MRI Liver Imaging Integrated with Texture Analysis in Native Liver Survivor Patients with Biliary Atresia after Kasai Portoenterostomy: Correlation with Medical Outcome after Surgical Treatment.

Kasai portoenterostomy (KP) plays a crucial role in the treatment of biliary atresia (BA). The aim is to correlate MRI quantitative findings of native liver survivor BA patients after KP with a medical outcome. Thirty patients were classified as having ideal medical outcomes (Group 1; n = 11) if laboratory parameter values were in the normal range and there was no evidence of chronic liver disease complications; otherwise, they were classified as having nonideal medical outcomes (Group 2; n = 19). Liver and spleen volumes, portal vein diameter, liver mean, and maximum and minimum ADC values were measured; similarly, ADC and T2-weighted textural parameters were obtained using ROI analysis. The liver volume was significantly (p = 0.007) lower in Group 2 than in Group 1 (954.88 ± 218.31 cm3 vs. 1140.94 ± 134.62 cm3); conversely, the spleen volume was significantly (p < 0.001) higher (555.49 ± 263.92 cm3 vs. 231.83 ± 70.97 cm3). No differences were found in the portal vein diameter, liver ADC values, or ADC and T2-weighted textural parameters. In conclusion, significant quantitative morpho-volumetric liver and spleen abnormalities occurred in BA patients with nonideal medical outcomes after KP, but no significant microstructural liver abnormalities detectable by ADC values and ADC and T2-weighted textural parameters were found between the groups.

Crohn disease-like enterocolitis remission after empagliflozin treatment in a child with glycogen storage disease type Ib: a case report.

BACKGROUND: Besides major clinical/biochemical features, neutropenia and inflammatory bowel disease (IBD) constitute common complications of Glycogen storage disease type Ib (GSD Ib). However, their management is still challenging. Although previous reports have shown benefit of empagliflozin administration on neutropenia, no follow-up data on bowel (macro/microscopic) morphology are available. We herein present for the first time longitudinal assessment of bowel morphology in a GSD Ib child suffering from Crohn disease-like enterocolitis treated with empagliflozin. CASE PRESENTATION: A 14-year-old boy with GSD Ib and severe IBD was (off-label) treated with empagliflozin (20 mg/day) after informed oral and written consent was obtained from the patient's parents. No adverse events were noted. Clinical symptoms and stool frequency improved within the first week of treatment. Pediatric Crohn disease activity index (PCDAI) normalised within the first month of treatment. Abdomen magnetic resonance imaging (MRI) performed 3 months after treatment initiation showed dramatic decrease in disease activity and length. Similar findings were reported on histology at 5.5 months. At 7.5 months hemoglobin levels normalised and fecal calprotectin almost normalised. Improved neutrophil count, metabolic control and quality of life were also noted. G-CSF dose was decreased by 33% and the patient was partly weaned from tube feeding. CONCLUSIONS: This is the first report presenting extensive gastrointestinal morphology follow-up in a GSD Ib patient receiving empagliflozin. The present case suggests that empagliflozin can be safe and effective in inducing IBD remission in GSD Ib patients and can even postpone surgery. Future studies are required to confirm its effect over time and assess its benefit in various disease stages. The development of an international collaborating networks for systematic data collection is worthy.

Imaging prediction with ultrasound and MRI of long-term medical outcome in native liver survivor patients with biliary atresia after kasai portoenterostomy: a pilot study.

