Identification of fractures on pediatric foot radiographs: do localization cues improve diagnostic accuracy and reduce interpretation time?

OBJECTIVE: To investigate the diagnostic accuracy and time in the detection of fractures on pediatric foot radiographs marked without and with localization cues. METHOD: One-hundred randomly selected foot radiographic examinations that were performed on children (<18 years old) after injury and with at least 4 weeks of follow-up were included. Blinded to history and diagnosis, 4 readers (one each: medical student, pediatrician, pediatric orthopedic surgeon, and pediatric musculoskeletal radiologist) retrospectively and independently reviewed each examination twice (without and with cue, at least 1 month apart, and after randomization). Each reader recorded the presence or absence of a fracture, fracture location, diagnostic confidence, and the total (interpretation) time spent on each study. Diagnostic accuracy, reader confidence, and interpretation time were compared between examinations without and with cues. RESULTS: Our study included 59 examinations without and 41 with fractures (21 phalangeal, 18 metatarsal, and 2 tarsal fractures). Localization cues improved inter-reader agreement (κ=0.36 to 0.64), overall sensitivity (68 to 72%), specificity (66 to 73%), and diagnostic accuracy (67 to 73%); thus, overcalled and missed rates also improved from 34 to 27% and 32 to 28%, respectively. Reader confidence improved with cue (49 to 61%, p<0.01) with higher incremental improvement with younger children (30% for 1-6 years; 14% for 7-11 years; and 10% for 12-17 years). Interpretation time decreased by 40% per examination (40±22 s without to 24±13 s with cues, p<0.001). CONCLUSION: Localization cues improved diagnostic accuracy and reader confidence, reducing interpretation time in the detection of pediatric foot fractures.

MRI features of spinal chronic recurrent multifocal osteomyelitis/chronic non-bacterial osteomyelitis in children.

BACKGROUND: Spinal lesions in pediatric chronic recurrent multifocal osteomyelitis/chronic non-bacterial osteomyelitis (CRMO/CNO) can cause permanent sequelae; thus, early recognition of these is vital for management. OBJECTIVE: To characterize the MR imaging features and patterns of pediatric spinal CRMO/CNO. MATERIALS AND METHODS: This cross-section study received IRB approval. The first available MRI with documented spine involvement in children with CRMO/CNO was reviewed by a pediatric radiologist. Descriptive statistics were used to describe the characteristics of vertebral lesions, disc involvement, and soft tissue abnormality. RESULTS: Forty-two patients were included (F:M, 30:12); median age was 10 years (range 4-17). At diagnosis, 34/42 (81%) had spine involvement. Kyphosis in 9/42 (21%) and scoliosis in 4/42 (9.5%) patients were present at the time of spinal disease recognition. Vertebral involvement was multifocal in 25/42 (59.5%). Disc involvement was found in 11/42 (26%) patients, commonly in the thoracic spine and often with adjacent vertebrae height loss. Posterior element abnormalities were present in 18/42 patients (43%) and soft tissue involvement in 7/42 (17%). One hundred nineteen vertebrae were affected, commonly the thoracic vertebrae (69/119; 58%). Vertebral body edema was focal in 77/119 (65%) and frequently superior (42/77; 54%). Sclerosis and endplate abnormality were present in 15/119 (13%) and 31/119 (26%) vertebrae, respectively. Height loss was present in 41/119 (34%). CONCLUSION: Chronic non-bacterial osteomyelitis of spine is usually thoracic. Vertebral body edema is often focal at the superior vertebral body. Kyphosis and scoliosis occur in a quarter and vertebral height loss in a third of children at spinal disease recognition.

MRI findings of growth plate fractures of the knee: are there age- and fracture-dependent differences?

