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THE JOURNAL OF THE JAPAN PEDIATRIC SOCIETY

Vol.123, No.1, January 2019


Original Article
1. Review of Five Years After Establishment of an Outpatient Clinic for Fetal Echocardiography and Its Implications for Perinatal Medicine
2. Prognosis of Patients with Trisomy 18 Who Underwent Pulmonary Artery Banding
3. Study of 112 Pediatric Patients with Eating Disorders at the Department of Pediatrics, Okayama University Hospital
Case Report
1. A Child in Whom a Paradoxical Brain Embolism Developed with a Sax Performance
2. A Case of Invasive Meningococcal Disease Managed by Rapid Diagnosis and Early Treatment Intervention
3. Endotracheal Tumor Detected in a 14-year-old Girl Treated for Wheezing
4. Idiopathic Intervertebral Disc Calcification in Childhood: A Case Report and Review
Brief Report
Positivity Rate and Costs of Pre-surgical Screening for Infectious Diseases at a Pediatric Tertiary Care Hospital


Original Article
Title
Review of Five Years After Establishment of an Outpatient Clinic for Fetal Echocardiography and Its Implications for Perinatal Medicine
Author
Takeshi Kumagai1) Nobuyuki Kakimoto1) Takayuki Suzuki1) Mitsuhiko Riko1) Yoshinobu Tsuno1) Takuya Sugimoto1) Mina Ueda1) Tomohiro Suenaga2) Takashi Takeuchi2) and Hiroyuki Suzuki2)
1)NICU division, Department of Perinatal Medicine, Wakayama Medical University
2)Department of Pediatrics, Wakayama Medical University
Abstract
In June 2012, we established an outpatient clinic for fetal echocardiography in our obstetrics department. During the past 5 years, there were 207 referrals for fetal echocardiography; the number of the introduced pregnant women has been gradually increasing and the fetal diagnosis ratio of critical congenital heart disease (critical CHD) of patients hospitalized during the neonatal period has increased rapidly from 10% to over 70%. The critical CHD patient ratio of neonatal transfer cases has significantly decreased because of the fetal echocardiography and maternal transfer before birth. However, some cases of critical CHD transferred from other institutes were not diagnosed during fetal life, and 3 cases were misdiagnosed and needed surgical treatment.
There are some problems, (1) misdiagnosed cases, (2) neonatal transfer cases with critical CHD who were not diagnosed antenatally, (3) opportunity of training for residents decreased dramatically.
Our aim is the diagnosis of critical CHD as early as possible when the condition is stable, and we hope that the use of fetal echocardiography will become more widespread in Wakayama prefecture. It has been realized perinatal care is effective to prevent newborns with critical CHD from requiring ambulance transportation.




Original Article
Title
Prognosis of Patients with Trisomy 18 Who Underwent Pulmonary Artery Banding
Author
Tomomi Hasegawa1)2) Yumi Sato3) and Toshikatsu Tanaka3)
1)Department of Pediatric Critical Care Medicine, Kobe Children's Hospital
2)Department of Cardiovascular Surgery, Kobe Children's Hospital
3)Department of Cardiology, Kobe Children's Hospital
Abstract
Although trisomy 18 (T18) has traditionally been considered as a lethal anomaly, recent reports have indicated that cardiac surgery improves survival and hospital discharge rates. This study aimed to investigate the prognosis of patients with T18 after they undergo pulmonary artery banding (PAB). Twenty-one consecutive patients with T18 who underwent PAB at Kobe Children's Hospital between 2006 and 2015 were enrolled in this retrospective study. The perioperative variables of patients with T18 were compared with those with trisomy 21 (T21). Intubation time and the length of ICU and hospital stay after PAB were significantly longer in patients with T18 than in those with T21. The percentage of patients with T18 who were discharged home was 76.2%. Of these, a considerable number required home medical care, including tube feeding and mechanical ventilation. The hospital readmission rate of patients with T18 was significantly higher than that with T21. In patients with T18, the cumulative 1- and 2-year survival rates were 74.5% and 50.9%, respectively, and the rate of total intracardiac repair was remarkably low at 18.8%. These findings suggest that cardiac surgeries such as PAB in patients with T18 are not ideal with respect to their improvement of the mid-term to long-term prognoses at present. Medical teams should share these findings and seek appropriate treatment for all patients with T18 and their families, whether they undergo PAB.




