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THE JOURNAL OF THE JAPAN PEDIATRIC SOCIETY
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Vol.118, No.9, September 2014
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Original Article
Title
Epidemiology of Status Epilepticus in Childhood
Author
Yoko Moriyama1) Kitami Hayashi2) Emiyu Matsuo1) Keiko Watanabe1) Yuri Shirato2) Takafumi Honda1) Ayako Muto1) Hiromichi Hamada1) and Masaru Terai1)
1)Department of Pediatrics, Tokyo Women's Medical University Yachiyo Medical Center
2)Division of Neurology and Developmental Pediatrics, Tokyo Women's Medical University Yachiyo Medical Center
Abstract
Objective: This study clarifies epidemiological characteristics of status epilepticus (SE) among Japanese children.
Methods: We analyzed retrospective data of children admitted with first episodes of SE from January 2007 through December 2011. All subjects were patients under 16 years old residing in Yachiyo City.
Results: A total of 59 children experienced SE for the first time in the 5-years period. (annual incidence: 41.0 per 100, 000 children annually). The SE incidence peaked at 12-23 months of age (19 patients, 32.2%), followed by 24-35 months of age (15 patients, 25%). There were 37 cases of prolonged febrile seizure (62.7%), 10 cases of acute symptomatic SE (16.9%), and 7 cases given a diagnosis of epilepsy (13.3%). Three cases were classified as acute on remote symptomatic. One case was considered to be remote symptomatic. Some kind of pathogen was detected in 28 patients. We detected the influenza virus including pdm influenza A H1N1 in 7 patients. Furthermore, 7 patients were diagnosed as having exanthema subitum. Three patients suffered from severe motor and mental disturbance as sequelae. No child died during the acute period of SE, but one patient who was later given a diagnosis of mitochondrial disorders had repeated SE and died of multiple cerebral infarction with SE.
Conclusions: The incidence of SE among Japanese children is higher than it is among children in Europe or the United States. One cause is the high frequency of febrile seizures. Furthermore, the incidence of SE is influenced by infectious epidemics.
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Original Article
Title
Diagnostic Accuracy of 18F-DOPA PET to Localize KATP-channel Hyperinsulinism and Post-treatment Prognosis
Author
Michiya Masue1) Hironori Nishibori2) Izaya Takada1) and Tohru Yorifuji3)
1)Department of Pediatrics, Kizawa Memorial Hospital
2)Department of Radiology, Kizawa Memorial Hospital
3)Department of Pediatric Endocrinology and Metabolism, Children's Medical Center, Osaka City General Hospital
Abstract
To evaluate the accuracy of 18F-fluoro-L-DOPA positron emission tomography (18F-DOPA PET) for diagnosing and localizing KATP-channel focal congenital hyperinsulinism in Japan, we performed 18F-DOPA PET scanning on 31 patients. Three of our patients had biparental mutations and they all showed diffuse uptake while the remaining 28 patients had paternal mutations. Seventeen out of 28 paternal mutations (61%) showed focal uptake. Twelve of these 17 patients (71%) showed uptake in the head of the pancreas. We diagnosed the pancreatic lesion correctly when 18F-DOPA PET showed a small focal uptake. However, the actual lesion borders were occasionally smaller or larger than shown on PET images.
Genetic testing had a 0% misdiagnosis rate but was useless for determining the lesion's location. It was useful to screen for PET scanning. The arterial stimulation venous sampling test (ASVS) occasionally provided more useful results than the 18F-DOPA PET test. However, ASVS is an invasive test. It is most useful in cases where 18F-DOPA PET and genetic test results are inconsistent.
Out of these 31 patients, two developed insulin-dependent diabetes mellitus following near total pancreatectomy. One patient (3%) showed epilepsy, but the other 30 patients showed no obvious neurological complications.
