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1.
Pediatrics ; 144(3)2019 Sep.
Artigo em Inglês | MEDLINE | ID: mdl-31455612

RESUMO

BACKGROUND: Diagnosis of tuberculosis should be improved in children infected with HIV to reduce mortality. We developed prediction scores to guide antituberculosis treatment decision in HIV-infected children with suspected tuberculosis. METHODS: HIV-infected children with suspected tuberculosis enrolled in Burkina Faso, Cambodia, Cameroon, and Vietnam (ANRS 12229 PAANTHER 01 Study), underwent clinical assessment, chest radiography, Quantiferon Gold In-Tube (QFT), abdominal ultrasonography, and sample collection for microbiology, including Xpert MTB/RIF (Xpert). We developed 4 tuberculosis diagnostic models using logistic regression: (1) all predictors included, (2) QFT excluded, (3) ultrasonography excluded, and (4) QFT and ultrasonography excluded. We internally validated the models using resampling. We built a score on the basis of the model with the best area under the receiver operating characteristic curve and parsimony. RESULTS: A total of 438 children were enrolled in the study; 251 (57.3%) had tuberculosis, including 55 (12.6%) with culture- or Xpert-confirmed tuberculosis. The final 4 models included Xpert, fever lasting >2 weeks, unremitting cough, hemoptysis and weight loss in the past 4 weeks, contact with a patient with smear-positive tuberculosis, tachycardia, miliary tuberculosis, alveolar opacities, and lymph nodes on the chest radiograph, together with abdominal lymph nodes on the ultrasound and QFT results. The areas under the receiver operating characteristic curves were 0.866, 0.861, 0.850, and 0.846, for models 1, 2, 3, and 4, respectively. The score developed on model 2 had a sensitivity of 88.6% and a specificity of 61.2% for a tuberculosis diagnosis. CONCLUSIONS: Our score had a good diagnostic performance. Used in an algorithm, it should enable prompt treatment decision in children with suspected tuberculosis and a high mortality risk, thus contributing to significant public health benefits.

2.
J Digit Imaging ; 2019 Jun 24.
Artigo em Inglês | MEDLINE | ID: mdl-31236743

RESUMO

Patient-specific 3D modeling is the first step towards image-guided surgery, the actual revolution in surgical care. Pediatric and adolescent patients with rare tumors and malformations should highly benefit from these latest technological innovations, allowing personalized tailored surgery. This study focused on the pelvic region, located at the crossroads of the urinary, digestive, and genital channels with important vascular and nervous structures. The aim of this study was to evaluate the performances of different software tools to obtain patient-specific 3D models, through segmentation of magnetic resonance images (MRI), the reference for pediatric pelvis examination. Twelve software tools freely available on the Internet and two commercial software tools were evaluated using T2-w MRI and diffusion-weighted MRI images. The software tools were rated according to eight criteria, evaluated by three different users: automatization degree, segmentation time, usability, 3D visualization, presence of image registration tools, tractography tools, supported OS, and potential extension (i.e., plugins). A ranking of software tools for 3D modeling of MRI medical images, according to the set of predefined criteria, was given. This ranking allowed us to elaborate guidelines for the choice of software tools for pelvic surgical planning in pediatric patients. The best-ranked software tools were Myrian Studio, ITK-SNAP, and 3D Slicer, the latter being especially appropriate if nerve fibers should be included in the 3D patient model. To conclude, this study proposed a comprehensive review of software tools for 3D modeling of the pelvis according to a set of eight criteria and delivered specific conclusions for pediatric and adolescent patients that can be directly applied to clinical practice.