OBJECTIVE: To investigate MRI findings in children with physeal fractures of the knee with respect to age, location, and articular involvement. METHODS: Children with physeal fractures who underwent knee MRI between 2008 and 2021 were included. Two radiologists retrospectively reviewed all examinations to determine articular involvement, findings of physeal instability (perichondral disruption, periosteal entrapment), and internal derangement (cruciate ligament injury, meniscal tear, chondromalacia). Independent samples t, Mann-Whitney U, Pearson's chi-square, and Fisher's exact tests were used to compare findings. RESULTS: Fifty-six patients (37 boys, 19 girls; mean age: 12.2 ± 2.5 years; 32 distal femur, 24 proximal tibial fractures) included 24(43%) intraarticular fractures. Fractures were more common in the tibia than the femur (67% versus 25%, p = 0.004) and intraarticular fractures were more common in older than younger children (13.1 ± 2.0 versus 11.5 ± 2.7 years, p = 0.01), to associate with chondromalacia (46% versus 12%, p = 0.02) and undergo surgery (33% versus 10%, p = 0.04) when compared to extraarticular fractures. Perichondral disruption (n = 44, 79%) and periosteal entrapment (n = 13, 23%) did not significantly differ based on location or articular involvement (p > 0.05). At a median follow-up of 17.5 months (interquartile range: 1.25-34), 3 patients (2 intraarticular, 1 extraarticular fractures) developed osteoarthritis, osteochondral lesion, and leg-length discrepancy from growth arrest, which required additional surgery. CONCLUSION: Intraarticular physeal fractures were more common with older children, associate with chondromalacia, and underdo surgical intervention when compared to extraarticular fractures of the knee. While MRI findings of physeal instability were common, no significant differences were found between fractures based on anatomic location or fracture pattern.

Pediatric hand fractures detection on radiographs: do localization cues improve diagnostic performance?

OBJECTIVE: To compare the diagnostic accuracy and interpretation time for detection of pediatric fractures on hand radiographs with and without localization cues. MATERIALS AND METHODS: Consecutive children, who underwent radiographic examinations after injury, over 2 years (2019-2021) and with > 2 weeks of follow-up to confirm the presence or absence of a fracture, were included. Four readers, blinded to history and diagnosis, retrospectively reviewed all images twice, without and with cue, at least 1 week apart and after randomization, to determine the presence or absence of a fracture, and if present, anatomic location and diagnostic confidence were recorded. Interpretation time for each study was also recorded and averaged across readers. Inter-reader agreement was calculated using Fleiss' kappa. Diagnostic accuracy and interpretation time were compared between examinations using sensitivity, specificity, and Mann-Whitney U correlation. RESULTS: Study group included 92 children (61 boys, 31 girls; 10.8 ± 3.4 years) with and 40 (31 boys, 9 girls; 10.9 ± 3.7 years) without fractures. Cue improved inter-reader agreement (κ = 0.47 to 0.62). While the specificity decreased (63 to 62%), sensitivity (75 to 78%), diagnostic accuracy (71 to 73%), and confidence improved (78 to 87%, p < 0.01), and interpretation time (median: 40 to 22 s, p < 0.001) reduced with examinations with localization cue. Specifically, examinations with fracture and cue had the shortest interpretation time (median: 16 s), whereas examinations without fracture and without cue had the longest interpretation time (median: 48 s). CONCLUSION: Localization cues increased inter-reader agreement and diagnostic confidence, reduced interpretation time in the detection of fractures on pediatric hand radiographs, while maintaining diagnostic accuracy.

Distribution of pediatric hand fractures: age- and bone maturation-dependent differences.

PURPOSE: To investigate the distribution and characteristics of fractures of bones of the hand on radiographs with respect to age and skeletal maturity of the fractured bone, and to identify predictors of surgery. METHODS: This cross-sectional, retrospective study included children (≤ 18 years) with hand fractures who underwent radiographic examinations (2019-2021). Fracture location, presence of displacement (≥ 2 mm), angulation (≥ 10°), articular extension, and if skeletally immature, then physeal involvement and Salter-Harris grade were recorded. Mann-Whitney U, Kruskal-Wallis, Fisher's exact, and chi-square tests as well as logistic regression analyses were used. RESULTS: Study group of 508 (350 boys, 158 girls; median age, 11.9 years) included 575 (63% phalangeal, 37% metacarpal, and 0.3% carpal) fractures. Younger children were more likely to sustain phalangeal and older children carpal and metacarpal fractures (median ages: 10.8 vs 12.3 and 13.8 years, p < 0.001); and fractures of the small finger accounted for 50% of metacarpal and 43% of phalangeal fractures. Fracture displacement (12% vs 22%, p = 0.02) and angulation (25% vs 49%, p < 0.001) were more common with mature than immature bones. A third of immature bones had physeal involvement and the most common pattern was Salter-Harris type II (89%). Surgical intervention was uncommon (11%) and independent predictors were displacement (OR = 3.99, 95% CI 1.95-8.19, p < 0.001) and articular extension (OR = 5.11, 95% CI 2.00-13.07, p < 0.001). CONCLUSION: While younger children were more likely to sustain phalangeal than metacarpal fractures and less likely to have displacement and angulation when compared to older children; only displacement and articular extension were significant independent predictors of surgery.

