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THE JOURNAL OF THE JAPAN PEDIATRIC SOCIETY
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Vol.118, No.5, May 2014
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Review
Title
Experience and Problems Concerning Short-stay Services at Developmental Rehabilitation Center
Author
Kiyoshi Takemoto Masahisa Funato Kiyoshi Baba Yoshitaka Iijima and Atsuko Kashiwagi
Department of Pediatrics, Osaka Developmental Rehabilitation Center
Abstract
[Objective] Investigation of short-stay service for children with severe motor and intellectual disabilities provided at Osaka Developmental Rehabilitation Center.
[Method] Retrospective analysis from the database of short-stay services from 2008 to 2012.
[Result] We have an enrollment of about 600 patients presently being provided with short-stay service, averaging 11.3 persons per day. Of these patients 49% stayed within 3 days, and 51% of the patients were under 18 years. Many of them required medical care. However, 17% of the patients were adults over 30 years of age. Mechanical ventilation was required by 12% of the total number of patients. The problems of management were: shortage of beds for short-stay service, difficulties with many sudden cancellations, medical treatment requirements arising from rapid changes in patient conditions, differences in methods of medical care not only among various hospitals but also among attending physicians or families, in addition to difficulties in the respective care related to each patient's daily life.
[Conclusion] The enhancement of short-stay services, including respite care is very important for supporting families taking care of children with severe disabilities, especially with those who require medical care at home.
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Original Article
Title
Nasopharyngeal Carriage of Streptococcus Pneumoniae Following the Introduction of the 7-valent Pneumococcal Conjugate Vaccine in Japanese Children Attending Day-care Centers
Author
Shinji Fujita Hiroko Sumita Ayako Saito Yutaka Minohara Shigeyoshi Yazaki Miyoshi Kitamura Shigehiko Kawai Kazumi Kamata and Hirosi Kitahara
Isogo Medical Association
Abstract
Day-care centers play an important role in maintaining the circulation of Streptococcus pneumoniae in the population. In Japan, the 7-valent pneumococcal conjugate vaccine (PCV7) was introduced in 2010. Because its use was initially optional, it was not widely distributed before 2011. Starting in February 2011, most children under the age of 5 were entitled to free PCV7 vaccinations. The rate of vaccination has subsequently increased significantly among children in this age group. We investigated the changes in the nasopharyngeal carriage of Streptococcus pneumoniae following the introduction of PCV7.
Methods: We collected nasopharyngeal specimens from 574 children (mean age 3.8 years) attending day-care centers (n=12) in Isogo-ku, Yokohama, between June and July 2012. The nasopharyngeal samples were obtained by pediatricians.
Written informed consent was obtained from the children's parents.
Results: Among the 574 children, 390 received at least one dose of the PCV7 vaccine. A total of 213 (37.1%) of the 574 children were colonized with Streptococcus pneumoniae.
Carriage of PCV7 serotypes was found in only 28 cases (13.1%), including the 6B serotype (n=10), the 19F serotype (n=12), and the 23F serotype (n=6).
Non-PCV7 serotypes were found in 185 cases (86.8%), including the 15A serotype (n=18), the 19A serotype (n=15), the 15B serotype (n=14), the 29 serotype (n=13), and the 15C serotype (n=9).
The prevalence of pneumococcal carriage exceeded 50% in the children less than 1 year of age and was highest among 1-year-olds then decreased gradually. However, no PCV7 serotypes were detected in children by age 1. The serotype of Streptococcus pneumoniae was quite different in each day-care center.
Conclusions: One year after the introduction of PCV7, the carriage of the PCV7 serotype decreased significantly.
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Original Article
Title
The Incidence and Symptoms/Signs of Acute Otitis Media among Patients Who Consulted Pediatric Outpatient Clinics
Author
Shinya Tsuchida
Japan Pediatric Research Group for Otitits Media, Tsuchida Children's Clinic
Abstract
Aims: To prospectively investigate the incidence and symptoms/signs of acute otitis media (AOM) among patients who consulted pediatric outpatient clinics due to respiratory tract infections.
