Pub Date : 2025-10-01DOI: 10.1016/j.arcped.2025.08.002
Alexandre Theron , Olivia Pineau , Sophie Bayart , Caroline Oudot , Sandrine Meunier , pediatric group of French Society of Thrombosis and Haemostasis
Hemophilia is the most prevalent constitutional bleeding disorder. Management of severe and moderate hemophilia has completely evolved in recent years due to major therapeutic innovations, such as antihemophilic factors with a prolonged half-life and non-substituted treatments, particularly emicizumab. Initial treatment takes place as early as possible in infancy to limit bleeding incidents and their consequences. In light of recent therapeutic changes, the pediatricians of the French Society of Thrombosis and Hemostasis have therefore carried out a national practice survey. Based on the survey results and a literature review, this article proposes suggestions for the initial management, prophylaxis, and monitoring of children with severe or moderate hemophilia. We recommend establishing early and long-term prophylaxis for all patients with severe or moderate hemophilia and a severe phenotype who have not been previously treated. The choice of treatment and prophylaxis regimen should be made through a shared decision-making process with the family. Close monitoring is recommended after prophylactic treatment initiation.
{"title":"How to start prophylaxis in untreated children with severe and moderate haemophilia in 2025, opinion of paediatricians from the French Society of Thrombosis and Haemostasis","authors":"Alexandre Theron , Olivia Pineau , Sophie Bayart , Caroline Oudot , Sandrine Meunier , pediatric group of French Society of Thrombosis and Haemostasis","doi":"10.1016/j.arcped.2025.08.002","DOIUrl":"10.1016/j.arcped.2025.08.002","url":null,"abstract":"<div><div>Hemophilia is the most prevalent constitutional bleeding disorder. Management of severe and moderate hemophilia has completely evolved in recent years due to major therapeutic innovations, such as antihemophilic factors with a prolonged half-life and non-substituted treatments, particularly emicizumab. Initial treatment takes place as early as possible in infancy to limit bleeding incidents and their consequences. In light of recent therapeutic changes, the pediatricians of the French Society of Thrombosis and Hemostasis have therefore carried out a national practice survey. Based on the survey results and a literature review, this article proposes suggestions for the initial management, prophylaxis, and monitoring of children with severe or moderate hemophilia. We recommend establishing early and long-term prophylaxis for all patients with severe or moderate hemophilia and a severe phenotype who have not been previously treated. The choice of treatment and prophylaxis regimen should be made through a shared decision-making process with the family. Close monitoring is recommended after prophylactic treatment initiation.</div></div>","PeriodicalId":55477,"journal":{"name":"Archives De Pediatrie","volume":"32 7","pages":"Pages 444-449"},"PeriodicalIF":1.3,"publicationDate":"2025-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145245890","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 : 2025-10-01DOI: 10.1016/j.arcped.2025.07.005
Auréliane Dantin , Sofia Guernouche , Sylvie Lorthois-Ninou , Vincent Des Portes , Etienne Javouhey , Carmine Mottolese , Laurence Lion-François , Federico Di Rocco
Objectives
The diagnosis of shaken baby syndrome (SBS) is difficult; the French High Authority for Health called “Haute Autorité de Santé” (HAS) introduced clinical and radiological criteria in 2017 to classify the diagnosis as “certain" or “probable." The aim of this study is to compare the clinical evolution of children with “certain" and “probable” diagnosis criteria of SBS.
Methods
Records of patients with a diagnosis of SBS according to HAS criteria, aged 1 to 16 months, admitted to a University Hospital between January 1st, 2008, and March 31st, 2018, were retrospectively analysed. Medical follow-up data were collected until December 31st, 2019. The primary endpoint was the Glasgow Outcome Scale (GOS) score at the end of hospitalization: the evolution was “favourable" if the GOS was equal to 5, and “unfavourable" if the GOS was lower than 5.
