Disseminated Mycobacterial Infection With Reactive Polyarthritis (Poncet's Disease) During Immune-suppressive Treatment Including Ustekinumab for Pediatric Crohn's Disease.

BACKGROUND: The incidence of pediatric inflammatory bowel disease is increasing. tumor necrosis factor alpha inhibitors medicines improved the prognosis of affected subjects. Nonetheless, a proportion of patients do not respond or lose response to treatment. Newer biologics, like ustekinumab, have been approved for adults. The pediatric off-label use of these drugs is increasing, despite limited safety evidence. We report a case of disseminated mycobacterial infection (MI) presenting with reactive polyarthritis (Poncet's disease, PD) in a girl with Crohn's disease receiving various immunosuppressants, including ustekinumab. CASE REPORT: A 12-year-old girl with Crohn's disease was admitted for acute-onset migratory polyarthritis of large and small joints and opioid-resistant pain. She had recently received adalimumab and methotrexate and was currently under treatment with ustekinumab. She was vaccinated with Bacillus Calmette-Guérin and screened for tuberculosis before starting immunosuppressants. Interferon-gamma release assay, Mantoux test and chest computed tomography scan were negative. Disseminated MI with PD was diagnosed following positive cultures for Mycobacterium tuberculosis complex in blood and intestinal biopsies (with negative in synovial fluid and gastric aspirate). Whole-exome sequencing did not identify any genetic susceptibility to MI. Antituberculosis treatment eradicated MI. CONCLUSIONS: Children with inflammatory bowel disease receiving combination immunosuppressive treatments including tumor necrosis factor alpha inhibitors and anti-IL-12/23 agents are at higher risk for MI. Disseminated MI should be considered and ruled out in these patients when presenting with pulmonary, extrapulmonary or unusual clinical manifestations, like PD. The collection of multiple specimens (including intestinal biopsies) for mycobacterial culture is recommended when mycobacterial disease is suspected.

COVID-19 musculoskeletal involvement in children.

Since the early phases of the COVID-19 pandemic, it has become clear that children are affected by mild respiratory symptoms rather than the critical pneumonia typical in adults. Nevertheless, it took longer to understand that pediatric patients with SARS-COV2 may develop a severe multisystem inflammatory response (a.k.a. multisystem inflammatory syndrome in children (MIS-C)), which can include musculoskeletal symptoms, and/or arthritis and myositis independently from MIS-C. Diagnostic imaging significantly contributed to the assessment of pulmonary disease due to COVID-19 but it has been rarely applied to evaluate musculoskeletal involvement in children with or without previous rheumatic diseases. Despite the paucity of radiological literature, muscle edema at magnetic resonance and synovitis at ultrasound have been described. Further use of diagnostic imaging for children with articular and muscular symptoms due to COVID-19 is strongly encouraged.

[18F]FDG PET-MR in the Evaluation and Follow-Up of Incidental Bone Ischemic Lesions in a Mono-Center Cohort of Pediatric Patients Affected by Hodgkin's Lymphoma.

Hodgkin's lymphoma (HL) is one of the neoplasms with the best prognosis in children, adolescents and young adults, but sufferers are burdened by the possibility of developing adverse effects such as Bone Ischemic Lesions (BILs) which are lesions of the bone caused by the loss of/reduction in blood flow. The main goal of this retrospective study was to evaluate the role of [18F]FDG-PET-MR in the early detection of BILs in a single-center cohort of uniformly treated pediatric HL patients. BILs were assessed through PET-MR images as the appearance of medullary lesion surrounded by a serpiginous, tortuous border. From 2017 to 2022, 10/53 (18.9%) HL patients developed BILs which were mostly (8/10 patients) multifocal. Overall, 30 lesions were identified in the 10 asymptomatic patients, all with the above-mentioned features at MR and with very low [18F]FDG uptake. BILs were incidentally detected during HL therapy (n = 6) and follow-up (n = 4), especially in the long bones (66.7%). No factors correlated with the occurrence of BIL were identified. No patients developed complications. PET-MR is a sensitive combined-imaging technique for detecting BILs that are asymptomatic and self-limiting micro-ischemic lesions. BILs can be monitored by clinical follow-up alone both during and after therapy.

Lung Ultrasound to Assess One Lung Ventilation: A Pediatric Case Series.

