|
THE JOURNAL OF THE JAPAN PEDIATRIC SOCIETY
|
Vol.121, No.6, June 2017
|
Review
Title
Outline and Practice of the International Neuroblastoma Pathology Classification
Author
Ryosuke Matsuno and Hiroyuki Shimada
Department of Pathology and Laboratory Medicine, Children's Hospital Los Angeles
Abstract
Peripheral neuroblastic tumors (pNTs including Neuroblastoma, Ganglioneuroblastoma, and Ganglioneuroma) constitute one of the most common extra-cranial solid tumors in children. They are composed of biologically distinct groups; i.e., Biologically Favorable Tumors with a benign or non-aggressive clinical course due to spontaneous regression or tumor maturation and Biologically Unfavorable Tumors with an aggressive behavior often leading to fatal outcome. In order to test clinical trials for developing more efficient and less toxic treatment protocol, the Children Oncology Group (COG) Neuroblastoma Biology Study stratified pNT patients into different risk groups, Low, Intermediate, and High, based on the combination of various prognostic factors. Our COG Neuroblastoma Pathology Reference Laboratory has been playing a critical role in this process by reviewing pathology material of the cases from the U.S., Canada, Australia, and New Zealand, and classifying them into Favorable Histology or Unfavorable Histology Group according to the International Neuroblastoma Pathology Classification (INPC). We have also been participating in the activities of translational research of this disease. In this manuscript, we outline histopathology/INPC, and discuss some of the important points we often encounter during the central pathology review at our Reference Laboratory.
|
|
Original Article
Title
Role of a Recovery Center for Medically-dependent Children in Osaka
Author
Masahisa Funato Kiyoshi Takemoto Yoshitaka Iijima Hiroshi Wada Waka Hatano Masumi Fujiwara and Tamami Katayama
Department of Pediatrics, Osaka Developmental Rehabilitation Center
Abstract
Aims. In Japan since the ending of the 20th Century, there have been increasing problems involved in the care for children staying in long-term situations in NICU (Neonatal Intensive Care Units) or pediatric wards. Intermediate facilities such as children's recovery centers or sub-acute centers have now become crucial for such cases. Therefore since 2011, our facility has received medically. dependent children and has assisted in the transfer process from hospital to home care.
Subject and method: Twenty-eight patients were referred mainly from NMCS (Neonatal Mutual Cooperative System) hospitals in Osaka for the following purposes; (1) provision of transfer support to home care, (2) comprehensive rehabilitation, (3) preparation for short-stay service. Multidisciplinary support was provided in the program during admission for 2-3 months.
Results: Our center received inquiry or referrals for 42 cases of patients from 18 hospitals. Of these we actually admitted 28 cases in the program over a 5-year period. Of these, 21 cases (75%) were transferred from hospital to home care after completion of the program. One patient was later admitted to a long-term facility after going back home, and another passed away after a 2-year home-stay. That family was provided with end-of-life care at home, supported by a visiting doctor and by nurses. Other patients have continued receiving home care by utilizing short-stay service and/or day care in our center or in other facilities.
Conclusion: Facilities for SMID (Severe Motor Intellectual Disorders) have expertise in the support of medically-dependent children, and have capability for providing rehabilitation or daily-life support by a multidisciplinary team in addition to medical support. If human resources or funds were adequately supplied, it might be possible for these facilities to provide a recovery center on the basis of the best interests of children and families.