Results
107 children were included. The mortality rate was 10 %. There was no significant difference in the GOS score at the end of hospitalization between children with “certain" diagnosis and those with “probable" diagnosis (p = 0.75). There was no significant difference at last follow-up between these two groups. Among the children with an “unfavourable" outcome, 68 % had psychomotor developmental delay, 57 % had motor sequelae, 38 % had visual impairments and 36 % had epilepsy. Among those with a “favourable" outcome, 31 % had language disorders and 13 % had learning disorders. The GOS score at the end of follow-up was identical to the GOS at the end of hospitalization for 76 % of patients. It had improved for 14 % of the patients and had deteriorated for 10 % of the patients.
Conclusions
The “certain" or “probable" diagnosis of SBS according to HAS criteria does not modify the risk of sequelae during follow-up. Mortality and morbidity remain significant. Multidisciplinary management and specialized medical long-term follow-up are essential in both conditions.
目的：摇晃婴儿综合征（SBS）诊断困难；2017年，法国卫生高级管理局（Haute autorit de sant， HAS）引入了临床和放射学标准，将诊断分为“确定”或“可能”。本研究的目的是比较具有SBS“确定”和“可能”诊断标准的儿童的临床演变。方法：回顾性分析2008年1月1日至2018年3月31日在某大学医院收治的符合HAS标准的1 ~ 16个月SBS患者的记录。医学随访数据收集至2019年12月31日。主要终点是住院结束时的格拉斯哥结局量表（GOS）评分：如果GOS等于5，则演变为“有利”，如果GOS低于5，则为“不利”。结果：共纳入107例患儿。死亡率为10%。“确定”诊断患儿与“可能”诊断患儿住院结束时GOS评分差异无统计学意义（p = 0.75）。两组在最后随访时无显著差异。在“不良”结果的儿童中，68%有精神运动发育迟缓，57%有运动后遗症，38%有视力障碍，36%有癫痫。在结果“良好”的患者中，31%有语言障碍，13%有学习障碍。随访结束时的GOS评分与76%患者住院结束时的GOS评分相同。14%的患者病情好转，10%的患者病情恶化。结论：根据HAS标准对SBS的“确定”或“可能”诊断并不能改变随访期间发生后遗症的风险。死亡率和发病率仍然很高。在这两种情况下，多学科管理和专门的医疗长期随访是必不可少的。
{"title":"« Certain » and « probable » shaken baby syndrome according to the HAS diagnostic criteria: a different neurological prognosis?","authors":"Auréliane Dantin , Sofia Guernouche , Sylvie Lorthois-Ninou , Vincent Des Portes , Etienne Javouhey , Carmine Mottolese , Laurence Lion-François , Federico Di Rocco","doi":"10.1016/j.arcped.2025.07.005","DOIUrl":"10.1016/j.arcped.2025.07.005","url":null,"abstract":"<div><h3>Objectives</h3><div>The diagnosis of shaken baby syndrome (SBS) is difficult; the French High Authority for Health called “Haute Autorité de Santé” (HAS) introduced clinical and radiological criteria in 2017 to classify the diagnosis as “certain\" or “probable.\" The aim of this study is to compare the clinical evolution of children with “certain\" and “probable” diagnosis criteria of SBS.</div></div><div><h3>Methods</h3><div>Records of patients with a diagnosis of SBS according to HAS criteria, aged 1 to 16 months, admitted to a University Hospital between January 1st, 2008, and March 31st, 2018, were retrospectively analysed. Medical follow-up data were collected until December 31st, 2019. The primary endpoint was the Glasgow Outcome Scale (GOS) score at the end of hospitalization: the evolution was “favourable\" if the GOS was equal to 5, and “unfavourable\" if the GOS was lower than 5.</div></div><div><h3>Results</h3><div>107 children were included. The mortality rate was 10 %. There was no significant difference in the GOS score at the end of hospitalization between children with “certain\" diagnosis and those with “probable\" diagnosis (<em>p</em> = 0.75). There was no significant difference at last follow-up between these two groups. Among the children with an “unfavourable\" outcome, 68 % had psychomotor developmental delay, 57 % had motor sequelae, 38 % had visual impairments and 36 % had epilepsy. Among those with a “favourable\" outcome, 31 % had language disorders and 13 % had learning disorders. The GOS score at the end of follow-up was identical to the GOS at the end of hospitalization for 76 % of patients. It had improved for 14 % of the patients and had deteriorated for 10 % of the patients.</div></div><div><h3>Conclusions</h3><div>The “certain\" or “probable\" diagnosis of SBS according to HAS criteria does not modify the risk of sequelae during follow-up. Mortality and morbidity remain significant. Multidisciplinary management and specialized medical long-term follow-up are essential in both conditions.</div></div>","PeriodicalId":55477,"journal":{"name":"Archives De Pediatrie","volume":"32 7","pages":"Pages 497-502"},"PeriodicalIF":1.3,"publicationDate":"2025-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145245904","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 : 2025-08-01DOI: 10.1016/j.arcped.2025.03.004
M. Battini , R. Maximilien-François , A. Paygambar , C. Laplace , M-E. Gatibelza , C. Trabanino , H. Coridon , C. Tolg , P. Grall , P. Guemaleu , A. Sika , R. Sfeir , F. Gottrand
Background
Esophageal atresia (EA) is a rare defect in esophageal continuity, frequently associated with tracheoesophageal fistula. Data on EA in children from French Guiana and the French West Indies are lacking. Objectives: The objective of this study was to compare characteristics and outcomes between patients with EA in three French Departments in the Americas (DFAs) and a cohort from metropolitan France.
