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Compartment syndrome caused by glucose acetate Ringer's solution in children has not been sufficiently reported. We report the cases of two children who developed compartment syndrome of the dorsum of the hand and forearm after receiving only glucose acetate Ringer's solution during hospitalization, with one case requiring a releasing incision. In recent years, glucose acetate Ringer's solution has been frequently used for maintenance infusion. However, it is not always safe and should be used with caution due to the risk of serious side effects caused by infiltration.
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BACKGROUND: Acute asthma exacerbation in children is often caused by respiratory infections. In this study, a coordinated national surveillance system for acute asthma hospitalizations and causative respiratory infections was established. We herein report recent trends in pediatric acute asthma hospitalizations since the COVID-19 pandemic in Japan. METHODS: Thirty-three sentinel hospitals in Japan registered all of their hospitalized pediatric asthma patients and their causal pathogens. The changes in acute asthma hospitalization in children before and after the onset of the COVID-19 pandemic and whether or not COVID-19 caused acute asthma exacerbation were investigated. RESULTS: From fiscal years 2010-2019, the median number of acute asthma hospitalizations per year was 3524 (2462-4570), but in fiscal years 2020, 2021, and 2022, the numbers were 820, 1,001, and 1,026, respectively (the fiscal year in Japan is April to March). This decrease was observed in all age groups with the exception of the 3- to 6-year group. SARS-CoV-2 was evaluated in 2094 patients from fiscal years 2020-2022, but the first positive case was not detected until February 2022. Since then, only 36 of them have been identified with SARS-CoV-2, none of which required mechanical ventilation. Influenza, RS virus, and human metapneumovirus infections also decreased in FY 2020. In contrast, 24% of patients had not been receiving long-term control medications before admission despite the severity of bronchial asthma. CONCLUSION: SARS-CoV-2 was hardly detected in children with acute asthma hospitalization during the COVID-19 pandemic. This result indicated that SARS-CoV-2 did not induce acute asthma exacerbation in children. Rather, infection control measures implemented against the pandemic may have consequently reduced other respiratory virus infections and thus acute asthma hospitalizations during this period. However, the fact that many hospitalized patients have not been receiving appropriate long-term control medications is a major problem that should be addressed.
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BACKGROUND: In Japan, the vaccination rate against the SARS-CoV-2 vaccine for children was low. Therefore, in this study we investigated the factors influencing guardians' decision-making regarding vaccination of their children. METHODS: From November 1, 2022 to March 31, 2023, pediatric clinics, departments, and midwifery clinics in Saitama Prefecture requested guardians of children under the age of 15 to complete an online questionnaire. RESULTS: Responses were obtained from 894 guardians of children aged 6 months to 15 years; 142 had had one of their children vaccinated at least once and 629 had not had any of their children vaccinated. Among guardians who had not had any of their children vaccinated, "the Age of children" was significantly younger (p < 0.001) and "Prevalence" (p < 0.001), "Free vaccination" (p < 0.001), and "Intentions of national and local governments" (p = 0.005) were selected as reasons significantly less frequently in comparison to guardians who had vaccinated their children. "Japanese adverse reactions" (p < 0.001), "Japanese effectiveness" (p < 0.001), "Adverse reactions" (p < 0.001), "History of adverse reactions" (p < 0.001), and "Reputation of friends" (p = 0.006) were selected significantly more frequently by guardians who had not had any of their children vaccinated. CONCLUSIONS: Guardians who had had one of their children vaccinated at least once emphasized the importance of prevalence and free vaccination. On the other hand, guardians who had not had any of their children vaccinated placed particular importance on adverse reactions and the Japanese data on effectiveness. To guide the decision-making of guardians, it is necessary to quickly collect and publish data on adverse reactions and effectiveness, particularly in Japanese individuals, so that citizens can decide whether to vaccinate themselves and their children.
