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THE JOURNAL OF THE JAPAN PEDIATRIC SOCIETY

Vol.125, No.11, November 2021


Review
1. Langerhans Cell Histiocytosis Associated CNS-neurodegenerative Disease
Original Article
1. Effect of Discontinuing Routine Gastric Aspirate And Skin Surface Cultures in Neonatal Early-onset Sepsis
2. Emergency Removal Is Not Required for Pediatric Asymptomatic, Gastric and Smaller-diameter Button Battery Ingestions
Case Report
1. Primary Ciliary Dyskinesia with Difficulty in Respiratory Management, Accompanied by Persistent Pulmonary Hypertension of the Newborn
2. A Boy Who Developed Drug-induced Hypersensitivity Syndrome (DIHS) after Treatment of Kawasaki Disease and Presented with Grave's Disease as Late-term Complication
3. Different Clinical Courses in Two Cases of Neonatal Marfan Syndrome with Identical FBN1 Mutation
4. Diabetic Ketoacidosis Complicated by Marked Hypernatremia
5. A Case of an 11-year-old Japanese Child with Multisystem Inflammatory Syndrome in Children Associated with Coronavirus Disease 2019 That was Treated as Kawasaki Disease
Brief Report
Mask-wearing Survey among Young Children and Parental Awareness during COVID-19


Review
Title
Langerhans Cell Histiocytosis Associated CNS-neurodegenerative Disease
Author
Kenichi Sakamoto1) Yoko Shioda1) Akira Morimoto2) and Shinsaku Imashuku3)
1)Children's Cancer Center, National Center for Child Health and Development
2)Department of Pediatrics, Jichi Medical University School of Medicine
3)Division of Laboratory Medicine, Uji-Tokushukai Medical Center
Abstract
Langerhans cell histiocytosis (LCH) is thought to be an inflammatory myeloid neoplasia derived from immature myeloid dendritic cells with the MAPK pathway gene mutation. Though the overall survival in patients with LCH is generally excellent, they may suffer various permanent consequences (PCs), which impairs the quality of life (QOL). Among PCs, the LCH associated CNS-neurodegenerative disease (LCH-ND) is the most serious and dismal disease. Here, we first review the pathology and the diagnosis of LCH-ND, and clinical course of the patients with this disease. Thereafter, we discuss the current studies on clarifying the mechanisms causing LCH-ND, various treatments attempted to date as well as some future proposals for prophylactic measures in preventing the occurrence of LCH-ND.




Original Article
Title
Effect of Discontinuing Routine Gastric Aspirate And Skin Surface Cultures in Neonatal Early-onset Sepsis
Author
Yukitsugu Nakamura1) Tadatomo Ohyanagi2) Maho Kobayashi1) Rei Fumimoto1) Takumi Moriuchi1) Ayano Shinagawa1) Ryo Niiya1) Miki Sunada3) Shiori Komachi3) Akane Hirose3) Emi Okishio3) Manami Suzuki3) Tomohiro Katsuta1) Isamu Hokuto3) and Naoki Shimizu1)
1)Department of Pediatrics, St. Marianna University School of Medicine
2)Department of Clinical Laboratory, St. Marianna University School of Medicine Hospital
3)Department of Neonatology, St. Marianna University School of Medicine
Abstract
Although gastric aspirate and skin cultures are often performed in addition to blood cultures in Japan, the positive predictive value (PPV) to isolate the causative agent in neonatal early-onset sepsis (EOS) is low. To evaluate the impact of discontinuation of routine gastric aspirate and skin cultures on the clinical practice of EOS, we investigated the characteristics in patients with suspected EOS before and after discontinuation. The duration of the study period was 1 year before and after discontinuation. Sixty-one patients were evaluated before and 52 patients after discontinuation. The number of confirmed cases of EOS was two, one each before and after discontinuation. The PPV to isolate the causative agent in EOS were low, at 10.0% for gastric aspirate and 4.2% for skin cultures. The median duration of antimicrobial therapy for EOS was 3 days before and after discontinuation, and the initial antimicrobial therapy was changed in 6 patients (9.8%) before and 3 patients (5.8%) after discontinuation. The utility of gastric aspiration and skin surface cultures in EOS management is low, and there was no worsening of patient outcomes after discontinuation. The discontinuation was appropriate.




