Sofia Maria Silva Faria, Sílvia Gomes Ferreira Duarte Costa, Carolina Isabel Lopes Pinto Da Costa, Cláudia Isabel Gomes de Sousa Ferraz, Maria Teresa Pinto Martins Cezanne, Catarina Homem Gouveia Neto Viveiros, Ana Isabel Aldeia Azevedo, Cecília de Sousa Pinto Martins Lopes
{"title":"Small Beginnings, Big Challenges: The Complexities of a Perinatal Stroke","authors":"Sofia Maria Silva Faria, Sílvia Gomes Ferreira Duarte Costa, Carolina Isabel Lopes Pinto Da Costa, Cláudia Isabel Gomes de Sousa Ferraz, Maria Teresa Pinto Martins Cezanne, Catarina Homem Gouveia Neto Viveiros, Ana Isabel Aldeia Azevedo, Cecília de Sousa Pinto Martins Lopes","doi":"10.1111/jpc.70212","DOIUrl":"10.1111/jpc.70212","url":null,"abstract":"","PeriodicalId":16648,"journal":{"name":"Journal of paediatrics and child health","volume":"62 1","pages":"123-126"},"PeriodicalIF":1.4,"publicationDate":"2025-10-11","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145274920","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}
{"title":"Potential Association Between Nonketotic Hyperglycinemia and Extensive Mongolian Spots: A Novel Dermatological Observation—Case Report","authors":"Ozge Serce Pehlevan, Seyma Karatas, Gulhan Karakaya, Ozlem Unal Uzun, Ayla Gunlemez","doi":"10.1111/jpc.70214","DOIUrl":"10.1111/jpc.70214","url":null,"abstract":"","PeriodicalId":16648,"journal":{"name":"Journal of paediatrics and child health","volume":"62 1","pages":"127-130"},"PeriodicalIF":1.4,"publicationDate":"2025-10-11","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145274988","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}
Acute refusal to weight-bear or limp is a commonly encountered presentation to the Paediatric Emergency Department (PED). Due to the extensive range of differential diagnoses that may be responsible for these symptoms in the paediatric population, such patients pose a diagnostic challenge. Furthermore, some of the potential causes can be extremely serious or even life-threatening. POCUS has been demonstrated to be useful in assisting and expediting decision making for paediatric patients presenting with lower limb complaints in the PED. This article describes a range of acute lower limb presentations to the PED in which POCUS played a central role in establishing the diagnosis, which was otherwise unclear or assisted as part of its management.
{"title":"The Role of Point of Care Ultrasound in Evaluating Paediatric Patients With Limp: An Illustrative Case Series","authors":"David J. McCreary, Peter J. Snelling","doi":"10.1111/jpc.70207","DOIUrl":"10.1111/jpc.70207","url":null,"abstract":"<div>\u0000 \u0000 <p>Acute refusal to weight-bear or limp is a commonly encountered presentation to the Paediatric Emergency Department (PED). Due to the extensive range of differential diagnoses that may be responsible for these symptoms in the paediatric population, such patients pose a diagnostic challenge. Furthermore, some of the potential causes can be extremely serious or even life-threatening. POCUS has been demonstrated to be useful in assisting and expediting decision making for paediatric patients presenting with lower limb complaints in the PED. This article describes a range of acute lower limb presentations to the PED in which POCUS played a central role in establishing the diagnosis, which was otherwise unclear or assisted as part of its management.</p>\u0000 </div>","PeriodicalId":16648,"journal":{"name":"Journal of paediatrics and child health","volume":"61 12","pages":"1826-1834"},"PeriodicalIF":1.4,"publicationDate":"2025-10-09","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145251648","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}
Xin Yee Chiew, Kok Joo Chan, Norazah Zahari, Nurliza Khaliddin, I-Ching Sam, Yao Mun Choo, Azanna Ahmad Kamar
{"title":"Intravitreal Bevacizumab in Multisystem Inflammatory Syndrome-Induced Cardiomyopathy","authors":"Xin Yee Chiew, Kok Joo Chan, Norazah Zahari, Nurliza Khaliddin, I-Ching Sam, Yao Mun Choo, Azanna Ahmad Kamar","doi":"10.1111/jpc.70209","DOIUrl":"10.1111/jpc.70209","url":null,"abstract":"","PeriodicalId":16648,"journal":{"name":"Journal of paediatrics and child health","volume":"61 12","pages":"1939-1943"},"PeriodicalIF":1.4,"publicationDate":"2025-10-09","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145251666","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}
Nav La, Schawanya Kaewpitoon Rattanapitoon, Thawatchai Aeksanti, Nathkapach Kaewpitoon Rattanapitoon
We read with interest the study by Yitzhaki et al. evaluating ChatGPT-4 against a PICU specialist in answering open-ended medical education questions [1]. The authors' methodological strengths—such as the use of a closed question set not previously available to the model and a blinded multi-centre evaluation—offer valuable internal validity. Their conclusion that ChatGPT-4 tends to outperform on factual recall but lags on complex clinical reasoning aligns with emerging evidence from other specialties, where large language models (LLMs) frequently excel in structured factual tasks but underperform in nuanced, context-dependent reasoning [2, 3].
