High-resolution computed tomography findings in young infants with cystic fibrosis detected by newborn screening.

OBJECTIVE: High-resolution computed tomography (HRCT) allows the early detection of pathological changes in the lung structure, and reproducible scoring systems can be used to quantify chest computed tomography (CT) findings in patients with cystic fibrosis (CF). The aim of the study was to describe early HRCT findings according to a validated scoring system in infants with CF diagnosed by newborn screening (NBS). METHODS: This cross-sectional study included infants with CF diagnosed by NBS who were born between January 2013 and January 2017 and who underwent HRCT scanning within the first year after diagnosis when they were clinically stable. The CT scans were evaluated using the modified Bhalla score. RESULTS: Thirty-two subjects underwent HRCT scanning. The mean total-modified Bhalla score was 3.6±2.1, and 93.8% of the scans were abnormal. Pseudomonas aeruginosa airway colonization was associated with increased modified Bhalla score values. Bronchial wall thickening was the most common feature (90.6%), followed by bronchial collapse/consolidation (59.4%), mosaic attenuation/perfusion (50%), bronchiectasis (37.5%) and mucus plugging (15.6%). Bronchial wall thickening was diffuse in most of the patients. CONCLUSION: A substantial proportion of infants diagnosed with CF after detection by NBS already showed evidence of lung disease. P. aeruginosa colonization was associated with increased Bhalla scores, highlighting the importance of this CF pathogen in early structural lung disease. The presence of bronchial wall thickening at such a young age may reflect the presence of airway inflammatory processes. The detection and quantification of structural abnormalities with the modified Bhalla score may aid in the identification of lung disease before it is clinically apparent.

High-resolution computed tomography findings in young infants with cystic fibrosis detected by newborn screening

OBJECTIVE: High-resolution computed tomography (HRCT) allows the early detection of pathological changes in the lung structure, and reproducible scoring systems can be used to quantify chest computed tomography (CT) findings in patients with cystic fibrosis (CF). The aim of the study was to describe early HRCT findings according to a validated scoring system in infants with CF diagnosed by newborn screening (NBS). METHODS: This cross-sectional study included infants with CF diagnosed by NBS who were born between January 2013 and January 2017 and who underwent HRCT scanning within the first year after diagnosis when they were clinically stable. The CT scans were evaluated using the modified Bhalla score. RESULTS: Thirty-two subjects underwent HRCT scanning. The mean total-modified Bhalla score was 3.6±2.1, and 93.8% of the scans were abnormal. Pseudomonas aeruginosa airway colonization was associated with increased modified Bhalla score values. Bronchial wall thickening was the most common feature (90.6%), followed by bronchial collapse/consolidation (59.4%), mosaic attenuation/perfusion (50%), bronchiectasis (37.5%) and mucus plugging (15.6%). Bronchial wall thickening was diffuse in most of the patients. CONCLUSION: A substantial proportion of infants diagnosed with CF after detection by NBS already showed evidence of lung disease. P. aeruginosa colonization was associated with increased Bhalla scores, highlighting the importance of this CF pathogen in early structural lung disease. The presence of bronchial wall thickening at such a young age may reflect the presence of airway inflammatory processes. The detection and quantification of structural abnormalities with the modified Bhalla score may aid in the identification of lung disease before it is clinically apparent.

Neurogenic bladder findings in patients with Congenital Zika Syndrome: A novel condition.

INTRODUCTION: Congenital Zika Syndrome (CZS) has been associated with microcephaly and other central nervous system abnormalities including areas that have been implicated in the control of the lower urinary tract. As such, this descriptive case series has aimed to investigate whether CZS is linked with neurogenic bladder. Identifying such an association is paramount in the effort to recognize CZS complications that have putative treatment options that could mitigate the impact of CZS in infected children. METHODS: Following IRB approval, urological assessment was performed in all patients referred to our clinic between June 2016 and May 2017 who presented with confirmed CZS-associated microcephaly. The research protocol consisted of obtaining clinical history, laboratory tests, lower and upper urinary tract ultrasounds, as well as a diagnostic urodynamic evaluation. ZIKA virus infection was previously confirmed by maternal history and positive PCR in babies and mothers. Microcephaly and other central nervous system abnormalities were established based on neurological assessment and associated imaging of the central nervous system (CT head and/or Brain MRI). RESULTS: Twenty-two consecutive CZS patients were tested and confirmed to have neurogenic bladder. Of the 22 patients assessed, 21 presented with an overactive bladder combined with reduced bladder capacity and elevated detrusor filling pressures. Clinically significant increases in postvoid residual (PVR) were confirmed in 40% of cases while a urinary tract infection (UTI) was identified in 23% of cases. CONCLUSION: Neurogenic bladder, a known treatable health condition, was confirmed in 100% of patients tested in this study, most presenting with high-risk urodynamic patterns known to lead to renal damage when left untreated. Follow up studies are necessary to provide further insight onto long-term disease progression and to investigate the response to standard therapies for neurogenic bladder. Nonetheless, we emphasize the importance of proactive management of neurogenic bladder and prompt referral so as to help mitigate CZS disease burden for patients and their families.

