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THE JOURNAL OF THE JAPAN PEDIATRIC SOCIETY

Vol.127, No.3, March 2023


Original Article
1. Effects of Decoding Instruction Applications on Reading Tests
2. Spectrophotometric Hemoglobin Measurement for Noninvasive Anemia Screening in Children Younger than 2 Years
3. Usefulness of the Functional Independence Measure for Children (Wee FIM II®) in Children with Disabilities
Case Report
1. Acute Appendicitis during Acute Lymphoblastic Leukemia Treatment: A Case Report
2. Public Health Checkups for 18-month-old Infants Led to an Early Diagnosis of Fragile X Syndrome in a Boy Based on Facial Features and Family History
3. Fundus Findings Lead to Diagnosis of Cat Scratch Disease in a 1-year-old Boy
4. Out-of-hospital Cardiac Arrest in an Infant Diagnosed with Ileocecal Intussusception at Autopsy


Original Article
Title
Effects of Decoding Instruction Applications on Reading Tests
Author
Yuki Sekido and Tatsuya Koeda
Department of Psychosocial Medicine, National Center for Child Health and Development
Abstract
Background: This study investigates the effects of decoding instructions using a decoding application on oral reading test results.
Methods: A total of 162 patients who visited the outpatient clinic for dyslexia at the National Center for Child Health and Development between March 2016 and October 2019 participated in this study. The oral reading test was based on the practical guidelines for the diagnosis and treatment of specific developmental disorders. The inclusion criteria involved patients who had been diagnosed with dyslexia with or without comorbidities, who had taken two oral reading tests within 6 months from each other, and who had received only decoding instructions. The results of the first and second oral reading tests (SD: Standard Deviation values) were compared using corresponding t-tests. In addition, we divided the children into two groups: the lower and upper grade groups (grades 1-3 and 4-6, respectively), and examined the characteristics of the effects of the different age groups.
Results: The results were based on 51 patients. We observed a significant improvement in all time-related items. In terms of errors, a significant improvement could be observed in monosyllabic and nonsense.
Discussion: Decoding instructions using the oral reading instruction application improved oral reading skills of hiragana. The reading time and the number of mispronounced single syllables were substantially improved to <2 SD.
Conclusion: Application instructions are easy to understand and can be implemented at home.




Original Article
Title
Spectrophotometric Hemoglobin Measurement for Noninvasive Anemia Screening in Children Younger than 2 Years
Author
Shoko Matsu-uchi1) Ayako Sasaki2) Ukyo Sanjo1) Kuraaki Aoki1) Emi Saito1) Kazuhiro Akaba1) Kanae Kitaoka2) Takuya Abe2) Taketo Igarashi2) Yu Abiko2) Yosuke Sudo2) and Tetsuo Mitsui2)
1)Department of Pediatrics, Saiseikai Yamagata Saisei Hospital
2)Department of Pediatrics, Yamagata University Faculty of Medicine
Abstract
Adequate nutrition during infancy is essential for later growth and development. Both iron deficiency and iron-deficiency anemia can lead to irreversible cognitive defects. It is difficult to diagnose anemia solely from physical examination findings in routine clinical practice, and venipuncture for hematological screening is invasive. Although noninvasive spectrophotometric hemoglobin (SpHb) measurement by pulse co-oximeter has been used in the perioperative management of adults, the use of SpHb measurement for infants has not been fully investigated. This study included 52 participants (age 1-22 months) who underwent blood tests between April 2021 and March 2022, and we prospectively investigated the correlation between SpHb and venous hemoglobin (vHb) values to assess the feasibility of SpHb-based anemia screening in children younger than 2 years. SpHb was measured with a Rad-67TM Spot-check Pulse CO-Oximeter® (Masimo) and rainbow® DCI®-mini Sensor (Masimo) on the day of blood sample collection. The mean (±SD) SpHb and vHb values were 12.2 (±1.6) and 11.9 (±1.6) g/dL, respectively. SpHb and vHb were significantly and positively correlated (Pearson product-moment correlation coefficient 0.698, p<0.001). Bland-Altman analysis revealed a mean (±SD) bias of −0.353 (±0.953) g/dL in the difference between vHb and SpHb. Receiver operating characteristic curve analysis for SpHb-based detection of anemia (vHb cutoff <10.5 mg/dL) showed that SpHb (cutoff <11.6 mg/dL) could accurately predict anemia (sensitivity 83.3%, specificity 87.5%, and area under the curve 0.936 [95% confidence interval 0.87-1]). SpHb measurement can be a noninvasive screening method for detecting anemia in infants and toddlers.