Subjects and Methods: The subjects were 2,287 patients (3.7± 3.3 years old) who consulted one of 10 pediatric outpatient clinics between January and December 2009 due to respiratory tract infections.
Results: Middle ear effusion and AOM were observed in 352 (15.4%) and 122 (5.3%), patients, respectively. The peak age at onset of AOM was 1 year (8.7%), and it occurred most frequently in April (9.0%) and December (9.2%). Fifty-three patients (43%) had earache, including 13 patients (19%) aged less than 2 years. The most important symptoms to diagnose AOM were crying/bad temper (relative risk [RR]=6.5, 95% confidence interval [CI]=4.1-10.3) and nasal secretions (RR=3.0, CI=1.9-4.6). Fever (≥ 38.0°C) was not found to be a specific sign of AOM; it was actually associated with reduced likelihood of AOM, especially among patients aged less than 2 years (RR=0.5, CI=0.3-0.9).
Conclusions: There were many cases of AOM among those who consulted pediatric outpatient clinics due to respiratory tract infections. Regardless of the symptoms, otoscopic examination is strongly recommended for patients with respiratory tract infections.
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Original Article
Title
Evaluation of a Screening Checklist and New Diagnostic Criteria for Juvenile Orthostatic Dysregulation
Author
Norio Kazuma
Department of Pediatrics, Seibu General Hospital
Abstract
The Japanese clinical guidelines for juvenile orthostatic dysregulation (OD) were published in 2006. These guidelines recommend that the various subsets are determined by a new orthostatic test for subjects who have been selected on the basis of the former diagnostic criteria.
Tanaka recommended that a screening checklist can be used as the first step for diagnosis, and concluded that manifestation of more than three of the 11 former diagnostic criteria be employed (the Tanaka recommendation). On the other hand, Ohkuni recommended new diagnostic criteria for OD, which were focused on major manifestations (A: vertigo and dizziness on standing; B: fainting in a standing position), and removed all of the minor manifestations except for the results of the orthostatic test (the Ohkuni recommendation). The number of the subjects in the present study were 164 patients who were reassessed on the basis of both the Tanaka and Ohkuni recommendations. All of the subjects who were assessed as positive by the new orthostatic test were selected on the basis of the Tanaka recommendation. However, 17 (12%) of the subjects selected on the basis of the Ohkuni recommendation were negative when the same test was applied. Therefore, the Ohkuni recommendation requires further consideration before it can be adopted as the new criteria. Tanaka's method of selecting more than three of the 11 manifestations in the former diagnostic criteria is useful for screening of orthostatic dysregulation.
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Original Article
Title
Usefulness of the Bristol Stool Form Scale in the Diagnosis and Evaluation of the Severity Assessment of Pediatric Functional Constipation
Author
Yoshimitsu Fujii1) Sohsaku Yamanouchi1) Shogo Kato1) Ryuta Washio2) Tetsuya Kitao1) Taichi Ohmachi1) Jiro Kino2) Minoru Kino2) and Kazunari Kaneko1)
1)Department of Pediatrics, Kansai Medical University
2)Nakano Children's Hospital
Abstract
Constipation, the majority of which is functional constipation (FC), is a common disorder in childhood with the prevalence rate of about 30%. However, it is difficult for general pediatricians to make a diagnosis and severity assessment of FC. The Bristol Stool Form Scale (BSFS) is widely used for determining the shape and hardness of stools for irritable bowel syndrome, which are classified as type 1-7 from separate hard lumps to watery escretions with no solid pieces.
In order to apply the BSFS for assessment of childhood FC, we evaluated the usefulness of BSFS in 271 cases who met the Rome III diagnostic criteria for FC together with 54 cases who presented with a chief complaint of constipation but whose final diagnosis was not FC.