Methicillin-resistant Staphylococcus aureus in cystic fibrosis patients: do we need to care? A cohort study

ABSTRACT CONTEXT AND OBJECTIVE: The prevalence of a variety of potentially pathogenic microorganisms in cystic fibrosis patients, such as methicillin-resistant Staphylococcus aureus (MRSA), has increased over the past decade. Given the increasing prevalence of MRSA and the few data available in the literature, better understanding of the clinical repercussions of colonization by this bacterium in cystic fibrosis patients becomes essential. This study aimed to evaluate the repercussions of chronic colonization by MRSA in cystic fibrosis patients. DESIGN AND SETTING: Retrospective cohort study from January 2004 to December 2013 in a cystic fibrosis reference center. METHODS: Each patient with cystic fibrosis was evaluated for nutritional status (body mass index, BMI, and BMI percentile), pulmonary function and tomographic abnormalities (modified Bhalla scores) at the time of chronic colonization by MRSA or methicillin-susceptible Staphylococcus aureus (MSSA) and throughout the study period. RESULTS: Twenty pairs of patients were included. There were no significant differences between the groups regarding nutritional characteristics. Spirometric data showed a trend towards greater obstruction of the airways in patients with MRSA. Patients with MRSA presented greater structural damage to their lungs, demonstrated not only by the total Bhalla score but also by its parameters individually. CONCLUSIONS: Patients colonized by MRSA presented greater functional and structural respiratory impairment at the time of chronic colonization. Disease progression was also faster in patients chronically colonized by MRSA than in those with MSSA. This was shown through comparisons that avoided possible confounding variables.

Methicillin-resistant Staphylococcus aureus in cystic fibrosis patients: do we need to care? A cohort study.

CONTEXT AND OBJECTIVE: The prevalence of a variety of potentially pathogenic microorganisms in cystic fibrosis patients, such as methicillin-resistant Staphylococcus aureus (MRSA), has increased over the past decade. Given the increasing prevalence of MRSA and the few data available in the literature, better understanding of the clinical repercussions of colonization by this bacterium in cystic fibrosis patients becomes essential. This study aimed to evaluate the repercussions of chronic colonization by MRSA in cystic fibrosis patients. DESIGN AND SETTING: Retrospective cohort study from January 2004 to December 2013 in a cystic fibrosis reference center. METHODS: Each patient with cystic fibrosis was evaluated for nutritional status (body mass index, BMI, and BMI percentile), pulmonary function and tomographic abnormalities (modified Bhalla scores) at the time of chronic colonization by MRSA or methicillin-susceptible Staphylococcus aureus (MSSA) and throughout the study period. RESULTS: Twenty pairs of patients were included. There were no significant differences between the groups regarding nutritional characteristics. Spirometric data showed a trend towards greater obstruction of the airways in patients with MRSA. Patients with MRSA presented greater structural damage to their lungs, demonstrated not only by the total Bhalla score but also by its parameters individually. CONCLUSIONS: Patients colonized by MRSA presented greater functional and structural respiratory impairment at the time of chronic colonization. Disease progression was also faster in patients chronically colonized by MRSA than in those with MSSA. This was shown through comparisons that avoided possible confounding variables.

Mastopatia linfocítica simulando neoplasia de mama: relato de caso / Lymphocytic mastopathy mimicking breast malignancy: a case report

A mastopatia linfocítica acomete mulheres jovens e de meia idade e está frequentemente associada a doenças autoimunes. Seu diagnóstico é feito associando achados clínicos (espessamento ou nódulo mamário endurecido), radiológicos (aumento da densidade mamária, nódulo e calcificações), ultrassonográficos (nódulo com sombra acústica posterior), histopatológicos (fibrose e infiltrado linfocítico) e imuno-histoquímicos. É uma entidade benigna que pode simular carcinoma. Neste artigo, relata-se um caso de paciente com mastopatia linfocítica.

