Transcultural adaptation and validation of IMPACT-III and IMPACT-III-P in Spanish families: a multicenter study from SEGHNP.

IMPACT-III and IMPACT-III-P are health-related quality of life (HRQoL) questionnaires for patients with pediatric inflammatory bowel disease (p-IBD) and their parents/caregivers. We aimed to perform a transcultural adaptation and validation for the Spanish context. Translation, back-translation, and evaluation of the questionnaires were performed by an expert committee and 12 p-IBD families. We recruited p-IBD patients aged 10-17 and their parents/caregivers. Utility, content, and face validity were considered. Validation was performed with Cronbach's alpha coefficient and varimax rotation. We confirmed the adequacy of the factor analysis using Kaiser-Meyer-Olkin (KMO) and Bartlett's sphericity tests. A confirmatory factor analysis was performed using the following goodness indexes: chi-square, Normed Fit Index (NFI), Root Mean Square Error of Approximation index (RMSEA), Standardized Root Mean Square Residual (SRMR), and Comparative Fit Index (CFI). The correlation coefficient between IMPACT-III and IMPACT-III-P was analyzed. We included 370 patients and 356 parents/caregivers (37 hospitals). Both questionnaires had good content and face validity and were considered user-friendly. The KMO measure (0.8998 and 0.9228, respectively) and Bartlett's sphericity test (p-value < 0.001 for both) confirmed the adequacy of the factor analysis. The 4-factor model, complying with Kaiser's criterion, explained 89.19% and 88.87% of the variance. Cronbach's alpha (0.9123 and 0.9383) indicated excellent internal consistency. The CFA showed an adequate fit (NFI 0.941 and 0.918, RMSEA 0.048 and 0.053, SRMR 0.037 and 0.044, and CFI 0.879 and 0.913). The correlation coefficient was excellent (0.92). CONCLUSION: The SEGHNP versions of IMPACT-III and IMPACT-III-P are valid and reliable instruments for Spanish p-IBD families. WHAT IS KNOWN: • IMPACT-III and parent-proxy IMPACT-III (IMPACT-III-P) are useful questionnaires for assessing health-related quality of life (HRQoL) in pediatric inflammatory bowel disease (p-IBD) patients and their parents/caregivers and have been translated and validated in several countries. • To date, no transcultural adaptation and validation of these questionnaires have been published for Spanish patients with p-IBD and their families. WHAT IS NEW: • This is the first transcultural adaptation and validation of IMPACT-III and IMPACT-III-P for Spanish p-IBD families. • These are valid and reliable instruments for assessing HRQoL in Spanish families of patients with p-IBD.

Por fin responde a algo / Finally responds to something

La esofagitis eosinofílica es una enfermedad crónica inmunomediada cuya incidencia está aumentando a lo largo de los últimos años. Afecta preferentemente, pero no de manera exclusiva, a varones y a personas con antecedentes de atopia. Se estima que la prevalencia actual en los países desarrollados supera los 100 casos por cada 100 000 habitantes. Supone la causa más frecuente de disfagia e impactación alimentaria a lo largo de la edad pediátrica, y repercute negativamente sobre la calidad de vida. Afecta más a varones que a niñas. Su primer escalón terapéutico está constituido por: las dietas de exclusión, los inhibidores de la bomba de protones y los corticoides tópicos deglutidos. En casos refractarios, se puede emplear dupilumab, un biológico con capacidad anti-interleukina 4 y 13, con resultados muy prometedores. (AU)

Eosinophilic esophagitis is a chronic immune-mediated disease whose incidence has been increasing in recent years. It preferentially, but not exclusively, affects men and people with a history of atopy. It is estimated that the current prevalence in developed countries exceeds 100 cases per 100,000 inhabitants. It is the most common cause of dysphagia and food impaction throughout the pediatric age, and has a negative impact on quality of life. It affects boys more than girls. Its first therapeutic step consists of: exclusion diets, proton pump inhibitors and swallowed topical corticosteroids. In refractory cases, dupilumab, a biologic with anti-interleukin 4 and 13 capacity, can be used with very promising results. (AU)

Cirugía neuroendoscópica intraventricular en pacientes adultos con hidrocefalia obstructiva. Serie corta de casos

