Airway surface liquid pH in paediatric patients with cystic fibrosis / PH del líquido de la vía aérea en pacientes pediátricos con fibrosis quística

Background: Cystic fibrosis (CF) patients have more acidic airway surface liquid (ASL), which can denature antimicrobial defensins. Induced sputum is non-invasive and is as representative as bronchoalveolar lavage. Objectives: The objectives of this study were to analyse the ASL pH obtained by induced sputum and assess the relationship between clinical features in paediatric CF patients. Methods: This is a prospective observational study in CF paediatric patients. Sputum was induced in a patient by inhaling 4.5% hypertonic saline, the sputum was collected into a sterile container for pathological analysis, and the pH was measured from the liquid part (ASL) in a gas machine. Results: A total of 27 patients were included in the study: mean age (11.96 ± 3.9) years, mean sweat test (99.38 ± 17.76) ng/L, common mutation Del508F (N24, 88.8%), mean FEV1% 91.94% ± –12.6%, Staphylococcus colonization 14 (51.9%), normal chest CT 8 (29.6%), air trapping 12 (44.4%), bronchiectasis 6 (22.2%), and mean ASL pH 6.72 ± 0.06 (n = 15). A significant correlation was found between a higher sweat test and lower ASL pH (R = 0.683, p = 0.005). There were no differences between altered chest CT (p = 0.199) and positive Staphylococcus aureus (p = 0.17). Conclusion: This is the first publication that use induced sputum to obtain the ASL pH in CF patients. The ASL pH in CF patients is usually acidic and correlated with altered transmembrane function conductance. (AU)

Antecedentes: Los pacientes con fibrosis quística (FQ) tienen el líquido de la vía aérea (ASL) ácido, provocando desnaturalización de defensinas antimicrobianas. El esputo inducido es reproducible, no invasivo y la muestra es equiparable al lavado broncoalveolar. Objetivo: Evaluar el pH ASL obtenido por esputo inducido y analizar sus implicaciones clínicas, en niños FQ. Métodos: Estudio observacional prospectivo desarrollado en niños FQ. El esputo se indujo mediante inhalación de solución salina hipertónica 4,5%. Se recolectó el esputo y posterior al análisis patológico el ASL se colocó en una jeringa y se midió pH con una máquina de gases. Resultados: Se incluyeron 27 pacientes: edad (11,96 ± 3,9) años, test del sudor (99,38 ± 17,76) ng/L, variante común Del508F (N24, 88,8%), FEV1% 91,94% ± 12,6%, Staphylococcus 14 (51,9%), TAC pulmonar normal 8 (29,6%), atrapamiento 12 (44,4%), bronquiectasias 6 (22,2%), pH ASL 6,72 ± 0,06 (n = 15). Se observó correlación significativa entre niveles elevados del test del sudor y pH ASL ácido (R = 0,683, p = 0,005). No hubo correlación entre pH ASL y TAC pulmonar alterado (p = 0,199) o S. aureus positivo (p = 0,17). Conclusiones: Esta es la primera publicación que utiliza esputo inducido para medir pH del ASL en FQ. El pH ASL se correlacionó con la alteración del gen de conductancia transmembranal. (AU)

Successful removal of distal persistent foreign body airway with CO2 cryotherapy in a child.

We report a case of a nine-year-old boy with clinical evidence of foreign body (FB) aspiration with 3 months of delay in diagnosis. The bronchoscopy found soft tissue FB with surrounding inflamed granulation tissue at the entrance to the lateral segmental bronchus. Repeated attempts to remove the FB with flexible forceps were unsuccessful due to friable FB and granulation tissue. Ablation of the granulation tissue using nitrous oxide cryotherapy was then successfully performed and the distal and organic FB was extracted. Early diagnosis is important for minimizing granulation tissue development which complicates FB removal. Cryotherapy with a flexible bronchoscope is an option if organic FB cannot be removed using conventional bronchoscopic instrumentation.

Characterization of severe asthma in the pediatric population

Introduction and objectives Relationship between the causal mechanisms of pediatric severe asthma and severity of symptoms would be helpful for developing personalized strategies for treatment and prevention. Materials and methods For this study, 698 medical histories of asthmatics between 6 and 18 years of age were reviewed in a period of 2 years. Variables analyzed were: age, sex, ethnicity, perinatological history, allergy history, asthma predictive index (API), exposure to tobacco, heavy traffic or epithelium, lung function, age of onset of symptoms, hospitalization admissions/PICU, systemic corticosteroids, daily symptoms control, device prescribe for daily control, and adherence. Results A total of 86 children with severe asthma were included (12.3%). Mean age 13.3 +/− 1.86 years, sex ratio1:1, mean age of symptom onset 2.765 +/− 3.06 years, mean IgE 1076.18KU / L +/− 1136, mean eosinophils 604c / mcl +/− 511.9, mean of FEV1 93.15% +/− 16.3. Evidently, 70 children (81.4%) had positive API, 68 (79.1%) rhinitis, 34 (39.5%) atopic dermatitis. 73 (83.9%) sensitized to inhalants and 56 (65.1%) to dermatophagoides, 39 (45.3%) passive smokers, 19 (22.1%) exposure to heavy traffic; 55 (64%) showed symptoms with exercise, 35 (40.7%) had audible wheezing. The mean systemic corticosteroid cycles/year was 3.63 +/− 3.23, mean PICU admissions 0.36 +/− 0.83, mean hospital admissions 4.31 +/− 5.3, average emergency room visits/year 19.44 +/− 16.28. 38 (56.7%) had good adherence, 44 (51%) used an MDI device and 39 (45.3%) used dry powder. Conclusions Children with severe asthma meet the following criteria: premature, positive API, rhinitis, atopic dermatitis, high IgE, eosinophilia, passive smokers, exposure to heavy traffic, decreased lung function, and low adherence to controller medication (AU)