Pediatric Osteosarcoma: Correlation of Imaging Findings with Histopathologic Features, Treatment, and Outcome.

Osteosarcoma is the most common primary bone sarcoma in children. Imaging plays a pivotal role in diagnostic workup, surgical planning, and follow-up monitoring for possible disease relapse. Survival depends on multiple factors, including presence or absence of metastatic disease, chemotherapy response, and surgical margins. At diagnosis, radiography and anatomic MRI are used to characterize the primary site of disease, whereas chest CT and whole-body bone scintigraphy and/or PET are used to identify additional sites of disease. Treatment starts with neoadjuvant chemotherapy, followed by en bloc tumor resection and limb reconstruction, and finally, adjuvant chemotherapy. Preoperative planning requires precise tumor delineation, which traditionally has been based on high-spatial-resolution anatomic MRI to identify tumor margins (medullary and extraosseous), skip lesions, neurovascular involvement, and joint invasion. These findings direct the surgical approach and affect the options for reconstruction. For skeletally immature children, the risk of cumulative limb-length discrepancy and need for superior longevity of the reconstruction have led to the advent and preferential use of several pediatric-specific surgical techniques, including rotationplasty, joint preservation surgery, autograft or allograft reconstruction, and extendible endoprostheses. A better understanding of the clinically impactful imaging features can directly and positively influence patient care. Online supplemental material is available for this article. ©RSNA, 2022.

MRI evaluation of pediatric tibial eminence fractures: comparison between conventional and "CT-like" ultrashort echo time (UTE) images.

OBJECTIVE: UTE MRI offers a radiation-free alternative to CT for bone depiction, but data on children is lacking. The purpose of this study was to determine whether UTE images improve detection and characterization of pediatric tibial eminence fractures. METHODS: Fifteen MRIs with UTE from 12 children (10 boys, 2 girls; mean age: 12.6 ± 3.3 years) with tibial eminence fractures (2018-2020) and 15 age-matched MRIs without fractures were included. After randomization, 5 readers reviewed images without and with UTE, at least 1 month apart, and recorded the presence of fracture and preferred images. If fracture is present, radiologists also recorded fragment size, number, and displacement; surgeons assigned Meyers-McKeever grade and management. Disagreements on management were resolved through consensus review. Kappa and intra-class correlation (ICC), sensitivity, and specificity were used to compare agreement between readers and fracture detection between images without and with UTE. RESULTS: For fracture detection, inter-reader agreement was almost perfect (κ-range: 0.91-0.93); sensitivity and specificity were equivalent between images without and with UTE (range: 95-100%). For fracture characterization, UTE improved agreement on size (ICC = 0.88 to 0.93), number (ICC = 0.52 to 0.94), displacement (ICC = 0.74 to 0.86), and grade (ICC = 0.92 to 0.93) but reduced agreement on management (κ = 0.68 to 0.61), leading to a change in consensus management in 20% (3/15). Radiologists were more likely to prefer UTE for fracture and conventional images for non-fracture cases (77% and 77%, respectively, p < 0.001). CONCLUSION: While UTE did not improve diagnosis, it improved agreement on characterization of pediatric tibial eminence fractures, ultimately changing the preferred treatment in 20%.

Pre-treatment MRI of leukaemia and lymphoma in children: are there differences in marrow replacement patterns on T1-weighted images?