Introducción: la hidrocefalia es una patología neuroquirúrgica que presenta varias alternativas de tratamiento. A pesar de esto, el índice de disfunción y reintervención de los pacientes es considerablemente elevado. Objetivo: describir los resultados de la cirugía neuroendoscópica intraventricular en pacientes operados de hidrocefalia obstructiva. Materiales y métodos: se trata de un estudio observacional, descriptivo, transversal, de serie de casos, en el que se describen variables clínicas, de imagen y quirúrgicas de pacientes operados mediante neuroendoscopia intraventricular. Después de aplicar criterios de inclusión y exclusión, la muestra quedó conformada por cinco pacientes. Resultados: la escala de coma de Glasgow de los pacientes al momento de la cirugía, fue mayor de 12 puntos en todos los casos. El 60 % de los casos presentó un grado 4 en la escala de Frisen para evaluar la gravedad del papiledema. El promedio del índice de Evans medido por tomografía fue de 0,39; el del tercer ventrículo fue de 14,75 mm, mientras que el promedio de los cuernos temporales fue de 4,1 mm. En tres casos se presentaron variedades anatómicas dilatadas de los forámenes de Monro, mientras que en dos presentaban diámetros medianos. El tiempo quirúrgico promedio fue de 45 minutos. Entre las complicaciones posoperatorias estuvo la disfunción del estoma quirúrgico en un paciente con tercer ventriculostomía. Conclusiones: la cirugía neuroendoscópica intraventricular es un método efectivo para tratar pacientes con hidrocefalia obstructiva en pacientes seleccionados.

Introduction: hydrocephalus is a neurosurgical pathology that presents several treatment alternatives. Despite this, the rate of dysfunctions and re-interventions of the patients is considered high. Objective: to describe the results of intraventricular neuro-endoscopic surgery in patients operated on for obstructive hydrocephalus. Materials and methods: this is an observational, descriptive, cross-sectional study, of case series, where clinical, imaging and surgical variables of patients operated by intraventricular neurosurgery are described. After applying inclusion and exclusion criteria, the sample was made up of five patients. Results: the Glasgow coma scale of the patients at the moment of surgery was greater than 12 points in all cases. 60% of the cases presented a grade 4 on Frisen scale to assess the severity of papilledema. The average Evans index, measured by tomography was 0.39; that of the third ventricle was 14.75 mm, while the average of the temporal horns was 4.1 mm. In three cases there were dilated anatomical variants of the foramens of Monro f, while in two they presented medium diameters. The average surgical time was 45 minutes. Surgical stoma dysfunction in one patient with a third ventriculostomy was among the postoperative complications. Conclusions: intraventricular neuro-endoscopic surgery is an effective method for treating patients with obstructive hydrocephalus in selected patients.

Ethylenediamine-assisted solvothermal synthesis of ZnS/ZnO photocatalytic heterojunction for high-efficiency hydrogen production.

Organic-inorganic hybrid materials have emerged as a class of novel materials over the last two decades, as they combine functional organic components and inorganic building blocks into unique materials through various chemical or physical interactions. In the present work, the importance of the use of ethylenediamine in sulfided materials applied to photocatalytic processes in the H2 production is demonstrated. The ZnS/ZnO heterojunction was prepared by the solvothermal synthesis in the presence and absence of ethylenediamine. The photocatalytic behavior showed that the addition of ethylenediamine increases the photocatalytic efficiency up to eight times compared to the photocatalyst without the organic agent. The materials were characterized by X-ray diffraction, scanning electron microscopy, infrared and UV-visible spectroscopies of solids, N2 adsorption-desorption isotherms, X-ray photoelectron spectroscopy, and photoelectrochemical characterization. The ethylenediamine plays a double role: to stabilize the cubic phase of zinc sulfide and to act as a promoter molecule of charge transfer on the surface of ZnS/ZnO/en heterojunction, slowing down the rate of recombination of the electron-hole pair, which is reflected in a decrease in the resistance to transfer of charge carriers, improving the H2 production rate until 1564 µmol h-1 g-1.

Proton Pump Inhibitor Therapy in Pediatric Eosinophilic Esophagitis: Predictive Factors and Long-Term Step-Down Efficacy.

