support the use of this technique as a treatment of choice in these patients.

INTRODUCCION: La estenosis adquirida de la vía aérea es una complicación frecuente tras la intubación endotraqueal. La dilatación endoscópica ha sido aceptada como tratamiento de elección en los casos detectados de forma precoz. Nuestro objetivo es conocer el estado actual de los pacientes tratados en nuestro centro mediante dilatación endoscópica en los últimos 10 años. MATERIAL Y METODOS: Estudio de cohorte retrospectivo de pacientes con estenosis subglóticas y traqueales adquiridas (ESTA) tratadas endoscópicamente mediante dilatación con balón en nuestro centro en los últimos 10 años. Se realizaron broncoscopias de control a las 2 semanas, al mes, a los 3 y 6 meses postdilatación y posteriormente en función de la clínica. RESULTADOS: Se trataron 32 pacientes de ESTA de reciente aparición en dicho periodo. La mediana de edad fue de 4,5 (3-120) meses. Fueron necesarias 2,5 (1-5) dilataciones por paciente. Todos los pacientes fueron extubados en quirófano o en las 24 horas siguientes al procedimiento. No hubo complicaciones durante los procedimientos ni durante el postoperatorio. El tiempo de seguimiento fue de 6 (1-10) años. Solo 1 de los 32 pacientes presentó recidiva de la estenosis 2 años después que fue secundaria a reintubaciones por nuevas intervenciones quirúrgicas; la cual se dilató nuevamente. CONCLUSIONES: La dilatación endoscópica precoz en las estenosis adquiridas de la vía aérea es un procedimiento seguro y eficaz a largo plazo. Los resultados avalan el uso de esta técnica como tratamiento de elección en estos pacientes.

Exercise-induced asthma in asthmatic children. Predisposing factors.

BACKGROUND: Exercise-induced bronchoconstriction (EIB) has a high prevalence in children with asthma, and this is a common problem, even in case of controlled asthma, because of the high levels of physical activity in the childhood. OBJECTIVES: The aim of our study was to identify factors associated with the development of EIB in children with controlled asthma. METHODS: We studied children evaluated for asthma. A personal and familiar history was collected from each patient to estimate asthma severity, precipitating factors, exercise ability, immunotherapy treatment and atopic familiar disorders. Skin prick tests for inhalant allergens, pulmonary function tests (PFTs) and exercise challenge test (ECT) measurements were realized in every patient. We used the Chi Squared test to compare qualitative variables, the Student's-t test for quantitative variables and a logistic regression analysis to estimate the independent effect of the variables. RESULTS: We evaluated 132 asthmatic patients. Eighty-two, 6 to 14 years old (average 110 +/- 36.9 months), were included in the study. Forty one have coughing or wheezing with exercise at least three months ago, in addition to a positive ECT; 9 of these children had solitary EIB (group A), and 32 (group B) had controlled chronic asthma, 27 intermittent and 5 moderately persistent. Forty one controlled asthmatic children, 39 intermittent, 1 mildly persistent and 1 moderately persistent (group C) had a good tolerance for exercise with a negative ECT. No differences were found in familiar history, asthma severity or evolution time in B vs C group. We found that 35 patients (42,68 %) patients were sensitized to indoor allergens: 24 (58,53 %) were patients suffering EIB and 11 (26,8 %) allowed to group C. Precipitating factors of asthma were in group B: respiratory infections in 19 cases, pollen in 20 and in 10 indoor allergens exposure. In group C: 14 patients had asthmatic symptoms with viral respiratory infections, 32 with pollen and 2 with indoor allergens exposure. A patient from group A had allergy rhinitis after exposure to cats. Allergy to indoor allergens demonstrated an direct association to EIB suffering (p = 0,026). Twenty six patients with allergic asthma followed pollen immunotherapy treatment, 7 of group B (33,3 %) and 19 (59,3 %) of group C. This treatment was inversely associated with EIB suffering (p = 0,048). A logistic regression analysis confirmed the independence of both variables as predisposing and protecting factors in EIB suffering. CONCLUSIONS: Allergy to indoor allergens might be considered a risk factor for EIB. Immunotherapy treatment could be a protective factor against the development of EIB in children with allergic asthma.

[Clinical significance of episodes of apnea in babies]. / Significado clínico de los episodios de apnea en el lactante.

INTRODUCTION: The presence of episodes of apnea (EA) in infants is very alarming and requires rapid, precise determination of the etiology in view of the relationship with the syndrome of sudden death of infants. MATERIAL AND METHODS: We therefore studied 16 patients, aged between 1 and 12 months, admitted to to hospital after having episodes where they stopped breathing for more than 15 seconds, with or without associated bradycardia. Patients with previous or associated convulsions, premature birth or low birth weight for gestational age were excluded from the study. In all cases a prolonged EEG was recorded. RESULTS: The commonest age of presentation of EA was 3-4 months. In one case there was a history of a sudden infant death occurring in a brother. The etiology was determined in 12 cases: gastroesophageal reflex in 5, epilepsy in 3, hyponatremia in 2, drug reaction in 1, bronchiolitis in 1 and hypoglycemia in another. In three cases where no cause was found the EA was not repeated, although apnea monitorization was necessary for several months because of the anxiety of the family. CONCLUSIONS: Of the 3 cases in which EA was a critical epileptic sign, only 2 could be demonstrated on a Holter recording. In one of these, progress was poor and resistant crises of different morphology later occurred. We consider that EA in infants requires a thorough etiological study. Cases of unknown etiology require prolonged EEG recording to determine whether the apnea is cerebral in origin, followed by prolonged monitorization of apneas to avoid possible sudden death.

Histiocytic cytophagic panniculitis: report of a case in a 12-year-old girl.

We report a 12-year-old girl with nodular skin lesions, fever, pancytopenia, coagulation defects and a fatal outcome within 4 months. Histopathology revealed proliferation of histiocytes with haemophagocytosis in skin, perivisceral fat and haemopoietic organs. The symptoms were most consistent with cytophagic histiocytic panniculitis.

[3 patients with maple syrup urine disease]. / Estudio de tres pacientes con la enfermedad de jarabe de arce.

Clinical manifestations, biochemistry and treatment of three patients with the inherited alteration of the so called maple syrup urine disease are presented. Diagnosis was based upon both detection of high concentrations of leucine, isoleucine, valine and their corresponding alpha-ketoacids in plasma and urine, and existence of a peculiar odor as well as an intense, progressive, neurological alteration. Considering the early onset of clinical symptoms, low protein tolerance and very reduced rate of leucine decarboxylation in cultured fibroblasts, the three patients exhibited grade I, classical type of this disease. None of them responded favorably to suprapharmacological doses of thiamine. Treatment of the patients is discussed and compared with that recommended in the literature.