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Palermo, Italy

Vultaggio A.,Immunoallergology Unit | Azzari C.,Anna Meyer Childrens Hospital | Milito C.,University of Rome La Sapienza | Toppino C.,University of Turin | And 7 more authors.
Clinical Drug Investigation | Year: 2015

Background and Objectives: Subcutaneous immunoglobulin (SCIG) therapy is becoming increasingly popular as self-administration is possible because intravenous access is unnecessary, and there is a lower frequency of systemic adverse events. The aim of this study was to evaluate the shifting from intravenous immunoglobulins (IVIGs) replacement therapy to SCIG in patients with primary immunodeficiency (PID) in a routine real-life situation.Methods: In a multicenter prospective observational study, we enrolled 50 patients suffering from PID who were monitored for 24 months; 44 patients switched from IVIG and six from different SCIG preparations. The study preparation (human IgG 16 %, Vivaglobin®, CSL Behring GmbH, Germany) was subcutaneously infused weekly (maximum volume 15 mL/site; maximum infusion rate 22 mL/h). The study endpoints were: annual rate of severe bacterial infections (SBIs), local adverse reactions, quality of life, days off school/work, and days of hospitalization.Results: Thirty-three of 39 (84.6 %) patients who completed the study experienced an infection or signs thereof. Only five SBIs were observed, corresponding to an annual rate of 0.056 episodes per patient in 44 subjects [intention-to-treat (ITT) population]. A significant decrease in both days of hospitalization (1.93 ± 4.08 vs. 0.64 ± 2.94) and days off school/work (15.27 ± 23.17 vs. 2.26 ± 4.45) was recorded at 24 months. Local reactions were observed in 14/50 (28 %) patients, mainly consisting of skin manifestations at the injection site. Only three (6.8 %) patients discontinued due to infusion site reactions. In patients shifting from IVIG to SCIG, the total mean score of Life Quality Index (LQI) improved from 76.9 ± 16.8 to 90.7 ± 11.6 (P < 0.01) at 6 months; there was an improvement also in the overall patients’ evaluation.Conclusions: A total of 93.2 % patients tolerated the new route of administration and reported a significant improvement in their LQI. Our results from a routine clinical practice in a real-life population are consistent with those of phase III clinical studies. © 2015, The Author(s).

Pojero F.,University of Palermo | Casuccio A.,University of Palermo | Di Bassiano F.,ARNAS Civico | Gervasi F.,Uosd Laboratorio Specialistico Oncologia | And 2 more authors.
Immunity and Ageing | Year: 2015

Background: Multiple myeloma (MM) is a neoplastic disorder of plasma cells interesting mainly the elderly. MM remains an incurable disease, mostly because of the strong interplay between clonal plasma cells (cPCs) and bone marrow (BM) microenvironment. Multiparameter flow cytometry (MFC) allows the simultaneous study of the cPC immunophenotype and alterations involving other cells in BM, but rarely these data are interpreted as connected. One exception to this habit are previous studies about relationship between CD117 cPC positivity and hematopoietic progenitor cell (HPC) distribution in newly diagnosed patients. Thus we were interested in verifying the distribution of BM CD34+ HPCs in healthy controls, and monoclonal gammopathy of undetermined significance (MGUS) patients and various categories of responding/relapsing MM subjects divided according to CD117 positivity. Results: Our data completely agree with precedent reports as regards untreated patients. In the group with progression of disease, CD117- patients exhibited a lower CD34 + CD19-/CD34 + CD19+ ratio vs CD117+ subjects. Among CD117- cases, newly diagnosed patients exhibited differences in distribution of HPCs vs responding myeloma subjects and patients with progressive disease. These differences reached statistical significance comparing CD117- newly diagnosed with CD117- responding cases, as reflected by CD34 + CD19-/CD34 + CD19+ ratio. In turn, no differences emerged comparing CD117+ treated and untreated patients. Conclusions: We demonstrate that administration of treatment and depth of reached response/presence of relapse imply a distinct regulation in distribution of CD34+ HPC subsets in CD117- and CD117+ patients. These differences become evident comparing untreated and treated CD117- patients, but they are impossible to detect in CD117+ cases. © 2015 Pojero et al.

