50 results on '"Fuchigami, Tatsuo"'
Search Results
2. A pediatric case of autosomal dominant hypocalcemia type 2.
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Takahashi S, Fuchigami T, Suzuki J, and Morioka I
- Abstract
Objectives: Autosomal dominant hypocalcemia (ADH) is characterized by hypocalcemia and hyperphosphatemia secondary to hypoparathyroidism. It is classified as type 1, caused by gain-of-function mutations of the calcium-sensing receptor (CASR), and type 2, caused by activating mutations in GNA11 , which is a crucial mediator of CASR signaling. What is new? We report a rare pediatric case of ADH type 2., Case Presentation: The patient was a 15-year-old girl with short stature. Blood tests demonstrated hypocalcemia and hyperphosphatemia without elevated parathyroid hormone levels. Brain computed tomography revealed calcification in the bilateral basal ganglia. Genetic testing revealed the rare GNA11 mutation, c.1023C>G (p.Phe341Leu). The patient was diagnosed with ADH type 2. She had experienced numbness and tetany in her hands for several years, which improved with alfacalcidol therapy., Conclusions: Our patient is the third female and first pediatric reported case of a variant mutation in the GNA11 gene (ADH type 2), c.1023C>G (p.Phe341Leu)., (© 2023 Walter de Gruyter GmbH, Berlin/Boston.)
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- 2023
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3. Central Precocious Puberty (CPP) in Two Girls With Autism Spectrum Disorder (ASD).
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Moriuchi Y, Fuchigami T, Horie M, Yamada R, and Morioka I
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In recent years, some cases of central precocious puberty (CPP) have been reported in patients with autism spectrum disorder (ASD). Here, we report CPP in two girls with ASD. The first case was a girl, 7 years and 9 months of age. Breast budding was observed at 7 years and 2 months and pubic hair at 7 years and 8 months of age. She was diagnosed with CPP based on guidelines and ASD according to her developmental history. Considering the psychosocial burden caused by the discrepancy between her cognitive and behavioral development and the progression of secondary sex characteristics, gonadotropin-releasing hormone (GnRH) analog therapy was initiated. Case 2 was a girl, 9 years and 8 months of age. She was diagnosed with ASD according to her developmental history. Treatment using oral aripiprazole for hypersensitivity to touch and taste had been initiated, with the onset of menarche at 9 years and 10 months of age. Breast budding had been observed before 7 years and 6 months old. She was diagnosed with CPP based on guidelines. Considering that menarche was not a significant psychosocial burden and the difficulty for her and her family to attend regular follow-ups, GnRH analog therapy was not initiated. Although the pathophysiological pathway linking ASD and CPP remains to be elucidated clinically, attention to CPP in ASD is needed, considering the increase in reported cases. In addition, the indication of GnRH analog therapy should be judged considering the psychosocial burden associated with secondary sexual characteristics., Competing Interests: The authors have declared that no competing interests exist., (Copyright © 2023, Moriuchi et al.)
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- 2023
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4. A Pediatric Case of Autism Spectrum Disorder With a Prostatic Abscess.
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Takahashi S, Fuchigami T, Furuya T, Mizukoshi W, and Morioka I
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There are few reports of prostatic and periprostatic abscesses in children, and diagnosis is often difficult due to the lack of early symptoms. In addition, children with autism spectrum disorder may have difficulty reporting symptoms, with and without cognitive impairments. This article reports the case of a five-year-old boy with autism spectrum disorder and multiple prostatic abscesses caused by Pseudomonas aeruginosa . He also had various vitamin and mineral deficiencies, presumably related to an unbalanced diet. The patient was treated with antibiotics, vitamins, and trace elements. After his blood vitamin and trace element levels returned to normal, he experienced no fever or relapse. The cause of this prostatic abscess was suggested to involve vitamin and trace element deficiencies., Competing Interests: The authors have declared that no competing interests exist., (Copyright © 2022, Takahashi et al.)
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- 2022
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5. An Infant With Congenital Tracheal and Bronchial Stenosis Diagnosed by Chest Three-Dimensional Computed Tomography.
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Moriuchi Y, Fuchigami T, Mizukoshi W, and Morioka I
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In this case report, we describe the case of an infant with repeated wheezing diagnosed relatively early with congenital tracheal and bronchial stenosis after evaluation by chest three-dimensional computed tomography (3D-CT). The patient was a six-month-old male infant with a one-month history of cough and wheezing. His symptoms worsened the day before admission, and he was admitted with pneumonia and wheezing. However, wheezing continued after treatment with intravenous steroids and inhalation of a short-acting β2-stimulant. 3D-CT of the chest revealed tracheal stenosis, right bronchial stenosis, and right tracheobronchial bronchus. The patient was finally diagnosed with congenital tracheal and bronchial stenosis via bronchoscopy. A virtual bronchoscopic navigation image of the tracheal lumen was created based on the CT images. Although virtual bronchoscopic navigation is more difficult for the dynamic evaluation and evaluation of mucosal lesions than bronchoscopy, it has the advantage of not directly invading the airway. Therefore, if a fixed stenotic lesion is suspected at a facility where bronchoscopy is difficult, evaluation using chest 3D-CT and virtual bronchoscopic navigation may be helpful for diagnosis. In conclusion, congenital tracheal/bronchial stenosis should be considered in patients with prolonged wheezing and recurrent airway infections, and evaluation by chest 3D-CT and virtual bronchoscopic navigation may be helpful for diagnosis., Competing Interests: The authors have declared that no competing interests exist., (Copyright © 2022, Moriuchi et al.)
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- 2022
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6. Obturator pyomyositis and labium majus cellulitis: A case report and literature review.
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Moriuchi Y, Fuchigami T, Sugiyama C, Takahashi S, Ohashi Y, Yonezawa R, Mizukoshi W, and Morioka I
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Pyomyositis is a rare, subacute, deep bacterial infection of the skeletal muscle. When treatment is delayed, pyomyositis causes abscess formation and progresses to sepsis; therefore, its early diagnosis is important. However, the clinical presentation and laboratory findings of pyomyositis are not specific; hence, diagnosis often takes time. We encountered the case of a girl with obturator pyomyositis and redness and swelling of the labium majus, which we considered as potentially important symptoms for distinguishing obturator pyomyositis from septic hip arthritis. An 8-year-old Japanese girl presented to our hospital with fever and right hip pain. On physical examination, she had redness and swelling of the right labium majus and a right limp. She was diagnosed with obturator pyomyositis and labium majus cellulitis with magnetic resonance imaging. Her clinical presentation markedly improved after starting antibiotic therapy with intravenous cefazolin for 2 weeks and oral cefaclor for 1 week. Improvement in the inflammation of the obturator muscle and labium majus was confirmed with follow-up magnetic resonance imaging. She recovered fully with no long-term sequelae. In conclusion, obturator pyomyositis rather than septic hip arthritis should be considered in children with a limp and hip and perineal pain, particularly girls with redness and swelling of the labium majus. In addition, imaging studies, including magnetic resonance imaging, should be performed for early diagnosis., Competing Interests: Declaration of conflicting interests: The author(s) declared the following potential conflicts of interest with respect to the research, authorship, and/or publication of this article: The authors declare no conflicts of interest related to this study. However, outside the submitted work, Ichiro Morioka has received lecture fees from MSD Co., Ltd., Shionogi Co., Ltd., and AbbVie LLC and study grants from Atom Medical Corp., (© The Author(s) 2022.)
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- 2022
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7. Severe rotavirus gastroenteritis in children older than 5 years after vaccine introduction.
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Kyo K, Takano C, Kasuga Y, Ogawa E, Ishige M, Pham NTK, Okitsu S, Ushijima H, Urakami T, Fuchigami T, Hayakawa S, and Morioka I
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- Child, Child, Preschool, Feces, Genotype, Hospitalization, Humans, Infant, Japan epidemiology, Retrospective Studies, Gastroenteritis epidemiology, Gastroenteritis prevention & control, Rotavirus genetics, Rotavirus Infections epidemiology, Rotavirus Infections prevention & control, Rotavirus Vaccines
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Introduction: Rotavirus (RV) is the major pathogen responsible for acute gastroenteritis in infants. Since RV vaccines were introduced, a substantial decline in the incidence of severe RV infection has been reported. However, some burden still exists, even in developed countries, including Japan., Methods: We retrospectively surveyed 380 patients hospitalized for acute gastroenteritis from 2015 to 2019. In 2019, additional detailed clinical information of 21 patients with RV gastroenteritis was obtained to evaluate the efficacy of the RV vaccine. Nine fecal samples from those patients were collected to detect the RV genotypes., Results: Our data showed an increasing trend in hospitalizations for severe RV gastroenteritis in children older than 5 years. According to the Vesikari clinical severity scores in the older group (≥5 years), the gastrointestinal symptoms in vaccinated patients were less severe than those in unvaccinated patients (p = 0.014). The genotype analysis revealed that G9P[8]I1 was the major genotype in the recruited patients in 2019., Conclusions: This report warns that children older than 5 years could be affected by severe RV infection and suggests prompt intervention for this age group, similar to that in infants. In the new period in which the RV vaccine is included in Japanese national immunization programs beginning October 2020, continuous monitoring of patient clinical characteristics and RV epidemiology is required to determine the role of vaccines., Competing Interests: Declaration of competing interest None of the authors have any conflicts of interest to declare in this study. Outside the submitted work, Ichiro Morioka has received lecture fees from MSD Co., Ltd., Shionogi Co., Ltd., and AbbVie LLC and study grants from Atom Medical Corp., (Copyright © 2020 Japanese Society of Chemotherapy and The Japanese Association for Infectious Diseases. Published by Elsevier Ltd. All rights reserved.)
- Published
- 2021
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8. Mortality of Japanese patients with Leigh syndrome: Effects of age at onset and genetic diagnosis.
