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THE JOURNAL OF THE JAPAN PEDIATRIC SOCIETY
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Vol.117, No.10, October 2013
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Review
Title
Typical Dengue Fever in a Japanese Child
Author
Yuta Sasaoka1) Yoshiyuki Sakai1) Mayumi Ohno1) Minami Yoda1) and Hiroyuki Tsutsumi2)
1)Department of Pediatrics, Hakodate Municipal Hospital
2)Department of Pediatrics, Sapporo Medical University
Abstract
Dengue virus is an arthropod-borne virus carried by mosquitoes, which causes dengue fever or the more serious dengue hemorrhagic fever. It is prevalent in tropical zones of the world, but recently the number of patients diagnosed as dengue virus infection in Japan has increased.
A 12-year-old girl visited Thailand. After she returned to Japan, she developed a fever and was referred to our hospital from a clinic. Her white blood cell and platelet counts were decreased so she was admitted. The next day (day 7 of her illness), she developed a rash. Thereafter she was discharged from hospital without any further serious developments. We diagnosed her illness as dengue fever based on identifying dengue viral RNA in her blood on day 4, using the RT-LAMP method at the Institute of Tropical Medicine Nagasaki University.
Half a century ago Japan experienced an epidemic of dengue fever. After revision of the Infectious Diseases Control law in 1999, more and more cases have been reported, but only 19 pediatric cases have been included. They were all treated symptomatically, and nobody died. The diagnosis of dengue infection is mainly based on the detection of IgM antibody specific for dengue virus or detection of viral RNA with RT-PCR.
We gathered the reports of dengue infection around Japan, and believe that pediatricians should learn about its diagnosis and treatment.
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Original Article
Title
The Eradication Rate and Recurrence Rate between 10-day Penicillin and 5-day Cephalosporin Therapies for Treating Pharyngitis Caused by Group A Streptococcus
Author
Hiroyuki Shimizu1)2) Miwako Saito1) Megumi Atsumi1) Chidori Kubota1) and Masaaki Mori3)
1)Chidori Children's Clinic
2)Children's Medical Center, Fujisawa City Hospital
3)Department of Pediatrics, Yokohama City University Medical Center
Abstract
Recently, an increasing number of cephalosporins have been administered for treating bacterial pharyngitis caused by Group A streptococcus, in spite of penicillin being the mainstay of antibiotics over a long period. We compared the rate of bacterial eradication from the pharynx and the rate of recurrence of a 10-day treatment with amoxicillin (AMPC) and a 5-day short course treatment with oral cephalosporins. We studied 441 pediatric outpatients at Chidori Children's Clinic from October 2011 to November 2012. All patients were encouraged to return to the doctor after 2 weeks of completing the antibiotic therapy, and bacterial eradication was confirmed with a throat culture. We defined recurrence as the presence of any symptoms of pharyngitis reappearing within 2 weeks of antibiotic therapy completion and diagnosed using a rapid antigen detection test. The eradication rate of AMPC was 91.7%, followed by cefcapene pivoxil (CFPN-PI) at 82.5% and cefditoren pivoxil (CDTR-PI) at 80.4%. The total eradication rate of cephalosporins was 82.0% (p=0.01). The recurrence rate of AMPC was 3.1%, followed by cefcapene pivoxil (CFPN-PI) at 3.4% and cefditoren pivoxil (CDTR-PI) at 3.8%. The total recurrence rate of cephalosporins was 3.4% (p=0.93). In conclusion, we showed the advantage of using penicillin from the aspects of clinical efficacy, proper use of antibiotics and medical economics.