Methods and settings
This was a retrospective, multicenter descriptive study of all children born with EA in French Guiana, Martinique, or Guadeloupe between 2008 and early 2021. Data were extracted from the French register (from 38 French medical centers), which records characteristics and outcomes from the prenatal period through 1 year of age (with two questionnaires centralized by the National Reference Center for Esophageal Anomalies through an exhaustive search using several methods, with double-checking and verification). The characteristics of infants from the three DFAs were compared with those from metropolitan France.
Results
Overall, 39 children from the DFAs were included, among whom 36 had surgery at a median age of 3 days postnatally (0–81 days). At 1 year of age, eight children (21 %) had died and seven were lost to follow-up. There were no differences among the DFAs regarding mortality (p = 0.318) or morbidity, apart from a greater rate of loss to follow-up in French Guiana than in Guadeloupe (p < 0.01). Compared with metropolitan France (total of 2205 children; 1960 children with an anastomosis, 97 children died at 1 year of age), children from the DFAs had higher rates of complications (36 % vs. 24 % in metropolitan France, p = 0.04), higher mortality (21 % vs. 4 % in metropolitan France, p < 0.01), and fewer rehospitalizations per child (1 vs. 2 in metropolitan France, p < 0.01).
Conclusion
The three overseas groups have similar characteristics and outcomes but significantly higher morbidity and mortality rates compared with metropolitan France.
{"title":"Esophageal atresia in three overseas departments compared with metropolitan France","authors":"M. Battini , R. Maximilien-François , A. Paygambar , C. Laplace , M-E. Gatibelza , C. Trabanino , H. Coridon , C. Tolg , P. Grall , P. Guemaleu , A. Sika , R. Sfeir , F. Gottrand","doi":"10.1016/j.arcped.2025.03.004","DOIUrl":"10.1016/j.arcped.2025.03.004","url":null,"abstract":"<div><h3>Background</h3><div><span>Esophageal atresia<span> (EA) is a rare defect in esophageal continuity, frequently associated with tracheoesophageal fistula. Data on EA in children from French Guiana and the French West Indies are lacking. </span></span><em>Objectives:</em> The objective of this study was to compare characteristics and outcomes between patients with EA in three French Departments in the Americas (DFAs) and a cohort from metropolitan France.</div></div><div><h3>Methods and settings</h3><div>This was a retrospective, multicenter descriptive study of all children born with EA in French Guiana, Martinique, or Guadeloupe between 2008 and early 2021. Data were extracted from the French register (from 38 French medical centers), which records characteristics and outcomes from the prenatal period through 1 year of age (with two questionnaires centralized by the National Reference Center for Esophageal Anomalies through an exhaustive search using several methods, with double-checking and verification). The characteristics of infants from the three DFAs were compared with those from metropolitan France.