We propose that future work in PICU education should evaluate prospective interventions that operationalise these principles: (a) implement dual-response workflows with mandatory expert verification; (b) deploy retrieval-augmented models that return linked, labelled evidence; and (c) assess AI use as a reasoning scaffold with outcomes including trainee diagnostic accuracy, reasoning process measures, learner engagement, and indicators of automation bias. Such studies should include robust blinded assessment and both formative and summative educational endpoints.
In summary, Yitzhaki et al. provide an important empirical foundation showing that modern LLMs can contribute meaningfully to factual teaching in PICU settings but require structured oversight for reasoning tasks [1]. Rather than viewing LLMs as a binary replacement for educators, we suggest viewing them as adaptable partners within transparent, evidence-traceable learning ecosystems that amplify educator capacity while safeguarding clinical reasoning standards.
All authors have substantial contributions to conceptualization, literature review, writing – original draft, and writing – review and editing; and gave final approval of the version to be published.
The authors have nothing to report.
The authors declare no conflicts of interest.
Data sharing not applicable to this article as no datasets were generated or analysed during the current study.
{"title":"Expanding the Role of Generative AI in Paediatric Intensive Care Education: Beyond Factual Knowledge Toward Integrated Clinical Reasoning","authors":"Nav La, Schawanya Kaewpitoon Rattanapitoon, Thawatchai Aeksanti, Nathkapach Kaewpitoon Rattanapitoon","doi":"10.1111/jpc.70213","DOIUrl":"10.1111/jpc.70213","url":null,"abstract":"<p>We read with interest the study by Yitzhaki et al. evaluating ChatGPT-4 against a PICU specialist in answering open-ended medical education questions [<span>1</span>]. The authors' methodological strengths—such as the use of a closed question set not previously available to the model and a blinded multi-centre evaluation—offer valuable internal validity. Their conclusion that ChatGPT-4 tends to outperform on factual recall but lags on complex clinical reasoning aligns with emerging evidence from other specialties, where large language models (LLMs) frequently excel in structured factual tasks but underperform in nuanced, context-dependent reasoning [<span>2, 3</span>].</p><p>We propose that future work in PICU education should evaluate prospective interventions that operationalise these principles: (a) implement dual-response workflows with mandatory expert verification; (b) deploy retrieval-augmented models that return linked, labelled evidence; and (c) assess AI use as a reasoning scaffold with outcomes including trainee diagnostic accuracy, reasoning process measures, learner engagement, and indicators of automation bias. Such studies should include robust blinded assessment and both formative and summative educational endpoints.</p><p>In summary, Yitzhaki et al. provide an important empirical foundation showing that modern LLMs can contribute meaningfully to factual teaching in PICU settings but require structured oversight for reasoning tasks [<span>1</span>]. Rather than viewing LLMs as a binary replacement for educators, we suggest viewing them as adaptable partners within transparent, evidence-traceable learning ecosystems that amplify educator capacity while safeguarding clinical reasoning standards.</p><p>All authors have substantial contributions to conceptualization, literature review, writing – original draft, and writing – review and editing; and gave final approval of the version to be published.</p><p>The authors have nothing to report.</p><p>The authors declare no conflicts of interest.</p><p>Data sharing not applicable to this article as no datasets were generated or analysed during the current study.</p>","PeriodicalId":16648,"journal":{"name":"Journal of paediatrics and child health","volume":"62 1","pages":"148-149"},"PeriodicalIF":1.4,"publicationDate":"2025-10-09","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://onlinelibrary.wiley.com/doi/epdf/10.1111/jpc.70213","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145251618","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}
Fabricated or Induced Illness in Children (FIIC) is a complex form of child abuse. Harm to the child is both iatrogenic and psychological, driven by caregivers. Our aim was to characterise the clinical features and outcomes of FIIC cases in a tertiary Australian child protection unit (CPU).
Methods
Retrospective review of cases referred for concerns of FIIC over a 15-year period from 1 January 2006 to 31 December 2020. Cases were excluded if they did not meet the Royal College of Paediatrics and Child Health (RCPCH) definition of FIIC or if there was inadequate information available.
