Pub Date : 2025-02-22DOI: 10.1016/j.arcped.2024.12.006
Léa Bonneau, Maïlys Rupin-Mas, Magali Descamps, Marie Vincent, Bénédicte Romefort, Patrick Van Bogaert
Hutchinson-Gilford syndrome, also known as progeria, is a rare genetic disorder that causes premature and accelerated ageing from the neonatal period. The disease is caused by de novo mutations in the LMNA gene. Patients present with a range of symptoms, including skin, bone, joint and cardiac disorders, as well as characteristic facial dysmorphia. Intracranial hypertension is not a known symptom of this disease. To the best of our knowledge, no case of a patient with Hutchinson-Gilford syndrome presenting with intracranial hypertension without a traumatic context has been reported in the literature to date. This report presents the case of a child diagnosed with Hutchinson-Gilford syndrome who presented with intracranial hypertension at the age of three years, with no secondary cause identified. Long-term treatment with acetazolamide was required to control the intracranial hypertension. We hypothesise that the intracranial hypertension may be related to vascular abnormalities observed in Hutchinson-Gilford syndrome, which may cause a venous drainage dysfunction. To support the hypothesis of a non-random association between intracranial hypertension and Hutchinson-Gilford syndrome, further reports of similar cases in children with the syndrome are required.
{"title":"Intracranial hypertension in a patient with Hutchinson-Gilford progeria syndrome.","authors":"Léa Bonneau, Maïlys Rupin-Mas, Magali Descamps, Marie Vincent, Bénédicte Romefort, Patrick Van Bogaert","doi":"10.1016/j.arcped.2024.12.006","DOIUrl":"https://doi.org/10.1016/j.arcped.2024.12.006","url":null,"abstract":"<p><p>Hutchinson-Gilford syndrome, also known as progeria, is a rare genetic disorder that causes premature and accelerated ageing from the neonatal period. The disease is caused by de novo mutations in the LMNA gene. Patients present with a range of symptoms, including skin, bone, joint and cardiac disorders, as well as characteristic facial dysmorphia. Intracranial hypertension is not a known symptom of this disease. To the best of our knowledge, no case of a patient with Hutchinson-Gilford syndrome presenting with intracranial hypertension without a traumatic context has been reported in the literature to date. This report presents the case of a child diagnosed with Hutchinson-Gilford syndrome who presented with intracranial hypertension at the age of three years, with no secondary cause identified. Long-term treatment with acetazolamide was required to control the intracranial hypertension. We hypothesise that the intracranial hypertension may be related to vascular abnormalities observed in Hutchinson-Gilford syndrome, which may cause a venous drainage dysfunction. To support the hypothesis of a non-random association between intracranial hypertension and Hutchinson-Gilford syndrome, further reports of similar cases in children with the syndrome are required.</p>","PeriodicalId":55477,"journal":{"name":"Archives De Pediatrie","volume":" ","pages":""},"PeriodicalIF":1.3,"publicationDate":"2025-02-22","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143484701","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Pub Date : 2025-02-15DOI: 10.1016/j.arcped.2024.08.004
Sarah Amar, Blaise Corbery, François Bastard, Guillaume Podevin, Françoise Schmitt
Background: Minimally invasive surgery seems particularly suited to adolescents in view of the cosmetic improvements it provides. This study reports on our first experience of single-incision laparoscopic surgery for adjustable gastric banding (SILS-AGB) using a single-trocar approach and evaluates its safety and efficacy as compared with conventional four-trocar laparoscopy.
Methods: The data of adolescent patients who underwent SILS-AGB or conventional laparoscopy for adjustable gastric banding (CL-AGB) between 2014 and 2019 in our center were reviewed. Pre- and postoperative data on weight- and obesity-associated comorbidities were used to assess the efficacy of the surgical intervention. Perioperative and postoperative data on the duration of surgery, use of analgesics, hospital stay, and complications were compared between the SILS and CL groups along with an assessment of scar quality.