|
|
Original Article
Title
The Development and Standardization of the Questionnaire for Triage and Assessment: A Self-report Health-related Outcome Scale for Children with Somatization
Author
Ryuta Ishii1) Shinichiro Nagamitsu1) Rieko Sakurai2) Kenshi Koyanagi3) Yukiko Kanbara4) Junichi Furusho5) Nobuo Ishitani6) Tatsuyuki Kakuma2) Yushiro Yamashita1) Toyojiro Matsuishi7) Hidetaka Tanaka8) and The Japanese Society of Psychosomatic Pediatrics9)
1)Department of Pediatrics and Child Health, Kurume University School of Medicine
2)Biostatistics Center, Kurume University School of Medicine
3)Nagasaki Prefectural Center of Medicine and Welfare for Children
4)Department of Pediatrics, Yao Tokushukai General Hospital
5)College of Education, Psychology and Human Studies, Department of Education, Aoyamagakuin University
6)Ishitani Shonika Iin
7)Research Center for Children, Research Center for Rett Syndrome, St. Mary's Hospital
8)OD Clinic Tanaka
9)The Japanese Society of Psychosomatic Pediatrics
Abstract
The Japanese Society of Psychosomatic Pediatrics has developed a self-report child health-related outcome scale to screen children with somatization early and to assess psychosomatic symptom changes after treatment. This 30-item scale was named the Questionnaire for Triage and Assessment (QTA30). The QTA30 assesses children's positive attitude and their physical and psychiatric symptoms. Each item is scored on a 3-point Likert scale: "yes", "sometimes", and "no". The QTA30 was compared with the Japanese language version of KINDLR (which measures health-related quality of life in children and adolescents) to assess its validity and reliability for 5,778 school-aged children (aged 10 to 17 years). A factor analysis conducted to identify the structure of the QTA30 revealed five factors: "physical symptoms", "depressive symptoms", "self-efficacy", "anxiety", and "family function". The QTA30 demonstrated high reliability (Cronbach's alpha coefficient = 0.80) and test-retest reliability (Pearson's correlation coefficient = 0.84). Validity was confirmed by high correlations between the QTA30 and Japanese language version of KINDLR. The total QTA30 score was set as 0-92 by weighting the "physical symptoms" scale by 3. Receiver operating characteristic analysis determined an optimal QTA30 cut-off value of 37, with a sensitivity and specificity of 71% and 80%, respectively. These results indicate that the QTA30 is a useful questionnaire to screen children with psychosomatic problems and can be used as an outcome scale to assess changes in psychosomatic symptoms.
|
|
Original Article
Title
Antigen-specific Immune Response in Three Different Intervals between Two Doses of Varicella Vaccine
Author
Masahiro Ohashi1) Hiroki Miura2) Yoshiki Kawamura2) Ayano Fujita2) Hitoshi Ando1) and Tetsushi Yoshikawa2)
1)Department of Pediatrics, Toyokawa Municipal Hospital
2)Department of Pediatrics, Fujita Health University School of Medicine
Abstract
In order to elucidate an appropriate interval between two doses of varicella vaccine, immune responses against varicella zoster virus (VZV) were examined for three different interval groups. Thirty-nine healthy children (mean age: 16.05±6.15 months) were divided into 3 different interval groups (A: 3-4 months, B: 5-7 months, C: 8-14 months). Seroconversion rates of IAHA and gp-ELISA antibodies after a first dose of vaccine were 73.5% (25/34) and 76.3% (29/38), respectively. Based on both serological assays, the seroconversion rate after the second dose of vaccine was 100% in all three interval groups. The mean IAHA (log2) antibody titers after the second dose of vaccine were 5.83±1.11 (A), 6.33±1.62 (B) and 6.40±1.17 (C), and there were no significant differences among the three groups. The mean gp-ELISA (log10) antibody titers after the second dose were 3.63±0.31 (A), 3.86±0.42 (B), and 3.79±0.26 (C). Although the mean antibody titers in group B and C tended to be higher than in group A, there were no significant differences among the three groups. Fold increases in mean IAHA antibody titers by the second dose of vaccine were 6.72 (A), 13.79 (B) and 9.19 (C), and fold increases in mean gp-ELISA antibody titers were 24.6 (A), 67.7 (B) and 52.3 (C). There were also no significant differences among the three groups. In the VZV skin test, the overall positive rate after a second dose vaccine was 80.6% (29/36), and no significant differences was demonstrated in maximum erythema diameter or positivity among the 3 different interval groups.