OBJECTIVES: To assess the short- and long-term efficacy of proton pump inhibitor (PPI) therapy for pediatric eosinophilic esophagitis (EoE) in real-world practice with a step-down strategy, and to evaluate factors predictive of PPI responsiveness. METHODS: We collected data regarding the efficacy of PPIs during this cross-sectional analysis of the prospective nationwide RENESE registry. Children with EoE treated with PPI monotherapy were included. Histological remission was defined as a peak eosinophilic count of <15 eosinophils (eos)/high-power field (hpf). Factors associated with PPI responsiveness were identified using multivariate logistic regression analysis. RESULTS: After induction therapy, histological and clinico-histological remission were observed in 51.4% (n = 346) and 46.5% of children, respectively. Normal endoscopic appearance of the esophagus was associated with a higher possibility [odds ratio (OR), 9.20; 95% confidence interval (CI), 2.10-40.16], and fibrostenotic phenotype was associated with a lower possibility (OR, 0.36; 95% CI, 0.18-0.74) of histological remission. Long-term therapy with a step-down strategy effectively maintained histological remission in 68.5% and 85.3% of children at 7 months (n = 108) and 16 months (n = 34), respectively. Complete initial histological remission (≤5 eos/hpf) was associated with a higher possibility of sustained histological remission (OR, 5.08; 95% CI, 1.75-14.68). Adverse events were infrequent and mild. CONCLUSIONS: We confirmed the efficacy of PPIs for a large cohort of children with EoE with sustained histological remission using a step-down strategy. Children with fibrostenotic phenotypes are less likely to respond to induction therapy. Furthermore, patients with complete initial histological remission are more likely to experience long-term histological remission.

Surveillance of osteoarticular infections caused by Staphylococcus aureus in a paediatric hospital in Mexico City.

Staphylococcus aureus is the main aetiologic agent of osteoarticular infections (OAIs) in paediatric patients. The aim of this prospective unicenter study was to describe the phenotypic and genotypic characteristics of S. aureus isolates obtained from OAIs in paediatric patients admitted to tertiary care hospital. Through a surveillance program called OsteoCode, a multidisciplinary team was created and we identified 27 patients with OAIs caused by S. aureus from 2019 to 2021. The susceptibility profile, virulence factors, biofilm formation, pulsed-field gel electrophoresis (PFGE), clonal complex (CC) and sequence type (ST) were determined. In addition, the clinical characteristics and evolution of the patients presented six months after the diagnosis of OAIs were described. Ninety-two percent of the isolates were methicillin-sensitive S. aureus (MSSA). In methicillin-resistant S. aureus (MRSA), SCCmec-II and SCCmec-V were detected. The pvl gene was only observed in MSSA (18.5%) and was associated with highest fever (p=0.015), multiple localization (p=0.017), and soft tissue sites of infection beyond the bone (pyomyositis, pulmonary abscess) (p=0.017). Biofilm formation was detected in 55.6% of isolates. The most common CC were CC5 and CC30 which represent the most common linages for bone and joint infections worldwide. The isolates were distributed in different STs, and ST672 was predominant. MRSA were associated with a longer duration of intravenous treatment and a prolonged hospital stay (p=0.023). Recurrent infection occurred in five children and orthopaedic complications in 33.3% of patients. This is the first study that reflects the epidemiology of S. aureus in OAIs in paediatric patients in Mexico; a clear predominance of MSSA distributed in different STs was observed. Our findings highlight that a multidisciplinary team is required for the diagnosis and treatment of OAIs.

¿Sin salida? / No exit?