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Original Article
Title
Clinical and Laboratory Findings of Young Children with Occult Bacteremia in an Outpatient Clinic
Author
Masahiro Hiraoka
Aiiku Pediatric Clinic
Abstract
The incidence and clinical findings of occult bacteremia before the start of heptavalent streptococcal vaccine and Hib vaccine were retrospectively examined at our pediatric outpatient clinic. Subjects included young children aged 3 to 71 months who underwent blood cultures for fever of unknown origin. Of 340 blood cultures taken from 276 children, 25 cultures (7.4%) were positive; with Streptococcus pneumoniae in 24 and Hemophilus influenzae in one. Of these 25 children, 24 underwent bacterial culture of the nasopharynx, and all revealed the same bacterial species as the blood cultures. Compared with the non-bacteremia group, the bacteremia group was significantly younger, was examined earlier after the onset of fever, presented more often with rhinorrhea (lasting for one week or longer), had more leukocytes and granulocytes, and lower CRP values. Leukocytes and granulocytes decreased significantly on the next day in both groups, with higher values in the bacteremia group. CRP values increased significantly on the next day, with no significant difference between the two groups. Intravenous antibiotic administration at an outpatient clinic reduced the fever by the next morning in all but one child in the bacteremia group and in 241 (77.7%) of the 310 children of the non-bacteremia group. Young children with rhinorrhea appear to easily develop bacterial infection of the nasopharynx with the abrupt onset of fever and increased neutrophils, and some of these children could develop bacteremia.
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Original Article
Title
Simple Evaluation of Academic Skills in Japanese Language, Applicable to Pediatric Outpatients
Author
Hideo Nagao
Department of Special Support Medicine, Faculty of Education, Ehime University
Abstract
The purpose of this research is to clarify the validity and the meaning of the Japanese language academic skill level test that the author developed in 2009.
The subjects totaled 696 pupils, 343 boys and 353 girls, and consisted of 1th-6th grade pupils. They took two tests for Japanese language skills of each school grade; the author's test and a standardized achievement test called CRT-II.
The average rate of correct answers of the author's test ranged from 79.5% for the fifth grade to 95.4% for the second grade, and that for the standardized achievement test ranged from 84.9% for the third grade to 90.1% for the first grade. There was a statistically significant correlation between the two tests as to all four fields of hearing, reading, writing, and the skills in Chinese characters, of the Japanese language.
Therefore, it is thought that this study demonstrates that the author's test measures Japanese language academic skills as well as the standardized achievement test. Moreover, the author's test may be useful for identifying children with learning difficulties, because it can be executed in a short time, consists of short sentence questions, and can be completed freely. The author's test would be helpful for clinicians by showing the academic levels in each language skill, of each child, at any school grade, simply within 1 result sheet.
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Original Article
Title
Effectiveness of Prostaglandin E1 in the Treatment of Pediatric Hepatic Veno-occlusive Disease
Author
Moeko Hino1) Hidemasa Ochiai1) Kumiko Ando1) Harumi Kakuta2) Yuri Okimoto2) and Yoichi Kohno1)
1)Department of Pediatrics, Graduate School of Medicine, Chiba University
2)Division of Hematology Oncology, Chiba Children's Hospital
Abstract
Hepatic veno-occlusive disease (VOD) is characterized by jaundice, hepatomegaly, pain in the upper right quadrant and ascites. There are few reports about pediatric VOD in Japan, and little is known about the therapeutic effects of prostaglandin E1 (PGE1) for VOD. We investigated risk factors and characteristics of pediatric patients with VOD in Japan and the therapeutic effects of PGE1 in this disease. We analyzed 66 children with malignancies between June 2007 and December 2010. Six (10.5%) of 57 patients had VOD after stem cell transplantation and 3 of 9 with malignant tumors receiving chemotherapy also had VOD. As a result of multivariate analysis, infants tended to be at high risk of VOD. Six patients were refractory to platelet transfusion before the diagnosis of VOD. All patients were infused with PGE1 within a median of one day after diagnosis. Eight patients recovered and one required recombinant thrombomodulin-α. The number of platelets increased faster than the symptoms diminished. Adverse reactions of PGE1 were not significant. Platelet count trends were useful for the early diagnosis of pediatric VOD and to predict improvement. Infants should be carefully monitored. Early PGE1 administration was safe and effective for treating children with VOD. Further assessment of PGE1 therapy is warranted.