PURPOSE: To comparatively assess the role of abdominal ultrasound (US) and magnetic resonance imaging (MRI) in predicting long-term medical outcome in native liver survivor patients with biliary atresia (BA) after Kasai portoenterostomy (KP). METHODS: Twenty-four retrospectively enrolled patients were divided in two groups according to clinical and laboratory data at initial evaluation after KP (median follow-up = 9.7 years; range = 5-25 years) as with ideal (Group 1; n = 15) or non-ideal (Group 2; n = 9) medical outcome. All patients were re-evaluated for a period of additional 4 years using clinical and laboratory indices. US and MRI studies were qualitatively analyzed assessing imaging signs suggestive of chronic liver disease (CLD). RESULTS: At re-evaluation, 6 patients (40%) of Group 1 changed their medical outcome in non-ideal (Group 1A); the other 9 patients (60%) remained stable (Group 1B); the mean time to change the medical outcome in non-ideal status at re-evaluation was 43.5 ± 2.3 months. The area under the ROC curve was 0.84 and 0.87 for US and MRI scores to predict long-term medical outcome with the best cut-off value score > 4 for both modalities (p = 0.89). In Group 2, 6 (67%) patients showed a clinical progression (Group 2A) with a mean time of 39.8 ± 3.8 months; in the other 3 (33%) patients, no clinical progression was observed (Group 2B). CONCLUSION: In BA patients with ideal medical outcome after KP, US and MRI may both predict long-term outcome. US, non-invasive and widely available technique, should be preferred.

Precocious ischemia preceding bilateral adrenal hemorrhage: A case report.

We present a case of a middle-age male who presented in emergency room with nonspecific abdominal pain. A contrast-enhanced computer tomography (ceCT) scan showed a reduced perfusion of both adrenal glands. The clinical examinations and the laboratory tests were negative for an adrenal pathological process. To reassess the adrenal ischemia, a second ceCT scan was performed 5 days later showing an acute bilateral adrenal hemorrhage. These findings demonstrated that the previous adrenal hypoperfusion represented the prodromal manifestation of a hemorrhagic intraglandular process. This case suggests that adrenal hypoperfusion detected on tomographic imaging dictates a prompt clinical management finalized to strictly monitor the potential evolution towards a more aggressive pathological condition and confirms the pivotal role of imaging in the diagnosis of such uncommon disorder.

Ultrasound, shear-wave elastography, and magnetic resonance imaging in native liver survivor patients with biliary atresia after Kasai portoenterostomy: correlation with medical outcome after treatment.

BACKGROUND: Biliary atresia (BA) is a rare obliterative cholangiopathy and Kasai portoenterostomy (KP) represents its first-line treatment; clinical and laboratory parameters together with abdominal ultrasound (US) are usually performed during the follow-up. Shear-wave elastography (SWE) is able to evaluate liver parenchyma stiffness; magnetic resonance imaging (MRI) has also been proposed to study these patients. PURPOSE: To correlate US, SWE, and MRI imaging findings with medical outcome in patients with BA who are native liver survivors after KP. MATERIAL AND METHODS: We retrospectively enrolled 24 patients. They were divided in two groups based on "ideal" (n = 15) or "non-ideal" (n = 9) medical outcome. US, SWE, and MRI exams were analyzed qualitatively and quantitatively for imaging signs suggestive of chronic liver disease (CLD). RESULTS: Significant differences were found in terms of liver surface (P = 0.007) and morphology (P = 0.013), portal vein diameter (P = 0.012) and spleen size (P = 0.002) by US, liver signal intensity (P = 0.013), portal vein diameter (P = 0.010), presence of portosystemic collaterals (P = 0.042), and spleen size (P = 0.001) by MRI. The evaluation of portal vein diameter (moderate, κ = 0.44), of portosystemic collaterals (good, κ = 0.78), and spleen size (very good, κ = 0.92) showed the best agreement between US and MRI. A significant (P = 0.01) difference in liver parenchyma stiffness by SWE was also found between the two groups (cut-off = 9.6 kPa, sensitivity = 55.6%, specificity = 100%, area under the ROC curve = 0.82). CONCLUSION: US, SWE, and MRI findings correlate with the medical outcome in native liver survivor patients with BA treated with KP.

Comparison Between Multidetector CT and High-Field 3T MR Imaging in Diagnostic and Tumour Extension Evaluation of Patients with Cholangiocarcinoma.