4.
Pediatr Radiol ; 49(5): 575-585, 2019 05.
Artigo em Inglês | MEDLINE | ID: mdl-30652195

RESUMO

BACKGROUND: In children, idiopathic and heritable pulmonary arterial hypertension present echocardiographic and heart catheterization findings similar to findings in pulmonary veno-occlusive disease. OBJECTIVE: To provide a systematic analysis of CT angiography anomalies in children with idiopathic or heritable pulmonary arterial hypertension, or pulmonary veno-occlusive disease. We also sought to identify correlations between CT findings and patients' baseline characteristics. MATERIALS AND METHODS: We retrospectively analyzed CT features of children with idiopathic and heritable pulmonary arterial hypertension or pulmonary veno-occlusive disease and 30 age-matched controls between 2008 and 2014. We compared CT findings and patient characteristics, including gene mutation type, and disease outcome until 2017. RESULTS: The pulmonary arterial hypertension group included idiopathic (n=15) and heritable pulmonary arterial hypertension (n=11) and pulmonary veno-occlusive disease (n=4). Median age was 6.5 years. Children with pulmonary arterial hypertension showed enlargement of pulmonary artery and right cardiac chambers. A threshold for the ratio between the pulmonary artery and the ascending aorta of ≥1.2 had a sensitivity of 90% and a specificity of 100% for pulmonary arterial hypertension. All children with pulmonary veno-occlusive disease had thickened interlobular septa, centrilobular ground-glass opacities, and lymphadenopathy. In children with idiopathic and heritable pulmonary arterial hypertension, presence of intrapulmonary neovessels and enlargement of the right atrium were correlated with higher mean pulmonary artery pressure (P=0.011) and pulmonary vascular resistance (P=0.038), respectively. Mediastinal lymphadenopathy was associated with disease worsening within the first 2 years of follow-up (P=0.024). CONCLUSION: CT angiography could contribute to early diagnosis and prediction of severity in children with pulmonary arterial hypertension.


Assuntos
Angiografia por Tomografia Computadorizada , Hipertensão Pulmonar/diagnóstico por imagem , Pneumopatia Veno-Oclusiva/diagnóstico por imagem , Adolescente , Cateterismo Cardíaco , Estudos de Casos e Controles , Criança , Pré-Escolar , Diagnóstico Precoce , Hipertensão Pulmonar Primária Familiar/diagnóstico por imagem , Hipertensão Pulmonar Primária Familiar/genética , Feminino , Humanos , Hipertensão Pulmonar/genética , Lactente , Masculino , Valor Preditivo dos Testes , Pneumopatia Veno-Oclusiva/genética , Interpretação de Imagem Radiográfica Assistida por Computador , Estudos Retrospectivos , Sensibilidade e Especificidade
5.
Nature ; 558(7711): 540-546, 2018 06.
Artigo em Inglês | MEDLINE | ID: mdl-29899452

RESUMO

CLOVES syndrome (congenital lipomatous overgrowth, vascular malformations, epidermal naevi, scoliosis/skeletal and spinal syndrome) is a genetic disorder that results from somatic, mosaic gain-of-function mutations of the PIK3CA gene, and belongs to the spectrum of PIK3CA-related overgrowth syndromes (PROS). This rare condition has no specific treatment and a poor survival rate. Here, we describe a postnatal mouse model of PROS/CLOVES that partially recapitulates the human disease, and demonstrate the efficacy of BYL719, an inhibitor of PIK3CA, in preventing and improving organ dysfunction. On the basis of these results, we used BYL719 to treat nineteen patients with PROS. The drug improved the disease symptoms in all patients. Previously intractable vascular tumours became smaller, congestive heart failure was improved, hemihypertrophy was reduced, and scoliosis was attenuated. The treatment was not associated with any substantial side effects. In conclusion, this study provides the first direct evidence supporting PIK3CA inhibition as a promising therapeutic strategy in patients with PROS.

6.
Pediatr Emerg Care ; 34(6): e102-e103, 2018 Jun.
Artigo em Inglês | MEDLINE | ID: mdl-29762333

RESUMO

Tension pneumoperitoneum is a relatively rare occurrence in the pediatric population. However, tension pneumoperitoneum is associated with significant morbidity and mortality if it is not promptly diagnosed and treated. A 5-year-old girl was admitted to emergency department with abdominal pain. She was in shock, and the radiograph film of the abdomen revealed a voluminous tension pneumoperitoneum. Aggressive fluid challenges were performed in intensive care followed by urgent laparotomy. Primary abdominal compartment due to trapped gas caused a decreased venous return and visceral perfusion. In the absence of hemodynamic improvement after vascular filling, needle decompression was performed before surgery.


