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THE JOURNAL OF THE JAPAN PEDIATRIC SOCIETY
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Vol.129, No.8, August 2025
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Original Article
Title
Life-Threatening Arrhythmias in Pediatric Cardiomyopathy: A Case Series Highlighting the Challenges of Management in School-Aged Children
Author
Takuto Yamada1)2) Yohei Yamaguchi1) Taku Ishii3) and Susumu Hosokawa1)4)
1)Department of Pediatrics, Institute of Science Tokyo Hospital
2)Department of Pediatrics, Tsuchiura Kyoudo Hospital
3)Department of Pediatrics, Perinatal and Maternal Medicine (Ibaraki), Institute of Science Tokyo
4)Department of Pediatrics, Musashino Red Cross Hospital
Abstract
Life-threatening arrhythmias are known to occur frequently in pediatric cardiomyopathy, but detailed reports on these events are limited. We present four cases of cardiomyopathy diagnosed before the age of 15, all experiencing life-threatening arrhythmias. The cases included three with hypertrophic cardiomyopathy and one with arrhythmogenic right ventricular cardiomyopathy. The median age at diagnosis was 13.6 years (range: 6.6-14.6 years), and the median age at the first arrhythmic event was 14.4 years (range: 12.7-14.6 years). All initial events occurred during physical activity at school, and each patient received an implantable cardioverter-defibrillator (ICD) after successful resuscitation. In two cases, cardiomyopathy was diagnosed following the arrhythmic event. During the follow-up period of 3.0-11.9 years (median: 6.4 years), all patients experienced appropriate ICD interventions, totaling nine episodes. Triggers included exceeding prescribed activity limits (two episodes), mild exercise within limits (three episodes), minor exertion during daily activities (five episodes), and rest (one episode). Of the 13 life-threatening arrhythmic episodes recorded, eight occurred at school, with a peak incidence around age 14. Despite intensified medication after each ICD activation, all patients experienced further appropriate ICD interventions, even on dual therapy. Two patients received amiodarone, with no symptomatic ventricular arrhythmias during its use. Managing life-threatening arrhythmias in early-onset cardiomyopathy is challenging, as strict medication adherence and exercise restrictions alone may not suffice, highlighting the importance of daily risk management.
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Original Article
Title
Assessment of Intensive Drug Overdose by Strengths and Difficulties Questionnaire: A Single-center, Retrospective Study
Author
Kisho Shimura1) Ryosuke Fukushima1) and Takuma Sakon2)
1)Pediatric Emergency Department, Fujisawa City Hospital
2)Pediatrics Department, Fujisawa City Hospital
Abstract
Purpose: As the deterioration of mental health among youths has been noted, the number of children who abuse drugs has increased. A retrospective single-center study was conducted to assess whether the Strengths and Difficulties Questionnaire (SDQ) could assess cases of intentional drug overdoses.
Methods: This study included patients aged 11-15 years who visited or were admitted to our hospital between January and March 2024 and answered the SDQ. Patients were divided into two groups: an overdose (OD) group and a control group composed of patients other than OD. Characteristics and SDQ scores, especially the total difficulties score (TDS), the assessment score of conduct and emotional dysregulation (SDQ-DP), and SDQ subscales, were compared between the two groups.
Results: There were 6 cases in the OD group (5 girls) and 46 in the control group (27 girls). The median age was 14.5 and 13 years in the OD and control groups, respectively. Regarding the SDQ scores, TDS was 19.5 in the OD group and 11 in the control group, and SDQ-DP was 4 in the OD group and 2 in the control group. Both scores were significantly different between the two groups. Among the subscales of SDQ-DP, emotional symptoms and hyperactivity/inattention subscales had a tendency of higher scores in the OD group. In the multi-regression analyses adjusted for age and sex, both TDS and SDQ-DP significantly differed between the two groups.
Conclusion: SDQ scores, especially TDS and SDQ-DP, may be higher in cases of intentional drug overdose. More cases need to be accumulated for these scores to be used as a universal assessment tool for patients with overdose. In particular, the risk of intentional overdose may be higher in patients with severe mood [affective] disorders who tend to be hyperactive, and the SDQ may be useful in screening such patients.