OBJECTIVES: To investigate the prevalence and distribution of specific marrow patterns on pre-treatment magnetic resonance imaging (MRI) examinations in children with leukaemia and lymphoma and with respect to the anatomic location. MATERIALS AND METHODS: This retrospective IRB-approved and HIPAA-compliant study included children with leukaemia or lymphoma who underwent pre-treatment MRI examinations over 18 years (between 1 January 1995 and 31 August 2013). Two radiologists blinded to the clinical diagnosis reviewed each study to determine the presence or absence of abnormal marrow signal and, when present, sub-categorised the pattern into diffuse, patchy, or focal abnormal marrow. Chi-square and Fisher's exact tests were used to compare marrow patterns between leukaemia and lymphoma. RESULTS: The study included 50 children (32 males and 18 females; mean age 9.5 ± 5.3 years) with 54 MRI examinations (27 leukaemia and 27 lymphoma) that included 26 spine and 28 non-spine studies. Marrow replacement was present on 43 (80%) studies, significantly more common with leukaemia than with lymphoma (p = 0.039). The diffuse replacement pattern was significantly more common with leukaemia when compared to lymphoma (p < 0.001) and the focal pattern was only observed with lymphoma. In the spine, the diffuse pattern was observed with lymphoma (3/14, 21%). All patients with leukaemia and MRI outside of the spine showed marrow involvement. CONCLUSION: Marrow replacement is common on MRI from children with leukaemia and lymphoma. A diffuse pattern was significantly associated with leukaemia on studies outside of the spine and a focal pattern was only observed with lymphoma, independently of the anatomic location. KEY POINTS: • Bone marrow replacement on pre-treatment MRI examinations in children with leukaemia and lymphoma was observed in 93% (25/27) and 67% (18/27), respectively. • Diffuse pattern of marrow replacement was significantly more common in leukaemia even though this pattern was also observed with lymphoma on the spine MRI studies. • Focal pattern of marrow replacement was present only with lymphoma and not with leukaemia regardless of the anatomic location.

Lateral Meniscus Posterior Root Injury: MRI Findings in Children With Anterior Cruciate Ligament Tear.

BACKGROUND. Undiagnosed and unrepaired root tears are increasingly recognized as a preventable cause of accelerated osteoarthritis. Preoperative MRI findings of lateral meniscus posterior root tears in children with concomitant anterior cruciate ligament (ACL) injury are not well described. OBJECTIVE. The purpose of this study was to investigate the performance of preoperative MRI for identifying concomitant lateral meniscus posterior root injuries in pediatric patients with ACL tears with arthroscopy as the reference standard. METHODS. Consecutively registered children who underwent MRI within 90 days before arthroscopic primary ACL reconstruction between March 2017 and December 2019 were included. Two radiologists assessed MRI examinations for direct signs involving the root proper and for findings associated with lateral meniscus posterior root tears. Kappa coefficients for MRI findings were computed. Findings in patients with root tears and intact roots were compared by independent-samples t test, Mann-Whitney U test, chi-square test, Fisher exact test, and multivariable logistic regression analysis. RESULTS. At arthroscopy, 39 children (18 boys, 21 girls; mean age, 15.2 ± 1.4 years) had lateral meniscus posterior root tears; 51 (22 boys, 29 girls; mean age, 15.7 ± 1.8 years) had intact roots. Kappa coefficients ranged from 0.65 to 0.92, aside from tears involving the entheseal segment (κ = 0.55) or popliteomeniscal fascicles (κ = 0.45). MRI findings that were predictors of arthroscopically diagnosed root tear (p < .05) were lateral meniscus root tear in any segment (odds ratio [OR], 16.8; 95% CI, 5.6-50.1), degeneration in any segment (OR, 3.9; 95% CI, 1.6-9.6), coronal cleft sign (OR, 5.7; 95% CI, 2.0-16.7), sagittal ghost sign (OR, 4.8; 95% CI, 1.2-19.1), and axial radial defect sign (OR, 7.1; 95% CI, 2.4-20.5). Tear involving any segment of the root proper had the highest PPV, 82%, with 79% NPV. The coronal cleft, sagittal ghost, and axial radial defect signs had specificities of 88%, 94%, and 88% but sensitivities of 44%, 23%, and 49%. The only significant independent predictor on preoperative MRI was root tear in any segment (OR, 15.8; 95% CI, 2.7-137.5; p = .003). CONCLUSION. Among MRI findings evaluated for preoperative diagnosis of lateral meniscus posterior root tear, tear involving any segment of the root proper had the strongest performance; associated findings had high specificity but low sensitivity. CLINICAL IMPACT. Accurate identification of lateral meniscus posterior root tears on preoperative MRI can aid in operative planning and reduce treatment delay.

Radiographic characterization of acute scaphoid fractures in children under 11 years of age.

