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THE JOURNAL OF THE JAPAN PEDIATRIC SOCIETY

Vol.122, No.9, September 2018


Original Article
1. School-based Cardiovascular Screening: An Examination of Electrocardiographic Findings Useful for the Accurate Diagnosis of Atrial Septal Defect
2. Assessment of Psychosocial Function in Children with Daytime Urinary Incontinence
3. An Approach to the Establishment of a Pediatric Emergency System for the Centralization of Care for Critical Children in Rural Areas
Case Report
1. Successful Treatment of Pneumonia Associated with Ureaplasma Infection Using Azithromycin in Two Newborns
2. A Girl Complaining of Back Pain Diagnosed with Sickle Cell Disease
3. A Case of Wide-range Subcapsular Splenic Infarction Associated with Primary Epstein-Barr Virus Infection
4. HLA-B27-associated Reactive Arthritis in a Case Following Salmonella Enteritis: A Case Report
5. Five Cases of Preterm Infant with Biliary Atresia


Original Article
Title
School-based Cardiovascular Screening: An Examination of Electrocardiographic Findings Useful for the Accurate Diagnosis of Atrial Septal Defect
Author
Yuji Morito Masahiro Kamada Naomi Nakagawa Yukiko Ishiguchi Kengo Okamoto and Yoshimi Matsumoto
Department of Pediatric Cardiology, Hiroshima City Hiroshima Citizen's Hospital
Abstract
Background: Electrocardiographic (ECG) findings associated with atrial septal defect (ASD) include: V1 rSR' pattern (rSR'), isolated negative T-wave (IT), and QRS notching patterns at the inferior leads (crochetage). In school-based cardiovascular screening, however, it is not uncommon for such patterns to be missed.
Objectives: To avoid the failure of pediatric ASD diagnosis, we attempted to elucidate the connections between the mode of ECG examination, patient age, and pulmonary to systemic blood flow ratio (Qp/Qs).
Materials and methods: We evaluated 124 ASD patients, aged < 18 years, and in whom catheter tests were performed between 2010-2016 were included. These patients' ECG findings were classified into 5 types, type 1: rSR', type 2: IT, type 3: crochetage, type 4: a wide S-wave at the V6 lead (SV6), or type 5 which showed no abnormalities. The sensitivity and specificity of ASD diagnosis were calculated for different combinations of ECG findings.
Results: The rSR' positivity rate did not exceed 42% even in those with Qp/Qs ≥ 1.5. The combinations with the most positive rates were crochetage or SV6, and IT or crochetage or SV6. Even when ECG was restricted to only four leads, the combination of crochetage or SV6 showed high values for ASD diagnostic sensitivity (81%) and specificity (88%), respectively.
Conclusions: To avoid missing pediatric ASD during ECG, it is important to check for crochetage and/or wide S-waves at the V6 lead.




Original Article
Title
Assessment of Psychosocial Function in Children with Daytime Urinary Incontinence
Author
Hirokazu Ikeda1) and Setsuko Tamura2)
1)Department of Pediatrics, Showa University Fujigaoka Hospital
2)Department of Faculty of Applied Psychology, Tokyo Seitoku University
Abstract
[Objectives] Daytime urinary incontinence (DUI) significantly affects self-esteem, emotionally and behaviorally, and can impact the quality of life of patients and their families. However, there are few reports focusing on social functioning and school functioning in children with DUI.
[Method] We retrospectively collected data using the Pediatric Quality of Life Inventory 4.0 Generic Core Scales (PedsQL). A total of 233 children aged 5-15 years with a history of DUI experiencing more than one episode per day and their parents were enrolled in this study. The children and their parents completed the PedsQL questionnaire during their first visit. We performed a comparative analysis of the PedsQL in children with DUI and in 999 healthy children and 476 parents of healthy children.
[Results] The children with DUI had significantly lower quality of life in child self-reports for total score and psychosocial score than the healthy children. When compared with each school grade, emotional functioning was significantly lower in the third grade, social functioning was significantly lower in the first, second and third grades, while school functioning was significantly lower in the third and fourth grades. No significant differences were observed in the second- and third-grade elementary schoolchildren in the parent proxy-report.
[Conclusion] These results indicated that DUI episodes encountered in childhood daily life directly affected lower social functioning in the first grade. Early therapeutic intervention and psychosocial support for the children with DUI was crucial.




