Impact of 1-Year Supplementation with High-Rich Docosahexaenoic Acid (DHA) on Clinical Variables and Inflammatory Biomarkers in Pediatric Cystic Fibrosis: A Randomized Double-Blind Controlled Trial.

A randomized, double-blind, and placebo-controlled study was conducted to assess the effect of dietary supplementation with high-rich docosahexaenoic acid (DHA) (Tridocosahexanoin-AOX® 70%) at 50 mg/kg/day in pediatric patients with cystic fibrosis (CF) as compared with placebo. The duration of supplementation was 12 months. A total of 22 patients were included, with 11 in the DHA group and 11 in the placebo group. The mean age was 11.7 years. The outcome variables were pulmonary function, exacerbations, sputum cellularity, inflammatory biomarkers in sputum and peripheral blood, and anthropometric variables. In the DHA group, there was a significant increase in FVC (p = 0.004) and FVE1 expressed in liters (p = 0.044) as compared with placebo, and a lower median number of exacerbations (1 vs. 2). Differences in sputum cellularity (predominantly neutrophilic), neutrophilic elastase, and sputum and serum concentrations of resolvin D1 (RvD1), interleukin (IL)-8 (IL-8), and tumor necrosis factor alpha (TNF-α) between the study groups were not found. Significant increases in weight and height were also observed among DHA-supplemented patients. The administration of the study product was safe and well tolerated. In summary, the use of a highly concentrated DHA supplement for 1 year as compared with placebo improved pulmonary function and reduced exacerbations in pediatric CF.

Changes of Erythrocyte Fatty Acids after Supplementation with Highly Concentrated Docosahexaenoic Acid (DHA) in Pediatric Cystic Fibrosis: A Randomized Double-Blind Controlled Trial.

We characterized the fatty acid profiles in the erythrocyte membrane of pediatric patients with cystic fibrosis (CF) receiving highly concentrated docosahexaenoic acid (DHA) supplementation (Tridocosahexanoin-AOX® 70%) at 50 mg/kg/day (n = 11) or matching placebo (n = 11) for 12 months. The mean age was 11.7 years. The DHA group showed a statistically significant improvement in n-3 polyunsaturated fatty acids (PUFAs), which was observed as early as 6 months and further increased at 12 months. Among the n-3 PUFAs, there was a significant increase in DHA and eicosapentaenoic acid (EPA). Additionally, a statistically significant decrease in n-6 PUFAs was found, primarily due to a decrease in arachidonic acid (AA) levels and elongase 5 activity. However, we did not observe any changes in linoleic acid levels. The long-term administration of DHA over one year was safe and well tolerated. In summary, the administration of a high-rich DHA supplement at a dose of 50 mg/kg/day for one year can correct erythrocyte AA/DHA imbalance and reduce fatty acid inflammatory markers. However, it is important to note that essential fatty acid alterations cannot be fully normalized with this treatment. These data provide timely information of essential fatty acid profile for future comparative research.

Long-term outcome of omalizumab-assisted desensitisation to cow's milk and eggs in patients refractory to conventional oral immunotherapy: real-life study.

BACKGROUND: Oral immunotherapy (OIT) is a promising approach to cow's milk and egg allergies, but reactions are frequent and some patients cannot be desensitized. OBJECTIVE: To evaluate long-term OIT outcomes with omalizumab (OMZ) in paediatric patients with severe egg and/or milk allergies. METHODS: This retrospective real-life study analysed findings in children with Immunoglobulin E-mediated allergy to cow's milk and/or hen eggs refractory to conventional OIT, who underwent OIT with OMZ in our department between 1 January 2010 and 31 December 2015. RESULTS: In all, 41 patients were included (median age: 7 years; interquartile range [IQR]: 5.5-9.5); 26/41 (63.4%) underwent OIT for milk, 8/41 (19.5%) for egg and 7/41 (17.1%) for both. The median time between initiation of OMZ and OIT was 27 weeks (IQR: 22-33). Forty (97.56%) patients reached the maintenance phase (200 mL of cow's milk and 30 mL of raw egg or 1 cooked egg) in a median time of 27 weeks (IQR: 18-37). The median total time with OMZ was 117 weeks (IQR: 88-144). During the OMZ period, 2.44% (1/41) of patients presented anaphylaxis. After discontinuation of OMZ, 29.3% (12/41) presented anaphylaxis, 50% of them had a poor adherence to daily ingestion. One patient (2.44%) was diagnosed with eosinophilic esophagitis after 2 years of discontinuation of OMZ. Currently, after a median time of 9 years (IQR: 7-10) since the initiation of OMZ, 75.6% (31/41) are desensitized (27/31 without OMZ). CONCLUSIONS: Omalizumab allows desensitisation of children with severe allergies to cow's milk and/or egg without developing severe reactions while receiving this treatment. However, discontinuation of OMZ leads to severe allergic reactions, and hence must be monitored carefully.

