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THE JOURNAL OF THE JAPAN PEDIATRIC SOCIETY
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Vol.126, No.9, September 2022
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Review
Title
Quality of Pediatric Health Care and Its Evaluation: From the Perspective of Patient- Centeredness
Author
Yu Inata1)2) and Etsuko Nakagami-Yamaguchi2)3)
1)Department of Intensive Care Medicine, Osaka Women's and Children's Hospital
2)Department of Medical Quality and Safety Science, Osaka City University (Osaka Metropolitan University) Graduate School of Medicine
3)Department of Quality and Safety Management, Osaka Metropolitan University Hospital
Abstract
With the recent decline in mortality rate in children, functional outcomes and quality of life have become important to healthcare providers, let alone to patients and their families. In addition, high-quality medical care based on what the patient values is increasingly desired. Healthcare quality is defined as "the degree to which health services for individuals and populations increase the likelihood of desired health outcomes and are consistent with current professional knowledge." There is growing acknowledgment that quality health services should be effective, safe, and patient-centered. Meanwhile, the health system needs to be efficient to maximize the social value of healthcare. To improve healthcare quality, its measurement is essential and must be promoted. However, we must not lose sight of patient-centeredness in the process. To assess the quality of care from a patient and family perspective, patient-reported outcome measures and patient-reported experience measures have been developed and utilized. For these measures to be truly meaningful to patients and families, their participation in the selection and development of these measures is required. However, such patient and family participation cannot be achieved without protecting children's rights. For patient- and family-centered care, the child should be placed at the center of pediatric care with support from family members. For better quality of pediatric care in Japan, it is essential to respect children's rights and promote patient and family participation in healthcare facilities. Their participation would inevitably raise the awareness of both healthcare professionals and patients/families regarding safety and efficacy; thereby leading to improvement in the quality of healthcare.
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Original Article
Title
Atypical Kawasaki Disease Initially Presenting with Abdominal Pain and Peritoneal Irritation Sign: A Case Report and Literature Review
Author
Hiroki Kitaoka1)2) Yuko Kajiho1) Keiho Owada1) Eri Ohashi1) Chie Takahashi1) Syoichiro Kanda1) Yutaka Harita1) and Akira Oka1)
1)Department of Pediatrics, The University of Tokyo Hospital
2)Department of Pediatrics, Yaizu City Hospital
Abstract
A 6-year-old boy was referred to our institute due to a history of high fever and abdominal pain for 2 days. Abdominal ultrasonography revealed intestinal edema in the ascending colon with no signs of appendicitis. We diagnosed the patient with bacterial colitis and administered cefmetazole and amikacin intravenously. Eight days after symptom onset, the patient developed conjunctival injection and brawny edema of the dorsa of the hands, due to which he was diagnosed with atypical KD on day 9. The fever and abdominal pain resolved on day 10 after administration of intravenous immunoglobulin and aspirin. Coronary artery aneurysm was not detected throughout the disease course. The diagnosis of KD was difficult in this case due to the predominance of abdominal symptoms, and the main symptoms of KD were revealed only in the latter half of the course. Our review and analysis of previous literature on KD presenting with abdominal pain at onset revealed a high incidence of atypical KD. It is therefore critical for physicians to consider that abdominal onset KD is associated with a higher incidence of atypical KD and a higher incidence of coronary aneurysms.
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Original Article
Title
Atlantoaxial Rotatory Fixation as a Complication of Kawasaki Disease: A Series of 9 Cases
Author
Kenta Igami Itsuhiro Oka Nozomi Satoh Musashi Hibio Asuka Fujii Shimpei Matsuda Mei Kato Kenji Oishi Yudai Toyama Nobuyasu Arai Noboru Yoshida Atsushi Tsujiwaki Koji Sakuraya Naho Ikeda Kyoko Suzuki and Yoshiyuki Ohtomo
Department of Pediatrics, Juntendo University Nerima Hospital
Abstract
Atlantoaxial rotary fixation (AARF) is a rare cause of torticollis in children, which sometimes presents as a complication of Kawasaki disease. We retrospectively reviewed 9 cases of AARF with Kawasaki disease, treated at our hospital over the last 7 years (2013−2020). There were 6 cases of Fielding classification type I, and 3 cases of Fielding classification type II. All patients recovered without surgery (rest: 4 cases, neck collar fixation: 4 cases, Glisson traction: 1 case) and did not relapse. A patient that required Glisson traction was presented to the hospital on day 11, which was later than the other 8 patients. Age and CRP were more elevated among Kawasaki disease patients with AARF than Kawasaki disease patients without AARF, and the difference was statistically significant. All patients presented with lymph node adenopathy on admission. Early intervention is key to early recovery. AARF patients will recover without any intervention, even if the CRP levels are significantly elevated, if they fall under Fielding classification type I.
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Case Report
Title
Three Cases of Growth Hormone Treatment for Short Stature after the Fontan Procedure
Author
Kazuhisa Naruse1)2) Noriko Nagai2) Yoshihiro Nagata2)3) Hiroshi Nishikawa3) Taichi Kato4) and Reizou Baba5)
1)Department of Pediatrics, Nakatsugawa Municipal General Hospital
2)Department of Pediatrics, Okazaki City Hospital
3)Department of Pediatric Cardiology, JCHO Chukyo Hospital
4)Department of Pediatrics, Nagoya University Hospital
5)College of Life and Health Sciences, Chubu University
Abstract
Patients who have undergone the Fontan procedure often have a short stature and are prescribed growth hormone (GH) therapy. However, GH has aldosterone-like effects, and its administration results in increased central venous pressure and fluid retention that may increase the risk of developing protein-losing gastroenteropathy (PLE). We administered GH to three patients who underwent the Fontan procedure. In the first case, GH treatment improved not only the patient's short stature but also heart failure. However, the other two patients developed PLE during the GH treatment, and the treatment had to be stopped. When GH treatment is administered to patients after the Fontan procedure, it should be followed carefully, with attention paid to the complications of PLE.