Results: Overall, 12 patients, with a mean body mass index (BMI) of 43.3 kg/m-² (37.0-55.5) at surgery were included in the SILS-AGB group and compared with 14 patients who underwent classic laparoscopy (CL-AGB group) and had an initial BMI of 39.5 kg/m-² (32.0-49.8). Median surgery time was 82 min (55-140) in the CL-AGB group and 106 min (75-159) in the SILS-AGB group (p = 0.04). Postoperative recovery was better in the SILS-AGB group with a mean duration of level-3 intravenous analgesia of 1.8 ± 0.4 days (vs. 2.4 ± 0.6 days, p = 0.02) and a median duration of hospitalization of 2 days (2-3) versus 3 days (2-5) (p = 0.0005). Mid-term follow-up showed equivalent efficacy in terms of weight loss, with a mean BMI at 12 months of 38.5 ± 6.0 kg m-1kg m-1² in both groups, and resolution of hyperinsulinism (92.3 % before surgery vs. 48 % at 6 months).
Conclusion: SILS for AGB placement appears to be as safe and effective as CL despite a slightly longer operative time. SILS was associated with faster recovery and better cosmetic results with a single scar.
{"title":"Preliminary experience of single-incision laparoscopic placement of adjustable gastric band in adolescents: Safe and feasible.","authors":"Sarah Amar, Blaise Corbery, François Bastard, Guillaume Podevin, Françoise Schmitt","doi":"10.1016/j.arcped.2024.08.004","DOIUrl":"https://doi.org/10.1016/j.arcped.2024.08.004","url":null,"abstract":"<p><strong>Background: </strong>Minimally invasive surgery seems particularly suited to adolescents in view of the cosmetic improvements it provides. This study reports on our first experience of single-incision laparoscopic surgery for adjustable gastric banding (SILS-AGB) using a single-trocar approach and evaluates its safety and efficacy as compared with conventional four-trocar laparoscopy.</p><p><strong>Methods: </strong>The data of adolescent patients who underwent SILS-AGB or conventional laparoscopy for adjustable gastric banding (CL-AGB) between 2014 and 2019 in our center were reviewed. Pre- and postoperative data on weight- and obesity-associated comorbidities were used to assess the efficacy of the surgical intervention. Perioperative and postoperative data on the duration of surgery, use of analgesics, hospital stay, and complications were compared between the SILS and CL groups along with an assessment of scar quality.</p><p><strong>Results: </strong>Overall, 12 patients, with a mean body mass index (BMI) of 43.3 kg/m<sup>-</sup>² (37.0-55.5) at surgery were included in the SILS-AGB group and compared with 14 patients who underwent classic laparoscopy (CL-AGB group) and had an initial BMI of 39.5 kg/m<sup>-</sup>² (32.0-49.8). Median surgery time was 82 min (55-140) in the CL-AGB group and 106 min (75-159) in the SILS-AGB group (p = 0.04). Postoperative recovery was better in the SILS-AGB group with a mean duration of level-3 intravenous analgesia of 1.8 ± 0.4 days (vs. 2.4 ± 0.6 days, p = 0.02) and a median duration of hospitalization of 2 days (2-3) versus 3 days (2-5) (p = 0.0005). Mid-term follow-up showed equivalent efficacy in terms of weight loss, with a mean BMI at 12 months of 38.5 ± 6.0 kg m<sup>-1</sup>kg m<sup>-1</sup>² in both groups, and resolution of hyperinsulinism (92.3 % before surgery vs. 48 % at 6 months).</p><p><strong>Conclusion: </strong>SILS for AGB placement appears to be as safe and effective as CL despite a slightly longer operative time. SILS was associated with faster recovery and better cosmetic results with a single scar.</p>","PeriodicalId":55477,"journal":{"name":"Archives De Pediatrie","volume":" ","pages":""},"PeriodicalIF":1.3,"publicationDate":"2025-02-15","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143434376","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Background: Abusive Head Trauma (AHT) is a leading cause of morbidity and mortality in infants requiring rapid neuroimaging performance and prognostic rapid diagnosis. The Pittsburgh Infant Brain Injury Score (PIBIS) clinical prediction rule (CPR) was derived to identify infants most likely to present brain injury, whose diagnosis would benefit from head CT. Our study aimed to externally validate the PIBIS CPR in a pediatric French population.
Methods: A retrospective study was conducted in a French pediatric emergency department between 2015 and 2017. We included all consecutive infants who underwent a neurological imaging. Medical data were collected, and PIBIS score was determined, both retrospectively.
Results: We included 129 infants among which 33 cases (including 20 with a diagnosis of AHT). The sensitivity and specificity of the PIBIS CPR were 75.8 % (95 % CI 57.7-88.9) and 61.4 % (51.0-71.2) and negative and positive predictive values 88.1 % (77.8-94.7) and 40.3 % (33.0-48.2). Among the 20 infants with a diagnosis of AHT, 19 (95.0 %) were correctly identified by the PIBIS CPR.