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.

Long-term outcome of omalizumab-assisted desensitisation to cow’s milk and eggs in patients refractory to conventional oral immunotherapy: real-life study

Background: Oral immunotherapy (OIT) is a promising approach to cow’s milk and egg aller-gies, but reactions are frequent and some patients cannot be desensitized. Objective: To evaluate long-term OIT outcomes with omalizumab (OMZ) in paediatric patients with severe egg and/or milk allergies.Methods: This retrospective real-life study analysed findings in children with Immunoglobulin E-mediated allergy to cow’s milk and/or hen eggs refractory to conventional OIT, who under-went OIT with OMZ in our department between 1 January 2010 and 31 December 2015. Results: In all, 41 patients were included (median age: 7 years; interquartile range [IQR]: 5.5–9.5); 26/41 (63.4%) underwent OIT for milk, 8/41 (19.5%) for egg and 7/41 (17.1%) for both. The median time between initiation of OMZ and OIT was 27 weeks (IQR: 22–33). Forty (97.56%) patients reached the maintenance phase (200 mL of cow’s milk and 30 mL of raw egg or 1 cooked egg) in a median time of 27 weeks (IQR: 18–37). The median total time with OMZ was 117 weeks (IQR: 88–144). During the OMZ period, 2.44% (1/41) of patients presented anaphy-laxis. After discontinuation of OMZ, 29.3% (12/41) presented anaphylaxis, 50% of them had a poor adherence to daily ingestion. One patient (2.44%) was diagnosed with eosinophilic esoph-agitis after 2 years of discontinuation of OMZ. Currently, after a median time of 9 years (IQR: 7–10) since the initiation of OMZ, 75.6% (31/41) are desensitized (27/31 without OMZ).Conclusions: Omalizumab allows desensitisation of children with severe allergies to cow’s milk and/or egg without developing severe reactions while receiving this treatment. However, dis-continuation of OMZ leads to severe allergic reactions, and hence must be monitored carefully.© 2022 Codon Publications. Published by Codon Publications (AU)

Respiratory outcomes of "new" bronchopulmonary dysplasia in adolescents: A multicenter study.

OBJECTIVE: Long-term respiratory consequences of bronchopulmonary dysplasia (BPD) in preterm infants born in the post-surfactant era ("new" BPD) remain partially unknown. The present study aimed to evaluate the respiratory outcomes of "new" BPD in adolescents who were born preterm. METHODS: This multicenter, cross-sectional study included 286 adolescents born between 2003 and 2005 (mean age: 14.2 years); among them, 184 and 102 were born extremely preterm (EP; <28 weeks' gestation) and moderate-late preterm (32 to <37 weeks' gestation), respectively. Among EP adolescents, 92 had BPD, and 92 did not. All participants underwent lung function tests, skin prick testing, and questionnaires on asthma symptoms and quality of life. RESULTS: EP adolescents with BPD had significantly lower forced expiratory volume in 1 s (FEV1 ), forced vital capacity (FVC), FEV1 /FVC ratio, and forced expiratory flow between 25% and 75% of FVC than other included adolescents. FEV1 /FVC ratios were below the lower limit of normal (z-score <-1.645) in 30.4% of EP adolescents with BPD, 13.0% of EP adolescents without BPD, and 11.8% of adolescents who were born moderate-late preterm. Bronchodilator response and air-trapping were significantly higher in BPD adolescents than in other adolescents. Diffusion capacity was significantly lower in EP adolescents than in moderate-late preterm adolescents. Asthma symptoms and quality-of-life scores were similar among groups. CONCLUSION: EP adolescents with "new" BPD had poorer pulmonary function than EP adolescents without BPD or moderate-late preterm adolescents. Further studies are needed to determine whether "new" BPD is associated with early-onset chronic obstructive pulmonary disease in adulthood.

Infección bronquial por Pseudomonas aeruginosa en los pacientes con fibrosis quística diagnosticados por cribado neonatal / Bronchial Infection due to Pseudomonas Aeruginosa in Patients with Cystic Fibrosis Diagnosed in Neonatal Screening

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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)