Non-contrast-enhanced magnetic resonance angiography for detecting crossing renal vessels in infants and young children: comparison with contrast-enhanced angiography and surgical findings.

BACKGROUND: Knowing that ureteropelvic junction obstruction is due to a crossing renal vessel is essential in choosing the appropriate surgical treatment. OBJECTIVE: To evaluate the diagnostic accuracy of non-contrast magnetic resonance (MR) angiography in identifying crossing renal vessels in children younger than 4 years old with unilateral hydronephrosis. MATERIALS AND METHODS: A retrospective review of preoperative MR urography of children with unilateral hydronephrosis was conducted by two independent readers. The presence or absence of crossing renal vessels was identified and compared with surgical findings. RESULTS: Twenty-nine patients were included. The disagreement between MR angiography with and without contrast enhancement in detecting a crossing renal vessel was 8%. The disagreement between non-contrast-enhanced MR and surgical findings was 17%. The disagreement between contrast-enhanced MR angiography and surgical findings was 25%. The balanced triggered angiography without contrast enhancement had a sensitivity of 70% (95% confidence interval [CI]: 35-93%) and a specificity of 93% (95% CI: 66-100%). Contrast-enhanced MR angiography had a sensitivity of 56% (95% CI: 21-86%) and a specificity of 91%. (95% CI: 59-100%). CONCLUSION: MR without contrast enhancement may be a reliable, valid and safe alternative to contrast-enhanced MR angiography for identifying crossing renal vessels.

Ultrasound changes in synovial abnormalities induced by treatment in juvenile idiopathic arthritis.

OBJECTIVES: To investigate the capacity of ultrasound (US) to detect improvement of synovial abnormalities induced by treatment in juvenile idiopathic arthritis (JIA). METHODS: Eighty-three joints (33 knees, 22 tibiotalar, 10 wrists, 9 elbows, 9 subtalar joints) of 33 patients with new-onset JIA were assessed by US at study entry and 6 months after a therapeutic intervention. Each joint was scored for grey-scale (GS) and power Doppler (PD) abnormalities according to a 4-point semiquantitative scale. Pre- and post-treatment US scores were compared and the sensitivity to change of GSUS and PDUS was estimated. Clinical response was assessed using the ACR paediatric (ACRp) response criteria. RESULTS: Seventeen patients (51.5%) underwent intra-articular corticosteroid injection (IACI) only, 15 (45.5%) were given IACI and systemic medications, and 1 (3.0%) was started with systemic therapy alone. Both GSUS and PDUS scores improved significantly (p<0.0001) from baseline to follow-up. US revealed strong sensitivity to change with standardised response mean for GSUS and PDUS of 2.44 and 1.23, respectively. At the follow-up visit, 13/20 (65.0%) joints with residual US abnormalities were judged in remission on clinical grounds. Six/21 (28.6%) patients who were ACRp90 responders did not display complete resolution of synovial abnormalities on US. CONCLUSIONS: US is a sensitive tool to assess therapeutic response in patients with JIA. Subclinical disease on US is common in joints with clinically-defined remission. An ACRp90 response may not be coupled with complete resolution of synovial abnormalities on US. Further studies are needed to establish the impact of US on therapeutic decision-making in JIA.

Epidemiology of invasive fungal diseases in children with solid tumours undergoing autologous haematopoietic stem cell transplantation: a 10-year experience in a tertiary Italian centre.

The objective of the study was to determine the incidence of invasive fungal disease (IFD) in children undergoing autologous haematopoietic stem cell transplantation (auHSCT) for solid tumours (ST). Retrospective study on auHSCT was performed in children with ST (January 2006-December 2015). Data on the number of patient-days at risk (pdr) during the first 30 and 90 days after auHSCT and cases of proven/probable IFDs were collected. Infection rate (IR, episodes/1000 pdr) and proportions and cumulative risk (CR) of IFD were evaluated. In 186 patients, 270 auHSCT were performed, for a total of 8327 pdr during the first 30 days and 24 366 up to day 90. Median age was 5 years (interquartile range 2;8), 63% were male. At day 30, seven procedures were complicated by IFD, with an IR of 0.84 (95% CI 0.66-1.02) and aCR of 2.6% (95% CI 1.4-5.4) at 18 days after HSCT. Within day 90, two further IFDs were detected with an IR of 0.37 (95% CI -0.49 to 1.23) and a CR of 3.3% (95% CI 1.7-6.3) at day 69. Children undergoing auHSCT for ST have a low incidence of IFDs in the first 90 days after the procedure.

