European Journal of Pediatrics最新文献

We aim to evaluate the Osteopontin (OPN) levels in patients with Celiac disease (CD) at diagnosis and exploring its association with mucosal atrophy and osteoporosis. The study included celiac patients at diagnosis and age and sex matched healthy controls. Subjects with additional intestinal pathology, other known inflammatory and autoimmune disease accompanying CD, and patients with signs of infection were excluded. Demographics, presenting symptoms, concurrent disorders, physical examination findings, laboratory results, celiac serology and histopathological assessment were recorded. A total of 36 celiac patients (23 girls, 13 boys, mean age 9.4 ± 4.5 years) and 36 healthy controls (22 girls, 14 boys, mean age 8.7 ± 4 years) were included. The median OPN level was significantly higher in patients [10.41 (9.34-13.47) ng/ml vs. 9.42 (7.56-10.19) ng/ml, p < 0.001]. The median OPN levels of patients with osteoporosis was significantly higher than those with normal BMD values [20.7 (10.12-21.22) vs. 9.87 (9.16-10.75), respectively, p = 0.006)]. A serum OPN level of 10.74 ng/ml was found to be a cut-off value for the patient with osteoporosis with 66.7% sensitivity, 66.7% specificity, 50% positive predictive value, and 80% negative predictive value. Patients' OPN levels were 9.53 (9.3-10.42) ng/ml in Marsh type 3a, 9.78 (8.62-15.28) ng/ml in Marsh type 3b and 11.51 (9.88-19.75) ng/ml in Marsh type 3c. Marsh-Oberhuber type 3c was found to have higher median OPN levels than type 3a and type 3b (p = 0.027). When patients were stratified based on their manifestations of either intestinal or extraintestinal symptoms, along with the presence of anemia, tissue TG IgA levels exceeding ten times the upper normal limit, and a deficiency in vitamin D, the median OPN levels exhibited no significant differences across these groups.

Conclusions:  In conclusion, it is suggested that serum OPN could potentially serve as an indicator of the extent of mucosal atrophy at the initial diagnosis and may have predictive value for osteoporosis.

What is known: • There is currently no routinely utilized marker that can ascertain the extent of histological involvement in celiac disease. • Bone mineral density evaluation is not routinely recommendable at diagnosis in celiac disease, as no clinical predictor is available for low bone mineral density in children.

What is new: • Celiac patients have a higher level of osteopontin at diagnosis. • Osteopontin could potentially serve as an indicator of the extent of mucosal atrophy at the initial diagnosis and may have predictive value for osteoporosis.

The purpose of this study is to evaluate the diagnostic and therapeutic yield of a specialized clinic for children with suspicion of a hereditary thoracic aortic disease (HTAD), including Marfan Syndrome (MFS), and to investigate the diagnostic value of presenting symptoms and findings during evaluation. This retrospective observational study included all patients younger than 18 years old at initial referral between 1998 and 2018. Clinical data, medical treatment, surgical interventions, and clinical events during surveillance were collected until December 2023. A case-control comparison between patients with and without an eventual diagnosis of HTAD was performed using logistic regression analysis to investigate the diagnostic value of collected variables. A total of 355 children were referred and evaluated at the clinic, resulting in 89 new diagnoses, with a diagnostic yield of 21% HTAD, including 59 cases of MFS. Younger age at referral, ectopia lentis, aortic dilatation, and facial features were among the strongest predictors of MFS and other HTAD, while pectus excavatum and arm span-height ratio had no predictive value at childhood age. Of patients with MFS, 65% received antihypertensive medication, and 8% of patients with HTAD underwent prophylactic aortic surgery, in some cases even during childhood. Conclusion: Evaluation of children for HTAD in our specialized Marfan clinic resulted in a high diagnostic yield and subsequent therapeutic implications. Indeed, early recognition of symptoms and signs and referral to such a specialized clinic may lead to early diagnosis, surveillance, and timely treatment, thereby possibly limiting acute aortic events and even mortality.