Lymphocytic mastopathy affects both young and middle-aged women and is frequently associated with autoimmune diseases. Diagnosis is done by associating clinical (breast tissue thickening or hardened breast lump), radiological (increased breast density, presence of mass and calcifications), sonographic (nodule with posterior acoustic shadowing), histopathological (fibrosis and lymphocytic infiltrate) and immunohistochemical findings. Lymphocytic mastopathy is a benign entity that may mimic carcinoma. The authors report the case of a patient with lymphocytic mastopathy.

Lymphocytic mastopathy mimicking breast malignancy: a case report.

Lymphocytic mastopathy affects both young and middle-aged women and is frequently associated with autoimmune diseases. Diagnosis is done by associating clinical (breast tissue thickening or hardened breast lump), radiological (increased breast density, presence of mass and calcifications), sonographic (nodule with posterior acoustic shadowing), histopathological (fibrosis and lymphocytic infiltrate) and immunohistochemical findings. Lymphocytic mastopathy is a benign entity that may mimic carcinoma. The authors report the case of a patient with lymphocytic mastopathy.

A mastopatia linfocítica acomete mulheres jovens e de meia idade e está frequentemente associada a doenças autoimunes. Seu diagnóstico é feito associando achados clínicos (espessamento ou nódulo mamário endurecido), radiológicos (aumento da densidade mamária, nódulo e calcificações), ultrassonográficos (nódulo com sombra acústica posterior), histopatológicos (fibrose e infiltrado linfocítico) e imuno-histoquímicos. É uma entidade benigna que pode simular carcinoma. Neste artigo, relata-se um caso de paciente com mastopatia linfocítica.

High-resolution computed tomography scores in cystic fibrosis patients colonized with Pseudomonas aeruginosa or Staphylococcus aureus.

OBJECTIVE: To compare HRCT findings in cystic fibrosis (CF) patients chronically colonized with Pseudomonas aeruginosa or Staphylococcus aureus using the modified Bhalla CT scoring system, as well as to evaluate intraobserver and interobserver reliability of the method. METHODS: This was a retrospective cross-sectional study involving 41 CF patients, 26 of whom were chronically colonized with P. aeruginosa (Pa group), and 15 of whom were colonized with S. aureus (Sa group).Two independent radiologists evaluated the HRCT scans of these patients using the modified Bhalla CT scoring system in two different moments. Intraobserver and interobserver reliability was calculated using the intraclass correlation coefficient (ICC). RESULTS: There was good intraobserver and interobserver agreement (ICC > 0.8). Scores were higher in the Pa group than in the Sa group for observer 1 (mean, 13.50 ± 3.90; median, 13.5 vs. mean, 5.00 ± 5.28; median, 3.0) and for observer 2 (mean, 11.96 ± 5.07; median, 12.0 vs. mean, 5.07 ± 5.65; median, 5.0). In addition, HRCT findings, such as bronchiectasis, bronchial wall thickening, mucus plugging, generation of bronchial divisions, and mosaic attenuation/perfusion pattern, were more prevalent in the Pa group. CONCLUSIONS: The modified Bhalla CT scoring system was reproducible and reliable for use in the evaluation of HRCT scans, allowing distinctions to be drawn between the two groups of patients under study. The higher scores in the Pa group provided evidence of greater pulmonary impairment in that group.

Escore tomográfico em pacientes com fibrose cística colonizados por Pseudomonas aeruginosa ou Staphylococcus aureus / High-resolution computed tomography scores in cystic fibrosis patients colonized with Pseudomonas aeruginosa or Staphylococcus aureus

OBJETIVO: Comparar achados de TCAR em pacientes com fibrose cística (FC) colonizados cronicamente por Pseudomonas aeruginosa ou Staphylococcus aureus, empregando o escore de Bhalla modificado, e avaliar as confiabilidades intraobservador e interobservador do método. MÉTODOS: Estudo transversal retrospectivo incluindo 41 pacientes portadores de FC, 26 dos quais colonizados cronicamente por P. aeruginosa (grupo Pa) e 15 por S. aureus (grupo Sa). Dois radiologistas analisaram independentemente em duas ocasiões, as imagens de TCAR desses pacientes e aplicaram o escore de Bhalla modificado. As confiabilidades intra e interobservador foram avaliadas segundo o coeficiente de correlação intraclasse (CCI). RESULTADOS: Houve boa concordância intraobservador e interobservador (CCI > 0,8). Os resultados dos escores do grupo Pa foram mais elevados que os do grupo Sa para o observador 1 (média de 13,50 ± 3,90 e mediana de 13,5 vs. média de 5,0 ± 5,28 e mediana de 3,0) e para o observador 2 (média de 11,96 ± 5,07 e mediana de 12,0 vs. média de 5,07 ± 5,65 e mediana de 5,0). Alterações tomográficas, como bronquiectasias, espessamento das paredes brônquicas, formação de tampões mucosos, comprometimento de gerações de divisões brônquicas e padrão de atenuação em mosaico, foram mais prevalentes no grupo colonizado por P. aeruginosa. CONCLUSÕES: O escore de Bhalla modificado se mostrou reprodutível e confiável para a avaliação de TCAR e permitiu a diferenciação entre os pacientes incluídos nos dois grupos. Escores mais altos no grupo Pa evidenciaram maior comprometimento estrutural pulmonar nesse grupo.

