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THE JOURNAL OF THE JAPAN PEDIATRIC SOCIETY
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Vol.122, No.1, January 2018
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Original Article
Title
Problems in Individuals with Severe Motor and Intellectual Disabilities (SMID) Requiring Additional Medical Treatment during a Short Stay at Our Institute
Author
Natsuko Arai1)2) Kyoko Kashii1) Yoshitaka Kim1) Kounosuke Nakada1) Bunsei Egawa1) Noriko Udagawa2) and Hitoshi Yamamoto2)
1)Medical and Educational Institute for Neurologically Impaired Persons, Soleil Kawasaki
2)Department of Pediatrics, St. Marianna University School of Medicine
Abstract
Purpose: We investigated the status of short stay services at our institute for those requiring additional medical treatment to consider the safety of medical management.
Materials and Methods: Between April 2012 and December 2016, out of 624 cases, 169 individuals with SMID registered for a short stay. Additional medical treatment was required in 16 cases.
Results: We noted a rapid increase annually especially in the ratio between the SMID-medical care dependent group (MCDG) and sub-SMID-MCDG. Most patients requiring additional medical treatment were children from SMID-MCDG and sub-SMID-MCDG, particularly those needing treatment during their first stay. Most patients required treatment for respiratory diseases, and 70% of hospitalized patients needed a longer-than-one-week stay.
Conclusions: We have to make efforts to develop greater practical skills for their clinical management. Effective communication between their caregivers and doctors is imperative, as well as monitoring patients showing changes in their physical conditions. Attention must be focused on the high-risk group - "Children", "SMID-MCDG" and "First time users of the service". Establishing a community-based cooperative hospital model would be useful.
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Original Article
Title
Dramatic Increase in the Incidence of Upper Urinary Tract Infection Caused by Extended-spectrum Beta-lactamase-producing Escherichia Coli in Children in Izumo City
Author
Akiyoshi Horie1) Daisuke Koike1) Tomohiro Hirade1) Kae Suemitsu1) Akiyoshi Nariai1) Ritsuko Kitamura2) and Fumihide Katou2)
1)Divisions of Pediatrics, Shimane Prefectural Central Hospital
2)Department of Neonatology, Shimane Prefectural Central Hospital
Abstract
At our hospital, the number of hospitalized patients in the pediatric department because of upper urinary tract infection (UUTI) dramatically increased in 2016. In the first contraction of urinary tract infection, 68% of cases were UUTI caused by extended-spectrum beta-lactamase (ESBL)-producing Escherichia coli.
In UUTI caused by ESBL-producing E. coli, fever is alleviated 50.78±37.78 hours after treatment with cefotaxime sodium or ceftriaxone disodium, which is significantly longer than that for fever in UUTI caused by ESBL-non-producing E. coli. We analyzed the antibiotic susceptibilities of ESBL-producing E. coli in our hospital and changed the primary antibiotics to flomoxef (FMOX) for treatment of UUTI caused by gram-negative rod bacilli. After initiation of FMOX treatment, the time to alleviation of fever significantly shortened to 18.78±14.62 hours.
Among the O antigens of ESBL-producing E. coli isolated in 2014 to 2016, we found O1, O6, and O25 antigens; E. coli with these antigens are considered uropathogenic. In particular, the ratio of ESBL-producing E. coli, which has the O6 antigen, significantly increased.
These data show that ESBL-producing E. coli is the main cause of community-acquired UUTI in children. Even in the first contraction of UUTI in children, ESBL-producing E. coli should be suspected and the choice of antibiotics should be based on local antibiotic susceptibilities.
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Original Article
Title
Knowledge, Attitude, and Practices in Injury Prevention among Japanese Pediatricians
Author
Shun Kishibe1) Yoshihiko Morikawa2)3) Masako Tomotsune2) and Nobuaki Inoue1)4)
1)Department of Pediatric Emergency and Critical Care Medicine, Tokyo Metropolitan Children's Medical Center
2)Clinical Research Support Center, Tokyo Metropolitan Children's Medical Center
3)Tokyo Pediatric Clinical Research Network
4)Bureau of International Health Cooperation, National Center for Global Health and Medicine
Abstract
[INTRODUCTION] Pediatricians in Japan are generally unaware of how to prevent pediatric injuries. The present study was based on a survey investigating Japanese pediatricians' knowledge, attitudes, and practices in injury prevention.
[METHODS] Pediatricians at seven Tokyo metropolitan government hospitals with more than three years' medical experience were included. The questionnaire was based on checklists used widely for health examinations and health care visits both within Japan and overseas.
[RESULTS] One hundred thirty-nine participants were enrolled. More than half of the respondents were unable to answer ten of the 15 questions pertaining to knowledge of injury prevention. Factors in the respondents' background contributed to the quality of the responses. Ninety-three% of respondents reported having a positive attitude to injury prevention, 77% were concerned about difficulties in obtaining information for parents to prevent injuries, and 50% recommended the senior residency as an appropriate time for this purpose.
