Mariana Tresoldi das N. Romaneli

Fatores associados ao óbito em lactentes após eventos com aparente risco de morte (apparent life-threatening event, ALTE)

OBJECTIVE To detect factors associated with greater risk of death in infants after an apparent life-threatening event (ALTE). METHODS This cross-sectional, retrospective, descriptive and analytic study evaluated infants younger than 12 months who had a sudden event of cyanosis, pallor, hypotonia or apnea and were seen in the emergency department of a tertiary university hospital. Forward stepwise logistic regression (Wald) was used to calculate and adjust odds ratios to evaluate associations. RESULTS Mean age of the 145 patients included in the study was 105 days (median = 65 days). Eleven (7.6%) died, and their mean age was 189 days (median = 218 days). Mean age of survivors was 98 days (median = 62 days) (p = 0.003). Activity before the event, prematurity and number of events were not associated with death. A significant association was found with pallor. Of the 11 infants, 3 had spontaneous resolution of ALTE, whereas 8 patients [27.6%; p < 0.001; OR = 14.3 (95%CI 3.51-58.3)] did not. The associations with respiratory or cardiovascular disease were also significant. In multivariate analysis, immediate spontaneous resolution [p = 0.015; OR = 6.06 (95%CI 1.02-35.94)] and diagnosis of cardiovascular disease [p = 0.047; OR = 164.27 (95%CI 7.34-3.673.78)] remained statistically significant. CONCLUSION Infants who experienced an ALTE had a higher risk of subsequent death when their age was greater than 6 months and the event had a long duration, particularly when ALTE was associated with cardiovascular disease.OBJECTIVE: To detect factors associated with greater risk of death in infants after an apparent life-threatening event (ALTE). METHODS: This cross-sectional, retrospective, descriptive and analytic study evaluated infants younger than 12 months who had a sudden event of cyanosis, pallor, hypotonia or apnea and were seen in the emergency department of a tertiary university hospital. Forward stepwise logistic regression (Wald) was used to calculate and adjust odds ratios to evaluate associations. RESULTS: Mean age of the 145 patients included in the study was 105 days (median = 65 days). Eleven (7.6%) died, and their mean age was 189 days (median = 218 days). Mean age of survivors was 98 days (median = 62 days) (p = 0.003). Activity before the event, prematurity and number of events were not associated with death. A significant association was found with pallor. Of the 11 infants, 3 had spontaneous resolution of ALTE, whereas 8 patients [27.6%; p < 0.001; OR = 14.3 (95%CI 3.51-58.3)] did not. The associations with respiratory or cardiovascular disease were also significant. In multivariate analysis, immediate spontaneous resolution [p = 0.015; OR = 6.06 (95%CI 1.02-35.94)] and diagnosis of cardiovascular disease [p = 0.047; OR = 164.27 (95%CI 7.34-3.673.78)] remained statistically significant. CONCLUSION: Infants who experienced an ALTE had a higher risk of subsequent death when their age was greater than 6 months and the event had a long duration, particularly when ALTE was associated with cardiovascular disease.

Clinical features and outcome of children and adolescents hospitalized with influenza A (H1N1) virus infection compared with flu-like symptoms and negative rapid tests for influenza A (H1N1) admitted in the same period of time.

This report describes the clinical features and outcome of 61 pediatric hospitalized patients with influenza-like infection. Fever, cough and respiratory distress were the most common symptoms of the infection. Fifteen patients presented positive RT-PCR results for influenza A (H1N1). The group with positive results was compared with the negative one. The main significant difference was antibiotic usage and the need of mechanical ventilation in the patients with H1N1-virus infection. Among the 11 patients who required intensive care due to respiratory failure, 3 from the positive group died and none from the negative group.

Recurrent apparent life-threatening event as the first manifestation of congenital myasthenia

