Ana P Spizzirri1, Carlos J Cobeñas2, Laura F Alconcher3, Néstor Murray4, Claudia Zarate5, Laura Curutchet4, Emanuel De Rose2, María José Gogorza2, Lucas Lucarelli3, Javier Ruscasso2, Laura Lombardi2, Priscila Pereyra2, Javier Zalba2, Paula Risso6, Angela Suarez2. 1. Nephrology Department, Hospital de Niños "Superiora Sor María Ludovica", LaPlata, Buenos Aires, Argentina. anaspizzirri@hotmail.com. 2. Nephrology Department, Hospital de Niños "Superiora Sor María Ludovica", LaPlata, Buenos Aires, Argentina. 3. Nephrology Unit, Hospital Interzonal General Dr. José Penna. Bahía Blanca, Buenos Aires, Argentina. 4. Ophthalmology Department, Hospital de Niños "Superiora Sor María Ludovica", LaPlata, Buenos Aires, Argentina. 5. Ophthalmology Department, Hospital Interzonal General Dr. José Penna, Bahía Blanca, Buenos Aires, Argentina. 6. Cátedra de Bioestadística Bayesiana y Clásica, Facultad de Ciencias Veterinarias, Universidad Nacional de La Plata, La Plata, Argentina.
Abstract
BACKGROUND: Hemolytic uremic syndrome (HUS) is a systemic thrombotic microangiopathy characterized by hemolytic anemia, thrombocytopenia, and variable kidney involvement. Extrarenal thrombotic microangiopathy occurs in central nervous system (CNS), colon, and other organ systems, but ocular involvement is rarely recognized. This study aimed to analyze frequency and severity of ocular involvement in STEC-HUS, and the relationship between ocular involvement and disease severity, with emphasis on CNS, kidney, and colonic disease. METHODS: Prospective, longitudinal, observational study. INCLUSION CRITERIA: STEC-HUS patients September 2014-January 2019. Funduscopic examination (FE) was performed within 48 h of admission. We evaluated severity of CNS disease, kidney involvement, and presence of hemorrhagic colitis (HC). RESULTS: Ninety-nine patients were included (female 52), mean age 39.4 months (DE: 29.8; range 9-132). Thirteen patients (13.1%) had abnormal FE, 10 showing variable degrees of hemorrhagic exudates and 2 with typical Purtscher-like retinopathy. Other findings included tortuous vascularity, cotton wool spots, and transient retinal edema. CNS involvement was present in 16/99 patients, severe in 12 (75%). Abnormal FE occurred in 5/12 (31%) patients with severe CNS involvement vs. 8/87 (9.2%) with mild, moderate, or no CNS disease (p = 0.0191). Abnormal FE was present in 2/33 (6%) patients without dialysis vs. 11/66 (16.6%) requiring dialysis (p = 0.20). Finally, there were FE abnormalities in 6/20 patients with HC vs. 7/79 without HC (p = 0.012). CONCLUSIONS: FE abnormalities were present in 13% of HUS patients. Abnormal FE significantly associated with more severe disease, including severe CNS involvement and HC. We suggest FE should be performed in severe HUS, especially in cases with severe CNS disease. A higher resolution version of the Graphical abstract is available as Supplementary information.
BACKGROUND: Hemolytic uremic syndrome (HUS) is a systemic thrombotic microangiopathy characterized by hemolytic anemia, thrombocytopenia, and variable kidney involvement. Extrarenal thrombotic microangiopathy occurs in central nervous system (CNS), colon, and other organ systems, but ocular involvement is rarely recognized. This study aimed to analyze frequency and severity of ocular involvement in STEC-HUS, and the relationship between ocular involvement and disease severity, with emphasis on CNS, kidney, and colonic disease. METHODS: Prospective, longitudinal, observational study. INCLUSION CRITERIA: STEC-HUS patients September 2014-January 2019. Funduscopic examination (FE) was performed within 48 h of admission. We evaluated severity of CNS disease, kidney involvement, and presence of hemorrhagic colitis (HC). RESULTS: Ninety-nine patients were included (female 52), mean age 39.4 months (DE: 29.8; range 9-132). Thirteen patients (13.1%) had abnormal FE, 10 showing variable degrees of hemorrhagic exudates and 2 with typical Purtscher-like retinopathy. Other findings included tortuous vascularity, cotton wool spots, and transient retinal edema. CNS involvement was present in 16/99 patients, severe in 12 (75%). Abnormal FE occurred in 5/12 (31%) patients with severe CNS involvement vs. 8/87 (9.2%) with mild, moderate, or no CNS disease (p = 0.0191). Abnormal FE was present in 2/33 (6%) patients without dialysis vs. 11/66 (16.6%) requiring dialysis (p = 0.20). Finally, there were FE abnormalities in 6/20 patients with HC vs. 7/79 without HC (p = 0.012). CONCLUSIONS: FE abnormalities were present in 13% of HUS patients. Abnormal FE significantly associated with more severe disease, including severe CNS involvement and HC. We suggest FE should be performed in severe HUS, especially in cases with severe CNS disease. A higher resolution version of the Graphical abstract is available as Supplementary information.
Authors: Sjoukje E Loudon; Eiske M Dorresteijn; Coriene E Catsman-Berrevoets; Rob M Verdijk; Huibert J Simonsz; A J Gerard Jansen Journal: J Pediatr Date: 2014-05-17 Impact factor: 4.406
Authors: A A Novillo; L E Voyer; R Cravioto; M C Freire; G Castaño; R Wainstein; N Binztein Journal: Pediatr Nephrol Date: 1988-07 Impact factor: 3.714
Authors: Ricardo C Rahman; Carlos J Cobeñas; Ricardo Drut; Oscar R Amoreo; Javier D Ruscasso; Ana P Spizzirri; Angela Del C Suarez; Javier H Zalba; Celia Ferrari; Marcela C Gatti Journal: Pediatr Nephrol Date: 2011-08-02 Impact factor: 3.714