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THE JOURNAL OF THE JAPAN PEDIATRIC SOCIETY
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Vol.129, No.4, April 2025
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Original Article
Title
A Questionnaire Survey on Nutritional Management by Pediatric Endocrinologists to Prevent Late Effects in Pediatric and Adolescent or Young Adult Cancer Patients
Author
Yoko Miyoshi1)2)3) Makiko Tachibana2) Naoko Nagai4) Tadashi Akao5) Masanori Adachi3) Junko Ito3) Kenichi Kashimada3) Junko Kanno3) Takeshi Sato3) Shizuyo Nagai3) Tomohiro Hori3) Reiko Horikawa3) Koji Muroya3) Shuntaro Morikawa3) and Takeshi Okada3)
1)Laboratory of Clinical Nutrition and Development, Department of Health and Nutrition, Osaka Shoin Women's University
2)Department of Pediatrics, Graduate School of Medicine, The University of Osaka
3)CCS and Endocrine Tumor Committee in the Japanese Society of Pediatric Endocrinology
4)Division of Nutritional Management, The University of Osaka Hospital
5)First Laboratory of Institutional Food Service Management, Department of Health and Nutrition, Osaka Shoin Women's University
Abstract
Pediatric and adolescent or young adult (AYA) cancer patients are at significant risk of late effects following treatment. Nutritional management, including diet, is crucial in preventing and managing endocrine and metabolic disorders, which are common in such patients. To assess the status and problems regarding the nutritional management of cancer patients by pediatric endocrinologists, we conducted a questionnaire survey in 2022. The primary survey was distributed to 171 council members of the Japan Society of Pediatric Endocrinology, and 158 responses (response rate: 92.4%) were obtained. Among these, 141 (89.2%) had experienced receiving requests for nutritional guidance, and 118 (74.7%) had treated pediatric and AYA cancer patients. A second survey was then conducted among the 107 respondents who had experience treating cancer patients and approved an additional survey, to which 104 responded (response rate: 97.2%). Among the secondary survey participants, 43 (41.3%) had requested nutritional guidance for cancer patients, with more than half making 1-9 requests per year. These requests typically included dietary management advice for obesity and food intake. The time of the request was one year or more after discharge. Among the 104 respondents in the secondary survey, the main symptoms that caused problems with nutritional management were obesity, growth disorders, loss of appetite, and weight loss. The main problems in nutritional management were lack of knowledge and understanding by health care providers and low awareness of nutritional guidance. This survey highlighted the need to raise awareness and improve nutritional management to prevent late complications in pediatric and AYA cancer patients.
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Original Article
Title
The Prevalence of Pediatric Brain-death Patients and the Factors That Prevent Organ Donation in a Children's Hospital: A Descriptive Study
Author
Chisato Akita Mitsunori Sato Ken Yakame Yosuke Nakano Saori Aiga Tadashi Oi Sanae Sato and Tatsuya Kawasaki
Shizuoka Children's Hospital, Pediatric Intensive Care Unit
Abstract
The number of pediatric brain-dead organ donors (ped-donors) in Japan remains low, and the gap between the number of ped-donors and that of patients on the transplant waiting list is widening. Various factors have been reported that prevent an increase in the number of ped-donors, but it is unclear to what extent these factors are influencing it. In this study, we describe the epidemiological profile of determination of brain-death, provision of information related to organ donation (OP), and the reasons for choosing not to donate organs in our children's hospital over a 12-year period from the enactment of the revised Organ Transplant Law (July 10, 2011) till July 9, 2022. We then investigated the modifiable factors that could increase the number of ped-donors. Patients diagnosed as neurologically-determined death accounted for 8% (35/435) of all in-hospital deaths. The rate of general brain-death determination (GBDD) was 44%, of which 43 patients were suspected to be brain-dead but did not undergo GBDD. The reasons why 29 of them did not undergo GBDD were not documented. Among the 16 patients who declined to donate organs, 6 patients pointed to psychological resistance to organ procurement surgery, which was the most common reason. In this study, the factor that could most strongly affect the number of ped-donors was the lack of GBDD. The number of ped-donors could be increased by ensuring that all the patients suspected brain-dead should undergo GBDD and that their families are informed of opportunities for organ donation whenever it is feasible.