Median BSFS score for the 271 FC and 54 non-FC cases was type 1.0 and type 4.0, respectively, indicating that cases with FC had significantly harder stools. The area under the receiver operating characteristic curve of BSFS for the diagnostic accuracy of FC was 0.825, and the cut-off point was set at BSFS type 2. With this cut-off point, the sensitivity was 0.730, the specificity was 0.796, the positive predictive value was 0.947, the negative predictive value was 0.368, and odds ratio was 10.5. The Spearman rank correlation test revealed that there were significant relationships between the BSFS scores and the indices of the severity of FC, such as defecation frequency, treatment period, and the number of medications administered.
Thus, we conclude that BSFS is a useful scale for the diagnosis and the severity assessment of FC.
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Original Article
Title
A Trial to Determine the Clinical Reference Ranges of Rapid Turnover Proteins
Author
Yukie Higashiyama1) Masaru Kubota1) Moeri Kai1) Ayako Nagai1) Akihiro Kataoka2) Yasuhiro Mizushima2) and Yosihiro Wakazono2)
1)Department of Human Life and Environment, Nara Women's University
2)Department of Pediatrics, Kyoto Katsura Hospital
Abstract
Rapid turnover proteins (RTP) such as transthyretin (TTR) and retinol-binding protein (RBP) are important in the clinical setting as sensitive indicators of nutritional status because of their short half-lives. However, reference values for RTP in children have not been fully established. Considering the difficulty of drawing blood samples from healthy children, we examined serum TTR and RBP levels in pediatric patients for the purpose of establishing, the age-related clinical reference ranges of these two indicators. After excluding 40 samples with any malnutrition risks, liver disorders or elevation of CRP more than 3.0 mg/dL, 114 samples aged 0-15 years were enrolled. TTR and RBP levels showed significant linear increases with age (R=0.48 p< 0.01, R=0.45 p< 0.01, respectively). When all samples were divided into 6 groups based on their age, the median of these indicators exhibited significantly higher levels at samples in the 13-15 years age-group than groups under 6 years of age (p< 0.05).
This study clarified that TTR and RBP values gradually increased until the age of 10 years, and reached the values of adulthood reference values at approximately the age of 15. We speculate that the present data are useful in nutritional evaluation for children to utilize the standard values in childhood instead of those in adulthood.
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Original Article
Title
A Case of Mitochondrial Respiratory Chain Complex I Deficiency: Lactate Accumulation Identified Only by Magnetic Resonance Spectroscopy
Author
Yoshie Nakamura Akira Kumakura Yoshitaka Honda Masamitu Mikami Masatoshi Nakata Atsuko Hata Ken Watanabe and Daisuke Hata
Department of Pediatrics, Kitano Hospital Tazuke Kofukai Medical Research Institute
Abstract
Mitochondrial respiratory chain disorders (MRCD) are caused by a dysfunction in mitochondrial energy metabolism. Most patients with MRCD show elevated lactate levels in their serum and cerebrospinal fluid (CSF). However, here we describe a patient with MRCD who had normal serum and CSF lactate levels.
A 5-year-old boy was examined in our hospital because of sudden headaches and vomiting. His blood tests showed slightly elevated levels of C-reactive protein. His CSF test showed an increased number of cells and high levels of interleukin 6. Magnetic resonance imaging (MRI) of his head revealed bilaterally symmetric hyperintense areas centering on the pyramidal tract. He was asymptomatic the next day. Nevertheless, the abnormal MRI findings remained unchanged. Throughout the course of treatment, his serum and CSF lactate levels were normal. Other metabolic screening tests showed no findings suggestive of metabolic disorders, except for sensory deafness. The MRI findings led us to suspect a mitochondrial disease. Therefore, magnetic resonance spectroscopy (MRS) was conducted, the results of which showed a clear lactate peak in the area of the lesions. We examined the mitochondrial respiratory chain function using dermal fibroblasts, and found that respiratory chain complex I activity was decreased. We therefore diagnosed complex I deficiency and concluded that MRCD cannot be excluded based only on the normal serum and CSF lactate levels. The use of MRS provides additional diagnostic help.