PURPOSE: To compare multidetector computed tomography (MDCT) and magnetic resonance imaging (MRI) with cholangiopancreatography (MRCP) findings in the diagnostic evaluation of patients with cholangiocarcinoma (CCA) to establish tumour resectability. METHODS: Thirty patients (22 M, 8 F) with pathologically proven CCA by post-surgical specimens (n = 20), core biopsy (n = 6) or cytology (n = 4) underwent both MDCT and MRI with MRCP. CCA lesions were classified on the basis of anatomical locations in intra-hepatic (iCCA), peri-hilar (pCCA) and distal (dCCA) tumours. Morphological tumour pattern, lesion size, biliary dilatation, tumour contrast enhancement type, lymph node involvement and vascular infiltration were directly compared between MDCT and MRI with MRCP. As a rule, a tumour resectability judgement for each patient was formulated by both imaging techniques comparing imaging results with direct surgical assessment (n = 20) or interventional procedures (n = 10). RESULTS: In terms of anatomical location, 14 iCCA, 8 pCCA and 8 dCCA were observed; both imaging techniques were concordant about the identification and morphological characterization of tumour lesions and in the evaluation of tumour features (lesion size, contrast enhancement pattern, capsular retraction, biliary dilatation, lymph node involvement and vascular infiltration) as well as in assessing lesion resectability; an excellent agreement (k = 1) for the assessment of all the parameters included in imaging analysis was observed. CONCLUSIONS: The comparative concordant results of our study suggest that MRI with MRCP represents a valid alternative to MDCT for the diagnostic evaluation of patients with CCA to establish tumour resectability providing multiplanar scanning of high-contrast imaging quality; MDCT should be preferred in uncooperative patients, in the presence of biliary stents or when MRI is absolutely contraindicated for incompatible medical devices.

Case report: horse or zebra, ascites or pseudo-ascites? Care for pictural details!

BACKGROUND: Pseudo-ascites is a very rare condition in children and remains a challenging diagnosis. Targeted imaging may be helpful, but a high index of clinical suspicion is often necessary to guide the investigations, as pseudo-ascites may efficiently mimic true ascites. To date, still many cases of pseudo-ascites suffer diagnostic and therapeutic delay, and some are only diagnosed during surgical exploration. We report the case of a patient with a late laparoscopic diagnosis of pseudo-ascites. We retrospectively review our patient's imaging findings and suggest new characteristic features which may help differentiate pseudo-ascites from true ascites. CASE PRESENTATION: A 7-month-old infant was referred for a progressive abdominal distention. Physical examination and initial ultra-sonographic findings evoked free ascites. An extensive diagnostic workup was then performed and was negative for hepatic, renal, cardiac, intestinal, pancreatic, inflammatory or infectious diseases, malignancy and congenital metabolic disorders. Pseudo-ascites was evoked and dedicated ultra-sonographic and magnetic resonance studies were repeated but could not confirm this diagnosis. Symptomatic diuretic treatment with spironolactone and furosemide was then started. A temporary and limited effect was noted but, with time, repeated paracenteses were necessary as the abdominal distention progressed causing discomfort and breathing difficulty. Last, because the patient's quality of life deteriorated, a peritoneal-venous shunting was proposed; as the operation started with a diagnostic laparoscopy, a benign giant cystic mesenteric lymphangioma was identified and totally excised. The resolution of symptoms was immediate and the patient remained symptom-free throughout the subsequent observation period that lasted more than 1 year. CONCLUSIONS: Increased awareness about pseudo-ascites is necessary, as the diagnosis is often overlooked, and treatment delayed. Targeted imaging may be helpful, as some specific, although not pathognomonic, features exist which may aid in the diagnosis.

Clinical benefits, echocardiographic and MRI assessment after pulmonary sequestration treatment.

BACKGROUND: This study aimed to explore systematically procedural results, clinical benefits with echocardiographic and chest-MRI assessment of pulmonary sequestration percutaneous treatment. METHODS: 13 consecutive infants and children with diagnosis of isolated pulmonary sequestration (PS) had percutaneous closure of the aberrant artery supplying pulmonary sequestration between 2010 and 2015. By protocol, echocardiographic and chest-MRI assessment was performed before and respectively at 6-12months and 1year with the aim to study the effects of embolization on heart volume overload and regression of pulmonary sequestration. RESULTS: Median age at diagnosis was 1year (95%CI 0-2.6); median age at treatment was 1.3years (95%CI1.01-2.85). In all pts the PS was confirmed by chest-MRI. Procedural success was 100%. After treatment, pts experiencing previously respiratory symptoms/infections remained asymptomatic at 2.9year follow-up. In pts with significant shunt due to PS, treatment resulted in amelioration in left or right cardiac chamber enlargement at 6 and 12month follow-up. At distance from PS closure (median 14months), chest-MRI confirmed the closure of the aberrant artery and PS regression in 12 patients. In one case, despite the acute procedural success and the supplying artery remained closed, MRI detected residual PS revascularization. CONCLUSIONS: Percutaneous PS closure in infants and children is safe and provide regression in respiratory symptoms and heart chamber dilatation if significant shunt is present. MRI is able to define aberrant artery course and PS parenchima, and might represent a valid instrument to study residual PS parenchima during growth.