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Original Article
Title
IgE-mediated Food Allergy in Children with Medical Complexity
Author
Daichi Suzuki1) Miori Sato1) Seiko Hirai1) Kenji Toyokuni1) Kiwako Yamamoto-Hanada1) Tatsuki Fukuie1) Tomoo Nakamura2) and Yukihiro Ohya1)
1)Allergy Center, National Center for Child Health and Development
2)Department of Home Care Department, Department of General Pediatrics & Interdisciplinary Medicine, National Center for Child Health and Development
Abstract
It is challenging to accurately assess and judge the condition of children with medical complexity at the onset of symptoms because of difficulties in communication, who often have various underlying diseases and multiple coexisting conditions. There have been no case series of IgE-mediated food allergies in children with medical complexity regarding causative food, severity of symptoms, oral food challenges (OFCs), and treatment progress.
We included 45 children with IgE-mediated food allergy and medical complexity, and conducted 54 OFCs on 24 children. Chicken eggs and cow's milk accounted for 60% of all OFCs. The results were positive in 18 (33%), negative in 32 (59%), and withheld in 4 (7%). The most common symptoms were skin symptoms in 9 out of 18 positive OFCs (50%), gastrointestinal symptoms in 7 OFCs (39%), and oral mucosal symptoms in 5 OFCs (28%). Neurological and circulatory symptoms were both present in one case of anaphylaxis. In 15 OFCs, the symptoms resolved without treatment. IgE-mediated food allergy children with medical complexity could safely undergo OFCs to assess the safe amount of food and adopt their diet based on their appropriate diagnosis.
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Case Report
Title
A Case of a 6-Year-Old Child Diagnosed with Bow Hunter Syndrome due to Repeated Cerebral Infarctions in the Posterior Circulatory System
Author
Erika Noda1) Yuki Tenma1) Kahori Namba1) Nobuyuki Mori2) Yohei Takenobu3) Kenji Hashimoto4) Ryuta Matsuoka5) Yasuhiro Takeshima5) and Hisanori Fujino1)
1)Department of Pediatrics, Osaka Red Cross Hospital
2)Department of Radiology, Osaka Red Cross Hospital
3)Department of Neurology, Osaka Red Cross Hospital
4)Department of Neurosurgery, Osaka Red Cross Hospital
5)Department of Neurosurgery, Nara Medical University Hospital
Abstract
Cerebral infarction in childhood is rare and caused by a variety of diseases, including heart disease, cerebral vascular malformation, thrombosis, and metabolic disorders. We report a case of a 6-year-old boy with recurrent cerebral infarctions in the posterior circulation, which were then diagnosed as Bow-Hunter's syndrome.
The patient was otherwise healthy and visited our hospital complaining of unsteadiness and gait disturbance. Head MRI revealed bilateral cerebellar infarctions. After initiating anticoagulant therapy, the patient's symptoms gradually improved without new neurological symptoms. However, a head MRI taken on the sixth day of hospitalization revealed a new cerebral infarction in the left occipital lobe and a thrombus in the left vertebral artery. Ultrasound showed that the flow velocity in the left vertebral artery decreased when the cervical spine was rotated to the right, and the patient was diagnosed with Bow hunter syndrome. Considering the risk of intraarterial thrombus and scattering, the patient was started on the oral administration of antiplatelet drugs and anticoagulants, as well as a cervical collar to restrict the rotation of the neck. After initiating the treatments, no new cerebral infarctions have been observed, and the thrombus in the left vertebral artery gradually disappeared within two months. To prevent recurrence, spinal fusion surgery was performed.
When a child presents with cerebral infarction in the posterior circulation, cervical ultrasound is a simple and useful screening test.
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Case Report
Title
A Neonatal Case of Intertrigo Caused by Streptococcus agalactiae
Author
Hiromu Watanabe1) Shigeki Ishii1) Tomoko Kourogi1) Yoshiko Yamamura1) Tomoko Ohhira1) Keigo Nakatani1) and Satoshi Nakano2)
1)Department of Pediatrics, Miyazaki Prefectural Miyazaki Hospital
2)National Institute of Infectious Diseases Drug Durability Research Center Room 7
Abstract
Intertrigo is a superficial dermatitis that occurs in intertriginous areas where skin surfaces are in close contact, such as the neck, axillae, and groin. It can potentially lead to secondary bacterial infections. Reports of intertrigo caused by Group A Streptococcus and Candida have been documented; however, to date, no cases of intertrigo caused by Streptococcus agalactiae (Group B Streptococcus; GBS) have been confirmed. Herein, we report a case of intertrigo caused by GBS in an 11-day-old healthy female neonate.