OBJECTIVE To alert pediatricians about the importance of a careful investigation on recurrent apparent life-threatening events. Reports of the association of these events with congenital myasthenic syndromes were not found. CASE DESCRIPTION A seven-month-old infant with recurrent apparent life-threatening events was admitted for investigation. During hospital stay, she presented cyanosis and respiratory failure, requiring mechanical ventilation for three days. After clinical improvement, hypotheses of gastroesophageal reflux and pulmonary aspiration were ruled out. The presence of eyelid ptosis, general hypotonia and weak crying led to the suspicion of congenital myasthenia, which was confirmed. Treatment with oral piridostigmine led to neurological and nutritional normalization, without any other apparent life-threatening event during the next three years. COMMENTS The careful etiological investigation of apparent life-threatening events may lead to rare diagnosis that requires specific treatments, such as congenital myasthenia.Objective: To alert pediatricians about the impor- tance of a careful investigation on recurrent apparent life-threatening events. Reports of the association of these events with congenital myasthenic syndromes were not found. Case description: A seven-month-old infant with re- current apparent life-threatening events was admitted for investigation. During hospital stay, she presented cyanosis and respiratory failure, requiring mechanical ventilation for three days. After clinical improvement, hypotheses of gastroesophageal reflux and pulmonary aspiration were ruled out. The presence of eyelid ptosis, general hypotonia and weak crying led to the suspicion of congenital myasthenia, which was confirmed. Treatment with oral piridostigmine led to neurological and nutritional normalization, without any other apparent life-threatening event during the next three years. Comments: The careful etiological investigation of apparent life-threatening events may lead to rare diagno- sis that requires specific treatments, such as congenital myasthenia.

TUBERCULOSE EM PACIENTES PEDIÁTRICOS: COMO TEM SIDO FEITO O DIAGNÓSTICO?

Objetivo: Descrever o quadro clinico, radiologico e o perfil epidemiologico e microbiologico de pacientes diagnosticados com tuberculose em um periodo de 15 anos. Metodos: Estudo retrospectivo de criancas e adolescentes menores de 18 anos diagnosticados com tuberculose no Hospital de Clinicas da Universidade Estadual de Campinas. Tuberculose ativa foi definida pela identificacao de M. tuberculosis em cultura, baciloscopia ou exame histopatologico ou, ainda, casos com quadro clinico e radiologico compativel e com historia de exposicao a tuberculose ou com teste tuberculinico positivo. Foi realizada analise estatistica, com comparacao entre os dados de criancas menores e maiores de 10 anos, ja que estas apresentam doenca com padrao mais proximo do adulto. Resultados: Foram identificados 145 casos, 61,4% em pacientes menores de 10 anos. Os principais sintomas relatados foram tosse (55,9%) e febre (46,9%), sendo que as variaveis febre, tosse, emagrecimento e dor se associaram a idade, estando mais presentes em criancas maiores de 10 anos. O diagnostico foi confirmado (cultura, baciloscopia ou histopatologico) em 67,6% dos casos. Os demais (32,4%) tiveram seu diagnostico realizado com base no quadro clinico, radiologico, epidemiologico e teste tuberculinico. A positividade da cultura, da baciloscopia e do teste tuberculinico foram, respectivamente, 65,8; 35,7 e 72,3%. Historia de contato com adulto tuberculoso foi confirmada em 37,2% dos casos. Conclusoes: O diagnostico de tuberculose em pediatria e complexo, considerando-se que todos os parâmetros usados tem baixa positividade. Os resultados apontam a necessidade de novos metodos diagnosticos e tambem de melhorias nas estrategias de busca de comunicantes.ABSTRACT Objective: To describe clinical, radiological, epidemiological, and microbiological characteristics of pediatric patients with diagnosis of tuberculosis in a period of 15 years. Methods: Retrospective study including children and adolescents younger than 18 years diagnosed with tuberculosis in the Clinical Hospital of the Universidade Estadual de Campinas in São Paulo State, Brazil. Active tuberculosis was defined by the identification of Mycobacterium tuberculosis in culture, microscopy, or histopathological examination. Children with positive clinical history and radiological tests who had been exposed to sick adults or with positive tuberculin skin test were also considered as having active tuberculosis. Statistical analysis compared the data obtained from children younger and older than 10 years of age, since they present a disease pattern more similar to adults. Results: There were 145 identified cases, 61.4% in patients under 10 years of age. The main symptoms reported were coughing (55.9%) and fever (46.9%), and the variables of fever, coughing, weight-loss, and pain were significantly influenced by age, with a higher frequency in older children. Diagnosis was confirmed by culture, microscopy, or histopathology in 67.6% of the cases. The other cases (32.4%) had the diagnosis of tuberculosis based on clinical, radiological, and epidemiological characteristics, as well as tuberculin skin test. The positivity for culture, microscopy, and tuberculin skin test was, respectively, 65.8, 35.7, and 72.3%. History of contact with a sick adult was confirmed in 37.2%, without influence of age. Conclusions: Diagnosis of tuberculosis in children is still a challenge, since all the confirmation tests have low positivity. These results demonstrate the need for new diagnostic methods and improved strategies for searching sick contacts.