Results
Twenty-two referrals fulfilled the criteria for diagnosis of FIIC, constituting 0.23% of all referrals for physical abuse and neglect. There was a 4-year duration between first presentation and first concern for FIIC being raised. Indirect evidence of FII was identified in 95% of cases, and only one case had direct evidence in the form of laboratory proof of poisoning. Additional child protection concerns were identified in 36% of cases. Statutory child protection reporting was made in most cases (77%). In 65% of the total cases, the child remained with the carer, who continued to seek excessive medical care—all were cases of indirect FIIC.
Conclusions
Children with indirect evidence of FIIC are at risk of further harm through unnecessary and extensive medical investigations. Statutory agency intervention is often required, and further research is needed to identify effective multi-agency management strategies.
{"title":"Fabricated or Induced Illness in Children: Experience of a Tertiary Australian Children's Hospital","authors":"Sandra Hanna, Ben W. R. Balzer, Lydia J. Garside","doi":"10.1111/jpc.70206","DOIUrl":"10.1111/jpc.70206","url":null,"abstract":"<div>\u0000 \u0000 \u0000 <section>\u0000 \u0000 <h3> Aims</h3>\u0000 \u0000 <p>Fabricated or Induced Illness in Children (FIIC) is a complex form of child abuse. Harm to the child is both iatrogenic and psychological, driven by caregivers. Our aim was to characterise the clinical features and outcomes of FIIC cases in a tertiary Australian child protection unit (CPU).</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Methods</h3>\u0000 \u0000 <p>Retrospective review of cases referred for concerns of FIIC over a 15-year period from 1 January 2006 to 31 December 2020. Cases were excluded if they did not meet the Royal College of Paediatrics and Child Health (RCPCH) definition of FIIC or if there was inadequate information available.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Results</h3>\u0000 \u0000 <p>Twenty-two referrals fulfilled the criteria for diagnosis of FIIC, constituting 0.23% of all referrals for physical abuse and neglect. There was a 4-year duration between first presentation and first concern for FIIC being raised. Indirect evidence of FII was identified in 95% of cases, and only one case had direct evidence in the form of laboratory proof of poisoning. Additional child protection concerns were identified in 36% of cases. Statutory child protection reporting was made in most cases (77%). In 65% of the total cases, the child remained with the carer, who continued to seek excessive medical care—all were cases of indirect FIIC.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Conclusions</h3>\u0000 \u0000 <p>Children with indirect evidence of FIIC are at risk of further harm through unnecessary and extensive medical investigations. Statutory agency intervention is often required, and further research is needed to identify effective multi-agency management strategies.</p>\u0000 </section>\u0000 </div>","PeriodicalId":16648,"journal":{"name":"Journal of paediatrics and child health","volume":"61 12","pages":"1872-1878"},"PeriodicalIF":1.4,"publicationDate":"2025-10-08","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145244688","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}
<p>Kawasaki disease (KD) is an acute autoimmune vasculitis that primarily affects small to medium-sized arteries and can lead to multiple organ dysfunction. The classic clinical manifestations of KD include fever, conjunctival injection, cervical lymphadenopathy, polymorphous rash and oropharyngeal changes. In developed countries, KD is the leading cause of acquired heart disease in children. Without treatment, approximately 15%–25% of affected children may develop coronary artery aneurysms. Administering intravenous immunoglobulin (IVIG) within the first 10 days significantly improves the prognosis. However, some patients may present with atypical features, complicating early diagnosis.