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Vacinas contra COVID-19 , COVID-19 , Vacinação , Criança , Humanos , COVID-19/epidemiologia , COVID-19/prevenção & controle , Vacinas contra COVID-19/administração & dosagem , Japão , SARS-CoV-2 , Vacinação/psicologia , Tomada de DecisõesRESUMO
BACKGROUND/AIM: Dysgeusia, one of the adverse effects of cancer chemotherapy, and anorexia due to taste disorder can significantly impair the quality of life of patients. However, an evaluation method for dysgeusia has not yet been established. The present prospective study aimed to utilize a combination of subjective and objective assessment methods to evaluate dysgeusia in patients with gastrointestinal cancer initiating chemotherapy, to determine chemotherapeutic drugs and regimens causing dysgeusia, and to assess whether dysgeusia was associated with zinc deficiency. PATIENTS AND METHODS: A total of 21 patients with newly diagnosed gastrointestinal cancer were registered between August 2020 to March 2021. The following regimens were also included in the evaluation if the patients did not develop dysgeusia. A total 30 regimens were administered to the patients during the study period. A salt-impregnated test paper (Salsave®) was used as a subjective assessment, and the chemotherapy-induced taste alteration scale was used as an objective assessment. RESULTS: Based on physician interviews, dysgeusia was diagnosed in 8 of 21 patients (38%) treated with 8 of 30 regimens (27%). All regimens that resulted in dysgeusia contained platinum-based drugs. The patients who developed dysgeusia had higher controlling nutritional status scores at the start of chemotherapy compared to those who did not develop dysgeusia. In both subjective and objective assessments, the patients with dysgeusia performed significantly worse than those without dysgeusia. Six of the eight patients who developed dysgeusia were administered Novelzine, which did not improve the taste disorder despite the improvement of serum zinc levels. CONCLUSION: The combined approach using subjective and objective taste assessment methods was useful in assessing chemotherapy-induced dysgeusia. Mechanisms other than hypozincemia should be considered as contributors to taste disorders caused by platinum-based drugs.
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Antineoplásicos , Neoplasias Gastrointestinais , Humanos , Disgeusia/induzido quimicamente , Disgeusia/tratamento farmacológico , Projetos Piloto , Qualidade de Vida , Estudos Prospectivos , Antineoplásicos/efeitos adversos , Neoplasias Gastrointestinais/complicações , Neoplasias Gastrointestinais/tratamento farmacológico , Distúrbios do Paladar/induzido quimicamente , Distúrbios do Paladar/complicações , Distúrbios do Paladar/tratamento farmacológico , ZincoRESUMO
BACKGROUND: Carnitine is essential for transporting long-chain fatty acids into mitochondria and promotes energy metabolism via ß-oxidation of long-chain fatty acids. Although carnitine is also present in the peripheral blood, 98% of total carnitine is stored in muscle tissue. Neuromuscular diseases accompanied by muscle atrophy are likely to lead to secondary carnitine deficiency, owing to the reduced amount of total carnitine stored in the body. CASE PRESENTATION: An 8-y-old Japanese boy with Fukuyama-type congenital muscular dystrophy accompanied by severe psychomotor retardation had been constantly bedridden, suffered from dysphagia, and had been fed through a gastrostomy tube since the age of 1 y. Regular oral carnitine supplementation (5 mg/kg/d of levocarnitine) was initiated at the age of 7 y, which increased serum carnitine value to within the normal range (serum total carnitine concentration, 58.5-60.9 µmol/L; acylcarnitine concentration, 45.8-55.0 µmol/L; free carnitine concentration, 5.9-12.7 µmol/L). He developed a fever, vomiting, and gastrointestinal bleeding at the age of 8 y. He fell into a coma and visited an emergency room 12 h later. Hypoglycemia and hypocarnitinemia (serum total carnitine concentration, 3.7 µmol/L; acylcarnitine concentration, 2.9 µmol/L; free carnitine concentration, 0.8 µmol/L; acyl-to-free carnitine ratio, 3.6) were observed, and he was found to be negative for urinary ketone bodies. CONCLUSIONS: Neuromuscular diseases accompanied by muscle atrophy may lead to acute carnitine deficiency, even if the serum carnitine concentration is within the normal range before onset. During sick days, it may be necessary to modify a patient's treatment, such as increasing both oral supplementation and intravenous administration of carnitine.
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Carnitina , Distrofias Musculares , Masculino , Humanos , Aminoácidos , Ácidos Graxos , Atrofia Muscular , Hemorragia Gastrointestinal , VômitoRESUMO
Background: Few pediatric cases with myositis and abscesses of the paraspinal muscles have been previously reported. Case Description: We herein report on a 3-year-old patient who developed an epidural abscess in a paraspinal muscle abscess, after a complication of infectious impetigo due to atopic dermatitis. The child improved through the administration of an antibacterial drug. The median age of seven patients with a history of paraspinal muscle inflammation and muscle abscess was 12 years old (3-15-years-old), few of which had underlying diseases, with methicillin-sensitive Staphylococcus aureus being the main causative agent. Although the prognosis was well in many cases following the administration of antibacterial agents, there were also cases in which extensive lesions were found and neurological sequelae remained. Conclusion: The current case was the youngest case ever reported of a paraspinal muscle abscess. Although pediatric cases are rare, in the episode of a fever of unknown origin and difficulty walking, paraspinal muscle abscesses should be cited as a differential diagnosis and appropriate early diagnostic imaging and treatment should be performed.
