Saitama Children's Medical Center Research Articles

Intravenous Lacosamide Therapy for Pediatric Patients With Cluster Seizures

BackgroundFew studies have investigated intravenous lacosamide use to treat cluster seizures in pediatric patients. Therefore, we aimed to investigate the efficacy and safety of intravenous lacosamide therapy in pediatric patients with cluster seizures. MethodsWe retrospectively evaluated the efficacy and safety of intravenous lacosamide therapy in 25 pediatric patients with cluster seizures at Saitama Children's Medical Center between March 2019 and June 2023. Cluster seizures were defined as a single seizure of less than five minutes duration, repeated three or more times within 12 hours, with recovery of consciousness between seizures. Response was defined as seizure freedom for at least 12 hours after lacosamide infusion. ResultsThe median age at onset of epilepsy was 1.5 (0.0 to 9.8) years. The median seizure frequency was 5 (3 to 20) times per 12 hours. The etiologies were remote (n = 17), acute (n = 4), and progressive (n = 4). The median age at which intravenous lacosamide therapy was administered was 4.2 (0.0 to 11.3) years. The median lacosamide dose was 2.6 (1.3 to 5.2) mg/kg. In total, 12 of 25 patients (48.0%) responded. Among patients treated with intravenous lacosamide as first-line therapy, nine of 17 (52.9%) had complete seizure remission. The frequency of complete seizure remission in patients with remote etiologies was 58.8% (10 of 17); among them, seven of 12 (58.3%) patients with structural abnormalities showed complete seizure remission. No adverse events were observed. ConclusionsIntravenous lacosamide therapy is a potentially useful treatment option for cluster seizures in pediatric patients.

Long-term analysis of adrenocorticotropic hormone monotherapy for infantile epileptic spasms syndrome with periventricular leukomalacia.

Infantile epileptic spasms syndrome (IESS) with periventricular leukomalacia (PVL) has a poor neurological prognosis. Adrenocorticotropic hormone (ACTH) and vigabatrin therapies are the recommended first-line treatments for IESS. However, ACTH monotherapy for IESS with PVL has not been studied in detail. We analysed long-term outcomes of ACTH monotherapy for IESS with PVL. We retrospectively examined 12 patients with IESS and PVL at Saitama Children's Medical Center between January 1993 and September 2022. We evaluated seizure outcomes 3 months post-ACTH therapy and at the last visit. We also assessed electroencephalography findings and developmental outcomes. A positive response was defined as complete remission of epileptic spasms, no other seizure types, and hypsarrhythmia resolution post-ACTH therapy. The median onset age of epileptic spasms was 7 (range: 3-14) months. The median age at initiation of ACTH therapy was 9 (7-17) months. Seven of 12 patients (58.3%) showed a positive response. The median age at the last visit was 5 years and 6 months (1 year and 5 months-22 years and 2 months). At the last visit, only 2 of 7 initial responders remained seizure-free who demonstrated normal electroencephalography findings within 1-month post-ACTH therapy. Patients with epileptic discharge in the parieto-occipital region within 1-month post-ACTH therapy showed relapse of epileptic spasms or other seizure types. Patients having epileptic discharge in the parietal or occipital regions on electroencephalography within 1-month post-ACTH therapy may be at a high risk of epileptic spasm recurrence or other seizure types in the long term.

Efficacy of antiseizure medications in Wolf-Hirschhorn syndrome.

Wolf-Hirschhorn syndrome (WHS) is caused by deletion of the terminal region of chromosome 4 short arm and is frequently associated with intractable epilepsy. To evaluate the clinical features of epileptic seizures in WHS and the therapeutic efficacy of oral antiseizure medications (ASMs) Methods: Patients with WHS who were treated for epilepsy at the Saitama Children's Medical Center under 5 years of age were included. WHS was diagnosed based on genetic tests and clinical symptoms. Medical records regarding the age of onset of epilepsy, seizure type, treatment of status epilepticus (SE), and effectiveness of ASMs were retrospectively reviewed. Oral ASMs were considered effective when seizures were reduced by at least 50% compared to the pre-medication level. Eleven patients were included in the study. The median age at the onset of epilepsy was 9 months (range: 5-32 months). Unknown-onset bilateral tonic-clonic seizure was the most common type of seizure, occurring in ten patients. Focal clonic seizures occurred in four patients. Ten patients exhibited recurrent episodes of SE, and its frequency during infancy was monthly in eight patients and yearly in two. SE occurrence peaked at 1 year of age and decreased after 3 years of age. The most effective ASM was levetiracetam. Although WHS-associated epilepsy is intractable with frequent SE occurrence during infancy, improvement in seizure control is expected with age. Levetiracetam may be a novel ASM for WHS.