</p><p>On rare occasions, patients with KD may experience hypotension, heart failure and reduced organ perfusion. In 2009, Kanegaye et al. defined this hemodynamically unstable form of KD as Kawasaki disease shock syndrome (KDSS) [<span>1</span>]. KDSS is a severe complication, occurring in 1% to 7% of KD cases [<span>2</span>]. While the mechanism and pathophysiology of KDSS remain unclear, a cytokine storm and capillary leak are speculated to be the underlying causes [<span>3</span>]. Previously reported cardiac damage in KDSS has been primarily attributed to systolic dysfunction or coronary artery dilation [<span>4</span>], with few cases involving abnormalities in the cardiac conduction system. Here, we report a case of KDSS with a severe second-degree atrioventricular block and a reduced ventricular rate.</p><p>A 10-year-old girl initially presented with cervical lymphadenopathy and fever. She had no prior medical or family history. On the third day of illness, she was admitted to the paediatric ward with a diagnosis of lymphadenitis. Her symptoms included swollen, painful cervical lymph nodes, elevated skin temperature in the neck and a high fever (40.4°C). At the time, KD was not suspected as she lacked typical features such as a strawberry tongue, cracked lips, or conjunctivitis. Laboratory tests showed a C-reactive protein (CRP) level of 49.9 mg/L, a white blood cell count of 12.54 × 10<sup>9</sup>/L, a haemoglobin level of 134 g/L and a platelet count of 184 × 10<sup>9</sup>/L. Her sodium level was 133 mmol/L, potassium was 4.24 mmol/L and transaminase levels were normal. PCR tests for viruses, including SARS-CoV2, influenza and adenovirus, were negative. The bacterial culture result was also negative. An electrocardiogram revealed a normal sinus rhythm. The preliminary diagnosis was cervical lymphadenitis and ceftriaxone was administered to treat the infection.</p><p>However, by the fifth day, her fever persisted and her CRP level had risen to 118.11 mg/L. As a result, her antibiotic regimen was changed to sulbactam and cefoperazone. On the sixth day, her condition worsened. Her fever remained above 39°C, and she developed abdominal pain, oliguria and hypotension (blood pressure of 62/31 mmHg). Immediate fluid resuscitation and administration of dopamin
川崎病（Kawasaki disease， KD）是一种急性自身免疫性血管炎，主要影响中小动脉，可导致多器官功能障碍。KD的典型临床表现包括发热、结膜注射、颈淋巴肿大、多形皮疹和口咽改变。在发达国家，KD是儿童获得性心脏病的主要原因。如果不进行治疗，大约15%-25%的患儿可能发展为冠状动脉瘤。在头10天内静脉注射免疫球蛋白（IVIG）可显著改善预后。然而，一些患者可能表现出非典型特征，使早期诊断复杂化。在极少数情况下，KD患者可能会出现低血压、心力衰竭和器官灌注减少。2009年，Kanegaye等人将这种血流动力学不稳定的KD形式定义为川崎病休克综合征（Kawasaki disease shock syndrome， KDSS）[1]。KDSS是一种严重的并发症，发生在1%至7%的KD病例中。虽然KDSS的机制和病理生理尚不清楚，但细胞因子风暴和毛细血管泄漏被推测是导致[3]的潜在原因。先前报道的KDSS心脏损伤主要归因于收缩功能障碍或冠状动脉扩张[4]，少数病例涉及心脏传导系统异常。在这里，我们报告一例KDSS伴有严重的二度房室传导阻滞和心室率降低。一名10岁女孩最初表现为颈部淋巴结病和发烧。她没有既往病史或家族史。在患病的第三天，她被诊断为淋巴结炎而住进儿科病房。她的症状包括颈部淋巴结肿胀、疼痛、颈部皮肤温度升高和高烧（40.4°C）。当时，由于没有草莓舌、裂唇、结膜炎等典型特征，没有怀疑是KD。实验室检查显示c反应蛋白（CRP）水平为49.9 mg/L，白细胞计数为12.54 × 109/L，血红蛋白水平为134 g/L，血小板计数为184 × 109/L。钠133 mmol/L，钾4.24 mmol/L，转氨酶水平正常。包括SARS-CoV2、流感和腺病毒在内的病毒PCR检测均为阴性。细菌培养结果也为阴性。心电图显示窦性心律正常。初步诊断为宫颈淋巴结炎，给予头孢曲松治疗。然而，到第五天，她的发烧持续存在，她的CRP水平上升到118.11 mg/L。因此，她的抗生素治疗方案改为舒巴坦和头孢哌酮。第六天，她的病情恶化了。患者高烧39°C以上，出现腹痛、少尿和低血压（血压62/31 mmHg）。立即进行液体复苏并给予多巴胺和去甲肾上腺素。进行了血液和心脏检查，超声心动图显示射血分数为60%，无冠状动脉扩张，有轻度二尖瓣反流。怀疑感染性休克，我们开始用血管活性药物，利奈唑胺和美罗培南治疗。肺部CT扫描显示肺炎和胸腔积液。肾功能检查提示急性肾损伤，血尿素氮14.16 mg/dL，血清肌酐249 μmol/L。她的血压有所改善，但出现心律失常，心电图显示早搏、房室传导阻滞和左心轴。(图1)。渐渐地，KD的症状开始出现，包括结膜注射和草莓样的舌头。基于这些发现，诊断为KDSS。第7天静脉注射免疫球蛋白和阿司匹林控制炎症。发热消退，肾功能逐渐好转。患者血检结果见表1。在患病的第六天，出现了新的异常症状。患者出现不寻常的心律失常，最初表现为频繁室性早搏。心肌检查显示肌钙蛋白（0.049 ng/mL）和BNP （35 000 ng/L）水平升高，提示KDSS所致心肌损伤。静脉注射免疫球蛋白（IVIG）后，肌钙蛋白水平降至0.012 ng/mL。然而，心电图显示完全房室传导阻滞（图2）。到第8天，患者的心室率明显减慢，最低心率降至每分钟40次。心电图示房室分离（图3）。考虑到心律失常的严重程度，在第8天和第9天静脉注射类固醇脉冲治疗（10mg /kg），同时使用异丙肾上腺素来增加心率。 同时24小时动态心电图监测显示16例心房早搏，间歇性1度房室传导阻滞，7例2度房室传导阻滞，435次室性漏搏和间歇性QT间期延长。幸运的是，到第11天，她的心率稳定在每分钟80次的窦性心律，此后一直保持稳定。连续超声心动图检查显示，第11天心包少量积液，左冠状动脉扩张至0.34 cm （Z = 1.1）。左前降支内径0.23 cm (Z = 0.1)，旋支内径0.19 cm (Z =−0.6)，右冠状动脉近端开口0.28 cm (Z = 0.4)，右冠状动脉远端开口0.36 cm （Z = 3.2）。在随后的随访中没有观察到进一步的冠状动脉扩张。在继续阿司匹林治疗和逐渐减少类固醇治疗期间，患者仍无症状。出院后随访9个月，超声心动图显示无冠状动脉瘤及扩张，LVEF为64%。我们描述了一个罕见的病例，一个年轻的女孩与KD谁发展KDSS伴有严重的心律失常。最初因其非典型表现而被误诊，她的最初症状是发烧和颈部淋巴结炎。抗生素治疗被证明无效，在患病的第六天发生了休克。在出现草莓舌和结膜炎后才怀疑是KD。当典型的临床表现尚未出现时，早期诊断KD具有挑战性。