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THE JOURNAL OF THE JAPAN PEDIATRIC SOCIETY
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Vol.125, No.4, April 2021
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Review
Title
Novel Biomarkers in Terms of Pathogenesis in IgA Vasculitis
Author
Yukihiko Kawasaki
Department of Pediatrics, Sapporo Medical University School of Medicine
Abstract
IgA vasculitis is a typical vasculitis affecting predominantly the skin, joints, and gastrointestinal tract in childhood. About 30-60% of children with IgA vasculitis have nephritis and the prognosis of IgA vasculitis is largely predicted by the severity of renal involvement. As a biomarker for assessing the disease activity of IgA vasculitis, an index relating to CD4+T cell subset, aberrantly glycosylated IgA1 antibody, anti-endothelial cell IgA antibody, E-selectin, thrombomodulin, the innate immune activity factors such as complement and macrophage, and the degree of tissue damage, degree of mesangial transformation and macrophage infiltration in renal biopsy tissue, and coagulation abnormality were reported. In order to improve the prognosis of IgA vasculitis with nephritis, it is necessary to evaluate the disease activity of IgA vasculitis by using these biomarkers and provide appropriate treatment.
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Original Article
Title
Randomized Controlled Trial of Fluticasone/Formoterol Combination Drug for Long-term Control of Pediatric Asthma
Author
Toshio Katsunuma1) Naoya Kitamura2) Miho Kamata2) and Yasuyuki Ishikawa2)
1)Department of Pediatrics, Daisan Hospital, The Jikei University School of Medicine
2)Clinical Development Center, Kyorin Pharmaceutical Co., Ltd.
Abstract
In Japan, the only approved combination of inhaled corticosteroid and long acting β2 agonist for pediatric asthma is fluticasone propionate/salmeterol xinafoate (FP/SM). To see if fluticasone propionate/formoterol combination drug (FP/FM) could be a new treatment option for pediatric asthma, we conducted a crossover comparative study and a long-term administration study. Here, we report on the crossover comparative study with FP/SM.
Multicenter randomized administration of FP/FM (200/20 μg/day) or FP/SM (200/100 μg/day) for 2 weeks each in Japanese asthma patients between the ages of 5 and 16 years. An open-label, active-control, two-group, two-period, crossover comparative study was conducted. The primary endpoint was the change of the morning peak expiratory flow (mPEF) from the baseline in 7 days before the end of each treatment period. The other evaluation items were evening peak expiratory flow, symptom scores, adverse drug reactions, laboratory tests and the 12-lead electrocardiogram.
Non-inferiority of the FP/FM group to FP/SM group was confirmed in the change of mPEF value from the baseline (LS Mean, 0.93 L/min; 95% CI, −4.57 to 6.43 L/min; non-inferiority margin, −15 L/min). No major differences were observed on the other efficacy endpoints and the safety profiles between the two groups.
FP/FM can be a new treatment option for pediatric asthma. (JapicCTI registration number: 173632)
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Original Article
Title
Post-travel Health Consultations for Children through 12 Years of Age at the Reference Center of Travel Medicine in Japan
Author
Kei Yamamoto1) Nozomi Takeshita1) Norio Ohmagari1) and Shuzo Kanagawa1)2)
1)Disease Control and Prevention Center, National Center for Global Health and Medicine
2)Tokyo Business Clinic
Abstract
The number of pediatric patients after international travel is expected to increase in Japan because of the increasing number of international travelers. However, the epidemiology of pediatric patients from foreign countries is not always clear.
We reviewed the medical records of patients aged <18 years who visited our department between January 2005 and December 2016, retrospectively.
In total, there were 269 patients (154 males) aged <18 years. Regarding age distribution, 101, 83, and 85 patients were aged between 0 and 5 years (A), between 6 and 11 years (B), and between 12 and 17 years (C), respectively. Further, 180 travelers (67%) were from Asia. Although the most frequent purpose of travel was tourism, expatriate visit, visiting friends and relatives (VFR), and study were frequently reported in groups A, B, and C, respectively. Many patients were given diagnoses of respiratory or gastrointestinal infections. There were only 14 (5.9%) cases of tropical diseases. There was a significantly lower number of patients who were short-term tourists (2.4%) than those who were long-term tourists, VFR, and visitors from foreign countries (9.0%, p = 0.04). Although 22 patients were admitted to the hospital, all were discharged without any sequelae or death.