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Ogawa E, Fushimi T, Ogawa-Tominaga M, Shimura M, Tajika M, Ichimoto K, Matsunaga A, Tsuruoka T, Ishige M, Fuchigami T, Yamazaki T, Kishita Y, Kohda M, Imai-Okazaki A, Okazaki Y, Morioka I, Ohtake A, and Murayama K
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- Adolescent, Adult, Age of Onset, Child, Child, Preschool, DNA genetics, DNA, Mitochondrial genetics, Female, Humans, Infant, Japan epidemiology, Kaplan-Meier Estimate, Leigh Disease diagnosis, Magnetic Resonance Imaging, Male, Mutation genetics, Phenotype, Survival Rate, Young Adult, Leigh Disease genetics, Leigh Disease mortality
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Leigh syndrome is a major phenotype of mitochondrial diseases in children. With new therapeutic options being proposed, assessing the mortality and clinical condition of Leigh syndrome patients is crucial for evaluating therapeutics. As data are scarce in Japan, we analysed the mortality rate and clinical condition of Japanese Leigh syndrome patients that we diagnosed since 2007. Data from 166 Japanese patients diagnosed with Leigh syndrome from 2007 to 2017 were reviewed. Patients' present status, method of ventilation and feeding, and degree of disability as of April 2018 was analysed. Overall, 124 (74.7%) were living, 40 (24.1%) were deceased, and 2 (1.2%) were lost to follow-up. Median age of living patients was 8 years (1-39 years). Median length of disease course was 91 months for living patients and 23.5 months for deceased patients. Nearly 90% of deaths occurred by age 6. Mortality rate of patients with onset before 6 months of age was significantly higher than that of onset after 6 months. All patients with neonatal onset were either deceased or bedridden. MT-ATP6 deficiency caused by m.8993T>G mutation and MT-ND5 deficiency induced a severe form of Leigh syndrome. Patients with NDUFAF6, ECHS1, and SURF1 deficiency had relatively mild symptoms and better survival. The impact of onset age on prognosis varied across the genetic diagnoses. The clinical condition of many patients was poor; however, few did not require mechanical ventilation or tube-feeding and were not physically dependent. Early disease onset and genetic diagnosis may have prognostic value., (© 2020 The Authors. Journal of Inherited Metabolic Disease published by John Wiley & Sons Ltd on behalf of SSIEM.)
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- 2020
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9. Cerebral Autoregulation During Active Standing Test in Juvenile Patients with Instantaneous Orthostatic Hypotension.
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Ishii W, Fujita Y, Kawaguchi T, Kimura K, Fukuda A, Fuchigami T, and Morioka I
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- Adolescent, Child, Homeostasis, Humans, Oxyhemoglobins, Spectroscopy, Near-Infrared, Cerebrovascular Circulation physiology, Hypotension, Orthostatic physiopathology
- Abstract
Instantaneous orthostatic hypotension (INOH) is one of the main types of orthostatic dysregulation in children and adolescents. In patients with INOH arterial pressure drops considerably after active standing and is slow to recover. We investigated changes in cerebral oxygenation in the bilateral prefrontal cortex during an active standing test in juvenile INOH patients to evaluate changes in cerebral oxygen metabolism. We enrolled 82 INOH patients (mean age 13.8 ± 2.2 years, 52 mild and 30 severe patients) at Nihon University Itabashi Hospital from October 2013 to April 2018. We measured cerebral oxygenated hemoglobin, deoxygenated hemoglobin, and total hemoglobin levels in the bilateral prefrontal cortex using near-infrared spectroscopy during an active standing test. In severe INOH patients, cerebral oxygenation of the right prefrontal cortex remained constant when blood pressure dropped; however, de-oxy-Hb significantly increased. These findings confirm that there is asymmetrical autoregulation between the right and left prefrontal cortex.
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- 2020
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10. Infantile cerebral infarction caused by severe diabetic ketoacidosis in new-onset type 1 diabetes mellitus.
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Suzuki J, Fuchigami T, Kawamura K, Aoki M, Urakami T, and Morioka I
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- Age of Onset, Cerebral Infarction pathology, Female, Humans, Infant, Prognosis, Cerebral Infarction etiology, Diabetes Mellitus, Type 1 physiopathology, Diabetic Ketoacidosis complications
- Abstract
Background Diabetic ketoacidosis (DKA) is a common complication of type 1 diabetes mellitus (T1DM). Infants and children with new-onset T1DM may present with DKA, and the risk of cerebral edema is high in infantile DKA. What is new? Neurological deterioration during an episode of DKA is usually attributed to cerebral edema and cerebrovascular accidents. However, cerebral infarction is a very rare complication in infantile DKA. Case presentation We describe a rare case of infantile cerebral infarction caused by severe DKA in a patient with new-onset T1DM. Conclusions Cerebral infarction is an important intracranial complication in infantile DKA. Careful observation and treatment for DKA during the first 24 h of therapy are necessary in infants with new-onset T1DM because the risk of cerebral infarction is highest during this timeframe.
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- 2019
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11. Primary carnitine deficiency with severe acute hepatitis following rotavirus gastroenteritis.
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Ishige M, Fuchigami T, Furukawa M, Kobayashi H, Fujiki R, Ogawa E, Ishige N, Sasai H, Fukao T, Hashimoto K, Inamo Y, and Morioka I
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- Acute Disease, Cardiomyopathies virology, Female, Gastroenteritis virology, Hepatitis virology, Humans, Hyperammonemia virology, Infant, Muscular Diseases virology, Rotavirus pathogenicity, Cardiomyopathies etiology, Carnitine deficiency, Gastroenteritis complications, Gastroenteritis etiology, Hepatitis etiology, Hyperammonemia etiology, Muscular Diseases etiology, Rotavirus Infections complications
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Rotavirus infection is a major cause of gastroenteritis, which occurs mainly in children. Liver dysfunction due to rotavirus gastroenteritis has been reported; however, acute hepatitis due to this disease is very rare. We present a rare case in which rotavirus gastroenteritis led to sequential diagnosis of acute hepatitis and systemic primary carnitine deficiency (CDSP) in a 1-year-old girl. The patient's symptoms (hypoglycemia, hepatomegaly, and elevated levels of serum transaminases and creatinine kinase) suggested a steatosis causing liver dysfunction. She was initially considered to have a beta oxygenation defect or secondary carnitine deficiency caused by pivalic acid-containing antibiotics; however, repetitive carnitine analysis and free carnitine clearance measurement confirmed primary carnitine deficiency (carnitine transporter deficiency). Children with severe liver dysfunction due to rotavirus infection and presenting with liver steatosis should undergo blood acyl carnitine analysis to detect potential carnitine or other beta oxidation deficiencies, especially if newborn screening for these diseases is not available., (Copyright © 2019 Japanese Society of Chemotherapy and The Japanese Association for Infectious Diseases. Published by Elsevier Ltd. All rights reserved.)
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- 2019
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12. A case of confusional migraine with transient increased cerebral blood flow.
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Momoki E, Fuchigami T, Kasuga Y, Kimura K, Ishii W, Fukuda A, Fujita Y, and Morioka I
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- Child, Confusion physiopathology, Electroencephalography methods, Female, Humans, Magnetic Resonance Angiography methods, Magnetic Resonance Imaging methods, Migraine Disorders blood, Migraine Disorders complications, Tomography, Emission-Computed, Single-Photon methods, Cerebrovascular Circulation physiology, Migraine Disorders physiopathology
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Background: Confusional migraine is a rare type of migraine presenting as an acute confusional state. However, the mechanism of this confusional state remains unclear., Subject and Methods: We examined an 11-year-old girl with confusional migraine, using electroencephalography, brain magnetic resonance imaging, cerebrovascular magnetic resonance angiography, and single-photon emission computed tomography to investigate cerebral blood flow changes., Results: Our findings revealed vessel narrowing in the left middle and posterior cerebral artery territory, indicating vasospasm and suggesting that the confusion was caused by hypoperfusion. However, abnormal increased cerebral blood flow in the left middle and posterior cerebral artery territory was observed during the non-confusional state., Conclusion: The recorded cerebral blood flow changes are similar to those associated with migraine attacks, gradually changing from abnormally low to abnormally high during the confusional and post-confusional state., (Copyright © 2019 The Japanese Society of Child Neurology. Published by Elsevier B.V. All rights reserved.)
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- 2019
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13. Agraphia with reversible splenial corpus callosum lesion caused by hypoglycemia.
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Miyakawa Y, Fuchigami T, Aoki M, Mine Y, Suzuki J, Urakami T, and Takahashi S
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- Adolescent, Agraphia physiopathology, Agraphia therapy, Corpus Callosum physiopathology, Diabetes Mellitus, Type 1 complications, Diabetes Mellitus, Type 1 diagnostic imaging, Diabetes Mellitus, Type 1 physiopathology, Diabetes Mellitus, Type 1 therapy, Diffusion Magnetic Resonance Imaging, Female, Humans, Hypoglycemia physiopathology, Hypoglycemia therapy, Agraphia diagnostic imaging, Agraphia etiology, Corpus Callosum diagnostic imaging, Hypoglycemia complications, Hypoglycemia diagnostic imaging
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Background: Neurological manifestations caused by hypoglycemia range from reversible focal deficits and transient encephalopathy to irreversible coma or death. Recently, high signal intensity lesions in the splenium of the corpus callosum on diffusion-weighted magnetic resonance imaging were reported in adults experiencing hypoglycemia. However, patients presenting with agraphia are rare., Subject and Methods: We examined a 17-year-old left-handed female patient with type 1 diabetes who exhibited transient left agraphia with a reversible splenium lesion of the corpus callosum on diffusion-weighted imaging caused by hypoglycemia, which was improved with blood glucose management alone., Conclusion: This rare case indicates that agraphia, a sign of callosal disconnection syndrome, can result from a reversible splenial lesion of the corpus callosum caused by hypoglycemia., (Copyright © 2018 The Japanese Society of Child Neurology. Published by Elsevier B.V. All rights reserved.)