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BACKGROUND: The clinical features of coronavirus disease 2019 (COVID-19) in children have been changing because of the emergence and rapid spread of variants of concern (VOC). The increase in cases infected with VOC has brought concern with persistent symptoms after COVID-19 in children. This survey aimed to analyze the clinical manifestations and persistent symptoms of pediatric COVID-19 cases in Japan. METHODS: We analyzed the clinical manifestations of pediatric COVID-19 cases reported between February 2020 and April 2022 in Japan, using a dedicated database updated voluntarily by the members of the Japan Pediatric Society. Using the same database, we also analyzed persistent symptoms after COVID-19 in children who were diagnosed between February 2020 and November 2021. RESULTS: A total of 5411 and 1697 pediatric COVID-19 cases were included for analyzing clinical manifestations and persistent symptoms, respectively. During the Omicron variant predominant period, the percentage of patients with seizures increased to 13.4% and 7.4% in patient groups 1-4 and 5-11 years of age, respectively, compared with the pre-Delta (1.3%, 0.4%) or Delta period (3.1%, 0.0%). Persistent and present symptoms after 28 days of COVID-19 onset were reported in 55 (3.2%). CONCLUSIONS: Our survey showed that the rate of symptomatic pediatric COVID-19 cases increased gradually, especially during the Omicron variant predominant period, and a certain percentage of pediatric cases had persistent symptoms. Certain percentages of pediatric COVID-19 patients had severe complications or prolonged symptoms. Further studies are needed to follow such patients.
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COVID-19 , Humanos , Criança , Japão , SARS-CoV-2 , Bases de Dados FactuaisRESUMO
Background: Kikuchi's disease with only extracervical lymphadenopathy is rare. Case Presentation. A 15-year-old male has presented with a fever lasting more than 1 week and right axillary lymphadenopathy. An axillary lymph node biopsy revealed coagulation necrosis, nuclear decay products, infiltration of histiocytes, and enlarged lymphocytes; he was diagnosed with Kikuchi's disease. The only four adult patients with Kikuchi's disease presenting without cervical lesions have been previously reported. Conclusion: This is the only pediatric case of Kikuchi's disease presenting without cervical lymphadenopathy. Kikuchi's disease should be included in the differential diagnosis even in cases of extracervical lymphadenopathy alone.
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Hemorrhagic cystitis is a diffuse inflammatory disease of the urinary bladder associated with macrohematuria. Several cases of hemorrhagic cystitis caused by penicillin G have been reported in adults but not children. Here we describe the first pediatric case of clinically-diagnosed penicillin G-induced hemorrhagic cystitis. The patient was a 9-year-old boy with a ventricular septal defect, chromosomal abnormalities, and infective endocarditis caused by Abiotrophia defectiva. After approximately four weeks of penicillin G administration, he had a culture-negative major hemorrhage with a clot. The hematuria resolved one week after penicillin G discontinuation, and a drug lymphocyte stimulation test for penicillin G was positive. In conclusion, penicillin G can also induce hemorrhagic cystitis in children. When large doses of penicillin G are used for long periods in adults or children, the patient should be monitored for hemorrhagic cystitis.
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BACKGROUND: The COVID-19 pandemic has affected the lives of people of all ages. Most reports on pediatric cases suggest that children experience fewer and milder symptoms than do adults. This is the first nationwide study in Japan focusing on pediatric cases reported by pediatricians, including cases with no or mild symptoms. METHODS: We analyzed the epidemiological and clinical characteristics and transmission patterns of 840 pediatric (<16 years old) COVID-19 cases reported between February and December 2020 in Japan, using a dedicated database which was maintained voluntarily by members of the Japan Pediatric Society. RESULTS: Almost half of the patients (47.7%) were asymptomatic, while most of the others presented mild symptoms. At the time of admission or first outpatient clinic visit, 84.0% of the cases were afebrile (<37.5°C). In total, 609 cases (72.5%) were exposed to COVID-19-positive household members. We analyzed the influence of nationwide school closures that were introduced in March 2020 on COVID-19 transmission routes among children in Japan. Transmission within households occurred most frequently, with no significant difference between the periods before and after declaring nationwide school closures (70.9% and 74.5%, respectively). CONCLUSIONS: COVID-19 symptoms in children are less severe than those in adults. School closure appeared to have a limited effect on transmission. Controlling household transmission from adult family members is the most important measure for prevention of COVID-19 among children.
