gakkaizashi


THE JOURNAL OF THE JAPAN PEDIATRIC SOCIETY

Vol.120, No.6, June 2016


Original Article
1. Current Utilization and Factors Associated with the Dissatisfaction Over Social Services for Medically Complex Children with Special Health Care Needs
2. Investigation of the Clinical Characteristics Associated with Bladder and Bowel Dysfunction and the Therapeutic Effects of Defecation Control
3. Effects of Public Subsidy and Routine Immunization for Varicella Vaccine
Case Report
1. Emery-Dreifuss Muscular Dystrophy with Rapidly Worsened Heart Failure
2. SCN2A Mutation Causes Neonatal-onset, Lidocaine-dependent, Intractable Epilepsy
3. Two Cases of Human Metapneumovirus Infection with Suspected Virus-associated Hypercytokinemia
4. Successful Management of Severe Malnutrition According to World Health Organization Guidelines
5. Smooth Transportation of a Pediatric Myocarditis Patient through Multiprofessional Coordination Resulting in Favorable Prognosis


Original Article
Title
Current Utilization and Factors Associated with the Dissatisfaction Over Social Services for Medically Complex Children with Special Health Care Needs
Author
Nobuyuki Yotani1)2) Akira Ishiguro1) Tomoo Nakamura1) and Hirokazu Sakai1)
1)Department of General Pediatrics and Interdisciplinary Medicine, National Center for Child Health and Development
2)Department of Palliative Medicine, Kobe University Graduate School of Medicine
Abstract
The number of medically complex children with special health care needs (CSHCN) requiring home-based life-support and life-long medical care is increasing. However, current social services are underdeveloped and provide insufficient support. The purpose of this study was to elucidate the current status of utilization of social services for CSHCN and the factors associated with the dissatisfaction over such services. The CSHCN and their families were recruited from two children's hospitals, one university hospital and six rehabilitation centers. A medical information handbook was distributed to the families at enrollment, followed by a self-reporting questionnaire one year later. The analysis included 76 medically complex CSHCN and their families. The mean age of the CSHCN was 16.5± 12.2 years. The rates of current utilization/dissatisfaction over the social services were as follows: physician home visit, 23/54%; home-visit nursing, 63/36%; home-visit rehabilitation, 34/32%; assisted bathing, 44/32%; transportation, 25/41%; day service, 15/23%; day service at home, 16/38%; ambulatory rehabilitation, 64/21%; short-stay, 66/11%; housekeeping, 5/64%; and -/74% in institutionalization. Dissatisfaction over physician home visits was significantly negatively correlated with having younger siblings (odds ratio [OR]=0.29, p=0.05). Dissatisfaction over day service at home and housekeeping were significantly negatively correlated with home mechanical ventilation with tracheostomy (OR=0.19, p=0.03; OR=0.30, p=0.05). There was a significant negative correlation between dissatisfaction over institutionalization and having younger siblings (OR=0.17, p=0.03). Family circumstances were an important factor associated with a dissatisfaction over social services. We propose that family circumstances should be considered when developing social resources.




Original Article
Title
Investigation of the Clinical Characteristics Associated with Bladder and Bowel Dysfunction and the Therapeutic Effects of Defecation Control
Author
Hirokazu Ikeda Tsuneki Watanabe Masaki Fuyama and Keiichi Isoyama
Department of Pediatrics, Showa University Fujigaoka Hospital
Abstract
[Objectives] Bladder and bowel dysfunction (BBD) is a term used to describe the pathological condition in which lower urinary tract dysfunction (LUTD) is complicated by bowel dysfunction.
[Method] The subjects of the study were 5-14-year-old pediatric patients who presented with daytime incontinence (DI) between April 2012 and March 2014 at our outpatient department. The patients were first diagnosed with LUTD using excretion records and the Rome III criteria, and were subsequently divided into the following two groups: a BBD group (with concomitant bowel dysfunction) and non-BBD group (without concomitant bowel dysfunction).
[Results] There were a total of 206 subjects: 95 subjects (46.1%) were in the BBD group and 111 (53.9%) in the non-BBD group. When the two groups were compared, the mean age of patients in the BBD group was significantly lower than that in non-BBD group. A history of urinary tract infections (UTIs) or recurrent UTIs was significantly more common in the BBD group than in the non-BBD group. After 12 weeks of treatment for constipation, a review of the available excretion records showed that DI was no longer present in 23 of 63 subjects (36.5%), and the frequency of DI decreased by ≥50% in 17 of 63 subjects (26.9%).
[Conclusion] Constipation was present in 43.3% of pediatric LUTD outpatients, and these patients belonged to the BBD group. The frequency of DI is higher in patients with BBD, and this can lead to recurrent UTIs. Therefore, it is important to diagnose concomitant constipation and prioritize its treatment while treating patients for LUTD.