Long-Term Extracorporeal Membrane Oxygenation as Bridging Strategies to Lung Transplantation in Rapidly Devastating Isolated Langerhans Cell Histiocytosis.

Isolated pulmonary involvement in pediatric Langerhans cell histiocytosis (LCH) is extremely rare. While the multisystem-LCH course varies from spontaneous remission to rapid deterioration with lethal outcome, single system involvement is generally associated with favorable prognosis. A child with isolated pulmonary LCH had an extremely rapid progression leading to respiratory failure, despite treatment with prednisone and vinblastine. Since lung hyperinflation and cystic degeneration contraindicated conventional mechanical ventilation, extracorporeal membrane oxygenation (ECMO) was chosen for 50 days as a bridge to lung transplantation. The mechanisms involved in disease progression and the usefulness of long-term ECMO are discussed.

Whole-body MRI in the assessment of disease activity in juvenile dermatomyositis.

OBJECTIVE: To compare whole-body MRI (WB-MRI) with clinical examination in the assessment of disease activity in juvenile dermatomyositis (JDM). METHODS: WB-MR images were obtained from 41 JDM patients and 41 controls using a 1.5 T MRI scanner and short τ inversion recovery sequences. 18 patients had follow-up WB-MRI. Muscle, subcutaneous tissue and myofascial signal abnormalities were scored in 36 muscular groups and on proximal and distal extremities. WB-MRI and clinical assessments were performed concurrently and results compared. Validation procedures included analysis of feasibility, reliability, construct validity, discriminative ability and responsiveness. RESULTS: WB-MRI revealed distal legs (26/41 patients) and forearm (19/41 patients) muscle inflammation undetected during clinical examination and allowed an accurate assessment of subcutaneous (23/41 patients) and myofascial involvement (13/41 patients). 27 patients showed a patchy distribution of muscle inflammation while in seven the abnormal hyperintense areas tended to be homogeneously distributed. The inter-reader agreement for muscular, subcutaneous and myofascial WB-MRI scores was excellent. Correlations between WB-MRI muscle score and disease activity measures were excellent (Manual Muscle Test: rs=-0.84, Childhood Myositis Assessment Scale: rs=-0.81). WB-MRI score was higher in JDM active patients when compared with the control group (pB<0.0001) and the inactive patients (pB=0.004), and showed an excellent responsiveness (standardised response mean=1.65). Follow-up WB-MRI showed resolution of inflammation in nine patients whereas clinical criteria for remission were satisfied in five. CONCLUSIONS: WB-MRI provides additional information to clinical evaluation and represents a promising tool to estimate total inflammatory burden, tailor treatment and monitor its efficacy.

Magnetic resonance urography and laparoscopy in paediatric urology: a case series.

Paediatric urology often presents challenging scenarios. Magnetic resonance urography (MRU) and laparoscopy are increasingly used. We retrospectively studied children affected by a disease of the upper urinary tract who after MRU were elected for laparoscopic treatment. This pictorial essay draws on our experience; it illustrates some specific MRU findings and highlights the usefulness of MRU for the diagnosis of upper urinary tract pathology in children. It also offers some examples of the potential additional diagnostic information provided by laparoscopy as well as its therapeutic role.

Predictive Value of Magnetic Resonance Imaging in Patients With Juvenile Idiopathic Arthritis in Clinical Remission.