Objectives: One lung ventilation (OLV) is the preferred ventilation technique for thoracoscopy as it provides a better exposure of the operative field and grants the protection of the healthy lung. Preoperative evaluation of lung exclusion is necessary and different methods are available. In recent years lung ultrasound (US) gained popularity and its use for monitoring the endotracheal tube position is widely reported. The existing evidence on adults addresses lung US as effective, yet only few data are available in children. Therefore, we present our experience with lung US as verification method for pediatric OLV. Methods: All patients undergoing OLV for video-assisted thoracoscopic surgery from January 2019 to May 2021 and for whom lung exclusion was confirmed through lung US were involved. Lung exclusion was considered effective when absence of lung motion and presence of lung pulse were encountered. When lung US did not match these criteria, repositioning of the endobronchial device followed by US verification was performed. When lung US met the exclusion criteria surgery was started and direct thoracoscopic observation was used to verify lung exclusion. Results: A total of 20 patients, accounting for 22 procedures, were involved. Absence of lung motion and presence of lung pulse were assessed in the operative-side lung for all patients. Lung exclusion was confirmed through thoracoscopy. Postoperative lung US proved the reappearance of lung motion in the previously excluded lung. Conclusions: In our center experience lung US resulted to be a safe, effective, and time-saving verification method for OLV. Further studies are needed to define its sensitivity and specificity.

Osteoperiostitis in children: proposal for a diagnostic algorithm.

Juvenile osteoperiostites (JOP) are a group of inflammatory bone diseases whose differential diagnosis is often difficult. The main conditions are acute osteomyelitis (AOM), chronic non-bacterial osteomyelitis (CNO) and the Goldbloom syndrome (GS). The study was aimed to develop an algorithm to enable an early diagnosis of JOP. Clinical records of patients with AOM, CNO and GS, followed at our Center over the past 10 years, were reviewed. Twelve additional patients with GS were selected from PubMed/MEDLINE literature search. Data collected included demographics, clinical manifestations, laboratory and instrumental investigations at disease onset. The association between categorical variables was investigated, and the segmentation of patients with different diagnoses was analyzed through a classification tree model (CTREE package) in order to build up a diagnostic algorithm. Ninety-two patients (33 CNO, 44 AOM, 15 GS) entered the study. Among 30 variables considered at onset, nine (age at onset, fever, weight loss, symmetry, focality, functional limitation, anemia, elevated ESR, CRP) resulted statistically significant in differentiating the three clinical entities from each other and were chosen to build up a decisional tree. Three variables, symmetry of bone involvement, presence of fever and age at disease onset, resulted significant to discriminate each of the three diseases from the others. The performance of the diagnostic algorithm was validated by comparing the diagnoses provided by the model with the real diagnoses and showed 85.9% accuracy.Conclusion: We propose a diagnostic algorithm, based on simple clinical data, which can help guide a prompt and appropriate diagnosis of JOP. What is Known: • Juvenile osteoperiostitis (JOP) are a group of inflammatory bone diseases followed by various pediatric specialists. • The distinction between these conditions is not easy as clinical and laboratory features often overlap. What is New: • We propose a diagnostic algorithm, based on clinical data of real patients, with high degree accuracy. • This instrument can help guide the prompt and appropriate diagnosis of JOP.

Sarcopenia in juvenile localized scleroderma: new insights on deep involvement.

OBJECTIVES: Juvenile localized scleroderma (JLS) is a rare chronic autoimmune disease which can also affect bones and muscles. Nevertheless, muscle loss was not previously investigated in patients with JLS. Thus, the aim of this study was to retrospectively evaluate deep involvement and assess and quantify sarcopenia in JLS patients using magnetic resonance imaging (MRI). METHODS: Fourteen children with JLS (nine females, mean age ± SD, 7.1 ± 3.6 years) referring to our tertiary center from January 2012 to January 2018 who underwent at least one MRI examination including axial T1-weighted and short tau inversion recovery images were included. Two readers assessed in consensus superficial and deep involvement. Muscle edema, muscle fatty infiltration, and sarcopenia were independently scored (absent, moderate, or severe) and the Cohen's kappa coefficient computed. Skin perimeter, subcutaneous area, muscle area, and muscle volume were independently measured using the contralateral unaffected extremity as reference (paired Student's t test, p < 0.05). The intraclass correlation coefficient (ICC) was used to investigate the reliability of the measurements. RESULTS: All patients showed superficial involvement with subcutaneous fat atrophy being the most common finding (13 patients). Bone marrow edema occurred in five patients. Muscle edema affected ten children (moderate in seven, severe in three; k = 0.89), muscle fatty replacement occurred in one case (severe; k = 1.00), and sarcopenia was detected in eight patients (severe in two; k = 0.78). All quantitative parameters were lower on the affected side than on the unaffected contralateral limb (p < 0.05, each) and all measurements showed a high reliability (ICC > 0.750, each). CONCLUSION: Patients with JLS can be affected by sarcopenia and quantitative analyses allow a robust characterization of such finding. KEY POINTS: • Deep involvement in juvenile localized scleroderma is frequently characterized by sarcopenia. • In juvenile localized scleroderma, muscle edema and sarcopenia are mostly moderate while fatty infiltration, even if rare, can be severe. • Sarcopenia can be reliably quantified in children with juvenile localized scleroderma using MRI.