BACKGROUND: Delayed diagnosis of scaphoid fractures can lead to long-term morbidity. While radiography is the preferred screening examination, there is a relative paucity of literature that examines fracture visibility in younger children, who have smaller ossification centers, an abundance of unossified cartilage and fractures that preferentially involve the distal scaphoid. OBJECTIVE: To characterize acute scaphoid fractures in younger children on radiographs with observer agreement and with respect to fracture location. MATERIALS AND METHODS: This institutional review board (IRB)-approved and Health Insurance Portability and Accountability Act (HIPAA)-compliant cross-sectional study included children (≤10 years of age) with acute scaphoid fractures (≤7 days), who underwent radiographic examinations at a tertiary children's hospital between December 2008 and June 2019. Three readers (two pediatric radiologists and one orthopedic surgeon) reviewed each examination to determine fracture visibility on each radiographic view and fracture location. Kruskal-Wallis, Fisher exact and Cochran-Armitage tests were used to compare fracture visibility and location, and Kappa tests were used to calculate observer agreement. RESULTS: Twenty-eight children (15 boys, 13 girls; mean age: 9.5±0.6 years) with 10 (36%) distal corner, 11 (39%) distal body and 7 (25%) mid-body fractures, underwent 7 (25%) 4-view, 18 (64%) 3-view and 3 (11%) 2-view examinations. Twenty-six (93%) fractures were visible on at least one view with six (21%) fractures visible on all available views. No significant association was found between fracture visibility and fracture location (P=0.32). Observer agreement was substantial to almost perfect. CONCLUSION: Only 7% of these acute scaphoid fractures in younger children are inconspicuous on the initial radiographic examination.

Hand Bone Age Radiography: Comparison Between Slot-scanning and Conventional Techniques.

BACKGROUND: Determination and longitudinal monitoring of progressive skeletal maturity are essential in the management of children with scoliosis. Although different methods for determining skeletal maturity exists, the most widely practiced method relies on the ossification pattern of the bones of the hand and wrist, which is traditionally acquired using conventional techniques and after the acquisition of the spine using the low-dose slot-scanning technique. Whereas the existing published literature has published promising results on the use of the slot-scanning technique to acquire these hand and wrist radiographs, image quality and radiation dose have not been systematically compared between these techniques. Thus, the objective of our study is to compare image quality, interpretation reliability, and radiation dose of hand bone age radiographs between slot-scanning and conventional techniques using age- and sex-matched children. METHODS: This retrospective study included children who underwent hand radiographs using slot-scanning between October 1, 2019 and December 31, 2019; and matched children who underwent conventional radiography. Blinded to technique, 5 readers reviewed all radiographs after randomization to rate image quality and to determine bone age using the Greulich and Pyle classification. Dose area product was recorded. Mann-Whitney and t tests were used to compare variables between techniques and intraclass correlation (ICC) to determine observer agreement. RESULTS: Our study cohort of 194 children (128 girls, 66 boys; mean age: 13.7±2.3 y) included 97 slot-scanning and 97 conventional radiographs. One (1%) slot-scanning and no conventional radiograph was rated poor in image quality. There was almost perfect interpretation reliability with slot-scanning with high interobserver (ICC=0.948) and intraobserver (ICC=0.996) agreements, comparable with conventional radiographs (ICCs=0.919 and 0.996, respectively). Dose area product (n=158) was lower (P<0.002) with slot-scanning than with conventional techniques. CONCLUSION: Almost perfect interobserver reliability and intraobserver reproducibility with slot-scanning radiographs (performed using significantly lower radiation doses) suggest that this technique for hand bone age determination can be a reliable adjunct to scoliosis monitoring. LEVEL OF EVIDENCE: Level III.

Pediatric scaphoid fracture: diagnostic performance of various radiographic views.

PURPOSE: The purpose of this study was to systematically investigate the performance of different radiographic views in the identification of scaphoid fractures in children. METHODS AND MATERIALS: This case-control study compared 4-view radiographic examinations of the wrist between children with scaphoid fracture and age- and sex-matched children without fractures performed between January 2008 and July 2019. After randomization, each examination was reviewed 3 times, at least 1 week apart, first using each view separately and later using multiple views without (3-view) and with the posteroanterior (PA) scaphoid view (4-view), to determine the presence or absence of a scaphoid fracture. Sensitivity, specificity, positive predictive value (PPV), and negative predictive value (NPV) were calculated with inter-rater agreement. RESULTS: The study group of 58 children (48 boys and 10 girls; mean age 13.1 ± 2.1 years) included 29 with scaphoid fractures (8 corner, 9 distal pole, 10 waist, and 2 proximal pole) and 29 without fractures. Multiple views had higher sensitivity (3-view, 93.0%; 4-view, 96.5%) for fracture identification when compared to individual views (41.0-89.6%). The oblique view was 100% specific for the identification of a scaphoid fracture, but it lacked sensitivity. The PA scaphoid view had the highest sensitivity (89.6%) and NPV (90%) when compared to other individual views and its inclusion in the 4-view examinations produced the highest inter-rater agreement (93%, κ = 0.86). CONCLUSION: Multiple radiographic views of the wrist with the inclusion of a PA scaphoid view (4-view) produced the highest sensitivity, NPV, and inter-rater agreement for the identification of a scaphoid fracture in children.