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Original Article
Title
Long-term Outcome after Initial Steroid Treatment of Body Surface Area-versus Body Weight-based Dosing in Idiopathic Nephrotic Syndrome
Author
Daishi Hirano1)2) Shuichiro Fujinaga1) Mayu Nakagawa1) Tsuneki Watanabe1) Akira Ito1)2) and Hiroyuki Ida2)
1)Division of Nephrology, Saitama Children's Medical Center
2)Department of Pediatrics, Jikei University School of Medicine
Abstract
Two corticosteroid regimens, body surface area (BSA)-based dosing and body weight (BW)-based dosing, for the initial treatment of idiopathic nephrotic syndrome have been equivalently used. However, little is known regarding the long-term outcome after the different regimens. To evaluate whether the initial dose difference affects clinical outcome, we retrospectively analyzed a cohort of 46 children (median age, 4.0 years) weighing < 30 kg who were treated with BSA- or BW-based dosing regimens. Although there were no significant differences in median age at onset, sex, or median number of days to achieve remission after initial steroid therapy between the two groups, the median time to develop first relapse was significantly shorter in the BW-based group than in the BSA-based group. Furthermore, the risk of steroid dependency was significantly higher in the BW-based group than in the BSA-based group. The initial dose difference appears to influence the clinical outcome in young children with idiopathic nephrotic syndrome.
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Original Article
Title
Effect of Chronic Functional Constipation on Acquisition of Toileting Skills in Childhood
Author
Yasuko Wakabayashi1) Kazuko Okada1)2) and Shigetaka Sugihara1)
1)Department of Pediatrics, Tokyo Women's Medical University, Medical Center East
2)Okada Pediatric Clinic
Abstract
It has been pointed out that chronic functional constipation (CFC) may cause toilet refusal in childhood toilet training (TT), therefore, we conducted this study to clarify the association between CFC and acquisition of toileting skills. The subjects included children ranging in age from 2.5 years to less than 7 years with constipation (constipation group; n=53) and without constipation (control group; n=82). The parents of these children were interviewed to determine the details of the TT. The survey results revealed toilet refusal during TT in 66% of the constipation group and 24% of the control group, being significantly higher in the constipation group (P< 0.01). In addition, the median age at completion of stool TT was 3 years 6 months in the constipation group and 2 years 11 months in the control group. On the other hand, the median age at completion of daytime bladder TT was 3 years in the constipation group and 2 years 11 months in the control group. Thus, completion of stool TT was significantly delayed in the constipation group as compared with that in the control group (P< 0.01). In the constipation group, particularly in the 30 children who first visited to our hospital for the constipation during the TT phase between 2 years and less than 4 years of age, the median age at completion of stool TT was 4 years. Thus, this group lagged behind overall, and all of the 8 children in whom stool TT could only be completed very late, that is, over 5 years of age, were in this group. Also, from the finding that completion of stool TT was delayed in all of the 17 children with a history of anal bleeding during evacuation in this group, it was concluded that constipation posed an interference with the completion of stool TT. However, in 86% of children under therapeutic management of constipation during this phase, stool TT could be completed without delay, suggesting the importance of treatment for constipation during the TT phase.
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Original Article
Title
Clinical Presentations and Outcomes of Severely Handicapped Children Transferred from Acute-care Hospital Pediatric Wards Including Neonatal Intensive Care Units
Author
Michiko Inoue
Department of Pediatrics, National Hospital Organization Minami-Okayama Medical Center
Abstract
The clinical presentations and outcomes were investigated in cases involving 30 children younger than 13 years old, who were transferred from acute-care hospital pediatric wards including neonatal intensive care units (hereinafter, abbreviated as NICU) to a ward for children and persons with severe motor and intellectual disabilities in national hospital organization hospitals in the Chugoku and Shikoku regions from April 2005 to March 2010. 60% of the transferred children were younger than 3 years old, with 76.7% suffering from severe motor and intellectual disabilities in the medical care dependent group and 66.7% on a ventilator. After the transfer, the general condition of 4 among 30 patients became unstable, with 5 patients becoming temporarily unstable. The age of the patients that became unstable was significantly younger than that of patients that were stable, even after the transfer. Moreover, a tendency was observed in the patients that became unstable in the cases involving a direct transfer from the neonatal unit. Respiratory tract infection and respiration failure were the most common difficult complications following the transfer. Among 30 transferred children, 8 (26.7%) died, with the most common reason of death being pneumonia and respiration failure (including progression to multiple organ failure).