Although pediatric cases of tropical diseases are rare, pediatricians should pay attention to these diseases when they see patients after long-term travel, those who are VFR, or those from a foreign country.
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Original Article
Title
Mesalazine Intolerance in Pediatric Inflammatory Bowel Disease: A Single-center Experience
Author
Yuki Yamakawa Tatsuki Mizuochi Hirotaka Sakaguchi Jun Ishihara and Yushiro Yamashita
Department of Pediatrics and Child Health, Kurume University School of Medicine
Abstract
Background: Prevalence of inflammatory bowel diseases (IBD) has been increasing in not only adults but also children in Japan. Mesalazine, which is a key drug for IBD, has intolerance and its symptoms are mimicking with IBD. The aim of this study was to clarify clinical features of mesalazine intolerance in pediatric patients with IBD.
Methods: We retrospectively reviewed medical records of pediatric patients (under 16 years old) with IBD treated with mesalazine at the Department of Pediatrics, Kurume University Hospital between January, 2010 and December, 2019.
Results: Nine of 85 children (11%) with IBD were diagnosed with mesalazine intolerance (8% in UC and 15% in CD). Median age was 13 years (range, 5-15 years). Median time from initiation of mesalazine to onset of mesalazine intolerance was 26 days (range, 6-837 days) and half (56%) was occurred within 4 weeks. Clinical findings of mesalazine intolerance with elevation of C-reactive protein, abdominal pain, diarrhea, fever, and bloody stool were respectively noted in 78%, 67%, 56%, 44% and 0% of patients with IBD. Drug-induced lymphocyte stimulation test was positive in 4 of 7 (57%).
Conclusions: Prevalence of mesalazine intolerance was 11% in pediatric IBD at our hospital and half of them occurred within 4 weeks.
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Case Report
Title
A Case of Developmental and Epileptic Encephalopathy Associated with ATP1A3 Mutation
Author
Ayaka Ono1) Yuji Fujii1) Tomoki Sato1) Ayako Tanimoto1) Yuko Yamane1) Ryouji Futagami1) Shuji Yoshino1) Hiroyuki Shimozono1) Keita Matsubara1) Rika Okano1) Hideyuki Asai2) Nodoka Hinokuma2) and Mitsuhiro Kato2)
1)Department of Pediatrics, Hiroshima City Funairi Citizens Hospital
2)Department of Pediatrics, Showa University School of Medicine
Abstract
The ATP1A3 gene, which was reported as a causative gene of rapid-onset dystonia-parkinsonism in 2004, is responsible for a wide range of neurological diseases such as alternating hemiplegia of childhood. Recently, cases characterized by respiratory failure, intractable early-life epilepsy, involuntary movement, nystagmus, and developmental disability were reported as a new phenotype of the ATP1A3-related disorder spectrum. We report a case of early-onset encephalopathy with a mutation in the ATP1A3 gene (c.2116G>A, p.G706R, de novo). p.G706R was previously reported in a typical AHC case, suggesting that it is a pathogenic mutation. The male patient is now two years old. He presented with respiratory failure, nystagmus, and epilepsy at one month, and now has moderate developmental disabilities. His seizures remained intractable to multiple anti-epileptic drugs. However, after potassium bromide was administered when he was one year and three months old, the frequency of his seizures significantly decreased. There are no previous reports of patients with mutation in the ATP1A3 gene treated by potassium bromide. This case suggests that potassium bromide is effective for treating intractable epilepsy in patients with such mutations. Mutations in the ATP1A3 gene should be considered in patients who present with respiratory failure, early-life epilepsy, nystagmus, involuntary movement, and developmental disabilities.
