National survey of hospital child protection teams in Japan.

This study aimed to investigate the penetration rate of child protection teams (CPTs) in medical institutions and associations between CPT functions and hospital services. We collected data in October of 2015 from 377 hospitals in Japan offering pediatric organ transplantation. The questionnaire included questions regarding the existence of a CPT, the number of child maltreatment cases discussed and reported per year, CPT functions including 21 items about staffing, manuals, meeting, prevention, education, and collaboration, and the services provided by the hospital. Of the 377 institutions, 122 (32.4%) answered the survey. There were significant associations between CPT functions and the number of pediatric beds (r = .27), number of pediatricians (r = .27), number of outpatients (r = .39), number of emergency outpatients (r = .28), and emergency medical care (p = .009). In a multiple regression analysis, CPT functions were significantly associated with the number of CPT members, pediatric outpatient numbers, and pediatric emergency outpatient numbers. Japan has no CPT guidelines that outline what CPTs should offer in terms of structure, staffing, functions, and systems. Hospitals with many pediatric and emergency outpatients are expected to play major roles in providing services such as specialty care, intensive care, and education. They are also expected to play a role in detecting and managing child maltreatment, and have, by their own initiative, improved their capacities to achieve these goals.

Recurrence of Fever After Initial Intravenous Immunoglobulin Treatment in Children With Kawasaki Disease.

The standard treatment for Kawasaki disease (KD) is high-dose intravenous immunoglobulin (IVIG). Some patients experienced recurrent fever after IVIG following defervescence. However, little is known about the frequency of such episodes and the clinical outcome for such patients. We classified 195 KD patients into 4 groups based on their fever patterns after initial IVIG treatment: group NR (no response), group EF (early recurrent fever within 72 hours after defervescence), group LF (late recurrent fever >72 hours after defervescence), and group GR (good response). We compared the clinical characteristics and laboratory data among these groups retrospectively. Nearly a third of patients had recurrent fever (group EF, n = 45; group LF, n = 11). In two-thirds of these patients, the fever had subsided spontaneously without retreatment; 2 patients in Group LF with smoldering KD symptoms had developed coronary artery lesions (CALs) even after additional IVIG. Recurrent fever and smoldering symptoms might be risk factors for CAL.

Training program for Japanese medical personnel to combat child maltreatment.

BACKGROUND: In 2014, we created a training program for personnel in medical institutions in Japan to combat child maltreatment. The aim of the present study was to document the effectiveness of this program. METHODS: Participants completed a questionnaire before and after the training lecture. The questionnaire designed for the training program included demographic questions such years of practice and area of specialty (i.e. physician, nurse, social worker, public health nurse, technician, and others), as well as experience of suspected child maltreatment cases and training in dealing with such cases. The questionnaire included 15 statements designed to ascertain practical knowledge and attitudes relevant to addressing child maltreatment. Baseline score measured before the lecture was compared with that obtained after the lecture. RESULTS: A total of 760 participants completed the survey, including 227 physicians, 223 nurses, 38 technologists, 27 social workers, 11 public health nurses, and 174 with other occupations, and 60 participants who left their occupation as blank. There was a significant difference between the baseline score of participants with versus without experience in suspected child maltreatment or training to deal with child maltreatment (F = 16.3; P < 0.001). After the lecture, the average score rose above the baseline (11.18 vs 10.57). The rate of correct answers for nine questionnaire items increased significantly. CONCLUSIONS: 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.

Decreased levels of inflammatory cytokines in immunoglobulin-resistant Kawasaki disease after plasma exchange.

The pathogenesis of coronary artery aneurysm (CAA) formation in Kawasaki disease (KD) remains unknown. However, inflammatory cytokines are thought to play an important role in KD. Patients with intravenous immunoglobulin (IVIG)-resistant KD are more likely to develop CAA. For such refractory patients, steroids and emerging infliximab (IFX) are used; however, further verification is required for their efficacy and safety. Plasma exchange (PE), which removes various inflammatory cytokines, has been used in Japan for over 15 years to prevent CAA in IVIG-resistant KD patients. The sequential change in inflammatory cytokines during the time course of PE has yet to be investigated. In this study, we measured plasma levels of 13 cytokines in nine children with IVIG-resistant KD before the start of PE (day 0: D0), as well as at 1 or 2 days (D1/2), and 4 or 5 days (D4/5) after starting PE. The median age of onset was 8 months (range: 3-53 months). Before PE, patients were treated with IVIG (median dose: 4 g/kg, range: 3-4 g/kg). The median starting period of PE was 8 days after the onset of fever (range: 6-21 days), while its duration was 3 days (range: 2-5 days). Among the 13 cytokines, interleukin-6, tumor necrosis factor-α, tumor necrosis factor receptor I (TNFR1), TNFR2, granulocyte colony-stimulating factor, and IL-17 were significantly lower at D4/5 compared with D0 and/or D1/2, reflecting the potential central efficacy of PE. While three patients developed moderate CAA, their condition regressed within 1 year. The removal of inflammatory cytokines could be the central efficacy of PE against refractory KD.