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- 2018
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14. Hypocarnitinemia Observed in an Infant Treated with Short-Term Administration of Antibiotic Containing Pivalic Acid.
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Nakazaki K, Ogawa E, Ishige M, Ishige N, Fuchigami T, and Takahashi S
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- Carbon chemistry, Carnitine analogs & derivatives, Humans, Hypoglycemia diagnosis, Infant, Male, Oxygen chemistry, Tandem Mass Spectrometry, Anti-Bacterial Agents adverse effects, Carnitine blood, Carnitine urine, Metabolic Diseases chemically induced, Metabolic Diseases diagnosis, Pentanoic Acids adverse effects
- Abstract
Carnitine is a water-soluble amino acid derivative required for β-oxidation of long-chain fatty acids. In carnitine cycle abnormalities and low carnitine states, fatty acid β-oxidation is inhibited during fasting, resulting in hypoglycemia. Pivalic acid is a substance used in prodrugs to increase absorption of parent drugs, and antibiotics containing pivalic acid are frequently used as wide spectrum antibiotics for pediatric patients in Japan. Pivalic acid released after absorption is conjugated with free carnitine to form pivaloylcarnitine, which is then excreted in urine. As a consequence, long-term administration of pivalic acid containing antibiotics has been associated with depletion of free carnitine, inhibition of energy production and subsequent hypoglycemia. Here we report a case of a 23-month-old boy treated with an antibiotic containing pivalic acid for 3 days for upper respiratory tract infection. Laboratory data at referral indicated hypoglycemia, decreased free carnitine and elevated five-carbon acylcarnitine. Isomer separation confirmed the major component of increased five-carbon acylcarnitine to be pivaloylcarnitine, thereby excluding the possibility of a genetic metabolic disorder detected with similar acylcarnitine profile. The level of carnitine was normal when the antibiotic was not administered. Our case shows that the use of antibiotics containing pivalic acid in young children requires consideration of hypocarnitinemia, even with short-term administration.
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- 2018
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15. Clinical validity of biochemical and molecular analysis in diagnosing Leigh syndrome: a study of 106 Japanese patients.
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Ogawa E, Shimura M, Fushimi T, Tajika M, Ichimoto K, Matsunaga A, Tsuruoka T, Ishige M, Fuchigami T, Yamazaki T, Mori M, Kohda M, Kishita Y, Okazaki Y, Takahashi S, Ohtake A, and Murayama K
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- Adolescent, Adult, Asian People, Child, Electron Transport genetics, Female, Fibroblasts physiology, Humans, Leigh Disease genetics, Male, Mitochondria genetics, Mitochondrial Proton-Translocating ATPases genetics, Muscle, Skeletal physiology, Mutation genetics, Oxygen Consumption genetics, Young Adult, Leigh Disease diagnosis
- Abstract
Leigh syndrome (LS) is a progressive neurodegenerative disorder of infancy and early childhood. It is clinically diagnosed by typical manifestations and characteristic computed tomography (CT) or magnetic resonance imaging (MRI) studies. Unravelling mitochondrial respiratory chain (MRC) dysfunction behind LS is essential for deeper understanding of the disease, which may lead to the development of new therapies and cure. The aim of this study was to evaluate the clinical validity of various diagnostic tools in confirming MRC disorder in LS and Leigh-like syndrome (LL). The results of enzyme assays, molecular analysis, and cellular oxygen consumption rate (OCR) measurements were examined. Of 106 patients, 41 were biochemically and genetically verified, and 34 had reduced MRC activity but no causative mutations. Seven patients with normal MRC complex activities had mutations in the MT-ATP6 gene. Five further patients with normal activity in MRC were identified with causative mutations. Conversely, 12 out of 60 enzyme assays performed for genetically verified patients returned normal results. No biochemical or genetic background was confirmed for 19 patients. OCR was reduced in ten out of 19 patients with negative enzyme assay results. Inconsistent enzyme assay results between fibroblast and skeletal muscle biopsy samples were observed in 33% of 37 simultaneously analyzed cases. These data suggest that highest diagnostic rate is reached using a combined enzymatic and genetic approach, analyzing more than one type of biological materials where suitable. Microscale oxygraphy detected MRC impairment in 50% cases with no defect in MRC complex activities.
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- 2017
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16. A case of classical maple syrup urine disease that was successfully managed by living donor liver transplantation.
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Takano C, Ishige M, Ogawa E, Usui H, Kagawa R, Tajima G, Fujiki R, Fukao T, Mizuta K, Fuchigami T, and Takahashi S
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- Child, Preschool, Female, Humans, Magnetic Resonance Imaging, Liver Transplantation methods, Living Donors, Maple Syrup Urine Disease diagnostic imaging, Maple Syrup Urine Disease surgery
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Classical MSUD is often fatal without appropriate medical interventions because of metabolic crisis. There are numerous reports suggesting the therapeutic potential of deceased donor liver transplantation for MSUD. However, the usefulness of LDLT for MSUD is unknown. We report a case of classical MSUD, which was successfully managed by LDLT from the patient's father at 1 year of age. Abnormal brain findings, which were cured with effective treatment, gradually disappeared after LDLT. The patient then developed normally. Findings from this case suggest the importance of LDLT for maintaining low leucine levels and subsequent normal neurological development. Although LDLT involves a modest surgical insult, LDLT with a related donor achieves acceptable leucine levels for life., (© 2017 John Wiley & Sons A/S. Published by John Wiley & Sons Ltd.)
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- 2017
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17. Familial unilateral carpal bone dysplasia in mother and daughter.
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Fuchigami T, Fujita Y, Oyama M, Inamo Y, Takahashi S, Yamaguchi T, Miyazaki O, and Nishimura G
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- Adult, Child, Preschool, Female, Humans, Magnetic Resonance Imaging, Mothers, Nuclear Family, Phenotype, Radiography, Bone Diseases, Developmental diagnosis, Carpal Bones abnormalities
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- 2017
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18. Acute Focal Bacterial Nephritis Associated With Central Nervous System Manifestations: A Report of 2 Cases and Review of the Literature.
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Kasuga Y, Fuchigami T, Fukuda A, Takahashi S, Murai T, Yonezawa R, Miyashita M, Kohira R, Fujita Y, and Takahashi S
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- Anti-Bacterial Agents therapeutic use, Bacterial Infections drug therapy, Brain Diseases drug therapy, Central Nervous System diagnostic imaging, Cerebrospinal Fluid chemistry, Cerebrospinal Fluid cytology, Child, Preschool, Diffusion Magnetic Resonance Imaging methods, Female, Humans, Nephritis diagnosis, Tomography, X-Ray Computed methods, Treatment Outcome, Bacterial Infections complications, Brain Diseases complications, Central Nervous System pathology, Encephalitis complications, Nephritis microbiology
- Abstract
Acute focal bacterial nephritis (AFBN) is a localized bacterial infection of the kidney presenting as an inflammatory mass without frank abscess formation. In children, most patients with AFBN present with nonspecific conditions, such as fever, vomiting, and abdominal pain. A small number of reported cases are accompanied by neurological symptoms, including meningeal irritation, unconsciousness, and seizures. We experienced 2 rare cases of AFBN associated with central nervous system lesions. The first case was a 3-year-old girl who had neurological symptoms, including unconsciousness and seizures, with AFBN associated with acute reversible encephalopathy. The second case was a 5-year-old girl who had neurological symptoms, including unconsciousness, with AFBN accompanied by clinically mild encephalitis/encephalopathy with a reversible splenial lesion.
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- 2017
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19. Severe Acute Subdural Hemorrhages in a Patient with Glutaric Acidemia Type 1 under Recommended Treatment.
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Ishige M, Fuchigami T, Ogawa E, Usui H, Kohira R, Watanabe Y, and Takahashi S
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- Amino Acid Metabolism, Inborn Errors complications, Amino Acid Metabolism, Inborn Errors diagnostic imaging, Brain Diseases, Metabolic complications, Brain Diseases, Metabolic diagnostic imaging, Craniocerebral Trauma complications, Craniocerebral Trauma diagnostic imaging, Hematoma, Subdural, Acute complications, Hematoma, Subdural, Acute diagnostic imaging, Humans, Infant, Male, Treatment Outcome, Amino Acid Metabolism, Inborn Errors surgery, Brain Diseases, Metabolic surgery, Craniocerebral Trauma surgery, Glutaryl-CoA Dehydrogenase deficiency, Hematoma, Subdural, Acute surgery, Severity of Illness Index
- Abstract
Glutaric acidemia type 1 is a rare autosomal recessive disease caused by a deficiency of glutaryl-CoA dehydrogenase. Previous studies have reported subdural hemorrhage in untreated patients with glutaric acidemia type 1. However, there is only one report of severe acute subdural hemorrhage after minor head trauma in a patient with glutaric acidemia type 1 under guideline-recommended treatment. We report a second case of life-threatening severe acute subdural hemorrhage after a minor head trauma in a patient with glutaric acidemia type 1. This patient was previously diagnosed by newborn screening, and treatment began at 25 days of age. Early diagnosis and guideline-recommended treatment produce better outcomes for patients with glutaric acidemia type 1, although the risk of subdural hemorrhage remains., (© 2016 S. Karger AG, Basel.)
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- 2017
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20. Extrapontine myelinolysis associated with severe hypernatremia in infancy.