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Case Report
Title
A Case of Desmoid-type Fibromatosis Presenting as Trismus
Author
Ayako Iemura1) Masakatsu Yanagimachi1) Akihisa Tasaki2) Yuka Hirota3) Iichiro Onishi3) Takahiro Kamiya1) Fumihiko Tsushima4) Hiroyuki Harada4) Izumi Kinoshita5) Kenichi Kohashi5) Yoshinao Oda5) Masatoshi Takagi1) Takumi Akashi3) Takahiro Asakage2) and Tomohiro Morio1)
1)Department of Pediatrics and Developmental Biology, Graduate School of Medical and Dental Sciences, Tokyo Medical and Dental University
2)Department of Head and Neck Surgery, Graduate School of Medical and Dental Sciences, Tokyo Medical and Dental University
3)Department of Pathology, Graduate School of Medical and Dental Sciences, Tokyo Medical and Dental University
4)Division of Oral and Maxillofacial Surgery, Department of Oral Health Sciences, Graduate School of Medical and Dental Sciences, Tokyo Medical and Dental University
5)Department of Anatomic Pathology, Pathological Sciences, Graduate School of Medical Sciences Kyushu University
Abstract
Trismus is an essential sign that is related to disorders of the temporomandibular joint, nerves and muscle or the adjacent structures. It is important to include rare tumors in the differential diagnosis of trismus. The present case was a 3-year-boy in whom trismus was noted soon after birth. He was referred to our hospital, and "desmoid-type fibromatosis (DF)" responsible for his trismus, was diagnosed. Treatment was a wait-and-see strategy, and spontaneous tumor regression was observed one year later.
DF is a rare tumor that has a tendency to invade locally, with occasional post-operative recurrence, while spontaneous regression may not infrequently occur. Due to this unpredictable clinical course, there is no established or evidence-based treatment approach for DF. Accurate diagnosis and appropriate treatment in pediatric patients is important.
When children with trismus are examined at a general practice, it should be kept in mind that some responsible tumors might exhibit malignant behavior.
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Case Report
Title
Pediatric Mandibular Osteomyelitis Caused by Mycobacterium fortuitum
Author
Natsumi Fujiyama1) Naoki Yogo1) Natsumi Kataoka1) Takahiro Nishihara1) Kenta Kawahara2)3) Akiyuki Hirosue2)3) Michiko Nagamine4) Yuichiro Muto1) Katsuki Hirai1) and Masahiro Migita1)
1)Department of Pediatrics, Japanese Red Cross Kumamoto Hospital
2)Department of Oral and Maxillofacial Surgery, Faculty of Life Sciences, Kumamoto University
3)Department of Oral and Maxillofacial Surgery, Japanese Red Cross Kumamoto Hospital
4)Department of Pathology, Japanese Red Cross Kumamoto Hospital
Abstract
Pediatric non-tuberculous mycobacterial (NTM) infection is very rare. Infection of pulmonary lesions is common among adults, while that of extrapulmonary lesions, involving the skin and lymph nodes, is common among children. However, osteomyelitis is rare and mandibular osteomyelitis is extremely rare. We report a case of mandibular osteomyelitis caused by Mycobacterium fortuitum after dental treatment. This case pertains to a 12-year-old girl who complained of pain and swelling in the right submandibular region. Antibiotic treatment was administered and she underwent incisional drainage. Since her condition did not improve, she was referred to our hospital. A diagnosis of mandibular osteomyelitis was made based on contrast-enhanced magnetic resonance imaging findings. The right mandibular first molar was extracted and pulpotomy was performed on the mandibular region around the involved tooth. A granuloma consisting of epithelioid cells and Langhans giant cells was detected, and M. fortuitum was isolated from the culture. The patient was treated with amikacin, imipenem, and ciprofloxacin for 6 weeks, and switched to oral administration of faropenem and ciprofloxacin. She is currently recovering well without any signs or symptoms of recurrence.
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Case Report
Title
Pediatric-onset Eosinophilic Esophagitis: A Single-center Experience in Japan
Author
Takuro Sato1) Ichiro Takeuchi1) Hirotaka Shimizu1) Natsuki Ito1) Masaaki Usami1) Hiroya Ogita2) Tatsuki Fukuie2) Ichiro Nomura2) Yukihiro Ohya2) Takako Yoshioka3) and Katsuhiro Arai1)2)
1)Division of Gastroenterology, National Center for Child Health and Development
2)Division of Allergy, National Center for Child Health and Development
3)Department of Pathology, National Center for Child Health and Development
Abstract
Eosinophilic esophagitis (EoE) is an eosinophilic gastrointestinal disorder in which eosinophilic infiltration is limited to the esophageal mucosa. The number of EoE patients has been increasing in Japan in recent years, and we encountered five cases of pediatric-onset EoE over the last 2.5 years.
