Pediatric EBV Positive Mucocutaneous Ulceration in Stomach a Rare Entity.

Epstein Barr virus (EBV) related lymphoproliferative diseases may occur in immunocompromised patients or patients with a history of drug use causing immunodeficiency. EBV positive mucocutaneous ulceration in the new classification of lymphoproliferative diseases in 2016 is very rare in children. Involvement occurs in the skin, oral mucosa, and gastrointestinal system. Gastric involvement is very rare in the literature. There is no case of gastric involvement in children. There are no specified modalities in the treatment of EBV positive mucocutaneous ulceration. We presented our pediatric patient with ataxia telangiectasia who presented with abdominal pain and difficulty swallowing and diagnosed with EBV positive mucocutaneous ulceration in the stomach. We started brentuximab vedotin during the treatment process, and complete remission was achieved after 6 cures of treatment. Our patient is the first case of EBV positive mucocutaneous ulceration in the pediatric case series.

Nutritional characteristics of patients with functional constipation aged 4 years and older.

BACKGROUND: Dietary modifications may have role in prevention and treatment of functional constipation. Macronutrient, extrafluid, and fiber intake have been evaluated and the results are conflicting. The aim of our study was to define the nutritional features associated with functional constipation aged 4 years and older. METHODS: This is a cross-sectional descriptive study. Forty-one patients with functional constipation and 55 age-gender matched controls between 4-18 years old were enrolled. Demographic data, duration of breast-feeding, defecation pattern in the first year of life, physical activity, socioeconomic parameters, and anthropometric measurements were noted. Mean daily macronutrient and micronutrient consumption from the 5-day dietary records were calculated by Nutrition Information System - BEBIS 7.2 version. RESULTS: There were no differences between two groups in energy, water, protein, and fiber consumption. However, in 4-7 years old constipated female and male group, the percentage of carbohydrate was higher (P=0.010, P=0.049, respectively) but fat was lower (P=0.011, P=0.032, respectively). All patients except 4-7 years old boys of both groups got less energy than the reference values. The mean daily protein intake was higher than required in the 4-7 years old constipated and control groups. There was no significant difference in fiber consumption between 2 groups. Breastfeeding >18 months was more common in controls (P=0.039). The constipated group used the squatting toilet more frequently (P=0.002). Lower family income (P<0.001) and parental education levels (P<0.001) were associated with FC. CONCLUSIONS: Dietary habits may be a risk factor for functional constipation, especially, in rapid growth period.

Desenlace de 102 pacientes con linfoma de Hodgkin menores de 5 años / Outcome of 102 patients under 5 years of age with Hodgkin lymphoma

Antecedentes. El linfoma de Hodgkin (LH) es uno de los tipos de cáncer pediátrico con más posibilidades de curación. Sin embargo, es raro entre los niños menores de 5 años, y aún se desconocen los factores pronósticos de la supervivencia debido a su baja frecuencia en este grupo etario. Objetivos. El objetivo de este estudio fue evaluar las características clínicas, los esquemas de tratamiento y el desenlace de los pacientes con LH menores de 5 años. Métodos: se realizó una evaluación retrospectiva de los pacientes con diagnóstico de LH entre 1972 y 2013. Todos los pacientes recibieron quimioterapia, con o sin radioterapia. Resultados. Participaron 102 pacientes; la mediana de edad fue de 4 años (intervalo: 2-4,9). La mediana de seguimiento fue de 13 años. Veintitrés pacientes tenían síntomas B, 15 pacientes tenían neoplasia maligna con gran masa tumoral, y los estadios I y II fueron los más frecuentes. Las tasas de supervivencia global (SG) fueron significativamente diferentes según el estadio del cáncer (p = 0,008). Si bien no se observaron diferencias estadísticamente significativas, la presencia de neoplasia maligna con gran masa tumoral y de síntomas B estuvo asociada con un mal pronóstico. Conclusión. En este estudio unicéntrico se incluyó la mayor cantidad de pacientes con LH menores de 5 años. El estadio fue el principal factor de predicción de SG; por otro lado, la presencia de síntomas B y neoplasia maligna con gran masa tumoral también afectó el pronóstico.

Background. Hodgkin's lymphoma (HL) is one of the most curable pediatric cancers, however it is rare among children under five years of age and prognostic factors for survival rate are still unknown due to low frequency in this age group. Objectives. The aim of this study was to evaluate clinical characteristics, treatment regimens, and outcome of patients under five years of age with HL. Methods. Patients diagnosed with HL between 1972 and 2013 were retrospectively evaluated. All patients were treated with chemotherapy with or without radiotherapy. Results. There were 102 patients with a median age of 4 years (range: 2 to 4.9). The median followup time was 13 years. Twenty-three patients had B symptoms, 15 patients had 'bulky disease' and the most common stages were stage I and II. Overall survival (OS) rates were significantly different according to the stage of the cancer (p = 0.008). Although there were no statistically significant differences; the positivity of 'bulky disease' and B symptoms were associated with poor prognosis. Conclusion. Our single-center study included the largest number of patients under five years of age with HL. The stage was the main predictor for OS; on the other hand, the presence of B symptoms and bulky disease has also affected the prognosis.