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Case Report
Title
Kawasaki Disease with Marked Gallbladder Enlargement during Adenovirus Infection
Author
Masako Koga1) Tatsuya Miyata1) Kanako Higashi1) Yumi Keida1) Junko Fujiyoshi1) Yuki Matsushita1) Hiroki Kai2) and Shunsuke Kanno1)
1)Department of Pediatrics, NHO Kyushu Medical Center
2)Department of Pediatric Surgery, NHO Kyushu Medical Center
Abstract
We report a case of 3-year-old boy with Kawasaki disease (KD), which was finally diagnosed on day 8, with marked gallbladder enlargement during adenovirus infection. The patient was admitted on day 6 due to high fever with remarkable inflammatory response. Although conjunctivitis was observed, we considered it was due to adenovirus infection by the positive result of the rapid antigen test. Because of abdominal distension, jaundice, and significant gallbladder enlargement detected by ultrasonography, we started intravenous antimicrobiotics according to acute cholecystitis therapy. The patient did not respond to treatment, and red lips and changes in his extremities became apparent. Therefore, we diagnosed KD on day 8. Symptoms improved with oral aspirin and two courses of intravenous immunoglobulin on day 8 and 10, and no coronary artery lesion was observed until two months after the onset. The gallbladder size reduced on ultrasonography 1 month after the onset, and neither gallstone nor pancreaticobiliary maljunction was observed on MR cholangiopancreatography, suggesting that gallbladder enlargement was a complication of KD.
Symptoms of adenovirus infection can mimic the major symptoms of KD, therefore early diagnosis of KD is challenging. Although gallbladder enlargement is one of the useful diagnostic signs of KD, marked gallbladder enlargement, as in the present case, is unusual. Because therapeutic delay in KD can lead to clinically serious outcomes such as coronary artery aneurysms, it is important to understand the findings which makes differentiation from KD difficult, as in this case.
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Case Report
Title
A Case of Relapsed Hodgkin Lymphoma Treated with HLA-haploidentical Allogeneic Hematopoietic Stem Cell Transplantation with Post-transplant Cyclophosphamide after Immune Checkpoint Inhibitor Therapy
Author
Yuki Sakaki Kazumi Okamura Mutsuki Nakano Narumi Takemoto Hiroyoshi Watanabe and Maki Urushihara
Department of Pediatrics, Tokushima University Hospital
Abstract
Anti-programmed cell death protein-1 (PD-1) antibodies, which are immune checkpoint inhibitors, are an option for treating relapsed or refractory Hodgkin lymphoma. They promote the activation of T cells and exhibit antitumor effects. While their use before allogeneic hematopoietic stem cell transplantation is expected to enhance antitumor immunity, it may exacerbate graft-versus-host disease (GVHD). The patient was a 16-year-old girl who had nodular sclerosing Hodgkin lymphoma (stage IIA) that was resistant to chemotherapy. She was originally treated with brentuximab vedotin (BV), but the disease recurred after treatment. BV was again administered, but the treatment was ineffective. She was switched to nivolumab and achieved partial remission. Because of the absence of complete remission, she underwent peripheral blood stem cell transplantation from an HLA haploidentical mother using fludarabine, melphalan, and 2 Gy of total body irradiation. Post-transplant cyclophosphamide (PTCy) was used to prevent GVHD. The interval between the last dose of nivolumab and transplantation was 80 days. Blood cell engraftment was uneventful and no acute GVHD was observed. Chronic GVHD (skin) resolved quickly with oral prednisolone. She remains in remission over 1 year post-transplantation. Allogeneic hematopoietic stem cell transplantation with an HLA haploidentical donor is a good option for patients with relapsed or refractory Hodgkin lymphoma if a sufficient withdrawal period is provided after anti-PD-1 antibody therapy, and PTCy is used for GVHD prophylaxis.