Original Article
Title
Emergency Removal Is Not Required for Pediatric Asymptomatic, Gastric and Smaller-diameter Button Battery Ingestions
Author
Keiichi Tomita1) Shunsuke Amagasa1) Shima Ohnishi1) Satoshi Tsuji1) Satoko Uematsu1) Mitsuru Kubota2) Shunsuke Nosaka3) and Akihiro Fujino4)
1)Division of Pediatric Emergency and Transport Services, National Center for Child Health and Development
2)Department of General Pediatrics & Interdisciplinary Medicine, National Center for Child Health and Development
3)Department of Radiology, National Center for Child Health and Development
4)Division of Surgery, National Center for Child Health and Development
Abstract
Objective: For button battery ingestions (BBIs) in children, there is no universal recommendation for management of patients with asymptomatic gastric BBIs. Since 2014, the National Center for Child Health and Development has introduced clinical guidelines for BBIs, and observation has been chosen over emergency removal of asymptomatic gastric BBIs. Method: We compared the changes in clinical outcomes (rate of removal, rate of consultation with pediatric surgeons, and clinically significant complications) before and after its introduction in a retrospective study of asymptomatic 44 children <18 years of age who had gastric BBIs at our hospital. Result: The respective rates (before and after guideline introduction) of removal (68.2% and 31.8%; p = 0.016) and consultation with pediatric surgeons (90.9% and 40.9%; p < 0.001) decreased significantly, whereas the rate of clinically significant complications did not increase. However, in the study period, only 2 patients had batteries with diameters of ≥15 mm. Conclusion: It may be possible to safely follow-up patients with asymptomatic gastric BBIs without emergency removal if the battery diameter is <15 mm, which may minimize the need for invasive procedures in children.




Case Report
Title
Primary Ciliary Dyskinesia with Difficulty in Respiratory Management, Accompanied by Persistent Pulmonary Hypertension of the Newborn
Author
Yoshikazu Otsubo1) Taizou Kirino1) Fumiko Kinoshita2) and Yusuke Yamane3)
1)Department of Pediatrics, Sasebo City General Hospital
2)Department of Pediatrics, Nagasaki University Hospital
3)Department of Pediatric Surgery, Nagasaki University Hospital
Abstract
Primary ciliary dyskinesia (PCD) is characterized by both ciliary structural and functional dysfunction, which is an autosomal recessive disorder that involves repeated chronic respiratory tract infections. We encountered a case of PCD accompanied by persistent pulmonary hypertension after birth with severe neonatal asphyxia. Long-term respiratory management was required, therefore tracheotomy was performed two months after birth. A bronchial biopsy led to a diagnosis of PCD. In addition to complications of the respiratory tract, gastroesophageal reflux disease and severe sensorineural deafness were confirmed. Mechanical in-exsufflator in addition to administering macrolide and expectorants were ineffective. The patient was never able to be removed from mechanical ventilation and died due to respiratory dysfunction five months after birth. The long-term mechanical ventilation using high-pressure, high-concentration oxygen immediately after birth was considered to be another cause of the aggravation of illness in this case. A genetic panel test of PCD after death found no mutations among 32 known genes. The addition of respiratory exacerbation factors to neonates with PCD as an underlying disorder may lead to a more serious clinical course. PCD might therefore be included in the differential diagnosis in neonates presenting with unexpected respiratory failure.




Case Report
Title
A Boy Who Developed Drug-induced Hypersensitivity Syndrome (DIHS) after Treatment of Kawasaki Disease and Presented with Grave's Disease as Late-term Complication
Author
Akemi Ono Shuji Kondo and Kazuhiro Mori
Department of Pediatrics, Tokushima Prefectural Central Hospital
Abstract
Drug-induced hypersensitivity syndrome (DIHS) is a severe drug eruption with viral reactivation, and is known to be complicated by autoimmune diseases during the recovery period. Few pediatric cases with DIHS have been described, particularly cases with late complications of autoimmune disease after DIHS. The patient in our case was an 18-month-old boy who was diagnosed with Kawasaki disease (KD) and treated with aspirin and γ-globulin. Although initial therapy resoled his KD symptoms once, recurrence of fever, rash, cervical lymphadenopathy, hepatic disorder, and pleural effusion and ascites were observed on day 20. Recurrent KD was initially considered, but symptoms exacerbated and eosinophilia and atypical lymphocytes increased in peripheral blood following additional treatment for KD. Atypical DIHS was finally diagnosed based on the diagnostic criteria, although reactivation of human herpesvirus 6 could not be demonstrated, and the patient improved with steroid treatment. Twenty-two months after onset of DIHS, Graves' disease with symptoms of tachycardia and excessive sweating was identified, and is currently being treated. To the best of our knowledge, this is the first report of DISH occurring during the treatment of KD, with Graves' disease developing in the distant period.
This case emphasizes the need for DIHS to be differentiated when exacerbation of symptoms occurs with high atypical lymphocyte and eosinophil counts during Kawasaki disease treatment. Furthermore, patients with DIHS require long-term follow-up as it may be complicated by autoimmune diseases during the recovery phase.