Japanese patients with congenital hyperinsulinism typically have relatively mild symptoms. Most of the patients who receive pharmacological therapy show spontaneous remission at 2 to 6 years of age. Our study suggests that continuous medical treatments such as octreotide therapy until spontaneous resolution can be a useful alternative to surgery for patients with diffuse or head accumulation of 18F-DOPA PET, because surgery is highly complex and there is risk of lasting complications.
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Original Article
Title
Ventilator-associated Pneumonia of Children in a Single Pediatric Intensive Care Unit
Author
Emily Knaup Mafumi Shinohara Takashi Muguruma Nobuyuki Nosaka Kazunori Aoki and Shuji Kuga
National Center of Child Health and Development Division of Critical Care and Anesthesia
Abstract
The medical records of patients with ventilator-associated pneumonia (VAP) treated in our intensive care unit from 2009 through 2011 were reviewed. VAP was diagnosed in 74 patients (6.4% of all cases, 7.7 cases/1, 000 ventilator days). The median duration from intubation to onset was nine (2-77) days. Both the numbers of ventilator and PICU days were greater in the VAP cases than in the non-VAP cases. In the late VAP cases, mortality was higher than expected. The initial antibiotics were selected based on the findings of Gram's staining of endotracheal aspirates. H. influenzae was detected most frequently in cultures, followed by S. aureus. In the early VAP cases, many types of bacteria causing community-acquired pneumonia were found. Meanwhile, the percentage of bacteria causing nosocomial infections tended to increase in the late VAP cases. Following initial antibiotic treatment, the white blood cell count and body temperature significantly decreased, while the PaO2/FIO2 significantly increased. The initial choice of antibiotics made based on Gram's staining of endotracheal aspirates usually resulted in a good clinical course, although in 5 of 74 cases, it was necessary to escalate the antibiotic regimen. Although the treatments selected based on analyses of endotracheal aspirates achieved a higher response rate, the numbers of PICU and ventilator days increased following the development of VAP. An effective plan to prevent VAP in children is necessary.
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Case Report
Title
A Case of Idiopathic Pulmonary Arterial Hypertension Well Controlled after Transition from Intravenous Epoprostenol to Oral Therapy
Author
Hideki Matsumoto Naoki Kuwabara Atsushi Terazawa Kuniko Tokoro Kentaro Omoya Hiroko Goto and Takashi Kuwahara
Department of Pediatric Cardiology, Gifu Prefectural General Medical Center
Abstract
A 6-year-old girl who started epoprostenol (EPO) continuous intravenous infusion therapy for idiopathic pulmonary arterial hypertension with World Health Organization pulmonary hypertension functional class III, succeeded in transition from intravenous EPO to oral therapy of sildenafil and bosentan at the age of 14 years. She was given EPO at a dose of 16 ng/kg/min, and test results revealed World Health Organization pulmonary hypertension functional class I, a 6-minute walk distance of 560 meters, BNP of 51.8 pg/ml, mean pulmonary arterial pressure of 30 mmHg and cardiac index of 5.31 L/min/m2, before discontinuance of EPO. The EPO dose was reduced and oral therapy of sildenafil was started. At the time EPO was discontinued, oral therapy of bosentan was added. Since then, the patient has been doing well with only oral medication more than 3 years after the discontinuance of EPO. We concluded that early administration of EPO improved pulmonary vascular remodeling and the patient responded well to the multidrug therapy administered after the transition from EPO. Since there are few reports on long-term outcomes after the transition from EPO, the further accumulation of cases is necessary.