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Aoki R, Morimoto T, Takahashi Y, Saito H, Fuchigami T, and Takahashi S
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- Humans, Hypernatremia blood, Hypernatremia diagnosis, Infant, Magnetic Resonance Imaging methods, Male, Myelinolysis, Central Pontine diagnosis, Severity of Illness Index, Hypernatremia etiology, Myelinolysis, Central Pontine complications, Sodium blood
- Abstract
Extrapontine myelinolysis (EPM) is an uncommon disorder in children, with few pediatric cases reported to date. We report the first case of an infant with EPM without central pontine myelinolysis (CPM) presenting with severe hypernatremia. On admission, the infant had impaired consciousness, mild dehydration, and severe hypernatremia (190 mmol/L). The following day, the patient developed abnormal involuntary movements. Brain magnetic resonance imaging (MRI) confirmed EPM without CPM. He recovered without sequelae, and clinical examinations were within normal limits approximately 6 months after discharge. Brain MRI at 1 year after onset showed complete disappearance of the previous EPM regions. To the best of our knowledge, this represents the youngest patient with EPM without CPM presenting with severe hypernatremia. Given that treatment for osmotic demyelination syndrome (ODS) is yet to be established, preventing the development of ODS is crucial., (© 2016 Japan Pediatric Society.)
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- 2016
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21. Severe hypoglycemic encephalopathy due to hypoallergenic formula in an infant.
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Ogawa E, Ishige M, Takahashi Y, Kodama H, Fuchigami T, and Takahashi S
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- Brain Diseases blood, Brain Diseases diagnosis, Female, Humans, Hypoglycemia blood, Hypoglycemia diagnosis, Infant, Magnetic Resonance Imaging, Blood Glucose analysis, Brain diagnostic imaging, Brain Diseases etiology, Hypoglycemia complications, Infant Formula adverse effects
- Abstract
A 7-month-old girl was brought to hospital due to vomiting. Upon admission, she was in a convulsive state and stupor with extremely low blood glucose. Head computed tomography showed brain edema, and comprehensive treatment for acute encephalopathy was initiated immediately. Severe hypoglycemia, metabolic acidosis, elevation of ammonia and serum transaminases and creatine kinase suggested metabolic decompensation. Infusion of a high-glucose solution containing vitamins, biotin, and l-carnitine resolved the metabolic crisis quickly, but brain damage was irreversible. She was found to have been fed exclusively on a hypoallergenic formula (HF) for 7 months, although she was found later to be non-allergic. Evidence of inborn metabolic diseases was absent, therefore biotin deficiency and carnitine deficiency were concluded to be a consequence of reliance on a HF for a prolonged period. Health-care professionals should warn parents of the consequences of using HF., (© 2016 Japan Pediatric Society.)
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- 2016
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22. Affinity for music in Wolf-Hirschhorn syndrome: two case reports.
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Arakawa C, Fujita Y, Fuchigami T, Kawamura Y, Ishii W, Endo A, Kohira R, and Takahashi S
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- Adult, Child, Female, Humans, Music Therapy, Wolf-Hirschhorn Syndrome therapy, Young Adult, Music psychology, Wolf-Hirschhorn Syndrome psychology
- Abstract
Background: Wolf-Hirschhorn syndrome is a congenital malformation syndrome resulting from deletion of the short arm of chromosome 4. Individuals with Wolf-Hirschhorn syndrome may have a "Greek warrior helmet" appearance, growth retardation, developmental delay, muscular hypotonia, epilepsy, and difficulty with language including verbal communication. An affinity for music has not previously been reported in these patients., Patients: We describe two patients with Wolf-Hirschhorn syndrome who both have a strong affinity for music. One patient is a 20-year-old woman who likes to listen to music all day and can hum many tunes. The other patient is a 9-year-old girl who is calmed by music and received music therapy, with subsequent improvement in her communication skills (eye contact, joint attention, and vocalizations to request music)., Conclusions: Individuals with Wolf-Hirschhorn syndrome may have a strong affinity for music and may benefit from music therapy. Additional studies are needed to investigate the interest in music in individuals with Wolf-Hirschhorn syndrome., (Copyright © 2014 Elsevier Inc. All rights reserved.)
- Published
- 2014
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23. A 3-year-old boy with Guillain-Barré syndrome and encephalitis associated with Mycoplasma pneumoniae infection.
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Hanzawa F, Fuchigami T, Ishii W, Nakajima S, Kawamura Y, Endo A, Arakawa C, Kohira R, Fujita Y, and Takahashi S
- Subjects
- Child, Preschool, Humans, Male, Encephalitis microbiology, Guillain-Barre Syndrome microbiology, Pneumonia, Mycoplasma complications
- Abstract
Mycoplasma pneumoniae is a common cause of respiratory tract illness in children. Among the most common extrapulmonary manifestations are disorders of the central nervous system, including meningitis, meningoencephalitis, cerebellitis, polyneuropathy, acute disseminated encephalomyelitis, and Guillain-Barré syndrome. Guillain-Barré syndrome, also known as acute inflammatory demyelinating polyradiculoneuropathy, is an acute-onset, immune-mediated disorder of the peripheral nervous system. The central nervous system is usually intact in patients with Guillain-Barré syndrome. However, there have been some reports of an association of Guillain-Barré syndrome with central nervous system involvement in children. We report a 3-year-old boy with M. pneumoniae infection associated with Guillain-Barré syndrome and encephalitis. Both serum anti-GM1 ganglioside (IgG and IgM) and anti-galactocerebroside IgG antibodies were detected in our patient: the former in the earlier stage of the disease, and the latter in the later stage. We speculate that anti-GM1 ganglioside was associated more with encephalitis, and anti-galactocerebroside antibody was associated more with GBS in our case. Our patient is the youngest report of Guillain-Barré syndrome with central nervous system involvement, and the first report of a pediatric patient with associated M. pneumoniae infection. Such cases are rarely reported, but highlight the need for awareness of the association of the infection with Guillain-Barré syndrome with central nervous system involvement., (Copyright © 2013 Japanese Society of Chemotherapy and The Japanese Association for Infectious Disease. Published by Elsevier Ltd. All rights reserved.)
- Published
- 2014
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24. Effect of dalteparin, a low-molecular-weight heparin, as adjunctive therapy in patients with Kawasaki disease: a retrospective study.
- Author
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Inamo Y, Saito K, Hasegawa M, Hayashi R, Nakamura T, Abe O, Ishikawa T, Yoshino Y, Hashimoto K, and Fuchigami T
- Subjects
- Chemotherapy, Adjuvant, Child, Preschool, Coronary Artery Disease etiology, Coronary Artery Disease prevention & control, Female, Humans, Immunoglobulins, Intravenous therapeutic use, Infant, Male, Mucocutaneous Lymph Node Syndrome complications, Pilot Projects, Retrospective Studies, Anticoagulants therapeutic use, Dalteparin therapeutic use, Mucocutaneous Lymph Node Syndrome drug therapy
- Abstract
Background: Dalteparin, a low-molecular-weight heparin, has anticoagulant and anti-angiogenic activity. This study investigated whether dalteparin reduced coronary artery lesion (CAL) prevalence, and resistance to intravenous immunoglobulin (IVIG) therapy in Kawasaki disease (KD)., Methods: This retrospective study comprised two parts. In the first cohort, 126 patients with KD (68 male, 58 female; median age: 22 months, range: 1-67 months) admitted to Nihon University Nerima-Hikarigaoka Hospital from January 2004 to June 2008, received either dalteparin 75 IU/kg/day, IVIG 400 mg/kg/day for 5 consecutive days, and aspirin 30 mg/kg/day, or dalteparin 75 IU/kg/day and aspirin 30 mg/kg/day, until clinical improvement. Control data came from the 2005-6 Nationwide KD survey. In the second cohort, 112 patients with KD (59 male, 53 female; median age: 19 months, range: 1-66 months) admitted from June 2010 to February 2012, received either dalteparin 75 IU/kg/day, IVIG 2.0 g/kg over 12 h, and aspirin 30 mg/kg/day, or dalteparin 75 IU/kg/day and aspirin 30 mg/kg/day. Control data came from the 2009-10 Nationwide KD survey. No patients enrolled in the nationwide surveys received dalteparin. All patients at our institution were given dalteparin in their combination therapy., Results: A comparison of the first cohort with controls in the nationwide survey showed that the prevalence of initial administration of IVIG was 80.2% versus 86.0%; the rate of additional IVIG administration was 7.1% versus 14.0% (p = 0.03); CAL prevalence in the acute period was 4.8% versus 11.9% (p < 0.01); and the prevalence of cardiovascular sequelae was 0% versus 3.8% (p < 0.05). A comparison of the second cohort with controls in the nationwide survey showed that the rate of initial administration of IVIG was 92.9% versus 89.5%; the rate of additional IVIG administration was 8.9% versus 17.1% (p = 0.02); the prevalence of resistance to IVIG was 3.6% versus 14.9% (p < 0.001); and CAL prevalence in the acute period was 2.7% versus 8.6% (p = 0.03)., Conclusions: This study found that adjunctive dalteparin was associated with a lower prevalence of IVIG resistance and CAL in young children with KD., Trial Registration Umin-Ctr: UMIN000010349.
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- 2014
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25. Asymmetrical changes in cerebral blood oxygenation induced by an active standing test in children with postural tachycardia syndrome.