Radical surgery for congenital diaphragmatic hernia in a 23-week preterm infant.

Pediatrics InternationalVolume 64, Issue 1 e15032 Clinical Notes Radical surgery for congenital diaphragmatic hernia in a 23-week preterm infant Toshiyuki Imanishi, Corresponding Author Toshiyuki Imanishi [email protected] orcid.org/0000-0003-2667-1139 Division of Neonatology, Department of Maternal and Perinatal Center, Saitama Children's Medical Center, Saitama, Japan Correspondence: Toshiyuki Imanishi, MD, Division of Neonatology, Department of Maternal and Perinatal Center, Saitama Children's Medical Center, 1-2 Shintoshin, Chuo, Saitama 330-8777, Japan. Email: [email protected]Search for more papers by this authorJun Unemoto, Jun Unemoto Division of Neonatology, Department of Maternal and Perinatal Center, Saitama Children's Medical Center, Saitama, JapanSearch for more papers by this authorChika Kanno, Chika Kanno Division of Neonatology, Department of Maternal and Perinatal Center, Saitama Children's Medical Center, Saitama, JapanSearch for more papers by this authorMasayuki Kanno, Masayuki Kanno Division of Neonatology, Department of Maternal and Perinatal Center, Saitama Children's Medical Center, Saitama, JapanSearch for more papers by this authorMasaki Shimizu, Masaki Shimizu Division of Neonatology, Department of Maternal and Perinatal Center, Saitama Children's Medical Center, Saitama, JapanSearch for more papers by this author Toshiyuki Imanishi, Corresponding Author Toshiyuki Imanishi [email protected] orcid.org/0000-0003-2667-1139 Division of Neonatology, Department of Maternal and Perinatal Center, Saitama Children's Medical Center, Saitama, Japan Correspondence: Toshiyuki Imanishi, MD, Division of Neonatology, Department of Maternal and Perinatal Center, Saitama Children's Medical Center, 1-2 Shintoshin, Chuo, Saitama 330-8777, Japan. Email: [email protected]Search for more papers by this authorJun Unemoto, Jun Unemoto Division of Neonatology, Department of Maternal and Perinatal Center, Saitama Children's Medical Center, Saitama, JapanSearch for more papers by this authorChika Kanno, Chika Kanno Division of Neonatology, Department of Maternal and Perinatal Center, Saitama Children's Medical Center, Saitama, JapanSearch for more papers by this authorMasayuki Kanno, Masayuki Kanno Division of Neonatology, Department of Maternal and Perinatal Center, Saitama Children's Medical Center, Saitama, JapanSearch for more papers by this authorMasaki Shimizu, Masaki Shimizu Division of Neonatology, Department of Maternal and Perinatal Center, Saitama Children's Medical Center, Saitama, JapanSearch for more papers by this author First published: 28 April 2022 https://doi.org/10.1111/ped.15032Read the full textAboutPDF ToolsRequest permissionExport citationAdd to favoritesTrack citation ShareShare Give accessShare full text accessShare full-text accessPlease review our Terms and Conditions of Use and check box below to share full-text version of article.I have read and accept the Wiley Online Library Terms and Conditions of UseShareable LinkUse the link below to share a full-text version of this article with your friends and colleagues. Learn more.Copy URL No abstract is available for this article. Volume64, Issue1January/December 2022e15032 RelatedInformation

Coronavirus disease 2019-associated multisystem inflammatory syndrome and Kawasaki disease.