Original Article
Title
Usefulness of the Functional Independence Measure for Children (Wee FIM II®) in Children with Disabilities
Author
Mizuki Sugiyama1)2) Keiji Hashimoto1)3) and Nobuyuki Kawate1)
1)Department of Rehabilitation Medicine, Showa University School of Medicine
2)Division of Otolaryngology, National Center for Child Health and Development
3)Division of Orthopaedic Surgery, National Center for Child Health and Development
Abstract
The purpose of this study was to examine the usefulness of the Functional Independence Measure for Children (Wee FIM) II® in children with disabilities. We evaluated 395 children of 6 months to 5 years of age who were referred to the Developmental Evaluation Center of the National Center for Child Health and Development and administered both the Kyoto Scale of Psychological Development (KSPD) and Wee FIM II®. We calculated the overall development quotient (DQ) and scores for each of the three DQ domains of the KSPD, and the total score and scores for each item of the Wee FIM II®. There were 262 cases in the DQ ≥70 group (KSPD DQ ≥70) and 133 cases in the DQ <70 group (KSPD DQ <70). In the DQ <70 group, there was a correlation between the posture-motor DQ of the KSPD and all items of the Wee FIM II® at 1–4 years of age, between the cognitive-adaptive DQ and all items at 3–5 years of age, and between the language-social DQ and all items at 4–5 years of age. In the DQ ≥70 group, there were limited correlations by age and item, and many items showed low or negligible correlations between each domain of DQ in the KSPD and Wee FIM II®. This study suggests that the assessment of ADL by the Wee FIM II® is useful as a developmental assessment for children with a low DQ.




Case Report
Title
Acute Appendicitis during Acute Lymphoblastic Leukemia Treatment: A Case Report
Author
Tomoki Yaguchi1) Shota Kato1) Yoshiko Nakano1)2) Moe Hidaka1) Masahiro Sekiguchi1) Kentaro Watanabe1) Mitsuteru Hiwatari1)2) Hideyuki Yokokawa3) Kanako Omata3) Mai Kutsukake3) Yusuke Watanabe4) Masaki Suzuki5) Shinya Takazawa3) Mariko Yoshida3) Jun Fujishiro3) and Motohiro Kato1)
1)Department of Pediatrics, The University of Tokyo Hospital
2)Department of Cell Therapy and Transplantation Medicine, The University of Tokyo Hospital
3)Department of Pediatric Surgery, The University of Tokyo Hospital
4)Department of Radiology, The University of Tokyo Hospital
5)Department of Pathology, The University of Tokyo Hospital
Abstract
Identifying the root of infection during neutropenia is often difficult due to ambiguous physical findings. However, infections during immunosuppression in patients with acute leukemia could result in severe and often fatal outcomes. Therefore, detecting the focus of infection and providing appropriate treatment is critical. Here, we report a case of acute appendicitis that was diagnosed using contrast-enhanced computed tomography (CECT) during acute leukemia treatment.
A 2-year-old boy with B-cell precursor acute lymphoblastic leukemia on the early consolidation therapy showed persistent fever and high inflammatory responses under severe neutropenia. His physical examination did not reveal findings that are suggestive of a specific focus of infection; however, a CECT scan revealed an enlarged appendix, confirming the acute appendicitis diagnosis. Post-diagnostic examination showed only a slight right lower quadrant abdominal tenderness. After conservative management with systemic antibiotics, the patient recovered without perforation or abscess formation, and appendectomy was performed after bone marrow recovery. The delay period of chemotherapy due to appendicitis treatment was considered to be about one week.
In this case, few signs could suggest the focus of infection, and a CECT scan was essential for acute appendicitis diagnosis. The patient was successfully treated with antibiotic therapy, and appendectomy avoided the risk of acute appendicitis recurrence during the following chemotherapy. Imaging studies should be performed to determine the appropriate treatment in cases of infection during bone marrow suppression, even in the absence of specific physical findings.