Esclerosi múltiple: un repte diagnòstic a pediatria / Esclerosis múltiple: un reto diagnóstico en pediatría / Multiple sclerosis: a diagnostic challenge in pediatrics

Introducció: l'esclerosi múltiple (EM) és la malaltia desmielinitzant primària del sistema nerviós central (SNC) més comuna. Sol manifestar-se amb episodis de focalitat neurològica amb curs remitentrecurrent. Entre el 2 i el 5% dels casos debuten en la infància. Es descriu un cas de malaltia primària de l'SNC suggestiu d'EM, en què es remarca la importància de fer un bon diagnòstic diferencial i es ressalten els nous criteris diag-nòstics d'EM pediàtrica. Cas clínic: nena de 12 anys que es presenta amb clínica d'una setmana d'evolució d'hipoestèsia en territori trigemi-nal esquerre i extremitat superior dreta. Herpes labial actiu. Vacunada del virus de l'hepatitis B i del virus del papil•loma humà vuit mesos abans. Estudi de neuroimatge amb ressonància magnètica nuclear (RM) cerebral que mostra lesions desmielinitzants a substància blanca en hemisferi cerebral esquerre i hemiprotuberància dreta, algunes amb signes d'activitat. Líquid cefaloraquidi (LCR) sense proteïnoràquia ni pleocitosi, amb bandes oligoclonals d'IgG de síntesi intratecal. Reacció en cadena de polimerasa (PCR) d'herpes virus en LCR negativa. El quadre clínic i les lesions de l'RM orienten al diagnòstic amb alta sospita d'EM. Comentaris: les malalties desmielinitzants de l'SNC plante-gen un ampli diagnòstic diferencial. La presència de serologies positives en sèrum orienta a una malaltia desmielinitzant com l'encefalomielitis aguda disseminada (EMAD) en què és freqüent un mecanisme postinfecciós. La neuroimatge és molt útil en el diagnòstic de les malalties desmielinitzants. Les bandes oligoclonals, els anticossos anti-AQP4/NMO (neuromielitis òptica) i els anticossos anti-MOG (mielina de l'oligodendròcit) poden ser de gran ajuda en el diagnòstic de les malalties desmielinitzants

Introducción. La esclerosis múltiple (EM) es la enfermedad desmielinizante primaria del sistema nervioso central (SNC) más común. Suele manifestarse con episodios de focalidad neurológica con curso remitente-recurrente. Entre el 2 y el 5% de los casos debutan en la infancia. Se describe un caso de enfermedad primaria del SNC sugestivo de EM, remarcando la importancia de hacer un buen diagnóstico diferencial y resaltando los nuevos criterios diagnósticos de EM pediátrica. Caso clínico. Niña de 12 años que se presenta con clínica de una semana de evolución de hipostesia en territorio trigeminal izquierdo y extremidad superior derecha. Herpes labial activo. Vacunada de virus de la hepatitis B y de virus del papiloma humano ocho meses antes. Estudio de neuroimagen con resonancia magnética nuclear (RM) cerebral que muestra lesiones desmielinizantes en sustancia blanca de hemisferio cerebral izquierdo y hemiprotuberancia derecha, algunas con signos de actividad. Líquido cefalorraquídeo (LCR) sin proteinorraquia ni pleocitosis, con bandas oligoclonales de IgG de síntesis intratecal. Reacción en cadena de polimerasa (PCR) de herpes virus en LCR negativa. El cuadro clínico y las lesiones de la RM orientan al diagnóstico con alta sospecha de EM. Comentarios. Las enfermedades desmielinizantes del SNC plantean un amplio diagnóstico diferencial. La presencia de serologías positivas en suero orienta a una enfermedad desmielinizante como la encefalomielitis aguda diseminada (EMAD), en que es frecuente un mecanismo post-infeccioso. La neuroimagen es muy útil en el diagnóstico de las enfermedades desmielinitzantes. Las bandas oligoclonales, los anticuerpos anti-AQP4/NMO (neuromielitis óptica) y los anticuerpos anti-MOG (mielina del oligodendrocito) podrían ser de gran ayuda en el diagnóstico de enfermedades desmielinizantes (AU)

Introduction. Multiple sclerosis (MS) is the most common primary demyelinating disease of the central nervous system (CNS). It usually manifests with episodes of neurological deficits that follow a relapsing-remitting course, and approximately 2-5% of the cases have an onset during childhood. We describe a case of primary demyelinating disease of the CNS suggestive of MS, we emphasize the importance of a proper differential diagnosis, and highlight the new diagnostic criteria for pediatric MS. Case report. A 12-year old girl presented with a one-week history of hypoesthesia in the left trigeminal dermatome and the right upper extremity, and active cold sores. There was history of hepatitis B and human papilloma virus immunization eight months prior. A brain magnetic resonance imaging (MRI) showed demyelinating lesions in the left hemisphere and right medulla oblongata; some of them enhanced with gadolinium. Cerebrospinal fluid (CSF) showed normal protein and absence of pleocytosis, but with oligoclonal bands of intratechal synthesis. Polymerase chain reaction for herpes virus was negative. The clinical and MRI lesions suggested the diagnosis or a primary CNS demyelinating disease, with high suspicion for MS. Comments. Demyelinating diseases of the CNS include a broad differential diagnosis. The presence of positive serology may suggest the diagnosis of acute disseminated encephalomyelitis (ADEM), which is a post-infectious process. Neuroimaging may be very useful in the diagnosis of demyelinating diseases. In addition, oligoclonal bands, anti-AQP4/NMO (neuromyelitis optica) antibodies, and anti-MOG (myelin oligodendrocyte) antibodies may be helpful in the diagnosis (AU)