The radiological aspects of chest complication in childhood measles

Chest radiographs of 335 Nigerian children presenting with pulmonary and cardiac complications of measles were studied systematically. Perihilar (88%) and diffuse (69%) pulmonary infiltrates were the commonest findings. Other abnormalities included segmental consolidation (34%), emphysema (20%), cardiomegaly (12%) and atelectasis (10%). Pneumothorax and pleural effusion were seen in 24 (7%) and 14 (4%) children respectively. Eight children (2.5%) had subcutaneous and mediastinal emphysema. In patients with atelectasis and segmental consolidation there was a striking preponderance of right sided lesions.     

Allergic bronchopulmonary aspergillosis in two patients with cystic fibrosis

Allergic bronchopulmonary aspergillosis (ABPA) is hypersensitivity to Aspergillus fumigatus which manifests as episodic wheezing, usually refractory to bronchodilator therapy, with fixed and transient pulmonary infiltrates, central bronchiectasis, blood eosinophilia, elevated serum IgE level, immediate skin reactivity to an A. fumigatus antigen and precipitating antibodies to A. fumigatus. It is an unusual complication of asthma and cystic fibrosis (CF). We present two cystic fibrosis patients with ABPA treated successfully with prednisone and, in Case 1 also with itraconazole. The physician should be alert to the possibility of ABPA whenever CF patients present with the new infiltrates, high serum total IgE and other positive parameters of A. fumigatus sensitization. Treatment with systemic steroids should be started in order to prevent irreversible lung damage.     

The role of advanced imaging techniques in cystic fibrosis follow-up: is there a place for MRI?

Cystic fibrosis (CF) lung disease is caused by mutations in the CFTR-gene and remains one of the most frequent lethal inherited diseases in the Caucasian population. Given the progress in CF therapy and the consecutive improvement in prognosis, monitoring of disease progression and effectiveness of therapeutic interventions with repeated imaging of the CF lung plays an increasingly important role. So far, the chest radiograph has been the most widely used imaging modality to monitor morphological changes in the CF lung. CT is the gold standard for assessment of morphological changes of airways and lung parenchyma. Considering the necessity of life-long repeated imaging studies, the cumulative radiation doses reached with CT is problematic for CF patients. A sensitive, non-invasive and quantitative technique without radiation exposure is warranted for monitoring of disease activity. In previous studies, MRI proved to be comparable to CT regarding the detection of morphological changes in the CF lung without using ionising radiation. Furthermore, MRI was shown to be superior to CT regarding assessment of functional changes of the lung. This review presents the typical morphological and functional MR imaging findings with respect to MR-based follow-up of CF lung disease. MRI offers a variety of techniques for morphological and functional imaging of the CF lung. Using this radiation free technique short- and long-term follow-up studies are possible enabling an individualised guidance of the therapy.     

Wertigkeit bildgebender Verfahren zum Nachweis von morphologischen Veränderungen bei Zystischer Fibrose

Numerous imaging modalities play an important role in the management of patients with cystic fibrosis (CF). Thus diagnosis, therapy, and prognosis is influenced by the morphologic findings depict by these modalities. The aim of this article is the describe the role of conventional radiographic studies, computed tomography (CT), and magnetic resonance imaging (MRI) in the evaluation of pulmonary, gastrointestinal, and skeletal manifestations seen by CF. The role of the imaging modalities will be compared and discussed with clinical diagnostic procedures.     

纤维支气管镜术在104例儿童肺不张诊断及治疗中的应用

目的 探讨纤维支气管镜(简称纤支镜)术在肺不张患儿诊断和治疗中的作用.方法 对2006年1月至2010年5月入住我科的104例行纤支镜检查的肺不张患儿临床资料进行回顾性分析.结果 经支气管镜检查,104例中,痰液堵塞引起肺不张76例(73%),支气管肺发育异常13例(13%),其中1岁以内的婴幼儿占9例;支气管异物12例(12%),其中3岁以内婴幼儿占8例.经纤支镜治疗后104例中有100例肺叶复张.痰液堵塞导致肺不张的76例中,65例接受1次纤支镜术后肺叶复张,8例接受2次后复张,2例接受3次后复张;肺不张病程在3周以内的患儿1次纤支镜术后的肺叶复张率明显高于病程在3周或3周以上的患儿.结论 不同年龄儿童肺不张的病因构成不同,支气管肺发育异常在1岁以内患儿多见;1到3岁患儿异物吸入占比例较大,3岁以上患儿中痰液堵塞是常见原因.大多数痰堵导致肺不张患儿经一次纤支镜治疗就能够复张,肺不张病程越短,一次复张率越高.纤支镜术在肺不张患儿的病因诊断及治疗中发挥了重要作用.     

