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THE JOURNAL OF THE JAPAN PEDIATRIC SOCIETY
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Vol.121, No.10, October 2017
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Original Article
Title
Results of Five Years of School-based Electrocardiographic Screening
Author
Haruka Obinata Noriko Motoki Shoko Yamazaki Akira Hachiya Yohei Akazawa Satoshi Matsuzaki and Kenichi Koike
Department of Pediatrics, Shinshu University School of Medicine
Abstract
Background: A school-based electrocardiographic screening program has been developed in Japan to prevent the sudden cardiac death of children and adolescents with life-threatening heart disease. In the city of Matsumoto, Nagano prefecture, cardiologists review and evaluate all abnormal electrocardiographic results.
Purpose: We assessed the findings of the school-based electrocardiographic screening program in Matsumoto obtained over the last 5 years.
Methods and results: We retrospectively reviewed the medical records of all students registered in the school screening program. Of 33,625 students, 352 (1.0%) were diagnosed with borderline or abnormal electrocardiographic findings. Ultimately, 124 (0.37%) were diagnosed as having a cardiac abnormality. One hundred and five children had arrhythmia or electrocardiographic abnormalities, such as premature ventricular contractions, Wolf-Parkinson-White (WPW) syndrome, and long QT. Two subjects with WPW syndrome needed catheter ablation for paroxysmal supraventricular tachycardia. A KCNQ1 mutation was identified in 1 student with long QT. Seven children were diagnosed as having congenital heart disease: five possessed an atrial septum defect and 5 required surgical intervention. Cardiomyopathy was diagnosed in 2 children. We encountered no cases of cardiac sudden death, but a student with hypertrophic cardiomyopathy experienced cardiac arrest during exercise and needed resuscitation.
Conclusion: The electrocardiographic screening program in Matsumoto may be effective for early diagnosis of heart disease and prophylactic intervention. High-risk patients should undergo meticulous follow-up equivalent to that for clinically identified patients.
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Original Article
Title
Simplification of the CRH Stimulation Test
Author
Masahiro Goto Nao Shibata and Yukihiro Hasegawa
Division of Endocrinology and Metabolism, Tokyo Metropolitan Children's Medical Center
Abstract
The CRH stimulation test is an established tool to assess the adrenocortical function safely. However, it takes 120 minutes to accomplish the test. This retrospective study was conducted to examine the suitability of shortening the test to 60 minutes through re-evaluating the results of the tests completed in our hospital. The subjects were 377 individuals who took the tests from March 2010 to October 2014. A total number of 648 tests were performed (male 404, female 244; ages at the test ranged from 0.1-24.4 yrs, median 5.8 yrs). Based on the data of serum ACTH and cortisol (F) levels, their peak values were compared between the simplified method applying values of 0, 15, 30, and 60 minutes and the conventional method using all the values from 0 to 120 minutes. Peak values of ACTH and F in the simplified method were identical with those in the conventional method in 96% and 93% of the tests, respectively. Peak values of ACTH in the simplified method were less than 0.4 times those in the conventional method only in 0.6% of the tests. The simplified method increased the diagnosis of adrenocortical insufficiency only in 0.6% of the tests (4 tests). This simplified method may be useful in screening children with adrenocortical insufficiency.
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Original Article
Title
Effectiveness of Rotavirus Vaccines for Decrease of Rotavirus Gastroenteritis in the Pediatric Outpatient Clinic
Author
Shuji Nakata1) Takeshi Tsugawa2) Mayumi Ono2) and Masatoshi Tatsumi3)
1)Nakata Pediatric Clinic
2)Department of Pediatrics, Sapporo Medical University
3)Department of Pediatrics, Otaru Kyokai Hospital
Abstract
The reduction of rotavirus-associated hospitalization has been reported in countries where two kinds of rotavirus vaccines were introduced in the national immunization program. To investigate the efficacy of these vaccines in reducing office visits for rotavirus gastroenteritis (RVGE), fluctuations in the number of RVGE were analyzed in one pediatric clinic in Sapporo during 7 years before and 4 years after vaccine introduction. Twelve hundred and eighty-nine stool samples (98-147/ year) were collected from the patients aged 2 months to 12 years old visiting the clinic between January 2005 and September 2015 and were tested for the rotavirus antigen by immunochromatography. Presumed uptake rates of two vaccines in Sapporo and in the clinic were calculated. In the 2014 and 2015 seasons when the vaccine uptake rates reached 50% or more, the rotavirus-positive number decreased to 25 and 34, respectively, compared with 59 of the median number during 7 years (46-68/ year) before vaccine introduction, showing 58% and 42% reduction, respectively. The number of patients mainly reduced among children aged between 7 and 23 months old. Seasonality and the peak of rotavirus detection were similar before and after vaccine introduction. In the 2014 and 2015 seasons, the rate of RVGE patients who received full dose of RV vaccines was 24% and 15 %, respectively, and they were not hospitalized. The rate of intravenous drip infusion diminished from 52% to 25% among them. Rotavirus vaccine can reduce office visits for RVGE and decrease the severity of disease in the pediatric outpatient clinic.
