scholarly journals A cross-sectional quantitative analysis of the natural history of Farber disease: an ultra-orphan condition with rheumatologic and neurological cardinal disease features

2017 ◽  
Vol 20 (5) ◽  
pp. 524-530 ◽  
Author(s):  
Matthias Zielonka ◽  
Sven F Garbade ◽  
Stefan Kölker ◽  
Georg F Hoffmann ◽  
Markus Ries
Author(s):  
Matthias Zielonka ◽  
Sven F. Garbade ◽  
Stefan Kölker ◽  
Georg F. Hoffmann ◽  
Markus Ries

2019 ◽  
Vol 14 (1) ◽  
Author(s):  
Robert J. Pignolo ◽  
Geneviève Baujat ◽  
Matthew A. Brown ◽  
Carmen De Cunto ◽  
Maja Di Rocco ◽  
...  

2018 ◽  
Vol 2018 ◽  
pp. 1-4 ◽  
Author(s):  
Ala I. Sharara ◽  
Nathalie Ziade ◽  
Rani H. Shayto ◽  
Luma Basma O. Rustom ◽  
Hamed Chehab ◽  
...  

Background. The natural history of colonic diverticulosis is unclear.Methods. Patients with incidental diverticulosis identified in a previous prospective cross-sectional screening colonoscopy study were evaluated retrospectively for clinic or hospital visit(s) for diverticular disease (DD= acute diverticulitis or diverticular bleeding) using review of electronic health records and patient phone interview.Results. 826 patients were included in the screening colonoscopy study. Three were excluded for prior DD. In all, 224 patients (27.2%; mean age 62.3 ± 8.2) had incidental diverticulosis distributed in the left colon (67.4%), right colon (5.8%), or both (22.8%). Up-to-date information was available on 194 patients. Of those, 144 (74.2%) could be reached for detailed interview and constituted the study population. Over a mean follow-up of 7.0 ± 1.7 years, DD developed in 6 out of 144 patients (4.2%) (4 acute cases of diverticulitis, 1 probable case of diverticular bleeding, and 1 acute case of diverticulitis and diverticular bleeding). Two patients were hospitalized, and none required surgery. The time to event was 5.1 ± 1.6 years and the incidence rate was 5.9 per 1000 patient-years. On multivariate analysis, none of the variables collected at baseline colonoscopy including age, gender, obesity, exercise, fiber intake, alcohol use, constipation, or use of NSAIDs were associated with DD.Conclusion. The natural history of incidental diverticulosis on screening colonoscopy was highly favorable in this well-defined prospectively identified cohort. The common scenario of incidental diverticulosis at screening colonoscopy makes this information clinically relevant and valuable to physicians and patients alike.


2015 ◽  
Vol 17 (12) ◽  
pp. 965-970 ◽  
Author(s):  
Konstantin Mechler ◽  
William K. Mountford ◽  
Georg F. Hoffmann ◽  
Markus Ries

2011 ◽  
Vol 34 (1) ◽  
pp. 75-91 ◽  
Author(s):  
Todd L. McKerchar ◽  
Edward K. Morris ◽  
Nathaniel G. Smith

2007 ◽  
Vol 7 ◽  
pp. 860-868 ◽  
Author(s):  
Alessandro Volpe

Incidentally detected, small renal masses (SRMs) have been increasing significantly in recent years due to the widespread use of improved cross-sectional imaging. A significant number of incidental SRMs are diagnosed in elderly patients who are more likely to undergo imaging for other medical issues. The natural history of SRMs has not been historically well understood because most masses are surgically removed soon after diagnosis.Several reports of surveillance of SRMs have been published in the last few years. When followed conservatively with serial imaging, SRMs have variable growth rates with an average of 0.28 cm/year, according to a recent meta-analysis. Larger series with longer follow-up are needed, but a significant number of small tumors seem to have an indolent behavior with a slow growth rate and a limited tendency to progress. The standard of care for enhancing SRMs is surgery. Up to one-third of surgically removed, <4-cm tumors are histologically benign. The outcomes of current surgical treatment of histologically confirmed, <4-cm, renal cell carcinomas are excellent, but this has not led to a decrease in mortality. Based on these considerations and on the available data on the natural history of SRMs, it seems reasonable to consider that we may be overtreating these lesions. This is especially true for elderly or unfit patients who have a decreased life expectancy. In these selected patients and in patients who refuse active treatment, it seems reasonable to propose an initial period of active surveillance for incidental SRMs, with delayed intervention for those tumors that will exhibit fast growth during follow-up. Percutaneous needle biopsies of renal tumors can be safely performed with the use of modern techniques and have the potential to characterize SRMs at histologically diagnosis, thereby allowing a better selection of the conservative or active treatment that is best suited for each individual patient.


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