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Case Report
Title
Report of Two Pediatric Cases of Primary Ciliary Dyskinesia Caused by Novel Copy Number Variation in DRC1 in One Family
Author
Hirotoshi Makino Hisashi Nishimori Atsushi Koso Tomoya Harada Katsuhiko Ito Mai Kobayashi Naofumi Suzuki Keiko Yamaguchi Yusuke Omori Hodaka Ota and Kenji Sugiyama
Mie Prefectural General Medical Center
Abstract
Primary ciliary dyskinesia (PCD) is a genetic disorder of ciliary motility. PCD has diverse characteristic clinical manifestations, such as chronic sinusitis, otitis media, bronchiectasis, infertility and situs inversus.
We report two pediatric PCD cases without situs inversus in siblings. They suffered from recurrent productive cough, rhinorrhea and nasal obstruction since infancy. They had been diagnosed with bronchitis and sinusitis and were treated with bronchodilators, expectorants and suction of nasal fluids. Although PCD was suspected clinically, it could not be diagnosed definitively for many years because of non-specific electron microscopy (EM) findings. A definitive diagnosis of PCD was finally made when whole-exome sequencing revealed novel copy number variations of DRC1.
Recent advances in gene sequencing techniques have led to the identification of a growing number of PCD-associated genes. Situs inversus is often absent in PCD patients with the DRC1 alteration, which is one of the most prevalent causes of PCD in Japanese populations. PCD patients present several nonspecific clinical symptoms, such as cough and rhinorrhea. Insufficient care, which can lead to bronchiectasis and impaired pulmonary function, should be avoided. Patients with neonatal respiratory distress and persistent rhinosinusitis during infancy should be followed carefully, and diagnostic procedures for PCD, such as analysis of nasal nitric oxide levels, EM analysis and genetic analysis, should be performed when indicated.
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Case Report
Title
A Child with Severe Developmental Delay and NACC1 Missense Variant Mosaicism
Author
Yutaro Hara1) Masahiro Toyoshima1) Yoshitaka Tanaka1) Hiromu Ukai1) Saki Noda1) Hideaki Fukushima1) Natsuo Nanki1) Shota Kawasaki1) Natsumi Hada1) Hiroyuki Kawabe1) Jun Okamura1) Yoshiaki Nagaya1) Tsuyoshi Sahashi1) Yoshishige Miyake1) Tomoo Ogi2) and Shinji Saito3)
1)Department of Pediatrics, Ichinomiya Municipal Hospital
2)Department of Genetics, Research Institute of Environmental Medicine, Nagoya University
3)Department of Pediatrics and Neonatology, Nagoya City University Graduate School of Medical Sciences
Abstract
A pathological variant of the nucleus accumbens associated 1 gene (NACC1) (MIM *619672) causes neurodevelopmental disorder with epilepsy, cataracts, feeding difficulties, and delayed brain myelination (MIM #617393). However, to the best of our knowledge, only a few cases have been reported to date, and none in Japan. We encountered, for the first time in Japan, a patient presenting with severe mental retardation, intractable epilepsy, cataracts, etc., and who was found to have a missense variant (NM_052876.4: c.892C>T [p.Arg298Trp]), and herein report our findings. The frequency of the variant allele was 12.2%, and the possibility of mosaicism was considered. Unlike previously reported cases, this case did not demonstrate a feeding disorder, dysphagia, nor severe food intolerance requiring gastrostomy, suggesting a relationship between mosaicism and clinical symptoms.
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Case Report
Title
A Case of Multiple Intestinal Perforations Caused by Accidental Ingestion of a Large Number of Neodymium Magnets in an Infant
Author
Iori Wakabayashi1) Emi Ishibazawa1) Yoichiro Yoshida1) Tsunehisa Nagamori1) Daisuke Ishii2) Hisayuki Miyagi2) Masatoshi Hirasawa2) and Satoru Takahashi1)
1)Department of Pediatrics, Asahikawa Medical University
2)Division of Pediatric Surgery, Department of Surgery, Asahikawa Medical University
Abstract
Accidental ingestion of foreign bodies among children is a frequently encountered disorder. As for magnets, a single piece is expected to be excreted, however, it becomes a critical condition when multiple pieces are ingested, because of the possible complications of intestinal ulceration and perforation. Here, we report a case of an infant who ingested a remarkably large number of magnets causing multiple intestinal perforations.
An 11-month-old male infant swallowed neodymium magnets while playing with them, but did not have dyspnea or abdominal symptoms. Therefore, the patient was placed under observation. On the third sick day, vomiting was observed, and he was transferred to our hospital because of the possibility of surgical treatment. He underwent an emergency operation at the day of transport because he presented acute abdomen. Intraoperative findings showed 58 magnet balls in the small intestine and stomach, and many intestinal perforations in the small intestine.
In recent years, the Consumer Affairs Agency in Japan has issued an investigation report in response to consecutive serious accidents due to the ingestion of neodymium magnets in infants and children. In addition to proper management, and enlightening the general public about this dangerous condition, establishment of legal restrictions for distribution in accordance with international standards may be required.
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