|
|
Original Article
Title
Factors Related with Taking CT Scan in Children with Suspected Cellulitis around the Eyes
Author
Keisuke Shimizu1) Kenta Ito2) Hiroki Sakurai2) Satoko Suzuki1) Toshirou Terakawa1) and Yuho Horikoshi2)
1)Department of General Pediatrics, Tokyo Metropolitan Children's Medical Center
2)Division of Infectious Diseases, Department of Pediatrics, Tokyo Metropolitan Children's Medical Center
Abstract
A computed tomography (CT) scan is recommended as a means of differentiating orbital cellulitis and orbital abscesses from preseptal cellulitis in children. However, which factors prompted physicians to use the CT scan in such cases were not known. Our aim was to examine the factors prompting physicians to use the CT scan to examine children presenting suspected cellulitis around the eyes at an emergency center and to compare those factors with the findings of previous studies. A total of 108 children with suspected cellulitis around the eyes were retrospectively examined through their medical records at Tokyo Metropolitan Children's Medical Center between March 2010 and May 2015. Of these patients, those who had received a CT and those who had not were compared in terms of their demographic data, physical findings, laboratory data, and definitive diagnosis. There was no statistically significant difference between the two groups in terms of the factors previously reported as significant: proportion of patients ≥3 years old, absolute neutrophil count > 10,000/μ l, and previous antibiotic administration. Multiple logistic regression analysis found that body temperature ≥38.0°C was significantly related to the use of the CT scan. (Adjustment odds ratio 6.0, 95% confidence interval 1.5-24.4, P=0.012). This finding indicated that our physicians were most likely to use the CT scan in febrile patients although the presence of fever did not predict orbital cellulitis or abscess in previous studies.
|
|
Original Article
Title
First-line Eradication Therapy for Helicobacter Pylori Infection Based on Antibacterial Susceptibility Testing
Author
Satoru Ishikawa1)2) Shin-ichiro Hagiwara1) Ryusuke Nambu1) Tomoko Hara1) Ryo Matsuoka1)2) Mitsuru Kubota3) Seiichi Kagimoto1) and Hiroyuki Ida2)
1)Division of General Pediatrics, Saitama Children's Medical Center
2)Department of Pediatrics, The Jikei University School of Medicine
3)Department of General Pediatrics and Interdisciplinary Medicine, National Center for Child Health and Development
Abstract
Background and Objective: As the proportion of pediatric patients with antibiotic-resistant Helicobacter pylori (H. pylori) has increased, first-line eradication has become unsuccessful. In our hospital, we employed regimens based on antibacterial susceptibility testing of gastric biopsy specimens. The purpose of the present study was to investigate the antibiotic-resistance rates of H. pylori and the efficacy of the eradication therapy based on antibacterial susceptibility testing.
Methods: We performed a retrospective study of the clinical records of 19 patients with H. pylori infection from 2007 to 2014. When the patients had clarithromycin (CAM)-resistant H.pylori, they were treated with amoxicillin (AMPC), metronidazole (MNZ) and a proton pump inhibitor as a first-line eradication therapy.
Results: Antibacterial susceptibility testing was performed in 13 cases. Resistance to AMPC, CAM, and MNZ was observed in 23% (3/13), 38% (5/13), and 0% (0/13) of patients, respectively. Eradication rates were 94.4% (17/18) using the first-line eradication therapy and 100% (1/1) using the second-line eradication therapy.
