SummaryThe age at which it is necessary to start Cardiovascular Magnetic Resonance (CMR) T2* screening in thalassaemia major (TM) is still uncertain. To clarify this point, we evaluated the prevalence of myocardial iron overload (MIO), function and fibrosis by CMR in TM patients younger than 10 years. We retrospectively selected 35 TM patients enrolled in the Myocardial Iron Overload in Thalassaemia network. MIO was measured by T2* multislice multiecho technique. Biventricular function parameters were evaluated by cine images. To detect myocardial fibrosis, late gadolinium enhancement images were acquired. Patients' age ranged from 4Á2 to 9Á7 years. All scans were performed without sedation. Nine patients showed no MIO, 22 patients had heterogeneous MIO with a T2* global value ≥20 ms; two patients had heterogeneous MIO with a T2* global value <20 ms and two patients showed homogeneous MIO. No patient showed myocardial fibrosis. Among the patients with heart T2*<20 ms, the youngest was 6 years old, none showed heart dysfunction and the iron transfused was <35 g in all cases. Cardiac iron loading can occur much earlier than previously described. The first cardiac T2* assessment should be performed as early as feasible without sedation, especially if chelation is started late or if poor compliance is suspected.
rimary chest wall tumors are rare and constitute 1% to 2% of all thoracic tumors. 1 In 15% to 20% of cases, they are asymptomatic neurogenic tumors, 2,3 and their presentation is often that of an incidental finding on chest radiography. 2Magnetic resonance imaging and computed tomography (CT) are commonly used in the diagnostic workup of these lesions.3,4 However, it is often difficult preoperatively to clarify whether they are benign or malignant, 1 and CT-guided needle biopsy can also be nondiagnostic. 3 We report a case in which contrast-enhanced ultrasonography (CEUS) and CEUSguided needle biopsy played a key role in diagnosing a schwannoma of the chest wall. Medicine (G.G., G.T., A.D.G., F.F.) and Pathology (G.Q., S.R.) Received June 4, 2009, from the Section of Interventional Ultrasound (P.T., S.S., S.P.) and Departments of Internal Case ReportA 78-year-old woman was admitted to our department for left-sided chest pain. Physical examination, electrocardiographic, and routine laboratory test findings were normal. Chest radiography showed prominence of the left superior mediastinum; the lung fields were clear. Contrast-enhanced CT of the thorax revealed a 4.5-cm slightly inhomogeneous, weakly enhanced extraparenchymal intrathoracic chest wall mass close to the fourth and fifth dorsal vertebrae (Figure 1). An ultrasonographic examination of the thorax was then performed with tissue harmonic imaging using a real-time ultrasonography system and a 5-MHz convex transducer (MyLab 70XVG; Esaote SpA, Genoa, Italy). Thoracic ultrasonography showed a 4-cm hypoechoic paravertebral mass indenting the pleural line in the fourth and fifth posterior intercostal spaces, with acoustic shadowing from nearby vertebrae in the medial margin (Figure 2).
The objective of this study was to identify prognostic factors for children and adolescents with relapsed or progressive classical Hodgkin’s lymphoma (cHL) to design salvage therapy tailored to them. We analyzed a homogeneous pediatric population, diagnosed with progressive/relapsed cHL previously enrolled in two subsequent protocols of the Italian Association of Pediatric Hematology and Oncology in the period 1996–2016. There were 272 eligible patients, 17.5% of treated patients with cHL. Overall survival (OS) and event-free survival (EFS) after a 10-year follow-up were 65.3% and 53.3%, respectively. Patients with progressive disease (PD), advanced stage at recurrence, and ≥5 involved sites showed a significantly worse OS. PD, advanced stage, and extra-nodal involvement at recurrence were significantly associated with a poorer EFS. Multivariable analysis identified three categories for OS based on the type of recurrence and number of localizations: PD and ≥5 sites: OS 34%; PD and <5 sites: OS 56.5%; relapses: OS 73.6%. Four categories were obtained for EFS based on the type of recurrence and stage: PD and stage 3–4: EFS 25.5%; PD and stage 1–2: EFS 43%; relapse and stage 3–4: EFS 55.4%; relapse and stage 1–2: EFS 72.1%. Patients with PD, in advanced stage, or with ≥5 involved sites had a very poor survival and they should be considered refractory to first- and second-line standard chemotherapy. Probably, they should be considered for more innovative approaches since the first progression. Conversely, patients who relapsed later with localized disease had a better prognosis, and they could be considered for a conservative approach.
Purpose We aimed to evaluate the near-final height (nFHt) in a large cohort of pediatricpatients with growth hormone deficiency (GHD) and to elaborate a new predictive method of nFHt. Methods We recruited GHD patients diagnosed between 1987 and 2014 and followed-up until nFHt. To predict the values of nFHt, each predictor was run in a univariable spline. ResultsWe enrolled 1051 patients. Pre-treatment height was −2.43 SDS, lower than parental height (THt) (−1.09 SDS, p < 0.001). The dose of recombinant human GH (rhGH) was 0.21mg/kg/week at start of treatment. nFHt was −1.08 SDS (height gain 1.27 SDS), higher than pre-treatment height (p < 0.001) and comparable to THt. 1.6% of the patients were shorter than −2 SDS from THt. The rhGH dose at nFHt was 0.19 mg/kg/week, lower than at the start (p < 0.001). The polynomial regression showed that nFHt was affected by gender, THt, age at puberty, height at puberty, age at the end of treatment (F = 325.37, p < 0.0001, R 2 87.2%). Conclusion This large national study shows that GHD children can reach their THt. The rhGH/kg/day dose significantly decreased from the start to the end of the treatment. Our model suggests the importance of a timely diagnosis, possibly before puberty, the beneficial effect of long-term treatment with rhGH, and the key-role of THt. Our prediction model has a very acceptable error compared to the majority of other published studies.
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