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Original Article
Title
Outbreak of Leptospirosis in Children, 2016, Okinawa, Japan
Author
Tetsuhiro Sakihara1) Yutaka Kawamitsu1) Kouki Tomari2) Takuya Chuman2) Tsutomu Matsumora2) and Tomoko Kunishima1)
1)Department of Pediatrics, Social Medical Corporation Kariyushikai Heartlife Hospital
2)Department of General Pediatrics, Prefectural Okinawa Nanbu and Children's Medical Center
Abstract
We reviewed the clinical manifestations of eleven children who developed leptospirosis in an outbreak that occurred after recreational activity at a river in Okinawa prefecture. Subsequently, we performed a questionnaire survey of the recreational activity participants. In addition, we investigated the amount of rainfall and the number of typhoon events as risk factors of developing leptospirosis.
All 11 patients experienced spiking fever, and more than 80% of the patients presented with typical symptoms of leptospirosis such as conjunctivitis and calf pain. The patients were treated with bactericidal antibiotics, and 10 patients (90.9%) developed a Jarisch-Herxheimer reaction. Although none of the patients developed severe symptoms such as Weil syndrome, one patient presented with aseptic meningitis during the immune phase.
Sixty children participated in the recreational activity. Among the 58 subjects who completed the questionnaire, there were no significant correlations between the development of leptospirosis and sex, age, atopic dermatitis, lower extremity injuries, swimming, fresh water contact to conjunctivae, or accidental ingestion of fresh water. The incidence of leptospirosis in Okinawa prefecture showed no correlation with the amount of rainfall, but was negatively correlated with the number of typhoon events.
Although leptospirosis in children tends to present with non-specific clinical manifestations, our study subjects had a high incidence of conjunctivitis and/or calf pain comparable to adult cases. Further investigations are needed to elucidate the risk factors of leptospirosis.
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Original Article
Title
Evaluation of the Performance of the Preventive Measures Against Mother-to-child Transmission of HBV Infection and Clinical Course in Children Who Contracted HBV Infection from Their Mothers
Author
Tomoko Takano1) Hitoshi Tajiri1) Daiki Abukawa2) Ayano Inui3) Kosuke Ushijima4) Yuri Etani5) Mitsuyoshi Suzuki6) Yoko Miyoshi7) and Jun Murakami8)
1)Department of Pediatrics, Osaka General Medical Center
2)Department of General Pediatrics, Miyagi Children's Hospital
3)Department of Pediatric Hepatology and Gastroenterology, Saiseikai Yokohama City Tobu Hospital
4)Department of Pediatrics, Kurume University Medical Center
5)Department of Pediatric Gastroenterology, Nutrition and Endocrinology, Osaka Medical Center and Research Institute for Maternal and Child Health
6)Department of Pediatrics, Juntendo University School of Medicine
7)Department of Pediatrics, Osaka University Hospital
8)Division of Pediatrics and Perinatology, Faculty of Medicine, Tottori University
Abstract
This report showed the immunoprophylaxis in children (born from 1986 to 2013) with mother-to-child transmission of HBV infection from data of a nationwide multicenter survey. Although there was no case with complete immunoprophylaxis among children born in 1986-1990, cases with complete immunoprophylaxis gradually increased and became 40% among children born in 2011-2013. When compared with children with complete immunoprophylaxis, the age at diagnosis was younger in those with incomplete immunoprophylaxis and proportions of the cases with genotype A or B were higher than those with genotype C in children without immunoprophylaxis. The proportions of HBe seroconversion below the age of 15 years were higher in children with complete or incomplete immunoprophylaxis than those without immunoprophylaxis.
