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THE JOURNAL OF THE JAPAN PEDIATRIC SOCIETY

Vol.120, No.3, March 2016


Original Article
1. Prevalence of Hematologic and Biochemical Abnormalities in Children with Anorexia Nervosa Restricting Type on Admission
2. A Retrospective Cohort Study of Isotonic Versus Hypotonic Maintenance Intravenous Fluids for Hospitalized Children with Respiratory Tract Infection
3. Implementation and Effectiveness of Preventive Behaviors for Influenza in Kindergarteners and School Children
Case Report
1. A Case of Long QT Syndrome with Ventricular Fibrillation Treated with Defibrillation Using an Automatic External Defibrillator
2. A Pediatric Case of Facial Diplegia with Paresthesia: A Rare Subtype of Guillain-Barré Syndrome with Anti GD1b IgG Antibodies
3. PHACE Syndrome Treated with a Beta-blocker
4. Six Cases Experiences of Late-presenting Congenital Diaphragmatic Hernia


Original Article
Title
Prevalence of Hematologic and Biochemical Abnormalities in Children with Anorexia Nervosa Restricting Type on Admission
Author
Sadayuki Nagai1) Kousaku Matsubara1) Shizuo Takamiya2) Hidekazu Harigaya1) Hiroyuki Nigami1) Aya Iwata1) Eru Kozuki1) Yu Kawasaki1) Ken-ichi Isome1) Masayuki Hori1) Keiji Tasaka1) and Takashi Fukaya1)
1)Department of Pediatrics, Nishi-Kobe Medical Center
2)Department of Psychiatry, Nishi-Kobe Medical Center
Abstract
To determine the prevalence of hematologic and biochemical abnormalities in children with anorexia nervosa restricting type (ANR), we retrospectively analyzed the charts of 62 such children admitted between August 1994 and March 2015 to our center. Thirty-seven percent of the patients were leukopenic (WBC<4,000 /μl), 16% were anemic (Hb<12 g/dl), and 23% were thrombocytopenic (platelets<15×104/μl). As for biochemical parameters, 21% were hypoglycemic (<70 mg/dl) without any clinical symptoms. Hypertransaminasemia of AST>35 IU/L and ALT>35 IU/L developed in 26% and 34%, respectively, and hypercholesterolemia (>230 mg/dl) and increased BUN levels (>20 mg/dl) were noted in 53% and 47%, respectively. These 4 parameters significantly correlated with severity of ANR. The prevalences of abnormality in sodium levels, hypokalemia (<3.5 mEq/L), and hypophosphatemia (<lower limit of normal range) were 0%, 3%, and 13%, respectively. In a patient who developed liver failure on the third hospital day, multiple abnormalities, including AST 352 IU/L, ALT 328 IU/L, CK 700 IU/L, and BUN 59 mg/dl, had been observed on admission. The findings of this case suggest that the presence of serious biochemical abnormalities beyond multi-organs on admission may be an important indicator to identify at-risk patients who may subsequently develop refeeding syndrome. This is the largest study in Japan on the prevalence of hematological and biological abnormalities in ANR children. Our findings may help to better understand the distribution of laboratory parameter abnormalities of Japanese ANR children and the indications of hospitalization.




Original Article
Title
A Retrospective Cohort Study of Isotonic Versus Hypotonic Maintenance Intravenous Fluids for Hospitalized Children with Respiratory Tract Infection
Author
Akihiko Shimizu Seiji Kawamura Koji Uematsu Yuichi Ogawa Soichiro Toda Erika Obikane Shigeki Ichikawa and Takahiro Uehara
Department of Pediatrics, Kameda Medical Center
Abstract
Iatrogenic hyponatremia is a severe complication leading to neurologic sequelae or death. Many reports have noted that hypotonic maintenance intravenous fluid (IVF) is associated with hyponatremia. However, most of the studies were performed in tertiary care hospitals abroad and surgical patients account for a considerable portion of the subjects.
We conducted a retrospective cohort study of children from one month old to 18 years old hospitalized in Kameda Medical Center (Kamogawa, Chiba, Japan), the only community hospital in the area. Between April 1, 2007 and March 31, 2010, 331 pediatric patients with respiratory tract infection who needed maintenance IVF were included.
On admission, 241 patients had normal serum sodium concentration (136-147 mmol/L), while 133 patients received isotonic IVF and 7 (5.3%) of them developed hyponatremia (less than 136 mmol/L), and 108 patients received hypotonic IVF and 9 (8.3%) developed hyponatremia (p=0.36). Three (2.3%) cases with isotonic IVF developed hypernatremia (148 mmol/L or more), all of which had underlying disorders.
Among 331 subjects, 90 patients were hyponatremic on admission. 54 patients received isotonic IVF and 6 (11.1%) cases sustained hyponatremia after admission. 36 patients received hypotonic IVF and six (16.7%) cases sustained hyponatremia after admission (p=0.48). Isotonic IVF significantly increased the serum sodium concentration more than hypotonic IVF. Seven (13.0%) cases with isotonic IVF developed hypokalemia, although none developed hypokalemia among patients with hypotonic IVF (p=0.03).
In our current study, use of isotonic IVF did not have a significantly lower risk of hyponatremia and may be a risk factor to develop hypernatremia especially for patients with underlying diseases. Appropriate follow-up of serum electrolyte concentration and adjustment of maintenance IVF are indispensable for such patients to prevent disturbance of serum sodium concentration.