Four of the patients were male, and the median age of onset and age at diagnosis was 4.0 years and 9.1 years, respectively. Four had underlying allergic disorders, and one had Crohn's disease. Four patients had gastroesophageal reflux symptoms (vomiting, heartburn, chest pain, and postprandial coughing), two had dysphagia, and one had poor weight gain. Esophagogastroduodenoscopy at diagnosis showed linear furrowing and esophageal rings in all cases. The maximum eosinophil count in the esophageal mucosa was 35-83/HPF. All patients were initially treated with a proton-pump inhibitor, but only one responded adequately to it. Two improved with the addition of topical steroids.
With the increasing number of children with EoE in Japan, early endoscopy with histological evaluation in suspected cases would facilitate the diagnosis of EoE followed by optimization of treatment. The characteristics of EoE in Japan should be investigated further.
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Case Report
Title
Clinical Indicators for the Early Diagnosis of Macrophage Activation Syndrome as a Complication of Kawasaki Disease
Author
Asumi Jinkawa1) Masaki Shimizu1) Yuta Sakai1) Keigo Nishida1) Yasuhiro Ikawa1) Maiko Takakuwa2) Syuhei Fujita2) Kiyoshi Hatasaki2) Taizo Wada1) and Akihiro Yachie1)
1)Department of Pediatrics, School of Medicine, Institute of Medical, Pharmaceutical and Health Sciences, Kanazawa University
2)Department of Pediatrics, Toyama Prefectural Central Hospital
Abstract
Macrophage activation syndrome (MAS) is a fatal complication of rheumatic diseases. MAS is rarely a complication of Kawasaki disease (KD). A 5-year-old girl with KD was treated with intravenous immunoglobulin on the fifth day of presentation. Her symptoms improved; however, the patient developed high-grade fever and a rash. Laboratory findings showed pancytopenia, coagulopathy, an increase in serum transaminase and lactate dehydrogenase (LDH) levels, and hyperferritinemia. A diagnosis of MAS was established. Treatment with dexamethasone palmitate, cyclosporine, and heparin was initiated. Her symptoms improved promptly. Patients with KD and MAS may have a high risk of developing coronary artery lesions and high risk of mortality. Therefore, it is essential to select an appropriate therapeutic approach. Serial monitoring with platelet counts and serum LDH levels might be useful for the early diagnosis of MAS. In patients with thrombocytopenia and KD, careful monitoring of serum LDH and ferritin levels is necessary for the diagnosis of transition to MAS.
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Case Report
Title
The Psychologist's Role in Organ Donation from Brain-dead Children
Author
Akiko Bessho1) Takashi Araki2) Yoshio Sakurai1) and Kouichi Moriwaki3)
1)Center for Pediatric Critical Care Medicine, Saitama Medical University
2)Department of Emergency and Critical Care Medicine, Saitama Medical University
3)Department of Pediatrics, Saitama Medical University
Abstract
Parental grief following the decision of organ donation of their brain-dead child can be extremely difficult. However, no study has focused on the involvement of a clinical psychologist (CP). Therefore, in the present study, the role of CP in one pediatric organ donation is discussed.
In this case, the patient was hit by a car and diagnosed brain-dead after being admitted to the center for pediatric critical care medicine. A CP met the family right after the patient was admitted. The doctors, nurses, and CP discussed how to inform the family about the patient's condition after which the CP relayed the information, assessed whether the family comprehended the situation, and reported the findings to the doctors and nurses. On the sixth day, the family consented to organ donation. During the organ donation process, the CP consistently supported the family. The fact that the CP was involved from the beginning and had provided support to the family as well as to doctors and nurses, facilitated the necessary discussions and contributed to the smooth progression of the organ donation process.
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