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.

Imatinib melylate as second-line treatment of bronchiolitis obliterans after allogenic hematopoietic stem cell transplantation in children.

Background: The onset of bronchiolitis obliterans (BO) as a pulmonary manifestation of chronic graft vs host disease dramatically changes the prognosis of children undergoing allogeneic hematopoietic stem cell transplantation (allo-HSCT). This study aimed to evaluate the overall survival (OS) of children with BO treated with imatinib mesylate (IM).

Methods: This study included children who underwent allo-HSCTs between January 2000 and December 2016.

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.

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.

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.

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 m).

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.

Multimodality imaging of pediatric airways disease: indication and technique.

Congenital and acquired airway anomalies represent a relatively common albeit diagnostic and therapeutic challenge, even for the most skilled operators in dedicated centers. Airway malformations encompass a wide spectrum of pathologies involving the larynx, trachea and bronchi, esophagus, mediastinal vessels. These developmental lesions are often isolated but the association of two or more anomalies is not infrequent.

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.

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.

Delineating the Application of Ultrasound in Detecting Synovial Abnormalities of the Subtalar Joint in Juvenile Idiopathic Arthritis.

Objective: To investigate the frequency of ultrasound (US)-detectable involvement of the subtalar joint (STJ), to compare clinical versus US assessment of the STJ, and to compare different scanning approaches to the STJ in juvenile idiopathic arthritis (JIA).

Methods: Clinical and US assessments were performed independently in 50 ankles with clinically active JIA. US abnormalities of the STJ were investigated using a lateral, medial, and posterior scanning approach and scored semiquantitatively.

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.

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.

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.

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.

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.

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.

MRI of the wrist in juvenile idiopathic arthritis: proposal of a paediatric synovitis score by a consensus of an international working group. Results of a multicentre reliability study.

Background: MRI is a sensitive tool for the evaluation of synovitis in juvenile idiopathic arthritis (JIA).

Objective: The purpose of this study was to introduce a novel MRI-based score for synovitis in children and to examine its inter- and intraobserver variability in a multi-centre study.

Materials And Methods: Wrist MRI was performed in 76 children with JIA.

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.

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.

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.