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.
Subject(s)
Central Nervous System Diseases , Hemolytic-Uremic Syndrome , Shiga-Toxigenic Escherichia coli , Thrombotic Microangiopathies , Child, Preschool , Female , Hemolytic-Uremic Syndrome/complications , Hemolytic-Uremic Syndrome/therapy , Humans , Prospective Studies , Renal Dialysis , Thrombotic Microangiopathies/complicationsABSTRACT
BACKGROUND: We aimed to determine the prevalence of hypoalbuminemia in STEC-HUS patients with hemorrhagic colitis (HC) and whether serum albumin level (SAL), leukocyte count, hematocrit and serum sodium level (SSL) are prognostic markers of HC, central nervous system disease (CNSd) and/or dialysis requirement and evaluate if hypoalbuminemia is associated with fecal protein losses. METHODS: We prospectively evaluated STEC-HUS patients treated at our institution from 9/2011 to 2/2019, analyzing the presence of HC, CNSd and dialysis requirement and SAL, SSL, leukocytes, hematocrit and α1-antitrypsin clearance. RESULTS: We evaluated 98 patients, with mean age of 33.3 months. SAL ≤ 29.5 g/l, > 24,600 leukocytes/mm3 and hematocrit > 30% behave as independent prognostic markers for HC. SAL ≤ 28 g/l, > 25,200 leukocytes/mm3 and hematocrit > 30% behave as prognostic markers for CNSd. SAL ≤ 31.6 g/l, > 13,800 leukocytes/mm3, hematocrit > 18.9% and hyponatremia (≤ 132 mEq/l) behave as prognostic markers for dialysis requirement. However, in multivariate logistic regression models, only hypoalbuminemia behaved as a risk factor for HC, CNSd and dialysis. α1-antitrypsin clearance was performed in 69 patients and was high in 9/69 (13%), only 4 with HC. No significant association was observed between α1-antitrypsin clearance and albuminemia (χ2 = 0.1076, p = 0.7429) as well as α1-antitrypsin clearance and HC (χ2 = 1.7892, p = 0.1810). CONCLUSIONS: Almost all patients with HC had hypoalbuminemia, which behaves as a risk factor for HC, CNSd and dialysis requirement. No significant association was observed between elevated α1-antitrypsin clearance and hypoalbuminemia nor between elevated α1-antitrypsin clearance and HC. These findings could be related to the small number of evaluated patients.
Subject(s)
Hemolytic-Uremic Syndrome , Hypoalbuminemia , Shiga-Toxigenic Escherichia coli , Child, Preschool , Hemolytic-Uremic Syndrome/complications , Hemolytic-Uremic Syndrome/epidemiology , Humans , Hypoalbuminemia/complications , Hypoalbuminemia/epidemiology , Renal Dialysis , Risk FactorsABSTRACT
BACKGROUND: We performed a retrospective evaluation of patients with diarrhea-associated hemolytic uremic syndrome (D + HUS) with the aims of: (1) determining the rate of red blood cell (RBC) transfusions; (2) establishing the relationship between need for RBC transfusion and severity of renal involvement; (3) determining whether precise measurements of lactic dehydrogenase (LDH) levels can predict the rate of hemolysis and severity of renal disease. METHODS: A total of 288 patients with D + HUS were retrospectively divided into three groups based on dialysis treatment: group 1, no dialysis treatment (144 patients); group 2, dialysis for 1-10 days (67 patients); group 3, dialysis for ≥11 days (77 patients). RESULTS: Of the patients in groups 1, 2 and 3, 73.6, 86.5 and 83.1%, respectively, required at least one RBC transfusion. The number of RBC transfusions in groups 1, 2 and 3 was 163, 107 and 162, respectively. Comparison of the groups revealed that the number of RBC transfusions was significantly higher in patients in groups 2 and 3 than in those in group 1 (p = 0.0001). Most RBC transfusions (94.2%) occurred during the first 2 weeks of the disease. The median peak LDH level was 2091 U/l in 32 patients with no RBC transfusion (group A), 3900 U/l in 73 patients with one transfusion (group B) and 6378 U/l in 62 patients with two or more transfusions (group C). Patients who received two or more RBC transfusions had a significantly higher median peak LDH level than those who did not receive RBC transfusions or received only one transfusion. This difference was also observed between patients who received only one RBC transfusion and those who did not receive any transfusions (p < 0.00001). Comparison of LDH levels on admission and peak LDH levels among patients in groups A, B and C revealed that 28/32 patients in group A, 56/73 patients in group B and 33/62 patients in group C had a stable LDH level, suggesting that patients with a stable LDH level require fewer RBC transfusions (p ≤ 0.006). Finally, we evaluated the possibility of an association between peak LDH levels and the degree of renal disease. The median peak LDH level in patients of group 1, 2 and 3 was 3538 (range 756-9373), 5165 (451-9205) and 7510 (1,145-16,340) U/l, respectively. Patients with >10 days of dialysis (group 3) had the highest LDH levels, followed by patients with 1-10 days of dialysis (group 2) and then by patients with no dialysis requirements (group 1) (p < 0.00001). CONCLUSIONS: The rate of RBC transfusion was higher in patients with the most severe renal injury, and most were performed during the first 2 weeks of the disease. Patients with stable LDH levels seemed to require fewer RBC transfusions. Median peak LDH levels were significantly higher in the group of patients with the most severe renal disease.
