A Framework for Understanding and Improving Environmental Decision Making

This paper presents a framework for understanding and improving public sector environmental decision making. Within the framework, four interrelated components are discussed: (1) the environmental and cultural context-understanding this context includes understanding what people consider to be environmental problems, the goals and values that they bring to environmental problems and decision processes, specialized and common knowledge about environmental problems, and the institutional settings within which problems are addressed; (2) planning and appraisal activitiesthese activities include forecasting and monitoring exercises, evaluations of past decisions, and decisions that processes ought to be launched to solve specific environmental problems; (3) decision-making modes-these include six typical ways of conducting an environmental problem-solving process, modes which, in the framework, are called emergency action, routine procedures, analysis-centred, elite corps, conflict management and collaborative learning; (4) decision actions-these include five generic steps that are undertaken, formally or intuitively, in virtually any decision-making situation: issue familiarization; criteria setting; option construction; option assessment; and reaching a decision. In the course of describing the framework, we show a decision-making process can be adapted to incorporate sustainability concerns, including fostering sustainable environmental and social systems, meeting obligations to future generations, and searching for robust and reasonable (rather than rigidly optimal) decisions. The framework also helps to illuminate intriguing questions regarding institutional responsibility, decision process complexity and paradigms for environmental decision making.     

Etiology and management of early pregnancy renal anhydramnios: Is there a place for serial amnioinfusions?

Early pregnancy renal anhydramios (EPRA) comprises congenital renal disease that results in fetal anhydramnios by 22 weeks of gestation. It occurs in over 1 in 2000 pregnancies and affects 1500 families in the US annually. EPRA was historically considered universally fatal due to associated pulmonary hypoplasia and neonatal respiratory failure. There are several etiologies of fetal renal failure that result in EPRA including bilateral renal agenesis, cystic kidney disease, and lower urinary tract obstruction. Appropriate sonographic evaluation is required to arrive at the appropriate urogenital diagnosis and to identify additional anomalies that allude to a specific genetic diagnosis. Genetic evaluation variably includes karyotype, microarray, targeted gene testing, panels, or whole exome sequencing depending on presentation. Patients receiving a fetal diagnosis of EPRA should be offered management options of pregnancy termination or perinatal palliative care, with the option of serial amnioinfusion therapy offered on a research basis. Preliminary data from case reports demonstrate an association between serial amnioinfusion therapy and short-term postnatal survival of EPRA, with excellent respiratory function in the neonatal period. A multicenter trial, the renal anhydramnios fetal therapy (RAFT) trial, is underway. We sought to review the initial diagnosis ultrasound findings, genetic etiologies, and current management options for EPRA.     

A system-based approach to the genetic etiologies of non-immune hydrops fetalis

A wide spectrum of genetic causes may lead to nonimmune hydrops fetalis (NIHF), and a thorough phenotypic and genetic evaluation are essential to determine the underlying etiology, optimally manage these pregnancies, and inform discussions about anticipated prognosis. In this review, we outline the known genetic etiologies of NIHF by fetal organ system affected, and provide a systematic approach to the evaluation of NIHF. Some of the underlying genetic disorders are associated with characteristic phenotypic features that may be seen on prenatal ultrasound, such as hepatomegaly with lysosomal storage disorders, hyperechoic kidneys with congenital nephrosis, or pulmonary valve stenosis with RASopathies. However, this is not always the case, and the approach to evaluation must include prenatal ultrasound findings as well as genetic testing and many other factors. Genetic testing that has been utilized for NIHF ranges from standard chromosomal microarray or karyotype to gene panels and broad approaches such as whole exome sequencing. Family and obstetric history, as well as pathology examination, can yield additional clues that are helpful in establishing a diagnosis. A systematic approach to evaluation can guide a more targeted approach to genetic evaluation, diagnosis, and management of NIHF.