Original Article
Title
Effects of Public Subsidy and Routine Immunization for Varicella Vaccine
Author
Hiroko Yamamoto1) Masako Miyake1) Yoshiko Ueda1) Hisashi Takasugi1) and Takashi Nakano2)
1)Kibi Medical Association
2)Department of Pediatrics, Kawasaki Medical School
Abstract
In Soja City, public subsidies for children younger than 5 years have been provided since April 2013, prior to the routine immunization of the varicella vaccine in October 2014. Before and after the provision, we investigated the number of patients with varicella infection, their ages, their vaccination histories, the severity of the disease, and the number of students in nursery schools, kindergarten, elementary, and junior high schools who were absent because of varicella infection. In addition, we studied the effects of the popularization of varicella vaccination.
As of the end of March 2015, 80.9% of the children aged 1-5 years and had one or more vaccination records. The number of patients with varicella infection decreased from 342 in 2012 to 171 in 2014. Among these patients, the number of those younger than 5 years significantly decreased from 217 in 2012 to 135 in 2013 and to 69 in 2014. The number of students absent from nursery schools also significantly decreased. On the other hand, the numbers of patients and suspended students among the older children did not decrease. Attention is necessary in the future regarding whether the number of patients among older children whose vaccination rate is low will further increase.
Although we clearly confirmed that in the children who had at least a one vaccination record, the severity of varicella infection improved to a mild condition, the preventive effect of one-time vaccination on the infection was not enough, as these children accounted for 157 (37.8%) of the 415 patients. Thus, thorough implementation of two-time vaccination is deemed necessary to control the spread of varicella infection. Among the patients with varicella infection who had a one-time vaccination record, 0.9%, 7.0%, and 16.7% developed varicella infection within 3 months, between 3 and 6 months, and between 6 and 12 months after the vaccination, respectively. Therefore, we consider that a more significant decrease in the number of patients with varicella infection can be expected by giving a second inoculation immediately after 3 months have elapsed after the first vaccination.




Case Report
Title
Emery-Dreifuss Muscular Dystrophy with Rapidly Worsened Heart Failure
Author
Yuichiro Hashida1) Shinzi Sakata1) Hiroki Kuranobu1) Yoichi Mino1) Hiroaki Funata1) Yasuhiro Tsuzi1) Kentaro Shirai2) and Susumu Kanzaki1)
1)Division of Pediatrics and Perinatology, Faculty of Medicine, Tottori University
2)Division of Neurology, Institute of Neurology Science, Faculty of Medicine, Tottori University
Abstract
Emery-Dreifuss muscular dystrophy (EDMD) is a hereditary neuromuscular disorder characterized by progressive muscle weakness, joint contractures and cardiomyopathy with cardiac conduction defect. The prognosis is strongly associated with cardiac complications such as severe myocardial dysfunction and arrhythmias. We present the case of a 16-year-old girl with EDMD. She was found to have muscular dystrophy by muscle biopsy at the age of 4 years. An echocardiogram revealed an enlarged right atrium and severe tricuspid regurgitation at the age of 8 years. At 12 years old, she was found to have left ventricular dysfunction. An electrocardiogram revealed second-degree atrioventricular block and atrial fibrillation after the age of 11 years, and ultimately atrial standstill. She was diagnosed with EDMD based on her clinical course and symptoms. Her cardiac function progressively worsened despite medical therapy including a beta-blocker, and she had difficulty in weaning from an intravenous inotropic support. Therefore, we thought her to be a candidate for heart transplantation. She had an episode of cardiopulmonary arrest due to ventricular fibrillation, but it was successfully treated. She underwent implantation of a ventricular assist device (VAD) as a bridge to transplantation because of resistance to conservative management. However, she developed complications of cerebral hemorrhage and infarction, and died 44 days after cardiac surgery. Cardiac transplantation is the optimal therapy for EDMD with severe cardiomyopathy. In addition, effective treatment including VAD should be performed, when cardiac function shows resistance to conservative management due to progressive deterioration of it during waiting for cardiac transplantation.




