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THE JOURNAL OF THE JAPAN PEDIATRIC SOCIETY
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Vol.121, No.4, April 2017
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Original Article
Title
Social Outcomes of Adults with Pervasive Developmental Disorders Diagnosed and Treated in Childhood -A Parent Questionnaire Study-
Author
Shinichi Hirabayashi1) Noboru Fueki2) and Satoru Hirano3)
1)Division of Neurology, Nagano Children's Hospital
2)Department of Rehabilitation, Nagano Children's Hospital
3)Nakagawanosato Rehabilitation Center
Abstract
Purpose: To elucidate the social outcomes of adults with pervasive developmental disorders (PDD) who were diagnosed and treated in childhood.
Methods: Parents of adults with PDD who were diagnosed and treated by a children's hospital responded to semi-structured questionnaires, and the data were analyzed. General adaptability, composed of adaptive competence and emotional stability, was evaluated as an indicator of social outcomes.
Results: A total of 228 parents responded (62% response rate). Of them, 38 (17%) were students, 131 (57%) were employed, and 57 (25%) were non-employed. General adaptability was good in 145 (65%) and poor in 36 (16%). Multiple logistic regression analysis revealed that good general adaptability was positively correlated with employment and child-rearing attitude, but negatively correlated with a history of medical treatment (especially the use of anti-psychotic drugs) and low inter-personal skills. Intelligence level and academic career were not correlated with general adaptability. Five adults (3%) showed an "optimal outcome".
Conclusions: Social outcomes based on general adaptability were better in comparison with previous reports, despite some methodological limitations. Advice on rearing attitude and effective interventions for the secondary symptoms due to maladjustment were considered important for improving the social outcomes. Further studies could elucidate how medical support provided from childhood can improve the social outcomes of adults with PDD.
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Original Article
Title
Medical Care System for Adolescents and Young Adults with Cancers
Author
Akiko Takada1) Katsutsugu Umeda1) Koji Kawaguchi1) Atsushi Iwai1) Masamitsu Mikami1) Seishiro Nodomi1) Satoshi Saida1) Hidefumi Hiramatsu1) Ken-Ichiro Watanabe1)2) Toshio Heike1) and Souichi Adachi3)
1)Department of Pediatrics, Graduate School of Medicine, Kyoto University
2)Division of Hematology and Oncology, Shizuoka Children's Hospital
3)Human Health Sciences, Graduate School of Medicine, Kyoto University
Abstract
We retrospectively analyzed the medical care system for 170 adolescents and young adults with cancers, who were admitted to our hospital between January 2008 and December 2012. The patients' median age was 22 years and 10 months. The major disease groups consisted of brain and spinal cord tumors, hematological malignancies, musculoskeletal tumors, genital tumors, thyroid and neuroendocrine tumors and gastrointestinal tumors. Approximately 70% of patients aged 15-19 years had brain and spinal cord tumors, hematological malignancies, and musculoskeletal tumors, which decreased with age. By contrast, the proportion of patients with gastrointestinal tumors and thyroid and neuroendocrine tumors increased among patients older than 20 years. The 4-year overall survival rate of 170 patients was 79.4% ± 6.5%. Multimodality therapy consisting of surgery, radiation therapy, and chemotherapy was conducted in 91 patients (53.5%). Fifteen departments were involved in hospital treatment: 85 patients were treated in a single department, whereas 85 patients were treated in multiple departments. Pediatricians treated 7 patients as attending physicians and supported other departments in making decisions regarding treatment or hematopoietic stem cell transplantation in the remaining 23 patients. The cancer support team provided assistance in 35 of 170 patients, mainly to mitigate unpleasant physical or mental condition. Multimodality therapy was required in a high percentage of adolescents and young adults with cancers: therefore, pediatricians were charged with an important task for these patients, even if they were not the attending physician. Deeper cooperation with other departments and co-medicals will be required to improve the outcome and quality of treatment for patients of this generation.
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Original Article
Title
Immunogenicity of Two-dose Schedule for Mumps Vaccination
Author
Naoko Nishimura Takao Ozaki Kensei Gotoh Kei Kozawa Hiromi Hibino Masahiro Kawaguchi Tomoyasu Noguchi Naozumi Fujishiro and Koji Takemoto
Department of Pediatrics, Konan Kosei Hospital
Abstract
In Japan, mumps vaccine was introduced as voluntary vaccination of a single-dose schedule in 1981 and it has not been changed since then. We assessed the immune response of a two-dose schedule for mumps vaccination. During the 4-year period from April 2008 to March 2012, 182 children received the first dose of mumps vaccine at our hospital. In July 2013, a questionnaire survey was sent to investigate the occurrence of mumps after vaccination. In children without the breakthrough infection from whom consent of their guardians was obtained, a booster dose of mumps vaccine was administered. Just prior to booster vaccination and 4-6 weeks after vaccination, the IgG antibody titer against mumps virus was measured by enzyme-linked immunosorbent assay (ELISA). In addition, adverse reactions were investigated up to 4 weeks after vaccination.