Conclusion: Our external validation study found a lower diagnostic value of the PIBIS CPR than in the original study. This argues for adding biomarkers to improve its performance, notably in the context of suspected AHT.
{"title":"External validation of pittsburgh infant brain injury score in a French pediatric study.","authors":"Cécile Oger, Audrey Grain, Elise Launay, Christèle Gras-Leguen, Fleur Lorton, Pauline Scherdel","doi":"10.1016/j.arcped.2024.11.007","DOIUrl":"https://doi.org/10.1016/j.arcped.2024.11.007","url":null,"abstract":"<p><strong>Background: </strong>Abusive Head Trauma (AHT) is a leading cause of morbidity and mortality in infants requiring rapid neuroimaging performance and prognostic rapid diagnosis. The Pittsburgh Infant Brain Injury Score (PIBIS) clinical prediction rule (CPR) was derived to identify infants most likely to present brain injury, whose diagnosis would benefit from head CT. Our study aimed to externally validate the PIBIS CPR in a pediatric French population.</p><p><strong>Methods: </strong>A retrospective study was conducted in a French pediatric emergency department between 2015 and 2017. We included all consecutive infants who underwent a neurological imaging. Medical data were collected, and PIBIS score was determined, both retrospectively.</p><p><strong>Results: </strong>We included 129 infants among which 33 cases (including 20 with a diagnosis of AHT). The sensitivity and specificity of the PIBIS CPR were 75.8 % (95 % CI 57.7-88.9) and 61.4 % (51.0-71.2) and negative and positive predictive values 88.1 % (77.8-94.7) and 40.3 % (33.0-48.2). Among the 20 infants with a diagnosis of AHT, 19 (95.0 %) were correctly identified by the PIBIS CPR.</p><p><strong>Conclusion: </strong>Our external validation study found a lower diagnostic value of the PIBIS CPR than in the original study. This argues for adding biomarkers to improve its performance, notably in the context of suspected AHT.</p>","PeriodicalId":55477,"journal":{"name":"Archives De Pediatrie","volume":" ","pages":""},"PeriodicalIF":1.3,"publicationDate":"2025-02-14","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143426635","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Pub Date : 2025-02-01DOI: 10.1016/j.arcped.2024.10.006
Ai Tien Nguyen , Sibylle Jalon , Albane Simon , Maurine Jouret , Justin Le Lorier , Véronique Hentgen , Marie-Aliette Dommergues
Scurvy is now considered to be a rare disease in European countries, even among children, but it still exists. We report the case of an 18-month-old boy who was initially hospitalized for a walking disorder and ultimately diagnosed with scurvy. Radiographs were compatible with rickets, but biological analysis ruled out this diagnosis. The vitamin C deficiency was due to an inadequate diet of a young child without any underlying conditions. This child was exclusively breastfed until the age of 17 months and his mother was also likely vitamin C deficient, as she had a diet lacking in fruits and vegetables. Practitioners should become familiar with symptoms of scurvy to prevent the ordering of unnecessary tests and delays in diagnoses.
{"title":"Scurvy in an 18-month-old child mimicking a clinical presentation of rickets","authors":"Ai Tien Nguyen , Sibylle Jalon , Albane Simon , Maurine Jouret , Justin Le Lorier , Véronique Hentgen , Marie-Aliette Dommergues","doi":"10.1016/j.arcped.2024.10.006","DOIUrl":"10.1016/j.arcped.2024.10.006","url":null,"abstract":"<div><div>Scurvy is now considered to be a rare disease in European countries, even among children, but it still exists. We report the case of an 18-month-old boy who was initially hospitalized for a walking disorder and ultimately diagnosed with scurvy. Radiographs were compatible with rickets, but biological analysis ruled out this diagnosis. The vitamin C deficiency was due to an inadequate diet of a young child without any underlying conditions. This child was exclusively breastfed until the age of 17 months and his mother was also likely vitamin C deficient, as she had a diet lacking in fruits and vegetables. Practitioners should become familiar with symptoms of scurvy to prevent the ordering of unnecessary tests and delays in diagnoses.</div></div>","PeriodicalId":55477,"journal":{"name":"Archives De Pediatrie","volume":"32 2","pages":"Pages 138-142"},"PeriodicalIF":1.3,"publicationDate":"2025-02-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142900676","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Pub Date : 2025-02-01DOI: 10.1016/j.arcped.2024.08.009
Claire Koenig Zores , Nicolas Mura , Elodie Rabatel , Meggane Melchior , André Dufour , Pierre Kuhn
Background
Care procedures for preterm infants can induce stress that may disrupt homeostasis, possibly altering cerebral perfusion or oxygenation. We evaluated the physiological and cerebral oxygenation changes during the routine care of very preterm infants.
