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( Pilar Brito Zeron ),( Marta Perez De Lis Novo ),( Pilar Rosich ),( Carles Tolosa ),( Joaquim Oristrell ),( Luis Saez Comet ),( Mercedes Perez Conesa ),( Jose Antonio Vargas Hitos ),( Jose Mario Sabi 대한내과학회 2014 대한내과학회 추계학술대회 Vol.2014 No.1
Background: To analyze the outcomes and therapeutic management of affected pregnancies with autoimmune congenital heart block (aCHB). Methods: Retrospective multicenter study in Internal Medicine Departments; inclusion criteria consisted of: aCHB of any type (I, II or III), fetal EFE and/or cardiomyopathy, cardiac block diagnosed in utero or in the first postpartum month, and mothers carrying anti-Ro52, Ro60 and/or La autoantibodies. Results: A total of 25 pregnancies with aCHB were retrospectively analyzed in 21 anti-Ro/La+ mothers. The mean maternal age at the time of pregnancy with aCHB was 33,25 years. Only 2 mothers received treatment prior to the first affected pregnancy (hydroxychloroquine and hydroxychloroquine+prednisone). Cardiac block consisted of type I (n=1), type II (n=6) and type III (n=18). At diagnosis of aCHB, 15/22 women were treated with dexamethasone (one of them, along with IVIG) and 2/22 with ritodrine. Preventive treatment with IVIG was administered in 2 pregnancies in which a recurrence was observed. Pregnancy was interrupted in 7/25 pregnancies at a mean week of 23.43 (18.2-37), while 18/25 of pregnancies gave a live birth baby with a mean age of 35.71 weeks of birth (30-40). 11 babies required pacemaker implantation, 9 immediately after birth and 2 in the neonatal period (1 at 5 years of age and another at 12). Of the 15 pregnancies with aCHB treated with dexamethasone, 12 achieved pregnancy to term (1 type II disappeared, with no changes in the remaining cases) and there were 3 fetal deaths despite treatment. Of the 7 pregnancies not treated with dexamethasone, 3 babies were born alive (no reversal of the blockade) and there were 4 fetal deaths. Conclusions: aCHB is a serious problem with a fetal mortality of 28% and a high requirement for neonatal pacemaker placement (61%).
Ribociclib-Related Stevens–Johnson Syndrome: Oncologic Awareness, Case Report, and Literature Review
Victoria López-Gómez,Ramón Yarza,Héctor Muñoz-González,Enrique Revilla,Santos Enrech,Olga González-Valle,Pablo Tolosa,Eva Ciruelos 한국유방암학회 2019 Journal of breast cancer Vol.22 No.4
Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis belong to a severe dermatopathic spectrum that includes frequently fatal mucocutaneous manifestations consisting of whole epidermal necrosis and sloughing with bullous transformation, blistering, and further skin detachment. Notably, cancer patients are at higher risk of developing SJS than the general population as a consequence of both the nature of neoplastic disease and frequent exposure to anticancer drugs. Ribociclib is a newly approved cycline-dependent kinase inhibitor that has been recently associated with a single case of SJS. We hereby present a case of ribociclib-related SJS. Early detection of threatening skin lesions is crucial to permit the immediate discontinuation of ribociclib given the predictable and unacceptable risk level. In cases of established SJS, early aggressive support should be initiated, ribociclib should be abruptly discontinued, and specific treatment based on actual evidence should be started.