Pediatrics InternationalAccepted Articles e15349 Clinical NoteFree Access COVID-19-associated multisystem inflammatory syndrome and Kawasaki disease Yoji Uejima, Corresponding Author Yoji Uejima uejima.yoji@saitama-pho.jp orcid.org/0000-0002-0755-7560 Division of Infectious Diseases and Immunology, Saitama Children’s Medical Center, Saitama, Japan Department of Clinical Laboratory and Molecular Pathology, Faculty of Medicine, Academic Assembly, University of Toyama, Toyama, Japan Correspondence Yoji Uejima, MD, Division of Infectious Diseases and Immunology, Saitama Children's Medical Center, 1–2 Shintoshin, Chuou-ku, Saitama 330-8777, Japan, Tel: +81- 48-601-2200; Fax: +81-48-601-2201. Email: uejima.yoji@saitama-pho.jpSearch for more papers by this authorEisuke Suganuma, Eisuke Suganuma Division of Infectious Diseases and Immunology, Saitama Children’s Medical Center, Saitama, JapanSearch for more papers by this authorKaede Matsunaga, Kaede Matsunaga Department of Clinical Laboratory and Molecular Pathology, Faculty of Medicine, Academic Assembly, University of Toyama, Toyama, JapanSearch for more papers by this authorIsao Kitajima, Isao Kitajima Department of Clinical Laboratory and Molecular Pathology, Faculty of Medicine, Academic Assembly, University of Toyama, Toyama, JapanSearch for more papers by this authorHideki Niimi, Hideki Niimi Department of Clinical Laboratory and Molecular Pathology, Faculty of Medicine, Academic Assembly, University of Toyama, Toyama, JapanSearch for more papers by this author Yoji Uejima, Corresponding Author Yoji Uejima uejima.yoji@saitama-pho.jp orcid.org/0000-0002-0755-7560 Division of Infectious Diseases and Immunology, Saitama Children’s Medical Center, Saitama, Japan Department of Clinical Laboratory and Molecular Pathology, Faculty of Medicine, Academic Assembly, University of Toyama, Toyama, Japan Correspondence Yoji Uejima, MD, Division of Infectious Diseases and Immunology, Saitama Children's Medical Center, 1–2 Shintoshin, Chuou-ku, Saitama 330-8777, Japan, Tel: +81- 48-601-2200; Fax: +81-48-601-2201. Email: uejima.yoji@saitama-pho.jpSearch for more papers by this authorEisuke Suganuma, Eisuke Suganuma Division of Infectious Diseases and Immunology, Saitama Children’s Medical Center, Saitama, JapanSearch for more papers by this authorKaede Matsunaga, Kaede Matsunaga Department of Clinical Laboratory and Molecular Pathology, Faculty of Medicine, Academic Assembly, University of Toyama, Toyama, JapanSearch for more papers by this authorIsao Kitajima, Isao Kitajima Department of Clinical Laboratory and Molecular Pathology, Faculty of Medicine, Academic Assembly, University of Toyama, Toyama, JapanSearch for more papers by this authorHideki Niimi, Hideki Niimi Department of Clinical Laboratory and Molecular Pathology, Faculty of Medicine, Academic Assembly, University of Toyama, Toyama, JapanSearch for more papers by this author First published: 01 September 2022 https://doi.org/10.1111/ped.15349 This article has been accepted for publication and undergone full peer review but has not been through the copyediting, typesetting, pagination and proofreading process, which may lead to differences between this version and the Version of Record. Please cite this article as doi:10.1111/ped.15349 AboutPDF ToolsExport citationAdd to favoritesTrack citation ShareShare Give accessShare full text accessShare full-text accessPlease review our Terms and Conditions of Use and check box below to share full-text version of article.I have read and accept the Wiley Online Library Terms and Conditions of UseShareable LinkUse the link below to share a full-text version of this article with your friends and colleagues. Learn more.Copy URL Share a linkShare onFacebookTwitterLinked InRedditWechat Accepted ArticlesAccepted, unedited articles published online and citable. The final edited and typeset version of record will appear in the future.e15349 RelatedInformation

Periodic fever, aphthous stomatitis, pharyngitis, and cervical adenitis syndrome: Clinical characteristics and treatment outcomes - a single center study in Japan.

