Archives De Pediatrie最新文献

Introduction

Hypoglycemia is a common symptom in pediatrics that can lead to neurological sequelae. The etiologies are mostly benign, but hypoglycemia can be a symptom of severe underlying disease. To streamline the etiological investigations, a “hypoglycemia kit,” containing supplies needed to perform specific analyses quickly, was made available in the pediatric emergency department of the Rouen University Hospital in 2011. Since its introduction, this kit has been used to explore all cases of hypoglycemia regardless of the context. However, although very useful, these analyses are expensive. The objective of our study was to examine the cost-effectiveness of this kit and to refine its indications if necessary.

Methods

This was a non-interventional and retrospective single-center study. Digital records of patients for whom a hypoglycemia kit was used from September 2011 to August 2019 at the pediatric emergency department of Rouen University Hospital were used to retrieve clinical characteristics, laboratory results, and the causes of hypoglycemia.

Results

The study included 82 patients. The etiologic investigation concluded that 74 patients had functional hypoglycemia, and eight cases were attributed to other etiologies. In two cases, the kit led to a diagnosis, i.e., 2.4 % efficiency. A history of congenital malformations or previous hypoglycemia was significantly associated with severe etiologies. However, there was no significant association between hypoglycemia severity, age, sex, and these etiologies.

Conclusion

Our study reveals that the cost-effectiveness of the hypoglycemia kit is low in pediatric emergencies (2.4 %) at Rouen University Hospital, where functional hypoglycemia remains the leading cause of hypoglycemia. However, our results allow us to suggest a decision tree for refining the usability of this kit, which would considerably increase its efficiency.

Background

Suicide is one of the leading causes of death among adolescents. Students from intercultural families (ICFs) are hypothesized to be vulnerable to suicide. This study aimed to identify the current status of depression and suicide in students from ICFs and the risk of suicide according to family type.

Methods

Data from the Korea Youth Risk Behavior Web-based Survey (KYRBS) were used for this study. We selected 586,829 participants from 2011 to 2020. We analyzed the statistical differences between groups using the chi-square and Bonferroni tests. Last, multiple logistic regressions were performed.

Results

Regarding experiencing extreme sadness/desperation and suicidal ideation, the group with both parents born outside Korea had the highest rate (37.1 % and 24.7 %, respectively), followed by the father-only, non-ICF, and mother-only groups. The both-parents group had the highest risk for suicidal plan and attempts, and for suicidal attempts after hospital visits (17.2 %, 14.9 %, and 59.5 %, respectively), followed by the father-only, mother-only, and non-ICF groups. In particular, the both-parents group had 1.74, 3.40, 4.56, and 6.44 times higher odds for suicidal ideation, suicidal plan, and suicidal attempt, and hospital visit after suicidal attempt than the non-ICF group, respectively.

Conclusions

ICF students were more vulnerable to suicide than the non-ICF group, particularly the both-parents and father-only groups. Thus, adolescents from ICFs are a high-risk group for suicide and should be a top priority for intervention.

Background

A number of pediatric conditions are chronic, such as attention-deficit/hyperactivity disorder (ADHD), idiopathic epilepsies, or anxiety disorder. They all have an impact on self-esteem with consequences on the quality of life. Hypnosis is a therapeutic strategy that consists in putting into trance an individual who becomes receptive to appropriate suggestions. Such an approach is now considered a simple and safe therapy with limited cost. The aim of the present study was to show the feasibility of hypnosis for improving self-esteem in children with the aforementioned conditions.

Methods

We conducted a single-center study with prospectively collected data during routine care. Patients with ADHD, idiopathic epilepsies, or anxiety disorder and a low self-esteem were included between April 2018 and February 2020. They all underwent the same hypnosis protocol conducted by the same therapist. Self-esteem was assessed using two self-evaluation scales, the Jodoin 40 scale and Piers–Harris Self-Concept Scale, and a self-assigned self-esteem score at the beginning and at the end of the hypnosis session.

Results

Among the 14 children included, 11 were studied (6 ADHD, 1 anxiety disorder, 4 idiopathic epilepsies). The median age at inclusion was 12.2 years and the sex ratio was 4:3 (boys:girls). Final comparisons showed that self-esteem had improved, which was statistically significant regarding the Jodoin 40 scale and the self-assigned self-esteem score (p ≤ 0.05). Neither side effect nor disease worsening was observed.

Conclusion

This study illustrates the feasibility of therapeutic hypnosis in clinical practice for improving self-esteem in chronic pediatric conditions.