Distal forearm fractures are the most common pediatric fractures. Currently, the diagnostic reference standard is X-ray. However, there is growing evidence that point-of-care ultrasound can be used for the diagnosis of distal forearm fractures in children and adolescents with good accuracy. The objective of this scoping review was to explore the current evidence for the use of ultrasound for the diagnosis of pediatric distal forearm fractures and to identify the gaps in the literature for further research. We performed a scoping review searching on the following databases PubMed, MEDLINE, SCOPUS, EMBASE, and ClinicalTrials.gov. The main review question was "What is the evidence for using ultrasound to diagnose distal forearm fractures in patients < 18 years old?" All types of studies, including randomized clinical trials and prospective and retrospective observational studies (case-control, cohort, and cross-sectional studies, case series) were included. Twenty-three articles were included in the scoping review; only two articles were from a single randomized controlled trial. Our scoping review found high sensitivity (91.5-99.5%) and specificity (85-99.5%) of POCUS for distal forearm fracture diagnosis. All studies used a linear ultrasound probe, with an upper range frequency ranging from 5 to 15 MHz and typically used a six-view scanning protocol. The duration of the procedure was only a few minutes, and pain associated with ultrasound was usually mild. When compared with X-ray, an ultrasound first approach shortened the length of stay by an average of 15 min per participant. Conclusions: The evidence suggests that ultrasound can be used for the diagnosis of clinically nondeformed distal forearm fractures in children and adolescents by a variety of practitioners. However, the current gaps in the literature include its translation into clinical practice and its cost-effectiveness.

Current professional guidelines enable diagnosing pediatric Celiac Disease (CeD) without a biopsy, when tissue transglutaminase (TTG) IgA antibodies are >  × 10 the upper limit of normal (ULN) and anti-endomysial antibodies (EMA) are positive in a second sample. We compared baseline characteristics and serology normalization in children diagnosed with or without biopsies. A retrospective study of pediatric patients diagnosed with CeD during 2020: group A, no biopsy and group B, biopsy-based diagnosis. Baseline characteristics included demographics, anthropometrics, symptoms, family history, and celiac serology. Follow-up at 6-month intervals, up to 18 months, included dietary compliance, symptoms, and serology. Of 145 children diagnosed with CeD, 42 (29%) and 103 (71%) were from group A and B respectively. Mean age was 7.8 years (range 2.4-17.9 y), 91 (62.8%) females. Baseline symptoms or signs were present in 93 (64.1%) children, without significant difference between the groups. Baseline TTG levels were >  × 10 ULN in all patients in group A and 71 (68.9%) in group B. Among these patients, the rate of TTG decline during follow-up did not differ at any time point between patients diagnosed with and without biopsy, and between patients with and without symptoms. At the last follow-up visit, 24 (57%) children in group A and 46 (65%) in group B had TTG <  × 3 ULN without significant difference between the groups.

Conclusion: Rate of TTG decline did not differ between CeD patients diagnosed with and without biopsy, suggesting that, at least in short term, no biopsy approach may not change patients' adherence and families' attitude towards treatment.

What is known: • Based on current guidelines, there is a rise in the incidence of pediatric celiac disease (CeD) diagnosis without an intestinal biopsy. • There is insufficient data regarding patients' adherence to treatment, including pattern of serology decline, based on the method of CeD diagnosis.

What is new: • Children with CeD have similar baseline characteristics, including presence or absence of symptoms, whether diagnosed with or without biopsies. • During 18-month follow-up, the rate of celiac serology decline, and the reported adherence to treatment, do not differ between patients diagnosed based on biopsy or no biopsy approaches.

Understanding the cardiac risks of antipsychotic use is crucial for clinicians managing psychiatric conditions in children and adolescents. However, the effects on the QT interval in pediatric populations have been poorly investigated. We performed a systematic review to provide clinicians an updated source on the effects of antipsychotic medications on QTc and guide drug's choice. A literature search on the PubMed and Scopus databases was conducted from April 22, 1989, to May 28, 2023, for all studies investigating the effects of antipsychotic medications on the QTc interval in patients aged 0-18 years. A total of 10 articles including 523 patients and 7 different antipsychotic drugs met our search criteria. Among the included articles were three randomized clinical trials, five controlled trials without randomization or prospective comparative cohort trials, and two retrospective cohort studies. Clinical data emerging from these studies were classified according to the Oxford Centre for Evidence-Based Medicine. Risperidone and aripiprazole showed minimal to no significant QTc prolongation, whereas quetiapine, olanzapine, and ziprasidone showed variable effects on QTc. Haloperidol did not demonstrate significant QTc prolongation. In a prospective comparative cohort trial, pimozide exhibited significant QTc prolongation in a cohort with Tourette syndrome.

Conclusion: Only 10 studies have carefully addressed the effect of antipsychotic medications on QTc among pediatric patients, underscoring the need for further research. Personalized risk assessment and regular cardiac monitoring should be integrated into clinical management of pediatric and adolescent patients receiving antipsychotics to facilitate early detection of repolarization abnormalities and potential intervention.

