The authors examined two Japanese siblings with a recessive hereditary spastic paraplegia (HSP) with dementia and a thin corpus callosum. Both showed thalamic glucose hypometabolism on PET. Recessive HSP with a thin corpus callosum is a rare disorder, with less than 20 reported patients, that may be a Japanese subtype of HSP.

BackgroundChild abuse and/or neglect is a serious issue, and in many cases, parents are the perpetrators. Hospital-based child protection teams (CPTs) play pivotal roles in the management of not only abused and/or neglected children but also of their parents; this is generally conducted through multidisciplinary practice. The aim of this study is to survey hospital-based CPT members to determine the professions they perceive to be most applicable to participation in CPTs.Participants and methodsThe participants were members of CPTs affiliated with hospitals that had pediatric emergency departments and which were located in Chiba Prefecture; specifically, 114 CPT members from 23 hospitals responded to this survey. The two main questionnaire items concerned are as follows: 1) each respondent’s evaluation of conducting assessments, providing support, and implementing multidisciplinary collaborative practice in the treatment of abusive and negligent parents, and 2) each CPT member’s opinion on the professions that are most important for CPT activities. An exploratory factor analysis (EFA) was performed to explore the factor structure of the data, and a correlation analysis was performed using the result obtained.ResultsThe EFA returned two factors: multidisciplinary collaborative practice (α = 0.84) and assessment and support (α = 0.89). A correlational analysis showed that multidisciplinary collaborative practice had a positive correlation for obstetricians (r = 0.315, p = 0.001), neonatologists (r = 0.261, p = 0.007), midwives (r = 0.248, p = 0.011), and psychiatrists (r = 0.194, p = 0.048); however, assessment and support was only significantly correlated with midwives (r = 0.208, p = 0.039).ConclusionThis study showed that hospital-based CPT members highly evaluate multidisciplinary collaborative practice for the management of abusive and/or negligent parents, and they believe that, in addition to pediatric physicians and nurses, perinatal care and mental health professionals are the most important participants in advanced CPT activities.

Nontyphoidal Salmonella (NTS) can cause bacterial enterocolitis. Although some children with NTS infection develop bacteremia, its clinical manifestations have not been discussed adequately. Therefore, we examined children with NTS bacteremia. We retrospectively examined the medical records of 15 patients aged less than 15 years. Salmonella spp. were detected in the blood cultures of these patients between 1991 and 2014. We divided an additional sample group of 34 patients diagnosed with an NTS infection between 2005 and 2014, into 2 groups. Group bacteremia (B) included patients in whose blood cultures Salmonella spp. were detected, and group non-bacteremia (NB) included patients in whom Salmonella infection was not detected. We compared each group using Wilcoxon test and Fisher’s exact test. The number of patients with fever, diarrhea, or abdominal pain was 15 (100%), 13 (87%), and 9 (60%), respectively, in the first sample of patients. However, vomiting and bloody stool were observed in only 5 patients (33%). More than 70% of patients exhibited a reduced white blood cell count, while C-reactive protein levels were variable in the patients. Salmonella spp. were detected via stool culture in 10 patients (67%). Diarrhea persisted for more than 4 days more frequently in group B than group NB (p = 0.004). The number of patients whose fever persisted for more than 4 days was significantly higher in group B than group NB (p = 0.030). Therefore, if NTS bacteremia is suspected, blood cultures should be collected and antibiotics should be initiated in cases with diarrhea or fever for more than 4 days. Furthermore, a negative stool culture result does not preclude the possibility of NTS bacteremia.

Objectives:The objective of this study was to determine whether the rates of abdominal pain or irritability, vomiting, and hematochezia differ depending on the duration of symptoms and age of the children with ileocolic intussusception. Methods:We retrospectively investigated the charts of ileocolic intussusception children between January 2008 and December 2017 at a rural general hospital in Japan. Children were separated into 2 groups: the early visiting group, including children examined within 6 hours after onset, and the late visiting group, including children examined more than 6 hours after onset. We further separated them into 2 groups based on age: the infant group (age, <18 months) and the child group (age, ≥18 months). We compared clinical features, such as abdominal pain or irritability, vomiting, and hematochezia, between each group.Results: Among 105 children with ileocolic intussusception, 51 were in the early visiting group and 49 were in the infant group. Hematochezia less frequently occurred in the early visiting group than in the late visiting group (29% vs 50%, P = 0.046). Furthermore, abdominal pain or irritability occurred less frequently in the infant group than in the child group (79.6% vs 98.2%, P = 0.003). Conversely, vomiting and hematochezia were more frequent in the infant group than in the child group (83.7% vs 51.8%, P < 0.001; 55.1% vs 26.8%, P = 0.005).Conclusions: Clinical features of pediatric ileocolic intussusception may depend on symptom duration and age.

Professionals from a range of fields need clinical skills and judgement to decide if a child's injuries are due to maltreatment. The combination of increased clinical experience along with a high-quality didactic lecture, appears to be the most effective method of raising awareness and enhancing skills.