Age-dependent changes in pediatric scaphoid fracture pattern on radiographs.

OBJECTIVE: To systematically investigate age-dependent changes in scaphoid fracture prevalence and fracture patterns on radiographs in children under 15 years of age. METHODS AND MATERIALS: This retrospective study included children with scaphoid fractures, who underwent radiographic examinations between May 1, 2009, and August 31, 2019. Blinded to outcome, all radiographs were reviewed to determine fracture visibility on initial radiographs; to characterize fracture location (distal corner, distal, mid, and proximal body) and orientation (horizontal oblique, transverse, and vertical oblique); and to identify the presence or absence of gap, displacement, and concomitant fractures. Demographic information and information on weight and height were collected. Mann-Whitney U, Kruskal-Wallis rank sum, chi-square, and post hoc tests were used to investigate associations between age, fracture characteristics, and BMI percentile. RESULTS: The study included 180 children (134 boys and 46 girls; 12.3 ± 1.4 years) with 59 (33%) distal corner, 42 (23%) distal, 76 (42%) mid, and 3 (2%) proximal body fractures. Younger children were more likely to present with distal corner and distal body fractures while older children with mid and proximal body fractures (p = 0.035). No association was found between age and fracture visibility (p = 0.246), fracture orientation (p = 0.752), presence of gap (p = 0.130), displacement (p = 0.403), or concomitant fractures (p = 0.588). Younger children with scaphoid fractures were more likely to be obese (n = 117; p = 0.038). CONCLUSION: Scaphoid fractures of the distal corner and distal body were significantly more common in younger children, who are more likely to be obese.

Pancreatitis Subtypes Survey in 852 Childhood-Onset Systemic Lupus Erythematosus Patients.

OBJECTIVE: Pancreatitis is a rare and a life-threatening systemic lupus erythematosus (SLE) manifestation in childhood-onset SLE (cSLE). The objective of this study was to systematically classify pancreatitis in cSLE according to the International Study Group of Pediatric Pancreatitis and determine the overall prevalence, clinical features, laboratory, and first episode outcomes. METHODS: A multicenter cohort study in 10 pediatric rheumatology centers, including 852 patients with cSLE. RESULTS: Pancreatitis was diagnosed in 22 of 852 (2.6%) patients with cSLE. It was classified as acute pancreatitis in 20 (91%), acute recurrent pancreatitis in 2 (9%), and none of them had chronic pancreatitis. None of them had gallstones, traumatic pancreatitis, or reported alcohol/tobacco use. The comparison of patients with pancreatitis (first episode) and without this complication revealed a shorter disease duration (1 [0-10] vs 4 [0-23] years, P < 0.0001) and higher median of Systemic Lupus Erythematosus Disease Activity Index 2000 (21 [0-41] vs 2 [0-45], P < 0.0001). The frequencies of fever (P < 0.0001), weight loss (P < 0.0001), serositis (P < 0.0001), nephritis (P < 0.0001), arterial hypertension (P < 0.0001), acute renal failure (P < 0.0001), macrophage activation syndrome (P < 0.0001), and death (P = 0.001) were also higher in patients with pancreatitis. The frequencies of intravenous methylprednisolone use (P < 0.0001) and the median of prednisone dose (55 [15-60] vs 11 [1-90] mg/day, P < 0.0001) were significantly higher in patients with pancreatitis. Of note, the 2 patients with acute recurrent pancreatitis had 2 episodes, with pain-free interval of 1 and 4 years. CONCLUSIONS: This was the first study characterizing pancreatitis using the International Study Group of Pediatric Pancreatitis standardized definitions in patients with cSLE showing that the predominant form is acute pancreatitis seen in association with glucocorticoid treatment and active severe disease.

Deep Learning-Assisted Diffusion Tensor Imaging for Evaluation of the Physis and Metaphysis.

