RESUMEN
BACKGROUND: Placenta accreta spectrum (PAS) has a high risk of maternal morbidity, and requires meticulous antenatal and peripartum management. AIMS: To compare the management and outcomes of PAS between women with and without antenatally suspected disease, and to evaluate the effect of multidisciplinary team (MDT) management. MATERIALS AND METHODS: A retrospective cohort study identified all hysterectomy specimens with a histopathological diagnosis of PAS in the Western Sydney Local Health District between January 2006 and December 2019, and analysed each patient's clinical course. RESULTS: Seventy patients had PAS diagnosed on hysterectomy specimens, of which 38 cases (54%) were antenatally suspected. Women with suspected PAS were more likely to have a previous caesarean section (100% vs 68%, P < 0.001), placenta praevia (92% vs 56%, P < 0.001) and anterior placenta (95% vs 66%. P = 0.011). Suspected PAS was associated with less maternal blood loss (median blood loss 2000 mL vs 4000 mL, P < 0.001), fewer red blood cell transfusions (median four units vs nine units, P < 0.001), and shorter intensive care or high dependency unit admission (mean stay one day vs three days, P = 0.037). There were no significant differences in other maternal morbidities. MDT management was associated with a clinically significant reduction in maternal blood loss (1500 mL vs 2520 mL, P = 0.09) and red blood cell transfusion (one unit vs six units, P = 0.04). The mean gestation of delivery was 37 weeks in both groups with no differences in neonatal morbidity. CONCLUSIONS: Both antenatally diagnosed PAS and MDT management reduced blood loss and blood transfusion. Elective delivery at 37 weeks gestation reduces the neonatal risks of preterm delivery.
Asunto(s)
Placenta Accreta , Placenta Previa , Hemorragia Posparto , Cesárea , Femenino , Humanos , Histerectomía , Recién Nacido , Placenta Accreta/cirugía , Hemorragia Posparto/epidemiología , Hemorragia Posparto/etiología , Hemorragia Posparto/terapia , Embarazo , Estudios RetrospectivosRESUMEN
Angelman syndrome (AS) is characterized by severe intellectual disability, limited, or absent speech and a generally happy demeanor. The four known etiological mechanisms; deletions, uniparental disomy, imprinting defects, and UBE3A mutation all affect expression of the UBE3A gene at 15q11-q13. An atypical phenotype is seen in individuals who are mosaic for a chromosome 15q11-q13 imprinting defect on the maternal allele. These patients present with a milder phenotype, often with hyperphagia and obesity or non-specific intellectual disability. Unlike typical AS syndrome, they can have a vocabulary up to 100 words and speak in sentences. Ataxia and seizures may not be present, and the majority of individuals do not have microcephaly. Here we review the current literature and present three individuals with atypical AS caused by a mosaic imprinting defect to demonstrate why DNA methylation analysis at the SNRPN locus needs to be considered in a broader clinical context. © 2017 Wiley Periodicals, Inc.