OBJECTIVE: To define the prevalence of subclinical synovitis on magnetic resonance imaging (MRI) in a large cohort of patients with juvenile idiopathic arthritis (JIA) in clinical remission and to evaluate its predictive value in terms of disease flare and joint deterioration. METHODS: Ninety patients with clinically inactive JIA who underwent a contrast-enhanced (CE)-MRI of a previously affected joint were retrospectively included. Each joint was evaluated for synovitis, tenosynovitis, and bone marrow edema. Baseline and follow-up radiographs were assessed to evaluate structural damage progression. RESULTS: CE-MRI was acquired in 45 wrists, 30 hips, 13 ankles, and 2 knees. Subclinical synovitis was detected in 59 (65.5%) of 90 patients and bone marrow edema in 42 (46.7%) of 90 patients. Fifty-seven of 90 (63.3%) patients experienced a disease flare during follow-up. Forty-four of 59 (74.6%) patients with subclinical synovitis experienced a disease flare versus 13 (41.9%) of 31 patients with no residual synovitis on MRI (P = 0.002). The presence of subclinical synovitis was the best predictor of disease flare on multivariable regression analysis (hazard ratio [HR] 2.45, P = 0.003). Baseline and follow-up radiographs were available for 54 patients, and 17 (31.5%) of 54 patients experienced radiographic damage progression. The presence of bone marrow edema (HR 4.40, P = 0.045) and being >17 years old (HR 3.51, P = 0.04) were strong predictors of joint damage progression in the multivariable analysis. CONCLUSION: MRI-detected subclinical inflammation was present in a large proportion of patients with JIA despite clinical remission. Subclinical synovitis and bone marrow edema have been shown to play a role in predicting the risk of disease relapse and joint deterioration, with potential implications for patients' management of the disease.

Mycobacterium tuberculosis pneumonia and bacteremia after allogeneic hematopoietic stem cell transplant: report of an instructive pediatric case.

Pulmonary infections often complicate hematopoietic stem cell transplantation (HSCT) outcome. Uncommon aetiologies, like Mycobacterium tuberculosis, should be considered when the clinical conditions do not fully improve with standard antimicrobial therapy and microbiological evaluations are repeatedly negative for bacteria and fungi. We describe an interesting pediatric case of miliary lung tuberculosis after HSCT, which was successfully treated after administering the appropriate therapy.

Development and preliminary validation of a paediatric-targeted MRI scoring system for the assessment of disease activity and damage in juvenile idiopathic arthritis.

OBJECTIVES: To develop and validate a paediatric-targeted MRI scoring system for the assessment of disease activity and damage in juvenile idiopathic arthritis (JIA). To compare the paediatric MRI score with the adult-designed. Outcome Measures in Rheumatology Clinical Trials-Rheumatoid Arthritis MRI Score (RAMRIS), whose suitability for assessing growing joints was tested. METHODS: In 66 patients with JIA the clinically more affected wrist was studied. Thirty-nine patients had a 1-year MRI follow-up. Two readers independently assigned the paediatric score and the RAMRIS to all studies. Validation procedures included analysis of reliability, construct validity and responsiveness to change. A reduced version of the bone erosion score was also developed and tested. RESULTS: The paediatric score showed an excellent reproducibility (interclass correlation coefficient >0.9). The interobserver agreement of RAMRIS was moderate for bone erosions and excellent for bone marrow oedema (BMO). The paediatric score and RAMRIS provided similar results for construct validity. The responsiveness to change of the paediatric score was moderate for synovitis and bone erosion, and poor for BMO and did not improve when RAMRIS was applied. The reduced version of the bone erosion was valuable for the assessment of joint damage, and provided time-saving advantages. CONCLUSION: The results demonstrate that the paediatric MRI score is a reliable and valid method for assessing disease activity and damage in JIA. Unexpectedly, the RAMRIS provides acceptable suitability for use in the paediatric age group. Further work, especially in a longitudinal setting, is required before defining the most suitable MRI scale for assessing growing joints.

Effect of the Inclusion of the Metacarpophalangeal Joints on the Wrist Magnetic Resonance Imaging Scoring System in Juvenile Idiopathic Arthritis.

OBJECTIVE: To extend the magnetic resonance imaging (MRI) score for assessment of wrist synovitis in juvenile idiopathic arthritis (JIA) by inclusion of the metacarpophalangeal (MCP) joints, and to compare the metric properties of the original and the extended score. METHODS: Wrist MRI of 70 patients with JIA were scored by 3 independent readers according to (1) the wrist component of the rheumatoid arthritis MRI synovitis score (comprising distal radioulnar, radiocarpal, and combined midcarpal and carpometacarpal joints); and (2) an extended score including the MCP joints. Thirty-eight patients had a 1-year MRI followup. The concordance between the readers [intraclass correlation coefficient (ICC), 95% limits of agreement (LOA), and weighted Cohen's κ], correlations with clinical variables (Spearman's ϱ), and the sensitivity to change [standardized response mean (SRM)] were calculated for both scores. RESULTS: The interreader agreement was moderate for the original score (ICC 0.77; 95% CI 0.68-0.84) and good for the extended score (ICC 0.86; 95% CI 0.80-0.91). Using 95% LOA, the aggregate score variability was less favorable with relatively wide LOA. Weighted Cohen's κ of the individual joints indicated good agreement for the original score and good to excellent agreement for the extended score. Correlations with clinical variables reflecting disease activity improved for the extended score and its SRM was higher compared to that of the original score. CONCLUSION: The extended score showed better reliability, construct validity, and sensitivity to change than the original. Inclusion of the MCP joints should be considered for a more accurate assessment of disease activity and treatment efficacy in JIA.