[CONCLUSIONS] Pediatricians had a positive attitude towards injury prevention but had insufficient knowledge and felt that opportunities for obtaining information on this topic were limited. The findings suggest that appropriate education on this topic should be incorporated into the training of pediatric residents.
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Original Article
Title
Japanese Herbal Kampo Medicine for Pediatric Inpatients in Japan: A Retrospective Nationwide Study
Author
Nobuaki Michihata
Department of Health Services Research, Graduate School of Medicine, The University of Tokyo
Abstract
Background: Japanese herbal Kampo medicine is used widely for pediatric patients in Japan. Little is known about how often Kampo medicine is used for pediatric inpatient care. The aim of this study was to examine the current status of using Kampo medicine for pediatric inpatients in Japan.
Methods: Using the Diagnostic Procedure Combination database, we extracted all inpatients aged under 20 years from April 2014 to March 2015. We examined the proportions of prescribing Kampo medicine in patients with different backgrounds. We also examined which Kampo medicines were prescribed frequently and what diseases were recorded for patients who used Kampo medicines.
Results: We identified 744,917 pediatric inpatients during the study period; 1.6% patients of them used some Kampo medicines. Older patients were significantly more likely to receive Kampo medicine (p< 0.001). Dai-kenchu-to, rikkunshi-to and gorei-san were most frequently prescribed Kampo medicines. Kampo medicines were significantly more likely to be used for patients with some devices including tracheostomy and gastrostomy tubes, and those with diseases, for which previous studies showed Kampo medicines were effective.
Conclusion: The present study showed Kampo medicine was frequently used for pediatric inpatients in Japan. Kampo medicines for gastrointestinal symptoms were used most frequently in pediatric inpatients. Patients with some devices tended to use Kampo medicines, possibly because most of them were bedridden and had gastrointestinal symptoms.
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Case Report
Title
Long QT Syndrome Treated with Subcutaneous Implantable Cardioverter Defibrillator
Author
Yuta Ariyama1) Masaru Miura2) Ken Kato3) Kohichi Miyata2) Hiroki Nagamine2) and Hirotaka Ohki2)
1)Department of General Pediatrics, Tokyo Metropolitan Children's Medical Center
2)Department of Cardiology, Tokyo Metropolitan Children's Medical Center
3)Department of Cardiology, Tokyo Metropolitan Tama Medical Center
Abstract
The subcutaneous implantable cardioverter defibrillator (S-ICD) is a new device capable of defibrillating the heart without touching it via a generator and lead implanted subcutaneously. In Japan, S-ICD use has increased in adults since its introduction in 2016, but its pediatric use has not yet been reported. We report the case of an 11-year-old boy with an S-ICD implantation. He had been treated with oral propranolol and restricted physical activity for long QT syndrome type 1. While running, he experienced ventricular fibrillation with Torsades de pointes, and was transferred to our hospital after spontaneous circulation was restored by cardioversion. Because he recovered without neurological sequelae, we planned an ICD implantation and chose S-ICD as better suited to children. To decrease the risk of a malfunction during activity, the patient underwent a screening test by treadmill. Absorbable sutures were used to lock the S-ICD leads to prevent pain from lead malpositioning and traction due to growth. Eight months after the operation, the patient has not experienced pain or defibrillation. Fixing S-ICD's leads with absorbable sutures may reduce the pain of traction. S-ICD is suitable for children because the durable leads rarely fail, managing infections is relatively easy, and radiation exposure is unnecessary. Drawbacks are the possible malpositioning of the leads or generator with growth and the exacerbation of pain or hematomas due to the large generator size relative to pediatric patients. As more children receive S-ICD implantations, guidelines for its appropriate management need to be clarified through the accumulation of data.
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Case Report
Title
Identification of CTLA4 Mutation in a Patient with Autoimmune Hemolytic Anemia
Author
Shiro Iwawaki1) Genki Yamato1) Norio Shiba1) Kazuhiro Muramatsu1) Jun Kawashima1) Mikiko Koitabashi1) Haruna Okuno1) Hisaya Nakadate2) Nao Nishimura3) Akihiro Hoshino4) Kohsuke Imai4) Makoto Kuwashima5) Hirokazu Kanegane4) and Hirokazu Arakawa1)
1)Department of Pediatrics, Gunma University Hospital
2)Department of Hematology, National Center for Child Health and Development
3)Department of Intensive Care Unit, National Center for Child Health and Development
4)Department of Pediatrics, Tokyo Medical and Dental University
5)Department of Pediatrics, Kiryu Kosei General Hospital
Abstract
Cytotoxic T lymphocyte antigen-4 (CTLA-4) deficiency is characterized by an immune defect and immune dysregulation syndrome. We present a case of CTLA-4 deficiency with severe autoimmune hemolytic anemia (AIHA). A 15-year-old boy was admitted with fever, impaired consciousness, and severe anemia. His medical history included chronic immune thrombocytopenic purpura and pneumonia. We diagnosed based on AIHA by blood cell count and serum immunological and biochemical findings. The patient was initially treated with 60 mg/day of prednisolone, plasma exchange, and immunoglobulin; however, his anemia and consciousness deteriorated. He was transferred to a pediatric intensive care unit center and given pulse methylprednisolone. Thereafter, his condition gradually improved. We suspected that his severe and multiple autoimmune diseases might be associated with primary immunodeficiency disease, and identified the heterozygous mutation for c.232delG, p.D78TfsX4 in CTLA4 and low CTLA-4 protein expression in regulatory T cells. Therefore, he was given a diagnosis of CTLA-4 deficiency. These findings suggest that CTLA-4 deficiency should be considered in patients with autoimmune diseases and immunodeficiency.