Case Report
Title
SCN2A Mutation Causes Neonatal-onset, Lidocaine-dependent, Intractable Epilepsy
Author
Keisuke Shoji1) Naka Saito1) Sho Horiguchi1) Kei Arai1) Seiko Sato1) Koichi Sato1) Hiroshi Yoshida1) Sueshi Ito1) Shinichi Magara2) Masaki Wada3) Kazuyuki Nakamura4) and Mitsuhiro Kato4)
1)Department of Pediatrics, Tsuruoka Municipal Shonai Hospital
2)Department of Child Neurology, Nishi-Niigata Chuo National Hospital
3)General Center for Perinatal, Maternal and Neonatal Medicine, Niigata University Medical & Dental Hospital
4)Department of Pediatrics, Yamagata University Faculty of Medicine
Abstract
SCN2A encodes Nav1.2, a voltage-gated sodium channel α subunit expressed in the human brain. Mutations in SCN2A cause various pathologic conditions, including epileptic syndromes and developmental disorders. Here, we report a case of lidocaine-dependent intractable epilepsy associated with SCN2A mutation. The patient was a boy aged 22 months who developed clustered seizures and had an abnormal amplitude-integrated electroencephalogram on day 2. The attacks were almost clustered partial seizures, but for a period of time migrating partial seizures. The clustered seizures persisted despite treatment with various antiepileptic drugs. Continuous intravenous infusion of lidocaine resulted in resolution of seizures. The seizures remained resolved after use of lidocaine tape therapy, and the patient was discharged. The patient remained on lidocaine tape therapy and was seizure-free but had developmental delay. Whole-exome sequencing revealed a heterozygous de novo missense mutation, c.788C> T, which causes an amino acid change, p.Ala263Val, in SCN2A. This mutation has been previously reported and is highly likely pathogenic in our patient as well. Lidocaine sodium-channel blocker tape therapy is a possible treatment for intractable epilepsy caused by sodium-channel dysfunction due to the SCN2A mutation.




Case Report
Title
Two Cases of Human Metapneumovirus Infection with Suspected Virus-associated Hypercytokinemia
Author
Hiroyuki Shimizu1) Hidenori Sugawara1) Yu Kamigaki1) Masaki Shimizu2) and Masaaki Mori1)3)
1)Children's Medical Center, Yokohama City University Medical Center
2)Department of Pediatrics, School of Medicine, Institute of Medical, Pharmaceutical, and Health Sciences, Kanazawa University
3)Department of Pharmacovigilance, Graduate School, Tokyo Medical and Dental University
Abstract
We report two cases of human metapneumovirus infection with suspected virus-associated hypercytokinemia. Many viruses can cause virus-associated hemophagocytic syndrome, but to the best of our knowledge, there are no case reports of hypercytokinemia and lymphohistiocytosis associated with human metapneumovirus infection. Here we describe two such patients. Case 1 was a 4-year-old boy with spondyloepiphyseal dysplasia congenita. He had prolonged high fever and was given a diagnosis of human metapneumovirus pneumonia. Laboratory blood tests showed increased AST at 121 IU/l, LDH at 1,065 IU/l and ferritin at 737 ng/ml. Case 2 was a 12-year-old girl with West syndrome and cerebral palsy. She had fever and wheezing, and we diagnosed with human metapneumovirus pneumonia and bronchial asthma. She had increased AST at 53 IU/l, LDH at 946 IU/l and ferritin at 518 ng/ml. In both patients, IL-6, neopterin and sTNFR (soluble tumor necrosis factor receptor II) were elevated in the acute phase and promptly decreased in the convalescent phase. These cytokine profiles were similar to those of other known virus-associated lymphohistiocytosis. In conclusion, clinicians need to be aware of the possibility of hypercytokinemia induced by human metapneumovirus infection.