Ofatumumab-associated acute pneumonitis: Not new but still the first case.

Ofatumumab is an anti-CD20 humanized monoclonal antibody utilized in the treatment of several clinical conditions resistant to other treatments. In spite there was a general expectation that ofatumumab was less toxic compared to rituximab, side effects have been reported that resemble those of its anti-CD20 chimeric precursor. Here, we describe the first case of Ofatumumab associate lung injury occurring in a 14-year-old boy affected by nephrotic syndrome dependent to prednisone plus cyclosporine A who had been treated with the dose of drug utilized in nephrotic syndrome (1500 mg/173 m2). The patient developed the full blown picture of rituximab associated lung injury (RALI) after 45 days from ofatumumab infusion at the end of the steroid tapering: severe exertional dyspnea, mild fever and cyanosis, with CT scan showing diffuse ground glass areas in both lungs and DLCO (diffusing capacity of transfer factor of the lung for carbon monoxide) test suggestive for reduction of CO diffusion. Clinical outcome was good with rapid improvement and normalization of all parameters without any specific therapy. After 60 days, chest CT and CO diffusion tests were normal. In conclusion, we describe here the first case of acute pneumonitis associated with ofatumumab that presents the same clinical, laboratory, and radiology features of the lung injury reported for rituximab. Like RALI occurring in patients treated for nephrotic syndrome, this case had a mild clinical expression and recovered in a few months.

Short-length ligamentum arteriosum as a cause of congenital narrowing of the left main stem bronchus.

An entity that has received little attention as cause or recurrent respiratory disorder is the narrowing of the left main stem bronchus. When not associated with congenital heart disorders, this condition has been ascribed to primary localized malacia of the bronchial cartilages or to the anterior displacement of the descending aorta in front to the adjacent vertebral bodies. Four girls were evaluated for recurrent/chronic respiratory symptoms. A pulsatile extrinsic compression on the posterior bronchial wall of the left main stem bronchus was detected, pressed between the descending aorta, posteriorly, and the left pulmonary artery, anteriorly. The two arteries were closely linked together by a short-length ligamentum that was resected, allowing the mobilization of the aorta with posterior aortopexy, stabilizing the space created between the pulmonary artery and the descending aorta. The reduced compression on the left main bronchus resulted in the enlargement of its caliber and in a marked improvement of the respiratory symptoms. Pediatr Pulmonol. 2016;51:1356-1361. © 2016 Wiley Periodicals, Inc.

Mild tracheal compression by aberrant innominate artery and chronic dry cough in children.

BACKGROUND: In children with aberrant innominate artery (AIA) one of the most prevalent respiratory symptom is dry cough. How frequently this mediastinal vessels anomaly, that can induce tracheal compression (TC) of different degree, may be detected in children with chronic dry cough is not known. METHODS: In a 3-year retrospective study, the occurrence of mediastinal vessels abnormalities and the presence and degree of TC was evaluated in children with recurrent/chronic dry cough. RESULTS: Vascular anomalies were detected in 68 out of the 209 children evaluated. A significant TC was detected in 54 children with AIA, in eight with right aortic arch, in four with double aortic arch but not in two with aberrant right subclavian artery. In AIA patients, TC evaluated on computed tomography scans, was mild in 47, moderate in six and severe in one. During bronchoscopy TC increased in expiration or during cough, but this finding was more pronounced in children with right aortic arch and double aortic arch in which a concomitant tracheomalacia was more evident. Comorbidities were detected in 21 AIA patients, including atopy, reversible bronchial obstruction and gastroesophageal reflux. Aortopexy was performed in eight AIA patients, while the remaining AIA patients were managed medically and showed progressive improvement with time. CONCLUSION: Mild TC induced by AIA can be detected in a sizeable proportion of children with recurrent/chronic dry cough. The identification of this anomaly, that may at least partially explain the origin of their symptom, may avoid further unnecessary diagnostic examinations and ineffective chronic treatments.