Perino A.,University of Palermo | Forlani F.,University of Palermo | Lo Casto A.,University of Palermo | Cali G.,ARNAS Civico | And 3 more authors.
Gynecological Surgery | Year: 2014

To comment on the prevalence, diagnosis, and treatment of the septate uterus, with special reference to hysteroscopic metroplasty and its effect on reproductive outcome, we searched publications in PubMed and Embase. Original articles, meta-analysis, reviews, and opinion articles were selected. The studies suggest that the prevalence of the septate uterus is increased in women with repeated pregnancy loss and infertility. Reliable diagnosis depends on accurate assessment of the uterine fundal contour and uterine cavity by means of magnetic resonance and three-dimensional ultrasound. Pertinent published data comparing pregnancy outcome before and after hysteroscopic metroplasty indicated a marked improvement after surgery. Magnetic resonance and three-dimensional ultrasound represent the gold standard for diagnosis of septate uterus. Hysteroscopic metroplasty with its simplicity, minimal postoperative sequelae, and improved reproductive outcome is the gold standard for treatment, not only in patients with recurrent pregnancy loss and premature labor but also in patients with infertility, especially if in vitro fertilization is being contemplated. © 2014 Springer-Verlag.

Terenziani M.,Fondazione Istituto Nazionale Dei Tumori | D'Angelo P.,ARNAS Civico | Inserra A.,Ospedale Pediatrico IRCCS Bambino Gesu | Boldrini R.,Ospedale Pediatrico IRCCS Bambino Gesu | And 12 more authors.
Pediatric Blood and Cancer | Year: 2015

Background: Teratomas demonstrate a benign clinical behavior, however they may recur with malignant components or as teratoma, and in a small group of patients prognosis could be fatal. After the first Italian study, we collected cases of teratoma, alongside the protocol for malignant germ cell tumors. Procedure: Patients with teratoma were collected from 2004 to 2014. Teratomas were classified according to the WHO classifications, as mature and immature. Patients with pathological aFP and/or bHCG, and those with a malignant germ cell component were not included. Results: The study enrolled 219 patients (150 mature, 69 immature teratomas) with a median age at diagnosis of 42 months. The primary sites involved were: 118 gonadal and 101 extragonadal teratomas. Two females with ovarian teratoma had a positive family history. Complete and incomplete surgeries were performed in 85% and 9% of cases. Seventeen events occurred: six females had a second metachronous tumor (5 contralateral ovarian teratoma, 1 adrenal neuroblastoma) and 11 teratomas relapsed/progressed (3 mature, 8 immature teratomas). Two patients died, one of progressive immature teratoma and one of surgical complications. At a median follow up of 68 months, the event-free, relapse-free, and overall survival rates were 90.6%, 94.3%, 98.6%, respectively. Conclusions: Teratomas show a good prognosis, especially the mature ones: surgery and follow-up remain the standard approach. Incomplete surgery in immature teratoma is the group at greatest risk of relapse. Bilateral ovarian tumors are a possibility, and the rare family predisposition to ovarian mature teratoma warrants further analyses. Pediatr Blood Cancer 2015;62:1202-1208. © 2015 Wiley Periodicals, Inc.

Cali G.,ARNAS Civico | Giambanco L.,ARNAS Civico | Puccio G.,University of Palermo | Forlani F.,University of Palermo
Ultrasound in Obstetrics and Gynecology | Year: 2013

Objectives To evaluate the diagnostic accuracy of two-dimensional (2D) gray-scale and color Doppler and three-dimensional (3D) power Doppler sonographic criteria for morbidly adherent placenta (MAP), and to identify criteria to help distinguish placenta accreta from placenta percreta. Methods We enrolled 187 patients with placenta previa and history of uterine surgery and performed transabdominal and transvaginal ultrasound examination for early detection of MAP. With 2D gray-scale transabdominal and transvaginal ultrasonography, we investigated loss/irregularity of the echolucent area between the uterus and the placenta ('clear space'), thinning or interruption of the hyperechoic interface between the uterine serosa and the bladder wall and the presence of turbulent placental lacunae with high-velocity flow (>15 cm/s). Using transabdominal 3D power Doppler, we evaluated the hypervascularity of the uterine serosa-bladder wall interface and irregular intraplacental vascularization. Ultrasound findings were reviewed against the final diagnosis made during Cesarean section (CS). Results MAP was detected on CS in 41 patients. All of them had an anterior placenta previa (34 major and seven minor) and had undergone at least one previous CS. The evaluated sonographic criteria showed good diagnostic performance; in MAP patients at least two out of five criteria were detected, with at most one of the criteria present in patients without MAP. Loss/irregularity of clear space used as a single criterion was responsible for the most false positives, demonstrating a low positive predictive value. Irregular intraplacental vascularization with tortuous confluent vessels affecting the entire width of the placenta, and hypervascularity of the entire uterine serosa-bladder wall interface, were only detected, on 3D power Doppler, in cases of placenta percreta. Conclusions The reviewed ultrasound criteria may be useful for the prenatal diagnosis of MAP and to differentiate between placenta accreta and placenta percreta; 3D power Doppler techniques were an important aid in the diagnosis. Copyright © 2013 ISUOG. Published by John Wiley & Sons, Ltd.

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