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THE JOURNAL OF THE JAPAN PEDIATRIC SOCIETY
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Vol.128, No.8, August 2024
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Review
Title
Pediatric Neurocritical Care for Brain Protection
Author
Masahiro Nishiyama1)3) Kazunori Aoki2) Hiroshi Kurosawa2) Azusa Maruyama1) and Hiroaki Nagase3)
1)Department of Neurology, Hyogo Prefectural Kobe Children's Hospital
2)Department of Pediatric Critical Care Medicine, Hyogo Prefectural Kobe Children's Hospital
3)Department of Pediatrics, Kobe University Graduate School of Medicine
Abstract
In Japan, the number of pediatric intensive care units (PICU) has increased, and a system for the intensive treatment of severe cases has been established, making it possible to save patients who would have been difficult to save in the past. However, many survival children have serious neurological sequelae, and there is an increasing need for pediatric neurocritical care for brain protection. The mechanism of brain injury is a complex and evolutionary process. Hypoxia, cerebral perfusion deprivation, brain edema, inflammation, excitatory amino acid accumulation, oxidative stress, and mitochondrial dysfunction are complicatedly involved, leading to neuronal cell death with the depletion of adenosine triphosphate (ATP). The mechanism of cerebral protection is to provide a stable supply of oxygen to the brain and to suppress the demand for oxygen in the brain to prevent secondary injury. For this purpose, therapeutic strategies to achieve stable cerebral blood flow, suppress intracranial pressure, manage body temperature, and avoid status epilepticus are attempted. While all patients admitted to the PICU are potentially eligible for cerebral protection, the evaluation to identify those at high risk for sequelae and preventable brain injury is important. Early intervention is desirable for brain protection. Treatments prior to the PICU in the emergency room and pre-hospital are also important. Therefore, to achieve discharge without sequelae, the concept of pediatric neurocritical care needs to be widely spread in Japan. Moreover, it is necessary for all medical staff involved in pediatric care to have a brain protection perspective.
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Original Article
Title
Survey for the Establishment of a System of Mental Health Care for Children by General Pediatricians
Author
Yuya Saito Yukiko Osawa and Toshimasa Obonai
Department of Pediatrics, Tokyo Metropolitan Tama-Hokubu Medical Center
Abstract
There is a need to improve the system for children with mental health problems, but the shortage of medical doctors and facilities to care for these children has become a problem. In our clinic, medical care was provided by doctors specializing in mental health (specialists), but since 2020, general pediatricians (non-specialists) have taken the lead in providing medical care. In this study, we examined the relationship between the presence or absence of a specialist and the number of patients, prescriptions of psychotropic drugs, and reimbursement for medical care. Subjects were those who visited an outpatient clinic for mental health problems between April 2011 and March 2023. There were 1,948 patients with mental health problems, of whom 1,258 (64.6%) were treated by specialists and 690 (35.4%) by non-specialists. Comparing 2016, the year with the highest number of patients, with 2021, the year with the lowest number of specialists (odds ratio (OR), 0.04, 95% confidence interval (CI), 0.023-0.068), the number of new patient visits for mental health problems decreased from 226 to 191, but the ratio of new patient visits for mental health problems (OR, 2.07; 95% CI 1.69-2.54), and the proportion of psychotropic drug prescriptions tended to decrease (OR, 0.58; 95% CI 0.34-1.00), but the proportion of pediatric-specific disease counseling fees received remained unchanged (OR, 1.07; 95% CI 0.73-1.57). Establishing a mental health care system among general pediatricians could maintain the number of new patients and reimbursement fees. Assessing and improving the quality of medical care is an issue to be addressed in the future.