El síndrome de obstrucción intestinal distal (SOID) es una complicación digestiva propia de los pacientes con fibrosis quística. Se caracteriza por la acumulación de materia fecal viscosa y secreciones espesas. Afecta a íleon distal, ciego o colon ascendente. Puede derivar en la aparición de obstrucción intestinal total o parcial. Para su tratamiento, se aconseja administrar polietilenglicol oral o a través de una sonda nasogástrica. La cirugía se reserva para los casos refractarios (AU)

Distal intestinal obstruction syndrome (SOID) is a digestive complication specific of patients with cystic fibrosis, characterized by the accumulation of viscous fecal matter and thick secretions. It can lead to the appearance of total or partial intestinal obstruction, affecting the distal ileum, cecum, or ascending colon. For its treatment, it is suggested the administration of oral polyethylene glycol or through a nasogastric tube. Surgery is reserved for refractory cases. (AU)

Ictericia en el lactante: más allá de la atresia de vías biliares / Jaundice in infants: beyond biliary atresia

La colestasis es una entidad que se define por la elevación de bilirrubina conjugada sérica (más de 2 mg/dl o más del 20% del total) y que suele acompañarse de clínica de ictericia, coluria y acolia/hipocolia. Existen múltiples entidades causantes de tal cuadro, pero en el caso de la colestasis neonatal, es fundamental descartar con urgencia la atresia de vías biliares extrahepáticas (AVBE), ya que precisa una intervención quirúrgica de derivación de flujo biliar de forma temprana por sus implicaciones pronósticas futuras. Ante una colestasis neonatal en la que se ha descartado la AVBE, el diagnóstico diferencial se realizará en función de la evaluación de distintos factores, tales como la cifra de gamma-glutamil transferasa (GGT), el valor de los ácidos biliares, si hay o no sospecha de enfermedad metabólica o por la presencia de otras anomalías asociadas. (AU)

Cholestasis is a condition defined by elevated direct bilirubin serum levels (>2 mg/dl or >20% of total bilirubin) and is usually accompanied by jaundice, dark urine, and acholia. Many diseases that cause this condition, but in the case of neonatal cholestasis, it is essential to rule out extrahepatic bile duct atresia (EBVA) on an urgent basis, since it requires early surgical intervention to divert bile flow due to its impact on future outcomes. In the presence of neonatal cholestasis in which EBVA has been ruled out, the differential diagnosis is based on the evaluation of different factors, such as gamma-glutamyl transferase (GGT) and bile acid levels, the suspicion of metabolic disease or the presence of other associated abnormalities. (AU)

Neuromodulación eléctrica en el tratamiento del dolor neuropático refractario. Colocación del primer estimulador medular en Cuba / Electric neuromodulation in the treatment of refractory pain. Placement of the first spinal stimulator in Cuba

El dolor es una causa importante de sufrimiento físico y emocional. El tratamiento médico de los pacientes con dolor crónico refractario es un gran reto. Se presenta el caso de una paciente de 19 años con un cuadro radicular compresivo secundario a Hernia discal L5-S1 derecha, que se le aplicó una discectomía L5-S1 por técnica de Caspar. Al mes de evolución regresa con igual sintomatología. A pesar de múltiples terapias farmacológicas y procederes intervencionistas, el dolor neuropático no mejora, después de múltiples estudios y discusiones en colectivo se determina la posibilidad de la colocación de un neuroestimulador medular, proceder que se lleva a cabo con mejoría considerable de su cuadro doloroso(AU)

Pain is a major cause of physical and emotional suffering. The management of patients with refractory chronic pain is a great challenge. The case is presented of a 19-year-old female patient with compressive radicular symptoms secondary to right L5-S1 disc herniation, who underwent L5-S1 discectomy with Caspar technique. After one month of evolution, she returned with the same symptoms. Despite multiple pharmacological therapies and interventional procedures, the neuropathic pain did not improve. After multiple studies and collective discussions, the possibility of placing a spinal neurostimulator was decided. After the procedure, the patient improved considerably with respect to her painful symptoms(AU)

Duodenitis, la gran desconocida / Duodenitis, the great unknown

Debido a la relación que mantiene el duodeno con el resto de los órganos vecinos, el abordaje de la duodenitis implica realizar un amplio diagnóstico diferencial. Las patologías de este sector del intestino delgado son muy diversas e incluyen procesos congénitos, inflamatorios, traumáticos y neoplásicos. En el presente caso, comentamos una duodenitis erosiva secundaria a una infección por H. pylori (AU)

Due to the relationship of the duodenum with all other adjacent organs, the approach to duodenitis requires a broad differential diagnosis. Diseases in this part of the small intestine are diverse and include congenital, inflammatory, traumatic and malignant processes. Here, we discuss a case of erosive duodenitis secondary to Helicobacter pylori infection. (AU)

