BACKGROUNDPatients older than 55 years of age with acute myelogenous leukemia (AML) are less likely to achieve complete remission and more likely to experience toxicity with conventional induction chemotherapy than younger patients. Azacitidine administered in the outpatient setting is well tolerated and can induce complete hematological remission in patients with myelodysplastic syndromes (MDS). At higher doses, azacitidine has activity in AML.METHODSTwenty patients were retrospectively identified who had been treated with azacitidine with bone marrow blast counts between 21 and 38%. Patients with blast counts up to 29% were initially treated as MDS, but by WHO now meet criteria for AML. Patients with blast counts over 29% were treated with azacitidine after being deemed poor candidates for induction chemotherapy. Azacitidine 75 mg/m2/day was administered subcutaneously for 7 days every 4 weeks, which was defined as 1 cycle.RESULTSThe overall response rate was 60% (12/20): complete response (CR; n = 4; 20%); partial response (PR; n = 5; 25%); hematologic improvement (HI; n = 3; 15%). The median survival of responders was 15+ months compared with 2.5 months for nonresponders (P = .009). During therapy, responders had an Eastern Cooperative Oncology Group (ECOG) performance status of 1 or 0. The most common toxic event was infection (n = 8). Four patients were hospitalized during the first cycle of treatment.CONCLUSIONSAzacitidine administered in the outpatient setting can induce remission in AML. The therapy is well tolerated and might be an alternative for patients unlikely to tolerate standard induction chemotherapy. Cancer 2006. © 2006 American Cancer Society.

The name Fryns syndrome was given to a new variable multiple congenital anomaly syndrome, almost always lethal, described in 1978, and now known to be autosomal recessive. Since that date, 20 patients have been reported in the literature. We describe 8 new cases, 6 of which were diagnosed in a series of 112276 consecutive births (livebirths and perinatal deaths). The prevalence of this syndrome can be estimated to be around 0.7 per 10000 births. These new cases confirm that the most frequent anomalies are diaphragmatic defects, lung hypoplasia, cleft lip and palate (often bilateral), cardiac defects (septal defects and aortic arch anomalies), renal cysts (type II, III or IV), urinary tract malformations, and distal limb hypoplasia. Most patients also have hypoplastic external genitalia and anomalies of internal genitalia (bifid or hypoplastic uterus, immature testes). The digestive tract is also often abnormal: duodenal atresia, pyloric hyperplasia, malrotation and common mesentery are present in half of the patients. When the brain was examined, more than half were abnormal (Dandy‐Walker anomaly and agenesis of corpus callosum). A few patients demonstrated cloudy cornea. We examined the eyes of three patients histologically: two of them showed retinal dysplasia with rosettes and gliosis of the retina, thickness of posterior capsula of lens and irregularities of the Bowman membrane. Four of our cases were diagnosed prenatally between 24 and 27 weeks. It is to be expected that prenatal diagnosis will be made often and earlier in the future, as the spectrum of anomalies of the Fryns syndrome can easily be evidenced by sonography.

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Across human history, civilizations have responded to disasters and outbreaks of disease with increasingly complex, systematic approaches as a means of organizing chaos and protecting human life. The SARS-CoV-2 coronavirus (COVID-19) pandemic provides a unique opportunity to learn from the practice of disaster management and crisis-driven changes to patient care processes in hospital and emergent care environments worldwide. COVID-19 acts as an accelerant for process change and the need for redesign in systems where classical, linear evaluation methods most often inform carefully implemented service improvements. Strikingly, many innovative approaches and valuable lessons come from all over the globe where technology and access to resources have been most limited. This article answers the question, what can we learn about how to respond to future disasters from the evolution of disaster management as performed by helping professionals and policymakers during the past hundred-plus years and best practices seen today?Macro practitioners have co-created unique approaches within several global communities to help cope with COVID-19 and other disasters despite limited resources and seemingly unlimited needs. Referencing existing case studies of patient care responses during COVID-19 in Italy, Nigeria, South Africa, South Korea, and the United States, the authors document innovative practices and use of diverse technologies in local patient care systems. The article concludes by suggesting best practices for designing more robust, adaptive, and crisis ready responses to patient care, as well as the use of developmental evaluation as an agile approach to evaluating and improving patient services. It also suggests roles that helping professionals can play in the translation of big data systems of disaster management from organizations such as the Center for Disease Control, World Health Organization, non-governmental organizations (NGOs), and selected think tanks, among others.