She was brought to a local physician due to erythema and erosion in both axillae and was subsequently referred to our hospital with a suspected diagnosis of staphylococcal scalded skin syndrome. On examination, well-demarcated erythema with exudate localized to the intertriginous areas was observed, leading to a diagnosis of intertrigo. Considering her neonatal status, she was admitted to the hospital, and intravenous antibiotics were administered, resulting in rapid improvement of her skin lesions.
Skin cultures obtained from the axillae later identified GBS as the causative pathogen. The characteristic skin findings and the favorable clinical course confirmed GBS as the etiological agent of intertrigo in this case.
Intertrigo is typically a superficial skin infection without systemic symptoms. However, GBS infections in neonates can potentially lead to severe complications. Fortunately, this patient's clinical course was favorable, with prompt improvement following treatment. Despite the benign nature of most intertrigo cases, this report highlights the need to consider GBS as a potential pathogen, particularly in neonates, to ensure appropriate diagnosis and management.
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Case Report
Title
A Patient with Severe Motor and Intellectual Disabilities That Received Ethics Committee Approval Owing to the Absence of Familial Consent
Author
Shinji Yamakura
Tsubasa Shizuoka
Abstract
The decisions of the ethics committee addressed situations involving children and adults with severe motor and intellectual disabilities (SMID) who had no immediate family, such as parents or siblings, and were residing in a care facility. The decision-making process involved four conferences attended by doctors and nurses, during which the on-site staff reached a consensus in favor of proceeding with the surgery. Additionally, the director of A Hospital and the head of its urology department were consulted to assess the necessity of the procedure. Permission to perform the surgery was obtained prior to convening an ethics committee meeting. The ethics committee included both the facility's administrative staff and external members, such as the honorary director of A Hospital, the facility's legal advisor, the principal of a special needs school, a Christian church pastor, the child's legal guardian, the head of the urology department at A hospital, and the nurse responsible for the case. After a thorough discussion, the ethics committee granted their consent for the surgery, which was later performed successfully. In cases where children and adults with SMID are unable to make medical decisions and have no relatives to provide consent, it is recommended that decisions be made on the patient's behalf through collaborative discussions among relevant stakeholders. These discussions should prioritize the patient's best interests and involve an ethics committee with legal representation to be consulted, discussed, and approved.
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Brief Report
Title
The Use of Epinephrine in In-Hospital Cardiopulmonary Resuscitation Events from a Multi-Center Resuscitation Registry in Japan
Author
Yoko Miyahara1)2)3) Jun Honma1) Naoki Shimizu1)3) Satoshi Nakagawa2) Atsushi Kawaguchi4)5)6) and Japan National Registry of Cardio-Pulmonary Resuscitation: J-NRCPR
1)Department of Intensive Care, Tokyo Metropolitan Children's Medical Center
2)Division of Critical Care Medicine, Department of Critical Care and Anesthesia, National Center for Child Health and Development
3)Department of Pediatrics, St. Marianna University School of Medicine
4)Division of Pediatric Critical Care Medicine, Department of Pediatrics, St. Marianna University School of Medicine
5)Department of Anesthesia and YCU Center for Novel and Exploratory Clinical Trials, Yokohama City University
6)CHU Sainte Justine Research Centre, University of Montreal
Abstract
Adrenaline is the first-line drug for cardiopulmonary resuscitation in children and is widely used in Japan. However, there are no comprehensive reports on its actual use in Japanese institutions. Using data from the Pediatric In-Hospital Cardiopulmonary Resuscitation Registry, we investigated the use of epinephrine. Sixty-seven percent of patients received the appropriate dose of epinephrine (0.01±0.005 mg/kg) based on the Pediatric Advanced Life Support Recommendations, but 7% received a 10-fold overdose (≥0.1 mg/kg). In 23% of the epinephrine cases, it took more than 5 minutes from the start of chest compressions to the first dose, and in 5%, it took 20 minutes or more.
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