A case of macrophage activation syndrome developing in a patient with chronic granulomatous disease-associated colitis.

Although macrophage activation syndrome (MAS) develops in some patients with chronic granulomatous disease (CGD), all of the reported cases have been associated with pathogenic microbial infections. We report a 2-year-old boy with CGD-associated colitis who suffered from MAS without any clinical signs of a microbial infection. He was treated with 1 course of methylprednisolone pulse therapy and the clinical symptoms improved; however, the colitis was difficult to control even with immunosuppressive drugs, and he eventually required hematopoietic stem cell transplantation 1 year after the onset of MAS. It is likely that MAS develops in patients with CGD colitis independent of microbial infections.

Comparison of patients with Kawasaki disease with retropharyngeal edema and patients with retropharyngeal abscess.

UNLABELLED: Kawasaki disease with retropharyngeal edema (KD with RPE) is a rare complication, and it is diagnosed by neck CT. Most reported cases had a delayed diagnosis because those patients' conditions were misdiagnosed as retropharyngeal abscess (RPA). The purpose of this study was to differentiate KD with RPE from RPA. We performed a retrospective case-control study comparing children with KD with RPE to those with RPA hospitalized at the tertiary pediatric hospital in Tokyo between 2005 and 2011. The 39 patients revealing RPE on neck CT were divided into two groups: group A was classified as KD (n = 21) and group B was classified as non-KD (n = 18). Patients in group B were finally evaluated as having RPA clinically and were treated with antibiotic therapy. A significantly higher proportion of patients in group B complained of dysphagia (11 patients vs. 5 patients; p = 0.0170) and neck pain (17 patients vs. 12 patients; p = 0.0106). Neck CT revealed a ring enhancement (16 patients vs. no patients; p < 0.0001) and mass effect in a greater proportion of patients in group B (11 patients vs. 1 patient; p < 0.0003). CONCLUSION: Careful attention to manifestations and close analyses of CT imaging may allow clinicians to differentiate KD with RPE from RPA.

Late development of coronary artery abnormalities could be associated with persistence of non-fever symptoms in Kawasaki disease.

BACKGROUND: Persistent fever after intravenous immunoglobulin (IVIG) is considered to be a major criterion of IVIG resistance in Kawasaki disease (KD), and a risk factor for the development of coronary artery abnormalities (CAA). However, the importance of persistent non-fever symptoms after defervescence has not yet been investigated. We examined the relationship between persistent non-fever symptoms and CAA in KD. METHODS: We conducted a retrospective cohort study of patients hospitalized with KD at the National Center for Child Health and Development between 1 April 2008 and 31 March 2009. Patients were divided into two groups; group A included patients who still had non-fever symptoms one month after onset of the illness and group B included patients who did not have persistent non-fever symptoms. Demographic, clinical variables were compared between the groups. RESULTS: Seventy-seven KD patients treated with IVIG were retrospectively analyzed. Patients were divided into two groups; group A included 12 (15.6%) patients and group B 65 (84.4%) patients. Demographic data, baseline laboratory data, and fever duration did not differ between the groups. In group A patients the most common persistent non-fever symptoms were lip erythema (n = 6) and bulbar conjunctivitis (n = 8). One month after onset of the illness CAA developed in seven of 77 patients (9.1%), four (33%) in group A and three (4.6%) in group B (odds ratio 10.3; 95% CI 1.9-54.8). Three patients in group A and one patient in group B developed CAA after the resolution of fever. CONCLUSIONS: Persistence of non-fever symptoms after IVIG may suggest persistence of latent inflammation, which may increase the risk of CAA. Therefore, patients with persistent non-fever symptoms may be at risk of developing CAA, even after defervescence. A prospective trial of additional IVIG for such patients should be considered.

