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THE JOURNAL OF THE JAPAN PEDIATRIC SOCIETY
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Vol.129, No.7, July 2025
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Original Article
Title
Survey on the Current Situation and Difficulties in Transitioning Children with Advanced Cancer to Home
Author
Tomoko Yokosuka1) Nobuyuki Yotani2) Yuko Cho3) Mai Ito4) and Tomoo Osumi5)6)
1)Department of Hematology/Oncology, Kanagawa Children's Medical Center
2)Department of Palliative Medicine, National Center for Child Health and Development
3)Department of Pediatrics, Hokkaido University
4)Department of Child Life, National Center for Child Health and Development
5)Children's Cancer Center, National Center for Child Health and Development
6)Home Care Clinic Aozora, Medical Incorporated Foundation Harutaka Kai
Abstract
Some children with advanced cancer hope to spend the rest of their time at home, but the process of returning home is complicated. This study aimed to clarify the current situation of home transition for children with advanced cancer and examine the difficulties doctors face. A total of 532 pediatric hematologists and oncologists in Japan were included in this study, half of whom were certified by the Japanese Society of Pediatric Hematology and Oncology, and the other half were not. We mailed a questionnaire and a return envelope to each physician, asking the following questions: their views toward pediatric cancer treatment, their perspectives on end-of life discussions with patients and their families, and the current situation and difficulties of sharing information with local medical teams. In total, 291 physicians responded (response rate: 55%). Seventy-five percent of the physicians had experiences with home transition for children with advanced cancer. Compared to the certified pediatric oncologists, the non-certified ones found it more difficult to decide when to make transition to home (P<0.01). Forty-two percent of the physicians were concerned that the family might feel transition to home would mean physicians giving up on providing care. Only 40% of physicians confirmed how well the patient understood his/her medical condition. Several factors could be barriers to transiting to home, including values and conflicts the physicians have, lack of discussion with the patient, insufficiency of home resources, and lack of information provided to the patient.
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Original Article
Title
A Questionnaire on Mental and Physical Health for School Medical Check-ups
Author
Chizu Habukawa1)2) Toru Nakamura2) Kenshi Koyanagi3) and Katsumi Murakami4)
1)Department of Pediatric Allergy, Minami Wakayama Medical Center
2)Osaka University Graduate School of Engineering Science
3)Nagasaki Prefectural Center of Medicine and Welfare for Children
4)Department of Psychosomatic Medicine, Sakai Sakibana Hospital
Abstract
To prevent school absenteeism, we have developed a self-completed health questionnaire for early detection of mental and physical disorders during a school health examination. First, a basic questionnaire consisting of 46 items was created by specialists using a 4-point Likert scale: "yes,""somewhat applicable,""not very applicable," and "not applicable." Next, the questionnaire was compared with the Questionnaire for Triage and Assessment with 30 items (QTA30) and the Questionnaire on Child Strength and Difficulties (SDQ) to probe its properties, validity and reliability for 4,306 students from the 5th grade of elementary school to the 3rd grade of junior high school. The questionnaire was then checked in a test-retest for 2,355 junior high school students. Factor analysis of the structure of the 25-item scale of the questionnaire revealed three factors: "degree of nervousness" (9 items), "life rhythm / physical hardship" (10 items) and "problems at school" (6 items). The questionnaire showed high reliability (Cronbach's alpha = 0.84, 0.82, 0.74, respectively) and test-retest reliability (Pearson's correlation coefficient = 0.88). Validity was confirmed by the high correlations of the questionnaire with the QTA30 and SDQ (0.81, 0.66, respectively). A cutoff for the questionnaire (sensitivity 84.1%, specificity: 85.1%, AUC 0.92) was calculated using the QTA30 cutoff of 37 points for distinguishing between children with psychosomatic disorders and healthy children. The questionnaire can identify problems faced by children in school life based on three factors and offer appropriate support for mental and physical health problems in school life.