Methods
We analyzed the changes in heart and respiratory rates and in systemic and regional cerebral oxygen saturation of 27 very preterm infants, defining three care periods of 5 min each: 30 min before care, 30 min during care, and 30 min after care. Mean maximum and minimum values for each parameter during the defined care periods were compared by analysis of variance (ANOVA) for repeated measures.
Results
The mean heart rate was significantly higher during (160 ± 8 bpm) than before and after care (151 ± 21 and 151 ± 6 bpm, respectively). The mean respiratory rate decreased during care and increased afterward: 44 ± 2.2, 40.6 ± 3.2, and 46.7 ± 3.4 cycles/min, respectively (p < 0.05). The mean regional cerebral oxygen and systemic saturation did not vary significantly. Mean minimum and maximum values for each parameter varied during and after care as compared with before care (all p < 0.01). The mean minimum cerebral and systemic saturation was lower after care than before care: 59 ± 8 % versus 63 ± 8 % and 83 ± 3 % versus 90 ± 7 %, respectively (p < 0.05).
Conclusions
During routine care procedures for very preterm infants, the change in physiological parameters suggested an autonomic stress reaction. Cerebral desaturation may occur during and after the care of such infants and call for specific attention to better support the physiological and cerebral well-being of these infants during standard care procedures.
{"title":"Physiological and cerebral hemodynamic changes during routine nursing procedures for very preterm infants","authors":"Claire Koenig Zores , Nicolas Mura , Elodie Rabatel , Meggane Melchior , André Dufour , Pierre Kuhn","doi":"10.1016/j.arcped.2024.08.009","DOIUrl":"10.1016/j.arcped.2024.08.009","url":null,"abstract":"<div><h3>Background</h3><div>Care procedures for preterm infants can induce stress that may disrupt homeostasis, possibly altering cerebral perfusion or oxygenation. We evaluated the physiological and cerebral oxygenation changes during the routine care of very preterm infants.</div></div><div><h3>Methods</h3><div>We analyzed the changes in heart and respiratory rates and in systemic and regional cerebral oxygen saturation of 27 very preterm infants, defining three care periods of 5 min each: 30 min before care, 30 min during care, and 30 min after care. Mean maximum and minimum values for each parameter during the defined care periods were compared by analysis of variance (ANOVA) for repeated measures.</div></div><div><h3>Results</h3><div>The mean heart rate was significantly higher during (160 ± 8 bpm) than before and after care (151 ± 21 and 151 ± 6 bpm, respectively). The mean respiratory rate decreased during care and increased afterward: 44 ± 2.2, 40.6 ± 3.2, and 46.7 ± 3.4 cycles/min, respectively (<em>p</em> < 0.05). The mean regional cerebral oxygen and systemic saturation did not vary significantly. Mean minimum and maximum values for each parameter varied during and after care as compared with before care (all <em>p</em> < 0.01). The mean minimum cerebral and systemic saturation was lower after care than before care: 59 ± 8 % versus 63 ± 8 % and 83 ± 3 % versus 90 ± 7 %, respectively (<em>p</em> < 0.05).</div></div><div><h3>Conclusions</h3><div>During routine care procedures for very preterm infants, the change in physiological parameters suggested an autonomic stress reaction. Cerebral desaturation may occur during and after the care of such infants and call for specific attention to better support the physiological and cerebral well-being of these infants during standard care procedures.</div></div>","PeriodicalId":55477,"journal":{"name":"Archives De Pediatrie","volume":"32 2","pages":"Pages 120-125"},"PeriodicalIF":1.3,"publicationDate":"2025-02-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143061535","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Pub Date : 2025-02-01DOI: 10.1016/j.arcped.2024.12.005
Elsa Riche , Aurelie Morand , Sophie Fruscione , Fabrice Michel , Aurelie Boutin , Valerie Bremond , Valerie Arnoux , Philippe Minodier
Objective
The management of a child presenting with a critical medical or surgical condition is a scarce event in the pediatric emergency department (PED). In this one year retrospective study, we have tried to better characterize the profile and care pathway of children who had been transferred to the neonatal or pediatric intensive care or critical care units (PICCU) after a visit to the PED, or died in PED.