The reply rate to the questionnaire was 51% (93/182) and 5 children (5.4%) developed mumps after the initial vaccination. Booster vaccination was conducted in 32 children. Their mean age at booster vaccination was 5.8 years (3.5 to 11.6 years) and the mean interval between the first and booster doses was 3.1 years (1.4 to 5.4 years). The seropositive rate after initial vaccination (ELISA titer ≥400) was 94% (30/32) and the seropositive rate prior to booster vaccination was 75% (24/32). After booster vaccination, the seropositive rate was 100% and the mean antibody titer (log10) was 3.25 which was significantly higher than the mean antibody titer 2.93 after the first dose (p<0.001). As adverse reactions, fever (≥37.5°C) was observed in 13%, local reactions in 6%, and parotid swelling in 3%. A two-dose schedule for mumps vaccination was considered to be effective because of high immunogenicity.
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Original Article
Title
A Review of 27 Cases of Foreign Body Ingestion in Children
Author
Hitoshi Honma Mai Kusakari Mayu Hanamura Sawako Kato Yoshiko Kurashige Nao Hidaka and Yoshiko Nakayama
Department of Pediatrics, Shinshu University School of Medicine
Abstract
Background and Aims: Foreign body (FB) ingestion is a common clinical problem among children. The indication of intervention depends on the age and the clinical condition of the patient, as well as the type and location of the FB, and the time since ingestion, in addition to other factors. Herein, we report our experience in the management of FB ingestion in children, in comparison to NASPGHAN-Guidelines.
Methods: Pediatric patients who presented with FB ingestion were retrospectively reviewed from medical records between April 2005 and April 2015, at the tertiary care center in Japan.
Results: There were 27 cases of FB ingestion. FB materials included; superabsorbent polymer (SAP), food impaction, coins, button batteries, and other objects, found in 2, 3, 4, 6 and 12 cases, respectively. Endoscopic intervention was performed in 17 cases, and the ingested FBs were successfully removed in 11 of them. No major complications were reported. The indication of endoscopy in our cases was consistent with that of NASPGHAN-Guidelines in regard to food impaction, coins, and SAP ingestion, however, it was different in the setting of button batteries in stomach, magnets, and some blunt object ingestion. Of note, a one-year-old boy developed duodenal obstruction after accidental ingestion of SAP.
Conclusions: The indication of endoscopy in our series of patients was consistent in most cases with that of NASPGHAN-Guidelines in reference to FB ingestion. The SAP has the potential risk of causing bowel obstruction in accordance to its expandable susceptibility and thus public awareness is highly recommended.
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Original Article
Title
Usefulness of Testing Postprandial Blood Glucose Levels for Screening Lifestyle-related Disease in Schoolchildren
Author
Ayumi Miyazaki1) Ayako Oguri2) and Shoetsu Shimura3)
1)Department of Pediatrics, Japan Community Health Care Organization Takaoka-Fushiki Hospital
2)Oguri Pediatric Clinic
3)Shimura Pediatric Clinic
Abstract
Background: In this study we evaluated the usefulness of testing postprandial blood glucose (PG) levels in children because PG, as a cardiovascular risk factor, is now recognized to be a better predictor of death in adults for all causes, including cardiovascular disease, than is fasting blood glucose (FG).
Methods: A total of 8,119 fourth and seventh grade schoolchildren were included in the screening for lifestyle-related disease from 2013 to 2015 in Takaoka City. Their PG levels were tested within 2 hours of eating a school-lunch. The relationship between PG and FG was also evaluated in 194 subjects who were re-examined because of obesity, dyslipidemia, etc.
Results: The differences in mean PG levels among three years were significant but small. The range of values of all three years was greater in the seventh grade students than in the fourth grade students, especially in girls. The 95th percentile values of fourth grade boys, girls, seventh grade boys, girls, and all subjects were 132 mg/dl, 138 mg/dl, 146 mg/dl, 155 mg/dl, and 143 mg/dl respectively, which approximated the 140 mg/dl for the 2-hour glucose intolerance level in adults. Simple regression analysis revealed a low contribution ratio between PG and FG among re-examined subjects (r2=0.056). Among the individuals with PG levels of more than 200 mg/dl, three were diabetic, including two who were newly diagnosed non-obese girls.