</div></div><div><h3>Results</h3><div>Overall, 39 children from the DFAs were included, among whom 36 had surgery at a median age of 3 days postnatally (0–81 days). At 1 year of age, eight children (21 %) had died and seven were lost to follow-up. There were no differences among the DFAs regarding mortality (<em>p</em> = 0.318) or morbidity, apart from a greater rate of loss to follow-up in French Guiana than in Guadeloupe (<em>p</em><span> < 0.01). Compared with metropolitan France (total of 2205 children; 1960 children with an anastomosis, 97 children died at 1 year of age), children from the DFAs had higher rates of complications (36 % vs. 24 % in metropolitan France, </span><em>p</em> = 0.04), higher mortality (21 % vs. 4 % in metropolitan France, <em>p</em> < 0.01), and fewer rehospitalizations per child (1 vs. 2 in metropolitan France, <em>p</em> < 0.01).</div></div><div><h3>Conclusion</h3><div>The three overseas groups have similar characteristics and outcomes but significantly higher morbidity and mortality rates compared with metropolitan France.</div></div>","PeriodicalId":55477,"journal":{"name":"Archives De Pediatrie","volume":"32 6","pages":"Pages 374-382"},"PeriodicalIF":1.3,"publicationDate":"2025-08-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144512798","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 : 2025-08-01DOI: 10.1016/j.arcped.2025.05.002
Cassandra Varoqui , Franck Thollot
Context
Vaccination of traveling children does not seem to be the primary concern for families when preparing for a trip abroad. Few data concerning the hepatitis A vaccine are available in minors traveling outside France.
Objectives
The present study aimed to assess whether the vaccination recommendation against hepatitis A was followed in the population of children traveling outside of France. The secondary objectives were to explore the reasons for non-compliance with vaccination recommendations and assess the frequency of minors travelling abroad in the study settings.
Population and methods
This was a monocentric observational study conducted within a pediatric practice located in the Nancy metropolitan area through the distribution of a questionnaire upon arrival in the waiting room. Inclusion criteria were: age between 1 and 18 years, regular pediatric follow-up within the practice, and absence of language barriers.
Results
477 questionnaires were included, evaluating the proportion of traveling children (outside of France) at 59 % of our population (n = 282). The leading destination remained Western Europe (36 %), but 64 % of children have already traveled to an area where vaccination against hepatitis A before departure is recommended. However, only 10.5 % of patients had received one dose of vaccination before traveling, and only 2.2 % had completed vaccination. The reasons for non-vaccination were a lack of information or a lack of anticipation before departure. After distributing the questionnaire, 101 prescriptions were issued at the parents' request.
Conclusion
The vaccination recommendations against hepatitis A are not followed in our population of travelling children, with only 10 % of them vaccinated, while 50 % have been travelling to a high-risk country. The reasons for non-vaccination may be multifactorial, but this study also highlights the importance of a preventive period to improve the population's knowledge and help them anticipate their travel plans. Although time-consuming, this prevention was profitable since 101 vaccine doses were prescribed out of 477 questionnaires collected. A display could be put up in the waiting room to draw families' attention to the need to anticipate travel.