Methods
Retrospective study of children who has been transferred to PICCU from the two PED of Marseille's University Hospital from the 1st of January 2022 until the 31st of December 2022.
Results
Among the 82,962 children who consulted the two PED of Marseille's University Hospital in 2022, 260 (0.3 %) were transferred to PICCU or died in PED. The mean age was 42 months. There were 17 % newborns and 12 % infants aged 1 to 3 months old. The main reasons for referral was respiratory troubles (bronchiolitis 27.3 %, asthma 21.9 %), and neurological impairment (14.6 %). Thirty percent of children were previously followed for a chronic illness. In 45 %, a critical intervention was required: non-invasive or controlled ventilation, extracorporeal membrane oxygenation, use of amines and/or blood transfusion, surgery, and/or external cardiac massage. Two children died in the PED and six during the hospitalization. The mean duration of hospitalization in PICCU was 4.2 days.
Conclusion
In Marseille, during 2022, the occurrence of a critical child in PED was estimated at one every 300 children. These children were generally young and presented with respiratory or neurological pathologies. Care might require extensive resources or simple surveillance. Mortality was low (2.3 %).
{"title":"From pediatric emergency department to pediatric intensive care unit: a retrospective study in a French Tertiary University hospital","authors":"Elsa Riche , Aurelie Morand , Sophie Fruscione , Fabrice Michel , Aurelie Boutin , Valerie Bremond , Valerie Arnoux , Philippe Minodier","doi":"10.1016/j.arcped.2024.12.005","DOIUrl":"10.1016/j.arcped.2024.12.005","url":null,"abstract":"<div><h3>Objective</h3><div>The management of a child presenting with a critical medical or surgical condition is a scarce event in the pediatric emergency department (PED). In this one year retrospective study, we have tried to better characterize the profile and care pathway of children who had been transferred to the neonatal or pediatric intensive care or critical care units (PICCU) after a visit to the PED, or died in PED.</div></div><div><h3>Methods</h3><div>Retrospective study of children who has been transferred to PICCU from the two PED of Marseille's University Hospital from the 1<sup>st</sup> of January 2022 until the 31<sup>st</sup> of December 2022.</div></div><div><h3>Results</h3><div>Among the 82,962 children who consulted the two PED of Marseille's University Hospital in 2022, 260 (0.3 %) were transferred to PICCU or died in PED. The mean age was 42 months. There were 17 % newborns and 12 % infants aged 1 to 3 months old. The main reasons for referral was respiratory troubles (bronchiolitis 27.3 %, asthma 21.9 %), and neurological impairment (14.6 %). Thirty percent of children were previously followed for a chronic illness. In 45 %, a critical intervention was required: non-invasive or controlled ventilation, extracorporeal membrane oxygenation, use of amines and/or blood transfusion, surgery, and/or external cardiac massage. Two children died in the PED and six during the hospitalization. The mean duration of hospitalization in PICCU was 4.2 days.</div></div><div><h3>Conclusion</h3><div>In Marseille, during 2022, the occurrence of a critical child in PED was estimated at one every 300 children. These children were generally young and presented with respiratory or neurological pathologies. Care might require extensive resources or simple surveillance. Mortality was low (2.3 %).</div></div>","PeriodicalId":55477,"journal":{"name":"Archives De Pediatrie","volume":"32 2","pages":"Pages 126-131"},"PeriodicalIF":1.3,"publicationDate":"2025-02-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143061518","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Myelin oligodendrocyte glycoprotein (MOG) antibody-associated disease (MOGAD) is a neuro-inflammatory condition affecting adults and children. The presentations vary and include acute disseminated encephalomyelitis, optic neuritis or transverse myelitis. Optic neuritis associated with anti-MOG antibodies is typically bilateral, anterior and initially severe but usually resolves quickly and completely. Here, we describe 3 children with pseudotumoural optic neuritis associated with anti-MOG antibodies and their outcome. Recognizing these unusual presentations may reduce unnecessary work-ups and improve functional prognosis by implementing rapid and adequate treatment.