Diarrea grave en una lactante previamente sana: un reto diagnóstico / Severe diarrhoea in a previously healthy infant: a diagnostic challenge

Aunque la causa más frecuente de los procesos diarreicos infantiles son las infecciones, ante cuadros graves y prolongados en el tiempo en los lactantes hay que descartar causas congénitas como trastornos genéticos o inmunodeficiencias. La colitis por citomegalovirus (CMV) es una causa poco frecuente de diarrea crónica en niños inmunocompetentes. Por lo general, su curso es leve y autolimitado, por lo que debemos pensar la posibilidad de que exista una inmunodeficiencia en los casos con una evolución más grave. Se recomienda realizar un estudio endoscópico en estos pacientes, precisando tratamiento antiviral aquellos con un curso de la enfermedad más grave o en caso de confirmarse situación de inmunodepresión (AU)

Although the most frequent cause of childhood diarrheal processes is infection, in the case of severe and prolonged symptoms in infants, congenital causes such as genetic disorders or immunodeficiencies must be ruled out. Cytomegalovirus (CMV) colitis is a rare cause of chronic diarrhoea in immunocompetent children. In general, it is a mild and self-limiting disease, so the possibility of immunodeficiency should be considered in cases with a more severe course. Performance of an endoscopic examination is recommended in these patients, and antiviral treatment is required in those with more severe forms of disease or with confirmed immunosuppression. (AU)

Úlcera rectal solitaria secundaria a prolapso rectal recurrente / Solitary rectal ulcer secondary to recurrent rectal prolapse

El síndrome de úlcera rectal solitaria (SURS) es una entidad excepcional en Pediatría, en especial si se acompaña de prolapso rectal recurrente. Es habitual que se confunda con otras entidades, como la enfermedad inflamatoria intestinal (EII), lo que puede conllevar retraso en el diagnóstico. Clínicamente, se manifiesta como tenesmo, urgencia defecatoria, rectorragia leve que puede estar acompañada de moco y sensación de evacuación incompleta. Para el diagnóstico es fundamental la realización de una rectoscopia con toma de biopsias. El tratamiento es controvertido, ya que no existen guías consensuadas para el manejo de esta entidad. La presencia de prolapso recurrente suele requerir cirugía (AU)

Solitary rectal ulcer syndrome (SURS) is a rare disease in paediatrics, especially when it is associated with recurrent rectal prolapse. It can be easily confused with other conditions, such as inflammatory bowel disease (IBD), which can lead to a delayed diagnosis. It manifests in the form of tenesmus, faecal urgency, mild rectal bleeding possibly accompanied by mucus and incomplete evacuation. Performance of a rectoscopy with collection of biopsy samples is essential for diagnosis. Its treatment is still subject to controversy, as there are no consensus guidelines for the management of this disease. If there is recurrent prolapse, surgery is usually required. (AU)

Infección por SARS-CoV-2 en neonatos menores de 28 días. Serie de casos multicéntrica / SARS-CoV-2 infection in infants aged 28 days and younger. A multicentre case series

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Trauma craneoencefálico penetrante por una herida de arpón. Presentación de un caso / Penetrating cranioencephalic trauma due to harpoon wound. Presentation of a case

RESUMEN Las heridas craneocerebrales penetrantes más frecuentes son las provocadas por armas de fuego; las restantes resultan de rara frecuencia. Se presentó un caso que recibió agresión craneoencefálica por arpón, de forma accidental, fuera del agua. Se describieron los detalles del suceso, los exámenes complementarios, la conducta adoptada, el manejo neuroquirúrgico, y la sorprendente evolución postoperatoria del paciente (AU).

ABSTRACT The most frequent penetrating craniocerebral wounds are those caused by firearms; the remaining ones are rare. We presented a case that received accidental cranioencephalic aggression by harpoon, an event that occurred out of the water. Details of the event, complementary examinations, adopted behavior and neurosurgical management that were decided, as well as the surprising post-operative evolution of the patient were described (AU).