Assuntos
Hipertensão Intra-Abdominal/diagnóstico , Pneumoperitônio/complicações , Choque/etiologia , Pré-Escolar , Descompressão Cirúrgica/métodos , Feminino , Hemodinâmica , Humanos , Hipertensão Intra-Abdominal/etiologia , Hipertensão Intra-Abdominal/terapia , Laparotomia/métodos , Pneumoperitônio/terapia , Choque/terapia
7.
Clin Gastroenterol Hepatol ; 16(7): 1089-1097.e4, 2018 Jul.
Artigo em Inglês | MEDLINE | ID: mdl-29501599

RESUMO

BACKGROUND & AIMS: Bowel healing is an important goal of therapy for patients with Crohn's disease (CD). Although there have been many studies of mucosal healing, transmural healing (ie, in the bowel wall) has not been investigated in children. We analyzed data from the ImageKids study to determine associations among mucosal, transmural healing and levels of calprotectin and C-reactive protein in children with CD. METHODS: We collected data from a multi-center study designed to develop 2 magnetic resonance enterography (MRE)-based measures for children with CD (6-18 years old). In our analysis of 151 children (mean age, 14.2 ± 2.4 years), all patients underwent MRE and a complete ileocolonoscopic evaluation; fecal levels of calprotectin and blood levels of C-reactive protein were measured. Mucosal healing was defined as simple endoscopic severity index in CD score below 3, transmural healing as an MRE visual analogue score below 20 mm, and deep healing as a combination of transmural and mucosal healing. RESULTS: We identified mucosal healing with transmural inflammation in 9 children (6%), transmural healing with mucosal inflammation in 38 children (25%), deep healing in 21 children (14%), and mucosal and transmural inflammation in 83 children (55%). The median level of calprotectin was lowest in children with deep healing (mean level, 10 µg/g; interquartile range, 10-190 µg/g), followed by children with either transmural or mucosal inflammation, and highest in children with mucosal and transmural inflammation (810 µg/g; interquartile range, 539-1737 µg/g) (P < .001). Fecal level of calprotectin identified children with deep healing with an area under the receiver operating characteristic curve value of 0.93 (95% CI, 0.89-0.98); level of C-reactive protein identified children with deep healing with an area under the receiver operating characteristic curve value of 0.81 (95% CI, 0.71-0.9). A calprotectin cutoff value of 100 µg/g identified children with deep healing with 71% sensitivity and 92% specificity; a cutoff value of 300 µg/g identified children with mucosal healing with 80% sensitivity and 81% specificity. CONCLUSIONS: In a prospective study of children with CD, we found that one-third have healing in only the mucosa or the bowel wall (not both). Levels of fecal calprotectin below 300 µg/identify children with mucosal healing, but a lower cutoff value (below 100 µg/g) is needed to identify children with deep healing. Clinicaltrials.gov no: NCT01881490.

8.
Eur J Cardiothorac Surg ; 54(2): 246-251, 2018 Aug 01.
Artigo em Inglês | MEDLINE | ID: mdl-29506211

RESUMO

OBJECTIVES: Bronchopulmonary sequestration (BPS) is the second most common congenital lung malformation, with an estimated incidence ranging from 0.15% to 1.8%. Surgical treatment is elective in patients with symptoms, but the management of asymptomatic patients remains controversial. METHODS: We retrospectively reviewed the medical records of 99 patients treated for BPS in our institution from January 2000 to December 2015. BPS was diagnosed prenatally in 86 (87%) cases. Management throughout this 16-year period was based on 3 interventions: resection by open surgery, resection by thoracoscopy and embolization. RESULTS: Among the 86 patients with a prenatal diagnosis of BPS, 14% had symptoms at birth and 10% had delayed symptoms at a median delay of 8 months (4.5-42 months). For the other 13 patients, symptoms occurred at a median age of 34 months (range 3-96 months). Embolization of the feeding vessel was performed in 46 patients with 6 secondary surgical resections (13%). A total of 59 patients were operated on: 23 cases by open surgery and 36 cases by thoracoscopy. The mean hospitalization stay was significantly longer for open surgery: 4.8 ± 1.3 days vs 4.1 ±1.5 days, respectively (P = 0.03). Differences in hospitalization stay were also found between asymptomatic and symptomatic patients: 3.5 ± 1.2 vs 5.1 ±1.6 days, respectively (P = 0.002). Two of the operated patients died. CONCLUSIONS: When surgery is chosen, thoracoscopy appears to be a valuable procedure. A better understanding of the natural history of BPS is still needed to define the optimal management and the respective roles of surgery, embolization or non-interventional follow-up.