Original Article
Title
An Approach to the Establishment of a Pediatric Emergency System for the Centralization of Care for Critical Children in Rural Areas
Author
Shunsuke Amagasa Masatomo Kitamura Masanori Sato Norimasa Kurosaka and Hikoro Matsui
Pediatric Intensive Care Unit, Nagano Children's Hospital
Abstract
Background: The centralization care of critically ill children, which is a significant factor in order to obtain better outcomes, remains a challenging task, although many local governments have set out medical policies for pediatric emergencies.
Methods: We have established a pediatric emergency system for centralization of critically ill children in Nagano by improving three topics regarding the patient transfer system and interhospital collaboration. First, to provide prompt transport, we speeded up and diversified the existing transport system. Second, to strengthen communications, we discussed all transferred cases at regular clinical conferences in regional hospitals. Third, to transport patients to suitable tertiary medical facilities, we established an agreement with the university hospital.
Results: The numbers of transport and cases administered to our pediatric intensive care unit increased significantly (R2=0.86). The increases were observed at the time when we systematized patient transfer system and started the regular clinical conferences. Although the Pediatric Index of Mortality 2 of patients admitted to the PICU increased from 6.4% to 8.5%, the Standardized Mortality Ratio improved from 0.68 to 0.52, during the period from 2011 to 2014.
Conclusion: Improvement of transport systems and interhospital collaborations provided centralization of critically ill children, resulting in better quality of pediatric emergency care in Nagano. Our experience suggests that not only pediatric medical policy but also specific plans are vital for the establishment of a critical care system for children in rural areas.




Case Report
Title
Successful Treatment of Pneumonia Associated with Ureaplasma Infection Using Azithromycin in Two Newborns
Author
Ryohei Suzuki1) Airi Konishi1) Masumi Seki1) Yuriko Sekiguchi1) Yoko Saito1) Nagatoshi Sagara1) Takanori Motoki1) Kenichi Akashi1) Itaru Yanagihara2) and Toshio Katsunuma1)
1)Department of Pediatrics, The Jikei University Daisan Hospital
2)Department of Developmental Medicine, Osaka Medical Center and Research Institute for Maternal and Child Health
Abstract
Ureaplasma spp., a type of normal vaginal bacterial flora, can cause infections such as pneumonia and meningitis in newborns via vertical transmission. Here, we report two cases of pneumonia associated with Ureaplasma infection in late preterm infants who required respiratory management with a ventilator. Immediately after birth, both patients exhibited respiratory difficulty and were therefore intubated to start ventilator management. However, their respiratory condition progressively worsened because of increased airway secretions. Ureaplasma spp. were detected in respiratory secretions by culture and polymerase chain reaction (PCR). We diagnosed pneumonia associated with Ureaplasma infection based on their clinical course. Treatment with oral azithromycin improved their respiratory condition. Both patients were subsequently withdrawn from mechanical ventilation and were discharged with no further requirement for oxygen supplementation. While respiratory disorders due to Ureaplasma infection in neonates with extremely low birth weight have been widely described, the cases reported here suggest that Ureaplasma infection should be evaluated in late preterm infants for whom respiratory management is difficult, and that treatment with macrolide antibiotics should be considered.




Case Report
Title
A Girl Complaining of Back Pain Diagnosed with Sickle Cell Disease
Author
Shunsuke Noda Kanae Hirabayashi Takefumi Ishida Masayoshi Takayama and Yoshiro Amano
Department of Pediatrics, Nagano Red Cross Hospital
Abstract
In Japan, sickle cell disease (SCD) is uncommon. A 6-year-old girl, who suffered from back pain was admitted to the hospital. Her medical history revealed that her Brazilian father had sickle cell trait. She suffered from diarrhea, low grade fever, and pantalgia for 4 days before admission, and experienced severe back pain during sleep the night before admission. She was suspected to have SCD because of the presence of significantly low hemoglobin A1c (HbA1c) titer, and the appearance of sickle cells observed by the sickle cell test. The diagnosis for SCD was confirmed by β -globin sequencing. She was identified as having a compound heterozygote of Hb S [Codon 6 GAG (Glu)→ GTG (Val)] and β +-thalassemia (-31A→ G), which were inherited from her father and mother, respectively. Thoracic magnetic resonance imaging revealed a combination of high signal and low signal intensity in the thoracic vertebra suggesting microvascular vaso-occlusion. The treatment consisted of continuous infusion and antibiotics for 2 weeks, and her malady quickly improved. In conclusion, because of the increasing number of non-Japanese, and the subsequent increase in the number of children of Japanese and non-Japanese parents in Japan, sickle cell disease should be considered when patients who complain of sudden back, chest, or extremity pain are assessed.




