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lifetimes and spectral features of soft drusen and hyperpigmentation in age-related macular degeneration.

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BackgroundMutations in the CRB1 gene are associated with variable phenotypes of severe retinal dystrophies, and retinal dystrophies resulting from CRB1 mutations may be accompanied by specific fundus features such as coat’s like vasculopathy in retinitis pigmentosa patients. This is the first report of the occurrence of coat’s like vasculopathy in a patient diagnosed with Leber congenital amaurosis caused by a CRB1 mutation.Case presentationAn 18-year old Syrian female patient presented with bilateral gradual loss of vision since early childhood, with recent deterioration in her left eye. She appeared to have an asymmetric bilateral coat’s like vasculopathy which was more severe in the left eye. The diagnosis of Leber congenital amaurosis was suggested, and a genetic CRB1 sequencing for the patient and her two younger siblings, who also had severe vision loss, was done, upon which the diagnosis of Leber congenital amaurosis associated with exudative retinal detachment due to coat’s like vasculopathy was made. Treatment with panretinal photocoagulation was attempted in the worse left eye, but with no improvement. As the disease suddenly progressed in both eyes, pars plana vitrectomy with endolaser and silicone oil tamponade was performed in the better right eye which led to anatomical stabilization of the case without improvement in the visual acuity.ConclusionLeber congenital amaurosis is reported to be associated with multiple systemic and ocular findings, none of which is coat’s like vasculopathy. CRB1 gene mutations are associated with remarkable retinal findings in patients with retinitis pigmentosa and other fundus dystrophies. In this unique case we are reporting the incidence of coat’s like vasculopathy in a patient diagnosed with Leber congenital amaurosis caused by CRB1 gene mutation, and its management. CRB1 mutant patients should be followed up closely as sudden progression can have permanent poor outcomes and as early management is vital in such cases.

Background Congestive heart failure (CHF) is the most common cause of hospitalization in the US for people older than 65 years of age. It has the highest 30-day re-hospitalization rate among medical and surgical conditions, accounting for up to 26.9% of the total readmission rates. We conducted a quality improvement project at our hospital with the objective to reduce the 30-day all-cause readmissions of patients with CHF by improving the transition of care and setting up scheduled follow-up appointments within two weeks of patient discharge. Method Retrospective data were collected to understand the pattern of admission for CHF during November 2017. Data on 30-day readmission post-discharge was also collected to understand readmission rates. Similarly, all patients who were admitted with acute CHF exacerbation to our hospital during the month of November 2018 were included in our intervention cohort. The 30day readmission rates of these patients post-intervention were calculated and compared to the initial cohort. Results As part of our study, we ensured that 58% of the enrolled patients had a follow-up appointment scheduled within two weeks of discharge compared to only 30% in 2017. Also, 56% of the enrolled patients kept their follow-up appointments compared to 37% in 2017. The 30-day readmission rate of CHF patients was reduced in half after the implementation of our project, with a 14% readmission rate for our study patients compared to 28% in 2017. Conclusion Patient education and measures to augment post-discharge follow-up appointments can lead to substantial reductions in the readmission rates of heart failure (HF) patients.