在这种情况下，诊断一直不确定，直到休克发作。在临床实践中，KDSS经常被误诊为感染性休克[5]，因为这两种情况都表现为外周循环功能障碍、低血压、CRP升高和白细胞介素-10 (IL-10)[6]升高。然而，KDSS更可能涉及心肌功能障碍和冠状动脉扩张[7]。KDSS的确切病因尚不清楚，但它通常与毛细血管渗漏、细胞因子风暴和心肌功能障碍有关。区分KDSS和儿童多系统炎症综合征（MIS-C）也很重要，因为它们具有几乎相同的临床特征。然而，MIS-C与SARS-CoV-2有关，而KDSS往往在亚洲国家更频繁发生。在我们的病例中，这名女孩没有被诊断为misc，因为她的SARS-CoV-2检测结果为阴性。此外，MIS-C在西方国家（欧洲和美洲）更为常见，而KDSS在亚洲国家的发病率更高。另一个显著特征是misc更频繁地与巨噬细胞激活综合征（MAS）[8]相关。本病例的临床和实验室特征更符合KDSS的诊断标准，而非MIS-C。KDSS可导致心肌功能障碍，典型表现为射血分数降低。据我们所知，这是首次报道的与心脏传导功能异常相关的KDSS病例。据报道，约80%的KDSS患者表现为肌酸激酶- mb和肌钙蛋白- t[9]水平升高，提示心肌细胞损伤。在日本的一个病例中，心肌活检显示间质心肌有轻度纤维化和巨噬细胞和t淋巴细胞[10]浸润。然而，其他研究表明，肌钙蛋白水平升高并不总是常见的。心脏传导系统损伤的报道则更为罕见。在本病例中，心电图最初显示完全房室传导阻滞，并发展为房室分离。在给予大剂量IVIG和静脉类固醇脉冲治疗后，患者窦性心律恢复，避免了人工起搏器的植入。心电图变化与治疗过程相对应，我们认为心律失常是KDSS的一种表现。在这种情况下早期使用糖皮质激素为未来类似病例的管理提供了有价值的见解。KDSS的发病率相对较低，一些研究建立了logistic回归预测模型进行分析。与典型的KD不同，KDSS患者通常表现为血小板计数较低。这种下降可能与凝血异常活跃引起的血小板消耗有关，d -二聚体水平升高可能支持这种可能性。此外，另一项研究发现血清钠降低是KDSS[7]的独立危险因素。我们的病人大体上符合这些预测模型；然而，与以往报道不同的是，她的转氨酶和心肌酶水平保持正常。这些差异突出了不同KDSS患者之间的异质性。冠状动脉异常更容易发生在KDSS。
{"title":"Kawasaki Disease Shock Syndrome With Progressive AV Block and Atrioventricular Dissociation: Case Report","authors":"Chengjun Dai, Jiao Chen, Yutong Chen, Yingchao Ying, Yanlan Fang, Zihao Yang, Chunlin Wang","doi":"10.1111/jpc.70210","DOIUrl":"10.1111/jpc.70210","url":null,"abstract":"<p>Kawasaki disease (KD) is an acute autoimmune vasculitis that primarily affects small to medium-sized arteries and can lead to multiple organ dysfunction. The classic clinical manifestations of KD include fever, conjunctival injection, cervical lymphadenopathy, polymorphous rash and oropharyngeal changes. In developed countries, KD is the leading cause of acquired heart disease in children. Without treatment, approximately 15%–25% of affected children may develop coronary artery aneurysms. Administering intravenous immunoglobulin (IVIG) within the first 10 days significantly improves the prognosis. However, some patients may present with atypical features, complicating early diagnosis.</p><p>On rare occasions, patients with KD may experience hypotension, heart failure and reduced organ perfusion. In 2009, Kanegaye et al. defined this hemodynamically unstable form of KD as Kawasaki disease shock syndrome (KDSS) [<span>1</span>]. KDSS is a severe complication, occurring in 1% to 7% of KD cases [<span>2</span>]. While the mechanism and pathophysiology of KDSS remain unclear, a cytokine storm and capillary leak are speculated to be the underlying causes [<span>3</span>]. Previously reported cardiac damage in KDSS has been primarily attributed to systolic dysfunction or coronary artery dilation [<span>4</span>], with few cases involving abnormalities in the cardiac conduction system. Here, we report a case of KDSS with a severe second-degree atrioventricular block and a reduced ventricular rate.</p><p>A 10-year-old girl initially presented with cervical lymphadenopathy and fever. She had no prior medical or family history. On the third day of illness, she was admitted to the paediatric ward with a diagnosis of lymphadenitis. Her symptoms included swollen, painful cervical lymph nodes, elevated skin temperature in the neck and a high fever (40.4°C). At the time, KD was not suspected as she lacked typical features such as a strawberry tongue, cracked lips, or conjunctivitis. Laboratory tests showed a C-reactive protein (CRP) level of 49.9 mg/L, a white blood cell count of 12.54 × 10<sup>9</sup>/L, a haemoglobin level of 134 g/L and a platelet count of 184 × 10<sup>9</sup>/L. Her sodium level was 133 mmol/L, potassium was 4.24 mmol/L and transaminase levels were normal. PCR tests for viruses, including SARS-CoV2, influenza and adenovirus, were negative. The bacterial culture result was also negative. An electrocardiogram revealed a normal sinus rhythm. The preliminary diagnosis was cervical lymphadenitis and ceftriaxone was administered to treat the infection.</p><p>However, by the fifth day, her fever persisted and her CRP level had risen to 118.11 mg/L. As a result, her antibiotic regimen was changed to sulbactam and cefoperazone. On the sixth day, her condition worsened. Her fever remained above 39°C, and she developed abdominal pain, oliguria and hypotension (blood pressure of 62/31 mmHg). Immediate fluid resuscitation and administration of dopamin","PeriodicalId":16648,"journal":{"name":"Journal of paediatrics and child health","volume":"62 1","pages":"118-122"},"PeriodicalIF":1.4,"publicationDate":"2025-10-07","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://onlinelibrary.wiley.com/doi/epdf/10.1111/jpc.70210","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145238983","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}