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COVID-19 , Adolescente , Adulto , Criança , Humanos , Japão/epidemiologia , Pandemias , SARS-CoV-2 , Instituições AcadêmicasRESUMO
BACKGROUND: There have been no reports on both anaphylaxis incidence rate in schools, kindergarten, and nurseries, or how teachers have treated these children. This study was a fact-finding survey aimed at determining if appropriate responses to anaphylaxis onset were implemented in Oita Prefecture, Japan. METHODS: The Oita Prefectural Allergy Control Committee administered a questionnaire using Google forms to all public and private schools, public and private kindergartens, certified child-care facilities, and day-care centers in the prefecture. RESULTS: Responses to the questionnaire were obtained from 597 institutions, of which 125 890 children were affiliated with the responding institutions. Forty-eight children developed symptoms for which an adrenaline auto-injector was recommended in an Oita guideline. Among these children, three used the adrenaline auto-injector, three were prescribed the adrenaline auto-injector but were unable to use it, 27 were unable to use it as they were not prescribed an adrenaline auto-injector, and the final 15 responded that they handled their symptoms via another method because none of the above options apply. CONCLUSIONS: Most children who developed symptoms which an adrenaline auto-injector was recommended had no prescription for an adrenaline auto-injector. There is thus a need for appropriate response training to anaphylaxis whether or not an adrenaline auto-injector was prescribed.
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Anafilaxia , Berçários para Lactentes , Anafilaxia/tratamento farmacológico , Anafilaxia/epidemiologia , Escolaridade , Epinefrina/uso terapêutico , Humanos , Lactente , Instituições AcadêmicasRESUMO
Few cases of pediatric psoriasis with short stature, possibly resulting from chronic systemic inflammation, have been reported. We present the case of a child with short stature occurring after the onset of psoriasis wherein treatment with adalimumab resulted in the improvement of not only the psoriasis but also the child's short stature. Pediatric psoriasis associated with short stature may benefit from the early induction of biologic therapy.
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Nanismo , Psoríase , Adalimumab/efeitos adversos , Anticorpos Monoclonais , Terapia Biológica , Criança , Humanos , Psoríase/tratamento farmacológicoRESUMO
BACKGROUND: Poor preoperative nutritional and immunological status are major risk factors for postoperative complications in patients with various malignancies. Lower preoperative prognostic nutrition index (PNI) is associated with higher rates of postoperative complications and poorer prognosis in those patients. The aim of this study was to analyze the predictive value of the PNI for post-hepatectomy complications in patients with hepatocellular carcinoma (HCC), and evaluate its utility in the surgical procedure. METHODS: This retrospective study included 510 patients who underwent open hepatectomies for HCC. The predictive value of the preoperative nutritional and immunological status for postoperative complications was assessed using the PNI. Postoperative complications were defined as grade II or higher per the Clavien-Dindo classification. Postoperative complication rates were compared according to surgical procedure (major hepatectomy vs minor hepatectomy). RESULTS: Patients with postoperative complications had significantly lower PNIs than those without (43.1 ± 5.5 vs 47.0 ± 5.7, P < 0.001). In the multivariate analysis, low preoperative PNI (< 45) was an independent risk factor for postoperative complications after hepatectomy (hazard ratio, 3.85). When patients were classified per their PNI (high vs low) and extent of surgical procedures (major vs minor), there were more complications among patients with low PNI than those with high PNI, regardless of the extent of surgical procedures. Specifically, the group of patients with low PNI who underwent major hepatectomy had significantly higher rates of postoperative complications than the other groups. CONCLUSIONS: Adding the resection range to the PNI is useful for predicting the postoperative morbidities of hepatectomy patients.
