A regional multidisciplinary network enhances child abuse case management.

BACKGROUND: We previously reported an inadequate response to intracranial hemorrhage (ICH) cases under 24 months of age in Yokohama from 2011 to 2013. Hence, it is very important to evaluate how the establishment of a regional multidisciplinary network for child abuse affects the response to ICH cases in medical institutions. METHODS: We conducted a questionnaire survey of ICH cases under 24 months of age from 2014 to 2016 using a regional multidisciplinary network for child abuse established in Yokohama in September 2013. We investigated the patients' characteristics, examinations to identify inflicted injury, and reports made to the hospital-based child protection team (CPT) or regional child protective service (CPS), and compared the results of a previous study and the current study, which corresponds to before and after the establishment of the regional network, respectively. RESULTS: The total number of ICH cases was 50 in 3 years. The number of cases surveyed for covert fracture and fundus hemorrhage increased significantly after the establishment of the regional network (P = 0.0001 and P = 0.0182, respectively). The number of cases reported as suspected child abuse was 41 (82%) to the hospital-based CPTs and 27 (54%) to the regional CPSs. There were significant differences between before and after the establishment of the regional network regarding CPT (P = 0.0062) and CPS (P = 0.0215) reports. CONCLUSIONS: A regional multidisciplinary network can enhance response and cooperation to address child abuse. It deepens our understanding of such care and improves awareness by hospital personnel of child abuse.

Epidemiology of child mortality and challenges in child death review in Japan: The Committee on Child Death Review: A Committee Report: The Committee on Child Death Review: A Committee Report.

We performed a retrospective survey and verification of the medical records of death cases of children (and adolescents; aged <18 years) between 2014 and 2016 in pediatric specialty training facilities in Japan. Of the 2,827 registered cases at 163 facilities, 2,348 cases were included. The rate of identified deaths compared with the demographic survey, was 18.2%-21.0% by age group. The breakdown of deaths was determined as follows: 638 cases (27.2%) were due to external factors or unknown causes, 118 (5.0%) were suspected to involve child maltreatment, 932 (39.7%) were of moderate or high preventability or were indeterminable. Further detailed verification was required for 1,333 cases (56.8%). Comparison of the three prefectures with high rates of identified deaths in Japan revealed no significant differences, such as in the distribution of diseases, suggesting that there was little selection bias. The autopsy rate of deaths of unknown cause was 43.4%, indicating a high ratio of forensic autopsies. However, sufficient clinical information was not collected; therefore, thorough evaluations were difficult to perform. Cases with a moderate or high possibility of involvement of child maltreatment accounted for 5%, similar to previous studies. However, more objective evaluation is necessary. Preventable death cases including potentially preventable deaths accounted for 25%, indicating that proposals need to be made for specific preventive measures. Individual primary verification followed by secondary verification by multiple organizations is effective. It is anticipated that a child death review (CDR) system with such a multi-layered structure will be established; however, the following challenges were revealed: The subjects of CDR are all child deaths. Even if natural death cases are entrusted to medical organizations, and complicated cases to other special panels, the numbers are very high. Procedures need to be established to sufficiently verify these cases. Although demographic statistics are useful for identifying all deaths, care must be taken when interpreting such data. Detailed verification of the cause of death will affect the determination of subsequent preventability. Verification based only on clinical information is difficult, so a procedure that collates non-medical information sources should be established. It is necessary to organize the procedures to evaluate the involvement of child maltreatment objectively and raise awareness among practitioners. To propose specific preventive measures, a mechanism to ensure multiprofessional diverse perspectives is crucial, in addition to fostering the foundation of individual practitioners. To implement the proposed measures, it is also necessary to discuss the responsibilities and authority of each organization. Once the CDR system is implemented, verification of the system should be repeated. Efforts to learn from child deaths and prevent deaths that are preventable as much as possible are essential duties of pediatricians. Pediatricians are expected to undertake the identified challenges and promote and lead the implementation of the CDR system. This is a word-for-word translation of the report in J. Jpn. Pediatr. Soc. 2019; 123 (11): 1736-1750, which is available only in the Japanese language.

Mutation of junctophilin type 2 associated with hypertrophic cardiomyopathy.

Junctophilin subtypes, designated as JPH1 approximately 4, are protein components of junctional complexes and play essential roles in cellular Ca2+ signaling in excitable cells. Knockout mice lacking the cardiac-type Jph2 die of embryonic cardiac arrest, and the mutant cardiac myocytes exhibit impaired formation of peripheral couplings and arrhythmic Ca2+ signaling caused by functional uncoupling between dihydropyridine and ryanodine receptor channels. Based on these observations, we hypothesized that mutations of JPH2 could cause human genetic cardiac diseases. Among 195 Japanese patients (148 index cases and 47 affected family members) with hypertrophic cardiomyopathy (HCM), two heterozygous nonsynonymous nucleotide transitions, G505S and R436C, were newly found in JPH2. When Fisher's exact test was used to compare index cases with HCM to unrelated Japanese healthy controls in the frequencies of mutant alleles, only the G505S mutation showed statistical significance (4/296 HCM patients and 0/472 control individuals, P=0.022). This result was still significant after Bonferroni's correction for multiple comparisons (P=0.044). To the best of our knowledge, this is the first report on JPH2 mutation associated with HCM.

A rare case of Salmonella soft-tissue abscess.

A healthy 6-year-old boy had complained of fever and chest pain for 3 days. On admission, he had a mass on the sternum, 3.7 x 2.5 cm in size. Abnormal laboratory findings included a white blood cell count of 12,900/microl, erythrocyte sedimentation rate (ESR), 74 mm/h, and C-reactive protein (CRP), 9.7 mg/dl. Ultrasound examination of the chest revealed a hypoechoic lesion on the sternum that was 30 x 15 mm in size. Chest computed tomography (CT) scan showed no bone fracture or bone erosion. The patient received cefpirome, given intravenously at 60 mg/kg per day for 10 days. Incision and drainage was performed on the seventh day in the hospital, and we collected 0.5 ml of pus. Salmonella enteritidis was detected from the drainage. However, the patient had no gastrointestinal symptoms. He was discharged on the fourteenth hospital day, as he was asymptomatic. Results of all physical and laboratory examinations including blood and stool cultures and ultrasound examinations, were within the normal limits upon discharge.