Lung structure and function similarities between primary ciliary dyskinesia and mild cystic fibrosis: a pilot study.

BACKGROUND: Primary ciliary dyskinesia (PCD) and cystic fibrosis (CF) are increasingly compared. There are no chest magnetic resonance imaging (MRI) comparative studies of PCD and CF. We assessed clinical, functional, microbiological and MRI findings in PCD and mild CF patients in order to evaluate different expression of lung disease. METHODS: Twenty PCD (15.1 years) and 20 CF subjects with mild respiratory impairment (16 years, 70% with pancreatic insufficiency) underwent MRI, spirometry, and sputum cultures when clinically stable. MRI was scored using the modified Helbich system. RESULTS: PCD was diagnosed later than CF (9.9 versus 0.6 years, p = 0.03), despite earlier symptoms (0.1 versus 0.6 years, p = 0.02). In the year preceding the study, patients from both groups underwent two systemic antibiotic courses (p = 0.48). MRI total scores were 11.6 ± 0.7 and 9.1 ± 1 in PCD and CF, respectively. FEV1 and FVC Z-scores were -1.75 (range, -4.6-0.7) and -0.6 (-3.9-1.8) in PCD, and -0.9 (range, -5.4-2.3) and -0.3 (-3.4-2.5) in CF, respectively. No difference was found between lung function or structure, despite a higher MRI subscore of collapse/consolidation in PCD versus CF (1.6 ± 0.1 and 0.6 ± 0.2, p < 0.001). These findings were confirmed after data-control for diagnostic delay. Pseudomonas aeruginosa and Staphylococcus aureus were more frequent in CF than in PCD (p = 0.05 and p = 0.003, respectively). CONCLUSIONS: MRI is a valuable radiation-free tool for comparative PCD and CF lung disease assessment. Patients with PCD may exhibit similar MRI and lung function changes as CF subjects with mild pulmonary disease. Delay in PCD diagnosis is unlikely the only determinant of similarities.

Nuclear imaging to characterize adrenal tumors: Comparison with MRI.

AIM: To describe the role of nuclear imaging modalities using nor-cholesterol, metaiodobenzylguanidine (MIBG) and fluorine-deoxy-glucose (FDG) in adrenal tumors for lesion characterization in comparison with magnetic resonance (MR). METHODS: Population was classified in group 1 consisting of 30 patients with non-hypersecreting unilateral adrenal masses, in group 2 consisting of 34 patients with hypersecreting (n = 19) or non-hypersecreting (n = 15) adrenal adenomas and in group 3 consisting of 18 patients with chromaffin-tissue tumors (CTT), of which 14 were pheochromocytomas while 4 were paragangliomas (n = 4). All patients underwent MR and nuclear studies (nor-cholesterol, MIBG and FDG). Pathology samples (n = 63) or follow-up data in adenomas (n = 19) were used as standard of reference for imaging studies interpretation. RESULTS: In group 1, MR findings were not highly accurate for lesion characterization, while the results of nuclear scans showed abnormal nor-cholesterol, MIBG and FDG concentration in all cases of adenomas, pheos and malignant tumors, respectively. In group 2, no differences in MR parameters were found between hyperfunctioning and non-hyperfunctioning adenomas, while nor-cholesterol uptake was significantly higher in hyperfunctioning compared to non-hyperfunctioning lesions. In group 3, no differences in MR parameters were found between benign and malignant CCT, while MIBG uptake was significantly higher in malignant compared to benign tumors. CONCLUSION: On the basis of our findings, nuclear imaging modalities using specific target agents are able to better characterize adrenal tumors, compared with MR. In particular, radionuclide techniques are able to identify the nature of adrenal incidentalomas and to differentiate between hypersecreting and non-hypersecreting adenomas as well as between benign and malignant CTT.