{"title":"Traveling children: Current situation and compliance with hepatitis A vaccination recommendations","authors":"Cassandra Varoqui , Franck Thollot","doi":"10.1016/j.arcped.2025.05.002","DOIUrl":"10.1016/j.arcped.2025.05.002","url":null,"abstract":"<div><h3>Context</h3><div>Vaccination of traveling children does not seem to be the primary concern for families when preparing for a trip abroad. Few data concerning the hepatitis A vaccine are available in minors traveling outside France.</div></div><div><h3>Objectives</h3><div>The present study aimed to assess whether the vaccination recommendation against hepatitis A was followed in the population of children traveling outside of France. The secondary objectives were to explore the reasons for non-compliance with vaccination recommendations and assess the frequency of minors travelling abroad in the study settings.</div></div><div><h3>Population and methods</h3><div>This was a monocentric observational study conducted within a pediatric practice located in the Nancy metropolitan area through the distribution of a questionnaire upon arrival in the waiting room. Inclusion criteria were: age between 1 and 18 years, regular pediatric follow-up within the practice, and absence of language barriers.</div></div><div><h3>Results</h3><div>477 questionnaires were included, evaluating the proportion of traveling children (outside of France) at 59 % of our population (<em>n</em> = 282). The leading destination remained Western Europe (36 %), but 64 % of children have already traveled to an area where vaccination against hepatitis A before departure is recommended. However, only 10.5 % of patients had received one dose of vaccination before traveling, and only 2.2 % had completed vaccination. The reasons for non-vaccination were a lack of information or a lack of anticipation before departure. After distributing the questionnaire, 101 prescriptions were issued at the parents' request.</div></div><div><h3>Conclusion</h3><div>The vaccination recommendations against hepatitis A are not followed in our population of travelling children, with only 10 % of them vaccinated, while 50 % have been travelling to a high-risk country. The reasons for non-vaccination may be multifactorial, but this study also highlights the importance of a preventive period to improve the population's knowledge and help them anticipate their travel plans. Although time-consuming, this prevention was profitable since 101 vaccine doses were prescribed out of 477 questionnaires collected. A display could be put up in the waiting room to draw families' attention to the need to anticipate travel.</div></div>","PeriodicalId":55477,"journal":{"name":"Archives De Pediatrie","volume":"32 6","pages":"Pages 363-367"},"PeriodicalIF":1.3,"publicationDate":"2025-08-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144859928","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}
Hypospadias is among the most common congenital anomalies of the male genitalia, with a multifactorial cause involving genetic, environmental, and maternal factors. In Algeria, the condition remains under-studied, with limited data on its epidemiology and risk factors.
Objective
To analyze the clinical characteristics, associated anomalies and key risk factors for hypospadias in Eastern Algeria over a 16-year period.
Methods
This retrospective case-control study included 483 hypospadias cases and 503 healthy controls. Cases were identified through medical records from the Pediatric Surgery Department, while controls were recruited via online questionnaires. Statistical analyses, including logistic regression, were performed to identify independent risk factors.
Results
Significant risk factors for hypospadias included low birth weight (<2500 g; OR = 11.86, p < 0.001), intrauterine growth restriction (IUGR) (OR = 2.36, p = 0.027), maternal gestational hypertension (OR = 2.65, p = 0.008), and rural residence (OR = 3.78, p < 0.001). Severe forms of hypospadias were associated with a higher prevalence of anomalies, particularly genitourinary anomalies (81.82 %).
Conclusion
Hypospadias in Eastern Algeria is strongly influenced by perinatal, maternal, and environmental factors. Enhancing prenatal care, enforcing stricter environmental regulations, and improving healthcare access in rural areas are crucial steps in reducing the burden of this condition and its complications.