Original Article
Title
Study of 112 Pediatric Patients with Eating Disorders at the Department of Pediatrics, Okayama University Hospital
Author
Ayumi Okada1) Chikako Fujii2) Yoshie Shigeyasu2) Akiko Sugihara2) Yasuko Tsurumaru2) Tomoko Akagi2) Aya Shimauti2) Mizuho Hosogi2) Eriko Munemori2) and Hirokazu Tsukahara1)
1)Department of Pediatrics, Okayama University Graduate School of Medicine Dentistry and Pharmaceutical Science
2)Department of Pediatrics, Okayama University Hospital
Abstract
The incidence of eating disorders among children is increasing, and their onset is occurring earlier. The purpose of this study was to investigate the characteristics of children with eating disorders and to evaluate the effectiveness of treatment by pediatric approaches.
We retrospectively reviewed the records of 112 pediatric patients diagnosed with eating disorders (at age< 18 years) who were treated as outpatients between 1999 and 2015 at the Department of Pediatrics, Okayama University Hospital. Among the 112 pediatric patients, 59 (52.7%) had anorexia nervosa (AN), 1 (0.9%) had bulimia nervosa (BN), and 52 (46.4%) had avoidant/restrictive food intake disorder (ARFID). Compared with patients with AN and BN, those with ARFID were younger, were more likely to be male, and had a lower median weight loss. Regarding outcome, eating disorders were relieved or improved in 69 (67.6%) patients with treatment. Among the remaining patients, there was aggravation in 1 (0.98%) patient, treatment was interrupted in 2 (2.0%), and 17 (16.7%) patients were referred to the Department of Psychiatry because of the worsening of their condition. Furthermore, 10 (9.8%) patients were referred elsewhere because they moved house and 3 (2.9%) were referred for physical consultation for a possible medical disorder. In our experience with pediatric patients with eating disorders, the proportion of those with ARFID was relatively high. Understanding the type of eating disorders is necessary to provide appropriate treatment. Treatment by pediatricians was effective in many cases, but pediatricians should consult psychiatrists when caring for adolescents with eating disorders.




Case Report
Title
A Child in Whom a Paradoxical Brain Embolism Developed with a Sax Performance
Author
Toshinobu Ifuku Toshihiro Nishiguchi Akihiko Yuge and Keigo Nakatani
Department of Pediatrics, Miyazaki Prefectural Miyazaki Hospital
Abstract
The causes of childhood brain infarction are diverse and often cannot be specified. We herein report the case of a 14-year-old girl who developed a paradoxical brain embolism due to Valsalva strain, which was complicated by a patent foramen ovale (PFO).
The patient developed photophobia of the right eye and cephalalgia while playing the saxophone at home. She had previously experienced similar symptoms while playing the saxophone. Cerebral MRI revealed an acute cerebral infarction in the left occipital lobe. We initiated the administration of edaravone and heparin.
Transthoracic echocardiography revealed a PFO. Following the injection of contrast agent, the application of Vasalva strain caused bubbles to appear inside the left atrium after one beats on transesophageal echocardiography. No arrhythmias, deep venous thrombosis, or arteriovenous fistula were detected.
We diagnosed paradoxical brain embolism due to a PFO. We suspected that the symptoms might recur because of her past history; thus, we performed the surgical closure of the PFO. No symptoms have occurred since.
In the present case, Valsalva strain due to playing the saxophone might have induced right to left shunt inside the atrium, leading to a paradoxical brain embolism. Children with PFO may develop paradoxical brain embolisms due to various types of stress.




Case Report
Title
A Case of Invasive Meningococcal Disease Managed by Rapid Diagnosis and Early Treatment Intervention
Author
Yoji Uejima1) Erica Hiwatari3) Koji Sakuraya4) Ryusuke Nambu3) Satoshi Sato1) Eisuke Suganuma1) Tadamasa Takano1) Shuichiro Fujinaga4) Takashi Arai2) Hideki Niimi5) Isao Kitajima5) Tsutomu Oh-ishi1) and Yutaka Kawano1)
1)Division of Infectious Diseases and Immunology, and Allergy, Saitama Children's Medical Center,
2)Clinical Laboratory, Saitama Children's Medical Center,
3)General Pediatrics, Saitama Children's Medical Center,
4)Division of Nephrology, Saitama Children's Medical Center,
5)Department of Clinical Laboratory and Molecular Pathology, Graduate School of Medical and Pharmaceutical Science, University of Toyama
Abstract
Invasive meningococcal disease rapidly causes deterioration of the body's system; thus, prompt and careful support is essential in the clinical setting. A six-month old girl was transferred to our hospital owing to her poor general condition, vomiting, and pale purpura. She developed fever the day before admission. We quickly confirmed bacteremia from her blood using the melting temperature mapping method, and identified Neisseria meningitidis from her blood using multiplex PCR. We diagnosed purpura fulminans and septic shock due to meningococcal infection. Her condition improved considerably after rapid fluid loading, catecholamine administration, and anti-disseminated intravascular coagulation therapy. Further treatment with continuous hemodialysis and polymyxin-B direct hemoperfusion stabilized her respiratory and circulatory dynamics. Blood cultures collected on admission were negative. Rapid identification of the pathogen ensured appropriate intervention and treatment in the early stage of the disease, thereby enabling her survival without major complications or sequelae. In addition, prophylactic administration among close contacts prevented secondary infection.