In order to prevent the worsening of conditions following transfer, it is believed that transferring children after they have reached 4 years of age or older and using a transitional facility (ward) between a neonatal unit and a ward for children and persons with severe motor and intellectual disabilities are helpful. Further, it has been indicated that expanding the maintenance of the respiratory care system is required in wards for children and persons with severe motor and intellectual disabilities.
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Original Article
Title
Two Cases of Congenital Central Hypoventilation Syndrome with a PHOX2B Gene Mutation Complicated by Hyperinsulinemic Hypoglycemia
Author
Ryojun Takeda1)5) Masahiro Goto1)2) Junko Igaki1) Masaki Takagi1) Nao Tachibana2) Hiroshi Yoshihashi2)3) Chikahiko Numakura6) Kiyoshi Hayasaka6) and Yukihiro Hasegawa1)2)4)
1)Division of Endocrinology and Metabolism, Tokyo Metropolitan Children's Medical Center
2)Department of Pediatrics, Tokyo Metropolitan Children's Medical Center
3)Division of Medical Genetics, Tokyo Metropolitan Children's Medical Center
4)Division of Genetic Research, Tokyo Metropolitan Children's Medical Center
5)Department of Pediatrics, Fussa Hospital
6)Department of Pediatrics, Yamagata University School of Medicine
Abstract
We report 2 pediatric cases of congenital central hypoventilation syndrome (CCHS) with a heterozygous PHOX2B gene mutation complicated by hyperinsulinemic hypoglycemia (HH). Both of them were diagnosed as CCHS and Hirschsprung's disease in the neonatal period because of their episodes of severe apnea and results of rectal biopsy. The complication of HH was diagnosed based on their hypoglycemic episodes (blood glucose levels were 49 mg/dL and 39 mg/dL) and simultaneous high serum insulin levels (3.3 μg/mL and 19 μg/mL) in infancy. A heterozygous 27-polyalanine repeat expansion mutation in the PHOX2B gene was confirmed in both cases.
CCHS is a rare disorder characterized by impaired automatic control of breathing and autonomic nervous system dysregulations. Heterozygous mutations of the PHOX2B gene are responsible for the disease. The gene is expressed in neural crest-derived cells. To the best of our knowledge, 7 cases, including the present one, of CCHS with heterozygous mutations of the gene have been reported to be complicated by HH.
PHOX2B gene products are supposed to regulate the secretion of insulin from endoderm-derived pancreatic beta cells by two mechanisms. First, this gene suppresses the function of the transcriptional factor NKX2.2, which accelerates the differentiation and proliferation of beta cells. Second, it activates dopamine beta-hydroxylase gene and accelerates the synthesis of norepinephrine in ectoderm-derived sympathetic nerve cells, thereby inhibiting insulin secretion by the pancreatic beta cells. Inactivating mutations of the PHOX2B gene is supposed to result in the impairment of these two inhibitory mechanisms of insulin secretion, which leads to HH.