{"title":"Pseudotumoural optic neuritis in myelin oligodendrocyte glycoprotein antibody-associated disease in children","authors":"Julia Roux , Domitille Bommier-Laur , Mariana Calota , Stéphane Auvin , Kumaran Deiva","doi":"10.1016/j.arcped.2024.11.005","DOIUrl":"10.1016/j.arcped.2024.11.005","url":null,"abstract":"<div><div>Myelin oligodendrocyte glycoprotein (MOG) antibody-associated disease (MOGAD) is a neuro-inflammatory condition affecting adults and children. The presentations vary and include acute disseminated encephalomyelitis, optic neuritis or transverse myelitis. Optic neuritis associated with anti-MOG antibodies is typically bilateral, anterior and initially severe but usually resolves quickly and completely. Here, we describe 3 children with pseudotumoural optic neuritis associated with anti-MOG antibodies and their outcome. Recognizing these unusual presentations may reduce unnecessary work-ups and improve functional prognosis by implementing rapid and adequate treatment.</div></div>","PeriodicalId":55477,"journal":{"name":"Archives De Pediatrie","volume":"32 2","pages":"Pages 143-146"},"PeriodicalIF":1.3,"publicationDate":"2025-02-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143061540","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Pub Date : 2025-02-01DOI: 10.1016/j.arcped.2024.12.003
Amélie Perriere , Karine Garcette , Nicolas Kalach , Patrick Tounian , Anaïs Lemoine
Background
Food protein-induced enterocolitis syndrome (FPIES) is a specific non IgE-mediated food allergy. The international consensus guidelines defined diagnosis criteria and management plan in 2017.
Objectives
To assess practices regarding FPIES in France and in French-speaking countries, according to those guidelines.
Methods and Setting
We carried out a 22-question online survey to assess practices of specialised physicians (paediatricians, allergists, gastroenterologists, members of 2 French-speaking learning societies and/or working at hospitals in paediatric allergy units) following patients with FPIES between August 2019 and February 2022.
Results
We received 92 replies to our survey, mostly from hospital practitioners following less than 10 patients with FPIES. Oral rehydration solution and/or Ondansetron were largely prescribed in the emergency kit (61/72, 84.7 % and 47/72, 65.3 % respectively). 20 practitioners declared never prescribing an emergency kit. There was some confusion when distinguishing between FPIES and an IgE-mediated food allergy, as suggested by the unnecessary prescription of an antihistamine (18/72, 25.0 %) and/or epinephrine (11/72, 15.3 %) in the emergency kit. An explanatory FPIES emergency management letter to physicians in case of allergic reactions was provided in 83.7 % (77/92) of patients. Oral food challenge (OFC) practices varied greatly concerning doses: most respondents used several doses (52/92, 56.5 %) during the same day (33/52, 63.4 %). Eleven responders (12.0 %) used the same protocol as for an IgE-mediated food allergy.
Conclusion
Our survey showed that practices of FPIES management in France are generally aligned with the international consensus guidelines. There are still pending issues to be standardised, such as the emergency kit prescription and its contents, as well as OFC management strategies. This work confirms the need for continuous training of physicians regarding FPIES management. Further guidelines are needed to improve standardisation of FPIES management.
{"title":"Food protein induced enterocolitis syndrome: French practices assessment in children","authors":"Amélie Perriere , Karine Garcette , Nicolas Kalach , Patrick Tounian , Anaïs Lemoine","doi":"10.1016/j.arcped.2024.12.003","DOIUrl":"10.1016/j.arcped.2024.12.003","url":null,"abstract":"<div><h3>Background</h3><div>Food protein-induced enterocolitis syndrome (FPIES) is a specific non IgE-mediated food allergy. The international consensus guidelines defined diagnosis criteria and management plan in 2017.</div></div><div><h3>Objectives</h3><div>To assess practices regarding FPIES in France and in French-speaking countries, according to those guidelines.</div></div><div><h3>Methods and Setting</h3><div>We carried out a 22-question online survey to assess practices of specialised physicians (paediatricians, allergists, gastroenterologists, members of 2 French-speaking learning societies and/or working at hospitals in paediatric allergy units) following patients with FPIES between August 2019 and February 2022.</div></div><div><h3>Results</h3><div>We received 92 replies to our survey, mostly from hospital practitioners following less than 10 patients with FPIES. Oral rehydration solution and/or Ondansetron were largely prescribed in the emergency kit (61/72, 84.7 % and 47/72, 65.3 % respectively). 20 practitioners declared never prescribing an emergency kit. There was some confusion when distinguishing between FPIES and an IgE-mediated food allergy, as suggested by the unnecessary prescription of an antihistamine (18/72, 25.0 %) and/or epinephrine (11/72, 15.3 %) in the emergency kit. An explanatory FPIES emergency management letter to physicians in case of allergic reactions was provided in 83.7 % (77/92) of patients. Oral food challenge (OFC) practices varied greatly concerning doses: most respondents used several doses (52/92, 56.5 %) during the same day (33/52, 63.4 %). Eleven responders (12.0 %) used the same protocol as for an IgE-mediated food allergy.