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Case Report
Title
Recurrent Urinary Tract Infections Due to Vitamin A Deficiency Caused by Picky Eating
Author
Hikari Ando1) Yoshinobu Moritoki2) Daisuke Nakata3) and Hidetaka Nakai1)
1)Department of Pediatrics, Toyokawa City Hospital
2)Department of Urology, Toyokawa City Hospital
3)Department of Ophthalmology, Toyokawa City Hospital
Abstract
Vitamin A deficiency causes a variety of symptoms. Ocular symptoms are particularly important as the initial manifestation. It begins with night blindness and photophobia, progresses from ocular dryness to corneal softening, and eventually blindness. Prolonged vitamin A deficiency can also be a risk factor for developing various infectious diseases due to keratinization of the mucous membranes. We report a case of a boy who developed ocular symptoms and recurrent urinary tract infections (UTIs) due to vitamin A deficiency caused by picky eating. The patient was a 6-year-old boy with autistic spectrum disorder and intellectual developmental disorder who had been an extremely picky eater since the age of 1 year. He had 11 UTIs in about a year prior to admission, 7 of which were upper UTIs and required 3 hospitalizations. Ultrasound examination revealed no abnormal findings in the renal urinary tract system and no VUR. He had been diagnosed with dry eyes by an ophthalmologist for 6 months, but the cause was unknown, and at the time of admission for this UTI, he was accompanied by eyelid-opening difficulties. He was diagnosed with a corneal ulcer on ophthalmologic examination, and a close examination revealed vitamin A deficiency. Subsequently, vitamin A supplementation and antimicrobial therapy improved the ocular symptoms and cured the UTI. If a child with intellectual developmental disorder has recurrent infections, such as UTI, the patient's ocular complaints may not be sufficient, ophthalmologic examination and a detailed dietary interview should be performed to differentiate vitamin A deficiency.
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Case Report
Title
Cervical Spinal Cord Injuries Due to Forward-facing Use of Infant Safety Car Seats during Traffic Accidents
Author
Masato Takemoto Naohiro Ikeda Teppei Tahara Yasuko Morita Daisuke Hazeki Ryo Kusubae and Yuichi Nomura
Department of Pediatrics, Kagoshima City Hospital
Abstract
Case: An 11-month-old infant seated in a forward-facing safety car seat installed in the back seat was injured in a car accident. The infant was transferred to our hospital under ventilatory management on the day of the accident, and magnetic resonance imaging showed C1 and C2 level cervical cord injuries. Since the 5th day of injury, episodes of severe bradycardia and cardiac arrest of 10-30 s occurred frequently. Regular administration of intravenous atropine sulfate was initiated, and the frequency of extreme bradycardia decreased until it was not observed on the 16th day after injury. The patient's consciousness improved and her facial muscle movement recovered. However, she required mechanical ventilation and her quadriplegia persisted.
Discussion: Infants and young children have large heads. Therefore, it is crucial to acknowledge that their necks are subjected to strong loads during forward collisions. Forward-facing use of a safety car seat, such as in this case, may pose a risk of cervical spinal cord injury. Severe bradycardia is often observed after severe cervical spinal cord injury and is challenging to treat. Atropine sulfate treatment was effective in this case. The use of safety rear- or side-facing car seats is recommended for infants weighing less than 10 kg; thus, their proper installation is essential for preventing cervical spinal cord injuries, such as those in this case.
Conclusion: The prognosis of cervical spinal cord injury is extremely serious and its prevention is important. Therefore, awareness-raising activities are needed to ensure proper installation of safety car seats, including rear-facing seats, for young children.
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