Case Report
Title
Public Health Checkups for 18-month-old Infants Led to an Early Diagnosis of Fragile X Syndrome in a Boy Based on Facial Features and Family History
Author
Nobuaki Tsuiki1) Hiroshi Terashima1)2) Teruyoshi Kurosawa1) Kohmei Ida1) Tetsuya Okazaki3) Kaori Adachi4) and Eiji Namba5)6)
1)Department of Pediatrics, Teikyo University Hospital, Mizonokuchi
2)Department of Pediatrics, National Rehabilitation Center for Children with Disabilities
3)Division of Clinical Genetics, Tottori University Hospital
4)Research Initiative Center, Organization for Research Initiative and Promotion, Tottori University
5)Otani Hospital
6)Organization for Research Initiative and Promotion, Tottori University
Abstract
Fragile X syndrome (FXS) is an X-linked genetic disorder, which is one of the most common inherited causes of intellectual disability. It affects one in 7,000 men and one in 11,000 women in Western countries, while it is estimated to affect one in 10,000 among Japanese men. The number of diagnosed cases of FXS in Japan is no more than 100, indicating that several cases may remain undiagnosed.
We diagnosed a boy with FXS at the age of 2 years, who was referred to us from routine checkups at the age of 18 months. After the diagnosis, his mother and maternal aunt were diagnosed as a premutation carrier. In recent years, genetic testing for FXS has been covered by national insurance in Japan, and a registry study is currently underway. FXS is one of the genetic disorders associated with intellectual disability which have been researched vigorously. Considering these facts, genetic testing, and proper diagnosis for patients with global developmental delay who have any physical characteristics or family history suggestive of FXS, can be beneficial for the patients as well as their families. Furthermore, access to genetic counseling can be valuable in such cases.




Case Report
Title
Fundus Findings Lead to Diagnosis of Cat Scratch Disease in a 1-year-old Boy
Author
Masato Itano1) Sayaka Kakei2) Daisuke Nakata2) and Hidetaka Nakai1)
1)Department of Pediatrics, Toyokawa City Hospital
2)Department of Ophthalmology, Toyokawa City Hospital
Abstract
Cat scratch disease (CSD) is a zoonosis caused by infection with Bartonella henselae through contact with cats, such as scratches and bites. Lymphadenopathy and fever are typical symptoms of CSD, but some cases present with various symptoms such as optic neuroretinitis, acute encephalopathy, aseptic meningitis, and hepatosplenic granuloma. Here, we describe a case of CSD in a young child that was diagnosed following fundus findings. The patient was a 16-month-old boy who was hospitalized for fever of unknown origin. He was found to have optic neuroretinitis in his right fundus, and it was also discovered that he kept cats in his home. From these facts, his diagnosis was suspected to be CSD with optic neuroretinitis. Therefore, he was treated with azithromycin, rifampicin, and prednisolone, which quickly resolved his fever and improved his fundus findings. Later, the diagnosis of CSD was confirmed based on serum antibody titers against Bartonella henselae. Complications of optic neuroretinitis in CSD are rare, and to the best of our knowledge, this is a report of the youngest patient in the world. When CSD is suspected in a young child who is unable to describe symptoms, an aggressive ophthalmologic examination should be performed even in the absence of ocular symptoms, because the treatment plan will depend on the presence of optic neuroretinitis.




Case Report
Title
Out-of-hospital Cardiac Arrest in an Infant Diagnosed with Ileocecal Intussusception at Autopsy
Author
Satoshi Tsuji1) Yoshiko Uchida1) Kino Hayashi2) and Mitsuru Kubota3)
1)Division of Pediatric Emergency and Transport Services, National Center for Child Health and Development
2)Tokyo Medical Examiner's Office
3)Department of General Pediatrics & Interdisciplinary Medicine, National Center for Child Health and Development
Abstract
Numerous cases of sudden death have been labeled as unspecified deaths in Japan; hence, pediatricians have little opportunity to determine the cause of death. By establishing a relationship with autopsy facilities, clinicians have come to appreciate the importance of investigating the cause of death in children and deepen mutual understanding. We describe the case of a 1-year-old boy with no previous medical history who developed sudden cardiac arrest at home. This patient did not respond to resuscitation procedures and was pronounced dead. Given our inability to identify the cause of death based on his medical history, physical examination, blood test findings, and imaging examination, the case was reported to the police as an unknown death. Subsequently, an administrative autopsy was performed at the Tokyo Metropolitan Medical Service after establishing interfacility collaboration. The autopsy revealed a duplication of the intestinal tract in the ileum with partial necrosis and food residue in the trachea. Histological examination revealed minimal inflammation at the site of intestinal strangulation. Viral isolation of tracheal secretions revealed adenoviruses, indicating the possibility of vomiting and asphyxia due to intestinal overload associated with adenovirus infection. Although autopsy studies on infantile sudden death have rarely reported intestinal tract obstruction, establishing a clinical diagnosis based on the physical findings, history, and laboratory and imaging findings remains difficult, suggesting the importance of autopsy in cases of sudden infant death.




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