Roentgenographic follow-up of acute pneumonia in children.

The need for follow-up roentgenograms documenting complete clearing of pulmonary infiltrates in the pediatric patient with acute pneumonia was studied prospectively. Seventy of 129 children enrolled in the study had a repeat roentgenogram within three to four weeks after initial diagnosis. Twenty percent of this group had residual pulmonary infiltrates. Of the two thirds of those who returned for a second follow-up roetgenogram, the infiltratrates had cleared completely within three months. Routine repeat chest roentgenograms may not be necessary unless there is clinical evidence of persistent respiratory difficulty or failure to thrive.     

Aortopulmonary collateral flow in cystic fibrosis assessed with phase-contrast MRI

Background

Cystic fibrosis (CF) is a common genetic disease in Caucasians. Chronic pulmonary disease with progressive destruction of the pulmonary parenchyma is two of the major morbidities, but the relationship between clinical severity of CF and aortopulmonary collateral blood flow has not been assessed.

Objective

The purpose of this study is to measure changes in aortopulmonary collateral blood flow by phase-contrast magnetic resonance imaging (MRI) in children with CF across the spectrum of disease severity as measured by the forced expiratory volume in one second as percent predicted value (FEV_1% predicted).

Materials and methods

Sixteen patients with CF were prospectively evaluated. Eight were classified as having mild CF lung disease (FEV₁ ≥80% predicted) and eight were classified as having moderate to severe CF lung disease (FEV₁ <80% predicted). Seventeen age- and gender-matched non-CF subjects without cardiac or lung disease served as controls. Phase-contrast flow was measured at the ascending aorta, main pulmonary artery and both pulmonary arteries. Aortopulmonary collateral blood flow was calculated for each subject. The relationship between collateral flow and FEV_1% predicted was modeled using nonparametric regression. Group differences were assessed by analysis of variance.

Results

Aortopulmonary collateral blood flow began to increase as FEV_1% predicted in subjects with CF fell below 101.5% with significant further increase in the aortopulmonary collateral blood flow in the subjects with CF with moderate to severe lung disease compared to controls (0.89 vs. 0.20 L/min, P?<?0.0001). Aortopulmonary collateral blood flow correlated negatively with FEV_1% predicted (r=0.70, P?=?0.0050) confirming its relationship to this established marker of disease severity. There was no statistically significant difference in results obtained from two independent observers.

Conclusion

These preliminary findings suggest that phase-contrast MRI can be performed reliably with consistent results and without interobserver variability. While the aortopulmonary collateral blood flow is within the normal range in subjects with mild CF disease, it begins to increase even when lung function is still in the normal range. A significant increase in the aortopulmonary collateral blood flow compared to controls is measured in patients with moderate to severe CF lung disease. The studies support the notion that aortopulmonary collateral blood flow may serve as a novel and sensitive biomarker of early pulmonary disease in cystic fibrosis.     

High-attenuation mucus plugs on MDCT in a child with cystic fibrosis: potential cause and differential diagnosis

High-attenuation mucus plugging is a rare finding in both adults and children. When it occurs, the field of differential diagnoses is typically quite small and includes acute hemorrhage, aspiration of radiodense material, and allergic bronchopulmonary aspergillosis (ABPA). The last of these three diagnoses is the most difficult to make, although ABPA is more commonly seen in children with cystic fibrosis (CF) or asthma. ABPA is radiographically characterized by recurrent mucus plugging, atelectasis, and central bronchiectasis. Thus far, high-attenuation mucus plugs have only been reported in adults. We report a rare case of a child with CF who had high-attenuation mucus plugs and atelectasis that raised the possibility of ABPA. We discuss the differential diagnoses of this finding and the role of multidetector CT in these children.     