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Kamiyama Y, Fujita Y, Fuchigami T, Kamiyama H, Takahashi S, and Sakatani K
- Subjects
- Adolescent, Child, Female, Humans, Male, Brain blood supply, Oxygen blood, Postural Orthostatic Tachycardia Syndrome blood, Posture
- Abstract
Near-infrared spectroscopy enables recognition of various brain conditions based on certain factors, such as oxygenated hemoglobin (oxy-Hb). Since July 2012, we have been trying to determine the mechanisms of autonomic function in Japanese children with orthostatic intolerance (also called orthostatic dysregulation) in Nihon University Itabashi Hospital in Tokyo, Japan. A total of 23 children aged 7-16 years diagnosed with postural tachycardia syndrome (POTS), a subtype of orthostatic dysregulation, were enrolled in the study. We evaluated the relation between asymmetry in frontal cortex activity and the automatic nervous system and compared oxy-Hb changes in the right and left frontal cortices during an active standing test. We observed that during active standing oxy-Hb decreased in the frontal cortex. The oxy-Hb changes were asymmetrical, with a significantly larger decrease in the left frontal cortex than in the right frontal cortex, suggesting that tachycardia during active standing in POTS patients might be caused by activation of the right frontal cortex, which induces sympathetic nervous system activity.
- Published
- 2014
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26. Changes in cerebral blood oxygenation induced by active standing test in children with POTS and NMS.
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Endo A, Fujita Y, Fuchigami T, Takahashi S, Mugishima H, and Skatani K
- Subjects
- Child, Female, Humans, Male, Cerebrovascular Circulation, Oxygen blood, Postural Orthostatic Tachycardia Syndrome blood, Posture, Syncope blood
- Abstract
Orthostatic dysregulation (OD) has been classified into subtypes by heart rate and blood pressure; however, the hemodynamics of brains have not yet been revealed. Therefore, we investigated changes in cerebral blood flow and oxygenation during an active standing test to clarify the pathophysiology of two subtypes: postural tachycardia syndrome (POTS) and neurally mediated syncope (NMS). We studied 31 children (15 boys, 16 girls; mean age, 14.0 ± 1.7 years) who presented with OD at the Department of Pediatrics and Child Health, Nihon University School of Medicine between 2009 and 2011. OD was diagnosed using the Japanese clinical guidelines for juvenile orthostatic dysregulation. After a 10-min resting period in the supine position, patients were asked to quickly stand up and keep upright for 10 min. Cerebral blood flow and cerebral oxygenation were measured using transcranial Doppler sonography and near-infrared spectroscopy. POTS showed a significant decrease of oxy-Hb and resistance index (RI), suggesting transient ischemia with maintainable cerebral autoregulation. NMS showed a decrease of oxy-Hb and an increase of RI, suggesting ischemia and impairment of autoregulation.
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- 2014
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27. [Clinical efficacy and pharmacokinetics of lamotrigine for childhood-onset intractable epilepsy].
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Ishii W, Fujita Y, Momoki E, Imai Y, Endo A, Arakawa C, Kohira R, Fuchigami T, and Mugishima H
- Subjects
- Adolescent, Age of Onset, Child, Drug Therapy, Combination methods, Female, Humans, Lamotrigine, Male, Treatment Outcome, Triazines therapeutic use, Valproic Acid administration & dosage, Valproic Acid therapeutic use, Seizures drug therapy, Triazines pharmacokinetics
- Abstract
Objective: We investigated the clinical efficacy and pharmacokinetics of lamotrigine (LTG) as an add-on therapy in childhood-onset intractable epilepsy., Methods: We reviewed the charts of 28 outpatients who had received LTG as an add-on therapy. The data collected included epilepsy type, seizure frequency, concomitant anti-epileptic drugs, dosage of LTG and LTG serum levels. Furthermore, we reviewed the relationship between the LTG serum levels (microg/ml) and dosage of LTG (mg/kg/day), as well as the relationship between the LTG serum levels (microg/ml) and clinical efficacy in the following 2 groups:the valproate sodium (VPA) combination group and the non-VPA combination group., Results: A reduction of 50% or more in seizure frequency was observed in 10 patients. In addition, there was a high correlation between the LTG serum levels and the dosage of LTG in each group. In the VPA combination group, the average of LTG serum levels in patients with adequate therapeutic response (50% reduction in seizure frequency) was higher than that in patients without adequate therapeutic response. In the non-VPA combination group, the average LTG serum level in adequate response patients was lower than that in patients without adequate therapeutic response. However, the epilepsy types of adequate response patients differed in the two groups., Conclusions: The LTG serum level is predictable based on the dosage of LTG. It was judged that the effective blood concentration of LTG differed when used with VPA, although factors other than the combined use of VPA should have been taken into consideration also.
- Published
- 2014
28. A 4-year-old girl with clinically mild encephalopathy with a reversible splenial lesion associated with rotavirus infection.
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Fuchigami T, Goto K, Hasegawa M, Saito K, Kida T, Hashimoto K, Fujita Y, Inamo Y, and Kuzuya M
- Subjects
- Brain Diseases virology, Child, Preschool, Diffusion Magnetic Resonance Imaging, Electroencephalography, Female, Gastroenteritis virology, Humans, Rotavirus genetics, Rotavirus Infections virology, Brain Diseases pathology, Corpus Callosum pathology, Gastroenteritis complications, Rotavirus isolation & purification, Rotavirus Infections complications
- Abstract
Rotavirus is a common cause of severe gastroenteritis in children. It is known that rotavirus gastroenteritis may be accompanied by neurological manifestations, including encephalitis/encephalopathy and seizures. We report a case of a 4-year-old girl with clinically mild encephalopathy with a reversible splenial lesion associated with rotavirus infection. She was admitted to our hospital because of reduced level of consciousness, seizures, diarrhea, and vomiting. Fecal rotavirus antigen testing was positive. Cell counts in the cerebrospinal fluid (CSF) were normal. She had a normal serum sodium level on admission. Brain computed tomography showed no cerebral edema. However, electroencephalography showed generalized high-voltage slow waves, and diffusion-weighted magnetic resonance imaging demonstrated a transient abnormality in the splenium of the corpus callosum. We diagnosed clinically mild encephalopathy with a reversible splenial lesion associated with rotavirus infection. She recovered well and exhibited no neurological sequelae. Rotavirus RNA and antigen were not detected in the CSF, suggesting that the reversible splenial change was caused by indirect effects on the central nervous system subsequent to viral infection. Her normal serum sodium level indicates that this change can occur without hyponatremia.
- Published
- 2013
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29. Acute encephalopathy with pandemic (H1N1) 2009 virus infection.
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Fuchigami T, Imai Y, Hasegawa M, Ishii W, Endo A, Arakawa C, Kohira R, Hashimoto K, Fujita Y, Inamo Y, and Mugishima H
- Subjects
- Child, Child, Preschool, DNA, Viral analysis, Electroencephalography, Encephalitis, Viral diagnosis, Encephalitis, Viral virology, Female, Humans, Influenza, Human epidemiology, Influenza, Human virology, Japan epidemiology, Magnetic Resonance Imaging, Male, Retrospective Studies, Tomography, X-Ray Computed, Encephalitis, Viral epidemiology, Influenza A Virus, H1N1 Subtype genetics, Influenza, Human complications, Pandemics
- Abstract
Objectives: In April 2009, a novel influenza A (H1N1) pdm virus was identified in Mexico and spread quickly around the world. However, the clinical features of acute encephalopathy associated with 2009 pandemic influenza have not yet been elucidated., Methods: We treated 8 patients (3 boys and 5 girls) aged 4 to 11 years (average age, 8 y 3 months) with influenza virus-associated encephalopathy, who presented at our 2 hospitals between July 2009 and March 2010. We investigated the clinical characteristics, treatments, and outcomes in the patients., Results: In all patients, brain computed tomography showed mild to severe diffuse cerebral edema, and electroencephalography revealed diffuse high-voltage slow waves. They were all treated with oseltamivir and methylprednisolone pulse therapy. Six patients recovered without any sequelae; however, the remaining 2 had residual neurological sequelae. These 2 patients presented with severe disturbance of consciousness, and their central nervous system symptoms appeared within 12 hours after the onset of fever. One patient had periventricular leukomalacia and symptomatic epilepsy by perinatal brain hypoxia, and the other patient had 1 complex febrile and 2 febrile seizures., Conclusions: This study showed that patients with influenza-associated encephalopathy caused by influenza A (H1N1) pdm infection were all older than those with seasonal influenza. Underlying neurological disease or history may be associated with poor prognosis.
- Published
- 2012
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30. A 7-year-old girl with subcutaneous emphysema, pneumomediastinum, pneumothorax, and pneumoretroperitoneum caused by Mycoplasma pneumoniae pneumonia.
- Author
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Inamo Y, Ishizuka Y, Hashimoto K, Hasegawa M, Saito K, Kida T, Nakamura A, Ishikawa T, and Fuchigami T
- Subjects
- Child, Female, Humans, Mediastinal Emphysema diagnostic imaging, Mediastinal Emphysema pathology, Mycoplasma pneumoniae, Pneumonia, Mycoplasma diagnostic imaging, Pneumonia, Mycoplasma microbiology, Pneumothorax diagnostic imaging, Radiography, Retropneumoperitoneum diagnostic imaging, Mediastinal Emphysema etiology, Pneumonia, Mycoplasma complications, Pneumothorax etiology, Retropneumoperitoneum etiology, Subcutaneous Emphysema diagnostic imaging, Subcutaneous Emphysema etiology
- Abstract
A 7-year-old girl presented with subcutaneous emphysema, pneumomediastinum (PM), pneumoretroperitoneum, and pneumothorax caused by Mycoplasma pneumoniae (MP). The patient had been treated with clarithromycin for pneumonia at another hospital; however, her condition deteriorated and complications developed. Soon after admission to our hospital, we started the patient on minocycline and prednisolone, and the complications improved promptly. Laboratory data showed serum ferritin and urinary beta-2-microglobulin levels were greatly elevated. We therefore speculated that the patient might have underlying hypercytokinemia. Prednisolone is an effective treatment for hypercytokinemia. We therefore recommend prednisolone treatment for cases of severe M. pneumoniae pneumonia that do not respond to antimicrobial agents.
- Published
- 2012
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31. Posterior reversible encephalopathy syndrome in childhood: report of four cases and review of the literature.