OBJECTIVE: To compare HRCT findings in cystic fibrosis (CF) patients chronically colonized with Pseudomonas aeruginosa or Staphylococcus aureus using the modified Bhalla CT scoring system, as well as to evaluate intraobserver and interobserver reliability of the method. METHODS: This was a retrospective cross-sectional study involving 41 CF patients, 26 of whom were chronically colonized with P. aeruginosa (Pa group), and 15 of whom were colonized with S. aureus (Sa group).Two independent radiologists evaluated the HRCT scans of these patients using the modified Bhalla CT scoring system in two different moments. Intraobserver and interobserver reliability was calculated using the intraclass correlation coefficient (ICC). RESULTS: There was good intraobserver and interobserver agreement (ICC > 0.8). Scores were higher in the Pa group than in the Sa group for observer 1 (mean, 13.50 ± 3.90; median, 13.5 vs. mean, 5.00 ± 5.28; median, 3.0) and for observer 2 (mean, 11.96 ± 5.07; median, 12.0 vs. mean, 5.07 ± 5.65; median, 5.0). In addition, HRCT findings, such as bronchiectasis, bronchial wall thickening, mucus plugging, generation of bronchial divisions, and mosaic attenuation/perfusion pattern, were more prevalent in the Pa group. CONCLUSIONS: The modified Bhalla CT scoring system was reproducible and reliable for use in the evaluation of HRCT scans, allowing distinctions to be drawn between the two groups of patients under study. The higher scores in the Pa group provided evidence of greater pulmonary impairment in that group.

A computed tomography scoring system to assess pulmonary disease among premature infants / Um sistema de escore por tomografia computadorizada para avaliação da doença pulmonar em lactentes prematuros

CONTEXT AND OBJECTIVE: High-resolution computed tomography (HRCT) is considered to be the best method for detailed pulmonary evaluation. The aim here was to describe a scoring system based on abnormalities identified on HRCT among premature infants, and measure the predictive validity of the score in relation to respiratory morbidity during the first year of life. DESIGN AND SETTING: Prospective cohort study in Instituto Fernandes Figueira, Fundação Oswaldo Cruz. METHODS: Scoring system based on HRCT abnormalities among premature newborns. The affected lung area was quantified according to the number of compromised lobes, in addition to bilateral pulmonary involvement. Two radiologists applied the score to 86 HRCT scans. Intraobserver and interobserver agreement were analyzed. The score properties were calculated in relation to predictions of respiratory morbidity during the first year of life. RESULTS: Most of the patients (85 percent) presented abnormalities on HRCT, and among these, 56.2 percent presented respiratory morbidity during the first year of life. Scores ranged from zero to 12. There was good agreement between observers (intraclass correlation coefficient, ICC = 0.86, confidence interval, CI: 0.64-0.83). The predictive scores were as follows: positive predictive value 81.8 percent, negative predictive value 56.3 percent, sensitivity 39.1 percent, and specificity 90.0 percent. CONCLUSION: The scoring system is reproducible, easy to apply and allows HRCT comparisons among premature infants, by identifying patients with greater likelihood of respiratory morbidity during the first year of life. Its use will enable HRCT comparisons among premature infants with different risk factors for respiratory morbidity.

