Pediatric Otorhinolaryngology Japan
Online ISSN : 2186-5957
Print ISSN : 0919-5858
ISSN-L : 0919-5858
Volume 37, Issue 3
Displaying 1-22 of 22 articles from this issue
Editorial
The 11th Conference on Pediatric Otorhinolaryngology Japan
Special Lecture
  • Yukuo Konishi
    Article type: Special Lecture
    2016 Volume 37 Issue 3 Pages 231-235
    Published: 2016
    Released on J-STAGE: March 23, 2017
    JOURNAL FREE ACCESS
     Autism spectrum disorders (ASDs) are complex, heterogeneous, and multifactorial disorders characterized by impaired social communication and repetitive stereotyped behaviors. However, there is growing interest in the role of biological and behavior rhythms such as cellular synchrony and synchrony of motor, emotional, and interpersonal rhythms in ASDs. Even in the neonatal period, sleep difficulties are frequently associated with ASD, and sleep difficulties might be one of the earliest symptoms of ASD. The role of melatonin in the ontogeny of circadian rhythms might be also involved in the synchrony of motor and interpersonal rhythms. These results suggest new hypotheses for the mechanisms of impairments of melatonin synthesis and provide a rationale for melatonin therapy in ASDs.
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Symposium 1—The role of otorhinolaryngologists in school health
Symposium 2—Treatment for children with swallowing disorders
Educational seminar 1
Educational seminar 2
Morning seminar
Luncheon seminar 1
Luncheon seminar 3
Clinical seminar
  • Hiroshi Kido
    Article type: Clinical seminar
    2016 Volume 37 Issue 3 Pages 305-311
    Published: 2016
    Released on J-STAGE: March 23, 2017
    JOURNAL FREE ACCESS
     Influenza A virus (IAV) is one of the most common infectious pathogens in humans, and it can cause significant morbidity and mortality in infants and the elderly with underlying diseases. The knowledge gained within the last decade in the IAV (H1N1) pandemic of 2009 and the highly pathogenic avian influenza outbreak improved our understanding not only of the viral pathogenicity but also the host cellular factors involved in the pathogenicity of multiple organ failure, such as cellular viral hemagglutinin processing proteases for viral multiplication, cytokine storm, metabolic disorders, and energy crises. Recently, a new concept of the pathogenicity of multiple organ failure has been proposed, the “influenza virus-cytokine-trypsin” cycle, involving up-regulation of ectopic pancreatic trypsin through pro-inflammatory cytokines, and potentiation of viral multiplication in various organs. We also found that the influenza virus-cytokine-trypsin cycle interrelated with the “metabolic disorders-cytokine” cycle, which is one of the principal mechanisms of the pathogenicity of multiple organ failure. These cycles provide new concepts for treatment of metabolic disorders and multiple organ failure, apart from antiviral neuraminidase inhibitors. Furthermore, we found that clarithromycin, an immunomodulating macrolide antibiotic, enhances mucosal and systemic immune responses and reduces the reinfection rate in pediatric patients with influenza treated with antiviral neuraminidase inhibitors. This review discusses IAV pathogenicity and available therapeutic options including immunomodulatory clarithromycin for prevention of IAV reinfection.
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Seminar with sweets
Original Articles
  • Takashi Ishino, Toru Sonoyama, Sachio Takeno, Katsuhiro Hirakawa
    Article type: Original Article
    2016 Volume 37 Issue 3 Pages 318-323
    Published: 2016
    Released on J-STAGE: March 23, 2017
    JOURNAL FREE ACCESS
     Enlarged Vestibular Aqueduct Syndrome (EVA) is an autosomal recessive deafness characterized by inner ear malformations. Pendred syndrome (PS) is an associated syndrome of EVA with goitrous change in the thyroid gland. Mutations in SLC26A4, which encodes the pendrin protein, are common causes of EVA and PS. These syndromes follow an autosomal recessive pattern of inheritance. Recently, a few studies have reported another type of inheritance for these syndromes.