Transpleural Pulmonary-to-Systemic Venous Collaterals in a Case With Obstructed Scimitar Vein

Scimitar syndrome is a rare cause of left-to-right shunting. Surgery is indicated for a pulmonary-to-systemic blood flow ratio greater than 1.5:1 and not infrequently is complicated by postoperative obstruction. This report presents a case of scimitar syndrome and reviews how magnetic resonance imaging (MRI) can be used for initial and follow-up assessment of the syndrome with emphasis on suspected pulmonary venous obstruction. Given the potential high incidence of postoperative occlusion, MRI provides hemodynamic and anatomic information for both initial and follow-up assessment of scimitar syndrome. MRI clearly demonstrated transpleural pulmonary-to-systemic venous collaterals draining the obstructed scimitar vein.     

Abnormal MIBG uptake in a neuroblastoma patient with right upper lobe atelectasis

A 3-month-old boy with a history of an abdominopelvic neuroblastoma presented 1?week after tumor resection for a routine follow-up 123-I Meta-iodobenzylguanidine (MIBG) scan to assess for residual mass. The study demonstrated abnormal radiotracer uptake in the right upper lobe, which correlated on the SPECT/CT to an area of airspace consolidation thought to be secondary to atelectasis. To the best of our knowledge, there is one published case of MIBG radiotracer uptake in the lung correlating with pneumonia; however, there are no reported cases to date in the literature of focal pulmonary MIBG uptake corresponding to atelectasis.     

Invasive diagnostic procedures for pulmonary infiltrates in pediatric hematopoietic stem cell transplant recipients

To evaluate the role of BAL, CTB, and OLB in the management of pulmonary infiltrates in pediatric HSCT recipients, we conducted a retrospective review of clinical records of pediatric HSCT recipients. Data were analyzed using Chi-square for dichotomous and anova for continuous variables. Logistic regression was used to adjust confounding variables for diagnostic yield. Forty patients underwent 44 separate procedures. Infections were the prevailing cause of infiltrates with a positive diagnostic yield (96%). CTB and OLB were performed more often in patients with focal infiltrates compared with BAL (100%, 71% vs. 22%; p < 0.01). Adverse events were not significantly different across the three procedures. OLB more often yielded information that led to change in medical management (71% vs. 0%, 34%; p < 0.05) compared with CTB and BAL. Patients who had a positive diagnostic yield had no apparent survival advantage when compared with those in whom a procedure yielded no information. Logistic regression demonstrated that focal infiltrate was the only independently predictive variable for identifying a cause of pulmonary infiltrate. In conclusion, all three invasive diagnostic procedures were safe. Having a focal infiltrate was independently and significantly associated with having a positive diagnostic yield.     

Mucoviscidosis and precapillary pulmonary hypertension

Various pathomechanisms are responsible for the development of pulmonary arterial hypertension (PAH) in cystic fibrosis (CF). Main factors are chronic hypoxia and the loss of peripheral vessels caused by recurrent infectious lung disease and lung fibrosis. Measurement of pulmonary artery pressure and pulmonary resistance provide reliable information on PAH. Many non invasive procedures, including echocardiography, myocardial and pulmonary imaging by radionuclide, proved to be not sensitive enough to diagnose PAH. Arterialized pO2 (taken from the hyperemized ear lobe), however, measured during standardized submaximum work load, seems to be a valuable parameter to estimate PAH (r = 0.92). Treatment of PAH in CF-patients is, at the moment, predominantly based on the improvement of the general therapeutical regimen of CF and on long-term oxygen insufflation, which should be initiated as soon as there are first signs of PAH.     

Allergic bronchopulmonary aspergillosis in paediatric cystic fibrosis patients

Allergic bronchopulmonary aspergillosis (ABPA) is a severe complication in children, adolescents and adults with cystic fibrosis (CF), the prevalence of which ranges from 6-25%. The disease is the result of the colonisation of the respiratory tract by fungi of the genus Aspergillus, commonly Aspergillus fumigatus, and subsequent host sensitisation to fungal antigens, accompanied by a Th2 CD4 type response mediated by the production of specific IgE. The consequent inflammatory and obstructive bronchopulmonary injury can progress to fibrosis. The diagnosis should be considered early in patients with CF who show wheezing, transient pulmonary infiltrates and reduced lung function. The objective diagnosis is not straightforward because of overlapping clinical and radiological signs, particularly the progression of bronchiectasis. Specific criteria are needed for the diagnosis of ABPA in patients with CF, such as those proposed by the Cystic Fibrosis Foundation. The study of specific IgE against recombinant antigens of A. fumigatus has contributed to the early diagnosis of ABPA with high sensitivity and specificity. The technique has also shown promise in the follow-up of patients after steroid therapy and the early detection of recurrences. Treatment consists of long-term systemic corticosteroid usage, the monitoring of their adverse effects, and of the measurement of total serum IgE levels. The concomitant use of oral itraconazole seems to promote a better control of the disease and to reduce the duration of systemic steroid therapy but its use continues to be controversial. Controlled studies involving larger numbers of patients are necessary if we are to better understand the management of ABPA.     

