1,720,968 research outputs found

    Ultrasound in the management of hepatic veno-occlusive disease in three children treated with dactinomycin and vincristine.

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    Three children, treated with dactinomycin and vincristine without radiotherapy showed hepatic toxicity consistent with diagnostic criteria for hepatic veno-occlusive disease (VOD). Two patients were affected with Wilms' tumor and the third with malignant fibromatosis. The clinical manifestations of VOD were mild and regressed in all patients after supportive therapy. Serial ultrasonography (US) was performed in all cases and was useful in confirming the diagnosis and in evaluating the severity of the disease. US features of VOD were hepatomegaly, gallbladder wall thickening, ill-defined borders of the hepatic vessels, and ascites. Parenchymal heterogeneity was still present after several months as a result of the hepatic injury. The hazy appearance of the portal vessels and the gallbladder wall thickening seemed directly correlated with the degree of hepatic involvement in the early phase of VOD

    Hypersensitivity to carboplatin in children.

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    . Abstract: Background. Hypersensitivity reactions are rare bur at times severe complications to cytostatic drugs. Procedure. The percentage of allergic reactions to carboplatin and their clinical features were evaluated in 185 children affected by different so lid tumors and treated with etoposide-carboplatin chemotherapy. Allergic reactions that occurred during or immediately following etoposide infusion (5 cases, 2.8%) were excluded from the study. Results. Seventeen out of 185 patients (9.2%) suffered from allergic responses to carboplatin. The first of these occurred after an average of 10.1 courses (range, 1-23; median, 9). The risk calculated according to the number of courses is 2% at 6 courses, 11.3% at 12 courses, and 47% at more than 12 courses. Conclusions. The high risk of allergic reactions to multiple courses of carboplatin should be kept in mind when developing treatment regimens that include the drug

    Presentazione atipica di neuropatia ottica di leber

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    Presentazione atipica di neuropatia ottica di leber Di Maggio C 1 , Nardecchia F 1 , Carrozzo R 3 , Pucci C1 , Mattiucci C4 , Varrasso G 4 , De Negri A 2 , Plateroti R 5 , Torraco A 3 , Bertini E 3 , Leuzzi V 1 , Moramarco A 5 1DAI Neurosc e Sal Ment, Univ Sapienza, Roma, 2UOC Oculistica, Osp San Cam Forl, Roma, 3Un Mal Musc e Neurodeg, OPBG, Roma, 4DAI Mat Infant e Sc Urol, Univ Sapienza, Roma, 5DAI Testa Collo, Oftalm, Univ Sapienza, Roma INTRODUZIONE/BACKGROUND: La neuropatia ottica ereditaria di Leber (LHON) è una malattia mitocondriale a trasmissione matrilineare, a penetranza incompleta e insorgenza tra i 15 e i 35 anni, caratterizzata da deficit binoculare del visus, acuto/subacuto, simultaneo o sequenziale. L’esordio in età infantile è raro. METODI / PAZIENTI: Segnaliamo un bambino di 12 anni che ha manifestato in pieno benessere un deficit acuto del visus (OD 4/10, OS 6/10) e concomitante congiuntivite di ndd.Nonna e cugino sulla linea materna con pregressa neuropatia ottica di ndd, in un caso regredita spontaneamente. Il bambino ha sofferto di epilessia idiopatica, in remissione dall’età di 11 anni. L’esame neurooftalmologico all’esordio mostrava assenza di deficit pupillare afferente; FOO: papille ottiche diffusamente rilevate, teleangectasie presenti tra le fibre ispessite, vasi venosi congesti, piccole emorragie lungo le fibre del polo superiore (OD) e al polo infero-temporale (OS). Ishihara alterato. Scotoma centrocecale bilateralmente. OCT: ispessimento dello strato delle fibre nervose e del nervo ottico bilateralmente, riduzione dello strato delle cellule ganglionari asimmetrico (OD>OS). Fluorangiografia: tenue diffusione papillare nelle fasi tardive, modesto turgore diffuso dei vasi venosi, papilla ottica a margini sfumati, congestione dei grossi vasi, rete capillare accentuata ed ectasica. PEV Pattern: latenza aumentata, in OD a basse frequenze, in OS a medie frequenze. Nella norma: RMN encefalo (mdc), PEV Flash, ERG, CSF (pressione liquorale, esame chimico-fisico, Ab antiacquaporina4 e anti-MOG), sierologia per EBV, CMV, HSV1-2, Morbillo, VZV, Borrelia. RISULTATI: La diagnosi è stata effettuata mediante sequenziamento con metodo Sanger che ha evidenziato la mutazione T14484C nel gene MT-ND6 in omoplasmia. DISCUSSIONE: Questo caso è peculiare per la presentazione atipica con un(o) (pseudo)edema papillare che può essere causa di ritardo diagnostico e terapeutico

    Evaluation of Nephron-Sparing Surgery as Potential Risk Factor for Relapse in Unilateral Wilms Tumor

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    Background: The aim of the study was to assess the prognostic significance of nephron-sparing surgery (NSS) without tumor size limits as a risk factor for relapse in children with unilateral Wilms Tumor (WT). Methods: A 28-y retrospective single-center review was performed. Prognostic relevance of age, gender, stage, histology, nephrectomy (N), and NSS was analyzed. Results: Sixty-nine cases (42 females and 27 males) with WT, off-therapy from 21 to 325 mo after chemotherapy mainly based on the International Society of Pediatric Oncology trials, were treated at our institution. Five cases were excluded (three children with synchronous bilateral WT and two adults with unilateral WT). Of 64 children with unilateral WT, 51 underwent N and 13 NSS without tumor size limits. Indeed, two-thirds of children who underwent NSS presented with a tumor diameter >4 cm. Overall, nine patients (14%) had a relapse (male-to-female ratio = 1:8). Initial surgery was N in eight cases and NSS in another one. Relapse rates in N and NSS groups were 15.7% and 7.7% (P = nonsignificant), respectively; the relapse rates in N and NSS groups were 8.6% and 7.7% (P = nonsignificant) for stages I-II unilateral WT cohort, respectively. On univariate analysis, factors correlated with probability of relapse were unfavorable histology (P < 0.002) and stage III disease (P < 0.01). Conclusions: In unilateral WT, NSS, whenever feasible, does not seem to increase the risk of recurrence. A multicenter prospective trial is required to carefully evaluate this risk

    Going Beyond Counting First Authors in Author Co-citation Analysis

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    The present study examines one of the fundamental aspects of author co-citation analysis (ACA) - the way co-citation counts are defined. Co-citation counting provides the data on which all subsequent statistical analyses and mappings are based, and we compare ACA results based on two different types of co-citation counting - the traditional type that only counts the first one among a cited work's authors on the one hand and a non-traditional type that takes into account the first 5 authors of a cited work on the other hand. Results indicate that the picture produced through this non-traditional author co-citation counting contains more coherent author groups and is therefore considerably clearer. However, this picture represents fewer specialties in the research field being studied than that produced through the traditional first-author co-citation counting when the same number of top-ranked authors is selected and analyzed. Reasons for these effects are discussed
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