Summary
Peripheral T‐cell lymphomas (PTCLs) are a heterogeneous group of haematological cancers with generally poor clinical outcomes. However, a subset of patients experience durable disease ...control, and little is known regarding long‐term outcomes. The International T‐cell Lymphoma Project (ITCLP) is the largest prospectively collected cohort of patients with PTCLs, providing insight into clinical outcomes at academic medical centres globally. We performed a long‐term outcome analysis on patients from the ITCLP with available 10‐year follow‐up data (n = 735). The overall response rate to first‐line therapy was 68%, while 5‐ and 10‐year overall survival estimates were 49% and 40% respectively. Most deaths occurred prior to 5 years, and for patients alive at 5 years, the chance of surviving to 10 years was 84%. However, lymphoma remained the leading cause of death in the 5‐ to 10‐year period (67%). Low‐risk International Prognostic Index and Prognostic Index for T‐cell lymphoma scores both identified patients with improved survival, while in multivariate analysis, age >60 years and Eastern Cooperative Oncology Group performance status 2–4 were associated with inferior outcomes. The favourable survival seen in patients achieving durable initial disease control emphasizes the unmet need for optimal front‐line therapeutic approaches in PTCLs.
The International T‐cell Lymphoma Project is the largest prospectively collected cohort of patients with peripheral T‐cell lymphomas (PTCLs). In the long‐term outcome analysis of patients with available 10‐year follow‐up data (n = 735), 5‐ and 10‐year overall survival (OS) estimates were 49% and 40% respectively. For patients alive at 5 years, the chance of surviving to 10 years was 84%, but lymphoma remained the leading cause of death in the 5‐ to 10‐year period (67%). The favourable survival seen in patients achieving durable initial disease control emphasizes the unmet need for optimal front‐line therapeutic approaches in PTCLs.
Purpose
International guidelines recommend salivary cortisol for the diagnosis of Cushing’s syndrome. Despite mass spectrometry-based assays are considered the analytical gold-standard, there is ...still the need to define reference intervals and diagnostic accuracy of such methodology.
Methods
100 healthy volunteers and 50 consecutive patients were enrolled to compare LC–MS/MS and electrochemiluminescence assay for the determination of late-night salivary cortisol and cortisone. Moreover, we aimed to determine reference intervals of salivary steroids in a population of healthy individuals and diagnostic accuracy in patients with suspected hypercortisolism and in a population including also healthy individuals.
Results
Method comparison highlighted a positive bias (51.8%) of immunoassay over LC–MS/MS. Reference intervals of salivary cortisol (0.17–0.97 µg/L), cortisone (0.84–4.85 µg/L) and ratio (0.08–0.30) were obtained. The most accurate thresholds of salivary cortisol for the diagnosis of hypercortisolism were 1.15 µg/L in the population with suspected hypercortisolism (AUC 1) and 1.30 µg/L in the population including also healthy individuals (AUC 1). Cut-off values of salivary cortisone (7.23 µg/L; Se 92.9%, Sp 97.2%, AUC 0.960 and Se 92.9%, Sp 99.1%, AUC 0.985 in suspected hypercortisolism and in overall population, respectively) and cortisol-to-cortisone ratio (0.20; Se 85.7%, Sp 80.6%, AUC 0.820 and Se 85.7%, Sp 85.5%, AUC 0.855 in suspected hypercortisolism and in overall population, respectively) were accurate and similar in both populations.
Conclusion
LC–MS/MS is the most accurate analytical platform for measuring salivary steroids. Obtained reference intervals are coherent with previously published data and diagnostic accuracy for diagnosis of overt hypercortisolism proved highly satisfactory.
This study aimed to compare the efficacy of osteopathic manipulative therapy (OMTh) versus light touch therapy (LTT) in reducing cranial asymmetries in infants with nonsynostotic plagiocephaly (NSP).
...A prospective, parallel-group, single-center, LTT-controlled randomized clinical trial was conducted in the Department of Neonatology of Sant'Anna Hospital in Turin, Italy, from September 6, 2016 to February 20, 2020. We enrolled infants of 1 to 6 months of age with NSP, who were then randomly assigned to the study group (repositioning therapy plus six sessions of OMTh) or the control group (repositioning therapy plus six sessions of LTT). The outcome was the reduction of the oblique diameter difference index (ODDI) score <104%, which was assessed at the end of the intervention protocol (at 3 months) and at 1 year of age.
A total of 96 infants were randomized, 48 in the OMTh group and 48 in the LTT group, with mean ages of 3.1 versus 3.2 months, and baseline ODDI score of 110.2 versus 108.7%. In the OMTh group, a significant reduction of the ODDI score <104%, compared with the LTT group, was observed in the intension-to-treat (ITT) and per-protocol (PP) analyses. The ITT analysis revealed an ODDI score <104% in the OMTh group at 3 months (risk difference: 0.41; 95% confidence interval CI: 0.25-0.53;
< 0.001) and at the follow-up at 1 year of age (risk difference: 0.47; 95% CI: 0.31-0.64;
< 0.001). The PP analysis at 3 months reported a risk difference of 0.44 (95% CI: 0.27-0.60;
< 0.001), and at 1 year of age, a risk difference of 0.54 (95% CI: 0.36-0.72;
< 0.001).