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Carcinoma Hepatocelular , Neoplasias Hepáticas , Carcinoma Hepatocelular/cirurgia , Hepatectomia/efeitos adversos , Humanos , Neoplasias Hepáticas/cirurgia , Morbidade , Avaliação Nutricional , Estado Nutricional , Prognóstico , Estudos RetrospectivosAssuntos
Eletroencefalografia/métodos , Fidelidade a Diretrizes/estatística & dados numéricos , Hipnóticos e Sedativos/uso terapêutico , Neurologistas , Padrões de Prática Médica/estatística & dados numéricos , Humanos , Japão , Neurologia/métodos , Neurologia/estatística & dados numéricos , Inquéritos e QuestionáriosRESUMO
BACKGROUND: Because of the overabundance of vaccination information on the internet, in the media, and on social media, providing clear and correct information on immunization is critical for parental decision-making. In 2018, the Japan Pediatric Society created and distributed a Vaccine Information Statement (VIS) to provide appropriate immunization information to caregivers. The objectives of the present study were to evaluate the effect of the VIS on immunization rates, adherence to schedule, and parental understanding of immunization in Japan. METHODS: This cross-sectional study was conducted at 18 centers in 2 prefectures in Japan. Caregivers were assigned to an intervention group, which received the VIS and a questionnaire when their child reached the age of 1 month, and a control group, which received only the questionnaire. Using the self-reported questionnaires, we evaluated vaccination rates and schedule adherence at age 2 months, and parental knowledge, attitudes, and beliefs regarding immunization. Three months later, the questionnaires were returned, and the findings were compared between the 2 groups. RESULTS: We contacted 422 and 428 persons in the intervention and control groups, respectively, and 111/422 (26.3%) and 119/428 (27.8%) returned the surveys. Vaccination rates and adherence rates for the first dose of 4 recommended vaccines did not differ significantly (P > 0.25); however, there were some positive effects on items related to vaccine knowledge (P = 0.03), perceived benefits (P = 0.02), perceived barriers (P < 0.001), and perceived behavioral control (P = 0.01). CONCLUSION: The VIS improved parent comprehension of infant immunization. Future studies should examine if the effects of such an intervention persist and affect vaccine uptake throughout childhood.
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Conhecimentos, Atitudes e Prática em Saúde , Vacinas , Criança , Estudos Transversais , Humanos , Imunização , Lactente , Japão , Pais , Inquéritos e Questionários , VacinaçãoRESUMO
22q13 deletion syndrome is a genetic disorder caused by the deletion or disruption of the segment of the long arm of chromosome 22. The characteristic clinical features of this syndrome include delayed expressive speech, autistic behavior and hypotonia, and clinically severe complications associated with autoimmunity are rarely reported. We herein report a girl with 22q13 deletion syndrome complicated with multiple inflammatory and autoimmune diseases during early childhood. We performed whole-exome sequencing to identify the genes responsible for her autoimmune diseases and identified the de novo variant p.R512W in the phosphatidylinositol-4,5-bisphosphate 3-kinase catalytic subunit delta (PIK3CD) gene. We suspected it to be the disease-causing variant at the conserved residue in PIK(3)C p110δ. Alternatively, haplo-insufficiency of SHANK3 or other genes by 22q13 deletion and the PIK3CD variant might have synergistically contributed to the onset of the distinctive clinical manifestations in this patient.
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Doenças Autoimunes/genética , Transtornos Cromossômicos/genética , Classe I de Fosfatidilinositol 3-Quinases/genética , Doenças Autoimunes/diagnóstico , Deleção Cromossômica , Transtornos Cromossômicos/diagnóstico , Transtornos Cromossômicos/imunologia , Cromossomos Humanos Par 22/genética , Cromossomos Humanos Par 22/imunologia , Feminino , Humanos , Lactente , MutaçãoRESUMO
BACKGROUND: In September 2015, Japan experienced an unusual increase in acute asthma hospitalizations of children that coincided with an enterovirus D68 (EV-D68) epidemic. The objective of this study is to investigate whether EV-D68 had a causal relationship with the spike in asthma hospitalizations. METHODS: A nation-wide retrospective survey of asthma hospitalizations of children was performed for the period from January 2010 through October 2015. The Japanese Society of Pediatric Allergy and Clinical Immunology asked its affiliated hospitals to report monthly numbers of hospitalizations, ICU admissions and mechanical ventilations due to acute asthma exacerbation. The data were retrieved from medical databases using predefined search criteria: diagnosis of asthma or asthmatic bronchitis, admission, and age <20 years. Monthly numbers of EV-D68 detection were also obtained from the Infectious Disease Surveillance Center of Japan. A Granger causality test was used to analyze the association of EV-D68 detections for asthma exacerbation. RESULTS: A total of 157 hospitals reported 87,189 asthma hospitalizations, including 477 ICU admissions and 1193 mechanical ventilations, during the survey period of 5 years and 10 months. The numbers of these events increased drastically in September 2015. The Granger causality test verified the association between EV-D68 and asthma hospitalizations/mechanical ventilations. The most-affected age group was 3-6 years old. CONCLUSIONS: The spike in pediatric asthma hospitalizations in Japan in September 2015 was found to be associated with the EV-D68 epidemic. Respiratory pathogens can cause "epidemics" of asthma exacerbation. Coordinated surveillance of infectious diseases and asthma may be beneficial for prevention and better control of both illnesses.