A chaperone enhances blood α-glucosidase activity in Pompe disease patients treated with enzyme replacement therapy.

Enzyme replacement therapy is currently the only approved treatment for Pompe disease, due to acid α-glucosidase deficiency. Clinical efficacy of this approach is variable, and more effective therapies are needed. We showed in preclinical studies that chaperones stabilize the recombinant enzyme used for enzyme replacement therapy. Here, we evaluated the effects of a combination of enzyme therapy and a chaperone on α-glucosidase activity in Pompe disease patients. α-Glucosidase activity was analyzed by tandem-mass spectrometry in dried blood spots from patients treated with enzyme replacement therapy, either alone or in combination with the chaperone N-butyldeoxynojirimycin given at the time of the enzyme infusion. Thirteen patients with different presentations (3 infantile-onset, 10 late-onset) were enrolled. In 11 patients, the combination treatment resulted in α-glucosidase activities greater than 1.85-fold the activities with enzyme replacement therapy alone. In the whole patient population, α-glucosidase activity was significantly increased at 12 hours (2.19-fold, P = 0.002), 24 hours (6.07-fold, P = 0.001), and 36 hours (3.95-fold, P = 0.003). The areas under the curve were also significantly increased (6.78-fold, P = 0.002). These results suggest improved stability of recombinant α-glucosidase in blood in the presence of the chaperone.

Diagnostic accuracy of MR imaging to identify and characterize focal liver lesions: comparison between gadolinium and superparamagnetic iron oxide contrast media.

To compare the diagnostic value of gadolinium (Gd) and ultrasmall superparamagnetic iron oxide (SPIO) contrast media for characterization of focal liver lesions (FLL), we retrospectively evaluated the results of magnetic resonance (MR) imaging in 68 patients (40 M, 28 F, age from 22 to 81 yrs) of which 36 with diagnosis of colo-rectal cancer, 26 with hepatic cirrhosis and 6 with incidental imaging detection of FLL. MR (Gyroscan Intera 1.5 T, Philips Medical Systems) study was performed using T1 and T2 fast-field-echo (FFE) and T2 turbo-spin-echo (TSE) sequences in axial and coronal views. Dynamic multi-phases gadolinium Gd-enhanced T1-FFE-Bh images were obtained in arterial, portal and equilibrium phases, followed by SPIO-enhanced T2-FFE scans. A qualitative analysis of pre- and post-contrast MR images to classify FLL as benign or malignant was performed using a 3-point scoring system: 0= benign; 1= suspicious for malignancy; 2= malignant. A total of 118 lesions were evaluated. In particular, histology (n=18), cytology (n=14) or clinical-imaging follow-up data (n=86) demonstrated 4 adenomas, 29 cysts, 3 focal steatosis, 25 hemangiomas, 1 focal vascular abnormality, 5 fibrotic lesions as well as 13 regenerative nodules, 6 dysplastic, 14 hepatocellular carcinomas (HCC), 17 metastasis and 1 cholangiocarcinoma. For MR imaging, diagnostic accuracy, sensitivity, specificity, positive (PPV) and negative (NPV) predictive values of Gd vs. SPIO images were respectively 83% vs. 92%, 79% vs. 74%, 85% vs. 99% (P=0.002), 68% vs. 96% (P=0.005) and 91% vs. 90%, respectively. The results suggest that SPIO-MR provides a diagnostic incremental value, as specificity and PPV, particularly to characterize FLL compared to Gd-MR; thus, we strongly recommend the use of SPIO when liver lesion characterization is requested and Gd images are uncertain.

Sleep disordered breathing and airway disease in primary ciliary dyskinesia.

