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THE JOURNAL OF THE JAPAN PEDIATRIC SOCIETY

Vol.128, No.3, March 2024


Original Article
1. A Single-center, Retrospective Study of Intracranial Abscesses in a Pediatric Tertiary Care Hospital
2. The Effectiveness of Spot Vision Screener in Ophthalmological Examinations for Health Checkups for 3-year-old Children
Case Report
1. A Case of a Double-chambered Right Ventricle with Systemic Circulatory Collapse due to Excessive Muscle Hypertrophy in the Right Ventricle and Spontaneous Closure of the Ventricular Septal Defect
2. Severe Perinatal Hypophosphatasia Over 9 Years of Enzyme Replacement Therapy
3. Crohn's Disease Associated with Systemic Lupus Erythematosus and Hypoalbuminemia
Brief Report
Detection of Dexamethasone in a Medicinal Tea Implying Effectiveness against Pollen Allergies


Original Article
Title
A Single-center, Retrospective Study of Intracranial Abscesses in a Pediatric Tertiary Care Hospital
Author
Ryoichi Yamaki1) Takuma Ohnishi1)2) Yoji Uejima1) Satoshi Sato1) Jun Kurihara3) Takashi Araki4) and Eisuke Suganuma1)
1)Division of Infectious Diseases, Immunology and Allergy, Saitama Children's Medical Center
2)Department of Pediatrics, Keio University School of Medicine
3)Department of Neurosurgery, Saitama Children's Medical Center
4)Department of Traumatology, Saitama Children's Medical Center
Abstract
Purpose: We conducted a retrospective study at our hospital to understand the clinical characteristics of patients with intracranial abscess.
Methods: Patients diagnosed with intracranial abscess at a tertiary children's hospital between January 1, 2013 and December 31, 2022 were included in this study. Patient background, clinical symptoms, various examination findings, and their treatment and progress was reviewed.
Results: Twelve patients were selected, and the median age of the patients was 10.5 years, with no differences in sex. Sinusitis was the most common causative disease in 5 cases. There were 3 cases of congenital heart disease, hematologic tumor, dental infection, and 1 case of meningitis. The median time from symptom onset to diagnosis was 5 days. Seven patients were treated surgically and 5 were treated medically. Causative organisms were identified in 5 cases. The median duration of antimicrobial therapy was 7.5 weeks. The prognosis was 1 death, and 4 cases of neurological sequelae: symptomatic epilepsy, higher functional impairment, and hemiplegia, respectively.
Conclusion: Intracranial abscess is a severe infectious disease that occurs in children with congenital heart disease or hematologic tumor, but it can also occur in healthy children due to sinusitis or dental caries. When symptoms such as fever and headache are prolonged in children with underlying diseases such as those described above, imaging evaluation at the appropriate time is important.




Original Article
Title
The Effectiveness of Spot Vision Screener in Ophthalmological Examinations for Health Checkups for 3-year-old Children
Author
Shiori Hamada1) Akiyoshi Kamada1) Keiko Kamimura2) Yumiko Sato2) Eiko Kojima2) Satoshi Kawaguchi3) Katsumi Azuma4) Yasuhiro Ogawa1) Satoru Shida1) Motoi Nishi5) and Naofumi Kajii2)
1)Department of Pediatric, Ebetsu City Hospital
2)Health Promotion Section and Health Center, Ebetsu City Office
3)Kawaguchi Clinic
4)Azuma Children and Family Clinic
5)Department of Biological Fundamental Science, Health Sciences University of Hokkaido
Abstract
Introduction: Ophthalmological examinations in health checkups for 3-year-old children is important for early detection and treatment of amblyopia. Most municipalities check children's vision using vision tests at home and medical examinations performed by pediatricians. However, it is hard to judge their vision troubles with this method due to difficulties in accurately testing at home or correctly conducting examinations by pediatricians who are not specialists in ophthalmology. In addition, in this method, it is impossible to examine refractometry which is important in diagnosing amblyopia. To resolve these problems, we introduced Spot Vision Screener (SVS) in health checkups for 3-year-old children.
Method: This study enrolled children in Ebetsu city who underwent health checkups for 3-year-olds between April 1, 2018 and March 31, 2019 as the control group, and who underwent the checkups between June 1, 2019 and March 31, 2020 as the SVS group. The control group answered a medical questionnaire, and underwent vision tests, and medical examinations. The SVS group also underwent the same exams and the SVS exam. Those who could not pass the exams were classified as being in the suspected group and referred to ophthalmologists.
Result: Those who were classified to be in the suspected group were 24/800 in the control group (3.0%), and 75/608 in the SVS group (12.3%). Those who required treatment were 6 in the control group and 22 in the SVS group.
Conclusion: In this study, the SVS test increased both the number of patients in the suspected group and the treatment-required group. SVS is useful for health checkups for 3-year-old children.