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Endo A, Fuchigami T, Hasegawa M, Hashimoto K, Fujita Y, Inamo Y, and Mugishima H
- Subjects
- Adolescent, Anemia, Aplastic surgery, Anticonvulsants therapeutic use, Antihypertensive Agents therapeutic use, Child, Cyclosporine adverse effects, Early Diagnosis, Epilepsy, Tonic-Clonic drug therapy, Epilepsy, Tonic-Clonic etiology, Female, Glomerulonephritis complications, Humans, Hypertensive Encephalopathy drug therapy, Hypertensive Encephalopathy pathology, IgA Vasculitis complications, Immunosuppressive Agents adverse effects, Intellectual Disability etiology, Male, Nephrotic Syndrome complications, Posterior Leukoencephalopathy Syndrome chemically induced, Posterior Leukoencephalopathy Syndrome drug therapy, Posterior Leukoencephalopathy Syndrome etiology, Posterior Leukoencephalopathy Syndrome pathology, Postoperative Complications chemically induced, Postoperative Complications diagnosis, Stem Cell Transplantation, Streptococcal Infections complications, Vision Disorders etiology, Hypertensive Encephalopathy diagnosis, Magnetic Resonance Imaging methods, Posterior Leukoencephalopathy Syndrome diagnosis
- Abstract
Background: Posterior reversible encephalopathy syndrome (PRES) is a recently described disorder with typical radiological findings of bilateral gray and white matter abnormalities in the posterior regions of the cerebral hemispheres and cerebellum. Its clinical symptoms include headache, decreased alertness, mental abnormalities such as confusion, diminished spontaneity of speech, and changed behavior ranging from drowsiness to stupor, seizures, vomiting, and abnormalities of visual perception such as cortical blindness. In this study, the clinical and radiological findings of 4 children with this syndrome due to a variety of conditions are reported., Methods: The records of 4 children with a diagnosis of PRES were retrospectively analyzed., Results: PRES is associated with a disorder of cerebrovascular autoregulation of multiple etiologies. Four patients with PRES who had primary diagnoses of severe aplastic anemia, nephritic syndrome, Henoch-Schönlein purpura, and acute poststreptococcal glomerulonephritis are presented. This syndrome has been described in numerous medical conditions, including hypertensive encephalopathy, eclampsia, and with the use of immunosuppressive drugs., Conclusions: Early recognition of PRES as a complication during different diseases and therapies in childhood may facilitate precise diagnosis and appropriate treatment.
- Published
- 2012
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32. Liver-specific mitochondrial respiratory chain complex I deficiency in fatal influenza encephalopathy.
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Arakawa C, Endo A, Kohira R, Fujita Y, Fuchigami T, Mugishima H, Ohtake A, Murayama K, Mori M, Miyata R, and Hatai Y
- Subjects
- Child, Preschool, Electron Transport Complex I deficiency, Electron Transport Complex I metabolism, Humans, Male, Electron Transport Complex IV metabolism, Encephalitis Viruses pathogenicity, Influenza, Human complications, Liver enzymology, Mitochondrial Diseases complications, Mitochondrial Diseases metabolism, Mitochondrial Diseases pathology
- Abstract
We report on a 4-year-old boy who died from influenza encephalopathy. The clinical course and microscopic findings of the autopsied liver were compatible with Reye's syndrome. We examined the mitochondrial respiratory chain function by blue native polyacrylamide gel electrophoresis (BN-PAGE), western blotting, and respiratory chain enzyme activity assays. The activity of liver respiratory chain complex (CO) I was markedly decreased (7.2% of the respective control activity); whereas, the other respiratory chain complex activities were substantially normal (CO II, 57.9%; CO III, 122.3%; CO IV, 161.0%). The activities of CO I-IV in fibroblasts were normal (CO I, 82.0%; CO II, 83.1%; CO III, 72.9%; CO IV, 97.3%). The patient was diagnosed with liver-specific complex I deficiency. This inborn disorder may have contributed to the fatal outcome. We propose that relying only on fibroblast respiratory chain complex activities may lead to the misdiagnosis of liver-specific complex I deficiency., (Copyright © 2011 The Japanese Society of Child Neurology. Published by Elsevier B.V. All rights reserved.)
- Published
- 2012
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33. Effectiveness of imiquimod 5% cream for treatment of perianal warts in a 28-month-old child.
- Author
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Masuko T, Fuchigami T, Inadomi T, Inamo Y, and Hashimoto K
- Subjects
- Child, Preschool, Condylomata Acuminata pathology, Female, Humans, Imiquimod, Ointments, Adjuvants, Immunologic administration & dosage, Aminoquinolines administration & dosage, Condylomata Acuminata drug therapy
- Published
- 2011
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34. Tipepidine hibenzate intoxication.
- Author
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Imai Y, Ishii W, Endo A, Arakawa C, Kohira R, Fujita Y, Fuchigami T, and Mugishima H
- Subjects
- Child, Preschool, Drug Overdose, Humans, Male, Antitussive Agents poisoning, Delirium chemically induced, Piperidines poisoning
- Published
- 2011
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35. Serum vitamin D concentrations and associated severity of acute lower respiratory tract infections in Japanese hospitalized children.
- Author
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Inamo Y, Hasegawa M, Saito K, Hayashi R, Ishikawa T, Yoshino Y, Hashimoto K, and Fuchigami T
- Subjects
- Acute Disease, Breast Feeding, Bronchiolitis epidemiology, Female, Hospitalization, Humans, Infant, Male, Radioimmunoassay, Respiration, Artificial, Respiratory Tract Infections epidemiology, Respiratory Tract Infections therapy, Retrospective Studies, Vitamin D analogs & derivatives, Vitamin D Deficiency epidemiology, Respiratory Tract Infections blood, Vitamin D blood
- Abstract
Background: Vitamin D is an immunomodulatory molecule related to innate immunity that may contribute to the increased occurrence of acute lower respiratory infection (ALRI) in children, one of the most common reasons for hospitalization and intensive care unit admission. In the present study, the association between vitamin D deficiency and the severity of respiratory infection was evaluated by determining serum concentrations of 25-hydroxyvitamin D (25(OH)D) in a group of hospitalized children with ALRI., Methods: Of the 28 children admitted to Nihon University Nerima-Hikarigaoka Hospital with ALRI over the period November 2008–May 2009, 26 were diagnosed as having bronchiolitis and two were found to have pneumonia. A competitive protein binding radioimmunoassay was used to determine serum 25(OH)D concentrations., Results: Mean 25(OH)D concentrations in breast-fed children with ALRI (n = 7) were significantly lower than those in children with ALRI who were bottle fed/weaned (n = 6) or on a regular diet (n = 15; 14.6 ± 9.7, 28.9 ± 6.9 and 24.6 ± 8.8 ng/mL, respectively). There was a significant correlation between vitamin D deficiency (<15 ng/mL) and the need for supplementary oxygen and ventilator management., Conclusion: Significantly more children with ALRI who needed supplementary oxygen and ventilator management were vitamin D deficient. These findings suggest that the immunomodulatory properties of vitamin D may influence the severity of ALRI.
- Published
- 2011
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36. Pandemic (H1N1) 2009-associated pneumonia in children, Japan.
- Author
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Hasegawa M, Okada T, Sakata H, Nakayama E, Fuchigami T, Inamo Y, Mugishima H, Tajima T, Iwata S, Morozumi M, Ubukata K, Watanabe H, and Takahashi T
- Subjects
- Antiviral Agents therapeutic use, Child, Disease Outbreaks, Female, Hospitalization statistics & numerical data, Humans, Influenza, Human virology, Japan epidemiology, Male, Pneumonia, Viral diagnosis, Pneumonia, Viral drug therapy, Pneumonia, Viral microbiology, Risk Factors, Time Factors, Influenza A Virus, H1N1 Subtype pathogenicity, Influenza, Human complications, Influenza, Human epidemiology, Pandemics, Pneumonia, Viral epidemiology
- Abstract
To describe clinical aspects of pandemic (H1N1) 2009 virus-associated pneumonia in children, we studied 80 such children, including 17 (21%) with complications, who were admitted to 5 hospitals in Japan during August-November 2009 after a mean of 2.9 symptomatic days. All enrolled patients recovered (median hospitalization 6 days). Timely access to hospitals may have contributed to favorable outcomes.
- Published
- 2011
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37. Improvement of intractable childhood epilepsy following acute viral infection.
- Author
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Fujita Y, Imai Y, Ishii W, Endo A, Arakawa C, Kohira R, Fuchigami T, Okubo O, and Mugishima H
- Subjects
- Acute Disease, Child, Preschool, Electroencephalography, Epilepsies, Myoclonic physiopathology, Female, Humans, Infant, Magnetic Resonance Imaging, Male, Spasms, Infantile physiopathology, Virus Diseases virology, Epilepsies, Myoclonic drug therapy, Epilepsies, Myoclonic etiology, Spasms, Infantile drug therapy, Spasms, Infantile etiology, Virus Diseases complications
- Abstract
In this study, we report 11 patients with intractable childhood epilepsy that improved following acute viral infection. The patients were 8 boys and 3 girls. Six of the 11 children were diagnosed as West syndrome (5 of the symptomatic type and 1 of the cryptogenic type). The remaining 5 children were myoclonic seizures. The patients became seizure free within 6 days following acute viral infections without an exchange or addition of antiepileptic drugs (AEDs). The types of acute viral infections were Exanthema subitum (Roseola infantum) in 5 patients, Rotavirus gastroenteritis in 2 patients, Measles infection in 2 patients, Herpetic stomatitis in 1 patient and Common cold in the remaining patient. Salaam seizures and/or tonic spasms disappeared within 6 days after the onset of viral infections, and hypsarrhythmia evolved to localized spikes on electroencephalography (EEG) in the patients with West syndrome. Epileptic seizures disappeared rapidly and EEG gradually normalized or improved in patients with myoclonic seizures. Four patients became seizure free for 5 years to 20 years. In 6 patients, seizures relapsed within 14 days to 1 month after the disappearance of seizures. One child remained seizure free for 12 months after viral infection. Common factors in 4 children who were continuously seizure free include (1) normal or almost normal findings of brain CT/MRI, (2) normal development prior to the onset of epileptic seizures, and (3) a short time interval between the onset of seizures and the acute viral infection. We propose several hypotheses including an immunological effect for the improvement of intractable childhood epilepsy following acute viral infection. Further study may provide important information concerning the mechanism of seizure control and the applicable to treatment for intractable childhood epilepsy., (Copyright © 2010 Elsevier B.V. All rights reserved.)