[Diagnostic value of brain biopsy in a pediatric multiple sclerosis mimicking brain stem glioma].

Diagnosis of multiple sclerosis (MS) is difficult when the lesion mimics glioma or cerebral enchephalitis. We report a case of pediatric MS initially suspected as brain stem glioma. An 11-year-old boy developed left foot joint pain followed by progressive symptoms such as left arm and leg weakness, dysarthria, paraplegia, and decreased level of consciousness. He subsequently developed respiratory distress requiring endotracheal intubation and mechanical ventilation. Magnetic resonance imaging showed a mass measuring 2 cm in the medulla oblongata. Although this mass was initially suspected as a glioma, the patient's acutely progressive disease course was not consistent with this diagnosis. Open biopsy revealed inflammation and demyelination, but no malignant cells were detected. He was treated with steroid pulse therapy, which showed dramatic effects. Nine months later, he developed another episode characterized by several neurological symptoms, and the diagnosis of MS was clinically confirmed. Open brain stem biopsy is technically demanding, but this case demonstrates that appropriate neurosurgical evaluation can play an important role in diagnosis by ruling out glioma and confirming MS.

Thalidomide attenuates excessive inflammation without interrupting lipopolysaccharide-driven inflammatory cytokine production in chronic granulomatous disease.

Chronic granulomatous disease (CGD) is a rare inherited disorder characterized by an inability to produce reactive oxygen species, resulting in recurrent life-threatening infections. Curiously, half of the patients with CGD suffer from aseptic bowel inflammation (CGD colitis) due to dysregulated inflammation induced by TNF-α and IL-1ß. Thus, developing therapies that regulate excessive inflammatory responses without interrupting antimicrobial immunity would benefit CGD colitis patients. Here, we show that thalidomide suppressed TNF-α-induced NF-κB activation and ATP-induced IL-1ß secretion, but did not interrupt the production of IL-1ß, IL-6, IL-8, and TNF-α in response to lipopolysaccharide in CGD monocytes. We report on a CGD colitis patient that showed decreased bowel inflammation characterized by reduced serum levels of inflammatory cytokines without evidence of progression of fungal and bacterial infections present at initiation of thalidomide therapy. Our results suggest that thalidomide could be an efficacious therapeutic option for patients with CGD colitis suffering from serious infections.

The impact of cardiac surgery in patients with trisomy 18 and trisomy 13 in Japan.

Congenital heart defects (CHD) are very common in patients with trisomy 18 (T18) and trisomy 13 (T13). The surgical indication of CHD remains controversial since the natural history of these trisomies is documented to be poor. To investigate the outcome of CHD in patients with T18 and T13, we collected and evaluated clinical data from 134 patients with T18 and 27 patients with T13 through nationwide network of Japanese Society of Pediatric Cardiology and Cardiac Surgery. In patients with T18, 23 (17%) of 134 were alive at this survey. One hundred twenty-six (94%) of 134 patients had CHDs. The most common CHD was ventricular septal defect (VSD, 59%). Sixty-five (52%) of 126 patients with CHD developed pulmonary hypertension (PH). Thirty-two (25%) of 126 patients with CHD underwent cardiac surgery and 18 patients (56%) have survived beyond postoperative period. While palliative surgery was performed in most patients, six cases (19%) underwent intracardiac repair for VSD. Operated patients survived longer than those who did not have surgery (P < 0.01). In patients with T13, 5 (19%) of 27 patients were alive during study period. Twenty-three (85%) of 27 patients had CHD and 13 (57%) of 27 patients had PH. Atrial septal defect was the most common form of CHD (22%). Cardiac surgery was done in 6 (26%) of 23 patients. In this study, approximately a quarter of patients underwent surgery for CHD in both trisomies. Cardiac surgery may improve survival in selected patients with T18.

A severe Salmonella enterica serotype Paratyphi B infection in a child related to a pet turtle, Trachemys scripta elegans.

Our report highlights a case of severe childhood salmonellosis related to a pet turtle, a red-eared slider (Trachemys scripta elegans). A 6-year-old girl had gastroenteritis complicated with sepsis caused by serotype Paratyphi B, which shared the same pulsed-field gel electrophoresis profiles with the organism isolated from a pet turtle. Based on our literature survey on childhood invasive salmonellosis acquired from reptiles, this case is the first documented reptile-associated salmonellosis including sepsis caused by this serotype.