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Case Report
Title
Prevention of Neonatal Hemochromatosis by Antenatal High-dose IVIG; Success in 2 Cases
Author
Yusuke Mitani1) Yuka Asakura1) Hidenori Iwasaki1) Misato Obata1) Akihiro Yachie1) Shinji Nakamura2) Takashi Kusaka2) and Atsuko Nakazawa3)
1)Department of Pediatrics, Kanazawa University
2)Department of Pediatrics, Kagawa University
3)Department of Pathology, National Center for Child Health and Development
Abstract
Neonatal hemochromatosis is a disease with poor prognosis in which depositions of iron are observed in several organs - mainly in the liver - in the fetal period, and hepatic failure occurs immediately after birth. Since antenatal high-dose IVIG was first reported to be effective in 2004, some treatment results have also been reported in Japan. However, reports have been limited. Antenatal high-dose IVIG was administered to two cases in our hospital. IVIG was initiated at 17-18 weeks of gestation, and 50 g was administered once a week until birth. At 36 weeks of gestation, planned caesarean sections were carried out. No hepatomegaly or bleeding tendency were seen in both of the neonates. A transient enhanced hepatic echo-intensity was observed in one case. The blood tests revealed normal AST and ferritin in both cases. The serum GGT and AFP levels were measured in both newborns. The laboratory abnormalities had improved one month after birth. Coagulant functions were normal in both cases. Based on the above, it was demonstrated that antenatal high-dose IVIG is a highly effective treatment method for the prevention of neonatal hemochromatosis. However, the pathogenesis of neonatal hemochromatosis and the optimal regimen of immunoglobulin therapy have not been sufficiently studied. Further research is therefore needed.
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Case Report
Title
A One-year-old Girl with Chiari Malformation Type 1 Showing Central Cord Injury without an Obvious Trigger
Author
Megumi Shiota1)2) Masato Mori1) and Tomohiko Tsuru1)
1)Department of Pediatrics, Children's Medical Center, Matsudo City Hospital
2)Department of Pediatrics, National Rehabilitation Center for Children with Disabilities
Abstract
We report a case of asymptomatic Chiari malformation type 1 that showed acute cervical central cord injury. The case was a 17-month-old girl with uneventful gestational, delivery, and developmental histories. After she returned home from an amusement park, her left lower limb temporarily became numb without an obvious cause. Next morning, her upper limbs exhibited flaccid paralysis. MR imaging showed cervical cord swelling with an abnormal signal and ptosis of the cerebellar tonsils. We diagnosed Chiari malformation type 1 with central cord injury caused by the presyrinx state. These symptoms and cord swelling with an abnormal signal on MR imaging improved without any therapy within a month. Central cord injury in infants is rare, however, it could be often caused by minor injury, and we thus should pay attention to congenital anomalies with a high risk of cervical cord injury such as an asymptomatic Chiari malformation.
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Case Report
Title
Prompt Improvement of Transplant-associated Thrombotic Microangiopathy by Eculizumab Administration for Neuroblastoma after Autologous Hematopoietic Stem Cell Transplantation
Author
Ai Yamada Hiroshi Moritake Mariko Kinoshita Daisuke Sawa Hideaki Imamura Sachiyo Kamimura Takao Konomoto and Hiroyuki Nunoi
Division of Pediatrics, University of Miyazaki
Abstract
Transplantation-associated thrombotic microangiopathy (TA-TMA) is known to be a fatal complication after hematopoietic stem cell transplantation. Treatment strategy for patients with TA-TMA has not been established. Recently, eculizumab, a humanized monoclonal antibody against the complement component C5, has been shown to be effective for patients with TA-TMA who have elevated plasma soluble membrane attack complex (sMAC), which suggests the presence of dysregulation of the complement system. We report a 2-year-old boy with metastatic neuroblastoma who received an autologous peripheral blood stem cell transplantation (aPBSCT) and was diagnosed to have TA-TMA when fulfilling the diagnostic criteria on day 23 after aPBSCT. The symptoms were progressive and the patient developed diffuse alveolar and vitreous hemorrhage in addition to marked anemia, thrombocytopenia, massive proteinuria, and severe hypertension. These critical conditions showing a bleeding tendency, respiratory failure, and circulatory failure did not indicate the patient should receive apheresis. As compared with the plasma level of sMAC of healthy volunteers, it was elevated in the patient at the time of diagnosis. Therefore, the patient was treated with eculizumab after approval of our Institutional Review Board and obtaining informed consent from his legal guardians. The frequency of platelet transfusion decreased and respiratory condition improved soon after administration of eculizumab. The symptoms of TA-TMA did not recur and eculizumab was discontinued after 3 doses. It is definitely important to administer eculizumab to appropriate patients only, however, we hope complementary-related examinations as well as sMAC measurement are standardized, and a novel classification is established to identify critical patients with TA-TMA for whom eculizumab is effective.
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Case Report
Title
Invasive Meningococcal Disease during an Eculizumab Treatment for Atypical Hemolytic Uremic Syndrome Despite a Neisseria Meningococcal Vaccine
Author
Tomoo Kise Hiroshi Yoshimura Shigeru Fukuyama and Masatsugu Uehara
Division of Pediatric Nephrology, Okinawa Prefectural Nanbu Medical Center-Children's Medical Center
Abstract
Atypical hemolytic uremic syndrome (aHUS) has a poor prognosis. Eculizumab is an effective treatment for aHUS, but increases the risk of invasive meningococcal disease (IMD).