Kimberley C. W. Wang, Philip J. Robinson, Alan L. James, Peter B. Noble, John G. Elliot
<p>Sudden infant death syndrome (SIDS) is a leading cause of infant deaths with a mortality rate of 20.98 per 100 000 infants worldwide [<span>1</span>]. The definition of SIDS is the sudden and unexplained death of a baby younger than 1 year of age after a thorough case investigation [<span>2</span>], no longer extending to ‘young child’ as outlined in the original classification. Sudden unexpected death in infancy (SUDI) is now the term used to capture all sudden, unexpected infant deaths, including SIDS [<span>3</span>]. Cases where children over 1 year of age die without a clear cause are classified as sudden unexplained death in childhood (SUDC) [<span>2</span>]. The clinical manifestation of SUDI/SUDC is heterogenous.</p><p>Of interest to the <i>Journal of Paediatric and Child Health</i> community, there is evidence for an effect of airway pathology that may enhance airway smooth muscle (ASM) shortening and lead to airflow limitation on SUDI/SUDC. For example, we have previously shown the layer of ASM in SIDS infants is thicker compared with controls [<span>4</span>]. Other studies report an increase in eosinophil numbers, T lymphocytes, B lymphocytes and peri-bronchial mast cells [<span>5</span>] in SIDS infants compared with control. An adverse in utero environment also affects the airway wall and is a known risk factor for SIDS. Infants who died from SIDS exposed to maternal smoking during and after pregnancy had increased inner airway wall thickness compared with SIDS cases who were not exposed to maternal smoking [<span>6</span>]. Prematurity is associated with a greater incidence of SUDI [<span>2</span>], although whether the effects of prematurity arise through alterations to airway wall structure is not known. The aim of the present study was therefore to examine the effect of prematurity on airway wall structure and ASM shortening in cases of SUDI or SUDC.</p><p>All infants involved in this study had post-mortem examinations performed by an experienced paediatric pathologist at the Victorian Institute of Forensic Medicine. Permission was obtained from the Institute's ethics committee to access the stored lung tissue from those post-mortems performed between 1991 and 1993 [<span>6</span>]. Approval for this study was obtained from the Royal Children's Hospital Ethics committee (94064C), Victorian Institute of Forensic Medicine Ethics Committee (Access Number 36/94, Human Ethics number 119/94) and Sir Charles Gairdner Hospital Human Research Ethics Committee (HREC 2015–053). Airways were available from 68 infants (post-mortem) who died between 1 and 18 months of age and were classified as SUDI or SUDC. The sample size in this study does not represent the total number of sudden infant deaths in Victoria between 1991 and 1993. Characteristics of the case series: 56% male (<i>n</i> = 38), average birth weight 3.23 ± 0.085 kg (±SEM) and 13% premature births (<i>n</i> = 9; < 37 gestational weeks). Cases examined in this study had simil