Periodic fever, aphthous stomatitis, pharyngitis, and cervical adenitis (PFAPA) syndrome is an autoinflammatory disease occurring in children. Although PFAPA is the most common periodic fever syndrome found in children, there are only a few studies defining the clinical characteristics and the efficacy of treatment strategies among Japanese children. This study aimed to clarify the demographic characteristics and clinical features of patients with PFAPA syndrome and to evaluate treatment efficacy. We retrospectively reviewed the clinical features of children with PFAPA who visited Saitama Children's Medical Center between January and December 2019. We also evaluated treatment strategies and their efficacy; abortive treatment with corticosteroids, prophylaxis with cimetidine or colchicine, and surgical management with tonsillectomy. A total of 100 Japanese children (61% male) with PFAPA were included. Median age of onset was 3 years, median duration of fever episodes was 5 days, and median interval between episodes was 4 weeks. The symptoms (frequencies) were pharyngitis (89%), exudate on tonsils (71%), cervical adenitis (50%), and aphthous stomatitis (49%). Approximately 37% of patients took prednisolone for aborting fever attacks, showing a 100% response; 93% were treated with cimetidine, showing an 79.6% response, and 18% were treated with colchicine, showing a 66.7% response. Only one patient underwent tonsillectomy. Among Japanese children with PFAPA, 28% of them were ≥5 years with a male predominance. Pharyngitis is the most frequent symptom associated with fever. Cimetidine is suitable for initial therapy because of its safety and efficacy.

Adjunctive perampanel therapy for patients with epileptic spasms

Perampanel is an antiepileptic drug. Some studies have documented the efficacy of perampanel in epileptic spasms. We aimed to evaluate the efficacy and safety of adjunctive perampanel therapy (PT) in patients with epileptic spasms. We retrospectively surveyed the efficacy and safety of adjunctive PT in 14 patients with epileptic spasms at the Saitama Children's Medical Center between June 2016 and September 2021. Seizure outcomes and safety were evaluated 12months after commencing PT. Response to perampanel was defined as complete remission of epileptic spasms for more than 3 months. The median age at onset of epileptic spasms was 0.4 years (range, 0.1-1.3 years). The etiology was structural in 11 patients, genetic in two, and unknown in one. The median age at the commencement of PT was 3.2 years (1.5-10.3 years). The initial and maintenance doses of perampanel were administered at 0.04 (range, 0.02-0.05) mg/kg/day and 0.12 (range, 0.03-0.24) mg/kg/day, respectively. Five of the 14 patients (35.7%) showed remission of epileptic spasms for more than 3 months at 12months after PT; these patients had a structural etiology. The median duration between commencement of perampanel and spasm remission was 2 months (range, 1-6 months). No serious adverse effects occurred. This is the first case series evaluating adjunctive PT for epileptic spasms. PT is worth investigating to treat epileptic spasms in patients with structural etiologies. As our study population primarily comprised children aged 2 years and older, PT may be useful for epileptic spasms beyond infancy.

Clostridioides difficile Infection in a Japanese Tertiary Children's Hospital.

PurposeToxins produced by Clostridioides difficile infection (CDI) can cause enteritis and diarrhea. Although the number of pediatric CDI cases is increasing, the clinical management of pediatric CDI, including patient characteristics and prognosis, remains unclear. This study aimed to elucidate the background and clinical course of patients with CDI and evaluate the reliability of diagnostic tests in a tertiary pediatric hospital in Japan.MethodsWe retrospectively analyzed the clinical data of children diagnosed with CDI between 2011 and 2021 at the Saitama Children’s Medical Center in Saitama, Japan.ResultsDuring the study period, 1,252 C. difficile antigen/toxin tests were performed, and 37 patients were diagnosed with CDI. The main underlying diseases among the patients were hematological and malignant disorders and gastrointestinal diseases, including inflammatory bowel disease (IBD) (59.4%). Two patients (5.4%) had an unremarkable medical history. Among the 37 patients, 27 (73.0%) were immunocompromised, 25 (67.6%) had a history of antibiotic use within the past two months, and 6 (16.2%) were negative on the initial test but were positive on the second test. Finally, 28 patients (75.7%) required primary antibiotic therapy only, and two patients with IBD required additional antibiotic therapy as secondary treatment.ConclusionThe number of pediatric patients with CDI is increasing. Both a comprehensive interview, including underlying diseases and history of antibiotic use, and an understanding of the features of clinical examinations should be emphasized to appropriately diagnose and treat CDI.

Telemedicine in epilepsy management during the coronavirus disease 2019 pandemic.