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Original Article
Title
Efficacy and Safety of Primary Combined Treatment of Kawasaki Disease with Prednisolone or Oral Cyclosporine
Author
Takeshi Koga Moeko Tanaka Tomoko Uramaru Nozomi Ryuno Satomi Ohtaki Moe Yoshimura Katsuhiko Tabata and Yuko Akioka
Department of Pediatrics, Saitama Medical University Hospital
Abstract
Prednisolone (PSL) and cyclosporine (CsA) have been recommended as the primary combined treatment for potential intravenous immunoglobulin (IVIG) nonresponders. However, to the best of our knowledge, no study has yet compared the efficacy and safety of the treatment methods, and their clinical superiority is unknown. In this study, we retrospectively compared PSL treatment according to the RAISE study (PSL treatment group: 2018-2019) with CsA treatment according to the KAICA trial (CsA treatment group: 2020-2021) to determine their clinical superiority and safety. Moreover, CsA blood concentration kinetics were evaluated for appropriate control. The results of this study revealed no significant differences in time to fever resolution; the CsA treatment group showed a shorter acute treatment period and a lower incidence of adverse events than the PSL treatment group. Conversely, the CsA treatment group had a higher relapse rate (PSL treatment group: 15%, CsA treatment group: 43%). The incidence of coronary artery abnormalities was noninferior. CsA blood concentration kinetics showed a peak at C1 in 9 of 14 patients, while AUC0-4 and C1 were highly correlated (r2 = 0.842). One patient in the CsA treatment group reported headache and nausea on the second day of treatment. C0 was as high as 202 ng/mL on the third day of CsA administration in this patient, therefore, the administration dose was reduced. The findings of this study indicate that CsA treatment shortens the acute treatment period and reduces the incidence of adverse events. Furthermore, appropriate monitoring methods should be considered in relation to the age groups and pathological conditions of patients with Kawasaki disease.
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Case Report
Title
Two Cases of Cerebrovascular Malformation with Subpial Hemorrhage in Infants
Author
Hiroki Nomura Naoki Okamoto Toshiki Takeo Michio Suzuki Tatsuya Fukasawa Tetsuo Hattori Yuichi Kato Yuji Miyajima and Tetsuo Kubota
Department of Pediatrics, Anjo Kosei Hospital
Abstract
Compared to subarachnoid hemorrhage, subdural hematoma and epidural hematoma, subpial hemorrhage is less well known among extraparenchymal hemorrhages. Although subpial hemorrhage has been increasingly reported in neonates in recent years, there are few reports of subpial hemorrhage as a uniform disease entity, and almost no reports in childhood. We report two cases of subpial hemorrhage: a 6-month-old infant with familial cavernous hemangioma and a 1-month-old infant with cerebral arteriovenous malformation. Impermeable subpial hemorrhage may cause cortical damage by compressing the brain parenchyma. We should be aware of the existence of subpial hemorrhage, which is still unknown.
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Case Report
Title
Cytokine Storm-associated Acute Encephalopathy during Remission Induction Therapy for Infant Leukemia
Author
Reina Murasawa1) Miho Ashiarai1) Hiroki Yoshihara1) Naoki Umehara1) Rintaro Ono1) Yosuke Hosoya1) Taiki Nozaki2) Mina Yokoyama1) Miwa Ozawa1) and Daisuke Hasegawa1)
1)Department of Pediatrics, St. Luke's International Hospital
2)Department of Radiology, St. Luke's International Hospital
Abstract
Acute myeloid leukemia (AML) with hyperleukocytosis is associated with a high risk of fatal complications such as leukostasis, tumor lysis syndrome, and disseminated intravascular coagulation (DIC). Although cerebrovascular diseases including intracranial hemorrhage are also known as central nervous system complications of AML with hyperleukocytosis, acute encephalopathy has rarely been reported.
A 2-month-old male infant presented with marked hepatosplenomegaly and white blood cell count of 580,000/μL. He was diagnosed with B-cell/monocyte type mixed-phenotype acute leukemia with KMT2A rearrangement. Low-dose cytarabine was gradually increased and etoposide was added on the third day of treatment. On day 4, massive tumor cell lysis led to the development of DIC accompanied with systemic inflammatory response syndrome. The patient showed prolonged disturbance of consciousness and convulsions. Brain MRI on day 10 showed loss of gray-white matter differentiation and diffuse cerebral edema. The elevation of pro-inflammatory cytokines in the serum and interleukin-18 in the cerebrospinal fluid were observed. Based on the clinical course and these findings, cytokine storm-associated acute encephalopathy was suspected.
