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1.
Rev. chil. obstet. ginecol ; 78(6): 447-450, 2013. ilus
Article in Spanish | LILACS | ID: lil-702351

ABSTRACT

Objetivo: Comparar la morbilidad neonatal y a seis meses de vida de hijos de pacientes con isoinmunización Rh que recibieron al menos una transfusión intrauterina (TIU), con aquellos que no la requirieron. Método: Estudio de caso y control de pacientes con diagnóstico de isoinmunización Rh controladas en la Unidad de Medicina Fetal del Hospital Clínico Universidad de Chile. Se comparó el resultado perinatal y hasta 6 meses de vida de recién nacidos (RN) con TIU (9 casos) y sin TIU (14 casos) entre los años 2004 y 2009. Resultados: Aunque la sobrevida a los 6 meses de los fetos con TIU fue alrededor de un 80 por ciento, solo una muerte puede atribuirse a la severidad de su condición de base. Los RN con TIU nacieron a una menor edad gestacional que los que no requirieron este tratamiento (34,4 +/- 2,2 sem vs. 37,4 +/- 0,6 sem; p=0,003). Al evaluar el manejo neonatal inmediato se observa que el 60 por ciento de los RN isoinmunizados sin TIU requirieron ser hospitalizados y requirieron fototerapia, mientras que todos los RN con antecedente de TIU fueron hospitalizados, recibieron fototerapia y 30 por ciento requirió una exanguineo transfusión. A los 6 meses de vida, 75 por ciento y 20 por ciento de los RN isoinmunizados, con y sin TIU, fueron hospitalizados para una nueva transfusión de GR y/o fototerapia, respectivamente. Conclusión: La isoinmunización Rh es una patología de alto riesgo, pero la terapia intrauterina, en los casos con anemia moderada y severa, permite llegar a edades gestacionales que dan una adecuada sobrevida.


Objective: To compare neonatal and six months of life morbidity of babies affected by Rh isoimmunization during pregnancy that required at least one intrauterine blood transfusion, with babies that did not required that procedure. Methods: Case control study of patients with diagnosis of Rh isoimmunization under control in the Fetal Medicine Unit at the University of Chile Hospital. Perinatal and until 6 months of life outcomes of isoimmunized newborns (NB) with (9 cases) and without intrauterine transfusion (IUT) (14 cases) between years 2004 and 2009 were compared. Results: Although six months of life survival of IUT babies was about 80 percent only one death was related to the severity of isoimmunization. Isoimmunized babies with IUT were delivered at a lower gestational age than those without IUT (34.4 +/- 2.2 vs. 37.4 +/- 0.6 weeks; p=0.003). At the immediate neonatal period only 60 percent of isoimmunized babies without IUT required hospitalization and phototherapy, in contrast to IUT babies where all of them were hospitalized and required phototherapy, and 30 percent required exchange transfusion. Until six months of life, 75 percent and 20 percent of NB with and without IUT required another hospitalization for a new transfusion and/or phototherapy respectively. Conclusion: Rh isoimmunization is a high risk disease, but intrauterine therapy in cases with moderate and severe fetal anemia increases gestational age at delivery with good survival rates.


Subject(s)
Humans , Female , Pregnancy , Infant, Newborn , Blood Transfusion, Intrauterine , Rh Isoimmunization/therapy , Case-Control Studies , Pregnancy Outcome , Prognosis , Survival Analysis
2.
West Indian med. j ; West Indian med. j;61(1): 81-83, Jan. 2012.
Article in English | LILACS | ID: lil-672854

ABSTRACT

Gilbert syndrome (GS) is a hereditary relatively common benign unconjugated hyperbilirubinaemia. The promoter region of uridine diphosphate glycosyltransferase 1 (UGT1A1) gene contains a normal A(TA)6 TAA element; variations in this motif (A(TA)7/8 TAA) are generally associated with this disorder. This is a report of the varied effects of GS in a Mexican Mestizo family with a non-common (TA)8 repeat in this population. T he proposita and her mother showed (TA)7 /(TA)8 genotype, while her father and sister were (TA)6 /(TA)7 , but only the proposita showed clinical manifestations. This report supports that the (TA)7 and (TA)8 are necessary, but not enough to explain the features of GS. There are probably additional genetic variations ie, the presence of "modifier" genes or one can speculate that an oligogenetic trait can contribute to the expression of the final phenotype.