Case Report
Title
Different Clinical Courses in Two Cases of Neonatal Marfan Syndrome with Identical FBN1 Mutation
Author
Nana Kushima1)3) Shuku Ishikawa1) Hidekatsu Nakamura1) Madoka Sawada2) Motoki Takamuro2) Hideomi Asanuma1) and Yukihiko Kawasaki3)
1)Department of Neonatology, Hokkaido Medical Center for Child Health and Rehabilitation
2)Department of Pediatric Cardiology, Hokkaido Medical Center for Child Health and Rehabilitation
3)Department of Pediatrics, Sapporo Medical University
Abstract
Neonatal Marfan syndrome (nMFS) is a rare genetic disease with a poor prognosis. We report two cases of nMFS. Neonate 1 had a senile appearance and long fingers. He was transferred to our hospital on the first day after birth. Echocardiography revealed dilatation of the aortic root, mitral valve regurgitation, and tricuspid valve regurgitation. Although he received medication for heart failure and mechanical ventilatory support, he died at the age of four years due to pneumonia. Neonate 2 was transferred to our hospital shortly after birth due to cyanosis and heart murmurs. She had a circular shunt through the large ductus arteriosus and patent foramen ovale due to the presence of severe tricuspid dysplasia, and underwent tricuspid valve surgery on day one. Although she received medication for heart failure, she presented with progressive heart and respiratory failure. She subsequently died because of a viral infection. Each neonate had a known missense mutation in exon 25 of FBN1, and each was diagnosed as nMFS. However, even with the same variant, their clinical courses differed. Here, we discuss the diagnosis of nMFS and the relevance of genotype-phenotype correlation.




Case Report
Title
Diabetic Ketoacidosis Complicated by Marked Hypernatremia
Author
Ryosuke Jokaku Yoshihiko Yuyama Yuko Hotta Satsuki Nishigaki and Tomoyuki Kawamura
Department of Pediatrics, The Osaka City University Graduate School of Medicine
Abstract
Diabetic ketoacidosis (DKA) commonly presents with apparent hyponatremia because hyperglycemia induces the transfer of free water into vessels. However, some patients with DKA present with hypernatremia and free water loss and require meticulous fluid and electrolyte management. We report our experience with a boy with new-onset type 1 diabetes and DKA complicated by marked hypernatremia in whom favorable outcomes were obtained by concurrent insulin and fluid therapy based on the predicted sodium dynamics through serum and urine electrolyte levels. A 14-year-old boy was transferred because of hyperglycemia and consciousness disturbance. Hypernatremia (174 mEq/L) was detected; thus, concurrent treatment for DKA and hypernatremia was required. We predicted the pre-treatment free water loss associated with hypernatremia and changes in serum sodium levels using the Adrogue-Madias formula and adjusted fluid replacement. Urine sodium excretion increased when the predicted fluid replacement volume was reached 52 hours after treatment initiation. After 90 hours, his serum sodium levels improved to 142 mEq/L. The patient was discharged without sequelae on day 9 of hospitalization and has resumed his usual insulin treatment. Preventing sudden changes in serum sodium levels is important when treating hypernatremia complicated by DKA and dehydration. This can be achieved by estimating the amount of free water loss before treatment initiation, monitoring serum and urine sodium levels, and adjusting fluid replacement while considering physiological sodium dynamics.




Case Report
Title
A Case of an 11-year-old Japanese Child with Multisystem Inflammatory Syndrome in Children Associated with Coronavirus Disease 2019 That was Treated as Kawasaki Disease
Author
Eriko Kudo Natsumi Ono Takeshi Yamazaki Tomoshiro Ito Kinya Hatakeyama and Hitomi Sano
Department of Pediatrics, Sapporo City General Hospital
Abstract
Multisystem inflammatory syndrome in children (MIS-C) has been primarily reported in Europe and the United States and causes severe inflammation of multiple organs associated with coronavirus disease 2019 (COVID-19). Though patients with MIS-C display features similar to those with Kawasaki disease, there are some differences between the two. Compared with the typical Kawasaki disease, MIS-C is more common in older children and is often accompanied by gastrointestinal and neurological symptoms, decreased blood pressure, and cardiac dysfunction. Further, it presents with laboratory features such as lymphopenia, thrombocytopenia, and markedly elevated inflammatory markers. Here, we present an 11-year-old Japanese boy who developed MIS-C and presented with the aforementioned clinical features 7 weeks after the onset of COVID-19. He was treated for Kawasaki disease because he also had typical Kawasaki disease symptoms. This is the first case of MIS-C associated with COVID-19 reported in Japan.




Brief Report
Title
Mask-wearing Survey among Young Children and Parental Awareness during COVID-19
Author
Asami Takasaki1)2) Hiromichi Taneichi1)2) Nami Takai1) Miku Ohashi3) and Shinichi Yagi1)4)
1)Toyama City School Novel Coronavirus Countermeasure Review Conference
2)Department of Pediatrics, University of Toyama
3)Child Life Specialist of Toyama University Hospital
4)Yagi Pediatric Clinic
Abstract
Individuals of all ages worldwide are wearing masks as a preventive measure against the COVID-19 virus during the pandemic. However, whether wearing masks should be mandatory for young children remains controversial.
We observed children playing while wearing masks at Toyama City Kindergarten and conducted a survey among their parents. The results revealed that it was difficult for children to wear masks effectively. Considering the disadvantages, we recommended that young children not wear masks, but found that many parents were concerned about this aspect. Mutual understanding was achieved through leaflets and media collaboration. We have not experienced any significant problems as of May 2021.
Although masking is a means of controlling infection, parents can undertake other practical measures, as per their child's developmental stage.




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