Subject(s)
Erythrocyte Transfusion/methods , Hemolytic-Uremic Syndrome/therapy , Kidney Diseases/complications , Acute Disease , Adolescent , Child , Child, Preschool , Diarrhea , Female , Hemolytic-Uremic Syndrome/complications , Humans , Infant , L-Lactate Dehydrogenase/blood , Male , Renal Dialysis , Retrospective Studies , Severity of Illness IndexABSTRACT
Little information exists regarding the efficacy, modifiers, and outcomes of anemia management in children with CKD or ESRD. We assessed practices, effectors, and outcomes of anemia management in 1394 pediatric patients undergoing peritoneal dialysis (PD) who were prospectively followed in 30 countries. We noted that 25% of patients had hemoglobin levels below target (<10 g/dl or <9.5 g/dl in children older or younger than 2 years, respectively), with significant regional variation; levels were highest in North America and Europe and lowest in Asia and Turkey. Low hemoglobin levels were associated with low urine output, low serum albumin, high parathyroid hormone, high ferritin, and the use of bioincompatible PD fluid. Erythropoiesis-stimulating agents (ESAs) were prescribed to 92% of patients, and neither the type of ESA nor the dosing interval appeared to affect efficacy. The weekly ESA dose inversely correlated with age when scaled to weight but did not correlate with age when normalized to body surface area. ESA sensitivity was positively associated with residual diuresis and serum albumin and inversely associated with serum parathyroid hormone and ferritin. The prevalence of hypertension and left ventricular hypertrophy increased with the degree of anemia. Patient survival was positively associated with achieved hemoglobin and serum albumin and was inversely associated with ESA dose. In conclusion, control of anemia in children receiving long-term PD varies by region. ESA requirements are independent of age when dose is scaled to body surface area, and ESA resistance is associated with inflammation, fluid retention, and hyperparathyroidism. Anemia and high ESA dose requirements independently predict mortality.
Subject(s)
Anemia/drug therapy , Hematinics/therapeutic use , Peritoneal Dialysis/adverse effects , Renal Insufficiency, Chronic/complications , Adolescent , Anemia/blood , Child , Child, Preschool , Female , Hematinics/administration & dosage , Hemoglobins , Humans , Infant , Infant, Newborn , Logistic Models , Male , Prevalence , Prospective Studies , Renal Insufficiency, Chronic/drug therapy , Renal Insufficiency, Chronic/therapy , Survival Analysis , Treatment Outcome , Young AdultABSTRACT
Hemorrhagic colitis (HC) is a severe manifestation of the hemolytic uremic syndrome (HUS). We performed a retrospective analysis of patients with HC with the following aims: (1) to characterize the clinicopathologic features; (2) to evaluate mortality rate; (3) to analyze severity of renal and central nervous system (CNS) disease. Patients with HC assisted between 1981-2009 were evaluated and compared with a control group of 137 patients without HC. Among 987 patients with diarrheal prodrome (D) + HUS, 54 (5.5%) presented HC. Clinical findings included abdominal pain (96%), distension (93%), hematochezia (44%), and abdominal mass (11%). Surgery was indicated in 35 patients (65%), and 17 (48.5%) required bowel resection. Transverse and ascending colon were most frequently affected. Macroscopic evaluation showed bowel necrosis (18) and perforation (12). Histologic evaluation (29) showed that 25 (86.2%) had necrosis of the affected segment (transmural in 21). A leukocyte count >20,000/mm(3) and hematocrit >30% were more common in HC patients than in controls (p < 0.001 and p < 0.0001, respectively). Mortality rate was higher in HC patients (33.3%) than in controls (1.4%; p < 0.0001). Dialysis >10 days, seizures, and coma were more frequent in HC patients than in controls (p < 0.0001). In summary, most patients had prominent abdominal findings, and almost 2/3 patients required surgery. Transverse/ascending colon was most affected, and the main histologic finding was transmural necrosis. Higher hematocrit and leukocytosis were frequent. Mortality rate was extremely high, and most had long-lasting anuria and severe neurologic involvement.