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Original Article
Title
An Infant with Knee Bacterial Arthritis Diagnosed as Kingella kingae by PCR
Author
Masahiro Nozawa1) Makiko Yoneta1) Junji Shimizu1) Noriko Nishikura1) Eisuke Ito1) Tohru Sugimoto1) Tsutomu Narita2) and Yoshihiro Takeuchi3)
1)Department of Pediatrics, Saiseikai Shiga Hospital
2)Department of Pediatrics, Takatsuki Red Cross Hospital
3)Department of Pediatrics, Shiga University of Medical Science
Abstract
A definitive diagnosis of bacterial arthritis may be difficult because of insufficient isolation of the bacterium by Gram stain or standard culture techniques. In childhood arthritis or osteomyelitis, Kingella kingae is an important causative bacterium that can be difficult to identify by conventional methods. Here, we report the case of a 1-month-old boy who developed knee bacterial arthritis. His left foot seemed paralyzed, and he cried on passive movement. Therefore, bacterial arthritis was suspected, and treatments consisted of empiric antimicrobial therapy and repeated draining and cleaning of synovial fluid were provided. In this case, the causative bacterium could not be identified by Gram stain or culture by the end of his treatment. Therefore, empiric antimicrobial therapy was continued for 4 weeks. Using a synovial fluid specimen stored before treatment, the bacterium was identified as Kingella kingae by PCR analysis. In cases of bacterial arthritis, it is difficult to determine the appropriate treatment and treatment termination based on the results of Gram stain or culture. Examination by PCR may allow the earlier detection and identification of the bacterium, which would aid in the choice of appropriate treatment.
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Original Article
Title
A 2-year-old Female Infant with Transplacental HIV Transmission Despite Mother-to-child Transmission Prophylaxis
Author
Kaori Okuma Moe Akahira-Azuma Yoshiaki Okuma Mizue Tanaka Masao Kaneshige Noriko Sato Shinichi Hosokawa and Takeji Matsushita
Department of Pediatrics, National Center for Global Health and Medicine
Abstract
We report a case of transplacental HIV transmission, despite prevention of mother-to-child transmission, in a 2-year-old female infant. We examined her treatment course, growth and neurological development. Anti-retrovirus therapy (ART) was delayed at 34 weeks of gestation because the mother did not receive prenatal care early in pregnancy. After initiating ART, the mother's serum HIV RNA level immediately decreased from 1.4× 104 copies/ml to 95 copies/ml. At 37+5 weeks of gestation, an elective cesarean section was performed and a female infant was born without complications. According to the standard 6-week zidovudine (AZT) chemoprophylaxis regimen, AZT was initiated to reduce the risk of perinatal transmission of HIV. The infant's HIV RNA was 85 copies/ml on day 1 and 150 copies/ml on day 8. HIV was diagnosed and ART was initiated (i.e., AZT, lamivudine [3TC], and lopinavir/tironavir) on day 9. During ART, she was switched to antiretroviral medications (abacavir, 3TC, and nevirapine) because of adverse effects (i.e., anemia and lactic acidosis). After receiving ART, her serum HIV RNA level remained undetectable from day 80 to 2 years of age. The child's growth and neurological development were appropriate for her age, and a cranial magnetic resonance imaging performed at 1.5 years of age showed no abnormal findings. We suspected that the cause of the child's HIV infection was placental transmission in utero before ART, rather than perinatal or breastfeeding transmission. In cases in which maternal ART is not sufficiently administered, we recommended that ART should be initiated in the newborn, immediately after birth. Additionally, treatment should be continued according to the results of diagnostic HIV testing.
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Original Article
Title
A Case of Cardiac Fibroma Diagnosed after Cardiopulmonary Arrest
Author
Megumi Hatano1) Naoya Fukushima2) Mika Saito2) Takuya Tamame2) Seiichiro Yokoyama2) Hirotaka Ooki2) Masaru Miura2) Kazuhiko Shibuya2) Ryuji Fukuzawa3) and Tatsuo Kono4)
1)Department of General Pediatrics, Tokyo Metropolitan Children's Medical Center
2)Department of Cardiology, Tokyo Metropolitan Children's Medical Center
3)Department of Pathology and Laboratory Medicine, Tokyo Metropolitan Children's Medical Center
4)Department of Radiology, Tokyo Metropolitan Children's Medical Center
Abstract
A previously healthy 14-month-old boy presented with cardiopulmonary arrest and ventricular fibrillation. A cardiac tumor measuring 2.5×3.5 cm was depicted in the intraventricular septum on echocardiography. A fibroma was diagnosed on the basis of the following magnetic resonance imaging (MRI) findings: intramyocardial location involving the intraventricular septum: isointensity and hyperintensity on T1- and T2-weighted images, respectively: hypoperfusion on first-pass myocardial perfusion; and delayed enhancement on the equivalent phase. Although a standard therapeutic strategy remains to be established, a cardiac fibroma associated with life-threatening arrhythmias was resected with a favorable outcome. MRI is the most useful diagnostic modality because of its capability to delineate pathological characteristics of cardiac tumors, even in children. Surgical resection is considered in symptomatic patients with a cardiac fibroma and arrhythmias.
