Nutritional Issues in Children with Dysphagia.

(1) Background: Pediatric dysphagia presents significant nutritional challenges, often impacting growth and development due to reduced oral intake, increased nutritional needs, and gastrointestinal complications; (2) Methods: This prospective quasi-experimental study assessed 117 children under 14 years old (20 patients were under 1 year old, 80 were aged 1-7 years, and 17 were older than 7 years), diagnosed with swallowing disorders, to analyze their caloric, macro-, and micronutrient intake and identify potential deficiencies. The severity of dysphagia was established using functional oral intake scales, and dietary records were reviewed over a 3-day period; (3) Results: The study revealed that 39.8% of participants did not meet their total energy expenditure (TEE), highlighting a high prevalence of malnutrition among these children. Furthermore, patients using feeding devices exhibited a significantly lower caloric intake, and over half required significantly modified food textures. After individualized speech therapy and nutritional rehabilitation, participants showed significant improvements in caloric intake, with their energy coverage increasing from 958% to 1198% of the daily requirement. Rehabilitation also improved tolerance to a broader range of food textures; (4) Conclusions: This research underscores the importance of multidisciplinary, individualized nutritional strategies to address the specific challenges of pediatric dysphagia, emphasizing the role of enteral nutrition and therapeutic interventions in improving the quality of life and nutritional outcomes of these children. Further studies are recommended to assess the long-term impact of such strategies.

Clinicopathological characterization of children with dysphagia, family impact and health-related quality of life of their caregivers.

INTRODUCTION: Oropharyngeal dysphagia (DOF) without proper evaluation can be underdiagnosed in certain groups of the pediatricpopulation. Meeting the needs of these patients can lead to an overload of their caregivers. OBJECTIVES: To describe the epidemiological and clinical characteristics of the patients evaluated after starting a monographic DOF clinic (C-DOF) and study whether there are changes at the nutritional level, as well as the burden and impact that caregivers find on quality of life related to health (HRQL). MATERIAL AND METHODS: Descriptive observational study of patients evaluated in a C-DOF from its start-up. To evaluate HRQoL, an ad hoc survey adapted from the Swallowing Quality of Life Questionnaire of the adult population was designed. RESULTS: 103 patients were evaluated, 85.4% presenting some neurological disease. A videofluoroscopic study was performed in 51 patients (49.5%), reporting combined alterations in both the oral and pharyngeal phases in 64.7% of them. There was a directly proportional correlation between the severity of the DOF and the presence of aspirations, as well as with the patient's motor impairment. Regarding the anthropometric evaluation, there was a trend towards improvement in weight z-score (+0.14 SD), height (+0.17 SD) and BMI (+0.16 SD). Out of 46.2% of the caregivers reported that the DOF problem interfered negatively in the basic activities of daily life. The increase in HRQOL, after the evaluation in the monographic DOF clinic, was statistically significant overall. CONCLUSIONS: The monographic DOF clinic provided specialized care, impacting positively at the nutritional status of patients, as well as perceived changes in HRQOL, with a probable impact on caregivers.

Caracterización clínico-patológica de niños con disfagia, impacto familiar y calidad de vida de sus cuidadores / Clinicopathological characterization of children with dysphagia, family impact and health-related quality of life of their caregivers

Introducción: La disfagia orofaríngea (DOF) puede resultar infradiagnosticada en determinados grupos de población pediátrica. Atender las necesidades de estos pacientes puede derivar en una sobrecarga de sus cuidadores. Objetivos: Describir las características epidemiológicas y clínicas de los pacientes evaluados tras la creación de una consulta monográfica de DOF (C-DOF), estudiar cambios a nivel nutricional, así como la repercusión en la calidad de vida relacionada con la salud (CVRS) de los cuidadores. Material y métodos: Estudio observacional descriptivo de los pacientes evaluados en una C-DOF desde su puesta en marcha. Para evaluar la CVRS, se diseña una encuesta ad hoc adaptada del Swallowing Quality of Life Questionnaire de población adulta. Resultados: Se evaluaron 103 pacientes (85,4% con patología neurológica de base). Se realizó estudio videofluoroscópico a 51 pacientes (49,5%), reportando alteraciones combinadas de fases oral y faríngea un 64,7%. Existió una correlación directamente proporcional entre la gravedad de la DOF y la presencia de aspiraciones, así como con la afectación motora del paciente. En cuanto a la evaluación antropométrica, se observó mejoría en z-score de peso (+0,14 DE), de talla (+0,17 DE) y de IMC (+0,16 DE). El 46,2% de los cuidadores refirieron que la DOF interfiere negativamente en las actividades básicas de la vida diaria. El incremento en calidad de vida, tras la evaluación en la C-DOF, de forma global resultó estadísticamente significativo. Conclusiones: La C-DOF proporciona una atención especializada, repercute positivamente en el estado nutricional de los pacientes, así como en los cambios percibidos en la CVRS, con probable impacto en los cuidadores. (AU)

