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THE JOURNAL OF THE JAPAN PEDIATRIC SOCIETY
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Vol.126, No.12, December 2022
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Original Article
Title
Effects of Revisions to the Medical Fee System on Additional Treatment Selection for Kawasaki Disease Patients Resistant to Initial Immunoglobulin Therapy
Author
Ushio Ohtaki1)2) Yoshikazu Nakamura1) and Mayumi Yashiro1)
1)Division of Public Health, Center for Community Medicine, Jichi Medical University
2)Department of Neonatal Health and Development, Aomori Prefectural Central Hospital
Abstract
[Purpose] The selection of additional treatment for patients resistant to initial intravenous immunoglobulin (IVIG) therapy was examined as an example of the effect of medical fee system revisions on treatment selection. [Subjects and methods] Using data for 4, 150 patients resistant to initial IVIG therapy from the 22nd nationwide survey of Kawasaki disease (January 2011 to December 31, 2012), odds ratios were calculated for additional treatments and the medical fee system. In addition, using the results of the 17th to 22nd surveys, the trends with time were shown for additional IVIG patients before and after the introduction of a diagnostic procedure combination/per-diem payment system (DPC/PDPS) at 82 advanced treatment hospitals. [Results] Of the 3, 798 patients (91.5%) who received addition administration of IVIG, the DPC/PDPS was used with 3, 339 patients and a fee-for-services system was used with 366 patients. The odds ratio was 0.68. Steroids were administered to 1, 245 patients (30.0%), among whom the DPC/PDPS was used for 1, 084 patients and a fee-for-services system was used for 139 patients. The odds ratio was 0.76. A temporary inhibitory effect was seen in advanced treatment hospitals for additional IVIG treatment. [Discussion] System revisions temporarily affect and may change treatment plan selection.
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Original Article
Title
Risk-stratification Treatment Based on the RAISE Study in Patients with Kawasaki Disease: Cardiac Prognosis Up to 1-year
Author
Akihiro Nakao Aya Iwata Kyouhei Iio Kaya Kim Akiyoshi Naito Yu Kawasaki Kenichi Isome Masayuki Hori Yoshimichi Yamaguchi Yoshimi Hiraumi and Kousaku Matsubara
Department of Pediatrics, Kobe City Nishi-Kobe Medical Center
Abstract
Since the publication of the RAISE study, an increasing number of Kawasaki disease (KD) patients have received primary adjunctive corticosteroid therapy. However, the RAISE study did not include incomplete KD cases or those with coronary artery dilation at diagnosis, and did not assess the long-term prognosis beyond the first month. We applied a steroid combination therapy based on the IVIG refractory score (Kobayashi score) in the RAISE study for all Kawasaki disease patients, including those with incomplete cases and those with pre-treatment coronary dilation, and retrospectively examined the IVIG refractory rate and coronary artery involvement from the acute phase to 1 year after onset. Between 2016 and 2020, a total of 254 KD patients (53 [20.9%] with incomplete cases and 98 [38.6%] with IVIG plus corticosteroid treatment) were identified. The IVIG refractory rate was 13.4%, and coronary artery dilation/aneurysm was observed in 4 patients (1.6%) after 1−2 months and in 1 patient (0.4%) after 1 year. The median coronary Z-scores of 98 KD patients with IVIG plus corticosteroid treatment did not increase from pre-treatment to the acute phase (pre-treatment/acute phase: segment #1, 0.8/0.8; #5, 0.7/0.8; #6, 0.6/0.6), and significantly decreased after 1−2 months (#1 0.5, #5 0.3, #6 0.2, each p<0.001) and after 1 year (#1 0.3, #5 0.2, #6 0.1, each p<0.001) compared with the pre-treatment phase. Of the 13 patients with coronary artery dilation at diagnosis, 4 had further dilation during the acute phase and only 1 with a low Kobayashi score had dilation after 1−2 months, but all patients normalized after 1 year. This study suggests that primary risk-stratification treatment based on the RAISE study is effective and results in a good 1-year cardiac prognosis in all KD patients.
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Case Report
Title
A 13-year-old Girl with Basedow Disease Presenting with Preceding Chronic Progressive Muscle Weakness
Author
Aiko Hirano Rika Hashi Ayumu Takehara Fumiko Torigoe Sanae Eto Koji Tsuchiya Atsushi Yamauchi Masahiko Kai and Takeshi Okinaga
Division of Pediatrics, Belland General Hospital
Abstract
Both hyperthyroidism and hypothyroidism often cause signs and symptoms of neuromuscular dysfunction, such as muscle weakness and cramps. Reports of thyrotoxic myopathy in children are extremely rare. Symptoms of thyrotoxic myopathy include chronic progressive muscle weakness, which mainly affects the proximal muscle groups, and symptoms of myopathy, which recover after the correction of hyperthyroidism.
We report a case of a 13-year-old girl who was diagnosed with Basedow disease who presented with preceding chronic progressive muscle weakness. Six months prior to admission, she was treated with midodrine hydrochloride for orthostatic disturbance due to difficulty waking up in the morning and dizziness; at that time, she was euthyroid. Despite medication, she felt weakness in her hand grip power and experienced general fatigue easily. One month prior to admission, she often fell down while running and experienced great fatigue even while walking. She was admitted to our hospital.