BACKGROUND AND OBJECTIVE: Sleep-disordered breathing (SDB) may develop in primary ciliary dyskinesia (PCD), leading to these diseases worsening one another. METHODS: Sixteen stable PCD patients (4.9-17.2 years) and 42 controls underwent overnight respiratory polysomnography (rPSG) and Sleep Disturbances Scale for Children (SDSC). In PCD we assessed nasal endoscopy, pulmonary function tests and chest high-resolution computed tomography (HRCT). RESULTS: Compared with controls, PCD had higher obstructive apnoea (4.7 vs 0.2, P < 0.001), central apnoea (0.8 vs 0.2, P < 0.001), hypopnoea (1.8 vs 0.2, P < 0.001), apnoea-hypopnoea (7.8 vs 0.6, P < 0.001), oxygen desaturation indexes (ODI; 0.7 vs 0.2, P = 0.002), and mean oxygen desaturation (4% vs 1%, P < 0.001), while mean and nadir oxygen saturation (97.1% vs 98.1, P < 0.001) (93% vs 97.2%, P < 0.001) were lower, respectively. In PCD, SDSC was unrelated to rPSG (P > 0.05), with total score and subscores of disorders in initiating and maintaining sleep, and sleep-wake transition lower than controls. PCD patients had chronic rhinosinusitis (100%) and adenoidal hypertrophy (50%). Total HRCT score was 7 (range 0-14). ODI correlated with functional residual capacity (r = 0.8, P = 0.02), total HRCT (r = 0.6, P = 0.03) and peribronchial thickening scores (r = 0.7, P = 0.02). Oxygen saturation was associated with bronchiectasis severity score (r = -0.6, P = 0.02). CONCLUSIONS: PCD's parents may underestimate SDB. As nocturnal desaturation is associated with lung function and structure abnormalities, SDB may significantly contribute to pulmonary morbidity.

Supratentorial and infratentorial damage in spinocerebellar ataxia 2: a diffusion-weighted MRI study.

Spinocerebellar ataxia type 2 (SCA2) is an autosomal-dominant degenerative disorder that is neuropathologically characterized primarily by infratentorial damage, although less severe supratentorial involvement may contribute to the clinical manifestation. Diffusion-weighted imaging (DWI)-Magnetic Resonance Imaging (MRI) studies of SCA2 have enabled in vivo quantification of neurodegeneration in infratentorial regions, whereas supratentorial regions have been explored less thoroughly. We measured microstructural changes in both infratentorial and supratentorial regions in 13 SCA2 patients (9 men, 4 women; mean age, 50 ± 12 years) and 15 controls (10 men, 5 women; mean age, 49 ± 14 years) using DWI-MRI and correlated the DWI changes with disease severity and duration. Disease severity was evaluated using the International Cooperative Ataxia Rating Scale and the Inherited Ataxia Clinical Rating Scale. Cerebral diffusion trace ( D¯) values were generated, and regions of interest (ROIs) and voxel-based analysis with Statistical Parametric Mapping (SPM) were used for data analysis. In SCA2 patients, ROI analysis and SPM confirmed significant increases in D¯ values in the pons, cerebellar white matter (CWM) and middle cerebellar peduncles. Moreover, SPM analysis revealed increased D¯ values in the right thalamus, bilateral temporal cortex/white matter, and motor cortex/pyramidal tract regions. Increased diffusivity in the frontal white matter (FWM) and the CWM was significantly correlated with ataxia severity. DWI-MRI revealed that both infratentorial and supratentorial microstructural changes may characterize SCA2 patients in the course of the disease and might contribute to the severity of the symptoms.

Non invasive assessment of lung disease in ataxia telangiectasia by high-field magnetic resonance imaging.