{"title":"Investigating risk factors for hypospadias: insights from a study in Eastern Algeria","authors":"Laouar Rania , Chellat-Rezgoune Djalila , Atrih Zoubir , Benhizia Yacine , Sifi Karima","doi":"10.1016/j.arcped.2025.06.001","DOIUrl":"10.1016/j.arcped.2025.06.001","url":null,"abstract":"<div><h3>Background</h3><div>Hypospadias is among the most common congenital anomalies of the male genitalia, with a multifactorial cause involving genetic, environmental, and maternal factors. In Algeria, the condition remains under-studied, with limited data on its epidemiology and risk factors.</div></div><div><h3>Objective</h3><div>To analyze the clinical characteristics, associated anomalies and key risk factors for hypospadias in Eastern Algeria over a 16-year period.</div></div><div><h3>Methods</h3><div>This retrospective case-control study included 483 hypospadias cases and 503 healthy controls. Cases were identified through medical records from the Pediatric Surgery Department, while controls were recruited via online questionnaires. Statistical analyses, including logistic regression, were performed to identify independent risk factors.</div></div><div><h3>Results</h3><div>Significant risk factors for hypospadias included low birth weight (<2500 g; OR = 11.86, <em>p</em> < 0.001), intrauterine growth restriction (IUGR) (OR = 2.36, <em>p</em> = 0.027), maternal gestational hypertension (OR = 2.65, <em>p</em> = 0.008), and rural residence (OR = 3.78, <em>p</em> < 0.001). Severe forms of hypospadias were associated with a higher prevalence of anomalies, particularly genitourinary anomalies (81.82 %).</div></div><div><h3>Conclusion</h3><div>Hypospadias in Eastern Algeria is strongly influenced by perinatal, maternal, and environmental factors. Enhancing prenatal care, enforcing stricter environmental regulations, and improving healthcare access in rural areas are crucial steps in reducing the burden of this condition and its complications.</div></div>","PeriodicalId":55477,"journal":{"name":"Archives De Pediatrie","volume":"32 6","pages":"Pages 389-394"},"PeriodicalIF":1.3,"publicationDate":"2025-08-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144838655","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 : 2025-08-01DOI: 10.1016/j.arcped.2025.05.001
Charlotte Suermondt , Charles de Marcellus , Dominique Vodovar , Charlotte Roy , Nathanael Shraer , Mehdi Oualha , Noémie de Cacqueray
A 20-month-old girl presented with organophosphate poisoning. Her clinical course was complicated by acute respiratory failure despite oxime administration, requiring mechanical ventilation within 24 h. Due to refractory acute respiratory distress syndrome (ARDS), the patient was placed on veno-venous extracorporeal membrane oxygenation (VV-ECMO) therapy on day 3. Successful rescue and complete recovery were achieved with VV-ECMO. Veno-venous ECMO may be an option for organophosphate poisoning in case of respiratory failure.
{"title":"Successful ECMO rescue for organophosphate poisoning causing refractory acute respiratory distress syndrome: a first pediatric case","authors":"Charlotte Suermondt , Charles de Marcellus , Dominique Vodovar , Charlotte Roy , Nathanael Shraer , Mehdi Oualha , Noémie de Cacqueray","doi":"10.1016/j.arcped.2025.05.001","DOIUrl":"10.1016/j.arcped.2025.05.001","url":null,"abstract":"<div><div><span>A 20-month-old girl presented with organophosphate poisoning<span><span>. Her clinical course was complicated by </span>acute respiratory failure despite </span></span>oxime<span> administration, requiring mechanical ventilation<span><span> within 24 h. Due to refractory acute respiratory distress syndrome<span> (ARDS), the patient was placed on veno-venous extracorporeal membrane oxygenation<span> (VV-ECMO) therapy on day 3. Successful rescue and complete recovery were achieved with VV-ECMO. Veno-venous ECMO may be an option for </span></span></span>organophosphate poisoning in case of respiratory failure.</span></span></div></div>","PeriodicalId":55477,"journal":{"name":"Archives De Pediatrie","volume":"32 6","pages":"Pages 423-426"},"PeriodicalIF":1.3,"publicationDate":"2025-08-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144499471","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}
A 4-month-old male born with a Binder Phenotype was admitted for the evacuation of a large subdural hematoma. The blood analysis revealed a prolonged prothrombin time due to vitamin K-dependent coagulation factor deficiency. Vitamin K participates in the embryonic development of the nasal cartilage. The genetic analysis of our patient revealed a rare genetic cause, responsible for the congenital Binder phenotype associated with a defect in the vitamin K metabolism, a pathogenic variant in the GGCX gene that has not been previously reported in the literature. All neonates presenting a Binder Phenotype would benefit from coagulation screening, an easy-access exam, in order to prevent severe and potentially dreadful hemorrhagic events.