What is known: • QT interval prolongation is a reported side effect of antipsychotics among adults especially with first-generation antipsychotics and parenteral infusion. • Understanding the effect of antipsychotics on QT is essential for clinical monitoring and avoidance of complications.

What is new: • Risperidone and aripiprazole were the most studied antipsychotics in pediatric patients and showed minimal QT prolongation. • The absence of a standardized protocol for assessing the effects on the QT interval makes comparisons between studies challenging and emphasizes the need for further research.

To investigate the impact of different types of exercise modalities on children and adolescents with developmental disorders. Data were obtained from randomized controlled trials retrieved from five databases. Following the PRISMA NMA guidelines, a Bayesian framework-based Markov chain Monte Carlo simulation was used for aggregation and analysis. The included studies were assessed for risk of bias and quality evaluation. A total of 68 studies were included. Moderate-quality evidence suggests that combative sports may be the best exercise for enhancing gross motor skills, ball sports are the most effective for improving executive function, neurodevelopmental motor training is the most effective for improving social skills, and aquatic exercise is the most effective for improving behavioral problems.

Conclusions: Combat sports, ball sports, neurodevelopmental motor training, and aquatic exercise may be effective exercise modalities for improving symptoms in children and adolescents with developmental disorders. However, the degree of improvement can vary among individuals with specific developmental disorders. Therefore, precise assessment of the individual symptoms of children or adolescents is crucial before selecting specific exercise interventions.

Trial registration: PROSPERO (CRD42024545673).

What is known: • Many studies indicate that exercise as an intervention can have positive effects on individuals with developmental disorders, such as ADHD and autism. However, reported effects vary, and there is no clear consensus on the optimal exercise intervention method yet.

What is new: • Through a comprehensive network meta-analysis, various exercise interventions for children and adolescents with developmental disorders were compared to determine the optimal approach. The study found that combat sports, ball sports, neurodevelopmental motor training, and aquatic exercise could potentially be effective modalities for improving symptoms in this population.

The aim of this study was to evaluate the cutaneous manifestations of immunoglobulin A vasculitis (IgAV) in terms of skin lesion type, distribution and persistence and to investigate the relationship between cutaneous manifestations and system involvement and treatment needs. This retrospective observational study was conducted with 489 IgAV patients who were followed-up for at least 6 months between 2013 and 2024. Demographic characteristics, detailed cutaneous manifestations, clinical findings and treatments were retrieved from electronic medical records. IgAV patients were divided into subgroups according to the presence or absence of vesicles/bullae, necrosis/ulcer, rash spreading above the buttocks and persistence. The groups were analyzed statistically for demographic findings, systemic involvement and treatments. Of 489 patients, 36 (7.4%) had vesicles/bullae and 22 (4.5%) had necrosis/ulcers. 345 (70.6%) patients had cutaneous manifestations limited to the lower extremities and buttocks, 144 (29.4%) had cutaneous manifestations spreading from the lower extremities to the trunk, upper extremities or face. 36 (7.4%) patients had persistent rash for more than 1 month. Patients with necrosis/ulcer had more genital tract involvement (p = 0.04). Patients with rash spreading above the buttocks had more gastrointestinal tract and genital tract involvement (p = 0.014, p = 0.003). Patients with persistent rash had more renal involvement (p = 0.05). Patients with vesicles/bullae, necrosis/ulcer, rash spreading above the buttocks and persistent rash required more steroid treatment (p = 0.003, p = 0.001, p < 0.001, p = 0.03).

Conclusions: The characteristics of cutaneous manifestations in IgAV patients may be helpful in predicting the course of the disease. The skin lesion type, distribution and persistence of cutaneous manifestations in IgAV are associated with system involvement. In addition, the need for intensive therapy increases in the presence of vesicles/bullae, necrosis/ulcer, rash spreading over the buttocks and persistent rash in IgAV.

What is known: • Palpable purpura localized to the lower extremities is the expected cutaneous manifestation of immunoglobulin A vasculitis.

What is new: • The type, distribution and duration of rash in IgAV have effects on the immunoglobulin A vasculitis disease course. • Genital tract involvement is more common in patients with necrosis/ulcers. • Gastrointestinal tract involvement and genital tract involvement are more common in patients with rash spreading above the buttocks. • Renal involvement is more common in patients with persistent rash.