10.
Lancet HIV ; 5(2): e87-e95, 2018 02.
Artigo em Inglês | MEDLINE | ID: mdl-29174612

RESUMO

BACKGROUND: Tuberculosis is a major cause of morbidity and mortality in HIV-infected children, but is difficult to diagnose. We studied mortality and its determinants in antiretroviral treatment (ART)-naive HIV-infected children presenting with suspected tuberculosis. METHODS: In this observational cohort study, HIV-infected children aged 13 years or younger with suspected tuberculosis were followed up for 6 months as part of the ANRS 12229 PAANTHER 01 cohort in eight hospitals in four countries (Burkina Faso, Cambodia, Cameroon, and Vietnam). Children started ART and antituberculosis treatment at the clinician's discretion and were retrospectively classified into one of three groups by tuberculosis documentation: confirmed by culture or Xpert MTB/RIF, unconfirmed, and unlikely. We assessed mortality and associated factors using Kaplan-Meier methods and Cox proportional hazard models. The ANRS 12229 PAANTHER 01 study is registered at ClinicalTrials.gov, number NCT01331811. FINDINGS: 266 (61%) of 438 children enrolled in the study between April 27, 2011, and May 31, 2014, were ART-naive and included in the analysis (40 had confirmed tuberculosis, 119 unconfirmed tuberculosis, and 107 unlikely tuberculosis). 112·5 person-years of follow-up were available. 154 children (58%) started antituberculosis treatment and 212 (80%) started ART. 50 children (19%) died. Mortality by 6 months was higher in children with confirmed tuberculosis (14 deaths; 2 month survival probability 65·0% [95% CI 50·2-79·8]) compared with unconfirmed tuberculosis (19 deaths; 83·5% [76·8-90·3]) and unlikely tuberculosis (17 deaths; 83·5% [76·3-90·7]; log-rank p=0·0141) and was lower in children with confirmed or unconfirmed tuberculosis who started antituberculosis treatment (p<0·0001 for both). In a multivariate analysis, ART started during the first month of follow-up (hazard ratio 0·08; 95% CI 0·01-0·67), confirmed tuberculosis (6·33; 2·15-18·64), young age (5·90; 2·02-17·19), CD4 less than 10% (2·63; 1·25-5·53), miliary features (4·08; 1·56-10·66), and elevated serum transaminases (4·40; 1·82-10·65) were all independently associated with mortality. INTERPRETATION: In our cohort, mortality was high in the first 6 months after suspicion of tuberculosis in ART-naive children. ART should be started early, particularly in children with factors associated with high mortality. Documented or empirical tuberculosis treatment decision should be accelerated to reduce mortality and allow early ART initiation. FUNDING: ANRS and Fondation Total.


Assuntos
Fármacos Anti-HIV/uso terapêutico , Antituberculosos/uso terapêutico , Infecções por HIV/tratamento farmacológico , Infecções por HIV/mortalidade , Tuberculose/tratamento farmacológico , Tuberculose/mortalidade , Adolescente , Burkina Faso , Camboja , Camarões , Criança , Pré-Escolar , Estudos de Coortes , Coinfecção , Feminino , Humanos , Estimativa de Kaplan-Meier , Masculino , Mortalidade , Vietnã
13.
J Inherit Metab Dis ; 38(2): 257-63, 2015 Mar.
Artigo em Inglês | MEDLINE | ID: mdl-25335805