CONTEXTO E OBJETIVO: Tomografia computadorizada de alta resolução (TCAR) é considerada o melhor método para avaliação pulmonar detalhada. O objetivo foi descrever um sistema de escore baseado em alterações identificadas nas TCAR de lactentes prematuros e medir a validade preditiva do escore em relação à morbidade respiratória no primeiro ano de vida. TIPO DE ESTUDO E LOCAL: Estudo de coorte prospectiva no Instituto Fernandes Figueira, Fundação Oswaldo Cruz. MÉTODOS: Sistema de escore baseado em alterações nas TCAR de lactentes prematuros. A área pulmonar alterada foi quantificada conforme o número de lobos alterados, acrescido do comprometimento pulmonar bilateral. Dois radiologistas aplicaram o escore em 86 TCAR. Foram analisadas as confiabilidades intraobservador e interobservador e calculadas as propriedades do escore em relação à predição da morbidade respiratória no primeiro ano de vida. RESULTADOS: A maioria (85 por cento) dos pacientes apresentou TCAR anormal, e dentre estes, 56,2 por cento apresentaram morbidade respiratória no primeiro ano de vida. Valores do escore variaram de zero a 12. Houve boa concordância entre os observadores (coeficiente de correlação intraclasse, CCI = 0,86, intervalo de confiança, IC: 0,64-0,83). Os valores preditivos do escore foram: valor preditivo positivo 81,8 por cento, valor preditivo negativo 56,3 por cento, sensibilidade 39,1 por cento e especificidade 90,0 por cento. CONCLUSÃO: O sistema de escore é reprodutível, de fácil aplicação e permite a comparação de TCAR de pacientes prematuros, identificando pacientes com maior probabilidade de morbidade respiratória no primeiro de vida. Seu uso permitirá a comparação de TC de lactentes prematuros com diferentes fatores de risco para morbidade respiratória.

Intra and interobserver reliability of the interpretation of high-resolution computed tomography on the lungs of premature infants.

CONTEXT AND OBJECTIVE: High-resolution computed tomography (HRCT) of the lungs is more sensitive than radiographs for evaluating pulmonary disease, but little has been described about HRCT interpretation during the neonatal period or shortly afterwards. The aim here was to evaluate the reliability of the interpretation of HRCT among very low birth weight premature infants (VLBWPI; < 1500 g). DESIGN AND SETTING: Cross-sectional study on intra and interobserver reliability of HRCT on VLBWPI. METHODS: 86 VLBWPI underwent HRCT. Two pediatric radiologists analyzed the HRCT images. The reliability was measured by the proportion of agreement, kappa coefficient (KC) and positive and negative agreement indices. RESULTS: For radiologist A, the intraobserver reliability KC was 0.79 (confidence interval, CI: 0.54-1.00) for normal/abnormal examinations; for each abnormality on CT, KC ranged from 0.05 to 1.00. For radiologist B, the intraobserver reliability KC was 0.79 (CI: 0.54-1.00) for normal/abnormal examinations; for each abnormality on CT, KC ranged from 0.37 to 0.83. The interobserver agreement was 88% for normal/abnormal examinations and KC was 0.71 (CI: 0.5- 0.93); for most abnormal findings, KC ranged from 0.51-0.67. CONCLUSION: For normal/abnormal examinations, the intra and interobserver agreements were substantial. For most of the imaging findings, the intraobserver agreement ranged from moderate to substantial. Our data demonstrate that in clinical practice, there is no reason for more than one tomographic image evaluator, provided that this person is well trained in VLBWPI HRCT interpretation. Analysis by different observers should be reserved for research and for difficult cases in clinical contexts.

Intra and interobserver reliability of the interpretation of high-resolution computed tomography on the lungs of premature infants / Confiabilidades intra e interobservador na interpretação da tomografia computadorizada de alta resolução do pulmão de lactentes prematuros

CONTEXT AND OBJECTIVE: High-resolution computed tomography (HRCT) of the lungs is more sensitive than radiographs for evaluating pulmonary disease, but little has been described about HRCT interpretation during the neonatal period or shortly afterwards. The aim here was to evaluate the reliability of the interpretation of HRCT among very low birth weight premature infants (VLBWPI; < 1500 g). DESIGN AND SETTING: Cross-sectional study on intra and interobserver reliability of HRCT on VLBWPI. METHODS: 86 VLBWPI underwent HRCT. Two pediatric radiologists analyzed the HRCT images. The reliability was measured by the proportion of agreement, kappa coefficient (KC) and positive and negative agreement indices. RESULTS: For radiologist A, the intraobserver reliability KC was 0.79 (confidence interval, CI: 0.54-1.00) for normal/abnormal examinations; for each abnormality on CT, KC ranged from 0.05 to 1.00. For radiologist B, the intraobserver reliability KC was 0.79 (CI: 0.54-1.00) for normal/abnormal examinations; for each abnormality on CT, KC ranged from 0.37 to 0.83. The interobserver agreement was 88 percent for normal/abnormal examinations and KC was 0.71 (CI: 0.5- 0.93); for most abnormal findings, KC ranged from 0.51-0.67. CONCLUSION: For normal/abnormal examinations, the intra and interobserver agreements were substantial. For most of the imaging findings, the intraobserver agreement ranged from moderate to substantial. Our data demonstrate that in clinical practice, there is no reason for more than one tomographic image evaluator, provided that this person is well trained in VLBWPI HRCT interpretation. Analysis by different observers should be reserved for research and for difficult cases in clinical contexts.

