ABSTRACT
UNLABELLED: PHACE syndrome comprises a spectrum of anomalies including posterior fossa malformations, haemangioma, arterial anomalies, cardiac defects and eye anomalies. PHACE should be considered in any patient with a large facial segmental infantile haemangioma (IH), and multidisciplinary management is crucial. Low-dose propranolol is effectively for the treatment of IH associated with PHACE syndrome. Recent evidence suggests IH is comprised of mesoderm-derived haemogenic endothelium. CONCLUSION: The embryonic developmental anomaly nature of IH provides an insight into the origin of PHACE syndrome.
Subject(s)
Aortic Coarctation , Eye Abnormalities , Neurocutaneous Syndromes , Adrenergic beta-Antagonists/therapeutic use , Aortic Coarctation/drug therapy , Aortic Coarctation/embryology , Eye Abnormalities/drug therapy , Eye Abnormalities/embryology , Female , Humans , Infant , Neurocutaneous Syndromes/drug therapy , Neurocutaneous Syndromes/embryologyABSTRACT
The authors present a case of PHACE(S) (posterior fossa malformations, hemangioma, arterial anomalies, cardiac defects, eye anomalies, and sternal cleft or supraumbilical raphe) syndrome with a right-sided segmental infantile hemangioma, and describe in detail, the associated absent ipsilateral intracranial internal carotid artery and anomalous Circle of Willis. Propranolol therapy led to accelerated, complete involution. Nadolol may reduce the theoretical risk of treating PHACE(S) patients with ß-blockers.
Subject(s)
Abnormalities, Multiple/diagnosis , Brain/abnormalities , Carotid Artery, Internal/abnormalities , Circle of Willis/abnormalities , Magnetic Resonance Imaging/methods , Female , Humans , Infant , Syndrome , Tomography, X-Ray ComputedABSTRACT
AIMS: Inadequate fruit and vegetable (F&V) consumption is an important dietary risk factor for disease internationally. High F&V prices can be a barrier to dietary intake and so to improve understanding of this topic we surveyed prices and potential competition between F&V outlet types. METHODS: Over a three week early autumn period in 2013, prices were collected bi-weekly for 18 commonly purchased F&Vs from farmers' markets (FM) selling local produce (n = 3), other F&V markets (OFVM) (n = 5), supermarkets that neighbored markets (n = 8), and more distant supermarkets (n = 8), (in urban Wellington and Christchurch areas of New Zealand). Prices from an online supermarket were also collected. RESULTS: A total of 3120 prices were collected. Most F&Vs (13/18) were significantly cheaper at OFVMs than supermarkets. Over half of the F&Vs (10/18) were significantly cheaper at nearby compared to distant supermarkets, providing evidence of a moderate 'halo effect' in price reductions in supermarkets that neighbored markets. Weekend (vs midweek) prices were also significantly cheaper at nearby (vs distant) supermarkets, supporting evidence for a 'halo effect'. Ideal weekly 'food basket' prices for a two adult, two child family were: OFVMs (NZ$76), online supermarket ($113), nearby supermarkets ($124), distant supermarkets ($127), and FMs ($138). This represents a savings of $49 per week (US$26) by using OFVMs relative to (non-online) supermarkets. Similarly, a shift from non-online supermarkets to the online supermarket would generate a $13 saving. CONCLUSIONS: In these locations general markets appear to be providing some substantially lower prices for fruit and vegetables than supermarkets. They also appear to be depressing prices in neighboring supermarkets. These results, when supplemented by other needed research, may help inform the case for interventions to improve access to fruit and vegetables, particularly for low-income populations.