Introduction: Oropharyngeal dysphagia (DOF) without proper evaluation can be underdiagnosed in certain groups of the pediatric population. Meeting the needs of these patients can lead to an overload of their caregivers. Objectives: To describe the epidemiological and clinical characteristics of the patients evaluated after starting a monographic DOF clinic (C-DOF) and study whether there are changes at the nutritional level, as well as the burden and impact that caregivers find on quality of life related to health (HRQOL). Material and methods: Descriptive observational study of patients evaluated in a C-DOF from its start-up. To evaluate HRQOL, an ad hoc survey adapted from the Swallowing Quality of Life Questionnaire of the adult population was designed. Results: 103 patients were evaluated, 85.4% presenting some neurological disease. A videofluoroscopic study was performed in 51 patients (49.5%), reporting combined alterations in both the oral and pharyngeal phases in 64.7% of them. There was a directly proportional correlation between the severity of the DOF and the presence of aspirations, as well as with the patient's motor impairment. Regarding the anthropometric evaluation, there was a trend toward improvement in weight z-score (+0.14 SD), height (+0.17 SD) and BMI (+0.16 SD). Out of 46.2% of the caregivers reported that the DOF problem interfered negatively in the basic activities of daily life. The increase in HRQOL, after the evaluation in the monographic DOF clinic, was statistically significant overall. Conclusions: The monographic DOF clinic provided specialized care, impacting positively at the nutritional status of patients, as well as perceived changes in HRQOL, with a probable impact on caregivers. (AU)

Sirolimus for the Treatment of Juvenile Polyposis in Childhood.

Juvenile polyposis syndrome (JPS) is a rare disease with an autosomal dominant inheritance pattern characterized by the development of multiple hamartomatous polyps in the gastrointestinal tract. The most frequent signs and symptoms are recurrent abdominal pain, rectal bleeding, anemia, and iron deficiency. The treatment of JPS is symptomatic, requiring serial endoscopic polypectomies or intestinal resections in the most severe cases. We describe the clinical case of a patient with JPS with a childhood juvenile polyposis phenotype because of a mutation on the SMAD4 gene, who received treatment with sirolimus successfully.

[Clinicopathological characterization of children with dysphagia, family impact and health-related quality of life of their caregivers]. / Caracterización clínico-patológica de niños con disfagia, impacto familiar y calidad de vida de sus cuidadores.

INTRODUCTION: Oropharyngeal dysphagia (DOF) without proper evaluation can be underdiagnosed in certain groups of the pediatric population. Meeting the needs of these patients can lead to an overload of their caregivers. OBJECTIVES: To describe the epidemiological and clinical characteristics of the patients evaluated after starting a monographic DOF clinic (C-DOF) and study whether there are changes at the nutritional level, as well as the burden and impact that caregivers find on quality of life related to health (HRQOL). MATERIAL AND METHODS: Descriptive observational study of patients evaluated in a C-DOF from its start-up. To evaluate HRQOL, an ad hoc survey adapted from the Swallowing Quality of Life Questionnaire of the adult population was designed. RESULTS: 103 patients were evaluated, 85.4% presenting some neurological disease. A videofluoroscopic study was performed in 51 patients (49.5%), reporting combined alterations in both the oral and pharyngeal phases in 64.7% of them. There was a directly proportional correlation between the severity of the DOF and the presence of aspirations, as well as with the patient's motor impairment. Regarding the anthropometric evaluation, there was a trend toward improvement in weight z-score (+0.14 SD), height (+0.17 SD) and BMI (+0.16 SD). Out of 46.2% of the caregivers reported that the DOF problem interfered negatively in the basic activities of daily life. The increase in HRQOL, after the evaluation in the monographic DOF clinic, was statistically significant overall. CONCLUSIONS: The monographic DOF clinic provided specialized care, impacting positively at the nutritional status of patients, as well as perceived changes in HRQOL, with a probable impact on caregivers.

The New Molecules Are Changing the Course of Pediatric Chronically Active Ulcerative Colitis: A Series of Pediatric Cases.

Chronically active ulcerative colitis (UC) constitutes a challenge in an era where medical therapeutic options have increased while experience with colectomies has decreased. The change in the therapeutic paradigm of the disease means that patients with chronically active UC are being managed waiting to find their therapeutic target. We present 2 cases of children with chronically active UC who did not respond to intravenous steroids nor sequential therapy. A response was obtained with ustekinumab and tofacitinib, 2 drugs widely used in adults but still with little evidence in children. Highlighting the important role of patients and their families helped decision-making, facilitating the work of the medical team. With multidisciplinary management and close follow-up, they have been able to avoid surgery entering complete clinical remission.

Central Venous Sinus Thrombosis in a Boy With Acute Severe Ulcerative Colitis.

Cerebral venous sinus thrombosis (CVST) in childhood is uncommon. Certain diseases predispose patients to CVST, such as inflammatory bowel disease (IBD), which is considered a risk factor for developing thrombosis, which in turn is considered an extraintestinal manifestation of IBD. The use of prophylaxis in certain patients is a controversial topic. We present the case of a 5-years-old child with ulcerative colitis, who presented with transverse sinus thrombosis immediately after colectomy. Considering the recent recommendations on prophylaxis in this disease, our patient and probably many others would benefit from establishing treatment with low-molecular-weight heparin. We believe that these recommendations should be known, with our case serving as an example, given that we are heading in a direction that has so far been controversial.