scholarly journals Long-term natural history of visual acuity in eyes with choroideremia: a systematic review and meta-analysis of data from 1004 individual eyes

2020 ◽  
pp. bjophthalmol-2020-316028
Author(s):  
Liangbo L Shen ◽  
Aneesha Ahluwalia ◽  
Mengyuan Sun ◽  
Benjamin K Young ◽  
Holly K Grossetta Nardini ◽  
...  
Keyword(s):  
Visual Acuity ◽  
Natural History ◽  
Primary Endpoint ◽  
Meta Analysis ◽  
Young Patients ◽  
Rapid Decline ◽  
Transition Age ◽  
Individual Level ◽  
History Of

Background/aimsBest-corrected visual acuity (BCVA) is the most common primary endpoint in treatment trials for choroideremia (CHM) but the long-term natural history of BCVA is unclear.MethodsWe searched in seven databases to identify studies that reported BCVA of untreated eyes with CHM. We sought individual-level data and performed segmented regression between BCVA and age. For eyes followed longitudinally, we introduced a horizontal translation factor to each dataset to account for different ages at onset of a rapid BCVA decline.ResultsWe included 1004 eyes from 23 studies. BCVA of the right and left eyes was moderately correlated (r=0.60). BCVA as a function of age followed a 2-phase decline (slow followed by rapid decline), with an estimated transition age of 39.1 years (95% CI 33.5 to 44.7). After the introduction of horizontal translation factors to longitudinal datasets, BCVA followed a 2-phase decline until it reached 0 letters (r2=0.90). The BCVA decline rate was 0.33 letters/year (95% CI −0.38 to 1.05) before 39 years, and 1.23 letters/year (95% CI 0.55 to 1.92) after 39 years (p=0.004).ConclusionBCVA in eyes with CHM follows a 2-phase linear decline with a transition age of approximately 39 years. Future trials enrolling young patients may not be able to use BCVA as a primary or sole endpoint, but rather, may need to employ additional disease biomarkers that change before age 39. BCVA may still have utility as a primary endpoint for patients older than 39 years who have measurable BCVA decline rates.

scholarly journals Long-term Natural History of Atrophy in Eyes with Choroideremia—A Systematic Review and Meta-analysis of Individual-Level Data

2020 ◽  
Vol 4 (8) ◽  
pp. 840-852 ◽  
Author(s):  
Liangbo L. Shen ◽  
Aneesha Ahluwalia ◽  
Mengyuan Sun ◽  
Benjamin K. Young ◽  
Holly K. Grossetta Nardini ◽  
...  
Keyword(s):  
Systematic Review ◽  
Natural History ◽  
Meta Analysis ◽  
Individual Level ◽  
Level Data ◽  
History Of

scholarly journals Bilateral visual acuity decline in males with choroideremia: a pooled, cross-sectional meta-analysis

2022 ◽  
Vol 22 (1) ◽  
Author(s):  
Duygu Bozkaya ◽  
Heng Zou ◽  
Cindy Lu ◽  
Nicole W. Tsao ◽  
Byron L. Lam
Keyword(s):  
Visual Acuity ◽  
Outcome Measure ◽  
Meta Analysis ◽  
Retinal Disease ◽  
Primary Outcome Measure ◽  
Cross Sectional ◽  
Data Set ◽  
Transition Age ◽  
Before And After ◽  
History Of

Abstract Background Choroideremia is a rare inherited retinal disease that leads to blindness. Visual acuity (VA) is a key outcome measure in choroideremia treatment studies, but VA decline rates change with age. An accurate understanding of the natural deterioration of VA in choroideremia is important to assess the treatment effect of new therapies in which VA is the primary outcome measure. We conducted a meta-analysis of data on individuals with choroideremia to determine the rate of VA deterioration between the better- and worse-seeing eye (BSE and WSE, respectively). Methods Data were collected from the prospective Natural History of the Progression of Choroideremia (NIGHT) study (613 eyes, baseline data only), studies included in a recent meta-analysis, and studies identified in a targeted literature search performed on March 25, 2020, including individual best-corrected VA (BCVA) and age data in male individuals with choroideremia. Best-corrected VA decline rates (measured by logMAR units) by age and trends in BCVA decline rates in the BSE and WSE were evaluated.  Results Data from 1037 males (1602 eyes; mean age, 41.8 years) were included. Before and after an age cutoff of 33.8 years, BCVA decline rates for the WSE were 0.0086 and 0.0219 logMAR per year, respectively. Before and after an age cutoff of 39.1 years, BCVA decline rates for the BSE were 0.00001 and 0.0203 logMAR per year, respectively. Differences in absolute BCVA and decline rates increased between the 2 eyes until age ~ 40; thereafter, differences in absolute BCVA and decline rates were similar between eyes. Conclusions Using the largest choroideremia data set to date, this analysis demonstrates accelerated BCVA decline beginning between 30 and 40 years of age. Disparate interocular progression rates were observed before the transition age, with similar interocular progression rates after the transition age.

