Nitisinone, although unapproved for use in alkaptonuria (AKU), is currently the only homogentisic acid lowering therapy with a potential to modify disease progression in AKU. Therefore, safe use of nitisinone off-label requires identifying and managing tyrosine keratopathy. A 22-year-old male with AKU commenced 2 mg daily nitisinone after full assessment. He was issued an alert card explaining potential ocular symptoms such as red eye, tearing, ocular pain and visual impairment and how to manage them. On his first and second annual follow-up visits to the National Alkaptonuria Centre (NAC), there was no corneal keratopathy on slit lamp examination. On his third follow-up annual visit to the NAC, he was found to have typical dendritiform corneal keratopathy in both eyes which was asymptomatic. Nitisinone was suspended until a repeat slit lamp examination, 2 weeks later, confirmed that the keratopathy had resolved. He recommenced nitisinone 2 mg daily with a stricter low protein diet. On his fourth annual follow-up visit to the NAC, a routine slit lamp examination showed mild corneal keratopathy in the left eye. This is despite him reporting no ocular symptoms. This case highlights the fact that corneal keratopathy can occur without symptoms and any monitoring plan with off-label use of nitisinone in AKU will need to take this possibility into account. This is also the first time that typical corneal keratopathy has been described with the use of low dose nitisinone in AKU without symptoms.
We describe a patient with ultra-rare disease, alkaptonuria, who developed tyrosine keratopathy following nitisinone therapy of 2 mg on alternate days. His vision became impaired approximately 7 weeks following the commencement of nitisinone and ophthalmological examination at week nine showed characteristic dendritic keratopathy associated with tyrosinaemia. The corneal lesion as well as his visual symptoms normalized completely following discontinuation of nitisinone. This is the first documented report of keratopathy due to acquired tyrosinaemia due to very low-dose nitisinone.
The lion (Panthera leo) is an iconic resident of zoos and wild animal parks throughout the world. Regular assessment of the morbidity of captive lions is necessary to address wellness concerns and improve the healthcare and management of this vulnerable species. In an effort to understand disease morbidity broadly and guide future inquiries into captive lion health, we distributed a questionnaire that emphasized diseases of organ systems rather than individual diagnoses. We sent the questionnaire to 108 American Zoo and Aquarium Association (AZA) institutions housing lions between 2001 and 2016. Fifty-six facilities responded of which 32 responses had usable data for 111 lions. Responses were compiled and analyzed with respect to age, class, and sex. Neoplasia, renal disease, and dental disease were the most common causes of morbidity in these captive lions. Older animals generally experienced a higher prevalence of multiple types of disease. Three cases of hypertrophic cardiomyopathies (HCM) were reported in nearly 40% (3/8) of cardiovascular diseases and 3% (3/111) of disease cases overall, which is the first report of HCM in lions, to the authors' knowledge. There is a relative paucity of literature on lion and large felid cardiovascular conditions, which suggests that this may be an understudied area of lion health. This broad assessment of morbidities present in captive lion populations aimed to identify key areas for further research and screenings. Management priorities of captive lions should include prevention strategies for dental disease and trauma, frequent screening for renal disease and neoplasia, and further research into cardiovascular health.
Healthcare providers and patients increasingly turn to the Internet—websites as well as social media platforms—for health-related information and support. Informed by research on audience behaviors and preferences related to digital health information, the National Kidney Disease Education Program (NKDEP) developed a comprehensive and user-friendly digital ecosystem featuring content and platforms relevant for each audience. NKDEP's analysis of website metrics and social media conversation mapping related to chronic kidney disease revealed gaps and opportunities, informing the development of a digital strategy to position NKDEP as a trustworthy digital source for evidence-based kidney disease information. NKDEP launched a redesigned website (www.nkdep.nih.gov) with enhanced content for multiple audiences as well as a complementary social media presence on Twitter and Facebook, serving to drive traffic to the website as well as actively engage target audiences in conversations about kidney disease. The results included improved website metrics and increasing social media engagement among consumers and healthcare providers. NKDEP will continue to monitor trends, explore new directions, and work to improve communication across digital platforms.
Background: To date there has been no randomised controlled trial demonstrating the safety and efficacy of macular relocation surgery (MRS) for age related macular degeneration (AMD). Vision can be improved in some patients and made worse in others despite successful surgery or because of complications. Purpose: To determine which patients would benefit from MRS. Methods: Twenty nine patients with exudative AMD took part in a prospective, non-comparative, interventional study. Macular relocation surgery involved phacoemulsification, vitrectomy, 360˚retinotomy, excision of choroidal neovascular membrane, and macular relocation using an infusion of 5-fluorouracil and low molecular weight heparin as adjuvant to prevent proliferative vitreoretinopathy. Patients underwent protocol refraction preoperatively and six-monthly postoperatively by designated optometrists. Preoperative fundus fluorescein angiograms were read by masked observers and the lesions were classified according to a set protocol. The main outcome measures were visual improvement, final vision of better than 20/400, reading speed, critical print size. Logistic and multiple stepwise linear regressions were used to identify independent factors which predicted the main outcomes. Results: Preoperative visual acuity (20/120 or worse) and lesion type (predominantly classic or submacular haemorrhage) were significantly associated with visual improvement (coefficient of regression B = 26.8, p,0.001 and B = 14.9 with p = 0.045 respectively). There were no significant independent factors which predicted a final distance logMAR visual acuity of 1.3 (20/400) or any arbitrary definition of blindness. Conclusions: The study showed that it was possible to select cases that were more likely to experience an improvement in vision following MRS.
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