In infants with NSP, a course of six OMTh sessions significantly reduced cranial asymmetries at both the 3-month and 1-year follow-up assessments, compared with LTT. This study is registered with ClinicalTrial.gov (identifier: NCT03970395; www.
gov ).
· OMTh plus repositioning therapy significantly decreased the risk and severity of NSP compared with LTT.. · OMTh reduced mild and moderate cranial asymmetries.. · The role of OMTh in severe cranial asymmetries should be investigated in a multicenter trial..
Aim of this retrospective-observational study was to evaluate fecal wet weight (WW), sodium (Na), and potassium (K) 24 h-excretions in patients with intestinal failure (IF) and insufficiency (INS), ...according to the residual length of short bowel, in the nutrition and clinical practice.
All in-patients with SBS – IICB, surgery > 3 months, admitted at the Unit of Clinical Nutrition were enrolled. They were divided into 3 groups according to clinical outcome as shown in table 1. Long term NPD treatment was administered in groups A and B. Pts in group C had an intestinal insufficiency that allowed them to preserve oral autonomy
All patients had a free oral diet without significant difference between the groups. At each stage, fecal WW, sodium and potassium were evaluated on three-days collections and mean values were calculated. Ion concentration was determined according to Mayo Clinic Laboratory method performed on the Roche Cobas 6000 (Voskoboev 2015).
Twenty five pts (M11/F14), age 54.6 ±28 yrs, were enrolled. Study results are shown in the table 2. Only in group B significant reductions of WW and Na were shown, contextually with intestinal rehabilitation.
Data suggest that WW and sodium excretion could be decisive biomarkers in the nutritional approach of these patients.
The spectrophotometric assay performed by Roche Cobas 6000, represents a simple and fast laboratory analysis which allows the clinician to have a routine tool in clinical management of patients.
Bone fibrous dysplasia is one of the main features of McCune-Albright syndrome, a rare genetic condition caused by constitutive activating mutations of Gs-protein and defined by skin dysplasia, bone ...fibrous dysplasia, and autonomous multiple endocrinopathies. Raised serum alkaline phosphatase (ALP) and urinary hydroxyproline levels indicating bone metabolic hyperactivity have been reported in these patients. Encouraging therapeutic results have been achieved, mainly in adults, with pamidronate, an aminobisphosphonate. In this study we investigate newer bone metabolic indices in a cohort of 11 children and adolescents treated with pamidronate. Tenfold increases of bone ALP and urinary pyridinoline cross-links were found and osteocalcin levels were twofold higher compared with reference values. After treatment, significant decreases in bone ALP and cross-links (Wilcoxon test P < 0.06) were found. Bone mineral density (BMD) significantly increased during treatment. There were signs of radiological healing as thickening of the cortical bone was found in some cases.
It is well known that change in apoptosis may modulate the natural story of illness, and that many drugs may act through modulation of apoptosis, but the role of steroids in acting through apoptosis ...in different settings, including renal diseases, has still to be elucidated. We studied the in vivo effects of steroids by oral assumption (10 to 25 mg/deltacortene) or by intravenous pulses (300 to 1000 mg/dose) on apoptosis and cellular subsets of peripheral lymphocytes, by evaluating DNA-fragmentation and lymphocyte subsets in 79 subjects: 22 controls and 57 patients with various renal diseases (25 Lupus-GN, 19 membranous-GN (MGN), 6 rapidly progressive-GN (RPGN), 2 acute interstitial nephritis (AIN), 5 on chronic dialysis. Baseline apoptosis was present in 1/22 (4.5%) of controls, 3/25 (12%) SLE, 2/6 (33.3%) RPGN and 10/19 (52.6%) MGN. A significant decrease in CD3+CD8+ cell count and a significant increase of the CD3+CD4/CD3+CD8+ ratio were found in apoptosis-positive subjects. DNA fragmentation did not change after oral steroids, paralleling a 22 to 32% decrease in total lymphocytes. Following intravenous methylprednisolone pulses, a deeper drop of all lymphocyte subsets was observed, while DNA fragmentation turned from present to absent in 2 MGN, but not in 2 RPGN, and from absent to present in 1 ARF and 1 SLE, independently of the dosage. We demonstrated that the presence of apoptosis in renal diseases is associated with decreased CD3+CD8+ cell count. Furthermore, steroid intravenous pulses, besides inducing a profound decrease in lymphocyte subsets, do exert a dual effect on baseline leukocyte apoptosis, eventually leading to a reversal of baseline patterns, either turning from negative to positive or from positive to negative. Oral steroid therapy did not influence baseline apoptosis.
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