RESUMO

BACKGROUND AND OBJECTIVES: Lysinuric protein intolerance (LPI) is a rare multisystemic metabolic disease. The objective of the study was to describe presentation and course of lung involvement in a cohort of ten children. PATIENTS AND METHODS: Retrospective review of patients followed at Necker-Enfants Malades University Hospital between 1980 and 2012 for a LPI. In patients with lung involvement, clinical data, chest radiographs, pulmonary function tests, bronchoalveolar lavages, and lung biopsies were analyzed. The first and last high-resolution computed tomography (HRCT) were also reviewed. RESULTS: Lung involvement was observed in ten of 14 patients (71 %). Five patients had an acute onset of respiratory symptoms, three had a progressive onset and two were free of symptoms. During the period studied, six patients (60 %) died, all in a context of respiratory failure. Clinical presentation and course were highly variable, even in the same family. HRCT were performed in seven cases, showing in all cases an interstitial pattern and fibrosis in four. All ten patients had pulmonary alveolar proteinosis (PAP) confirmed by histopathological analysis. Five patients had pulmonary fibrosis (at biopsy and/or HRCT scan). Two patients underwent whole lung lavages, without efficiency. CONCLUSION: PAP is a constant feature in children with LPI and lung involvement. Pulmonary fibrosis is frequent and these two pathologies may develop independently. This study shows the heterogeneity of presentation and outcome. Lung injury could be secondary to impaired phagocytic function and abnormal inflammatory and immune responses intrinsic to the SLC7A7 mutant phenotype. HRCT is recommended to detect lung involvement.


Assuntos
Erros Inatos do Metabolismo dos Aminoácidos/complicações , Doenças Autoimunes/etiologia , Pulmão , Proteinose Alveolar Pulmonar/etiologia , Fibrose Pulmonar/etiologia , Adolescente , Adulto , Erros Inatos do Metabolismo dos Aminoácidos/diagnóstico , Erros Inatos do Metabolismo dos Aminoácidos/mortalidade , Erros Inatos do Metabolismo dos Aminoácidos/terapia , Doenças Autoimunes/diagnóstico , Doenças Autoimunes/mortalidade , Doenças Autoimunes/fisiopatologia , Doenças Autoimunes/terapia , Biópsia , Lavagem Broncoalveolar , Criança , Pré-Escolar , Progressão da Doença , Feminino , Cadeias Leves da Proteína-1 Reguladora de Fusão/genética , Predisposição Genética para Doença , Hospitais Pediátricos , Hospitais Universitários , Humanos , Lactente , Recém-Nascido , Pulmão/diagnóstico por imagem , Pulmão/patologia , Pulmão/fisiopatologia , Masculino , Mutação , Paris , Valor Preditivo dos Testes , Proteinose Alveolar Pulmonar/diagnóstico , Proteinose Alveolar Pulmonar/mortalidade , Proteinose Alveolar Pulmonar/fisiopatologia , Proteinose Alveolar Pulmonar/terapia , Fibrose Pulmonar/diagnóstico , Fibrose Pulmonar/mortalidade , Fibrose Pulmonar/fisiopatologia , Fibrose Pulmonar/terapia , Testes de Função Respiratória , Insuficiência Respiratória/diagnóstico , Insuficiência Respiratória/etiologia , Estudos Retrospectivos , Fatores de Tempo , Tomografia Computadorizada por Raios X , Adulto Jovem
14.
Orphanet J Rare Dis ; 9: 85, 2014 Jun 14.
Artigo em Inglês | MEDLINE | ID: mdl-24927752

RESUMO

BACKGROUND: Pulmonary alveolar proteinosis (PAP) is very rare in children. Only a few small series have been published, with little information about long-term progression. The objective of our study was to describe the clinical, radiological and pathological features, and the long-term course of PAP in a cohort of 34 children from La Réunion Island. METHODS: Data were retrospectively collected from medical files. Radiological and pathological elements were reviewed by two pediatric radiologists and three pathologists, respectively. RESULTS: Thirteen cases were familial and 32/34 (94%) cases were family connected. Disease onset occurred in the first six months of life in 82% of the patients. Thoracic computed tomography scans showed the typical "crazy-paving" pattern in 94% of cases. Respiratory disease was associated with a liver disorder, with the detection of liver enlargement at diagnosis in 56% of cases. The course of the disease was characterized by frequent progression to chronic respiratory insufficiency, accompanied by the appearance of cholesterol granulomas and pulmonary fibrosis. Overall prognosis was poor, with a mortality of 59% and an overall five-year survival rate from birth of 64%. Whole-lung lavages were performed in 21 patients, with no significant effect on survival. Liver disease progressed to cirrhosis in 18% of children, with no severe complication. CONCLUSIONS: PAP in children from la Réunion Island is characterized by an early onset, associated liver involvement, poor prognosis and frequent progression to lung fibrosis, despite whole-lung lavages treatment. The geographic clustering of patients and the detection of many familial links between most of the cases strongly suggest a genetic etiology, with an autosomal recessive mode of inheritance.