BackgroundTelemedicine has spread rapidly during the coronavirus disease 2019 (COVID‐19) pandemic and shown its usefulness, particularly for patients with epilepsy, compared to face‐to‐face visits. We sought to evaluate the clinical features of patients with childhood onset epilepsy associated with consultations by telephone call during the COVID‐19 pandemic.MethodsWe retrospectively investigated the medical records of patients with childhood onset epilepsy who visited an outpatient clinic in Saitama Children's Medical Center, Saitama, Japan, from 1 March 2020 to 30 September 2020. To find the clinical features of patients who utilized telemedicine consultation (by telephone call), we divided the patients into the telemedicine group and the face‐to‐face group. We then reviewed the clinical features. Telemedicine consultation was not implemented for new patients.ResultsWe enrolled 776 outpatients in total, and 294 patients (37.9%) utilized telemedicine consultations. The total number of visits was 2,299 and the total number of telemedicine consultations was 373 (16.2%). No clinical feature was associated with telemedicine consultations except for age at onset of epilepsy. The number of oral antiepileptic drugs prescriptions decreased in 23 of 776 (3.0%) of the patients who did not experience seizure deterioration, including status epilepticus, or who visited the emergency room.ConclusionTelemedicine consultations were successfully utilized for epilepsy treatment at our outpatient clinic, regardless of epilepsy type, etiology, seizure frequency, comorbidities, and patients' residential areas. Thus, telemedicine by telephone call may be a useful resource in the management of patients with childhood onset epilepsy during the pandemic.

Efficacy, safety, and pharmacokinetics of oral valganciclovir in patients with congenital cytomegalovirus infection

ObjectivesValganciclovir (VGCV) has been shown to improve sensorineural hearing loss (SNHL) and neurological outcomes in patients with neonatal symptomatic congenital cytomegalovirus (cCMV) infection. However, reports on the pharmacokinetics, efficacy and safety of oral VGCV are limited. The aim of this study is to evaluate the pharmacokinetics of VGCV for use in the treatment of cCMV. MethodsThis was a single-center, retrospective observational study conducted at Saitama Children's Medical Center in Japan between 2012 and 2017. CMV DNA copy number, maximum plasma VGCV concentration (Cmax), and adverse events (ADEs) during treatment were evaluated. ResultsA total of 26 patients with cCMV who received VGCV were included in this study. The median age at VGCV initiation was 9.5 months (range 0–46). Twenty-one patients (81%) had SNHL at baseline. Of these, five patients (19%) presented with improved SNHL, and none experienced worsened SNHL during treatment. The mean VGCV Cmax was 3.5 μg/mL (range 2–5.3), with no significant variation among individual values, and the values were maintained during treatment. Furthermore, there were no correlations between the Cmax values and age, sex, SNHL improvement or ADEs. Neutropenia (<1000/mm3) was observed in six patients (23%); however, no serious ADEs occurred. ConclusionsVGCV prevented the progression of SNHL without serious ADEs due to its stable pharmacokinetics. This study provides safety and tolerability of VGCV for the treatment of cCMV patients.

Difference between early onset and late-onset pediatric ulcerative colitis.

Early onset pediatric ulcerative colitis (EO-UC) is distinguished from late-onset pediatric ulcerative colitis (LO-UC) by the effects of genetic predisposition, but there have been few reports on the clinical features of EO-UC in Asia. To describe and compare the presentation and disease course of EO-UC (age range, 0-7 years) with those of LO-UC (age range, 8-15 years), we retrospectively analyzed 63 children with UC who had been diagnosed between January 2004 and March 2014 at Saitama Children's Medical Center in Japan. Ten patients (16%) had EO-UC, and 53 (84%) had LO-UC. All patients in the EO-UC group and 70% in the LO-UC group had pancolitis (P = 0.05). The period from onset to diagnosis was 9.0 ± 14.1 months for EO-UC and 2.6 ± 3.5 months for LO-UC (P < 0.01). The prevalence of extra-intestinal complications at diagnosis was significantly higher for EO-UC than for LO-UC (50% vs 11%, respectively; P < 0.01). There were no significant differences in the use of corticosteroids, immunomodulators, immunosuppressants, or surgical risk between the groups but, in the EO-UC group, only one patient was treated with cytapheresis and none was treated with anti-tumor necrosis factor (TNF-α) antibodies. The EO-UC group had a higher incidence of pancolitis, longer diagnostic delay, and more extra-intestinal manifestations than the LO-UC group. Diagnosis and treatment may therefore be slightly more difficult for EO-UC than for LO-UC.