     Here, we report a heterozygous SLC26A4 c.919–2A>G (IVS7–2A>G) mutation in a patient with EVA. The patient presented with enlarged vestibular aqueduct in the CT scan and severe hearing loss in the auditory brainstem response (ABR) test, auditory steady-state response (ASSR) test, and conditioned orientation response audiometry (COR). Both, the genetic screening test for congenital hearing loss and next-generation sequencing (NGS) revealed only the presence of the heterozygous SLC26A4 c.919–2A>G (IVS7–2A>G) mutation. Mutations in other genes associated with both EVA and PS (FOXI1, KCNJ10, SIX1, ATP6V1B1, ATP6V0A4, and POU3F4) and genes associated with hearing loss (GJB2, OTOF, and mitochondrial rRNA) were not detected by NGS under ClinVar at NCBI. Direct sequencing revealed that only the mother carried the heterozygous SLC26A4 c.919–2A>G (IVS7–2A>G) mutation. A family pedigree analysis showed that the inheritance of hearing loss followed a non-autosomal recessive pattern that was dissimilar to the autosomal recessive pattern. The cause of hearing loss should differ between the patient and his mother because of the difference in their hearing pattern, despite no clear rejection of the mother's EVA by CT scan.
     Most studies have reported an autosomal recessive inheritance pattern for EVA and PS, but a few studies have showed that digenic inheritance of double-heterozygous mutations induced another pattern of inheritance in the presence of SLC26A4 mutations, as seen in this family. Thus, we cannot reject the possibility of inheritance induced by digenic double-heterozygous mutations along with heterozygous SLC26A4 mutations or together with a mutation in the promoter region of SLC26A4. However, the presence of the other accompanying mutation was not detected in our study. Therefore, we should consider genetic counseling with a medical geneticist if patients with EVA or PS show only heterozygous SLC26A4 mutations.
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  • Takashi Nasu, Makoto Kano, Seiji Kakehata
    Article type: Original Article
    2016 Volume 37 Issue 3 Pages 324-329
    Published: 2016
    Released on J-STAGE: March 23, 2017
    JOURNAL FREE ACCESS
     Purpose: Surgical closure of the larynx with removing of cricoid cartilage in infants that have severe motor and intellectual disabilities or belong to medical care-dependent groups (SMID–MCDG), who have immature larynxes is considered to be difficult because of the small size of the larynx in such patients. The aim of the present study was to analyze the cases of children with SMID–MCDG who underwent surgical closure of the larynx with removing of cricoid cartilage to clarify whether this operation is an appropriate technique for preventing aspiration in such patients.
     Methods: The medical records of five children under the age of seven with SMID–MCDG who underwent surgical closure of the larynx with removing of cricoid cartilage at Yamagata University Hospital were retrospectively reviewed and compared with those of five children over the age of seven with SMID–MCDG (the control group) who underwent the same procedure. The following clinical details were reviewed: sex, age, body height and weight, operative time, the amount of intraoperative bleeding, postoperative complications, the number of days spent in hospital, the number of tracheal cannula-free children, the number of children that required home health management, and outcomes.
     Results: The operative time of the five children with SMID–MCDG was not significantly longer than that of the control group. Similarly, no significant differences in the amount of intraoperative bleeding, the frequency of postoperative complications, or the number of days spent in hospital were detected between the two groups. The number of tracheal cannula-free children and the number of children that required home health management were the same in both groups.
     Conclusion: Surgical closure of the larynx with removing of cricoid cartilage is an appropriate method for preventing aspiration in children under the age of seven with SMID–MCDG.
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  • Nana Akagi Tsuchihashi, Noriomi Suzuki, Mariko Hara, Keisuke Yoshihama ...
    Article type: Original Article
    2016 Volume 37 Issue 3 Pages 330-335
    Published: 2016
    Released on J-STAGE: March 23, 2017
    JOURNAL FREE ACCESS
     Postcricoid cushion is a mass which is observed at postcricoid lesion of infants. This mass becomes obvious when they cry because of the dilation of venous flux of postcricoid lesion. It can be the cause of suckling or respiration disorder as a result of laryngeal stenosis which comes from venous pressure of the mass. The epidemiology and pathophysiology of postcricoid cushion has not been fully known because many cases are without any symptoms, and diagnosed as hemangioma. We report seven cases of postcricoid cushion. Its main symptoms are suckling and respiratory disorders accompanying secondary laryngeal stenosis in young children, and elder ones did not have apparent symptoms. Enhanced CT scan and MRI did not reveal masses under sedation. In five in seven cases, masses became smaller in size as they grew up, except for a case with achondroplasia which needed tracheotomy. Therefore, it is important to consider the treatment courses of postcricoid cushion.
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