Case Report
Title
A Case of Wide-range Subcapsular Splenic Infarction Associated with Primary Epstein-Barr Virus Infection
Author
Kouhei Ishida Keisuke Oyatani Syuhei Adachi Tugumi Takechi Yousuke Kosugi Mina Kosugi Hidekatu Nakamura Yusuke Daimon Minako Kihara Toshio Ohara and Yoshitaka Agatuma
Department of Pediatrics, Tomakomai City Hospital
Abstract
An 11-year-old girl was admitted to our hospital with a 3 day history of fever and left abdominal pain. She developed fever with sore throat about 2 weeks before admission. Abdominal contrast computed tomography (CT) showed an extensive hypo-enhanced subcapsular area of splenic tissue, which suggested splenic infarction. After a few days of conservative treatment, the patient's symptoms resolved. Later, she was serologically confirmed to have primary Epstein-Barr (EB) virus infection. Coagulation profiles indicated low plasma protein S and protein C activity, which normalized 1 week later.
Currently, contrast CT is the best noninvasive test to diagnose splenic infarction, which typically appears as a non-enhanced wedge-shaped area of splenic tissue. Because splenic infarction during EB virus infection has rarely been reported, its pathogenesis has been poorly understood. However, the reported histopathologic findings of the excised spleens from the patients consisted of diffuse reactive lymphoid hyperplasia, sinus congestion, and hypercellularity at the site of infarction. Contrast CT results of the present case would support these histopathologic findings.




Case Report
Title
HLA-B27-associated Reactive Arthritis in a Case Following Salmonella Enteritis: A Case Report
Author
Shuichi Tanese Katsuhiko Kitazawa Yukie Arahata Yuri Fujibe Akihiro Iguchi Hironobu Kobayashi Masayoshi Senda and Akihito Honda
Department of Pediatrics, Asahi General Hospital
Abstract
Reactive arthritis (ReA) is a sterile arthritis which develops after extra-articular infections such as bacterial enteritis and can be difficult to differentiate from juvenile idiopathic arthritis subtype enthesitis related arthritis (ERA) when accompanied by enthesitis. We report the case of an 11-year-old girl with HLA-B27-associated ReA following Salmonella enteritis. She developed right dorsal foot pain with swelling and erythema 10 days after the onset of enteritis, followed sequentially by left knee, right knee, left heel, and posterior neck pain during the subsequent two weeks. A diagnosis of arthritis in the right midfoot and both knees and enthesitis in the left sole and posterior neck was made. Joint stiffness was not present. Bilateral knee arthralgias were exacerbated by movement. Although her musculoskeletal pain, with the exception of her left knee arthralgia, resolved spontaneously within 3 weeks, her left knee arthralgia persisted and required treatment with ibuprofen. Stool culture performed 25 days after the onset of enteritis grew Salmonella species. HLA serotyping was positive for B27. Based on these findings, she was diagnosed with HLA-B27-associated ReA following Salmonella enteritis 5 weeks after the onset of arthritis. Her left knee arthralgia on walking gradually resolved for in the subsequent 5 months, and she exhibited no joint dysfunction in her left knee 2 years later. In this case, absence of joint stiffness, erythema over the affected joint, monophasic course, and a positive stool culture result demonstrating antecedent Salmonella infection were the crucial features in differentiating ReA from ERA.




Case Report
Title
Five Cases of Preterm Infant with Biliary Atresia
Author
Hiroko Miyatake1) Rieko Sakamoto1) and Kimitoshi Nakamura2)
1)Department of Neonatology, Kumamoto University Hospital
2)Faculty of Life Science, Department of Pediatrics, Kumamoto University
Abstract
There are few reports on premature infants of biliary atresia (BA). We encountered 5preterm infant cases that with BA. All cases were over 60 days-old as golden-time for BA treatment when they were diagnosed. Four cases underwent living donor liver transplantation (LDLT). One case underwent LDLT directly without being performed Kasai procedure because her liver showed severe fibrosis already. In preterm infants with jaundice, BA should be suspected even if they are older than the average age when full term newborns are diagnosed. We need to take BA into consideration as differential diagnosis when they showed elevated direct bilirubin (> 2.0 mg/dL). If we cannot exclude BA by various image examinations, it is necessary to promptly perform open biliary cholangiography to obtain definitive diagnosis.




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