- Published
- 2011
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38. Detection of group a rotavirus RNA and antigens in serum and cerebrospinal fluid from two children with clinically mild encephalopathy with a reversible splenial lesion.
- Author
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Arakawa C, Fujita Y, Imai Y, Ishii W, Kohira R, Fuchigami T, Mugishima H, Izumi H, and Kuzuya M
- Subjects
- Antigens, Antigens, Viral genetics, Antigens, Viral immunology, Brain pathology, Brain physiopathology, Capsid Proteins genetics, Capsid Proteins immunology, Child, Preschool, Enzyme-Linked Immunosorbent Assay, Feces virology, Humans, Male, Reverse Transcriptase Polymerase Chain Reaction, Rotavirus Infections pathology, Rotavirus Infections virology, Antigens, Viral analysis, Cerebrospinal Fluid virology, RNA, Viral analysis, Rotavirus isolation & purification, Rotavirus Infections diagnosis, Serum virology
- Abstract
We report on two children with mild encephalopathy with a reversible splenial lesion associated with group A rotavirus (GARV) infection. We examined stool, serum, and cerebrospinal fluid samples to determine the presence of the GARV VP7 gene and GARV antigen by reverse-transcription PCR and enzyme-linked immunosorbent assay, respectively. GARV antigen was detected in stool samples from both patients. The GARV G genotype was G9 in one child and G3 in the other. GARV antigens were also found in both serum samples. However, the GARV VP7 gene was detected in only one serum sample, which was collected on the first day of symptomatic illness. Neither GARV antigen nor the VP7 gene was detected in cerebrospinal fluid samples. Both patients had excellent outcomes. Our results suggest that the reversible splenial changes in our patients might have been caused by indirect effects to the central nervous system subsequent to viral infection.
- Published
- 2011
39. Henoch-Schönlein purpura in a child with Guillain-Barré syndrome.
- Author
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Fuchigami T, Hasegawa M, Hashimoto K, Fujita Y, and Inamo Y
- Subjects
- Child, Preschool, Factor XIII metabolism, Female, Guillain-Barre Syndrome blood, Humans, IgA Vasculitis blood, Guillain-Barre Syndrome complications, IgA Vasculitis complications
- Abstract
A case of Henoch-Schönlein purpura with Guillain-Barré syndrome in a 3-year-old-girl is presented. This association is extremely rare. During the course of Guillain-Barré syndrome, a decrease in plasma factor XIII activity was noted. When the Guillain-Barré symptoms improved, the factor XIII activity returned to normal. In the present case of Henoch-Schönlein purpura complicated by Guillain-Barré syndrome, the factor XIII level was measured, a novel feature of this study. The findings suggest the involvement of factor XIII, which is characteristically involved in Henoch-Schönlein purpura, also is involved in the associated Guillain-Barré syndrome., (Copyright © 2010 Elsevier Inc. All rights reserved.)
- Published
- 2010
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40. A pediatric patient with acute suppurative thyroiditis caused by Eikenella corrodens.
- Author
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Yoshino Y, Inamo Y, Fuchigami T, Hashimoto K, Ishikawa T, Abe O, Tahara D, and Hayashi K
- Subjects
- Anti-Bacterial Agents therapeutic use, Child, DNA, Bacterial genetics, Eikenella corrodens drug effects, Eikenella corrodens genetics, Gram-Negative Bacterial Infections drug therapy, Humans, Male, Microbial Sensitivity Tests, Neck diagnostic imaging, Radiography, Sequence Analysis, DNA, Thyroid Gland diagnostic imaging, Thyroiditis, Suppurative drug therapy, Eikenella corrodens isolation & purification, Gram-Negative Bacterial Infections microbiology, Thyroiditis, Suppurative microbiology
- Abstract
A previously healthy 6-year-old boy had continuous fever for 6 days before admission to our hospital. His general condition was good except for pyrexia. The left lobe of the thyroid gland was swollen, red, hot, and tender, and neck movement was limited. The provisional diagnosis was upper respiratory tract infection. We demonstrated the existence of an orifice of the left piriform sinus by esophagography in this case, and made a diagnosis of acute suppurative thyroiditis caused by a piriform sinus fistula in the hypopharynx. The causative organisms of acute suppurative thyroiditis include Peptostreptococcus, Staphylococcus haemolyticus, and α-streptococcus, but the organisms responsible are commonly undetectable in clinical cases. We detected Eikenella corrodens in the present patient. Although Eikenella species occasionally causes acute suppurative thyroiditis in adults, it is rare for this to happen in pediatric patients. Antibiotics were administered for 7 days. We also performed aspiration of the abscess on the 8th day of the illness. The abscess was reduced in size and tenderness was relieved after aspiration. In conclusion, if a pediatric patient has swelling, heat, tenderness, and redness of the anterior neck, we should keep in mind acute suppurative thyroiditis and the possibility of a fistula. If there is an abscess, we should immediately perform aspiration, culture, and isolation, and choose the appropriate antibiotics for the causative bacteria.
- Published
- 2010
- Full Text
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41. Protein-losing enteropathy associated with Henoch-Schönlein purpura.
- Author
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Nakamura A, Fuchigami T, and Inamo Y
- Abstract
The gastrointestinal manifestations of Henoch-Schönlein purpura include abdominal pain, gastrointestinal bleeding, intussusception, and perforation. Protein-losing enteropathy is rarely associated with Henoch-Schönlein purpura. Two pediatric patients with Henoch-Schönlein purpura who developed protein-losing enteropathy are reported. Tc-99m human serum albumin scintigraphy is useful in the detection of protein-losing enteropathy.
- Published
- 2010
- Full Text
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42. A case of D-lactic acid encephalopathy associated with use of probiotics.
- Author
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Munakata S, Arakawa C, Kohira R, Fujita Y, Fuchigami T, and Mugishima H
- Subjects
- Child, Preschool, Female, Humans, Neurotoxicity Syndromes physiopathology, Acidosis, Lactic complications, Acidosis, Lactic etiology, Neurotoxicity Syndromes etiology, Probiotics adverse effects, Short Bowel Syndrome complications
- Abstract
A five year old girl was admitted to the hospital for evaluation of intermittent ataxia. She had undergone serial resections of the small intestine after birth, resulting in short bowel syndrome. Lactomin was prescribed for watery diarrhea at twice the regular dose 2 weeks before the onset of neurologic symptoms. D-lactic acidosis was diagnosed on the basis of a plasma D-lactate level of 5.537 mmol/l. Lactomin was discontinued, and she was treated with sodium bicarbonate and oral antibiotics. The probiotics the patient had taken were likely the cause of D-lactic acidosis and should therefore be avoided in patients with short bowel syndrome., (Copyright 2009 Elsevier B.V. All rights reserved.)
- Published
- 2010
- Full Text
- View/download PDF
43. Henoch-schönlein purpura complicated by reversible posterior leukoencephalopathy syndrome.
- Author
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Fuchigami T, Inamo Y, Hashimoto K, Yoshino Y, Abe O, Ishikawa T, Fujita Y, and Mugishima H
- Subjects
- Child, Diagnosis, Differential, Female, Follow-Up Studies, Humans, IgA Vasculitis diagnosis, Magnetic Resonance Imaging, Posterior Leukoencephalopathy Syndrome diagnosis, IgA Vasculitis complications, Posterior Leukoencephalopathy Syndrome etiology
- Abstract
We report a 7-year-old girl with Henoch-Schönlein purpura who developed hypertensive encephalopathy. She showed a sudden onset of neurological symptoms, including hypertension, convulsions, disturbance of consciousness, and cortical blindness. Reversible posterior leukoencephalopathy syndrome was diagnosed from the findings on magnetic resonance imaging. Reports of this syndrome in patients with Henoch-Schönlein purpura are very rare. However, if symptoms suggest central nervous system involvement, neuroimaging should be done for rapid diagnosis, and immediate treatment should be provided to avoid sequelae.
- Published
- 2010
- Full Text
- View/download PDF
44. Fanconi syndrome caused by valproic acid.
- Author
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Endo A, Fujita Y, Fuchigami T, Takahashi S, and Mugishima H
- Subjects
- Adolescent, Anticonvulsants therapeutic use, Child, Epilepsy drug therapy, Fanconi Syndrome therapy, Female, Humans, Male, Time Factors, Valproic Acid therapeutic use, Anticonvulsants adverse effects, Fanconi Syndrome chemically induced, Valproic Acid adverse effects
- Abstract
Two severely disabled children with epilepsy, a 6-year-old boy and a 15-year-old girl, had been treated with valproic acid from the ages of 5 and 6 months, respectively, and developed Fanconi syndrome. Both patients were bedridden and fed by means of a nasogastric or gastrostomy tube. Because their Fanconi syndrome appeared to be caused by valproic acid, that treatment was stopped; for both patients, the Fanconi syndrome then resolved, in 6 months and 3 months, respectively. Severely handicapped children may be at risk for valproic acid-induced renal involvement. Although renal involvement is an uncommon adverse effect of valproic acid, Fanconi syndrome due to valproic acid therapy has rarely been reported. In the few previous cases, the patients, who were bedridden and fed through a nasogastric or a gastrostomy tube, had taken valproic acid for an average of approximately 7 years. Nonetheless, their valproic acid blood levels were normal. Urinary abnormalities disappeared in an average of 4 months, as also in the present two cases. The potential for Fanconi syndrome must be considered in children with epilepsy who are treated with valproic acid, especially in severely disabled children., (Copyright 2010 Elsevier Inc. All rights reserved.)