Original Article
Title
Implementation and Effectiveness of Preventive Behaviors for Influenza in Kindergarteners and School Children
Author
Nao Seki1) Atsushi Iwaya2) Minoru Okazaki3) Yugo Shobugawa4) Reiko Saito4) Naohito Tanabe5) and Hiroshi Suzuki6)
1)School of Health Sciences Faculty of Medicine, Niigata University
2)Department of Pediatrics, Ryotsu Hospital
3)Department of Pediatrics, Sado General Hospital
4)Division of International Health, Graduate School of Medical and Dental Sciences, Niigata University
5)Department of Health and Nutrition, Faculty of Human Life Studies, University of Niigata Prefecture
6)School of Nursing, Niigata Seiryo University
Abstract
In this study, we aimed to investigate the rates and effectiveness of influenza vaccination and implementation of non-pharmaceutical interventions such as hand washing in children, and to determine the actual situation in terms of going to a public location during influenza outbreak-related school or class closures. After the 2009/10 and 2011/12 influenza seasons, we conducted surveys for all kindergarteners and elementary school children in Sado Island; the survey recovery rates were 91% and 94%, respectively. Multivariate analysis indicated that "vaccination" (odds ratio [95% confidence interval]: 0.27 [0.23-0.31] (2009/10), 0.77 [0.67-0.89] (2011/12)) and "frequent hand washing" (0.67 [0.56-0.81], 0.71 [0.59-0.86]) lowered the risk significantly, although "wearing a mask" (1.41 [1.14-1.75], 1.35 [1.01-1.80]) and "gargling" (1.24 [1.03-1.50], 1.21 [1.00-1.45]) increased the risk. The implementation rates of "frequent hand washing" were 23% (2009/10) and 19% (2011/12). In both seasons, approximately 30% of children went to at least one public location, such as a shopping mall or cram school, during the influenza outbreak-related school or class closure. Thus, an education strategy for non-pharmaceutical interventions as well as the manner in which time can be spent when school is closed should be established to prevent an influenza epidemic.




Case Report
Title
A Case of Long QT Syndrome with Ventricular Fibrillation Treated with Defibrillation Using an Automatic External Defibrillator
Author
Tomoko Horinouchi1) Kiyoshi Hamahira1) Hiroyasu Uemura1) Yuichi Takami1) Tsuyoshi Egawa1) Hironobu Takahashi1) Tomoaki Ioroi1) Masaaki Kugo1) and Kenji Waki2)
1)Department of Pediatrics, Japanese Red Cross Society Himeji Hospital
2)Department of Pediatrics, Kurashiki Central Hospital
Abstract
In the present report, we describe the case of a 23-month-old female infant with long QT syndrome who presented with ventricular fibrillation and was treated with defibrillation using an automatic external defibrillator (AED). The chief symptoms in this patient were cardiopulmonary arrest and a sudden loss of consciousness. She was resuscitated by the ambulance crew, and the AED indicated a ventricular fibrillation rhythm. Subsequently, two electroshocks were delivered to the patient. The estimated brain ischemia time was 20 minutes, and therapeutic hypothermia was initiated using ventilation. The patient completely recovered without any neurologic impediments and was treated with a β-blocker; moreover, the patient's family was provided with an AED in case she required treatment at home.
This patient was rescued due to the spread of AEDs and the expansion of AED indications to a young population. In the future, AEDs may aid resuscitation of young patients with lethal arrhythmia causing sudden cardiac arrest. More clinical reports, such as our case, may disclose the cause of sudden death or sudden infant death syndrome.