Assuntos
Proteinose Alveolar Pulmonar/diagnóstico , Criança , Pré-Escolar , Feminino , França , Humanos , Lactente , Masculino , Linhagem , Proteinose Alveolar Pulmonar/diagnóstico por imagem , Proteinose Alveolar Pulmonar/genética , Radiografia , Estudos Retrospectivos
15.
Pediatr Radiol ; 44(7): 795-802, 2014 Jul.
Artigo em Inglês | MEDLINE | ID: mdl-24599270

RESUMO

BACKGROUND: Pulmonary alveolar proteinosis (PAP) is characterized by an abnormal accumulation of periodic acid-schiff-positive lipoproteinaceous material in the alveoli. Early diagnosis allows setting up of therapeutic lung lavages, which reduces the need for oxygen supplementation and weight gain. OBJECTIVE: To provide a description of radiological features by CT at the onset of primary PAP in children. MATERIALS AND METHODS: The clinical and radiological data of 24 patients, including 16 boys and 8 girls (median age: 12 months), diagnosed with a primary form of PAP between April 1992 and May 2012 in a tertiary referral hospital, were retrospectively reviewed. CT images were examined for the presence of alveolar and interstitial elementary lesions. Correlation between clinical and radiological findings was assessed. RESULTS: The types of elementary lesions detected were: ground-glass opacities (n = 24), intralobular lines (n = 24), thickened interlobular septa (n = 22), thickened fissures (n = 21), airspace consolidation (n = 16), hyperinflation (n = 16), cystic lesions (n = 2) and micronodules (n = 1). A crazy-paving pattern was found in 92% of cases. Consolidation and hyperinflation were especially detected in younger children (median age, 8 months, P < 0.01). A density dependent gradient was found. The distribution of the lesions was symmetrical. There was no correlation between radiological and clinical data of severity of the disease. CONCLUSION: CT findings are suggestive of diagnosis of PAP in immunocompetent children with chronic respiratory failure.


Assuntos
Proteinose Alveolar Pulmonar/diagnóstico por imagem , Tomografia Computadorizada por Raios X , Criança , Pré-Escolar , Feminino , Humanos , Lactente , Pulmão/diagnóstico por imagem , Masculino , Estudos Retrospectivos
16.
Pediatr Pulmonol ; 49(5): 512-4, 2014 May.
Artigo em Inglês | MEDLINE | ID: mdl-24022880

RESUMO

Extralobar pulmonary sequestration is a congenital lung malformation characterized by a non-functional lung segment with systemic feeding vessel. Over 90% of sequestrations are found in the thorax with less than 10% located in the abdomen. We present an unusual case of intra abdominal pulmonary sequestration, located suprarenally, adherent to both the stomach and the diaphragm. The malformation was surgically excised via laparoscopy in the second year of life, when no evidence of regression was found on follow up imaging. On the occasion of this description, the spectrum of bronchopulmonary foregut malformation is discussed.


Assuntos
Sequestro Broncopulmonar/diagnóstico por imagem , Diafragma/anormalidades , Pulmão/anormalidades , Estômago/anormalidades , Sequestro Broncopulmonar/cirurgia , Humanos , Recém-Nascido , Pulmão/cirurgia , Tomografia Computadorizada por Raios X , Ultrassonografia Pré-Natal
17.
Pediatr Radiol ; 43(5): 558-67, 2013 Mar.
Artigo em Inglês | MEDLINE | ID: mdl-23224105