Irritabilidad y síndrome de Williams / Irritability and Williams syndrome

El síndrome de Williams es una enfermedad genética poco frecuente que fue descrita por primera vez en 1961. Entre su espectro clínico, destaca la aparición de dismorfia facial, cardiopatía (en especial, estenosis aórtica supravalvular), discapacidad intelectual e hipercalcemia idiopática. En estos niños es frecuente la aparición de irritabilidad en el periodo lactante. Se presenta el caso de un lactante con este problema, y se aprovecha el mismo para hacer el diagnóstico diferencial de sus posibles orígenes (AU)

Williams syndrome is a rare genetic disease that was first described in 1961. Among its clinical spectrum, the appearance of facial dysmorphia, heart disease (especially supravalvular aortic stenosis), intellectual disability and idiopathic hypercalcemia stand out. In these children, irritability in the lactating period is frequent. We report the case of an infant with this problem, and it is used to make the differential diagnosis of its possible origins. (AU)

Complicaciones en la hemorragia intraventricular del recién nacido, a propósito de un caso / Complications in the intraventricular hemorrhage of the newborn, a propos of a case

RESUMEN La hemorragia interventricular es una complicación frecuente en el recién nacido prematuro. Se presentó el caso con el objetivo de describir las complicaciones en la hemorragia intraventricular en el recién nacido. Se trató de una recién nacida, producto de un embarazo de 30 semanas de gestación, con peso al nacer de 1 600 g. Desarrolló una hemorragia intraventricular e hidrocefalia que requirió diferentes intervenciones neuroquirúrgicas y desarrolló complicaciones sépticas graves. La ventriculitis y los abscesos cerebrales fueron las complicaciones más peligrosas. Se realizaron lavados ventriculares. Se utilizó antibióticos intraventriculares y también antibioticoterapia sistémica, cambios frecuentes de catéter de derivación al exterior y permanencia de una derivación ventricular externa por 102 días. Después de seis meses de evolución, de más de veinte intervenciones quirúrgicas, y de haber sufrido severas complicaciones sépticas, se logró realizar la derivación ventrículo-peritoneal definitiva, lográndose su egreso. Al año de vida, la paciente mantiene un desarrollo psicomotor adecuado (AU).

ABSTRACT The interventricular hemorrhage is a frequent complication in the premature newborn baby. The case was presente with the aim of describing the complications of intraventricular hemorrhage in the newborn baby. It dealed with a female newborn baby, product of a 30 weeks pregnancy, with weight at birth of 1 600 g. She developed intraventricular hemorrhage and hydrocephalus that required different neurosurgical interventions and developed serious septic complications. Ventriculitis and brain abscesses were the most dangerous complications. Ventricular lavages were performed. Intraventricular antibiotics were used as well as systemic antibiotic therapy, frequent changes of bypass catheter to the exterior and permanence of an external ventricular bypass for 102 days. And permanence of an external ventricular shunt for 102 days. After six months of evolution, more than twenty surgical interventions, and having suffered severe septic complications, it was possible to perform the definitive ventricular-peritoneal bypass, achieving her discharge. At a year of life, the patient maintains adequate psychomotor development (AU).

Síndrome de Joubert / Joubert syndrome

El síndrome de Joubert es una enfermedad multisistémica poco frecuente. Se caracteriza por una malformación congénita del tronco cerebral e hipoplasia del vermis cerebeloso. Estas alteraciones provocan, entre otros, problemas respiratorios, hipotonía y retraso del desarrollo psicomotor. Puede asociar alteraciones a nivel renal, hepático u ocular, entre otros. No existe un tratamiento curativo de la enfermedad; por ello es importante un diagnóstico precoz, para ofrecer un seguimiento multidisciplinar y así poder mejorar el pronóstico y la calidad de vida de los pacientes y sus familias. (AU)

Joubert syndrome is a rare neurological disorder. It is characterized by the absence or underdevelopment of the cerebellar vermis and a malformed brain stem. These abnormalities cause signs and symptoms that include abnormal breathing patterns, hypotonia and development delay. It may associate kidney, liver or eye abnormalities. Treatment is supportive and depends on the symptoms in each person. This is why early diagnosis is so important, to offer a multidisciplinary strategy for improving the prognosis and quality of life. (AU)