{"title":"Familial vitamin K metabolism deficiency responsible for a congenital binder phenotype","authors":"Konstantinos Grammatopoulos , Annie Harroche , Cristina Peduto , Nancy Vegas , Alix Mathonnet , Genevieve Baujat , Veronique Abadie , Alix Flamant","doi":"10.1016/j.arcped.2025.05.003","DOIUrl":"10.1016/j.arcped.2025.05.003","url":null,"abstract":"<div><div>A 4-month-old male born with a Binder Phenotype was admitted for the evacuation of a large subdural hematoma. The blood analysis revealed a prolonged prothrombin time due to vitamin K-dependent coagulation factor deficiency. Vitamin K participates in the embryonic development of the nasal cartilage. The genetic analysis of our patient revealed a rare genetic cause, responsible for the congenital Binder phenotype associated with a defect in the vitamin K metabolism, a pathogenic variant in the GGCX gene that has not been previously reported in the literature. All neonates presenting a Binder Phenotype would benefit from coagulation screening, an easy-access exam, in order to prevent severe and potentially dreadful hemorrhagic events.</div></div>","PeriodicalId":55477,"journal":{"name":"Archives De Pediatrie","volume":"32 6","pages":"Pages 420-422"},"PeriodicalIF":1.3,"publicationDate":"2025-08-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144499468","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 : 2025-08-01DOI: 10.1016/j.arcped.2025.06.004
Bruno Hoen
{"title":"Recent and important updates to the French meningococcal vaccination strategy","authors":"Bruno Hoen","doi":"10.1016/j.arcped.2025.06.004","DOIUrl":"10.1016/j.arcped.2025.06.004","url":null,"abstract":"","PeriodicalId":55477,"journal":{"name":"Archives De Pediatrie","volume":"32 6","pages":"Pages 361-362"},"PeriodicalIF":1.3,"publicationDate":"2025-08-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144849715","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}
Ovarian hyperstimulation syndrome is characterized by enlarged ovaries, multiple follicular cysts, and fluid leakage into the extravascular space caused by increased vascular permeability. Although controlled ovarian stimulation is often the main cause, spontaneous ovarian hyperstimulation syndrome is rare.
Observation and discussion
We present a seventeen-year-old patient with ovarian hyperstimulation syndrome and primary hypothyroidism due to autoimmune thyroiditis, who presented with galactorrhea and severe abdominal pain. The thyroid-stimulating hormone level was 713 uIU/ml. Ultrasonography and magnetic resonance imaging showed ovarian enlargement and multiple cysts. Our patient, who also had galactorrhea and hyperprolactinemia, was treated with levothyroxine and cabergoline. The ovaries, which were very large and multicystic before treatment, showed significant improvement after treatment.
Conclusion
Spontaneous ovarian hyperstimulation syndrome, although rare in childhood, should be considered in patients with primary hypothyroidism. Abdominal pain is an important symptom for diagnosis.
{"title":"Spontaneous Ovarian Hyperstimulation Syndrome and Hyperprolactinemia due to Primary Hypothyroidism: An Adolescent Case Report","authors":"Emel Hatun Aytaç Kaplan , Nazlı Gülsüm Akyel , Zümrüt Kocabey Sütçü","doi":"10.1016/j.arcped.2025.03.007","DOIUrl":"10.1016/j.arcped.2025.03.007","url":null,"abstract":"<div><h3>Introduction</h3><div>Ovarian hyperstimulation syndrome is characterized by enlarged ovaries, multiple follicular cysts, and fluid leakage into the extravascular space caused by increased vascular permeability. Although controlled ovarian stimulation is often the main cause, spontaneous ovarian hyperstimulation syndrome is rare.</div></div><div><h3>Observation and discussion</h3><div>We present a seventeen-year-old patient with ovarian hyperstimulation syndrome and primary hypothyroidism due to autoimmune thyroiditis, who presented with galactorrhea and severe abdominal pain. The thyroid-stimulating hormone level was 713 uIU/ml. Ultrasonography and magnetic resonance imaging showed ovarian enlargement and multiple cysts. Our patient, who also had galactorrhea and hyperprolactinemia, was treated with levothyroxine and cabergoline. The ovaries, which were very large and multicystic before treatment, showed significant improvement after treatment.</div></div><div><h3>Conclusion</h3><div>Spontaneous ovarian hyperstimulation syndrome, although rare in childhood, should be considered in patients with primary hypothyroidism. Abdominal pain is an important symptom for diagnosis.</div></div>","PeriodicalId":55477,"journal":{"name":"Archives De Pediatrie","volume":"32 6","pages":"Pages 427-431"},"PeriodicalIF":1.3,"publicationDate":"2025-08-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144849716","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 : 2025-08-01DOI: 10.1016/j.arcped.2025.05.004
Victor Sartorius , Ann-Marie Crowe , Alina Marilena Lazarescu , Laurence Labat , Anna Kaminska , Charles Joris Roux , Gilles A Orliaguet , Stéphane Blanot
Background
The determination of brain death (BD) is the primary pathway to organ donation in children. Thiopental is a sedative drug prescribed in critical situations potentially leading to BD and has been identified as a confounder during BD diagnosis. The implications of prior thiopental sedation on the diagnostic process of BD are poorly defined.