Conclusions: Testing PG levels may be useful for screening lifestyle-related disease, especially diabetes in children.
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Case Report
Title
Pulmonary Hypertension in a Liver Transplant Recipient with Hepatocerebral Mitochondrial DNA Depletion Syndrome due to MPV17 Gene Mutation
Author
Hitomi Masuda1) Hiroshi Ono1) Taiyu Hayashi1) Hiroshi Masuda2) Kazumi Sakashita2) and Hitoshi Kato1)
1)Division of Cardiology, National Center for Child Health and Development
2)Department of General Pediatrics, National Center for Child Health and Development
Abstract
Mitochondrial DNA depletion syndromes (MTDPS) are autosomal recessive disorders caused by a tissue-specific decrease in mitochondrial DNA copy number. Mutations in at least 14 nuclear genes have been found to be associated with the syndromes, which result in organ disorders including hepatocerebral disease, encephalopathy and myopathy. However, no patients with MTDPS has been reported to develop pulmonary hypertension. We report a boy with hepatocerebral MTDPS, in whom a mutation in MPV17 gene was confirmed by a liver biopsy at 2 months. He had developed fulminant hepatic failure and underwent a living donor liver transplantation at 4 months. Postoperatively, the liver graft function was favorable. At 14 months, the patient developed pulmonary hypertension; mean pulmonary artery pressure was 46 mmHg and pulmonary vascular resistance was 9.54 Wood Units• m2. Although sequential combination therapy with sildenafil, ambrisentan, and epoprostenol led to a reduction in echocardiographically tricuspid regurgitation pressure gradient, the patient died of sepsis at 21 months. In the follow-up of a liver transplant recipient with hepatocerebral MTDPS due to MPV17 gene mutations, the occurrence of pulmonary hypertension should be closely monitored. The fact that a patient with MTDPS developed pulmonary hypertension might provide a new insight into the role of mitochondria in the development of pulmonary hypertension.
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Case Report
Title
An Anti-GAD Antibody-associated Cerebellar Ataxia Case
Author
Kenji Iwai1) Kiyoko Amo1) Junichi Ishikawa1) Kiyohiro Kin2) Azumi Sakakibara2) Yukiko Hashimoto2) Rie Kawakita2) Yuki Hosokawa2) Tohru Yorifuji2) and Masao Togawa1)
1)Department of Pediatric Emergency Medicine, Osaka City General Hospital
2)Department of Pediatric Metabolism and Endocrinology, Osaka City General Hospital
Abstract
The anti-glutamic acid decarboxylase (GAD) antibody is associated with type 1 diabetes mellitus (DM). Although some adults are affected by anti-GAD antibody-related disease, children rarely develop anti-GAD cerebellar ataxia. An eight-year-old girl had been diagnosed with influenza infection two days after the onset of fever and was administered Zanamivir. However, she repeatedly had tonic clonic seizure and her, so she was admitted to the hospital. She was transferred to our hospital due to a lack of improvement in her level of consciousness. Spinal fluid assessment revealed highlevel of protein and pleocytosis. Brain MRI showed clinically mild encephalitis/encephalopathy with reversible splenial lesions (MERS). She also had metabolic acidosis and high levels of blood sugar, HbA1c, serum anti-GAD antibody, and anti-insulin antibody. So we diagnosed type1DM. Her level of consciousness gradually improved, but ataxic symptoms persisted. Brain MRI on hospital day 8 revealed an area of high intensity in the right side of the middle cerebellar peduncle. Anti-GAD antibodies associated cerebellar ataxia was diagnosed based on her spinal fluid being positive for anti-GAD antibody upon admission. Treatment with intravenous immunoglobulin (IVIG) and pulsed steroid significantly improved her symptoms. More information about children with anti-GAD cerebellar ataxia needs to be accumulated and analyzed.