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Case Report
Title
Diabetic Ketoacidosis Caused by a Delay in Insulin Pump Battery Replacement
Author
Junko Hanakawa Koji Muroya Takeshi Sato Yumi Asakura and Masanori Adachi
Department of Endocrinology and Metabolism, Kanagawa Children's Medical Center
Abstract
The patient was a 17-year-old Japanese girl with poorly-controlled type 1 diabetes mellitus who was hospitalized due to an episode of ketoacidosis. For two years prior to the episode, she had been on insulin pump therapy, using Medtronic MiniMed Paradigm 712. Her HbA1c (NGSP) level had been over 9%. Although she had noticed a message on the pump indicating "Low Battery", she ignored it. Seven days before the hospitalization, she finally exchanged the battery, because the battery had run out. After that, self-monitored blood glucose levels increased. She increased doses of bolus insulin injection at each meal without changing the basal injection rate. No other possible precipitating factors associated with ketoacidosis were noted. On admission, we checked the setting of the insulin pump, and found that the basal rate was set to be 0.0 U/h. After her recovery, we confirmed that she had never changed the basal rate setting. Thus, it is presumed that the basal rate setting was initialized to 0.0 U/h because the battery was dead for more than five minutes. Insulin pump users should check the setting of the pump whenever they exchange a battery.
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Case Report
Title
Brain Abscess Caused by Streptococcus in Two Patients with Right-to-left Interatrial Shunt
Author
Yuko Tasaki1)2) Noboru Igarashi1) Tatuya Kubo1) Shuhei Fujita1) Masaki Fukuda2) Yoko Imi2) Mondo Kuroda2) Masaki Shimizu2) and Akihiro Yachie2)
1)Department of Pediatrics, Toyama Prefectural Central Hospital
2)Department of Pediatrics, Kanazawa University
Abstract
We present two cases of brain abscess caused by Streptococcus, with right-to-left interatrial shunt. Case1: A 20-month-old boy was transferred to our hospital with fever and vomiting. The diagnosis of brain abscess and ventriculitis was made by magnetic resonance imaging. He was successfully treated with surgical drainage and intravenous antibiotic therapy. He had no neurologic sequela. The culture of pus from brain abscess revealed Streptococcus constellatus. Transesophageal echocardiography (TEE) with the Valsalva maneuver demonstrated right-to-left interatrial shunt through the patent foramen ovale (PFO). Case2: A 7-year-old girl was transferred to our hospital with coma and seizures. The diagnosis of brain abscess was made by contrast computed tomography. She was successfully treated with endoscopic drainage and intravenous antibiotic therapy. She had no neurologic sequelae. The culture of pus from brain abscess revealed α-Streptococcus. TEE with Valsalva maneuver demonstrated right-to-left interatrial shunt through atrial septal defect. Right-to-left interatrial shunt is an important risk factor of brain abscess. Evaluation for right-to-left interatrial shunt by echocardiography with Valsalva maneuver is recommended in patients with brain abscess.
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Brief Report
Title
Everolimus Improved Cognitive and Behavioral Symptoms in a Patient with Tuberous Sclerosis Complex
Author
Tomohei Nakao1) Ryuta Tanaka1) Eri Nishioka2) Keisuke Kato1) Ai Yoshimi1) Toshihiro Yanai3) Isho Izumi1) Nobuaki Iwasaki4) Kazutoshi Koike1) and Masahiro Tsuchida1)
1)Department of Pediatrics, Ibaraki Children's Hospital
2)Department of Clinical Psychology, Ibaraki Children's Hospital
3)Department of Pediatric Surgery, Ibaraki Children's Hospital
4)Department of Pediatrics, Ibaraki Prefectural University of Health Sciences
Abstract
Everolimus was recently approved for the treatment of renal angiomyolipoma (AML) and cerebral subependymal giant cell astrocytoma (SEGA) associated with tuberous sclerosis complex (TSC). Here we report the use of everolimus for an 11-year-old boy with TSC accompanied by both AML and SEGA who had severe motor and intellectual disabilities, epilepsy and autistic features, such as a short temper and self-injurious behavior. Three months after the initiation of everolimus therapy, the tumors regressed and his bad temper and self-injurious behavior ameliorated. Subsequently, he began smiling more often, showing a wider range of interests, and enjoying interpersonal relationships. No particularly harmful event has occurred since. Everolimus is expected to improve neuropsychiatric function because of its mechanism of action; therefore, further studies should evaluate cognitive-behavioral characteristics using a particular measurement scale in patients undergoing everolimus therapy for neuropsychiatric disorders.
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