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Original Article
Title
A Case of Sturge-Weber Syndrome with Neonatal Cerebral Calcification
Author
Junji Shimizu1) Noriko Nishikura1) Eisuke Ito1) Tohru Sugimoto1) Fukiko Ryujin2) Seiichiro Yoshioka2) Tomoyuki Takano2) and Yoshihiro Takeuchi2)
1)Department of Pediatrics, Saiseikai Shiga Prefectural Hospital
2)Department of Pediatrics, Shiga University of Medical Science
Abstract
A case of Sturge-Weber syndrome with neonatal cerebral calcification was presented. His facial capillary malformation was at the paramidline of the forehead. This patient was initially referred to our hospital due to high fever. Brain computed tomography showed cortical calcification and marked cerebral atrophy extending from the left frontal lobe to the parietal lobe. Brain magnetic resonance imaging revealed hypointensity in the atrophic area on T2-weighted images and hyperintense lesions along the cerebral sulcus on gadolinium-enhanced T1-weighted images, suggesting leptomeningeal capillary malformation compatible with Sturge-Weber syndrome. Although epileptic seizures have not been observed, electroencephalography showed low-voltage patterns in the left frontal and parietal regions, as well as sporadic spike waves in the right frontal region. Oral administration of phenobarbital was started. Epileptic seizures have not occurred and his psychomotor development was normal at the age of 12 months, although there was mild spastic hemiparesis of the right upper limb. The presence of the contralateral epileptic discharges to the leptomeningeal capillary malformation in the present case suggests a variety of epileptogenetic patterns in this syndrome.
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Original Article
Title
Romiplostim Therapy in a Child with Refractory Chronic Immune Thrombocytopenia
Author
Satoshi Hirase1) Kosuke Nishida2) Natsuki Matsunoshita1) Nobuyuki Yamamoto1) Takeshi Ninchoji1) Takeshi Mori1) Yoshinobu Oyazato2) Tomoko Yanai1) Akira Hayakawa1) Yasuhiro Takeshima1) and Kazumoto Iijima1)
1)Department of Pediatrics, Kobe University Graduate School of Medicine
2)Department of Pediatrics, Kakogawa West City Hospital
Abstract
Romiplostim is a thrombopoietin (TPO) receptor agonist that is attracting attention as a novel active agent for treatment of adult chronic immune thrombocytopenia (ITP). We report a case of romiplostim treatment of childhood refractory chronic ITP. A 1-year old boy presenting with multiple purpura was diagnosed with ITP and referred to our hospital. Intravenous injection of immunoglobulin (IVIg) and oral prednisolone had a small, transient effect. Bone marrow examination showed a marked increase in megakaryocytes and no abnormal cells. Multiple immunosuppressive drugs including rituximab and methylprednisolone pulse therapy were administered, all of which proved to be ineffective. Dexamethasone pulse therapy transiently increased platelet count to> 1× 104-2×104/μl. Extensive subcutaneous hematoma and nasal bleeding were frequently observed. Splenectomy was considered but rejected because of the low platelet count. Six months after onset, romiplostim (1 μg/kg) was started. After the dose was escalated to 10 μg/kg, the platelet count stabilized at more than 1×104/μl. The bleeding tendency was gradually decreased and steroids were no longer necessary. More than 1 year has passed since romiplostim treatment was started and no adverse event has been observed. However, ITP did not reach complete remission and may require further treatment in the future. Romiplostim may benefit childhood refractory chronic ITP as well as adult ITP. However only small studies have been conducted to date and indications, safety and long-term outcome remain to be clarified. A large study of romiplostim treatment for childhood is warranted.
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