Objective
To compare the timeline and process involved in BD diagnosis between patients who received thiopental sedation versus those who did not.
Methods and Setting
Retrospective analysis was carried out on the healthcare records of all children who underwent the process of BD evaluation and diagnosis in our institution, between January 2012 and April 2020.
Results
74 patients were included; 29 had been treated with thiopental and 45 had received other sedatives or none. Median [IQR] time from reporting of suspected BD patients to apnea test (19.5 [11.5–30.2] vs 11.5 [5.4–21.1] hours, p = 0.015) and to BD confirmatory test (24.2 [7.2–32.6] vs 13.2 [6.2–23.5] hours, p = 0.019) was significantly longer in the thiopental group. Organ allocation was also delayed in the thiopental group. Thiopental plasma level was measured before BD examination in 24 patients, and the median concentration of the last assay was 4.6 [0.8–8.8] mg.L-1. The BD diagnostic process included persistent signs of cerebral blood flow interruption on transcranial Doppler in 89.7 % of patients in the thiopental group vs 68.9 % in the other group (p = 0.049).
Conclusion
Prior administration of thiopental delays the process involved in the evaluation and diagnosis of BD in children in our intensive care unit. The diagnosis of BD after thiopental sedation requires a multimodal evaluation, which should include the monitoring of thiopental plasma levels and could be guided by transcranial Doppler.
{"title":"Brain death diagnostic process in children after thiopental sedation: a comparative retrospective cohort study","authors":"Victor Sartorius , Ann-Marie Crowe , Alina Marilena Lazarescu , Laurence Labat , Anna Kaminska , Charles Joris Roux , Gilles A Orliaguet , Stéphane Blanot","doi":"10.1016/j.arcped.2025.05.004","DOIUrl":"10.1016/j.arcped.2025.05.004","url":null,"abstract":"<div><h3>Background</h3><div>The determination of brain death (BD) is the primary pathway to organ donation in children. Thiopental is a sedative drug prescribed in critical situations potentially leading to BD and has been identified as a confounder during BD diagnosis. The implications of prior thiopental sedation on the diagnostic process of BD are poorly defined.</div></div><div><h3>Objective</h3><div>To compare the timeline and process involved in BD diagnosis between patients who received thiopental sedation versus those who did not.</div></div><div><h3>Methods and Setting</h3><div>Retrospective analysis was carried out on the healthcare records of all children who underwent the process of BD evaluation and diagnosis in our institution, between January 2012 and April 2020.</div></div><div><h3>Results</h3><div>74 patients were included; 29 had been treated with thiopental and 45 had received other sedatives or none. Median [IQR] time from reporting of suspected BD patients to apnea test (19.5 [11.5–30.2] vs 11.5 [5.4–21.1] hours, <em>p</em> = 0.015) and to BD confirmatory test (24.2 [7.2–32.6] vs 13.2 [6.2–23.5] hours, <em>p</em> = 0.019) was significantly longer in the thiopental group. Organ allocation was also delayed in the thiopental group. Thiopental plasma level was measured before BD examination in 24 patients, and the median concentration of the last assay was 4.6 [0.8–8.8] mg.L<sup>-1</sup>. The BD diagnostic process included persistent signs of cerebral blood flow interruption on transcranial Doppler in 89.7 % of patients in the thiopental group vs 68.9 % in the other group (<em>p</em> = 0.049).</div></div><div><h3>Conclusion</h3><div>Prior administration of thiopental delays the process involved in the evaluation and diagnosis of BD in children in our intensive care unit. The diagnosis of BD after thiopental sedation requires a multimodal evaluation, which should include the monitoring of thiopental plasma levels and could be guided by transcranial Doppler.</div></div>","PeriodicalId":55477,"journal":{"name":"Archives De Pediatrie","volume":"32 6","pages":"Pages 383-388"},"PeriodicalIF":1.3,"publicationDate":"2025-08-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144838624","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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