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Case Report
Title
A Patient with Type 1 Diabetes Complicated by Hemoglobinopathy
Author
Noriko Kinoshita Daisuke Yoshida and Masayuki Tanaka
Department of Pediatrics, National Hospital Organization, Higashi-Ohmi General Medical Center
Abstract
The patient was a 6-year-old girl of Brazilian descent. She was diagnosed with type 1 diabetes after experiencing frequent urination, and insulin treatment was initiated. No increase in the patient's glycated hemoglobin (HbA1c) level was detected on high performance liquid chromatography (HPLC) despite the presence of hyperglycemia, which was suggestive of fulminant type 1 diabetes mellitus, but this was ruled out based on the patient's course and test findings. Hemoglobinopathy was suspected because the patient's red blood cells were hypochromic and microcytic, and high-resolution hemoglobin separation and DNA sequencing of the hemoglobin β chain gene confirmed the diagnosis. HbA1c cannot be accurately measured using HPLC in hemoglobinopathy patients; therefore, diabetes should be controlled by measuring the HbA1c level with enzymatic or immunological methods, which are not influenced by hemoglobinopathy, or by using the glycoalbumin (GA) level as an index. The reductions in patients' insulin secretion capacities seen in the early phase of type 1 diabetes vary, leading to unstable blood glucose control. As monitoring the GA level is considered to facilitate the early detection of aggravation/improvement, we controlled our patient's diabetes based on this index, which has been demonstrated to be useful in neonates and infants. The prevalence of hemoglobinopathy is higher among non-Japanese people, but occasions to treat diabetic patients with concomitant hemoglobinopathy will increase with globalization. When divergence between the blood glucose and HbA1c levels is detected, it is necessary to understand the limitations of HPLC and consider the possibility of hemoglobinopathy.
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Case Report
Title
Cystic Fibrosis Presenting with Marked Steatosis of the Liver and Pancreas with No Respiratory Symptoms: A Case Report
Author
Yusuke Hoshi Takashi Honma Fumihiko Kakuta and Daiki Abukawa
Department of Gastoroenterology and Hepatology, Miyagi Children's Hospital
Abstract
Cystic fibrosis (CF) is one of the most common autosomal recessive inherited disorders among Caucasians, although it is considered to be rare among Asians. We report the case of a 5-year-old boy with CF who had steatorrhea and poor weight gain, but no respiratory symptoms. Imaging tests and a liver biopsy revealed marked steatosis of the liver and pancreas. The chloride level in his perspiration was abnormally high (122 mEq/L). CFTR gene analysis revealed compound heterozygous gene mutations that were inherited from each parent. He had a mutation in intron 6a (c.744-3C>G) of the CFTR gene, which represents a novel mutation in a Japanese patient. After the administration of a pancreatic enzyme supplement and multivitamins, his steatorrhea and fatty liver markedly improved, and his weight increased. When an Asian patient presents with fatty liver and steatorrhea without respiratory symptoms, a sweat test and CFTR gene analysis should be considered to diagnose CF.
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Case Report
Title
An Infant with Laryngopharyngeal Edema due to Laundry Detergent Pod Ingestion
Author
Shunsuke Shimazaki1) Itsuro Kazukawa1) Makiko Hai1) Takahiro Ishii1) Maine Ishida1) Masanori Minagawa1) and Yoko Sugimura2)
1)Department of Endocrinology, Chiba Children's Hospital
2)Department of Critical Care Medicine, Chiba Children's Hospital
Abstract
An 8-month-old boy was transferred to our hospital with respiratory failure due to ingestion of a laundry detergent pod. We diagnosed with laryngopharyngeal edema caused by the pod ingestion; laryngoscopy revealed a swollen epiglottis and oropharyngeal mucosal edema. He required emergency intubation and was given dexamethasone and antibiotics. He was extubated on hospital day 4 with improvement of mucosal edema evidenced by laryngoscopy. On hospital day 11 he was discharged and recovered without sequelae.
Laundry detergent pods were introduced in Japan in 2014 and became popular because of their ease of use. Increased home use and easy availability of the pods may have led to an increase in their accidental ingestion. We report this case because we suspect there are increased numbers of children in Japan who have required intensive care treatment for ingestion of laundry detergent pods.
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