{"title":"Increased Airway Smooth Muscle Shortening Is Associated With an Earlier Age of Sudden Unexpected Death in Infancy","authors":"Kimberley C. W. Wang, Philip J. Robinson, Alan L. James, Peter B. Noble, John G. Elliot","doi":"10.1111/jpc.70211","DOIUrl":"10.1111/jpc.70211","url":null,"abstract":"<p>Sudden infant death syndrome (SIDS) is a leading cause of infant deaths with a mortality rate of 20.98 per 100 000 infants worldwide [<span>1</span>]. The definition of SIDS is the sudden and unexplained death of a baby younger than 1 year of age after a thorough case investigation [<span>2</span>], no longer extending to ‘young child’ as outlined in the original classification. Sudden unexpected death in infancy (SUDI) is now the term used to capture all sudden, unexpected infant deaths, including SIDS [<span>3</span>]. Cases where children over 1 year of age die without a clear cause are classified as sudden unexplained death in childhood (SUDC) [<span>2</span>]. The clinical manifestation of SUDI/SUDC is heterogenous.</p><p>Of interest to the <i>Journal of Paediatric and Child Health</i> community, there is evidence for an effect of airway pathology that may enhance airway smooth muscle (ASM) shortening and lead to airflow limitation on SUDI/SUDC. For example, we have previously shown the layer of ASM in SIDS infants is thicker compared with controls [<span>4</span>]. Other studies report an increase in eosinophil numbers, T lymphocytes, B lymphocytes and peri-bronchial mast cells [<span>5</span>] in SIDS infants compared with control. An adverse in utero environment also affects the airway wall and is a known risk factor for SIDS. Infants who died from SIDS exposed to maternal smoking during and after pregnancy had increased inner airway wall thickness compared with SIDS cases who were not exposed to maternal smoking [<span>6</span>]. Prematurity is associated with a greater incidence of SUDI [<span>2</span>], although whether the effects of prematurity arise through alterations to airway wall structure is not known. The aim of the present study was therefore to examine the effect of prematurity on airway wall structure and ASM shortening in cases of SUDI or SUDC.</p><p>All infants involved in this study had post-mortem examinations performed by an experienced paediatric pathologist at the Victorian Institute of Forensic Medicine. Permission was obtained from the Institute's ethics committee to access the stored lung tissue from those post-mortems performed between 1991 and 1993 [<span>6</span>]. Approval for this study was obtained from the Royal Children's Hospital Ethics committee (94064C), Victorian Institute of Forensic Medicine Ethics Committee (Access Number 36/94, Human Ethics number 119/94) and Sir Charles Gairdner Hospital Human Research Ethics Committee (HREC 2015–053). Airways were available from 68 infants (post-mortem) who died between 1 and 18 months of age and were classified as SUDI or SUDC. The sample size in this study does not represent the total number of sudden infant deaths in Victoria between 1991 and 1993. Characteristics of the case series: 56% male (<i>n</i> = 38), average birth weight 3.23 ± 0.085 kg (±SEM) and 13% premature births (<i>n</i> = 9; < 37 gestational weeks). Cases examined in this study had simil","PeriodicalId":16648,"journal":{"name":"Journal of paediatrics and child health","volume":"61 11","pages":"1818-1820"},"PeriodicalIF":1.4,"publicationDate":"2025-10-07","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://onlinelibrary.wiley.com/doi/epdf/10.1111/jpc.70211","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145239014","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}
Şeyma Bütün Türk, Davut Bozkaya, Burçin Kurtipek, Turan Bayhan, İkbal Ok Bozkaya, Şerife Suna Oğuz, Gülsüm İclal Bayhan
{"title":"Causes of Blueberry Muffin Rash Beyond TORCH","authors":"Şeyma Bütün Türk, Davut Bozkaya, Burçin Kurtipek, Turan Bayhan, İkbal Ok Bozkaya, Şerife Suna Oğuz, Gülsüm İclal Bayhan","doi":"10.1111/jpc.70208","DOIUrl":"10.1111/jpc.70208","url":null,"abstract":"","PeriodicalId":16648,"journal":{"name":"Journal of paediatrics and child health","volume":"61 12","pages":"1944-1946"},"PeriodicalIF":1.4,"publicationDate":"2025-10-07","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145238954","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}