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Original Article
Title
Rapid Gender Assignment by Team Medicine in a Neonatal Case of 46, XY Robinow Syndrome
Author
Masahiro Goto1) Takashi Daitsu1) Junko Igaki1) Daisuke Ogata2) Hidehiro Takahashi2) Hiroshi Yoshihashi3) and Yukihiro Hasegawa1)4)
1)Division of Endocrinology and Metabolism, Tokyo Metropolitan Children's Medical Center
2)Division of Neonatology, Tokyo Metropolitan Children's Medical Center
3)Division of Medical Genetics, Tokyo Metropolitan Children's Medical Center
4)Department of Clinical and Basic Research, Tokyo Metropolitan Children's Medical Center
Abstract
Disorders of sex development (DSD) in neonates are medical emergencies requiring rapid and proper gender assignment. A one-day-old neonate was referred to our hospital for examination of DSD because of a completely buried micropenis. As the baby also had a dysmorphic face and mesomelic shortening, various specialists, including a medical geneticist and a radiologist from our hospital, collaborated and diagnosed SRY-positive Robinow syndrome by the age of 4 days. Consequently, we were able to recommend that the parents raise the baby as a boy at the age of 5 days. Based on the "Guidelines on the Early Management of DSD" from the Japanese Society for Pediatric Endocrinology, we organized a medical team of DSD specialists and were able to perform rapid gender assignment for this patient.
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Original Article
Title
A Case of Acute Encephalopathy with Secondary Carnitine Deficiency after Taking Pivalate-conjugated Antibiotics for 3 Days
Author
Masahiro Nishiyama1) Tsukasa Tanaka1) Kyoko Fujita1) Azusa Maruyama1) Hiroaki Nagase1) Hiroki Takeda2) Yoshiyuki Uetani2) and Yosuke Shigematsu3)
1)Department of Neurology, Hyogo Prefectural Kobe Children's Hospital
2)Department of Emergency and Critical Care Medicine, Hyogo Prefectural Kobe Children's Hospital
3)Department of Health Science, University of Fukui
Abstract
We report the case of a 19-month-old girl with acute encephalopathy after 3 days of treatment with the Tebipenem pivoxil. Upon arrival at a local hospital, she showed repeated convulsions and loss of consciousness. Laboratory data showed hypoglycemia (12 mg/dL), low free carnitine (5.1 μmol/L), and high C5 acylcarnitine (0.73 μmol/L). Intravenous administration of glucose was ineffective against the seizures and unconsciousness, and she was transferred to our hospital. Despite treatment by normothermia with barbiturate coma and carnitine supplementation, magnetic resonance imaging showed reduced diffusion in the deep white matter, as well as cortical lesions followed by brain atrophy. She was discharged on day 28 with severe neurological sequelae including paralysis on her left side. Her developmental quotient was 20 at the time of discharge. Acylcarnitine analysis in the newborn period and urinary organic acid analysis revealed no specific abnormalities. Carnitine deficiency was transient. Therefore, it was decided that the carnitine deficiency was induced by the antibiotic treatment. Acute encephalopathy-associated carnitine deficiency following long-term treatment with antibiotics containing pivalic acid has been reported previously. However, we should note that acute encephalopathy with secondary carnitine deficiency potentially occurs even after short-term use of antibiotics, e.g., 3 days in this case.