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Case Report
Title
A Young Child with Autoimmune GFAP Astrocytopathy with Bilateral Symmetrical Cerebellar Lesions
Author
Haruka Uehara1) Masato Mori1) Masaru Jinza1) Hiroaki Obayashi1) Hajime Ikehara1) and Harushi Mori2)
1)Department of Pediatrics, Matsudo City General Hospital, Children's Medical Centre
2)Department of Radiology, School of Medicine, Jichi Medical University
Abstract
Autoimmune GFAP (glial fibrillary acidic protein) astrocytopathy presents with meningoencephalomyelitis that is relatively steroid responsive, with symptoms such as impaired consciousness, dysuria, dyskinesia, cognitive dysfunction, and hyponatremia. Typical imaging findings include linear perivascular radial enhancement around the lateral ventricles; however, only a few cases have been reported in Japanese children. The patient was a 2-year-old boy who presented with fever and somnolence, and was admitted to the hospital on the 6th day because of difficulty in sitting and walking. Decreased speech and trunk ataxia, as well as deep tendon reflex hyperreflexia and bilateral foot clonus were observed. Examination revealed a mild increase in CSF cell count, and brain MRI showed asymmetric lesions in the subcortical white matter, corpus callosum, and spinal cord, and symmetric lesions in the cerebellar hemispheres. He was diagnosed with acute encephalomyelitis, and three courses of steroid pulse therapy were administered. Thirty days after admission, he had recovered to the same level as before the disease. The diagnosis of autoimmune GFAP astrocytopathy was made based on a positive spinal fluid anti-GFAPα antibody test. The patient was only 2 years old, and his symptoms lacked the characteristic findings that differentiate autoimmune GFAP astrocytopathy from other encephalomyelitis, and his imaging was characterized by symmetrical lesions in the cerebellar hemispheres, which had not been reported before. It is possible that children may have different clinical pictures or imaging findings from those of adults, and more cases need to be accumulated.
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Case Report
Title
Prader-Willi Syndrome with 11 Mb Interstitial Deletion of Chromosome 15 Including the SNORD116 Gene Cluster but the Intact SNRPN Gene
Author
Akari Nakagawa1) Miwako Nagasaka1)2) Yuka Yotsumoto1)2) Shinji Saitoh3) Keiko Matsubara4) and Tomoko Tamaoki2)
1)Department of Pediatrics, Takatsuki General Hospital
2)Center for Clinical and Molecular Genetics, Takatsuki General Hospital
3)Department of Pediatrics and Neonatology, Nagoya City University Graduate School of Medical Sciences
4)Division of Diversity Research, National Research Institute for Child Health and Development
Abstract
Prader-Willi syndrome (PWS) has traditionally been classified as an imprinting disorder linked to the SNRPN gene on chromosome 15q11.2. PWS diagnosis is confirmed by detecting SNRPN gene deletion using fluorescence in situ hybridization or identifying methylation abnormalities in the promoter region of the SNRPN gene via methylation testing. In 2020, we encountered a patient in whom PWS was clinically diagnosed during the neonatal period. Initial G-banding revealed a deletion at 15q12-14. However, neither deletion of the SNRPN gene nor any methylation abnormalities were detected, and a definitive diagnosis of PWS could not be reached. Around 2008, on the other hand, emerging reports began to suggest that microdeletions near the SNORD116 gene cluster, located downstream to the SNRPN gene, are responsible for the major symptoms of PWS. Therefore, we performed a custom array comparative genomic hybridization test when the patient was 7 months old, focusing on the 15q11-13 region. A deletion involving the SNORD116 gene cluster as the proximal break point was detected, finally leading to the diagnosis of PWS. Subsequent genome-wide microarray analysis revealed an 11 Mb deletion extended distal to the SNORD116 gene cluster, including the region responsible for the 15q13.3 microdeletion syndrome. After we finally diagnosed this case as PWS, the 2021 PWS consensus guidelines in Japan were published, which defined PWS as a disorder characterized by the loss of expression of paternally expressed genes, including the SNORD116 gene cluster. At three years old, this patient shows typical PWS symptoms without those of 15q13.3 microdeletion syndrome.
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Case Report
Title
Secondary Nephrogenic Diabetes Insipidus Induced by Tosufloxacin
Author
Marina Iwakami Naoto Sakumura Mariko Honda Yoshikatsu Takeda Yusuke Yachi Kazuyuki Ueno Kengo Miyashita and Shuhei Fujita
Toyama Prefectural Central Hospital, Pediatrics
Abstract
Nephrogenic diabetes insipidus is a disorder in which the sensitivity of the renal collecting duct principal cells to vasopressin (AVP) is reduced, resulting in an increased urinary volume. Early recognition is important because this condition can lead to dehydration and electrolyte abnormalities. We report two cases of a 4-year-old girl with otitis media and a 7-year-old boy with bronchitis. Both patients presented to our hospital because of thirst, polydipsia, and polyuria after taking one dose and four doses of tosufloxacin (TFLX), respectively. Serum osmolality was 284 and 285 mOsm/kgH2O, and AVP was increased to 7.6 and 4.0 pg/mL, respectively. The 7-year-old boy also had dehydration and prerenal acute kidney injury. Both patients showed improvement in thirst and polyuria on the day after discontinuation of oral medication, and at the second visit, AVP had decreased to 2.7 and 3.0 pg/mL, and serum osmolality was 279 and 280 mOsm/kgH2O, respectively. In the differential diagnosis for polyuria, diabetes mellitus and urinary tract infection were negative, and urine protein and occult blood disappeared on the next day. Both patients were suspected to have diabetes insipidus, plus both had a high AVP level and even had symptoms while sleeping at night. Thus, central and primary diabetes insipidus were ruled out, and drug-induced diabetes insipidus due to TFLX was considered likely. When thirst, polydipsia, and polyuria occur in a patient taking TFLX, nephrogenic diabetes insipidus should be considered, and dehydration and electrolyte abnormalities should be noted.