With over 1000 genetic causes for neurodevelopmental conditions, genetic testing (including exome sequencing) is recommended for people with intellectual disability to guide clinical care, as well as improve empowerment, connection to peer supports, and access to funded therapies. Many genetic neurodevelopmental conditions are inherited, with a parent sharing the genetic change identified in their child. However, despite showing interest in genetic medicine, many young people and adults with intellectual disability feel excluded from full participation. There is a lack of accessible resources to support people with intellectual disability in making informed choices about genetic tests and understanding their test results. There is also little training available to healthcare professionals to help them communicate with and support young people with intellectual disability and their parents about genetics. This situation reflects a broader exclusion of people with intellectual disability from equitable and respectful access to mainstream healthcare, as highlighted in the National Roadmap to Improving the Health of People with Intellectual Disability (2021) and the Royal Commission into Violence, Abuse, Neglect and Exploitation of People with Disability (2023). In this article, we discuss key approaches and co-produced resources (i.e., resources created together with people with intellectual disability, academic researchers, healthcare professionals, and teachers) to equip healthcare professionals to empower children, young people, and parents with intellectual disability to make informed decisions about genetic testing and understand their test results. We highlight the need for inclusive, person-centred, and respectful healthcare to ensure genetic medicine is equitable and accessible.
{"title":"Shared Decision-Making for Genetic Tests With Children and Young People With Intellectual Disability: Considerations for Inclusive, Person-Centred, and Respectful Approaches.","authors":"Manjekah Dunn, Iva Strnadová, Michelle Tso, Claudia Pantoja Mardones, Jackie Boyle, Erica Longhurst, Julie Loblinzk Refalo, Skie Sarfaraz, Bronwyn Terrill, Elizabeth Emma Palmer","doi":"10.1111/jpc.70202","DOIUrl":"https://doi.org/10.1111/jpc.70202","url":null,"abstract":"<p><p>With over 1000 genetic causes for neurodevelopmental conditions, genetic testing (including exome sequencing) is recommended for people with intellectual disability to guide clinical care, as well as improve empowerment, connection to peer supports, and access to funded therapies. Many genetic neurodevelopmental conditions are inherited, with a parent sharing the genetic change identified in their child. However, despite showing interest in genetic medicine, many young people and adults with intellectual disability feel excluded from full participation. There is a lack of accessible resources to support people with intellectual disability in making informed choices about genetic tests and understanding their test results. There is also little training available to healthcare professionals to help them communicate with and support young people with intellectual disability and their parents about genetics. This situation reflects a broader exclusion of people with intellectual disability from equitable and respectful access to mainstream healthcare, as highlighted in the National Roadmap to Improving the Health of People with Intellectual Disability (2021) and the Royal Commission into Violence, Abuse, Neglect and Exploitation of People with Disability (2023). In this article, we discuss key approaches and co-produced resources (i.e., resources created together with people with intellectual disability, academic researchers, healthcare professionals, and teachers) to equip healthcare professionals to empower children, young people, and parents with intellectual disability to make informed decisions about genetic testing and understand their test results. We highlight the need for inclusive, person-centred, and respectful healthcare to ensure genetic medicine is equitable and accessible.</p>","PeriodicalId":16648,"journal":{"name":"Journal of paediatrics and child health","volume":" ","pages":""},"PeriodicalIF":1.4,"publicationDate":"2025-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145199457","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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