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Recurrent ocular involvement in pediatric atypical hemolytic uremic syndrome. 2014

Xiaoyu Zheng, and Ian R Gorovoy, and Jianhua Mao, and Ji Jin, and Xi Chen, and Qi N Cui

Atypical hemolytic uremic syndrome (HUS) is a subtype of thrombotic microangiopathy associated with complement alternative pathway dysregulation. It is clinically characterized by a relapsing course and a poor prognosis. Multiple organ systems are commonly affected by thrombotic microangiopathy in pediatric atypical HUS; however, ocular involvement is rarely reported. The case of an 11-year-old girl diagnosed as having atypical HUS who presented with bilateral central retinal vein occlusions with macular subhyaloid hemorrhage during her initial onset and ophthalmoplegia, diplopia, and optic disc edema during her relapsing episode 1 year later is described. All ocular manifestations occurred in the convalescence phase of atypical HUS. No other extrarenal complications were found and full recovery was achieved following typical treatment for atypical HUS (ie, plasma infusion, steroid, and supportive therapy). This is thought to be the first reported case of recurrent ocular involvement in pediatric atypical HUS.

UI MeSH Term Description Entries
D008269 Macular Edema Fluid accumulation in the outer layer of the MACULA LUTEA that results from intraocular or systemic insults. It may develop in a diffuse pattern where the macula appears thickened or it may acquire the characteristic petaloid appearance referred to as cystoid macular edema. Although macular edema may be associated with various underlying conditions, it is most commonly seen following intraocular surgery, venous occlusive disease, DIABETIC RETINOPATHY, and posterior segment inflammatory disease. (From Survey of Ophthalmology 2004; 49(5) 470-90) Central Retinal Edema, Cystoid,Cystoid Macular Edema,Macular Edema, Cystoid,Cystoid Macular Dystrophy,Cystoid Macular Edema, Postoperative,Irvine-Gass Syndrome,Macular Dystrophy, Dominant Cystoid,Edema, Cystoid Macular,Edema, Macular,Irvine Gass Syndrome,Syndrome, Irvine-Gass
D002648 Child A person 6 to 12 years of age. An individual 2 to 5 years old is CHILD, PRESCHOOL. Children
D003937 Diagnosis, Differential Determination of which one of two or more diseases or conditions a patient is suffering from by systematically comparing and contrasting results of diagnostic measures. Diagnoses, Differential,Differential Diagnoses,Differential Diagnosis
D004172 Diplopia A visual symptom in which a single object is perceived by the visual cortex as two objects rather than one. Disorders associated with this condition include REFRACTIVE ERRORS; STRABISMUS; OCULOMOTOR NERVE DISEASES; TROCHLEAR NERVE DISEASES; ABDUCENS NERVE DISEASES; and diseases of the BRAIN STEM and OCCIPITAL LOBE. Double Vision,Polyopsia,Diplopia, Cortical,Diplopia, Horizontal,Diplopia, Intermittent,Diplopia, Monocular,Diplopia, Refractive,Diplopia, Unilateral,Diplopia, Vertical,Cortical Diplopia,Cortical Diplopias,Diplopias,Diplopias, Cortical,Diplopias, Horizontal,Diplopias, Intermittent,Diplopias, Monocular,Diplopias, Refractive,Diplopias, Unilateral,Diplopias, Vertical,Horizontal Diplopia,Horizontal Diplopias,Intermittent Diplopia,Intermittent Diplopias,Monocular Diplopia,Monocular Diplopias,Polyopsias,Refractive Diplopia,Refractive Diplopias,Unilateral Diplopia,Unilateral Diplopias,Vertical Diplopia,Vertical Diplopias,Vision, Double
D005260 Female Females
D006801 Humans Members of the species Homo sapiens. Homo sapiens,Man (Taxonomy),Human,Man, Modern,Modern Man
D012166 Retinal Hemorrhage Bleeding from the vessels of the retina. Hemorrhage, Retinal,Retinal Hemorrhages
D014057 Tomography, X-Ray Computed Tomography using x-ray transmission and a computer algorithm to reconstruct the image. CAT Scan, X-Ray,CT Scan, X-Ray,Cine-CT,Computerized Tomography, X-Ray,Electron Beam Computed Tomography,Tomodensitometry,Tomography, Transmission Computed,X-Ray Tomography, Computed,CAT Scan, X Ray,CT X Ray,Computed Tomography, X-Ray,Computed X Ray Tomography,Computerized Tomography, X Ray,Electron Beam Tomography,Tomography, X Ray Computed,Tomography, X-Ray Computer Assisted,Tomography, X-Ray Computerized,Tomography, X-Ray Computerized Axial,Tomography, Xray Computed,X Ray Computerized Tomography,X Ray Tomography, Computed,X-Ray Computer Assisted Tomography,X-Ray Computerized Axial Tomography,Beam Tomography, Electron,CAT Scans, X-Ray,CT Scan, X Ray,CT Scans, X-Ray,CT X Rays,Cine CT,Computed Tomography, Transmission,Computed Tomography, X Ray,Computed Tomography, Xray,Computed X-Ray Tomography,Scan, X-Ray CAT,Scan, X-Ray CT,Scans, X-Ray CAT,Scans, X-Ray CT,Tomographies, Computed X-Ray,Tomography, Computed X-Ray,Tomography, Electron Beam,Tomography, X Ray Computer Assisted,Tomography, X Ray Computerized,Tomography, X Ray Computerized Axial,Transmission Computed Tomography,X Ray Computer Assisted Tomography,X Ray Computerized Axial Tomography,X Ray, CT,X Rays, CT,X-Ray CAT Scan,X-Ray CAT Scans,X-Ray CT Scan,X-Ray CT Scans,X-Ray Computed Tomography,X-Ray Computerized Tomography,Xray Computed Tomography
D065766 Atypical Hemolytic Uremic Syndrome An hereditary hemolytic uremic syndrome associated with variations in the gene that encodes COMPLEMENT FACTOR H, or the related proteins CFHR1 and CFHR3. Disease often progresses to CHRONIC KIDNEY FAILURE without the prodromal symptoms of ENTEROCOLITIS and DIARRHEA that characterize typical hemolytic uremic syndrome. Atypical Hemolytic-Uremic Syndrome,Hemolytic Uremic Syndrome, Atypical,Non-Shiga-Like Toxin-Associated HUS,Non-Stx-Hus,Nonenteropathic HUS,Atypical Hemolytic-Uremic Syndromes,HUS, Non-Shiga-Like Toxin-Associated,HUS, Nonenteropathic,HUSs, Non-Shiga-Like Toxin-Associated,HUSs, Nonenteropathic,Hemolytic-Uremic Syndrome, Atypical,Hemolytic-Uremic Syndromes, Atypical,Non Shiga Like Toxin Associated HUS,Non Stx Hus,Non-Shiga-Like Toxin-Associated HUSs,Nonenteropathic HUSs,Syndrome, Atypical Hemolytic-Uremic,Syndromes, Atypical Hemolytic-Uremic,Toxin-Associated HUS, Non-Shiga-Like,Toxin-Associated HUSs, Non-Shiga-Like

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