Roentgenographic features of common pediatric viral respiratory tract infections

Viral infections of the respiratory tract in infants and children are common. Little has been reported on roentgenographic findings associated with infection caused by common viruses other than with respiratory syncytial virus. We studied chest roentgenograms from 128 previously healthy infants and children who were infected with respiratory syncytial virus, parainfluenza virus, influenza virus, or adenovirus. Four common roentgenographic findings were detected: parahilar peribronchial infiltrates, hyperexpansion, segmental or lobar atelectasis, and hilar adenopathy. Diffuse interstitial infiltrates and significant pleural fluid accumulations rarely occurred in our series. We confirmed the popular but not well-documented belief that other common respiratory viruses can be associated with roentgenographic findings similar to those caused by respiratory syncytial virus. However, respiratory syncytial virus infection is associated with more abnormal chest roentgenograms than any of the other viruses regardless of the clinical syndrome. Hilar adenopathy was more common in adenovirus infection. Young infants had significantly more abnormal chest roentgenograms, with more hyperexpansion and parahilar peribronchial infiltration than older children. We also found a significant correlation between lobar atelectasis and severity of the illness. In infants and children with viral infection of the lower respiratory tract, roentgenographic information can be a useful adjunct to clinical viral diagnosis.     

Subcutaneous panniculitis-like T-cell lymphoma in a child: whole-body MRI in the initial and follow-up evaluations

Subcutaneous panniculitis-like T-cell lymphoma (SPTCL) is classified as an unusual subtype of peripheral T-cell lymphoma that preferentially infiltrates the subcutaneous tissue without overt lymph node involvement. SPTCL is particularly rare in children, and there have been only a few case reports describing the US and CT findings of SPTCL. To our knowledge, the use of whole-body (WB) MRI as the initial and follow-up diagnostic method to assess the extent of disease and relapse of SPTCL has not been reported in children. In our case report involving one child, WB MRI was useful as both the initial and follow-up diagnostic method to assess the extent of disease and to monitor the patient’s response to therapy for SPTCL.     

Clinical Applications of Cardiac Magnetic Resonance Imaging After Repair of Tetralogy of Fallot

In the past 15 years, cardiovascular magnetic resonance (MR) has evolved into an imaging technique that provides adequate, and in part unique, information on residual problems in the follow-up of patients operated for tetralogy of Fallot. Spin-echo or gradient-echo cine magnetic resonance imaging allow detailed assessment of intracardiac and large vessel anatomy, which is particularly helpful in Fallot patients with residual abnormalities of right ventricular outflow and/or pulmonary artery. Multisection gradient-echo cine MRI can be used to obtain accurate measurements of biventricular size, ejection fraction, and wall mass. This allows serial follow-up of biventricular function. MR velocity mapping is the only imaging technique available that provides practical quantification of pulmonary regurgitation volume. MR velocity mapping can also be used to quantify right ventricular diastolic function in the presence of pulmonary regurgitation.     

Pulmonary function and oxygen uptake during exercise in 11-year-old patients with cystic fibrosis

Ten patients with cystic fibrosis (CF), 5 girls and 5 boys, 11 years of age, underwent pulmonary function and bicycle exercise testing. The material includes all but 2 CF patients in this age group in southern and eastern Norway. The CF children had a moderately reduced pulmonary function, the girls more pronounced than the boys. The mean maximal oxygen uptake (max VO2) in the CF boys was 55 ml/kg/min (94% of age predicted value; range 79-105%), and the CF girls 42 ml/kg/min (79% of age predicted value; range 67-89%). The mean maximal peak heart rate was 195 beats/min. Only one CF girl showed apparent pulmonary limitation during exercise. Increased ventilatory equivalent for oxygen was found during both rest and exercise for the CF girls, but only during rest for the CF boys. Max VO2 was fairly well related to the habitudinal physical activity of the children (r = 0.79).     