- Published
- 2010
- Full Text
- View/download PDF
45. Rotavirus antigenemia and genomia in children with rotavirus gastroenteritis.
- Author
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Fujita Y, Liu B, Kohira R, Fuchigami T, Mugishima H, Izumi H, Kuzuya M, Fujii R, Hamano M, and Ogura H
- Subjects
- Antigens, Viral genetics, Capsid Proteins genetics, Child, Preschool, Enzyme-Linked Immunosorbent Assay, Feces virology, Female, Humans, Infant, Male, Reverse Transcriptase Polymerase Chain Reaction, Rotavirus genetics, Rotavirus immunology, Viral Nonstructural Proteins genetics, Antigens, Viral blood, Blood virology, Gastroenteritis virology, RNA, Viral blood, Rotavirus isolation & purification, Rotavirus Infections virology
- Abstract
We investigated group A rotavirus (GARV) antigenemia and genomia in children with rotavirus gastroenteritis. A total of 16 patients (2-29 months old), who received a diagnosis of GARV gastroenteritis using a commercial rapid test, were enrolled in this study. The sera from the patients were tested for the presence of GARV antigen and the VP7 and NSP3 genes using an enzyme-linked immunosorbent assay (ELISA) and reverse transcription-polymerase chain reaction, respectively. Furthermore, when the VP7 gene was amplified, G type was identified and compared with that of GARV from the fecal samples of the patients. GARV antigen was detected in 12 of 16 serum samples (75.0%). No GARV antigen was found in infants that were 6 months old or younger. Thirteen of 16 serum samples (81.3%) were positive for GARV genes. In cases where both antigen and gene analyses were conducted, either GARV antigens or genes, or both, were detected in all cases. The GARV antigen levels of serum collected at 2 days of illness or more were significantly higher than were the levels in the samples obtained from the 1st day. Furthermore, the ELISA optical density values of patients with convulsion were significantly higher than were those of patients without convulsion, suggesting that the antigen level is associated with disease severity.
- Published
- 2010
46. Bronchial casts and pandemic (H1N1) 2009 virus infection.
- Author
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Hasegawa M, Inamo Y, Fuchigami T, Hashimoto K, Morozumi M, Ubukata K, Watanabe H, and Takahashi T
- Subjects
- Airway Obstruction pathology, Child, Humans, Influenza, Human therapy, Japan, Male, Respiration, Artificial, Airway Obstruction etiology, Influenza A Virus, H1N1 Subtype, Influenza, Human physiopathology, Mucus virology, Pulmonary Atelectasis etiology
- Published
- 2010
- Full Text
- View/download PDF
47. Event-related potentials in response to 3-D auditory stimuli.
- Author
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Fuchigami T, Okubo O, Fujita Y, Kohira R, Arakawa C, Endo A, Haruyama W, Imai Y, and Mugishima H
- Subjects
- Acoustic Stimulation, Adolescent, Adolescent Development, Adult, Age Factors, Child, Child Development, Child, Preschool, Doppler Effect, Electroencephalography, Female, Humans, Male, Reaction Time, Regression Analysis, Event-Related Potentials, P300 physiology, Evoked Potentials, Auditory physiology, Sound Localization physiology
- Abstract
To evaluate auditory spatial cognitive function, age correlations for event-related potentials (ERPs) in response to auditory stimuli with a Doppler effect were studied in normal children. A sound with a Doppler effect is perceived as a moving audio image. A total of 99 normal subjects (age range, 4-21 years) were tested. In the task-relevant oddball paradigm, P300 and key-press reaction time were elicited using auditory stimuli (1000 Hz fixed and enlarged tones with a Doppler effect). From the age of 4 years, the P300 latency for the enlarged tone with a Doppler effect shortened more rapidly with age than did the P300 latency for tone-pips, and the latencies for the different conditions became similar towards the late teens. The P300 of auditory stimuli with a Doppler effect may be used to evaluate auditory spatial cognitive function in children.
- Published
- 2009
- Full Text
- View/download PDF
48. Detection of rotavirus RNA and antigens in serum and cerebrospinal fluid samples from diarrheic children with seizures.
- Author
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Liu B, Fujita Y, Arakawa C, Kohira R, Fuchigami T, Mugishima H, and Kuzuya M
- Subjects
- Antigens, Viral genetics, Capsid Proteins genetics, Child, Preschool, Enzyme-Linked Immunosorbent Assay, Feces virology, Female, Genotype, Humans, Infant, Male, Reverse Transcriptase Polymerase Chain Reaction, Rotavirus genetics, Rotavirus immunology, Antigens, Viral blood, Antigens, Viral cerebrospinal fluid, Diarrhea etiology, RNA, Viral blood, RNA, Viral cerebrospinal fluid, Rotavirus isolation & purification, Rotavirus Infections complications, Seizures etiology
- Abstract
Group A rotavirus (GARV) genes (the VP7 and NSP3 genes) in acute-phase cerebrospinal fluid (CSF), sera and stool samples from 6 children with convulsions accompanied by GARV gastroenteritis were investigated by reverse transcription-polymerase chain reaction (RT-PCR). When the VP7 gene was amplified from the samples, the G genotype (G type) of GARV was determined by RT-PCR. GARV genes were detected in the CSF samples of all 6 children, in 2 of the 3 blood samples, and in all of 4 stool samples. The G typing of GARV from 12 of a total of 13 samples indicated that G3 was the predominant G type in all samples. GARV antigens were detected by enzyme-linked immunosorbent assay in all of the 3 tested sera samples, while no GARV antigens were detected in any of the 5 tested CSF samples. We confirmed the presence of GARV genomes in the CSF samples from all of the children with rotavirus-associated seizures, including encephalopathy. However, the relationship between convulsions and the existence of GARV RNA in CSF remains unclear and further study is required.
- Published
- 2009
49. The relationship between drug treatment and the clinical characteristics of febrile seizures.
- Author
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Haruyama W, Fuchigami T, Noguchi Y, Endo A, Hashimoto K, Inamo Y, Fujita Y, Takahashi S, and Mugishima H
- Subjects
- Child, Child, Preschool, Contraindications, Drug Therapy, Combination, Female, Humans, Infant, Male, Phenothiazines, Retrospective Studies, Theophylline, Time Factors, Histamine H1 Antagonists, Seizures, Febrile chemically induced
- Abstract
Background: Drugs such as theophylline, antihistamines, and antiallergics with anti-histaminic actions have been shown to induce febrile seizures. The relationship between febrile seizures and medications has not been actively investigated. The present study aimed to investigate the relationship between the clinical characteristics of febrile seizures and the use of medications., Methods: Two hundred and sixty-five children treated at our emergency room due to febrile seizures were studied to investigate the relationship between the clinical characteristics of febrile seizures, such as the type and duration of convulsions, and the drug treatment., Results: The duration of convulsions was longer among children who took theophylline and antihistamines than among children who did not take these medications. Of the antihistamines, mequitazine did not prolong the duration of convulsion., Conclusions: Theophylline should not be used in febrile children, particularly infants. Cautions should be taken in using histamine H1 antagonists in young infants because such drugs could potentially disturb the anticonvulsive central histaminergic system. However, mequitazine appears to be a suitable antihistamine for use in children with febrile seizures, since it does not prolong convulsions.
- Published
- 2008
- Full Text
- View/download PDF
50. Intravenous ulinastatin therapy for Stevens-Johnson syndrome and toxic epidermal necrolysis in pediatric patients. Three case reports.
- Author
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Inamo Y, Okubo T, Wada M, Fuchigami S, Hashimoto K, Fuchigami T, Takahashi S, Sawada S, and Harada K
- Subjects
- Animals, Child, Glycoproteins administration & dosage, Humans, Infant, Injections, Intravenous, Male, Treatment Outcome, Trypsin Inhibitors administration & dosage, Glycoproteins therapeutic use, Stevens-Johnson Syndrome drug therapy, Trypsin Inhibitors therapeutic use
- Abstract
Background: More effective therapy is needed for the treatment of Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN). The clinical efficacy of intravenous ulinastatin therapy was investigated in 3 Japanese pediatric patients with SJS or TEN., Methods: Ulinastatin was given to 1 pediatric SJS patient and 2 pediatric TEN patients within 7 days (patient 1; SJS), 6 days (patient 2; TEN), or 4 days (patient 3; TEN) after the onset of the skin rash. Ulinastatin was administered intravenously at a dose of 7,500 U/kg/day (maximum dose: 300,000 U/day). No corticosteroids were given. After the skin lesions resolved, the ulinastatin dose was reduced to between 2,500 and 5,000 U/kg/day as maintenance therapy and then the drug was withdrawn., Results: Erythema, fatigue, and fever improved within 12-36 h of starting the ulinastatin infusion, and the skin lesions resolved completely after 4-7 days of ulinastatin therapy. None of the patients had cutaneous or ocular sequelae. No patient developed secondary infection or relapse and ulinastatin therapy caused no side effects., Conclusion: Ulinastatin dramatically reduced the febrile period with no adverse effects and was very safe in this study. Ulinastatin appears to be a useful and effective therapy for controlling SJS and TEN without sequelae.
- Published
- 2002
- Full Text
- View/download PDF
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