CONTEXTO E OBJETIVO: Tomografia computadorizada de alta resolução dos pulmões (TCAR) é mais sensível que radiografias para avaliar doença pulmonar, entretanto, pouco tem sido descrito sobre a interpretação da TCAR no período neonatal ou imediatamente após. O objetivo foi avaliar a confiabilidade na interpretação da TCAR em lactentes prematuros de muito baixo peso (LPMBP, < 1500 g). TIPO DE ESTUDO E LOCAL: Estudo transversal sobre confiabilidade intra e interobservador da TCAR em LPMBP. MÉTODOS: 86 LPMBP foram submetidos a TCAR. Dois radiologistas pediátricos analisaram as tomografias. A confiabilidade foi medida pela proporção de concordância, coeficiente kappa (KC) e índices de concordância positiva e negativa. RESULTADOS: Para o radiologista A, na confiabilidade intraobservador o KC foi 0,79 (intervalo de confiança, IC: 0,54-1.0) para exames normais/anormais; para cada alteração tomográfica o KC variou de 0,05 a 1. Para o radiologista B, na confiabilidade intraobservador o KC foi 0,79 (IC: 0,54-1.0) para exames normais/anormais e variou de 0,37 a 0,83 para cada alteração tomográfica. Concordância interobservador foi de 88 por cento para exames normais/anormais e o KC foi 0,71 (IC: 0,5-0,93) e variou de 0,51-0,67 em muitos achados anormais. CONCLUSÃO: Para exames normais/anormais, as concordâncias intra e interobservador foram substanciais. Para muitos achados tomográficos, a concordância intraobservador variou de moderada a substancial. Nossos dados demonstram que, na prática clínica, não há razão para mais de um avaliador das imagens tomográficas, desde que este seja bem treinado na interpretação de TCAR de LPMBP. A análise por diferentes observadores estará reservada para pesquisa e casos difíceis no contexto clínico.

A computed tomography scoring system to assess pulmonary disease among premature infants.

CONTEXT AND OBJECTIVE: High-resolution computed tomography (HRCT) is considered to be the best method for detailed pulmonary evaluation. The aim here was to describe a scoring system based on abnormalities identified on HRCT among premature infants, and measure the predictive validity of the score in relation to respiratory morbidity during the first year of life. DESIGN AND SETTING: Prospective cohort study in Instituto Fernandes Figueira, Fundação Oswaldo Cruz. METHODS: Scoring system based on HRCT abnormalities among premature newborns. The affected lung area was quantified according to the number of compromised lobes, in addition to bilateral pulmonary involvement. Two radiologists applied the score to 86 HRCT scans. Intraobserver and interobserver agreement were analyzed. The score properties were calculated in relation to predictions of respiratory morbidity during the first year of life. RESULTS: Most of the patients (85%) presented abnormalities on HRCT, and among these, 56.2% presented respiratory morbidity during the first year of life. Scores ranged from zero to 12. There was good agreement between observers (intraclass correlation coefficient, ICC = 0.86, confidence interval, CI: 0.64-0.83). The predictive scores were as follows: positive predictive value 81.8%, negative predictive value 56.3%, sensitivity 39.1%, and specificity 90.0%. CONCLUSION: The scoring system is reproducible, easy to apply and allows HRCT comparisons among premature infants, by identifying patients with greater likelihood of respiratory morbidity during the first year of life. Its use will enable HRCT comparisons among premature infants with different risk factors for respiratory morbidity.

Avaliação das doses de radiação em uretrocistografia miccional de crianças / Evaluation of radiation dose in voiding cystourethrography in children

OBJETIVO: Analisar o produto dose-área, a dose de entrada na pele do paciente e as doses relativas à fluoroscopia e às radiografias em exames de cistouretrografia miccional em crianças. MATERIAIS E MÉTODOS: Foram avaliados os procedimentos em 37 pacientes, realizados por quatro médicos do serviço. As medições foram realizadas com um equipamento composto de uma câmara de ionização acoplada diretamente à saída do tubo de raios X e um eletrômetro (Diamentor) ligado diretamente ao computador, para a coleta dos dados. RESULTADOS: Foi observada alguma heterogeneidade na realização do procedimento, que não segue padrão de técnica radiográfica. São realizadas em média 11 radiografias por exame, usando tempo longo de fluoroscopia, com dose média final mais alta que a encontrada em referências da literatura. CONCLUSÃO: A adoção da técnica de alta quilovoltagem nas radiografias e o uso restrito da fluoroscopia podem proporcionar importante redução das doses durante a realização deste procedimento, porque o maior contribuinte para as altas doses verificadas foi a utilização da fluoroscopia.