儿童肺炎支原体肺炎合并肺不张临床特点及纤维支气管镜的诊疗分析

目的 探讨儿童肺炎支原体肺炎（MPP）合并肺不张的临床特点及纤维支气管镜（纤支镜）和肺泡灌洗的诊治效果及介入时机.方法 选取2012年6月至2013年3月住院的MPP合并肺不张行纤支镜和肺泡灌洗检查的患儿53例为试验组,选取同期住院MPP未合并肺不张患儿35例为对照组,根据平均介入时间将试验组患儿分为早期组和晚期组,总结分析临床、实验室及支气管镜检查特点.结果 试验组患儿平均发热时间、住院时间及血清C-反应蛋白（CRP）均高于对照组（P＜0.05）.试验组患儿肺不张部位多集中在右肺中叶（18例,33.9％）,支气管镜下均可见黏膜充血肿胀及分泌物附着,部分可见黏膜滤泡样增生（9例,17.0％）、黏膜糜烂（3例,5.7％）、黏液栓形成（7例,13.2％）、段支气管通气不良（4例,7.5％）,其肺泡灌洗液可见中性粒细胞升高（43例,81.1％）、吞噬细胞减少（31例,58.5％）.经过治疗,l例（1.8％）经纤支镜治疗1次无效自动出院,52例（98.1％）患儿经治疗后均复张;支气管镜介入早期组热退时间、平均住院时间均短于晚期组（P＜0.05）.结论 MPP合并肺不张患儿平均发热时间长,CRP升高明显,支气管镜介入治疗能够促进肺不张的恢复,早期行纤支镜介入治疗能有效缩短发热时间及住院时间.     

Pulmonary Function and Oxygen Uptake during Exercise in 11-Year-Old Patients with Cystic Fibrosis

ABSTRACT. Ten patients with cystic fibrosis (CF), 5 girls and 5 boys, 11 years of age, underwent pulmonary function and bicycle exercise testing. The material includes all but 2 CF patients in this age group in southern and eastern Norway. The CF children had a moderately reduced pulmonary function, the girls more pronounced than the boys. The mean maximal oxygen uptake (max VO₂) in the CF boys was 55 ml/kg/min (94% of age predicted value; range 79-105%), and the CF girls 42 ml/kg/min (79% of age predicted value; range 67-89%). The mean maximal peak heart rate was 195 beats/min. Only one CF girl showed apparent pulmonary limitation during exercise. Increased ventilatory equivalent for oxygen was found during both rest and exercise for the CF girls, but only during rest for the CF boys. Max VO₂ was fairly well related to the habitudinal physical activity of the children ( r =0.79).     

Structural and perfusion magnetic resonance imaging of the lung in cystic fibrosis

Background

Because of its absence of ionising radiation and possibility for obtaining functional information, MRI is promising for assessing lung disease in children who require repetitive imaging for long-term follow-up.

Objective

To describe MRI findings in children with cystic fibrosis and evaluate semi-quantitative dynamic contrast-enhanced lung perfusion.

Materials and methods

We retrospectively compared lung MRI in 25 children and young adults with cystic fibrosis (median age 3.7 years) to 12 children (median age 2 years) imaged for other pathologies. MRI at 1.5 T included respiratory-gated sequences and contrast-enhanced lung perfusion imaging. We described and graded any morphologic change. Signal enhancement and time to peak values of perfusion abnormalities were compared to those of normally enhancing lung parenchyma.

Results

Frequent findings in patients with cystic fibrosis were bronchial wall thickening (24/25, 96%), areas of consolidation (22/25, 88%), enlarged lymph nodes (20/25, 80%), bronchiectasis (5/25, 20%) and mucus plugging (3/25, 12%). Compared to normally enhancing lung, perfusion defects (21/25, 84%), characterised by decreased enhancement, showed prolonged time to peak. Areas of consolidation showed increased enhancement. While time to peak of procedure-related atelectasis was not significantly different from that of normal lung, disease-related consolidation showed prolonged time to peak (P=0.01).

Conclusion

Lung MRI demonstrates structural and perfusion abnormalities in children and young people with cystic fibrosis. Semi-quantitative assessment of dynamic contrast-enhanced perfusion imaging might allow differentiation between procedure-related atelectasis and disease-related consolidation.