Outcome of 102 patients under 5 years of age with Hodgkin lymphoma. / Desenlace de 102 pacientes con linfoma de Hodgkin menores de 5 años.

BACKGROUND: Hodgkin's lymphoma (HL) is one of the most curable pediatric cancers, however it is rare among children under five years of age and prognostic factors for survival rate are still unknown due to low frequency in this age group. OBJECTIVES: The aim of this study was to evaluate clinical characteristics, treatment regimens, and outcome of patients under five years of age with HL. METHODS: Patients diagnosed with HL between 1972 and 2013 were retrospectively evaluated. All patients were treated with chemotherapy with or without radiotherapy. RESULTS: There were 102 patients with a median age of 4 years (range: 2 to 4.9). The median followup time was 13 years. Twenty-three patients had B symptoms, 15 patients had 'bulky disease' and the most common stages were stage I and II. Overall survival (OS) rates were significantly different according to the stage of the cancer (p = 0.008). Although there were no statistically significant differences; the positivity of 'bulky disease' and B symptoms were associated with poor prognosis. CONCLUSION: Our single-center study included the largest number of patients under five years of age with HL. The stage was the main predictor for OS; on the other hand, the presence of B symptoms and bulky disease has also affected the prognosis.

Antecedentes. El linfoma de Hodgkin (LH) es uno de los tipos de cáncer pediátrico con más posibilidades de curación. Sin embargo, es raro entre los niños menores de 5 años, y aún se desconocen los factores pronósticos de la supervivencia debido a su baja frecuencia en este grupo etario. Objetivos. El objetivo de este estudio fue evaluar las características clínicas, los esquemas de tratamiento y el desenlace de los pacientes con LH menores de 5 años. Métodos: se realizó una evaluación retrospectiva de los pacientes con diagnóstico de LH entre 1972 y 2013. Todos los pacientes recibieron quimioterapia, con o sin radioterapia. Resultados. Participaron 102 pacientes; la mediana de edad fue de 4 años (intervalo: 2-4,9). La mediana de seguimiento fue de 13 años. Veintitrés pacientes tenían síntomas B, 15 pacientes tenían neoplasia maligna con gran masa tumoral, y los estadios I y II fueron los más frecuentes. Las tasas de supervivencia global (SG) fueron significativamente diferentes según el estadio del cáncer (p = 0,008). Si bien no se observaron diferencias estadísticamente significativas, la presencia de neoplasia maligna con gran masa tumoral y de síntomas B estuvo asociada con un mal pronóstico. Conclusión. En este estudio unicéntrico se incluyó la mayor cantidad de pacientes con LH menores de 5 años. El estadio fue el principal factor de predicción de SG; por otro lado, la presencia de síntomas B y neoplasia maligna con gran masa tumoral también afectó el pronóstico.

Assessment of cardiac function and electrocardiographic findings in patients with Wilson's disease.

BACKGROUND: This study evaluated cardiac function using tissue Doppler echocardiography and assessed electrocardiographic findings in children diagnosed with Wilson's disease. METHOD: Asymptomatic patients with a diagnosis of Wilson's disease (n = 43) were compared to healthy controls (n = 37) that were age and gender matched. RESULTS: The standard electrocardiographic and conventional echocardiographic examinations were similar in both groups. The left ventricular ejection fraction, shortening fraction, and diastolic function were not significantly different between the two groups. The Tei index for mitral lateral, mitral septal, tricuspid lateral, tricuspid septal, and inter-ventricular septum on tissue Doppler echocardiography was higher in the patient group, yet it did not reach statistical significance. Mitral lateral and septal systolic annular velocity values were significantly lower in the patient group when compared to the control group (p = 0.02 and 0.04, respectively). Also, mitral lateral and septal isovolumetric contraction time values were higher in the patient group (p = 0.04). Although the left ventricular values were not significantly different, relative left ventricular wall thickness was higher in the patient group when compared to the control group, and concentric remodelling in the left ventricle was found in 7 (16%) of 42 patients. QT interval (p = 0.02) and P-wave dispersion values (p = 0.04) were significantly higher in the patient group compared to the control group, and these tend to predict arrhythmias. CONCLUSION: Our study based on the tissue Doppler echocardiography assessment indicated a subclinical systolic, rather than diastolic, dysfunction in the myocardium with increased QT interval and P-wave dispersion, despite the young age of the patients and short disease duration.