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Case Report
Title
A Japanese Boy of Glucose-6-Phosphate Dehydrogenase Deficiency Identified by a Possibility of Aplastic Crisis
Author
Asuka Yamaguchi-Fujimaki1)2) Kei Osaka1) Mari Tanaka-Kubota1) Satoshi Miyamoto1) Noriko Mitsuiki1) Masatoshi Takagi3) Kohsuke Imai3) Mitsuru Kubota4) Hirokazu Kanegane1) and Tomohiro Morio1)
1)Department of Pediatrics and Developmental Biology, Graduate School of Medical and Dental Sciences, Tokyo Medical and Dental University
2)Division of Pediatrics, Tokyo-Kita Medical Center
3)Department of Community Pediatrics, Perinatal and Maternal Medicine, Graduate School of Medical and Dental Sciences, Tokyo Medical and Dental University
4)Division of General Pediatrics & Interdisciplinary Medicine, National Center for Child Health and Development
Abstract
Aplastic crisis is severe anemia caused by transient impaired hematopoiesis, and it is sometimes observed in patients with hereditary hemolytic anemia caused by certain infections, especially parvovirus B19 infection. The most common type of hereditary hemolytic anemia is hereditary spherocytosis in Japan, and this disease is often identified by a chance of aplastic crisis. Here we described a 3-year-old Japanese boy with aplastic crisis caused by parvovirus B19 infection, who was diagnosed with glucose-6 phosphate-dehydrogenase (G6PD) deficiency. The patient was admitted to our hospital due to severe anemia (Hb 3.4 g/dL), and was diagnosed with parvovirus B19 infection-associated aplastic crisis, and required blood transfusion. He had no family history of hemolytic anemia, normal erythrocyte morphology, and normal osmotic fragility, indicating no spherocytosis. The patient was later diagnosed with G6PD deficiency by a mean of hemolytic anemia screening test. G6PD deficiency is extremely rare in Japanese, but it should be considered as a differential diagnosis of hereditary hemolytic anemia associated with aplastic crisis. Galactosemia is screened by Beutler's test as newborn mass screening in Japan, and it is described that G6PD deficiency is rarely detected simultaneously. However, the patient revealed a normal result of newborn mass screening. We conclude that Beutler's test is not helpful to identify G6PD deficiency.
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Case Report
Title
A Rare Case of Summer-type Hypersensitivity Pneumonitis in a Child with Severe Motor and Intellectual Disabilities
Author
Yusei Baba Shinsuke Maruyama Akinori Miyazono Takayuki Tanabe Yasuko Morita Yuichi Nomura and Yoshifumi Kawano
Department of Pediatrics, Kagoshima University
Abstract
Hypersensitivity pneumonitis is a diffuse granulomatous interstitial pneumonia caused by fungal spores, organic dust, etc. Its symptoms include fever, cough, and exertional dyspnea. This disease is unusual in children because some are frequently diagnosed as having other respiratory diseases such as bronchial asthma; additionally, since there are patients in whom symptoms are seasonal, a diagnosis cannot be made since the symptoms disappear before an assessment can be performed. We describe the case of a 14-year-old girl with severe motor and intellectual disabilities who was diagnosed as having summer-type hypersensitivity pneumonitis. She had recurrent pneumonia 3 years ago. It took time to make the diagnosis of summer-type hypersensitivity pneumonitis. To date, there have been no other reports of patients with severe motor and intellectual disabilities who have been diagnosed as having hypersensitivity pneumonitis. Hypersensitivity pneumonitis is a recurrent lower respiratory tract disease; thus, it is important for physicians to consider it in patients A rare case of summer-type hypersensitivity pneumonitis in a child with severe motor and intellectual disabilities.
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Case Report
Title
Clinical Course and Endocrinological Characteristics of an Infant Case of Young-Simpson Syndrome
Author
Takashi Shima and Ayako Tanae
Department of Pediatric, Shonan Kamakura General Hospital
Abstract
Young-Simpson instances of syndrome are rare, and are characterized by short palpebral fissures, hypothyroidism, cryptorchidism, patellar hypoplasia, and mental retardation. It was first reported in 1987, and the responsible gene was identified as KAT6B in 2011. Approximately 30 cases have been reported in the world. We diagnosed a male infant with this syndrome, and report the endocrinological, ophthalmological, and urological findings. The patient had blepharophimosis at birth, but could only open his eyes slightly by age 1 month. The bulb and fundus were intact. He had primary hypothyroidism. An exposed glans at birth and a low serum testosterone level indicated testicular dysfunction. However, he showed a normal testosterone response to a human chorionic gonadotropin test at 4 months. Brain magnetic resonance imaging showed corpus callosum hypoplasia and periventricular cysts. He had general psychomotor retardation, and an acquired expressive but no receptive language disorder. There are few reports on this condition because it is rare. Accumulation of case reports will help clarify the pathology and psychomotor development, enabling appropriate management of such patients.
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