scholarly journals Clinical profile & long-term natural history of symptomatic coronary artery disease in young patients (<30 yr)

2020 ◽  
Vol 152 (3) ◽  
pp. 263
Author(s):  
Harikrishnan Sivadasanpillai ◽  
Arun Gopalakrishnan ◽  
Sanjay Ganapathi ◽  
KrishnaKumar Mohanan Nair ◽  
Sivasankaran Sivasubramonian ◽  
...  
Keyword(s):  
Coronary Artery Disease ◽  
Coronary Artery ◽  
Natural History ◽  
Young Patients ◽  
Clinical Profile ◽  
History Of ◽  
Symptomatic Coronary Artery Disease ◽  
Artery Disease

scholarly journals Long-term natural history of idiopathic epiretinal membranes with good visual acuity

Eye ◽  
2019 ◽  
Vol 33 (5) ◽  
pp. 714-723 ◽  
Author(s):  
Kieu-Yen Luu ◽  
Tynisha Koenigsaecker ◽  
Amirfarbod Yazdanyar ◽  
Lekha Mukkamala ◽  
Blythe P. Durbin-Johnson ◽  
...  
Keyword(s):  
Visual Acuity ◽  
Natural History ◽  
Epiretinal Membranes ◽  
Good Visual Acuity ◽  
History Of

Natural history of adrenal incidentalomas with and without mild autonomous cortisol excess; a systematic review and meta-analysis

2018 ◽  
Author(s):  
Yasir Elhassan ◽  
Fares Alahdab ◽  
Alessandro Prete ◽  
Danae Delivanis ◽  
Aakanksha Khanna ◽  
...  
Keyword(s):  
Systematic Review ◽  
Natural History ◽  
Meta Analysis ◽  
Adrenal Incidentalomas ◽  
History Of

The natural history of pituitary apoplexy: long term follow-up study

2019 ◽  
Author(s):  
Ayesha Shaikh ◽  
Natasha Shrikrishnapalasuriyar ◽  
Giselle Sharaf ◽  
David Price ◽  
Maneesh Udiawar ◽  
...  
Keyword(s):  
Natural History ◽  
Pituitary Apoplexy ◽  
Follow Up Study ◽  
Long Term Follow Up ◽  
History Of

scholarly journals Natural History of NAFLD

2021 ◽  
Vol 10 (6) ◽  
pp. 1161
Author(s):  
Raluca Pais ◽  
Thomas Maurel
Keyword(s):  
Liver Disease ◽  
Natural History ◽  
Disease Progression ◽  
Specific Treatment ◽  
Alcoholic Fatty Liver ◽  
Individual Level ◽  
Close Relationship ◽  
History Of ◽  
The Individual ◽  
The Impact

The epidemiology and the current burden of chronic liver disease are changing globally, with non-alcoholic fatty liver disease (NAFLD) becoming the most frequent cause of liver disease in close relationship with the global epidemics of obesity, type 2 diabetes and metabolic syndrome. The clinical phenotypes of NAFLD are very heterogeneous in relationship with multiple pathways involved in the disease progression. In the absence of a specific treatment for non-alcoholic steatohepatitis (NASH), it is important to understand the natural history of the disease, to identify and to optimize the control of factors that are involved in disease progression. In this paper we propose a critical analysis of factors that are involved in the progression of the liver damage and the occurrence of extra-hepatic complications (cardiovascular diseases, extra hepatic cancer) in patients with NAFLD. We also briefly discuss the impact of the heterogeneity of the clinical phenotype of NAFLD on the clinical practice globally and at the individual level.

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