Conclusion: First-line eradication therapy based on antibacterial susceptibility testing improved the successful eradication rate of H. pylori infections. Cohort studies involving greater numbers of cases are required to confirm our strategy.
|
|
Case Report
Title
A High Flow Nasal Cannula Improved Hypoventilation due to Thoracic Deformity in an Infant with Klippel-Feil Syndrome
Author
Shingo Yamada Tomoaki Maji Naoki Tsuboya Tomoki Hattori Minehiro Kurai Ayako Yoshino Kazuyuki Matsuda Mitsue Ito Ryoji Ichimi and Masamune Higashigawa
Department of Pediatrics/Neonatology, Japanese Red Cross Ise Hospital
Abstract
We report the first case of a male infant with Klippel-Feil syndrome who was transferred to home care after using a high flow nasal cannula (HFNC). His mother was 30 years old, gravida 0, para 0, and became pregnant by transfer of a thawed single blastocyst. Ultrasound and magnetic resonance imaging studies at 17 weeks of gestation indicated that the fetus had hypoplasia of the cerebellum. The infant was delivered via caesarean section at 37 weeks and 4 days of gestation because of fetal distress. His Apgar scores were 7 and 9, at 1 and 5 min, respectively. His body weight at birth was 2,358 g. Physical examination showed that he had a short neck, limited neck motion, and a low hairline. He received endotracheal intubation and mechanical ventilation for 7 days due to respiratory distress.
A three-dimensional computed tomography (3D-CT) scan of the head, neck, and thorax at 13 days of age revealed no airway obstruction but dysgenesis of the atlas and axis vertebrae, fused and butterfly shaped cervical vertebrae, and fusion and agenesis of the costal bones. A diagnosis of Klippel-Feil syndrome was made based on these physical and radiological findings.
After cessation of mechanical ventilation, he exhibited difficulty feeding and poor body weight gain because of tachypnea. A blood gas analysis showed hypercapnia. We considered that the restricted motion of the thorax caused by the rib abnormality reduced the tidal volume. We therefore treated him with an HFNC to excrete CO2 at 22 days of age. Feeding difficulties and hypercapnia promptly improved after the HFNC therapy. He was transferred to home care at 72 days of age because he was able to complete full oral feeding and had gained adequate body weight. We speculate that an HFNC is a useful device for patients with hypercapnia due to hypoventilation based on a thoracic deformity.
|
|
Case Report
Title
Bone Mineral Density Changes in Severely Neurologically Disabled Children with Sodium Valproate-induced Fanconi Syndrome
Author
Hitomi Maeda Junko Nakayama Tomohiro Nakayama Tatsuo Ito Kenji Shin and Nobuaki Iwasaki
Department of Pediatrics, Ibaraki Prefectural University of Health Sciences Hospital
Abstract
Fanconi syndrome (FS), which has been associated with osteomalacia, has been induced in severely disabled children receiving long-term treatment with sodium valproate (VPA), or with multiple antiepileptic drugs. However, as far as we know, there are no reported cases of bone mineral density changes in patients with VPA-induced FS. We report the cases of two boys who were diagnosed with FS, at the ages of 4 years and 7 months (case 1) and 11 years and 5 months (case 2), which was also associated with osteomalacia. Both boys were severely disabled and were treated with multiple antiepileptic drugs, including VPA. Regular evaluation of bone mineral density by dual X-ray absorptiometry (DXA) and urine β2-microglobulin were performed from prior to the onset of FS until approximately 18 months after symptom manifestation. In these two cases, FS was detected as a result of proteinuria and elevated β2-microglobulin in the urine, as determined by regular urinalysis. We found improvement of renal tubular function approximately two months after discontinuing VPA, and the decrease in bone mineral density was restored approximately 15-18 months after discontinuation. The restoration of bone mineral density was delayed compared with idiopathic adult FS, and bone fragility remained after improvement of the urinary biomarkers. Children with severe neurological disabilities have decreased bone density and show a greater risk of bone fracture. For these children, regular urinalysis including urine β2-microglobulin may lead to early diagnosis of FS, and regular bone mineral density evaluations may lead to improved care that prevents bone fracture.