Diffusion tensor imaging of physis and metaphysis can be used as a biomarker to predict height change in the pediatric population. Current application of this technique requires manual segmentation of the physis which is time-consuming and introduces interobserver variability. UNET Transformers (UNETR) can be used for automatic segmentation to optimize workflow. Three hundred and eighty-five DTI scans from 191 subjects with mean age of 12.6 years ± 2.01 years were retrospectively used for training and validation. The mean Dice correlation coefficient was 0.81 for the UNETR model and 0.68 for the UNET. Manual extraction and segmentation took 15 min per volume, whereas both deep learning segmentation techniques took < 1 s per volume and were deterministic, always producing the same result for a given input. Intraclass correlation coefficient (ICC) for ROI-derived femur diffusion metrics was excellent for tract count (0.95), volume (0.95), and FA (0.97), and good for tract length (0.87). The results support the hypothesis that a hybrid UNETR model can be trained to replace the manual segmentation of physeal DTI images, therefore automating the process.

Predictors of Effectiveness of Anakinra in Systemic Juvenile Idiopathic Arthritis.

OBJECTIVE: To seek predictors of therapeutic response to the interleukin (IL)-1 inhibitor anakinra in children with systemic-onset juvenile idiopathic arthritis (sJIA). METHODS: The clinical charts of all patients with sJIA who were newly treated with anakinra at our center between 2004 and 2017 were reviewed retrospectively. Predictors included baseline demographic, clinical, and laboratory variables as well as previous or concomitant therapies. The effectiveness of anakinra was assessed at 1 year after treatment start. Complete clinical response (CCR) was defined as absence of fever, physician's global assessment ≤ 1, count of active joints ≤ 1, negative C-reactive protein, and ≥ 75% reduction of corticosteroid dose. According to the intention-to-treat principle, patients who had anakinra discontinued before 1 year for any reasons other than disease remission were classified as nonresponders. Statistics included univariate and multivariable analyses. RESULTS: Of the 62 patients included in the study, 24 (39%) met the criteria for CCR at 1 year, whereas 38 (61%) did not. On multivariable analysis, independent correlations with achievement of CCR were identified for shorter disease duration, lower active joint count, higher ferritin level, and greater activity of systemic manifestations. The area under the curve of the model was 0.83. CONCLUSION: Our findings help to delineate the clinical profile of patients with sJIA who are more likely to benefit from IL-1 blockade. They also underscore the need for studies aimed at examining the therapeutic role of early IL-1 inhibition and to identify biomarkers predicting response to either IL-1 or IL-6 antagonists.

Anti-RO/SSA and anti-La/SSB antibodies: Association with mild lupus manifestations in 645 childhood-onset systemic lupus erythematosus.

BACKGROUND: To our knowledge there are no studies assessing anti-Ro/SSA and anti-La/SSB autoantibodies in a large population of childhood-systemic lupus erythematosus (cSLE) patients. METHODS: This was a retrospective multicenter cohort study performed in 10 Pediatric Rheumatology services, São Paulo state, Brazil. Anti-Ro/SSA and anti-La/SSB antibodies were measured by enzyme linked immunosorbent assay (ELISA) in 645 cSLE patients. RESULTS: Anti-Ro/SSA and anti-La/SSB antibodies were evidenced in 209/645 (32%) and 102/645 (16%) of cSLE patients, respectively. Analysis of cSLE patients with and without anti-Ro/SSA antibodies revealed higher frequencies of malar rash (79% vs. 71%, p=0.032), photosensitivity (73% vs. 65%, p=0.035), cutaneous vasculitis (43% vs. 35%, p=0.046) and musculoskeletal involvement (82% vs. 75%, p=0.046) in spite of long and comparable disease duration in both groups (4.25 vs. 4.58years, p=0.973). Secondary Sjögren syndrome was observed in only five patients with this antibody (2.5% vs. 0%, p=0.0035), two of them with concomitant anti-La/SSB. The presence of associated autoantibodies: anti-Sm (50% vs. 30%, p<0.0001), anti-RNP (39% vs. 21%, p<0.0001) and anti-ribossomal P protein (46% vs. 21%, p=0.002) was also significantly higher in patients with anti-Ro/SAA antibodies. Further evaluation of cSLE patients with the presence of anti-La/SSB antibodies compared to those without these autoantibodies showed that the frequency of alopecia (70% vs. 51%, p=0.0005), anti-Sm (59% vs. 31%, p<0.0001) and anti-RNP (42% vs. 23%, p<0.0001) were significantly higher in the former group. CONCLUSIONS: Our large multicenter cohort study provided novel evidence in cSLE that anti-Ro/SSA and/or anti-La/SSB antibodies were associated with mild manifestations, particularly cutaneous and musculoskeletal. Secondary Sjögren syndrome was rarely observed in these patients, in spite of comparable frequencies of anti-Ro/SSA and/or anti-La/SSB reported for adult SLE.

