Presentation
Pigmented paravenous retinochoroidal atrophy is an uncommon fundus disorder with a striking clinical presentation. [ncbi.nlm.nih.gov]
In the present case the visual acuity at presentation was poor caused by the macular involvement. There was no evidence of ocular inflammation. [ojoonline.org]
ORPHA:251295 Synonym(s): PPRCA Prevalence: Inheritance: Autosomal dominant or Not applicable Age of onset: All ages ICD-10: H35.5 OMIM: 172870 UMLS: C1868310 MeSH: - GARD: - MedDRA: - The documents contained in this web site are presented for information [orpha.net]
Psychiatrical
- Suggestibility
Our patients, and the preponderance of males in previously reported cases of this disease, suggest the possibility of Y-chromosome mediated male-to-male transmission. [ncbi.nlm.nih.gov]
[…] in nature. 12 Some reports suggest an inflammatory cause. [nature.com]
Workup
Systemic workup revealed QuantiFERON-gold positive. This is the first report of unilateral PPRCA secondary to presumed ocular tuberculosis. [Ophthalmic Surg Lasers Imaging Retina. 2017;48:345-349.]. [ncbi.nlm.nih.gov]
Results of laboratory studies, including a vasculitis workup, were within normal limits. A tuberculin skin testing result was 20 mm, erythrocyte sedimentation rate was 23 mm/hr, and a Quantiferon TB gold test (Qiagen) result was normal. [retinatoday.com]
Laboratory test Laboratory studies should be aimed towards narrowing the differential diagnosis depending on the presentation and given the overlap with inflammatory degenerative diseases a workup can include: comprehensive metabolic panel, complete and [eyewiki.org]
We referred the patient for a medical workup and asked her to return in six to eight weeks for close observation. The patient returned two years later with complaints of vision loss OS (20/100; OD remained 20/20). [reviewofoptometry.com]
Treatment
No effective treatment is available. [ncbi.nlm.nih.gov]
276 1552 Symptomatic Treatment 277 156 Differential Diagnosis 278 1563 Congenital Fibrosis of the Extraocular Muscles 279 Treatment of Specific Types of Nystagmus 283 161 Introduction 284 16211 Etiology 286 16212 Therapeutic Recommendations 287 1622 [books.google.com]
Prognosis
The visual prognosis for patients with pigmented paravenous retinochoroidal atrophy is quite good. [ncbi.nlm.nih.gov]
Prognosis - this condition recurs over months and frequent foveal involvement gives rise to a relatively poor prognosis. Multiple evanescent white dot syndrome (MEWDS) Nature - uncommon, idiopathic and self-limiting condition. [patient.info]
Prognosis Patients with PPRCA have good visual prognosis with slowly progressive disease. In patients without macular involvement, visual acuity is minimally adversely affected. [eyewiki.org]
Ischemic Visual Loss 235 1335 Orbital Manifestations 236 1343 LargeVessel Vasculitis 237 135 Laboratory Investigations in GCA 238 1352 CReactive Protein 239 1355 Anemia 240 1361 Temporal Artery Biopsy 241 1363 Role of Ultrasound 243 137 Treatment and Prognosis [books.google.com]
WFAF may also be helpful in determining prognosis in patients with AMD, although this needs confirmation in larger trials (i.e., AREDS2). [reviewofophthalmology.com]
Etiology
Etiology The etiology of PPRCA remains unknown with a presumed initial inflammatory insult that leads to the slow developmental of the retinal findings. [eyewiki.org]
Although earlier reports have suggested a possible post-inflammatory etiology such as tuberculosis, rubeola and syphilis as possible causes, a definite etiology remains elusive. [2] Typically, bone corpuscle pigmentation is seen along the distribution [ojoonline.org]
The etiology in this case could be inflammatory, infectious, or both. To the best of our knowledge after an extensive literature search, PPCRA with vasculitis has not been previously reported. [retinatoday.com]
The cause of the condition may be unknown or idiopathic, although a dysgenetic, degenerative, hereditary etiology or even an inflammatory cause has been hypothesized. [ingentaconnect.com]
We could not confirm the etiology as our patient could not undergo genetic studies. A long-term follow-up would reveal how the left eye condition progresses, particularly whether RP like degeneration develops in that eye. [ijo.in]
Epidemiology
Questions involved clinicians' perceptions of the epidemiology, diagnosis, treatment and prophylaxis of invasive candidosis. P < 0.0 Rev. Soc. Bras. Med. Trop.. Publicado em: 23/07/2013 3. [academicoo.com]
Ocular symptoms were analyzed using a validated ocular surface... article Adenoviral keratoconjunctivitis: An update The objective of this review is to describe the clinical and epidemiological characteristics of adenoviral conjunctivitis, as well as [infona.pl]
Relevant External Links for CRB1 Genetic Association Database (GAD) CRB1 Human Genome Epidemiology (HuGE) Navigator CRB1 Atlas of Genetics and Cytogenetics in Oncology and Haematology: CRB1 No data available for Genatlas for CRB1 Gene Clinical and molecular [genecards.org]
BACKGROUND Describe clinical and epidemiological characteristics of pediatric patients diagnosed with acute diarrhea and shock, admitted to the pediatric intensive care unit, in order to compare the evolution of clinical data between the survival and [pe.anoxia.us]
Pathophysiology
This case might provide preliminary information regarding the pathophysiology of PPRCA and improve our understanding of the nature of this disease. [ncbi.nlm.nih.gov]
Offers the most comprehensive content available on retina, balancing the latest scientific research and clinical correlations, covering everything you need to know on retinal diagnosis, treatment, development, structure, function, and pathophysiology. [books.google.com]
[…] ophthal #medicine #surgery #eyespecialist #eyemedicine #eyesurgeon #myophthalmologyresidency Retinitis Pigmentosa (RP) The term retinitis pigmentosa (RP) is semantically inaccurate: inflammation (implied by the suffix -itis) is not a prominent part of the pathophysiology [picuki.com]
Prevention
Prevention the secondary degeneration of cones in RP represents a very promising approach. This because such a therapy could be applied in a wide range of mutations expressed in rods, and even at late stages of the disease. [institut-vision.org]
BACKGROUND AND OBJECTIVES The presence of adverse reactions, inherent to all treatments, justifies the necessity of deep knowledge, by the medical team of the prevention and treatment of occasional organic dysfunctions, reducing its impact. [pe.anoxia.us]