Bilateral spontaneous chylothorax after severe vomiting in children

Abstract Objective: To report the case of a child with bilateral chylothorax due to infrequent etiology: thoracic duct injury after severe vomiting. Case description: Girl, 7 years old, with chronic facial swelling started after hyperemesis. During examination, she also presented with bilateral pleural effusion, with chylous fluid obtained during thoracentesis. After extensive clinical, laboratory, and radiological investigation of the chylothorax etiology, it was found to be secondary to thoracic duct injury by the increased intrathoracic pressure caused by the initial manifestation of vomiting, supported by lymphoscintigraphy findings. Comments: Except for the neonatal period, chylothorax is an infrequent finding of pleural effusion in children. There are various causes, including trauma, malignancy, infection, and inflammatory diseases; however, the etiology described in this study is poorly reported in the literature.

Evento com aparente risco de morte recorrente como manifestação inicial de síndrome miastênica congênita

OBJECTIVE To alert pediatricians about the importance of a careful investigation on recurrent apparent life-threatening events. Reports of the association of these events with congenital myasthenic syndromes were not found. CASE DESCRIPTION A seven-month-old infant with recurrent apparent life-threatening events was admitted for investigation. During hospital stay, she presented cyanosis and respiratory failure, requiring mechanical ventilation for three days. After clinical improvement, hypotheses of gastroesophageal reflux and pulmonary aspiration were ruled out. The presence of eyelid ptosis, general hypotonia and weak crying led to the suspicion of congenital myasthenia, which was confirmed. Treatment with oral piridostigmine led to neurological and nutritional normalization, without any other apparent life-threatening event during the next three years. COMMENTS The careful etiological investigation of apparent life-threatening events may lead to rare diagnosis that requires specific treatments, such as congenital myasthenia.Objective: To alert pediatricians about the impor- tance of a careful investigation on recurrent apparent life-threatening events. Reports of the association of these events with congenital myasthenic syndromes were not found. Case description: A seven-month-old infant with re- current apparent life-threatening events was admitted for investigation. During hospital stay, she presented cyanosis and respiratory failure, requiring mechanical ventilation for three days. After clinical improvement, hypotheses of gastroesophageal reflux and pulmonary aspiration were ruled out. The presence of eyelid ptosis, general hypotonia and weak crying led to the suspicion of congenital myasthenia, which was confirmed. Treatment with oral piridostigmine led to neurological and nutritional normalization, without any other apparent life-threatening event during the next three years. Comments: The careful etiological investigation of apparent life-threatening events may lead to rare diagno- sis that requires specific treatments, such as congenital myasthenia.

Evento com aparente risco de morte: uma revisão

OBJECTIVE: To perform a critical review by gathering all the available information about apparent life-threatening events. DATA SOURCES: Bibliographic review of the articles published in Portuguese, English and Spanish from the electronic databases Medline, Lilacs and SciELO, using the key-words: apparent life-threatening events, apparent life-threatening event, infant, apnea, monitoring, and cyanosis. DATA SYNTHESIS: Apparent life-threatening events define sudden events with, a combination of apnea, color change, and marked change in the muscle tone, that have various underlying causes. The real incidence remains unknown, and it affects infants from 11 to 12 weeks of age. There is no association between apparent life-threatening events and sudden infant death syndrome. There are many possible causes for the events, and they must be investigated even in apparently healthy infants, because the presence of a severe underlying disease associated with the event is possible. If the cause of the apparent life-threatening events is found, it must be treated properly. If there is no explainable cause, the event is considered idiopathic and generally has a benign course. CONCLUSIONS: It is necessary to investigate all the infants taken to the pediatric emergency unit after experiencing an apparent life-threatening event, since there is the risk of morbidity caused by an underlying disease or the event itself, as well as subsequent mortality. Consensus guidelines about the investigation in apparently healthy infants who experienced apparent life-threatening events are not available. Most authors recommend that careful observation and hospital monitoring should be performed for at least for 24 hours after the event.