Case Report
Title
Endotracheal Tumor Detected in a 14-year-old Girl Treated for Wheezing
Author
Yuko Mizutani1)2) Yasushi Kanda1) Mutsuo Ishii1) and Shinji Saitoh2)
1)Department of Pediatrics, Japanese Red Cross Nagoya Daini Hospital
2)Department of Pediatrics and Neonatology, Nagoya City University Graduate School of Medical Sciences
Abstract
Herein, we report a 14-year-old girl with an endotracheal tumor. She complained of persistent cough, wheezing, and intermittent hemoptysis for one and half months. She was initially given diagnosis of bronchial asthma, and intravenous glucocorticoids, oral and inhaled bronchodilators showed partial improvement of the symptoms. Despite the improvement, her lung function tests showed severe obstructive ventilatory dysfunction. Chest computed tomography revealed a 10 to 14 mm lesion in the right wall of the lower trachea. Tumor resection and tracheoplasty were performed and complete remission was achieved. Pathologically, the tumor was diagnosed as angiomatoid fibrous histiocytoma. Endotracheal tumor is extremely rare in the pediatric population, and is often misdiagnosed with bronchial asthma at the initial evaluation. However, rapid and accurate evaluation is crucial because of the danger of suffocation. It is thus important to examine chest computed tomography for patients who have clinical symptoms that can not be explained only by bronchial asthma even if chest X-ray is normal.




Case Report
Title
Idiopathic Intervertebral Disc Calcification in Childhood: A Case Report and Review
Author
Shin Kasai Yoshiyuki Furuichi Aya Itoyama Sayaka Kamei Toshie Nishijima and Tomoaki Sano
Department of Pediatrics, Yamanashi Red Cross Hospital
Abstract
Pediatric cervical intervertebral disc calcification has a good prognosis with conservative treatment. We report the case of 4-year-girl, who complained of neck pain, limitation of neck movement and fever. We diagnosed cervical intervertebral disc calcification radiologically and 21 months later, the calcification was resorbed with conservative treatment.
We researched 44 cases of children that have been reported from 1949 to 2017. The common age was 3-13 years (average 7.51) and male-to-female ratio was about 3: 2. The average term was 10.21 months for the calcifications to disappear. It was revealed that the disappearance term of calcifications was not affected by infection or trauma before or after onset. No changes in the term of disappearance within more than two disk spaces or one were noted.
We accessed the past cases and suggested 3 points, 1) over 10 years old, 2) calcifications exist under the lower thoracic spine, 3) neuropathy or dysphagia, that were significant to evaluate the prognosis. The cases in which these points may be related may have bad prognosis with conservative treatment. Moreover H2 blocker treatment possesses the potential to shorten the time to disappearance term of calcifications compared to conservative treatment.




Brief Report
Title
Positivity Rate and Costs of Pre-surgical Screening for Infectious Diseases at a Pediatric Tertiary Care Hospital
Author
Hidenori Nakagawa1) and Isao Miyairi1)2)
1)Office for Infection Control, National Center for Child Health and Development,
2)Division of Infectious Diseases, Department of Medical Subspecialties, National Center for Child Health and Development
Abstract
The rates and costs of positive pre-surgical testing for infectious diseases in Japan are unknown. During the study period between April 2008 and March 2015, pre-surgical screening tests for patients aged less than 18 years was performed for HBs antigen, HCV antibody, and TPLA of which, 15/16,106 (0.09%), 208/16,038 (1.3%), and 29/13,672 (0.21%) were positive, respectively. None of these revealed a new diagnosis and required re-testing in 172 cases. The total cost of these tests was 13 million yen per year and costs for re-testing was 0.4 million yen per year.
Screening tests for populations with a low pretest probability appears to have a number of disadvantages.




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