RESUMO
BACKGROUND: Pediatric procedural sedation (PPS) has been established worldwide as standard practice for several decades. However, there are no comprehensive guidelines or multi-facility databases of PPS in Japan, and the current status of PPS and PPS-related adverse events is unclear. The objectives of this study were to investigate the status of PPS in Japan and clarify the adverse events and risk factors. METHODS: This study was a single-facility, database survey performed at Oita University Hospital from September 2016 to March 2019. Children under 18 years of age who had been kept sedated for medical procedures with intravenous sedatives were enrolled in this study. Adverse events were recorded and defined according to the Quebec Guideline. RESULTS: During the study period, PPS was performed for 1,436 consecutive cases. The majority (94%) of the sedatives used were thiamylal alone or thiamylal combined with ketamine. There were a total of 253 adverse events in 233 cases (16.2%), including oxygen desaturation, airway hypersensitivity, and vomiting. Patients recovered from respiratory-related adverse events immediately with simple intervention. No patient required endotracheal intubation and no severe adverse event occurred. Four risk factors (a higher American Society of Anesthesiologists classification, longer procedure time, non-compliance of nil per os status, and no Pediatric Advanced Life Support certification for sedation personnel) were associated with the occurrence of adverse events. CONCLUSIONS: Adverse events occurred in 16.2% of all PPS cases. Further studies are needed to analyze the serious adverse events and risk factors for PPS in Japan.
Assuntos
Sedação Consciente/efeitos adversos , Hipnóticos e Sedativos/administração & dosagem , Hipnóticos e Sedativos/efeitos adversos , Administração Intravenosa , Adolescente , Criança , Pré-Escolar , Sedação Consciente/métodos , Quimioterapia Combinada , Serviço Hospitalar de Emergência , Feminino , Humanos , Lactente , Injeções Intravenosas , Japão , Ketamina/administração & dosagem , Ketamina/efeitos adversos , Masculino , Estudos Retrospectivos , Fatores de Risco , Tiamilal/administração & dosagem , Tiamilal/efeitos adversos , Vômito/epidemiologia , Vômito/etiologiaAssuntos
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: The typical non-muscle complications of long-surviving X-linked myotubular myopathy (XLMTM) include scoliosis, head deformity, macrocephaly, gastroesophageal reflux disease and peliosis hepatis. Recently, pulmonary blebs and recurrent pneumothorax have also been reported as uncommon complications, whereas no reports on autopsy cases have focused on lung lesions. CASE PRESENTATION: An 8-year-old boy with XLMTM presented recurrent pneumothorax requiring bleb resection and pleurodesis. He subsequently developed multiple pulmonary mass lesions. He died of hemorrhagic shock due to peliosis hepatis. Autopsy showed multiple peliosis-like hematomas in the blebs of the lung. The histopathological examination of the hematomas revealed pooled blood without a pathway to bronchus. No apparent increase in desmin- or α-smooth muscle actin (α-SMA)-positive cells, namely myofibroblasts, was observed around hematomas, suggesting that the mutation in the myotubularin gene was involved in the defective repair process in the liver and lung tissues. CONCLUSION: Recurrent pneumothorax should be considered as a non-muscle complication of XLMTM. Peliosis-like intrapulmonary hematoma may also be a critical complication caused by poor proliferation of myofibroblasts in the tissue repair process.
Assuntos
Hematoma/patologia , Pneumopatias/patologia , Miopatias Congênitas Estruturais/patologia , Pneumotórax/patologia , Autopsia , Criança , Hematoma/diagnóstico , Humanos , Pneumopatias/diagnóstico , Masculino , Miopatias Congênitas Estruturais/diagnóstico , Pneumotórax/diagnóstico , RecidivaRESUMO
To clarify the cause of deaths associated with pandemic (H1N1) 2009 among children in Japan, we retrospectively studied 41 patients <20 years of age who had died of pandemic (H1N1) 2009 through March 31, 2010. Data were collected through interviews with attending physicians and chart reviews. Median age of patients was 59 months; one third had a preexisting condition. Cause of death was categorized as unexpected cardiopulmonary arrest for 15 patients, encephalopathy for 15, and respiratory failure for 6. Preexisting respiratory or neurologic disorders were more frequent in patients with respiratory failure and less frequent in patients with unexpected cardiopulmonary arrest. The leading causes of death among children with pandemic (H1N1) 2009 in Japan were encephalopathy and unexpected cardiopulmonary arrest. Deaths associated with respiratory failure were infrequent and occurred primarily among children with preexisting conditions. Vaccine use and public education are necessary for reducing influenza-associated illness and death.