El síndrome de Gilberto (SG) es un hiperbilirubinemia no conjugada, benigna, relativamente común y hereditaria. La región promotora del gen (UGT1A1) de la uridina difosfato glicosiltransferasa 1, contiene un elemento normal A (TA)6 TAA. Las variaciones en este motivo (A (TA)7/8 TAA) se encuentran por lo general asociadas con este desorden. Éste es un reporte de los variados efectos del SG en una familia mestiza mexicana con una repetición (TA)8 no común en esta población. La probando y su madre mostraron el genotipo (TA)7 /(TA)7 , mientras su padre y hermana eran (TA)6 /(TA)7 , pero sólo la probando mostró manifestaciones clínicas. Este informe sostiene que el (TA)7 y (TA)8 son necesarios, pero no suficientes para explicar los rasgos del SG. Probablemente hay variaciones genéticas adicionales, es decir, la presencia de genes "modificadores", o se puede especular que un rasgo oligogenético puede contribuir a la expresión del fenotipo final.


Subject(s)
Adult , Female , Humans , Young Adult , Gilbert Disease/genetics , Glucuronosyltransferase/genetics , Alleles , Polymorphism, Genetic , Promoter Regions, Genetic
3.
Rev. Col. Bras. Cir ; 36(4): 339-346, jul.-ago. 2009. ilus, graf, tab
Article in Portuguese | LILACS | ID: lil-531029

ABSTRACT

OBJETIVO: Estudar a influência da icterícia obstrutiva sobre a capacidade reprodutiva e desenvolvimento fetal em ratas. MÉTODOS: Foram utilizadas 60 ratas sexualmente maduras e sabidamente férteis distribuídas em dois grupos: grupo 1 (n=30)- submetidas a ligadura do ducto biliopancreático e grupo 2 (n=30) -controles. A partir do 23? dia pós-operatório, as ratas foram acasaladas e seus ciclos estrais avaliados diariamente por meio de esfregaços vaginais, que permitiram determinar o dia da cópula e a idade gestacional em que foram mortas. Realizou-se estudo histológico dos corpos lúteos nos ovários de todas as ratas e analisou-se macroscopicamente a morfologia externa dos fetos. RESULTADOS: Observou-se que 23 ratas controle (92 por cento) e 11 ratas ictéricas (39,3 por cento) desenvolveram prenhez (p=0,0002). As 17 ratas com hiperbilirrubinemia e sem prenhez (60,7 por cento) apresentaram somente corpos lúteos com aspecto involutivo em seus ovários e sofreram modificações em seus ciclos estrais, permanecendo vários dias em proestro ou estro. As ratas prenhes com hiperbilirrubinemia não apresentaram alterações em seus corpos lúteos, porém os seus fetos eram anormais. CONCLUSÃO: Em presença de hiperbilirrubinemia, a fertilização é viável, a capacidade reprodutiva é muito reduzida, os ciclos estrais tornam-se irregulares, o epitélio vaginal permanece cornificado, os corpos lúteos ovarianos regridem, os corpos lúteos gravídicos não são alterados aumentando progressivamente durante a prenhez e o desenvolvimento fetal é gravemente alterado.


OBJECTIVE: To assess the influence of jaundice on the reproductive capacity and fetal development in rats. METHODS: 60 sexually mature rats were divided into two groups: Group 1 (n=30) - submitted to ligature of the biliopancreatic duct and Group 2 (n=30) -control- submitted only to sham operation. 23 days later, the animals were matted with sexually mature males for copulation. Vaginal smears were daily collected in order to verify the presence of spermatozoids and copula. The morphologic aspect of the ovaries and the corpi lutea diameter were studied. The morphology of the embryos of rats that became pregnant were assessed. Serum bilirubin levels were also determined. RESULTS: It was observed that 23 rats of the control group (92 percent) and 11 jaundiced rats (39.3 percent) became pregnant (p=0,0002). The 17 rats with hyperbilirubinemia that did not become pregnant (60.7 percent) present only involutive corpi lutea and had suffered modifications in their estrous cycles, remaining some days in proestro or estro. The pregnant rats with hyperbilirubinemia did not presented corpi lutea alterations, however their embryos were abnormal. CONCLUSION: In jaundice rats fertilization is viable, the reproductive capacity is intensive reduced, the estrus cycles becomes irregular, the corpi lutea is presented in regression, the gravidic lutea is not modified increasing gradually during the pregnancy and the fetal development is seriously impaired.


Subject(s)
Animals , Female , Rats , Fetal Development , Jaundice/complications , Jaundice/physiopathology , Ovary/anatomy & histology , Reproduction , Rats, Inbred Lew
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