Case Report
Title
Successful Management of Severe Malnutrition According to World Health Organization Guidelines
Author
Momoko Yokoyama1) Jun Mine1) Kazuko Kishi1)2) Akiyoshi Horie1)3) and Seiji Yamaguchi1)
1)Department of Pediatrics, Shimane University School of Medicine
2)Department of Child Psychiatry, Shimane University School of Medicine
3)Department of Pediatrics, Shimane Prefectural Central Hospital
Abstract
In developing countries, severe acute malnutrition is a significant factor in many cases of infant and child death. Studies have suggested that mortality could be decreased by following the World Health Organization (WHO) guidelines for the inpatient treatment of severely malnourished children. In Japan, the existence of these guidelines is not well known because severe acute malnutrition in infants and children is rare. However, with the recent increase in the number of abused children in Japan, the prevalence of severe acute malnutrition in infants and children is likely to increase. Here, we report the case of an 8-month-old severely malnourished girl who was successfully managed according to the WHO guidelines.
The patient had shown rapid weight loss from 4 months to 8 months of age due to parental neglect. At presentation, her weight for length was below -3SD of the mean, fulfilling the criteria for severe malnutrition according to the WHO guidelines. The patient was taken under the care of a child consultation center and was admitted to our hospital. She was successfully managed according to the WHO guidelines for malnourished children. Her weight increased steadily, without serious complications, and she was discharged on hospital day 62.
Severe malnutrition can be managed in several ways, but inappropriate treatment could increase mortality. Based on our experience with this case, we suggest that the WHO guidelines should be properly employed even in Japan, although some points might not be adapted to actual Japanese conditions.




Case Report
Title
Smooth Transportation of a Pediatric Myocarditis Patient through Multiprofessional Coordination Resulting in Favorable Prognosis
Author
Satoshi Matsui1) Satoshi Tsuji1) Tomoya Ito1) and Takashi Muguruma2)
1)Division of Emergency Medicine and Transport Service, National Medical Center for Children and Mothers
2)Pediatric Intensive Care Unit, National Medical Center for Children and Mothers
Abstract
Acute myocarditis in children calls for rapid transportation to facilities that can deploy extracorporeal auxiliary circulation, but also necessitates that the transportation be carried out by a specialized team capable of handling patients with unstable circulation. In this report, we describe our experience regarding multiprofessional coordination leading to the smooth transportation of a patient by a specialized team. The patient, a 3-year-old boy, experienced emesis and diarrhea on day 1 of the illness. On the night of day 3 of the illness, upward rolling of the eyeballs for a few seconds was observed multiple times. On day 4 of the illness, abnormal complexion and irregular pulse were detected, and the patient was referred to his previous doctor. The previous doctor diagnosed circulatory failure with third-degree atrioventricular block. Transcutaneous pacing under oral tracheal intubation was immediately initiated, and the doctor requested transportation to our hospital. The transport team was dispatched by land to the previous hospital immediately after the request. However, our hospital was located about 40 km away, and the previous doctor therefore requested the fire department for aerial transportation by helicopter. The transport team, the patient, and the helicopter gathered at a set rendezvous point, making the aerial transportation to our hospital possible. After admission to the intensive care unit, the transcutaneous pacing was rendered ineffective, which resulted in cardiac arrest. Cardiopulmonary resuscitation was initiated immediately, and the patient's heartbeat resumed in about 2 minutes. Although extracorporeal membrane oxygenation was necessary, on the fifth day after admission, it was no longer required. Eventually, 18 days after admission, the patient was discharged in an ambulatory state without any sequelae. Although this patient was transported smoothly by effective multiprofessional coordination, hereafter, it is necessary for each hospital to check transport options previously depending on weather and the referring hospital, and establish a coordination protocol to consistently ensure smooth transportation.




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