Background

Spinal muscular atrophy (SMA) is a neuromuscular disorder characterized by the degeneration of the anterior horn cells of the spinal cord. Nusinersen for the treatment of SMA has been covered by public healthcare in France since May 2017.

Objective

Our aim was to investigate whether there is a correlation between clinical and compound motor action potential (CMAP) measurements in SMA patients treated with nusinersen after 3  years’ follow-up.

Method

Motor skills were evaluated regularly between M0 and M36 using the Motor Function Measure (MFM) score. CMAP measurements were collected regularly between M0 and M22.

Results

Data for 10 patients with SMA type 2 were collected and divided into two age groups (< 5 years and > 5 years). Motor function improved, but not significantly, regarding distal motor skills (D3) in both groups, and in axial and proximal motor function (D2) in the younger group. CMAP measurements improved in all patients. CMAP increased significantly for the median nerve, and this improvement correlated significantly with global MFM and with axial and proximal tone (D2).

Conclusion

Our study shows gain in distal motor function with nusinersen, especially in younger patients with SMA type 2. These results encourage the screening of SMA patients and treatment as early as possible. CMAP measurements of the median nerve show clear improvement in patients treated with nusinersen and could be performed as routine follow-up.

Aims

This study analyzed the results of a transition program in a patient population with a rare liver disease of pediatric onset.

Method

Data were collected on the clinical course of an adolescent population with a rare disease of pediatric onset and enrolled in a transition program between 1994 and 2022.

Results

A total of 238 adolescents (including 34 having undergone a liver transplant on enrolling in the program) were included. Eight patients were lost to follow-up before the first transition consultation and 16 families requested follow-up in an adult hepatology department closer to their home. Overall, 214 initial transition consultations were carried out; 29 patients were subsequently lost to follow-up and 13 switched center. Overall, 15.4 % of the patients enrolled in our program were lost to follow-up. Five adult patients underwent a liver transplantation during this 28-year period. Overall mortality was 3.2 %, graft survival was 91.5 %, and posttransplant survival was 92 %. In total, the current active file represents 183 patients with a median age of 24.3 years (18–51) and a median follow-up period of 5.8 years (6 months to 28 years).

Conclusion

The implementation of a transition program to adult medicine for adolescents with a rare liver disease should follow the recommendations but must be adapted in line with local practice conditions. This process requires close collaboration between the pediatric and adult medicine teams based on a mutual desire to constantly improve practices and enhance knowledge.

Gastrocolic fistulas are very rare in children and their association with Helicobacter pylori is poorly described. We present the case of a 10-year-old boy with a history of chronic abdominal pain, diarrhea, halitosis, and growth delay diagnosed with H. pylori-associated gastritis and gastrocolic fistula. The boy recovered with resection surgery and antibiotic therapy for eradication of the pathogen. This is the first description of a gastrocolic fistula in pediatrics related to H. pylori.

Objective

In recent years, genomic information and technology have been increasingly integrated into healthcare services for the prevention of genetic diseases, screening, diagnosis, treatment selection, and follow-up of treatment efficacy. The aim of the study was to develop the Scale of Genetic/Genomic Awareness in Pediatric Nurses (SGAPN) and to evaluate its psychometric properties.

Material and methods

This was a methodological study covering the development of the SGAPN and testing its psychometric properties. SGAPN items were developed based on a literature review and were evaluated by content validity. The SGAPN was then tested for internal consistency coefficients, face validity, criterion validity, and construct validity in a convenience sample of 280 pediatric nurses.

Results

The SGAPN is a scale consisting of 32 items. Its Cronbach alpha value was calculated as 0.893. It has a structure with three factors including “genetic/genomic information” (13 items), “genetic transmission information” (7 items), and “genetic education and practices” (12 items). The Cronbach alpha values of the factors were 0.845, 0.600, and 0.893, respectively.

Conclusion

It is suggested that the SGAPN can be used as a valid and reliable scale in the evaluation of the genetic/genomic information, education, and practices of pediatric nurses.

The cases were a pair of siblings with a carnitine palmitoyltransferase (CPT2) deficiency detected by tandem mass spectrometry. Their C16 and C18:1 levels were both within the normal range, while C0 was low, and the (C16+C18:1)/C2 ratio was high. Following genetic testing, a novel CPT2 gene mutation was identified in both patients. The male patient had a normal growth rate during 5 years of follow-up after treatment. By contrast, the female patient did not take l-carnitine supplements and died after an infectious disease-associated illness when she was 1 year old. These data emphasize the need to raise awareness about CPT2 deficiency so as to correctly diagnose and accurately manage the disease.