OBJECTIVE: To evaluate dose-area product, skin entrance dose and doses from fluoroscopy and radiography in voiding cystourethrography studies of pediatric patients. MATERIALS AND METHODS: Procedures performed in 37 patients by four physicians of the institution were evaluated. Measurements were performed with an equipment including an ionization chamber directly coupled to the x-ray tube window and an electrometer (Diamentor) connected to a computer for data collection. RESULTS: Some procedures heterogeneity was observed and guidelines for good radiographic techniques were not followed. On average, 11 radiographies are performed for each study, with extended fluoroscopy time delivering a higher average final dose than those reported in the literature. CONCLUSION: The adoption of radiography with high kilovoltage technique and restricted utilization of fluoroscopy can result in a significant reduction of doses during this procedure, considering that the major contribution to the final dose comes from fluoroscopy.

Cholelithiasis and biliary sludge in Down’s syndrome patients

CONTEXT AND OBJECTIVE: Although studies have demonstrated increased frequency of gallbladder abnormalities among Down’s syndrome (DS) patients in some countries, there is only one paper on this subject in the Brazilian literature. The aim of this study was to demonstrate the prevalence, clinical characteristics and evolution of lithiasis and biliary sludge among DS patients in a maternity and children’s hospital in Rio de Janeiro. DESIGN AND SETTING: This was a cross-sectional study followed by a retrospective cohort study on all individuals with an ultrasound diagnosis of gallbladder abnormalities. METHODS: 547 DS patients (53.2 percent male, 46.8 percent female) attending the Instituto Fernandes Figueira in 2001 underwent abdominal ultrasound examination at ages of between one day and three years (mean: five months). Clinical and ultrasound data were analyzed. RESULTS: In 50 patients (9.1 percent), the ultrasound demonstrated gallbladder abnormalities (6.9 percent lithiasis and 2.1 percent biliary sludge). Spontaneous resolution was observed in 66.7 percent of the patients with biliary sludge and 28.9 percent with lithiasis. Cholecystectomy was carried out on 26.3 percent of the patients with gallstones. CONCLUSION: The results from this study and comparison with the literature suggest that DS patients are at risk of developing lithiasis and biliary sludge and should be monitored throughout the neonatal period, even if there are no known risk factors for gallstone formation. Most frequently, these gallbladder abnormalities occur without symptoms and spontaneously resolve in most non-symptomatic patients. DS patients should be monitored with serial abdominal ultrasound, and cholecystectomy is indicated for symptomatic cases or when cholecystitis is present.

OBJETIVO: Demonstrar prevalência, características clínicas e evolução de litíase e lama biliar em pacientes com síndrome de Down (SD) num hospital materno-infantil no Rio de Janeiro. Apesar de estudos revelarem aumento das anormalidades biliares em pacientes com SD em alguns países, no Brasil existe apenas um trabalho abordando o tema. TIPO DE ESTUDO E LOCAL: Estudo transversal seguido por um estudo de coorte retrospectivo de todos os indivíduos com diagnóstico ultra-sonográfico de anormalidades da vesícula biliar. MÉTODOS: Foram selecionados 547 pacientes com SD (53,2 por cento sexo masculino, 46,8 por cento feminino) atendidos no Instituto Fernandes Figueira, Fundação Instituto Oswaldo Cruz (IFF-Fiocruz) em 2001. Todos os pacientes incluídos neste estudo foram submetidos a ultra-sonografia abdominal quando tinham idades variando entre um dia e três anos (mediana cinco meses). Dados clínicos e ultra-sonográficos foram avaliados. RESULTADOS: Em 50 (9,1 por cento) crianças, a ultra-sonografia demonstrou alteração da vesícula biliar (6,9 por cento litíase e 2,2 por cento lama biliar). Houve resolução espontânea em 66,7 por cento dos pacientes com lama biliar e em 28,9 por cento dos pacientes com litíase. A colecistectomia foi realizada em 26,3 por cento dos pacientes com cálculo biliar. CONCLUSÃO: Resultados deste estudo e a comparação com a literatura sugerem que a SD deve ser considerada como fator de risco para desenvolvimento de litíase e lama biliar em crianças, sobretudo no período neonatal, sem que existam outros fatores predisponentes para formação de cálculo biliar. Na maioria das vezes, estas alterações são assintomáticas e, freqüentemente, têm evolução favorável, permanecendo desta forma ou tendo resolução espontânea. Pacientes devem ser acompanhados com ultra-sonografias seriadas. Tratamento cirúrgico está indicado para casos sintomáticos ou na presença de colecistite.

Cholelithiasis and biliary sludge in Downs syndrome patients.