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Asma/epidemiologia , Enterovirus Humano D , Infecções por Enterovirus/epidemiologia , Epidemias , Hospitalização , Doença Aguda , Adolescente , Adulto , Asma/terapia , Criança , Pré-Escolar , Infecções por Enterovirus/terapia , Feminino , Humanos , Lactente , Recém-Nascido , Japão/epidemiologia , MasculinoAssuntos
Agamaglobulinemia/tratamento farmacológico , Doenças Genéticas Ligadas ao Cromossomo X/tratamento farmacológico , Imunoglobulinas Intravenosas/efeitos adversos , Fatores Imunológicos/efeitos adversos , Nefrite Intersticial/induzido quimicamente , Adolescente , Criança , Evolução Fatal , Humanos , Imunoglobulinas Intravenosas/uso terapêutico , Fatores Imunológicos/uso terapêutico , Masculino , Nefrite Intersticial/diagnósticoRESUMO
BACKGROUND: Following the increase in the number of children with food allergies, support systems are now required for school lunches, but a large-scale factual investigation has not been carried out. OBJECTIVE: We evaluated the features of elimination diet due to food allergy and the support system in kindergartens and schools. METHODS: A prefecture-based questionnaire survey regarding measures for food allergies in school lunches of all kindergartens, public elementary schools, and public junior high schools (631 facilities) was conducted in Oita Prefecture, Japan. RESULTS: The recovery rate of the questionnaire was 99.5%, which included 106,008 students in total. A total of 1,562 children (1.5%) required elimination diets. The rate of children on elimination diets in kindergartens and elementary/junior high schools that required medical certification by a physician was 1.2% (324 among 27,761 children), which was significantly lower than the 1.8% of children (1,227 among 68,576 students) on elimination diets at the request of guardians without the need for medical certification (p < 0.0001). A total of 43.9% of the kindergartens and schools said that they would contact guardians if symptoms were observed after accidental ingestion, while a low 8.1% stated that they provided support to children themselves, including the administration of adrenaline auto-injectors. CONCLUSION: Medical certification reduces the number of children requiring elimination diets, but it has not been adequately implemented. Furthermore, waiting to contact guardians after symptoms are observed may lead to the delayed treatment of anaphylaxis. Cooperation between physicians and teachers is desired to avoid the overdiagnosis and undertreatment of children with food allergies.
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BACKGROUND: Bilateral adrenalectomy is performed in cases with infantile-onset Cushing syndrome due to bilateral adrenal hyperplasia in McCune-Albright syndrome (MAS) because severe Cushing syndrome with heart failure and liver dysfunction can have a lethal outcome. This procedure can completely ameliorate hypercortisolism, although lifetime steroid replacement therapy and steps to prevent adrenal crisis are necessary. Recently, the efficacy of unilateral adrenalectomy has been reported in adult cases of bilateral macronodular adrenal hyperplasia, but there is no consensus regarding the appropriate surgical treatment for bilateral adrenal hyperplasia in MAS. OBJECTIVE: A 6-month-old girl presented with café-au-lait spots, short stature, central obesity, a moon face, and hypertension. Endocrinological tests and imaging studies led to the diagnosis of ACTH-independent Cushing syndrome due to bilateral adrenal hyperplasia induced by MAS. "Three-quarters adrenalectomy", namely right-sided total adrenalectomy and left-sided half adrenalectomy, was carried out. An activating mutation of the GNAS1 gene (p.Arg201Cys) was identified in the adrenal tissues. Since the operation, our patient has been in a state of clinical remission for more than 2 years. CONCLUSION: Our original surgical intervention, three-quarters adrenalectomy, may be a new treatment option for Cushing syndrome associated with MAS.