Pediatric Surgery.

Asian Journal of Endoscopic SurgeryVolume 8, Issue 4 p. 390-407 JSES Guideline Pediatric Surgery Tadashi Iwanaka, Corresponding Author Tadashi Iwanaka Correspondence Tadashi Iwanaka (Saitama Children's Medical Center). Tel: +81-48-758-1811 Fax: +81-48-758-1818 Email: iwanaka.tadashi@pref.saitama.lg.jpSearch for more papers by this authorAtsuyuki Yamataka, Atsuyuki YamatakaSearch for more papers by this authorSadashige Uemura, Sadashige UemuraSearch for more papers by this authorHiroomi Okuyama, Hiroomi OkuyamaSearch for more papers by this authorOsamu Segawa, Osamu SegawaSearch for more papers by this authorMasaki Nio, Masaki NioSearch for more papers by this authorJoji Yoshizawa, Joji YoshizawaSearch for more papers by this authorMakoto Yagi, Makoto YagiSearch for more papers by this authorSatoshi Ieiri, Satoshi IeiriSearch for more papers by this authorHiroo Uchida, Hiroo UchidaSearch for more papers by this authorHiroyuki Koga, Hiroyuki KogaSearch for more papers by this authorMasahito Sato, Masahito SatoSearch for more papers by this authorHideki Soh, Hideki SohSearch for more papers by this authorHiroshi Take, Hiroshi TakeSearch for more papers by this authorRyuichiro Hirose, Ryuichiro HiroseSearch for more papers by this authorHiroaki Fukuzawa, Hiroaki FukuzawaSearch for more papers by this authorMasaru Mizuno, Masaru MizunoSearch for more papers by this authorToshihiko Watanabe, Toshihiko WatanabeSearch for more papers by this author Tadashi Iwanaka, Corresponding Author Tadashi Iwanaka Correspondence Tadashi Iwanaka (Saitama Children's Medical Center). Tel: +81-48-758-1811 Fax: +81-48-758-1818 Email: iwanaka.tadashi@pref.saitama.lg.jpSearch for more papers by this authorAtsuyuki Yamataka, Atsuyuki YamatakaSearch for more papers by this authorSadashige Uemura, Sadashige UemuraSearch for more papers by this authorHiroomi Okuyama, Hiroomi OkuyamaSearch for more papers by this authorOsamu Segawa, Osamu SegawaSearch for more papers by this authorMasaki Nio, Masaki NioSearch for more papers by this authorJoji Yoshizawa, Joji YoshizawaSearch for more papers by this authorMakoto Yagi, Makoto YagiSearch for more papers by this authorSatoshi Ieiri, Satoshi IeiriSearch for more papers by this authorHiroo Uchida, Hiroo UchidaSearch for more papers by this authorHiroyuki Koga, Hiroyuki KogaSearch for more papers by this authorMasahito Sato, Masahito SatoSearch for more papers by this authorHideki Soh, Hideki SohSearch for more papers by this authorHiroshi Take, Hiroshi TakeSearch for more papers by this authorRyuichiro Hirose, Ryuichiro HiroseSearch for more papers by this authorHiroaki Fukuzawa, Hiroaki FukuzawaSearch for more papers by this authorMasaru Mizuno, Masaru MizunoSearch for more papers by this authorToshihiko Watanabe, Toshihiko WatanabeSearch for more papers by this author First published: 28 December 2015 https://doi.org/10.1111/ases.12263Citations: 2Read the full textAboutPDF ToolsRequest permissionExport citationAdd to favoritesTrack citation ShareShare Give accessShare full text accessShare full-text accessPlease review our Terms and Conditions of Use and check box below to share full-text version of article.I have read and accept the Wiley Online Library Terms and Conditions of UseShareable LinkUse the link below to share a full-text version of this article with your friends and colleagues. Learn more.Copy URL Share a linkShare onFacebookTwitterLinkedInRedditWechat Citing Literature Volume8, Issue4November 2015Pages 390-407 RelatedInformation

Psychosocial Responses to being Identified as a Balanced Chromosomal Translocation Carrier: a Qualitative Investigation of Parents in Japan.