Case Report
Title
A Pediatric Case of Facial Diplegia with Paresthesia: A Rare Subtype of Guillain-Barré Syndrome with Anti GD1b IgG Antibodies
Author
Yoshihiro Takeshita Akiko Ikeda Tomoo Uenishi Takahiro Kiyomasu and Akio Sunakawa
Department of Pediatrics, Yamato Takada Municipal Hospital
Abstract
We report a pediatric case of facial diplegia with paresthesia, a rare subtype of Guillain-Barré syndrome associated with anti GD1b IgG antibodies.
A 5-year-old boy presented with left facial paralysis after an episode of infection. The next day, facial diplegia appeared, and the boy was admitted to our hospital.
He presented with facial diplegia, lower-extremity muscle weakness, and decreased deep tendon reflexes. Based on the results of motor nerve conduction velocity and cerebrospinal fluid examinations we diagnosed Guillain-Barré syndrome and treated him with intravenous immunoglobulin therapy.
Facial nerve paralysis improved slowly and resolved after 59 days. Facial diplegia is a rare clinical condition; therefore, when encountering this condition, it should be considered a subtype of Guillain-Barré syndrome and the patient's anti-ganglioside antibody levels should be measured.




Case Report
Title
PHACE Syndrome Treated with a Beta-blocker
Author
Masatoshi Nakata Mitsutaka Shiota Kuniaki Tanaka Yoichi Iki Akira Kumakura Hiroshi Mizumoto Takakazu Yoshioka Atsuko Hata Ken Watanabe and Daisuke Hata
Department of Pediatrics, Tazuke Kofukai Medical Research Institute, Kitano Hospital
Abstract
We report a case of PHACE syndrome in a 3-month-old girl treated with propranolol, which resulted in significant regression. She was admitted to our hospital for rapidly enlarging facial hemangiomas with ulceration. MRI showed multiple hemangiomas in the posterior portion of the neck, along the esophagus, in the thoracic vertebral canal, dorsal to the stomach, and in the liver. Immunohistochemical analysis of the cervical hemangioma was positive for GLUT-1, confirming the diagnosis of infantile hemangioma. MRI of the head revealed hypoplasia of the bilateral internal carotid arteries and right vertebral artery, mild coarctation of the aorta, and aberrant origin of the right subclavian artery. The anomalies were consistent with PHACE syndrome. The patient had no respiratory symptoms, but the hemangioma along the esophagus distorted the airway. She was started on oral propranolol therapy at a dose of 0.5 mg/kg/day in 3 divided doses. On day 3, the hemangiomas appeared discolored. On day 4 when propranolol was increased to 1.5 mg/kg/day, echography showed decreased flow in the left carotid artery. Three hours later, she had an apneic event. MRI of the head showed no change from baseline. Propranolol was discontinued because the apneic event was considered a transient ischemic attack. Propranolol was restarted on day 12 at 0.5 mg/kg/day, and increased to 1 mg/kg/day. Subsequently, there was significant regression of the hemangiomas. The patient tolerated propranolol without adverse events until 10 months of age.
The potential risk of stroke in cases of PHACE syndrome cannot be ignored. We advise an initial low dose of propranolol and slow upward dosage titration to avoid adverse events.




Case Report
Title
Six Cases Experiences of Late-presenting Congenital Diaphragmatic Hernia
Author
Yusuke Hoshi1) Masato Kimura1) Eichiro Kawai1) Takehiko Ohnoki1) Moriei Shibuya2) Yohei Watanabe2) Satoru Kumaki2) Setsuko Kitaoka2) and Shigeo Kure1)
1)Department of Pediatrics, Tohoku University School of Medicine
2)Department of Pediatrics, Sendai Medical Center
Abstract
Congenital diaphragmatic hernia (CDH) is a cause of severe respiratory distress in the newborn period and is associated with a high mortality. Late-presenting CDH, which is detected after 30 days of age, has better prognosis compared with neonatal CDH. However, diagnostic delays may lead to severe complications such as perforation and/or necrosis of the gastrointestinal tract. We report our experience in the diagnosis and management of 6 cases of late-presenting CDH in an institution for 15 years. They were diagnosed between 3 months and 4 year-old. Five were boys and one case was a girl. Only one case presented with respiratory symptom and two were with gastrointestinal symptoms. One patient was asymptomatic and chest X-ray showed a CDH unexpectedly. There were 5 left-sided CDH and only one right-sided case. The postoperative course was uneventful in each case, and no recurrence has been reported subsequently.




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