Monocytic subtypes of AML have a greater risk of hypercytokinemia. Monocytic leukemia cells accounted for approximately half of the entire cell population in the present case; therefore, the patient might have developed acute encephalopathy due to a cytokine storm associated with tumor lysis. Attention should be paid to cytokine storm-associated complications in the initial treatment of monocytic subtypes of AML with hyperleukocytosis.
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Case Report
Title
A Case of Severe Hypothyroidism Due to Pseudomalabsorption Diagnosed by Levothyroxine Absorption Test
Author
Yuka Imakawa1) Hiroto Idesawa1)2) and Isho Izumi1)
1)Department of Pediatrics, Ibaraki Children's Hospital
2)Department of Endocrinology and Metabolism, Tsukuba University Hospital Mito Clinical Education and Training Center, Mito Kyodo General Hospital
Abstract
In hypothyroid cases, pseudomalabsorption may be indistinguishable from true malabsorption when adequate daily doses of levothyroxine are administered. We report a case of a 13-year-old girl with severe hypothyroidism, which developed after total thyroidectomy. The patient was diagnosed with pseudomalabsorption using the levothyroxine absorption test.
She was diagnosed with Basedow's disease at the age of 12 y 10 m. She was treated with 15 mg thiamazole, but her blood test findings, thyroid-stimulating hormone (TSH) <0.010 μIU/mL and free levothyroxine (FT4) 2.5 ng/dL, did not improve. We added potassium iodide to the treatment regimen and gradually increased the dose of thiamazole up to 60 mg/day. However, thyrotoxicosis continued for four months, and total thyroidectomy was performed at the age of 13 y 3 m. After thyroidectomy, she developed hypothyroidism despite taking 100 μg/day of levothyroxine, which was gradually increased to 300 μg/day, but her blood test findings worsened to 21.410 μIU/mL and <0.40 ng/dL TSH and FT4 levels, respectively. We suspected malabsorption and therefore hospitalized the patient. She underwent the levothyroxine absorption test, which resulted in a favorable increase in FT4 levels; hence, she was diagnosed with pseudomalabsorption. We explained the test results to the patient and her mother. Since then, she has been taking 100 μg/day of levothyroxine and has been doing well.
We recommend the levothyroxine absorption test for patients with severe hypothyroidism whose conditions do not improve, even with adequate doses of levothyroxine, to identify true malabsorption before changing to suppositories or injectable preparations.
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Case Report
Title
A Case of Toxic Shock Syndrome Complicating with Coronary Artery Dilatation
Author
Takahiro Matsuoka Hitoshi Irabu Shuya Kaneko Asami Shimbo Masaki Shimizu and Tomohiro Morio
Department of Pediatrics and Development Biology, Graduate School of Medical and Dental Sciences, Tokyo Medical and Dental University
Abstract
Coronary dilation is a typical complication associated with Kawasaki disease. However, many acute febrile illnesses, such as rheumatic diseases or viral infections, also present with coronary artery lesions. Here, we report a case of a 13-year-old boy with toxic shock syndrome (TSS) complicated with coronary artery dilation. He was referred to us due to symptoms of fever, posterior neck pain, diarrhea, impaired consciousness, and hypotension. Laboratory findings in the peripheral blood showed severe inflammation and renal dysfunction. The echocardiography results indicated a dilated right coronary artery, whereas cardiac function was normal. An enhanced computed tomography scan of the neck showed posterior cervical lymphadenitis, which had to be differentiated from the Kawasaki disease shock syndrome; however, TSS was strongly suspected based on the skewed T cell receptor repertoire of Vbeta2 within the CD4 (+) T lymphocytes. Treatment, including intravenous immunoglobulin, clindamycin, and cefazolin, resolved the fever and improved the coronary artery dilation. Staphylococcus aureus non-producing TSS toxin-1 was identified in the skin culture of the neck, although other culture tests, including those of the blood, urine, and stool, were negative. Finally, desquamation in his hands and feet and a skin perforation of the abscess from the cervical lymphadenitis were observed; therefore, we made a definitive diagnosis of TSS. In children, acute febrile illnesses complicating with coronary artery lesions include various diseases, often having similar clinical symptoms. Although existing coronary artery dilation with fever often indicates Kawasaki disease, it is essential to make a proper diagnosis according to the pathophysiology of each case.
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