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Original Article
Title
Two Cases of Severe Mosquito Bite Allergy That Progressed to Chronic Active Epstein-Barr Virus Infection
Author
Masanobu Takeuchi1) Masakatsu Yanagimachi1) Takanori Yanai1) Koji Sasaki1) Hiromi Kato1) Tomoko Yokosuka1) Ryosuke Kajiwara1) Hisaki Fujii1) Hiroaki Goto1) Kenichi Imadome2) Hiroshi Kimura3) and Shumpei Yokota1)
1)Department of Pediatrics, Yokohama City University Hospital
2)Department of Infectious Diseases, National Research Institute for Child Health and Development
3)Depertment of Virology, Nagoya University Graduate School of Medicine
Abstract
Severe mosquito bite allergy (SMBA) is a disease with poor prognosis related to persistent infection with Epstein-Barr Virus (EBV). SMBA is characterized by intense local skin symptoms including bullae, ulcers, scarring of the mosquito bite site, and systemic high fever. SMBA does not often recover spontaneously, but usually progresses over a number of years to chronic active EBV infection (CAEBV), lymphoma, and hemophagocytic lymphohistiocytosis with a high mortality rate. We report two cases of SMBA that progressed to CAEBV. The first patient was considered for hematopoietic stem cell transplantation (HSCT) after the diagnosis of SMBA but her family refused HSCT and discontinued the patient's medical follow-up. She developed EBV-related malignant lymphoma one year after diagnosis and died with a fulminant clinical course, 6 years after lymphoma onset. The second patient received bone marrow transplantation from a matched sibling donor using a reduced intensity conditioning regimen, 6 months after SMBA diagnosis EBV-PCR was negative after preconditioning. Eighteen month follow-up showed the patient was healthy and without graft-versus-host disease or clinical symptoms of SMBA. In conclusion, SMBA can progress to an active phase such as CAEBV or malignant lymphoma, resulting in multiple organ failure. Therefore, it is important to perform HSCT at an early time-point after the diagnosis of SMBA.
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Original Article
Title
Successful Treatment with KTP Laser for an Infant with Extensive Subglottic Hemangioma
Author
Seiko Maeno1) Michiko Hayashidani1) Yutaka Nishimura1) Seishi Sumi1) Shinichirou Iwataki1) Shiho Nishimura1) Seiichi Hayakawa2) Yasuhiko Sera2) and Masao Kobayashi2)
1)Department of Newborn Care Unit, Hiroshima City Hospital
2)Department of Pediatrics, Hiroshima University Hospital
Abstract
We report the case of an infant presenting respiratory distress with noisy and labored breathing. Based on a diagnosis of croup, she was treated with nebulized epinephrine and systemic administration of dexamethasone. Her respiratory distress was temporally improved, however, it then progressed. Computed tomography showed a mass causing subglottic narrowing. The findings of laryngoscopy suggested extensive submucosal hemangioma. After admission to the intensive care unit, ablation using a KTP laser as well as the intravenous administration of prednisolone was performed under endotracheal intubation, resulting in the disappearance of her symptoms. Subsequently, she has been well for three years without any complications. Thus, endoscopic ablation with KTP laser may be recommended for the treatment of symptomatic airway hemangioma.
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Original Article
Title
Eosinophilic Pleural Effusion Following Iatrogenic Pneumothorax in a Patient with Severe Motor and Intellectual Disabilities
Author
Masahiro Kikuchi Yasuo Oyake Takumi Ishiodori Akiyoshi Hiraki and Naruyoshi Suwabe
Department of Pediatrics, Hitachi General Hospital
Abstract
We report a 17-year-old girl with severe motor and intellectual disabilities, who developed eosinophilic pleural effusion (EPE) after iatrogenic pneumothorax due to an unsuccessful central venous catheter insertion. Thoracentesis revealed massive, yellow sterile exudate containing 52% eosinophils, while peripheral blood contained 10% eosinophils. Pleural fluid IL-5 concentration was high (269 pg/ml), with an undetectable concentration (< 3.9 pg/ml) in the corresponding serum sample. Low levels of pleural fluid IL-4 and IgE suggested that preferential production of IL-5 in the pleural fluid was responsible for the pleural eosinophilia in the patient. Administration of a corticosteroid finally eliminated the pleural effusion with resolution of peripheral eosinophilia. EPE can be seen as a complication of cardiothoracic surgery, thoracic trauma, infection, or drug administration, which may be often encountered in patients with severe motor and intellectual disabilities. Respiratory status should be carefully monitored in handicapped patients who cannot express any distress.
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