|
|
Case Report
Title
Efficacy of Soluble IL-2 Receptor Level in a Pediatric Patient with Eosinophilic Cellulitis
Author
Keiko Nagahara1)2) Yoshifusa Abe1) Yuko Sato2) Tetsuomi Suzuki3) Katsuhiko Yamaguchi2) Yutaka Sato2) and Kazuo Itabashi1)
1)Department of Pediatrics, Showa University School of Medicine
2)Department of Pediatrics, Machida Municipal Hospital
3)Department of Pediatrics, Saiseikai Yokohamashi Nanbu Hospital
Abstract
Eosinophilic cellulitis (EC) was first described by Wells in 1971 as "recurrent inflammatory dermatosis of unknown pathogenesis." Few reports are available about the serum marker level reflecting the condition of EC. A 4-year-old girl presented with an onset of fever. On the second day, she developed pruritic inflammatory plaques on the trunk. On the fourth day, she was admitted to our hospital due to additional palpebral edema. Bacterial cellulitis was suspected, and antibiotic therapy was started. However, the skin rashes spread all over her body. On the seventh day, the level of serum soluble IL-2 receptor (sIL-2R) was found to be elevated (4,920 U/ml). On the eighth day, fever and rashes rapidly improved after steroid therapy. Hence, steroid therapy was discontinued on the 15th day despite her high level of serum sIL-2R (2,240 U/ml). On the 16th day, edematous erythema with vesicles appeared on her fingers and reinitiation of steroid therapy was necessary. The skin biopsy performed on the 27th day revealed the distinctive pathological findings of EC (eosinophil infiltration and "flame figures" between the dermis and the subcutaneous fat tissue) and she was finally given a diagnosis of EC. It was indicated that sIL-2R level was related to the condition of EC in her clinical course. Her serum sIL-2R level lowered to 705 U/ml on the 29th day. After five weeks of steroid therapy, she had no recurrence of the disease after treatment. Although EC is an uncommon condition in pediatric patients, the serum sIL-2R level in patients with EC may be a useful parameter in evaluation of the disease state and the efficacy of steroid therapy and in decision-making for complete medical treatment.
|
|
Case Report
Title
A Case of Bladder and Bowel Dysfunction (BBD) with Recurrent UTI Preventable by Constipation Treatment
Author
Yuta Oonuki Hirokazu Ikeda Chisato Oyake Chizu Hirabayashi Masaki Fuyama Tsuneki Watanabe and Keiichi Isoyama
Department of Pediatrics, Showa University Fujigaoka Hospital
Abstract
Bladder and Bowel dysfunction (BBD) is a syndrome including dyschezia and dysuria. BBD are often complicated by vesicoureteral reflux (VUR) and reflux nephropathy (RN). We present a 7-year-old girl with frequent UTI and VUR. She had been affected many times with unidentified fever from the neonatal period, and she recovered with antibiotics each time. Voiding cystourethrogram (VCUG) showed grade III bilateral VUR. Dimercapto-succinic acid scans (99mTc-DMSA) showed functional abnormality of right kidney. X-ray scans showed her constipation. We began treatment with lactulose and ordinary urination. After the initiation of therapy, UTI also did not occur once. In the case of VUR with BBD, we should treat BBD more aggressively.
|
|
Case Report
Title
Two Cases of Polycystic Ovarian Syndrome with School Absenteeism
Author
Hidetoshi Hagiwara1) Akira Motegi1)2) Ayako Ozawa2) Itsumi Hashimoto1) Shuichi Suzuki1) and Shigeaki Nonoyama1)
1)Department of Pediatrics, National Defense Medical College
2)Department of Pediatrics, National Hospital Organization Nishisaitama Chuo National Hospital
Abstract
Polycystic ovarian syndrome (PCOS) is an endocrine disorder, which causes menstrual disorder, polycystic ovary and hyperandrogenaemia. Although the etiology is still unknown, PCOS is well recognized as a syndrome leading to multiple clinical conditions. We reported two cases of polycystic ovarian syndrome with school absenteeism. One patient visited our hospital for obesity, secondary amenorrhea and school absenteeism, and was subsequently diagnosed with PCOS. The other patient, who had been receiving medical attention for school absenteeism and obesity, was diagnosed with PCOS derived from ovulatory dysfunction. It is not easy to diagnose PCOS in the early stage. However, our cases might suggest that school absenteeism, linked with obesity and ovulatory dysfunction, is one of the early signs that there might be a diagnosis of PCOS. This report shows the importance of early diagnosis and treatment for PCOS patients with school absenteeism, obesity and/or ovulatory dysfunction.