Undergoing chromosome analysis and receiving the results may have various psychosocial effects. To identify the impact on balanced translocation carriers identified through affected offspring, we conducted semi-structured interviews with eleven parents at Saitama Children's Medical Center. The results of the interviews were analyzed qualitatively by the KJ (Kawakita Jiro) method. Categories and subcategories of the various thoughts, emotions and responses experienced by balanced chromosomal translocation carriers were extracted. Participants' reactions were mixed, and appeared to be interrelated in some cases. Parents' reactions were sometimes ambivalent with regard to effects on reproductive issues and disclosure of test results. We recommend genetic counseling before and after carrier testing to help parents cope with the mixed and complex thoughts and feelings that arise upon being identified as a carrier.

Epilepsy and West syndrome in neonates with hypoxic–ischemic encephalopathy

Perinatal hypoxic-ischemic encephalopathy (HIE) has been linked to the development of late-onset seizures. The aim of the present study was to determine the incidence of epilepsy and West syndrome in children with perinatal HIE and identify factors associated with the development of postnatal seizure disorders. We retrospectively enrolled 208 term and late-preterm infants diagnosed with perinatal HIE from April 2000 to March 2009 at Saitama Children's Medical Center. Children with obvious multiple anomalies and known chromosomal abnormalities were excluded. A questionnaire was distributed to parents to determine seizure-related outcomes. Medical records were retrospectively reviewed and relevant clinical parameters were analyzed. In total, 162 questionnaires were answered (77.9%). Of the 162 subjects, 26 (16.0%) developed epilepsy, and eight subjects (4.9%) were diagnosed with West syndrome. Neonatal seizures occurred in 72 subjects (44.4%). The incidence of epilepsy and West syndrome was significantly higher in infants who experienced neonatal seizures than in those without seizure history. A total of 82 subjects were diagnosed with moderate (n = 52) or severe HIE (n = 30), of whom 57 subjects (69.5%) received therapeutic hypothermia. The incidence of epilepsy was significantly lower in these treated subjects. In addition, subjects with moderate or severe HIE were significantly more likely to develop late-onset epilepsy and West syndrome than those with mild HIE. The severity of perinatal HIE and neonatal seizures is a potential risk factor for the development of late-onset seizures. Therapeutic hypothermia may reduce the risk of the development of epilepsy in such cases.

埼玉県立小児医療センターにおける 診療時間外の耳鼻咽喉科救急疾患 看護師による電話相談の効果

埼玉県立小児医療センターにおける 診療時間外の耳鼻咽喉科救急疾患 看護師による電話相談の効果

Nephrotoxicity of once-daily cyclosporine A in minimal change nephrotic syndrome

Although once-daily cyclosporine (CsA) therapy may have greater nephrotoxic-sparing effects than standard twice-daily therapy, little information is available in children with steroid-dependent minimal change nephrotic syndrome (MCNS) regarding histological analysis after long-term once-daily administration. A prospective study of the clinical efficacy and comparison between pre- and post-treatment renal biopsy findings in ten children (mean age, 8.8 years) with steroid-dependent MCNS who were administered once-daily CsA therapy for more than 24 months (mean ± SD, 30 ± 3.7) was performed in Saitama Children's Medical Center. Administration of once-daily CsA therapy (mean dose, 2.8 ± 0.6 mg/kg/day; mean C2 levels, 670 ± 64 ng/ml) resulted in a significant reduction in the median relapse rate from 4.6 to 0.2 times per year, and five patients did not experience a relapse of NS. Furthermore, mean threshold of prednisolone dose significantly reduced from 1.2 to 0.02 mg/kg on alternate days. However, two patients showed evidence of chronic CsA nephrotoxicity (CsAN). Once-daily CsA therapy appears to be effective in children with steroid-dependent MCNS. However, follow-up renal biopsies should be performed to investigate the presence of CsAN after more than 24 months of treatment with once-daily regimen as well as with the conventional twice-daily regimen.