Association between single nucleotide polymorphisms in TLR4, TLR2, TLR9, VDR, NOS2 and CCL5 genes with acute viral bronchiolitis

Abstract Background Acute viral bronchiolitis is the leading cause of hospitalization among infants during the first year of life. Most infants hospitalized for bronchiolitis do not present risk factors and are otherwise healthy. Our objective was to determine the genetic features associated with the risk and a severe course of bronchiolitis. Methods We prospectively evaluated 181 infants with severe bronchiolitis admitted at three hospitals over a 2-year period, who required oxygen therapy. The control group consisted of 536 healthy adults. Patients were evaluated for the presence of comorbidities (premature birth, chronic respiratory disease, and congenital heart disease), underwent nasopharyngeal aspirate testing for virus detection by multiplex-PCR, and SNPs identification in immune response genes. Patient outcomes were assessed. Results We observed association between SNP rs2107538*CCL5 and bronchiolitis caused by respiratory syncytial virus(RSV) and RSV-subtype-A, and between rs1060826*NOS2 and bronchiolitis caused by rhinovirus. SNPs rs4986790*TLR4, rs1898830*TLR2, and rs2228570*VDR were associated with progression to death. SNP rs7656411*TLR2 was associated with length of oxygen use; SNPs rs352162*TLR9, rs187084*TLR9, and rs2280788*CCL5 were associated with requirement for intensive care unit admission; while SNPs rs1927911*TLR4, rs352162*TLR9, and rs2107538*CCL5 were associated with the need for mechanical ventilation. Conclusions Our findings provide some evidence that SNPs in CCL5 and NOS2 are associated with presence of bronchiolitis and SNPs in TLR4, TLR2, TLR9, VDR and CCL5 are associated with severity of bronchiolitis.

Hirschsprung's disease - Postsurgical intestinal dysmotility

Abstract Objective: To describe the case of an infant with Hirschsprungs disease presenting as total colonic aganglionosis, which, after surgical resection of the aganglionic segment persisted with irreversible functional intestinal obstruction; discuss the difficulties in managing this form of congenital aganglionosis and discuss a plausible pathogenetic mechanism for this case. Case description: The diagnosis of Hirschsprungs disease presenting as total colonic aganglionosis was established in a two-month-old infant, after an episode of enterocolitis, hypovolemic shock and severe malnutrition. After colonic resection, the patient did not recover intestinal motor function that would allow enteral feeding. Postoperative examination of remnant ileum showed the presence of ganglionic plexus and a reduced number of interstitial cells of Cajal in the proximal bowel segments. At 12 months, the patient remains dependent on total parenteral nutrition. Comments: Hirschsprungs disease presenting as total colonic aganglionosis has clinical and surgical characteristics that differentiate it from the classic forms, complicating the diagnosis and the clinical and surgical management. The postoperative course may be associated with permanent morbidity due to intestinal dysmotility. The numerical reduction or alteration of neural connections in the interstitial cells of Cajal may represent a possible physiopathological basis for the condition.

Reexpansion pulmonary edema in children

OBJECTIVE To present a case of a patient with clinical and radiological features of reexpansion pulmonary edema, a rare and potentially fatal disease. CASE DESCRIPTION An 11-year-old boy presenting fever, clinical signs and radiological features of large pleural effusion initially treated as a parapneumonic process. Due to clinical deterioration he underwent tube thoracostomy, with evacuation of 3,000 mL of fluid; he shortly presented acute respiratory insufficiency and needed mechanical ventilation. He had an atypical evolution (extubated twice with no satisfactory response). Computerized tomography findings matched those of reexpansion edema. He recovered satisfactorily after intensive care, and pleural tuberculosis was diagnosed afterwards. COMMENTS Despite its rareness in the pediatric population (only five case reports gathered), the knowledge of this pathology and its prevention is very important, due to high mortality rates. It is recommended, among other measures, slow evacuation of the pleural effusion, not removing more than 1,500 mL of fluid at once.ABSTRACT Objective: To present a case of a patient with clinical and radiological features of reexpansion pulmonary edema, a rare and potentially fatal disease. Case description: An 11-year-old boy presenting fe-ver, clinical signs and radiological features of large pleural effusion initially treated as a parapneumonic process. Due to clinical deterioration he underwent tube thoracostomy, with evacuation of 3,000 mL of fluid; he shortly presen-ted acute respiratory insufficiency and needed mechanical ventilation. He had an atypical evolution (extubated twice with no satisfactory response). Computerized tomography findings matched those of reexpansion edema. He recovered satisfactorily after intensive care, and pleural tuberculosis was diagnosed afterwards. Comments: Despite its rareness in the pediatric popula -tion (only five case reports gathered), the knowledge of this pathology and its prevention is very important, due to high mortality rates. It is recommended, among other measures, slow evacuation of the pleural effusion, not removing more than 1,500 mL of fluid at once.