Corticosteroids may favor proliferation of thoracic inflammatory myofibroblastic tumors.

Inflammatory myofibroblastic tumor (IMT) was thought to represent a benign post-infectious or post-inflammatory process cured by surgical resection. However, reports of cases with an aggressive clinical course suggest the need for caution about the prognosis. The treatment of choice is a complete surgical resection, while medical treatment options are limited. Corticosteroid therapy has been used with some success in unresectable lesion. However, rapid progression of lung IMT after prednisone treatment has been reported, raising the hypothesis that corticosteroids may favor a tumultuous proliferation of this lesion, possibly through immunosuppression. We here report a similar observation and suggest that other mechanisms may be involved. A 5-year and 6-month-old boy presented with a 72 hr history of breathlessness, initially responsive to albuterol and prednisone. He represented 15 days later with increasing symptoms despite further prednisone treatment. CT chest scan showed a mass lesion in the tracheal lumen, which on biopsy was found to be an IMT. The possibility that prednisone may have an enhancing effect on IMT cell proliferation is demonstrated through IMT cell culture and discussed.

Role of management strategies in reducing mortality from invasive fungal disease in children with cancer or receiving hemopoietic stem cell transplant: a single center 30-year experience.

BACKGROUND: In the last decades, several diagnostic and therapeutic strategies have been implemented for management of invasive fungal diseases (IFD) in patients with cancer or receiving allogeneic hemopoietic stem cell transplant. Few data are available on their impact on mortality in children. METHODS: All IFD episodes diagnosed at tertiary care center during a 30-year period between 1983 and 2012 were analyzed for 90-day mortality and risk factors. Diagnoses were coded according to international (European Organization for Research and Treatment of Cancer/Invasive Fungal Infections Cooperative Group and the National Institute of Allergy and Infectious Diseases Mycoses Study Group) criteria. Four treatment eras (1983-1990, 1991-1999, 2000-2005 and 2006-2012) were defined according to availability of diagnostic technologies, new antifungal drugs and use of a diagnostic-driven approach without empiric antifungal therapy. RESULTS: A total of 198 IFD were diagnosed in 191 patients; 71.2% were proven/probable infections; 39.9% were caused by yeasts and 31.3% by molds. Within 90 days from IFD diagnosis, 58 (30.4%) patients died for a 28.3% cumulative probability of death. A multivariable analysis showed that the highest risk of death was associated with alternative donor-hemopoietic stem cell transplant [hazard ratio (HR): 3.96] and mold etiology (HR: 1.34). The risk of death significantly decreased across the treatment eras, with almost a 3-fold reduced risk for patients diagnosed during the 2006-2012 period (HR: 0.24). Also if the variable year of diagnosis was considered as continuous, the hazard of death significantly decreased by 5% per year (HR: 0.95). CONCLUSIONS: New management strategies resulted in a better prognosis of IFD in children with cancer or hemopoietic stem cell transplant. A diagnostic-driven approach was not associated with an increase in mortality.

Invasive mould infections in newborns and children.

Invasive mould infections represent important complications of different pediatric conditions. Epidemiology and clinical features vary according to the type of underlying conditions that determine the risk of invasive mycosis. No pediatric study has specifically evaluated the efficacy of prophylaxis or therapy invasive moulds infections, while pediatric dosages for the treatment of invasive aspergillosis are available for drugs that produced positive results in clinical trials undertaken in adults.

Chronic eosinophilic ascites in a very young child.

UNLABELLED: A case of chronic eosinophilic ascites with onset in early infancy is described. An intensive diagnostic work-up ruled out other known causes of ascites in childhood. The final diagnosis was made at 2 years of age when a large number of eosinophils was detected in the ascitic fluid. The outcome was complicated by an ex vacuo intraperitoneal haemorrhage. Steroids were able to control the disease only after complete aspiration (1600 ml) of the ascitic fluid. On discontinuation of treatment, peritoneal inflammation recurred indicating steroid-dependency. CONCLUSION: eosinophilic ascites, a very rare disorder in children, should be considered in the differential diagnosis of even very young children presenting with ascites.