18F-FDG PET/MR in an Atypical Pediatric Solid Pseudopapillary Pancreatic Tumor.

MR and F-FDG PET/MR images of a 15-year-old boy with recurrent mild abdominal pain and hyperamylasemia demonstrating a pancreatic head lesion hypointense on the T1-weighted and slightly hyperintense on the T2-weighted images, with very small pseudocystic areas, restricted diffusion on the apparent diffusion coefficient map, and focal FDG uptake. No other pathologic findings such as enlarged lymph nodes, vascular invasion, metastases, dilatation of the main pancreatic duct, or pathologic uptake of the parotid glands were detected. The MR features, even if atypical, together with the focal uptake, allowed the diagnosis of pediatric solid pseudopapillary pancreatic tumor, which was then confirmed by histology.

Assessing pediatric ileocolonic Crohn's disease activity based on global MR enterography scores.

OBJECTIVES: This study was aimed at correlating a magnetic resonance index of activity (MaRIA) and a magnetic resonance enterography global score (MEGS) with activity indexes in a paediatric population with Crohn's disease (CD). METHODS: This retrospective study included 32 paediatric patients (median age 14.5 years, 18 male) with proven CD who underwent magnetic resonance enterography (MRE). A correlation analysis was performed on the MRE-based scores, the simplified endoscopic score for CD (SES-CD), the paediatric Crohn's disease activity index (PCDAI), and C-reactive protein (CRP) levels. Based on PCDAI, comparison of both global MaRIA and MEGS was made between patients with mild and moderate/severe disease activity. RESULTS: Global MaRIA correlated with SES-CD (r = 0.70, p = 0.001) and PCDAI (r = 0.42, p = 0.016). MEGS correlated with PCDAI (r = 0.46, p = 0.007) and CRP levels (r = 0.35, p = 0.046). MEGS differed significantly (p = 0.027) between patients grouped by clinical disease severity. CONCLUSIONS: MRE-based global scores correlated with clinical indexes of CD activity. Therefore, they represent a potential useful tool to predict CD activity and severity, as well as a possible promising alternative to endoscopy, to monitor paediatric patients with CD during their follow-up. KEY POINTS: • MRE is widely used to for accurate assessment of CD. • Global MaRIA and MEGS have been suggested as indicators of CD activity. • Paediatric studies comparing MRE-based global scores with clinical CD activity are lacking. • Such scores can serve as predictors of CD activity/severity in paediatric patients. • MRE offers an alternative to clinical score/endoscopy for paediatric CD monitoring.

Relationship between gastric pouch and weight loss after laparoscopic sleeve gastrectomy.

BACKGROUND: Laparoscopic sleeve gastrectomy (LSG) is considered safe and effective even as conversion procedure after primary bariatric operations. The correlation between gastric pouch volumes and patients weight loss remains unclear. METHODS: To assess a correlation between the gastric remnant size and the weight loss, we reviewed 49 consecutive barium swallow UGS performed at our institute from August 2012 through May 2014 in LSG patients with symptoms and/or unsatisfactory weight loss. The anteroposterior (AP), laterolateral (LL) and vertical (CC) diameters of the gastric pouch were measured to calculate the volume by the formula of the ellipsoid (AP × LL × CC × 0.5). Patients were divided in two groups: group 1 without gastric pouch (n = 36) and group 2 with gastric pouch (n = 13). Correlation between pouch volume and weight loss data was calculated with t Student's and Fisher tests to compare the percent excess body mass index (BMI) and percent excess body mass loss (EBL) between two groups, and P < 0.05 was considered statistically significant. RESULTS: The mean percent EBL was 26.54 ± 11.02 and 27.12 ± 12.35 kg/m(2) in groups with and without pouch, respectively. The mean volume of the pouch after LSG was 17.13 ± 21.56 mm(3). Pouch volume, when present, was not significantly correlated to weight loss (P = 0.88 95% CI, CL 19.88-33.20 group 2; CL 22.94-31.30 group 1). CONCLUSIONS: No statistical correlation was found between the volume of the gastric pouch and weight loss (percent EBL) after LSG in symptomatic or with unsatisfactory weight loss patients.