PURPOSE: A sensitive imaging technique that assesses ataxia telangiectasia (AT) lung disease without ionizing radiation is highly desirable. We designed a study to evaluate lung changes using magnetic resonance imaging (MRI), and to investigate the relationships among severity and extent of pulmonary abnormalities and clinical, microbiological and functional data in children and young adults with AT. METHODS: Fifteen AT patients (age, 11.3 years; range, 6-31) underwent 3.0-T MRI, spirometry, and deep throat or sputum culture. Images were scored using a modified Helbich score. RESULTS: Although only 8 patients (53 %) had recurrent/chronic respiratory symptoms, MRI identified lung abnormalities in all. Bronchiectasis, peribronchial thickening, mucous plugging, and collapse/consolidation were present in 60 %, 87 %, 67 %, and 13 % of cases, respectively, with no difference between subjects with or without respiratory symptoms. No difference in changes of specific scores was found between the two groups, but the total MRI score was higher in patients with respiratory symptoms (6.5 versus 5, respectively; p = 0.02). Total or specific MRI scores were not associated with patients' age. Of all scores, only mucous plugging subscore appeared significantly related to FEV1 (r = 0.7, p = 0.04) and FEF25-75% (r = 0.9, p = 0.001). MRI scores from patients with positive (n = 5) or negative (n = 10) sputum culture were not significantly different. CONCLUSIONS: MRI is valuable in the assessment of extent and severity of pulmonary changes in children and adults with AT. It represents an helpful tool for the longitudinal evaluation of patients and may be also used as an outcome surrogate to track the effects of medications.

Magnetic resonance imaging is an accurate and reliable method to evaluate non-cystic fibrosis paediatric lung disease.

BACKGROUND AND OBJECTIVE: Chest MRI is increasingly used to assess pulmonary diseases, but its utility compared with high-resolution computed tomography (HRCT) has never been evaluated in children using specific performance outcomes. The aim of this study was to assess the accuracy and reliability of MRI compared with HRCT in children with non-cystic fibrosis (CF) chronic lung disease. METHODS: Fifty subjects aged 5.9-20 years, with primary ciliary dyskinesia (n = 17), primary immunodeficiency (n = 17) or recurrent pneumonia (n = 16), underwent chest HRCT and MRI. The prevalence of lung abnormalities on HRCT was evaluated, and sensitivity, specificity, accuracy and positive and negative likelihood ratios for MRI versus HRCT were calculated. MRI and HRCT scans were also assessed using a modified Helbich score. RESULTS: Bronchiectasis, mucous plugging, peribronchial wall thickening, consolidation, bullae, abscesses and emphysema were detected by HRCT in 72, 68, 66, 60, 10, 8 and 8% of subjects, respectively. Sensitivity, specificity, accuracy and positive and negative likelihood ratios for MRI were good or excellent for most of the changes that were assessed. Median total Helbich scores for HRCT and MRI were 10 (range 0-20) and 10 (range 0-18), respectively. There was good-to-excellent agreement between the two techniques for all scores (r ≥ 0.8). A Bland-Altman plot confirmed this agreement between total scores (bias value: 0.2 ± 1.18; 95% limits of agreement of mean difference: -2.12-2.52). CONCLUSIONS: Chest MRI was equivalent to HRCT to determine the extent of lung disease in children with non-CF lung disease. The findings support the use of chest MRI as an alternative to HRCT in diagnostic pathways for paediatric chronic lung disorders.

Progression of lung disease in primary ciliary dyskinesia: is spirometry less accurate than CT?

Despite its extensive use, there is no evidence that spirometry is useful in the assessment of progression of lung disease in primary ciliary dyskinesia (PCD). We hypothesize that high-resolution computed tomography (HRCT) is a better indicator of PCD lung disease progression than spirometry. We retrospectively evaluated two paired spirometry and HRCT examinations from 20 PCD patients (age, 11.6 years; range, 6.5-27.5 years). The evaluations were performed in stable state and during unstable lung disease. HRCT scans were scored blind by two raters. Compared to the first assessment, at the second evaluation spirometry did not change while HRCT scores significantly worsened (P < 0.01). Age was significantly related to HRCT total (r = 0.5; P = 0.02) and bronchiectasis scores (r = 0.5; P = 0.02). At both evaluations, HRCT total score correlated with FEV(1) (r = -0.5, P = 0.01; r = -0.7, P = 0.001, respectively) and FVC Z scores (r = -0.6, P = 0.006; r = -0.7, P = 0.001, respectively), and bronchiectasis score was related to FEV(1) (r = -0.5, P = 0.03; r = -0.6; P = 0.002, respectively) and FVC Z scores (r = -0.6, P = 0.008; r = -0.7, P = 0.001, respectively). No relationship was found between the change in HRCT scores and the change in spirometry. In PCD, structural lung disease may worsen despite spirometry being stable.