Assuntos
Vírus da Influenza A Subtipo H1N1 , Influenza Humana/mortalidade , Pandemias , Adolescente , Causas de Morte , Criança , Pré-Escolar , Feminino , Humanos , Lactente , Vírus da Influenza A Subtipo H1N1/isolamento & purificação , Influenza Humana/epidemiologia , Japão/epidemiologia , MasculinoRESUMO
Breast feeding is known to be a major cause of vitamin D deficiency in infants because the content of vitamin D in breast milk is significantly lower than that in formula. We report a case of a 1-mo-old boy who developed hypocalcemic seizures and dilated cardiomyopathy caused by vitamin D deficiency despite being fed a sufficient amount of regular formula. The cause of vitamin D deficiency in this case was maternal vitamin deficiency due to severe hyperemesis and insufficient sunlight exposure, induced mainly by the malabsorption of fat-soluble vitamins caused by maternal cholestasis. We should carefully consider maternal conditions during pregnancy and the postpartum period to detect and prevent vitamin D deficiency in the fetus and infant.
RESUMO
We report a pediatric case of reversible cerebral vasoconstriction syndrome with focal seizures without a thunderclap headache. A 7-year-old girl had a mild acute headache with nausea after swimming. She subsequently developed hemi-convulsions followed by right hemiplegia. Brain magnetic resonance angiography revealed generalized vasoconstriction of the main cerebral peripheral arteries. Her hemiplegia was spontaneously resolved within 6h. Over the next 24h she suffered from recurrent and transient headaches, which recurred on days 3 and 5. Follow-up magnetic resonance angiography on day 3 documented the multifocal narrowing of the main cerebral arteries, which was observed to have diminished at 12weeks after her initial presentation. She did not have any headaches or neurological deficits after day 5. This case indicates that reversible cerebral vasoconstriction syndrome should be considered in children with focal seizures even when they do not present with thunderclap headaches. The timely and appropriate evaluation by magnetic resonance angiography and imaging is essential for diagnosing reversible cerebral vasoconstriction syndrome.
Assuntos
Transtornos Cerebrovasculares/diagnóstico por imagem , Transtornos da Cefaleia Primários/diagnóstico por imagem , Convulsões/diagnóstico por imagem , Angiografia Cerebral , Artérias Cerebrais/diagnóstico por imagem , Transtornos Cerebrovasculares/fisiopatologia , Criança , Diagnóstico Diferencial , Feminino , Seguimentos , Transtornos da Cefaleia Primários/fisiopatologia , Hemiplegia/diagnóstico por imagem , Hemiplegia/fisiopatologia , Humanos , Angiografia por Ressonância Magnética , Convulsões/fisiopatologiaAssuntos
Neoplasias das Glândulas Suprarrenais/genética , Neoplasias Primárias Múltiplas/diagnóstico , Neoplasias Primárias Múltiplas/genética , Neuroblastoma/genética , Cromossomo Filadélfia , Leucemia-Linfoma Linfoblástico de Células Precursoras/diagnóstico , Leucemia-Linfoma Linfoblástico de Células Precursoras/genética , Protocolos de Quimioterapia Combinada Antineoplásica/administração & dosagem , Quimioterapia Adjuvante , Transplante de Células-Tronco de Sangue do Cordão Umbilical/métodos , Progressão da Doença , Evolução Fatal , Feminino , Humanos , Lactente , Estadiamento de Neoplasias , Neuroblastoma/tratamento farmacológico , Neuroblastoma/patologia , Neuroblastoma/cirurgia , Leucemia-Linfoma Linfoblástico de Células Precursoras/terapia , Falha de TratamentoRESUMO
BACKGROUND: Every year, an estimated 200-500 children in Japan develop influenza-associated encephalopathy (IAE), and 10-30% of these children die. OBJECTIVE: To clarify the clinical features of a severe form of acute encephalopathy seen with 2009 pandemic influenza A (H1N1). STUDY DESIGN: This retrospective survey examined 20 children with acute encephalopathy associated with the 2009 pandemic influenza A (H1N1) who died or were in a prolonged deep coma with a flat electroencephalogram tracing and loss of spontaneous respiration. We obtained demographic, clinical, laboratory, and neuroimaging data through interviews with the attending physicians and chart reviews. RESULTS: Subjects were 13 boys and seven girls. Their median age was 45 (range 11-200) months. Five patients had one or more pre-existing conditions. Acute encephalopathy developed within 2 days after influenza onset in 16 patients. As the initial neurological symptom, delirious behavior was seen in six children, and brief seizures in six. Eighteen patients were comatose within 6h of the onset of encephalopathy. Marked brain edema on computed tomography (CT) was seen in all but one patient. Brainstem lesions on CT were recognized in 12 patients. Sixteen patients died 0-45 (median 2.5) days after the onset of acute encephalopathy, and the others remained in deep comas without spontaneous respiration. CONCLUSIONS: The clinical course of the patients was characterized by an onset with mild neurological symptoms and rapid deterioration of consciousness into coma. Head CT revealed marked cerebral edema, often associated with brainstem lesions.