[Childhood-onset systemic polyarteritis nodosa and systemic lupus erythematosus: an overlap syndrome?] / Poliartrite nodosa sistêmica e lúpus eritematoso sistêmico de início na infância: uma síndrome de sobreposição?

We described herein a patient who presented an overlap syndrome of childhood-onset systemic polyarteritis nodosa (c-PAN) and childhood-onset systemic lupus erythematosus (c-SLE). A 9-year-old girl presented tender subcutaneous nodules on feet, arterial hypertension, right hemiplegia and dysarthric speech. She was hospitalized due to stroke and left foot drop. Brain computer tomography showed ischemic stroke. Magnetic resonance angiography revealed stenosis in the middle cerebral and internal carotid arteries. Electroneuromyography identified a mononeuropathy of left posterior tibial nerve and she fulfilled the c-PAN validated criteria. She was treated with intravenous methylprednisolone pulse therapy followed by prednisone, that was progressively tapered, six months of intravenous cyclophosphamide and after that she received azathioprine for 19 months. At the age of 14 years and 9 months, she presented malar rash, photosensitivity, edema in lower limbs and arterial hypertension. The proteinuria was 1.7g/day. Antinuclear antibodies (ANA) were 1/1280 (homogeneous nuclear pattern) and anti-dsDNA antibodies were positive. Renal biopsy showed focal proliferative and membranous glomerulonephritis. Therefore, she fulfilled the American College of Rheumatology classification criteria for SLE and she was treated with prednisone, hydroxychloroquine and mycophenolate mofetil. In conclusion, we described herein a possible overlap syndrome of two autoimmune diseases, where c-PAN occurred five years before the c-SLE diagnosis.

[Liver and spleen biometrics in childhood-onset systemic lupus erythematosus patients]. / Biometria do fígado e do baço em pacientes com lúpus eritematoso sistêmico de início pediátrico.

OBJECTIVE: To evaluate liver and spleen dimensions in childhood-onset systemic lupus erythematosus (c-SLE) patients and healthy controls. METHODS: 30 c-SLE patients and 30 healthy control volunteers underwent abdominal ultrasound. The following two liver measurements were performed in left hepatic lobe: craniocaudal and anteroposterior and three in right hepatic lobe (RHL): posterior craniocaudal (PCC-RHL), anterior craniocaudal and anteroposterior. Three spleen dimension measurements were also evaluated: longitudinal, transverse and anteroposterior. Demographic, clinical and laboratorial data, SLEDAI-2K, ECLAM, SLAM and treatment were assessed. RESULTS: Mean current age was similar in c-SLE and controls (170.31 ± 27.81 vs. 164.15 ± 39.25 months; p = 0.486). The mean of PCC-RHL dimension was significantly higher in c-SLE compared to controls (13.30 ± 1.85 vs. 12.52 ± 0.93, p = 0.044). There were no differences between the other hepatic biometrics and splenic parameters (p > 0.05). Further analysis in c-SLE patients according to PCC-RHL dimension ≥ 13.3cm versus < 13.3 cm showed that the median of SLEDAI-2K [8(0-18) vs. 2(0-8), p=0.004], ECLAM [4(0-9) vs. 2(0-5), p = 0.019] and SLAM [5(1-13) vs. 2(0-14), p = 0.016] were significantly higher in patients with higher PCC-RHL dimension, likewise the frequencie of nephritis (77% vs. 29%, p = 0.010). Liver enzymes were similar in both groups (p > 0.05). Positive correlation was observed between SLEDAI-2K and PCC-RHL (p = 0.001, r = +0.595). Negative correlation was evidenced between disease duration and longitudinal dimension of spleen (p = 0.031, r = -0.394). CONCLUSION: Our data raises the possibility that disease activity could lead to a subclinical and localized hepatomegaly during the disease course. Long disease duration resulted to spleen atrophy in c-SLE patients.