Risk Factors for Hearing Loss after Pediatric Meningitis in Japan

We sought to identify predictors for hearing loss in Japanese children with meningitis. We analyzed 155 cases of pediatric meningitis without other entities causing hearing loss in children admitted to Saitama Children's Medical Center between 1990 and 2005 for potential risk factors for hearing loss, using multiple logistic regression. Auditory brain stem response tests were performed to evaluate hearing loss. Of 155 children, 35 (23%) developed hearing loss (21 unilaterally and 14 bilaterally). Profound hearing loss (greater than 90 dB normal hearing level) occurred in 15 patients (9.7%; 4 unilaterally and 11 bilaterally). Of 112 patients with positive cerebrospinal fluid cultures, 27 (24%) developed hearing loss and 13 (12%) showed profound loss. Of 22 patients with Streptococcus pneumoniae meningitis, 11 (50%) developed hearing loss and 7 (32%) showed profound loss. Of 54 patients with Haemophilus influenzae meningitis, 11 (20%) developed hearing loss and 4 (7.4%) showed profound loss. High serum C-reactive protein levels and cerebrospinal fluid cultures positive for Streptococcus pneumoniae were identified as significant risk factors for hearing loss. A high serum C-reactive protein level was first identified as a risk factor for hearing impairment after pediatric meningitis.

Juvenile dermatomyositis: clinical characteristics and the relatively high risk of interstitial lung disease

To clarify the clinical features of juvenile dermatomyositis (JDM) in Japanese cases, we retrospectively evaluated the characteristics of 16 children with JDM that were treated at Saitama Children's Medical Center between 1985 and 2004. The age at disease onset ranged from 3.5 to 14.1 years old (7 boys, mean age 7.9 years; 9 girls, mean age 9.2 years). In 14 patients more than two muscle enzymes were elevated at diagnosis. The antinuclear antibody at diagnosis was positive in all girls but one, while it was positive in only two boys (2/7; P<0.01). Three patients were complicated with interstitial lung disease (ILD) (18.8%) and their serum KL-6 levels were already elevated on admission. Our findings suggest that serum KL-6 levels seemed to be sensitive to the detection of ILD in an early phase, and the relatively high frequency of JDM-associated ILD indicated that a careful evaluation of the lungs was therefore required in any individuals with JDM. Of 16 patients, two boys showed a favorable improvement and prognosis without relapse for over 9 years after the termination of treatment. Overall, in girls, there is a tendency to be a delay in the diagnosis/treatment for JDM, and this disease also demonstrated a severe course.

The laparoscopic approach of neuroblastoma

Neuroblastoma is one of the most common solid tumors, and the biopsy and excision of this tumor are often required as part of a planned multimodal treatment. In 1995, Holcomb and coworkers first reported endosurgical procedures for the diagnosis and treatment of pediatric malignancies; however, the usefulness of laparoscopic procedures for abdominal neuroblastoma is still unclear. Twenty-five laparoscopic biopsies for advanced abdominal neuroblastoma and nine laparoscopic excisions for localized abdominal neuroblastoma performed at Saitama Children's Medical Center were evaluated. The laparoscopic procedures significantly reduce the time to start postoperative feeding as well as the time to start postoperative chemotherapy and the duration of hospital stay. Also, the blood loss of laparoscopic excision of localized neuroblastoma was significantly little compared with that of open excision. Precise indications of laparoscopic procedure for the diagnosis and treatment of abdominal neuroblastoma provide better prognosis and quality of life for infants and children.

Juvenile dermatomyositis: clinical characteristics and the relatively high risk of interstitial lung disease

To clarify the clinical features of juvenile dermatomyositis (JDM) in Japanese cases, we retrospectively evaluated the characteristics of 16 children with JDM that were treated at Saitama Children's Medical Center between 1985 and 2004. The age at disease onset ranged from 3.5 to 14.1 years old (7 boys, mean age 7.9 years; 9 girls, mean age 9.2 years). In 14 patients more than two muscle enzymes were elevated at diagnosis. The antinuclear antibody at diagnosis was positive in all girls but one, while it was positive in only two boys (2/7; P < 0.01). Three patients were complicated with interstitial lung disease (ILD) (18.8%) and their serum KL-6 levels were already elevated on admission. Our findings suggest that serum KL-6 levels seemed to be sensitive to the detection of ILD in an early phase, and the relatively high frequency of JDM-associated ILD indicated that a careful evaluation of the lungs was therefore required in any individuals with JDM. Of 16 patients, two boys showed a favorable improvement and prognosis without relapse for over 9 years after the termination of treatment. Overall, in girls, there is a tendency to be a delay in the diagnosis/treatment for JDM, and this disease also demonstrated a severe course.