Her serum creatine kinase and anti-acetylcholine receptor antibody levels, motor nerve conduction study results, and peripheral nerve repeated stimulation test results were normal. Elevated thyroid hormones and decreased thyroid stimulating hormone suggested the presence of thyrotoxic myopathy. Treatment with thiamazole (15 mg/day) led to a euthyroid status and recovery of muscle strength to a normal level after 4 months.
Thyroid function examinations should be considered for children who present with chronic progressive muscle weakness and treatment of Basedow disease should be prioritized over examinations, such as electromyography and muscle biopsy for patients diagnosed with thyrotoxic myopathy.
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Case Report
Title
A Recruitment Maneuver Improves Severe Atelectasis in a Pediatric Case of Neuromuscular Disease
Author
Chisato Sawatari1) Satoshi Hayano1) Satoru Matsushima2) Ryuhei Yasuoka3) Kei Asada2) Junichiro Katsuki1) Ryosuke Shiozawa1) Keigo Seki1) Soichiro Yata1) and Satoru Iwashima1)
1)Department of Pediatrics, Chutoen General Medical Center
2)Department of Emergency and Critical Care Medicine, Chutoen General Medical Center
3)Department of Pediatrics, Hamamatsu University School of Medicine
Abstract
Children are individuals at high risk for atelectasis. It is challenging to treat atelectasis in children with neuromuscular disease. Therefore, supportive measures, including positioning and cough-assist devices, are recommended in treating pediatric acute respiratory failure. Atelectasis in children with neuromuscular disease may not respond to the standard respiratory physiotherapy due to weakness in the respiratory muscle and poor airway clearance. A recruitment maneuver (RM) is a procedure for expanding alveoli by temporarily using high ventilation pressure for adult acute respiratory distress syndrome. There is a paucity of evidence of RM for treating severe atelectasis, though the preventive effect of RM was reported for atelectasis in children under general anesthesia. We report a 2-year-old boy with cortical dysplasia (polymicrogyria) who underwent RM for severe atelectasis. After undergoing tracheostomy with home mechanical ventilation, he was hospitalized because of hypoxemia due to atelectasis. Atelectasis was difficult to treat with standard respiratory physiotherapy, and oxygenation was unstable with a fraction of inspiratory oxygen (FIO2) of 0.45. RM was performed by gradually increasing the positive end-expiratory pressure (8−20 cmH2O) with a constant driving pressure (ΔP=10 cmH2O). Oxygenation immediately improved, and atelectasis disappeared after RM. RM is an effective intervention for severe atelectasis associated with pediatric neuromuscular disease.
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Case Report
Title
Acute Ethanol Intoxication Following Intentional Ingestion of Alcohol-based Hand Sanitizer
Author
Tomohiro Hirade Daisuke Koike Rikako Okamura Yasuhiro Haneda and Rie Kanai
Department of Pediatrics, Shimane Prefectural Central Hospital
Abstract
Since hand hygiene has been recommended to prevent SARS-CoV-2, alcohol-based hand sanitizer is prevalent and stored in public and domestic places. Therefore, the number of reports about pediatric ethanol intoxication induced by unintentional or intentional ingestion of hand sanitizer has increased worldwide. A 5-year-old girl was transported to our emergency department with unconsciousness from her kindergarten. After she recovered consciousness, she described that she had secretly ingested alcohol-based hand sanitizer in the kindergarten. As the symptoms of ethanol intoxication are not specific, the diagnosis may be difficult without a trace or witnesses. However, ethanol intoxication can also result in death due to hypoglycemia. Therefore, ethanol intoxication should be considered a differential diagnosis of unconsciousness in children. A rise in pediatric ethanol intoxication cases is expected during the COVID-19 pandemic. The most important precaution for pediatric ethanol intoxication related to hand sanitizers is to keep them out of reach and sight of children. Furthermore, pediatricians should educate society about the possibility of ethanol intoxication in children by ingesting even a small amount of alcohol-based sanitizer.
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Case Report
Title
An Infant with Severe Diaper Rash in the Context of Constipation with Overflow Incontinence and Medical Neglect
Author
Shota Hiruma1) Yukiko Kanna1) Takumi Fujimura2) Kotaro Umezawa1) Yoshinori Mishima1) Nao Akiyama1) Mizue Tomita1) Masako Ikemiyagi1) Maki Nakazawa1) and Isamu Kamimaki1)
1)Department of Pediatrics, National Hospital Organization Saitama Hospital
2)Department of Pediatric Surgery, National Hospital Organization Saitama Hospital
Abstract
A 3-year 5-month-old boy, presenting with frequent fecal incontinence, was referred to our hospital by the child guidance center on suspicion of neglect. On examination, multiple, scattered ulcers and granulations were noted on the buttock fissures that suggested granuloma gluteale infantum (GGI). An abdominal radiography revealed fecal retention in the colon, which was thought to be a symptom of overflow fecal incontinence and prolonged diaper rash. Hospitalization, treatment of constipation, and skin care improved the skin symptoms. GGI is a rare disease caused by negligence in addition to overflow fecal incontinence associated with severe constipation.
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