</div></div><div><h3>Conclusion</h3><div>Our survey showed that practices of FPIES management in France are generally aligned with the international consensus guidelines. There are still pending issues to be standardised, such as the emergency kit prescription and its contents, as well as OFC management strategies. This work confirms the need for continuous training of physicians regarding FPIES management. Further guidelines are needed to improve standardisation of FPIES management.</div></div>","PeriodicalId":55477,"journal":{"name":"Archives De Pediatrie","volume":"32 2","pages":"Pages 114-119"},"PeriodicalIF":1.3,"publicationDate":"2025-02-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143061459","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Pub Date : 2025-02-01DOI: 10.1016/j.arcped.2024.09.009
Fouzia Yous , Salim Zaabar , Omar Aoun , Nawel Adjeroud-Abdellatif , Radia Arab , Khodir Madani
Objective
Adolescents are meant to spend a large part of their time practicing a hobby or sport, but currently there is a decline in their level of physical activity and sport both inside and outside schools, which can lead to a risk of obesity. The objective of the present study was to examine physical activity in obese adolescents within the school establishments of the province of Bejaia (northeastern Algeria).
Method
To determine the physical activity level (PAL) in the school population, a sample of 3,038 students with obesity comprising 1,635 girls and 1,403 boys was selected. The study focused on (a) the comparison of the intensity of physical activities practiced by girls and boys, (ii) the determination of the duration, intensity, and level of physical activity.
Results
The results show that obese girls are less active than obese boys at all ages (from 11 to 23 years) and in all locations; PALs was steady with increasing age for girls, while PALs increase with age in boys from 17 to 21. We found that some factors are associated with a decrease in PAL, such as the time spent watching television. Doing only 30 min of physical activity once a week is associated with an increase in sedentary behavior. The mean PALs of middle school (1.54) and high school (1.50) girls were lower than those observed for middle school (1.75) and high school (1.65) boys.
Conclusions
Our results show that obese girls are less active than obese boys and spend time watching television and practicing sports only at school.
{"title":"Study of physical activity in obese adolescents in Algeria","authors":"Fouzia Yous , Salim Zaabar , Omar Aoun , Nawel Adjeroud-Abdellatif , Radia Arab , Khodir Madani","doi":"10.1016/j.arcped.2024.09.009","DOIUrl":"10.1016/j.arcped.2024.09.009","url":null,"abstract":"<div><h3>Objective</h3><div>Adolescents are meant to spend a large part of their time practicing a hobby or sport, but currently there is a decline in their level of physical activity and sport both inside and outside schools, which can lead to a risk of obesity. The objective of the present study was to examine physical activity in obese adolescents within the school establishments of the province of Bejaia (northeastern Algeria).</div></div><div><h3>Method</h3><div>To determine the physical activity level (PAL) in the school population, a sample of 3,038 students with obesity comprising 1,635 girls and 1,403 boys was selected. The study focused on (a) the comparison of the intensity of physical activities practiced by girls and boys, (ii) the determination of the duration, intensity, and level of physical activity.</div></div><div><h3>Results</h3><div>The results show that obese girls are less active than obese boys at all ages (from 11 to 23 years) and in all locations; PALs was steady with increasing age for girls, while PALs increase with age in boys from 17 to 21. We found that some factors are associated with a decrease in PAL, such as the time spent watching television. Doing only 30 min of physical activity once a week is associated with an increase in sedentary behavior. The mean PALs of middle school (1.54) and high school (1.50) girls were lower than those observed for middle school (1.75) and high school (1.65) boys.</div></div><div><h3>Conclusions</h3><div>Our results show that obese girls are less active than obese boys and spend time watching television and practicing sports only at school.</div></div>","PeriodicalId":55477,"journal":{"name":"Archives De Pediatrie","volume":"32 2","pages":"Pages 87-92"},"PeriodicalIF":1.3,"publicationDate":"2025-02-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142973367","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Myotonia is the main feature of both myotonic dystrophy (DM) and non-dystrophic myotonia (NDM). It is felt as stiffness, pain, fatigue, and weakness. In France, mexiletine, a non-selective voltage-gated sodium channel blocker, is approved for the treatment of myotonia in adults with NDM, and it has a temporary recommendation for use in the symptomatic treatment of DM in adults. However, it is not currently licensed for treating myotonia in children due to the lack of studies on its use in pediatrics. This has meant heterogeneous practices in its utilization and has led to prescriber reluctance, which has jeopardized accessibility. We undertook a professional practice survey of French pediatric neuromuscular centers to determine their prescribing habits for mexiletine, assessing indications, doses, efficacy, and tolerance.
