Anales de pediatria最新文献

Introduction

Survival in paediatric patients with Hodgkin lymphoma (HL) has increased over the last decades. However, these patients are at increased risk of developing late thyroid sequelae due to the treatment with irradiation and alkylating agents.

Methods

We conducted an observational and retrospective study in patients with a diagnosis of HL between 2007 and 2022, in a hospital that is a paediatric oncology reference centre, through the review of electronic health records. We collected data on demographic (age, sex), clinical, radiological and histopathological variables, the dosage of alkylating agents and radiotherapy (RT) and on thyroid disorders using Microsoft Excel®. The data analysis was conducted with SPSS version 17, using the Fisher exact test for qualitative data, a nonparametric test for quantitative data and Kaplan-Meier curves.

Results

Sixty patients received a diagnosis of HL from 2007 to 2022. The median duration of followup was 78.5 months. There were 4 detected cases of hypothyroidism, 5 of thyroid nodules and 1 of subclinical hyperthyroidism. Treatment with RT was significantly associated with the development of hypothyroidism (P=.026), thyroid nodules (P=.01) and thyroid disease overall (P=.003). We estimated that the risk of thyroid disease increased 8-fold with each additional Grey received (hazard ratio, 1.081; 95% CI, 1.014-1.152; P=.017).

Conclusion

Hodgkin lymphoma patients treated with RT are at increased risk of late thyroid disorders, mainly hypothyroidism and malignancy. This risk is greater the higher the RT dosage and the longer the followup. We did not find evidence of an association between the use of alkylating agents and an increase in the risk of thyroid disease.

Cornelia de Lange syndrome (CdLS) is a rare congenital developmental disorder with multisystemic involvement. The clinical presentation is highly variable, but the classic phenotype, characterized by distinctive craniofacial features, pre- and postnatal growth retardation, extremity reduction defects, hirsutism and intellectual disability can be distinguished from the nonclassic phenotype, which is generally milder and more difficult to diagnose. In addition, the clinical features overlap with those of other neurodevelopmental disorders, so the use of consensus clinical criteria and artificial intelligence tools may be helpful in confirming the diagnosis.

Pathogenic variants in NIPBL, which encodes a protein related to the cohesin complex, have been identified in more than 60% of patients, and pathogenic variants in other genes related to this complex in another 15%: SMC1A, SMC3, RAD21, and HDAC8. Technical advances in large-scale sequencing have allowed the description of additional genes (BRD4, ANKRD11, MAU2), but the lack of molecular diagnosis in 15% of individuals and the substantial clinical heterogeneity of the syndrome suggest that other genes and mechanisms may be involved.

Although there is no curative treatment, there are symptomatic/palliative treatments that paediatricians should be aware of. The main medical complication in classic CdLS is gastro-oesophageal reflux, which should be treated early.

Introduction and objective

Several studies have suggested that the hospitalization rate for COVID-19 in children and adolescents may reflect the prevalence of the infection rather than the severity of the disease. The aim of this study was to describe the clinical features of hospitalised paediatric patients with SARS-CoV-2 infection in order to understand if the infection was the reason for admission.

Methods

Retrospective cohort study including patients aged 0 to 18 years with SARS-CoV-2 infection or multisystem inflammatory syndrome in children (MIS-C) admitted to a tertiary care children's hospital in Spain between 01/01/2020 and 12/31/2021.

Results

228 patients were included, corresponding to 150 cases of COVID-related admission (SARS-CoV-2 infection as main cause of hospitalization) and 78 of non-COVID-related admission (SARS-CoV-2 infection unrelated to the hospitalization).

In the group of COVID-related admissions, 58 patients had comorbidities. Forty-nine patients had acute respiratory disease (pneumonia, bronchospasm or bronchiolitis). Multisystem inflammatory syndrome in children was diagnosed in 27 and was significantly more frequent in the first year of the pandemic (wild type virus). Eighty percent of patients with acute respiratory disease needed respiratory support, mostly low-flow oxygen therapy. The severity of the disease was similar in all virus variants. Two patients (both with severe comorbidities) died from COVID-related conditions.

Conclusions

In our study, one third of the patients were admitted with SARS-CoV-2 infection but not because of it. Acute respiratory disease was less frequent and had a better prognosis compared to the adult population, while MIS-C was a major cause of morbidity and hospitalization. The fatality rate was extremely low.

Rates of childhood cancer survival in developed countries have risen to over 80-85%. In consequence, the population of childhood cancer survivors (CCS) has grown considerably. Nevertheless, CCS present a high morbidity and mortality due to cancer or its treatment, with an increased risk of premature mortality, second primary tumors and late side effects, both physical and psychosocial, all of which decrease the quality of life. Long-term follow-up (LTFU) of CCS is recommended to prevent, detect and treat those health problems. Despite the advances achieved, the management of CCS is still not optimal. Among the areas for improvement discussed in this manuscript are: 1) Quantifying the real burden of morbimortality, by implementing new frequency measures (mean cumulative count and cumulative burden), to obtain more accurate assessments, and using simulation models, to determine individual risks. 2) Assessing the impact of risk factors for late side effects, related to the patient, tumor type, treatments, lifestyle, comorbidities, genetics and ageing. 3) Considering the impact of the international harmonisation of long-term follow-up guidelines, to generate homogeneous, evidence-based recommendations and an individualized LTFU. 4) Challenges to LTFU implementation, considering models of care adapted to patient risk and needs, with special attention to the transition to adult-care follow-up. Finally, we comment on the situation of CCS in Spain and consider future prospects for improving the health and quality of life of this population.

Introduction

Disordered Eating Attitudes and Behaviours (DEABs) can impact both the mental and physical health of children. Early detection is crucial to prevent complications and improve outcomes. The Eating Attitudes Test-26 (EAT-26) is a widely used, cost-effective tool for assessing DEABs.

Objective

To evaluate the psychometric properties of the EAT-26 by analysing its factor structure, internal consistency, convergent validity, and measurement invariance across sexes in Spanish schoolchildren.

Method

Validation study in a sample of 718 schoolchildren. The sample was randomly divided into two groups, each with 359 participants, and we carried out an exploratory factor analysis (EFA) and a confirmatory factor analysis (CFA) of the instrument. Subsequently, for the total sample, we assessed the internal consistency by means of the ordinal alpha, the convergent validity with the SCOFF questionnaire and the measurement invariance between the sexes.

Results

The results of the EFA and CFA supported a multidimensional structure of the EAT comprising six factors and 21 items. These factors underlie a second-order model of DEABs. The internal consistency was adequate for most factors. The SCOFF questionnaire showed a moderate convergent validity for most factors. We found strict invariance across the sexes.

Conclusions

The abbreviated EAT-21 scale exhibited modest and promising psychometric properties, making it a suitable instrument for assessing DEABs equitably in both sexes in educational settings.