We report a case of IMD during eculizumab treatment, despite previous inoculation with the Neisseria meningococcal vaccine, in a 5-year-old boy who recovered with rapid treatment and no sequelae. A clinical diagnosis of aHUS was made. Before beginning eculizumab therapy, the patient received a meningococcal conjugate vaccine (serogroups A, C, Y, and W135). He was admitted to our hospital with fever and vomiting. Because bacterial infection was suspected, he was given 180 mg/kg/day of cefotaxime starting 2 hours after admission. Consequently his symptoms improved immediately. However, the fever recurred in the afternoon of day 2, and Neisseria meningococcus serogroup B was detected in blood culture on day 3. We performed an examination of the cerebrospinal fluid. However, this culture was negative for Neisseria meningococcus. Subsequently, the patient was given 240 mg/kg/day of cefotaxime. He was discharged after 10 days and has remained without sequelae, and at the latest follow-up (11 months since diagnosis), he was relapse-free.
IMD is a severe disease, and the key to a successful outcome is the administration of appropriate antibiotics as early as possible. It is essential to inoculate patients with the Neisseria meningococcal vaccine before eculizumab treatment. However, the current Neisseria meningococcal vaccine is ineffective against serogroup B. Thus, because IMD onset is possible despite inoculation with the vaccine, we should rapidly treat fever during eculizumab treatment while considering IMD.
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Case Report
Title
Comparison and Examination of Treatment with or without Exchange Blood Transfusion Therapy for Two Cases of Severe Infantile Pertussis [K1]
Author
Hiroyuki Esaki Kunio Hashimoto Mari Yokokawa Michiyo Sasaki Tomoyuki Hasuwa Hideki Motomura and Hiroyuki Moriuchi
Department of Pediatrics, Nagasaki University Hospital
Abstract
Pertussis is potentially serious during the neonatal and early infantile periods due to complications such as refractory respiratory failure and pulmonary hypertension. Exchange blood transfusion therapy may be effective for severe pertussis. We report two severe cases of pertussis and discuss the significance of exchange blood transfusion.
Patient 1: A two-month-old girl with respiratory failure was admitted with pulmonary hypertension and marked leukocytosis of 82,000/μ L. Exchange blood transfusion was started immediately, along with azithromycin, dexamethasone, and intravenous immune globulin (IVIG). The pulmonary hypertension was controlled by milrinone and furosemide. The patient recovered and was discharged on day 25 with no significant sequelae.
Patient 2: A two-month-old girl presented with seizure and respiratory failure, requiring artificial ventilation. The leukocyte count was 66,000/μ L. She responded poorly to treatment with azithromycin, methylprednisolone pulse therapy, and IVIG, but her condition slowly improved after cyclosporine A and dexamethasone were added to the regimen. She spent 124 days in the hospital and had developmental regression and retardation as neurological sequelae, requiring long-term continuous rehabilitation over 8 years.
These two patients shared common clinicoepidemiological features such as age, profound leukocytosis, and severe respiratory failure. The initial treatment regimens were also comparable, except for the application of exchange blood transfusion only in Patient 1, who had a better outcome despite having more severe pulmonary hypertension than Patient 2. Given that the early application of exchange blood transfusion therapy may have been a critical prognostic factor, we recommend this therapy be considered for inclusion in the initial treatment regimen for severe pertussis with marked leukocytosis.
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Case Report
Title
A Case of Scolding-induced Avoidant/restrictive Food Intake Disorder Successfully Treated by Nutrition Support Team and Sand Play Therapy
Author
Mao Shimazaki1) Daisuke Tokuhara1) Rie Aimoto1) Hiroki Fujimoto2) Yuki Cho1) Dai Miyawaki3) and Haruo Shintaku1)
1)Department of Pediatrics, Osaka City University Graduate School of Medicine
2)Division of Nutrition, Osaka City University Hospital
3)Department of Neuropsychiatry, Osaka City University Graduate School of Medicine
Abstract
An 8-year-old girl complained of difficulty in swallowing solid food after being scolded by her grandmother and mother. Because she didnot fear weight gain, had no pathological findings on brain MRI and no difficulty in swallowing liquid, we diagnosed her as having an avoidant/restrictive food intake disorder possibly caused by scolding. After admission on day 7, we started nutrition support team (NST) intervention by introducing various types of food (taste, texture and solidity) to the patient. In addition, she received sand play therapy to enhance self-expression. She succeeded in ingesting a soft jelly on day 15, and gradually recovered swallowing solid food in parallel with self-expression, and was discharged on day 42. She has had no recurrence for at least 5 months after the discharge. Nutritional support for eating behavior by using various types of food (taste, texture and solidity) and a support for self-expression by using sand play therapy were effective methods in treating this patient with an avoidant/restrictive food intake disorder.
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