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Case Report
Title
A Child with Severe Motor and Intellectual Disabilities Presenting with Thyroid Dysfunction Due to Levothyroxine Malabsorption Induced by Food Additives
Author
Hiroki Kumazawa Toshiki Kasagi Tatsushi Tanaka and Tsuyoshi Ito
Department of Pediatrics, Toyohashi Municipal Hospital
Abstract
The patient was a 16-year-old male adolescent who had developed severe brain damage due to unexplained encephalopathy at 1 year and 5 months of age and required long-term home mechanical ventilation. For central hypothyroidism associated with brain damage, he was receiving levothyroxine sodium hydrate (LT4) supplementation. Nutritional management was provided via a gastrostomy tube, including a semi-elemental formula and a protein supplement.
During a routine checkup, an increased heart rate was observed. Blood tests revealed elevated levels of free triiodothyronine (fT3) and free thyroxine (fT4), leading to a reduction in the LT4 dose. Initially, the cause of hyperthyroidism was unknown. However, it was later discovered that approximately two weeks prior to this checkup, the protein supplement had been switched to a sister product. It was subsequently found that the previous product contained calcium carbonate and ferric pyrophosphate, whereas the sister product did not.
It has been reported that LT4 absorption in the gastrointestinal tract is inhibited when co-administered with calcium carbonate or iron. In this case, after switching to the sister product, which lacked these components, it was considered that the absorption rate of LT4 increased. LT4 is a widely used medication, and many drugs have been reported to reduce its absorption when co-administered. In particular, calcium carbonate is widely used as a food additive for nutritional enhancement and texture improvement in various foods. Therefore, special attention should be paid when LT4 is taken simultaneously with such food components.
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Case Report
Title
A Boy Diagnosed with Paratyphoid Fever Following a Visit to Relatives in Pakistan
Author
Mizuho Ichikawa1) Kenzaburo Koide2) Takashi Tamaki1) Masaki Kouketsu1) Yuki Kunisawa1) Yuichi Shirakawa1) Tomoki Ueda1) Kazunori Haruta1) Maiko Ikeda1) Sachie Ito1) Sachi Matsuoka1) Ryoko Kimura1) Masayuki Hasegawa1) and Shin Hoshino1)
1)Department of Pediatrics, Nagoya Ekisaikai Hospital
2)Center for Medical Education and Training, Nagoya Ekisaikai Hospital
Abstract
This case report concerns a 7-year-old boy, a Pakistani national residing in Japan, who had spent one and a half months in Pakistan with his family. Seventeen days after returning to Japan, he developed persistent fever and diarrhea, which continued until Day 6 of illness, when he visited our hospital's emergency department. After receiving intravenous fluids, and undergoing blood tests and blood and stool cultures, he was discharged. However, on Day 7 of illness, his blood culture came back positive, and ceftriaxone treatment was initiated. On Day 8, he was admitted to the hospital. Although he was able to walk, he appeared lethargic with a slightly pale complexion. His abdomen was mildly distended, but there was no hepatosplenomegaly or tenderness. Ceftriaxone was continued, and based on the culture results, paratyphoid fever was diagnosed. Following admission, his appetite and bowel movements improved, though it took approximately one week for his fever to subside. A second blood culture, performed the day after starting antibiotics, was also positive, but sensitivity testing showed susceptibility to both ampicillin and third-generation cephalosporins. On Day 13 of illness, oral amoxicillin was started, and the medication was completed by Day 22. He showed no recurrence of symptoms after discharge. With the increasing number of international travelers and foreign residents, it is expected that healthcare providers may encounter imported infections in general pediatric practice, necessitating increased awareness.
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