El granuloma central de células gigantes (GCCG) es una lesión ósea poco frecuente que asienta predominantemente en el hueso maxilar. Su principal tratamiento es la cirugía con márgenes amplios, por lo que en ocasiones condiciona gran morbilidad y alteraciones estéticas. Denosumab, un anticuerpo monoclonal inhibidor de RANK-ligando, se ha presentado como una alternativa terapéutica válida en el tratamiento de estas lesiones.Objetivo: describir la respuesta clínica y radiológica tras tratamiento con Denosumab en un paciente pediátrico con GCCG no resecado.Caso Clínico: adolescente de 12 años que consultó por tumefacción maxilar de 24 h de evolución, sin otra clínica asociada. A la exploración constatamos tumoración en región maxilar superior izquierda con abombamiento de encía ipsilateral. Se realizó una tomografía computarizada (TC) en la que se observó una gran lesión intraósea expansiva en borde alveolar maxilar, solicitándose posteriormente biopsia de la lesión con diagnóstico anatomopatológico de GCCG. Debido al tamaño y localización de la lesión se optó por tratamiento inicial con Denosumab. Tras 10 meses de tratamiento el paciente presentó respuesta clínica y radiológica favorable, con disminución del tamaño lesional y la actividad metabólica. Como efecto adverso asoció hipocalcemia leve, resuelta tras suplementación con calcio.Conclusión: el empleo de Denosumab como primera línea de tratamiento en Granuloma de Células Gigantes puede ser una opción terapéutica adecuada en adolescentes con lesiones difícilmente resecables.

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Sclerosing Mesenteritis (SM) is a rare diagnosis, particularly in pediatric patients, and is typically non-fatal when appropriately treated. Although molecular and immunohistochemical alterations have been described, no pathognomonic signature has been identified for this entity. This report presents a case of a seven-year-old boy who suffered sudden cardiorespiratory arrest. Upon autopsy, he was found to have multicentric SM on the upper mesentery, which led to bowel wall thinning and abdominal bleeding with bacterial translocation. We performed comprehensive morphological, immunohistochemical, and molecular analyses. SM is an atypical disorder with diverse clinical manifestations, including a rare but potentially fatal course. Early diagnosis is critical, given its potential severity. To our knowledge, this is the first case report of pediatric mortality linked to SM. Our findings emphasize the importance of increased awareness and early detection of SM in pediatric patients.

Background: An unbalanced dietary pattern, characterized by high animal protein content: may worsen metabolic control, accelerate renal deterioration and consequently aggravate the stage of the chronic kidney disease (CKD) in pediatric patients with this condition. Aim: to assess the effect of a registered dietitian (RD) intervention on the CKD children’s eating habits. Methods: Anthropometric and dietetic parameters, obtained at baseline and 12 months after implementing healthy eating and nutrition education sessions, were compared in 16 patients (50% girls) of 8.1 (1–15) years. On each occasion, anthropometry, 3-day food records and a food consumption frequency questionnaire were carried out. The corresponding relative intake of macro- and micronutrients was contrasted with the current advice by the European Food Safety Authority (EFSA) and with consumption data obtained using the Spanish dietary guidelines. Student’s paired t-test, Wilcoxon test and Mc Nemar test were used. Results: At Baseline 6% were overweight, 69% were of normal weight and 25% were underweight. Their diets were imbalanced in macronutrient composition. Following nutritional education and dietary intervention 63%, 75% and 56% met the Dietary Reference Values requirements for fats, carbohydrates and fiber, respectively, but not significantly. CKD children decreased protein intake (p < 0.001), increased dietary fiber intake at the expense of plant-based foods consumption (p < 0.001) and a corresponding reduction in meat, dairy and processed food intake was noticed. There were no changes in the medical treatment followed or in the progression of the stages. Conclusions: RD-led nutrition intervention focused on good dieting is a compelling helpful therapeutic tool to improve diet quality in pediatric CKD patients.

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