CONTEXT AND OBJECTIVE: Although studies have demonstrated increased frequency of gallbladder abnormalities among Downs syndrome (DS) patients in some countries, there is only one paper on this subject in the Brazilian literature. The aim of this study was to demonstrate the prevalence, clinical characteristics and evolution of lithiasis and biliary sludge among DS patients in a maternity and childrens hospital in Rio de Janeiro. DESIGN AND SETTING: This was a cross-sectional study followed by a retrospective cohort study on all individuals with an ultrasound diagnosis of gallbladder abnormalities. METHODS: 547 DS patients (53.2% male, 46.8% female) attending the Instituto Fernandes Figueira in 2001 underwent abdominal ultrasound examination at ages of between one day and three years (mean: five months). Clinical and ultrasound data were analyzed. RESULTS: In 50 patients (9.1%), the ultrasound demonstrated gallbladder abnormalities (6.9% lithiasis and 2.1% biliary sludge). Spontaneous resolution was observed in 66.7% of the patients with biliary sludge and 28.9% with lithiasis. Cholecystectomy was carried out on 26.3% of the patients with gallstones. CONCLUSION: The results from this study and comparison with the literature suggest that DS patients are at risk of developing lithiasis and biliary sludge and should be monitored throughout the neonatal period, even if there are no known risk factors for gallstone formation. Most frequently, these gallbladder abnormalities occur without symptoms and spontaneously resolve in most non-symptomatic patients. DS patients should be monitored with serial abdominal ultrasound, and cholecystectomy is indicated for symptomatic cases or when cholecystitis is present.

Instabilidade atlanto-axial na síndrome de Down: o que o radiologista precisa conhecer / Atlanto-axial instability in Down syndrome: what the radiologist should know

Este trabalho teve como principais motivações o grande número de pacientes com síndrome de Down acompanhados no Departamento de genética Médica do Instituto Fernandes Figueira e um certo desconhecimento por parte dos radiologistas gerais e demais profissionais de saúde que prestam assistência a esses pacientes sobre esta alteração da coluna cervical, que tem prevalência de 10% a 30% e pode ter evolução desfavorável com paraplegia ou tetraplegia em 1% a 2% dos casos. Os autores apresentam uma revisão bibliográfica sobre a instabilidade atlanto-axial em pacientes com síndrome de Down, descrevem as principais características desta síndrome e de sua associação com essa anormalidade da coluna cervical, apresemtam as particularidades anatômicas das primeiras vértebras cervicais da criança, descrevem a técnica do exame e os principais pontos para uma correta interpretação dos achados radiológicos, e apoiados na literatura, sugerem algumas diretrizes para o acompanhamento desses pacientes quer tenham exames radiográficos normais ou tenham instabilidade atlanto-axial.

The main motivations for this study about atlantoaxial instability in Down syndrome are the great number of individuals with this syndrome attending in the Medical Genetics Department at the Instituto Fernandes Figueira, Rio de Janeiro, Brazil, and certain unfamiliarity on the part of the radiologists and the others professional that gives assitance to these patients. A prevalence of 10-30% has been described amongst individuals with Down syndrome and 1-2% of persons can have spinal cord injury and adverse evolution to paraplegia e tetraplegia. The authors describe the main characteristics of this syndrome and its association with this cervical spine abnormality; they present the atlas and axis anatomical particularities in children ; describe the techique of the examination and the main references for a correct interpretation of the radiographic findings; and supported in the literature, they suggest some follow up recommendations for patients that have normal radiographic examination or have atlantoaxial instability.

Estudo de crianças com síndrome do desconforto respiratório agudo: correlaçäo anatomoclínica radiológica / A study of children with acute respiratory distress syndrome: histopathological, clincal and radiological correlation

Estudo retrospectivo com revisäo dos casos clínicos de Sídrome do Desconforto Respiratório Agudo, realizado no período de out/88 a dez/90 na Unidade de Pacientes Graves do Instituto Fernandes Figueira. Os autores estudaram as características clínicas, radiológicas e histopatológicas de acordo com o estágio evolutivo da doença. Dentre 459 casos estudados, foram selecionados 49 (11 por cento). Onze casos tiveram exame anatomopatológico [biopsia (4), necropsia (8) e foram classificados de acordo com o estágio evolutivo em: fase exsudativa inicial, fase proliferativa celular e fase proliferativa fibrótica. Houve correlaçäo clínica radiológica e anatomopatológica nos casos confirmados com exame histológico. Os autores consideram importante estudos futuros em que a interaçäo entre a pesquisa clínica e experimental permita o melhor conhecimento desta Síndrome na populaçäo pediátrica