|
|
Case Report
Title
Efficacy of Colchicine for Recurrent Abdominal Pain and Persistent Rash of Chronic IgA Vasculitis: A Case Report and Literature Review
Author
Yoko Yamaguchi Yusuke Okubo Takahiro Matsushima Ayako Nigo Satoko Suzuki Hiroshi Sakakibara Toshiro Terakawa and Hiroshi Hataya
Department of General Pediatrics, Tokyo Metropolitan Children's Medical Center
Abstract
IgA vasculitis is a common leukocytoclastic vasculitis in children, characterized by palpable purpura, arthritis, gastrointestinal symptoms, and nephritis. Corticosteroids are effective to treat gastrointestinal symptoms for limited time periods, however no therapy has yet been known to prevent recurrences of abdominal manifestations and decrease the duration of IgA vasculitis.
An 8-year-old boy presented with abdominal pain and typical palpable purpura over his legs. The diagnosis of IgA vasculitis was made, and intravenous corticosteroids were initiated. When the doses of corticosteroids were reduced, the skin lesions increased in number with recurrence of abdominal pain and vomiting over 4 months. We started colchicine in addition to corticosteroids, and most skin lesions dramatically diminished, and abdominal symptoms did not recur. The patient remained on a course of colchicine for the next 6 months with no recurrence. After this period, colchicine dose was tapered off, and he did not recur after that.
The patient with chronic IgA vasculitis was successfully treated with colchicine, added to corticosteroids, to control the longstanding symptoms. We reviewed the literatures of similar cases, and they showed excellent response to colchicine. Therefore, colchicine should be one of options for the treatment of chronic IgA vasculitis.
|
|
Brief Report
Title
Safety Study on Simultaneous Administration of Vaccines and Palivizumab in Children
Author
Satoshi Toishi1) Naruhiko Ishiwada2) Yoshiteru Ohsone3) Mamiko Endo4) Haruka Hishiki4) Hiromichi Nakajima5) Yasutaka Kawasoe6) Setsuo Ota7) Hiromichi Hamada8) Masaru Terai8) and Junichi Sato9)
1)Department of Neonatology, Narita Red-cross Hospital
2)Department of Infectious Diseases, Medical Mycology Research Center, Chiba University
3)Department of Neonatology, Kimitsu Chuo Hospital
4)Department of Pediatrics, Chiba University Hospital
5)Division of Cardiology, Chiba Children's Hospital
6)Department of Pediatrics, Chiba Cerebral and Cardiovascular Center
7)Department of Pediatrics, Teikyo University Chiba Medical Center
8)Department of Pediatrics, Tokyo Womens' Medical University Yachiyo Medical Center
9)Departemnt of Pediatrics, Funabashi Municipal Medical Center
Abstract
There is no data on the safety of simultaneous administration of vaccines and palivizumab. To investigate the safety of simultaneous administration of vaccines and palivizumab in children, we conducted a multicenter, nonblinded, observational clinical study. The simultaneous administration of vaccines and palivizumab were safe and well tolerated with no related serious adverse events. The simultaneous administration of vaccines and palivizumab is useful for the children and their guardians because of appropriate prevention of infectious diseases and reduction of the numbers of visits to medical care providers.
|
|
|
Back number |
|