Case Report
Title
A Case of a Double-chambered Right Ventricle with Systemic Circulatory Collapse due to Excessive Muscle Hypertrophy in the Right Ventricle and Spontaneous Closure of the Ventricular Septal Defect
Author
Rie Ogino1) Naomi Nakagawa1) Yosuke Fukushima1) Kengo Okamoto1) Hitoshi Uemasu1) Koichi Kataoka1) Masahiro Kamada1) and Nakao Konishi2)
1)Department of Pediatric Cardiology, Hiroshima City Hospital
2)Department of Pediatrics, Chugoku Rosai Hospital
Abstract
A 7-year-old boy was diagnosed with a ventricular septal defect (VSD) at birth, but he dropped out of regular check-ups during infancy because his family believed that the VSD would close spontaneously. Heart murmur and hypertrophy of the right ventricle were detected during a school cardiac screening, and he presented with pre-shock symptoms at his first visit. On echocardiography, the right ventricular outflow pressure gradient increased to 210 mmHg, which exceeded the left ventricular pressure at 117/64 mmHg. He was diagnosed with double-chambered right ventricle (DCRV) without shunting through the VSD, and was hospitalized for immediate surgery to avoid sudden death. He required continuous sedation for recurrent pre-shock symptoms. Urgent intracardiac repair involving the resection of the right ventricular outflow tract muscle bundle was performed on the fifth day of hospitalization, and he was discharged 11 days after the surgery. The postoperative course was good, and he has been able to live a normal school life without any limitations.
DCRV is known as a concomitant of the VSD. Patients may show cyanosis when the right ventricular pressure exceeds the left ventricular pressure due to excessive muscle hypertrophy in the right ventricle. Conversely, patients without significant shunting through VSDs may experience life-threatening low systemic circulation, leading to acute systemic circulatory collapse. Therefore, the presence of DCRV should be expected when hypertrophy of the right ventricle and/or systolic ejection murmur is present and pediatricians should focus on low cardiac output syndrome in patients with or without small shunting through the VSD.




Case Report
Title
Severe Perinatal Hypophosphatasia Over 9 Years of Enzyme Replacement Therapy
Author
Hideomi Asanuma1) Akira Ishii2) Masato Saito3) and Yoshihito Kurashige3)
1)Department of Neonatology, Hokkaido Medical Center for Child Health and Rehabilitation
2)Department of Pediatrics, Sapporo Medical University School of Medicine
3)Division of Pediatric Dentistry, Department of Oral Growth and Development, School of Dentistry, Health Sciences University of Hokkaido
Abstract
A male infant with severe perinatal hypophosphatasia, manifested by convulsions, started enzyme replacement therapy with asfotase alfa at the age of 3 months and was monitored for 9 years. The patient's physical development markedly improved within 1 year of initiating therapy, and the growth in height and weight was maintained thereafter. Although there were some weaknesses in movements requiring balance, the development of the patient's gross motor skills was only slightly delayed compared with that of similarly aged children, whereas the fine motor skills displayed remarkably weak development. Some deficits were observed in spatial cognition and visual perceptions, with difficulty in organizing information and making inferences from visual stimuli. Intellectual and cognitive development was slower than average, although the extent varied among different indicators. Language development was average with communication remaining at a level that did not hinder daily living. Dental examinations indicated that only two deciduous teeth had fallen out by 4 years of age. Delay of permanent teeth eruption was not observed, and permanent teeth without pulp cavity enlargement or delayed crown formation were observed, suggesting that enzyme replacement therapy was possibly effective. Despite varying catchup in several developmental domains after 9 years of enzyme replacement therapy, it remains a future issue how these weaknesses will evolve during school years or the extent to which the treatment will impact the patient's permanent teeth in particular. Further long-term monitoring of this case and accumulating studies in other patients are necessary.




Case Report
Title
Crohn's Disease Associated with Systemic Lupus Erythematosus and Hypoalbuminemia
Author
Yuka Ishii1) Tomoo Kise1) Masatsugu Uehara1) Shigeru Fukuyama1) and Toshifumi Yodoshi2)
1)Okinawa Prefectural Nanbu Medical Center & Children's Medical Center, Department of Pediatric Nephrology
2)Okinawa Chubu Hospital, Department of Pediatrics
Abstract
Lupus erythematosus (SLE) is an autoimmune chronic inflammatory disease with a wide variety of symptoms. The concurrence of SLE and Crohn's disease is rare, whereby gastrointestinal symptoms may be a trigger for the diagnosis. Here, we describe a case of Crohn's disease in a patient with a 9-year history of SLE, which was diagnosed based on hypoalbuminemia and the absence of gastrointestinal symptoms.
A 20-year-old woman with SLE and lupus nephritis IVG (A) +V had been receiving treatment since she was 11 years old. The patient complained of abdominal pain from October to November of 2010, had a decrease in serum albumin in November, and experienced a weight loss of 8 kg over a 2-month period in December. In this case, proteinuria did not reach the level of Nephrotic syndrome. As a result of a gastrointestinal scintigraphy, RI accumulation was observed from the sigmoid colon to the rectum, leading to the diagnosis of protein-losing gastroenteropathy. In addition to the endoscopic appearance of cobblestones and longitudinal ulcers, the pathology revealed significantly after she was referred to gastroenterologists and was administered Ustekinumab.
Although there is a paucity of reports regarding the concurrence of Crohn's disease and SLE, we should consider gastrointestinal endoscopy to rule out inflammatory bowel disease in patients with SLE who develop hypoalbuminemia and weight loss despite the absence of gastrointestinal symptoms. Furthermore, a potential benefit of Ustekinumab may be found in Crohn's disease which might be associated with SLE.




Brief Report
Title
Detection of Dexamethasone in a Medicinal Tea Implying Effectiveness against Pollen Allergies
Author
Satoshi Takakuwa
Department of Pediatrics, Hyogo Prefectural Nishinomiya Hospital
Abstract
A 13-year-old girl undergoing growth hormone replacement therapy was found to have adrenal insufficiency due to a decrease in serum dehydroepiandrosterone sulfate (DHEA-S) levels. Her adrenal function recovered after she stopped drinking a medicinal tea reputed to be effective against pollen allergies that she had been drinking during the course of her hormone therapy. It was suspected that the medicinal tea was contaminated with glucocorticoid analogues, and analysis of the tea indicated the presence of dexamethasone. If health hazards caused by foods and beverages are suspected, their causes must be investigated to prevent additional harm. In particular, it is necessary to establish a public system and raise awareness so that appropriate measure can be taken promptly when serious health hazards are suspected. One possible solution is to consult with the National Consumer Affairs Center, which conducted the analysis of this medicinal tea.




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