RESUMO

BACKGROUND: The potential effects of ionizing radiation are of particular concern in children. The model-based iterative reconstruction VEO(TM) is a technique commercialized to improve image quality and reduce noise compared with the filtered back-projection (FBP) method. OBJECTIVE: To evaluate the potential of VEO(TM) on diagnostic image quality and dose reduction in pediatric chest CT examinations. MATERIALS AND METHODS: Twenty children (mean 11.4 years) with cystic fibrosis underwent either a standard CT or a moderately reduced-dose CT plus a minimum-dose CT performed at 100 kVp. Reduced-dose CT examinations consisted of two consecutive acquisitions: one moderately reduced-dose CT with increased noise index (NI = 70) and one minimum-dose CT at CTDIvol 0.14 mGy. Standard CTs were reconstructed using the FBP method while low-dose CTs were reconstructed using FBP and VEO. Two senior radiologists evaluated diagnostic image quality independently by scoring anatomical structures using a four-point scale (1 = excellent, 2 = clear, 3 = diminished, 4 = non-diagnostic). Standard deviation (SD) and signal-to-noise ratio (SNR) were also computed. RESULTS: At moderately reduced doses, VEO images had significantly lower SD (P < 0.001) and higher SNR (P < 0.05) in comparison to filtered back-projection images. Further improvements were obtained at minimum-dose CT. The best diagnostic image quality was obtained with VEO at minimum-dose CT for the small structures (subpleural vessels and lung fissures) (P < 0.001). The potential for dose reduction was dependent on the diagnostic task because of the modification of the image texture produced by this reconstruction. CONCLUSIONS: At minimum-dose CT, VEO enables important dose reduction depending on the clinical indication and makes visible certain small structures that were not perceptible with filtered back-projection.


Assuntos
Algoritmos , Fibrose Cística/diagnóstico por imagem , Modelos Biológicos , Intensificação de Imagem Radiográfica/métodos , Interpretação de Imagem Radiográfica Assistida por Computador/métodos , Radiografia Torácica/métodos , Tomografia Computadorizada por Raios X/métodos , Criança , Simulação por Computador , Feminino , Humanos , Masculino , Estudos Prospectivos , Reprodutibilidade dos Testes , Sensibilidade e Especificidade
18.
Eur J Haematol ; 84(3): 259-65, 2010 Mar.
Artigo em Inglês | MEDLINE | ID: mdl-19912310

RESUMO

OBJECTIVES: In children with sickle cell disease (SCD), chronic transfusion to maintain haemoglobin S (HbS) below 30% markedly decreases both the risk of a first stroke when transcranial Doppler (TCD) ultrasonography shows abnormal cerebral blood flow velocities and the risk of recurrent stroke. Maintaining HbS below 30% may be difficult, especially in countries where blood donors and recipients belong to different ethnic groups and where the availability of closely matched blood products is limited. We assessed the feasibility and efficacy of chronic transfusion with an HbS target of 30% in children with SCD living in the Paris area. METHODS: We retrospectively studied 29 children aged 6.8 +/- 3.0 yr (3-15 yr) at inclusion who received chronic transfusion either because of abnormal TCD findings (primary prevention group, PPG, n = 17) or because of a previous cerebrovascular event (secondary prevention group, SPG, n = 12 including nine with a history of stroke and three of transient ischaemic attacks). RESULTS: Mean follow-up was 3.5 +/- 3.0 yr (0.5-12 yr). No cases of stroke occurred in the PPG. In the SPG, one patient with a history of stroke and severe cerebrovascular disease had a recurrence after 11 yr of chronic transfusion, when the HbS level was 20%. The stroke recurrence rate (SPG group) was 1.6/100 patient-years. Mean HbS levels before and after transfusion were 30 +/- 10% and 20.6 +/- 7%, respectively. Two patients acquired red-cell alloantibodies. Of the 29 patients, 22 required iron chelation. CONCLUSIONS: Regular transfusion maintaining HbS below 30% is feasible and safe in children with SCD in France and protects from overt stroke.


Assuntos
Anemia Falciforme/terapia , Transfusão de Sangue , Acidente Vascular Cerebral/prevenção & controle , Adolescente , Anemia Falciforme/complicações , Circulação Cerebrovascular , Terapia por Quelação , Criança , Pré-Escolar , Eritrócitos/imunologia , Estudos de Viabilidade , Feminino , Seguimentos , Hemoglobina Falciforme/análise , Humanos , Quelantes de Ferro/uso terapêutico , Sobrecarga de Ferro/tratamento farmacológico , Sobrecarga de Ferro/etiologia , Isoanticorpos/biossíntese , Imagem por Ressonância Magnética , Masculino , Paris , Estudos Retrospectivos , Prevenção Secundária , Acidente Vascular Cerebral/diagnóstico por imagem , Acidente Vascular Cerebral/etiologia , Ultrassonografia Doppler Transcraniana
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