Methods
One medical pediatric professional from each French pediatric neuromuscular center belonging to the national neuromuscular network (FILNEMUS) was invited to complete an anonymous questionnaire.
Results
In total, 34 healthcare professionals responded. Of these, 16 had already treated a child for myotonia with mexiletine. Mexiletine was prescribed in one third of pediatric patients with NDM, but it was used in only 3% of DM type 1 patients and in no DM type 2 patients. Pre-treatment assessment always included a cardiac evaluation; however, the method of introduction (inpatient vs. outpatient basis), dosage adjustment, and efficacy evaluation ranged widely. More than half of the respondents reported a high efficacy of mexiletine in their patients; only three reported moderate adverse events (dyspepsia, loss of appetite, and asthenia).
Conclusion
The findings of this first survey on mexiletine for pediatric myotonia in France lend support for the creation of future national guidelines.
{"title":"Evaluation of professional practices in the use of mexiletine for the management of childhood myotonia in French pediatric neuromuscular centers (MEXI-PEDI survey)","authors":"Sarah Barrière , Véronique Manel , Christine Barnerias , Karim Wahbi , Frédérique Audic , Claude Cances , Mondher Chouchane , Ivana Dabaj , Jean-Baptiste Davion , Isabelle Desguerre , Julien Durigneux , Caroline Espil-Taris , Gaelle Gousse , Cyril Gitiaux , Clémentine Lambert , Cécile Laroche , Vincent Laugel , Anne-Gaelle Le Moing , Yann Pereon , Susana Quijano-Roy , Catherine Sarret","doi":"10.1016/j.arcped.2024.10.009","DOIUrl":"10.1016/j.arcped.2024.10.009","url":null,"abstract":"<div><h3>Background</h3><div>Myotonia is the main feature of both myotonic dystrophy (DM) and non-dystrophic myotonia (NDM). It is felt as stiffness, pain, fatigue, and weakness. In France, mexiletine, a non-selective voltage-gated sodium channel blocker, is approved for the treatment of myotonia in adults with NDM, and it has a temporary recommendation for use in the symptomatic treatment of DM in adults. However, it is not currently licensed for treating myotonia in children due to the lack of studies on its use in pediatrics. This has meant heterogeneous practices in its utilization and has led to prescriber reluctance, which has jeopardized accessibility. We undertook a professional practice survey of French pediatric neuromuscular centers to determine their prescribing habits for mexiletine, assessing indications, doses, efficacy, and tolerance.</div></div><div><h3>Methods</h3><div>One medical pediatric professional from each French pediatric neuromuscular center belonging to the national neuromuscular network (FILNEMUS) was invited to complete an anonymous questionnaire.</div></div><div><h3>Results</h3><div>In total, 34 healthcare professionals responded. Of these, 16 had already treated a child for myotonia with mexiletine. Mexiletine was prescribed in one third of pediatric patients with NDM, but it was used in only 3% of DM type 1 patients and in no DM type 2 patients. Pre-treatment assessment always included a cardiac evaluation; however, the method of introduction (inpatient vs. outpatient basis), dosage adjustment, and efficacy evaluation ranged widely. More than half of the respondents reported a high efficacy of mexiletine in their patients; only three reported moderate adverse events (dyspepsia, loss of appetite, and asthenia).</div></div><div><h3>Conclusion</h3><div>The findings of this first survey on mexiletine for pediatric myotonia in France lend support for the creation of future national guidelines.</div></div>","PeriodicalId":55477,"journal":{"name":"Archives De Pediatrie","volume":"32 2","pages":"Pages 107-113"},"PeriodicalIF":1.3,"publicationDate":"2025-02-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143061357","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
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