Subject(s)
Colitis/etiology , Diarrhea/complications , Gastrointestinal Hemorrhage/etiology , Hemolytic-Uremic Syndrome/complications , Child , Child, Preschool , Colitis/mortality , Colitis/pathology , Female , Gastrointestinal Hemorrhage/mortality , Gastrointestinal Hemorrhage/pathology , Humans , Infant , Male , Necrosis , Retrospective StudiesABSTRACT
El síndrome nefrítico se caracteriza por la combinación de hematuria y proteinuria como manifestación de injuria glomerular, generalmente asociadas a oliguria, hipertensión arterial y edema. La causa mas común del síndrome nefrítico es la glomérulo nefritis pos estreptocócica, secundaria a sepas nefritogenicas de estreptococo ß hemolítico del grupo A. Causas menos frecuentes son: otras infecciones, glomérulo nefritis membrano-proliferativa, LES, endocarditis bacteriana, enfermedad de Berger, Síndrome de Schonlein-Henoch, vasculitis, etc.
Subject(s)
Child , Nephrotic SyndromeABSTRACT
El síndrome nefrítico se caracteriza por la combinación de hematuria y proteinuria como manifestación de injuria glomerular, generalmente asociadas a oliguria, hipertensión arterial y edema. La causa mas común del síndrome nefrítico es la glomérulo nefritis pos estreptocócica, secundaria a sepas nefritogenicas de estreptococo ß hemolítico del grupo A. Causas menos frecuentes son: otras infecciones, glomérulo nefritis membrano-proliferativa, LES, endocarditis bacteriana, enfermedad de Berger, Síndrome de Schonlein-Henoch, vasculitis, etc
Subject(s)
Child , Nephrotic SyndromeABSTRACT
Se denomina Síndrome Uréemico Helolítico (SUH) a una entidad clínica y anatomapatológica de origen infeccioso y de características endemo epidémicas, caracterizada por anemia hemolítica microangiopática y trombocitopenia, con grados variables de afectación de la función renal
Subject(s)
Humans , Child , Anemia, Hemolytic/complications , Anemia, Hemolytic/diagnosis , Food Contamination/prevention & control , Escherichia coli , Hemolytic-Uremic Syndrome/classification , ThrombocytopeniaABSTRACT
La infección urinaria (IU) constituye el conjunto de signos y síntomas resultantes de la multiplicación microbiana dentro del tracto urinario
Subject(s)
Humans , Child , Bacteriuria , Cystitis , Pyelonephritis , Urinary Tract , Urinary Tract InfectionsABSTRACT
Se denomina Síndrome Uréemico Helolítico (SUH) a una entidad clínica y anatomapatológica de origen infeccioso y de características endemo epidémicas, caracterizada por anemia hemolítica microangiopática y trombocitopenia, con grados variables de afectación de la función renal
Subject(s)
Humans , Child , Hemolytic-Uremic Syndrome/classification , Anemia, Hemolytic/complications , Anemia, Hemolytic/diagnosis , Thrombocytopenia/diagnosis , Escherichia coli/classification , Food Contamination/prevention & controlABSTRACT
La infección urinaria (IU) constituye el conjunto de signos y síntomas resultantes de la multiplicación microbiana dentro del tracto urinario
Subject(s)
Humans , Child , Urinary Tract Infections/classification , Cystitis/complications , Cystitis/classification , Pyelonephritis/classification , Bacteriuria/classification , Urinary Tract/anatomy & histologyABSTRACT
Actualización de Hipertensión Arterial. Guía de diagnóstico y tratamiento
Subject(s)
Humans , Child , Hypertension/diagnosis , Diagnosis , Blood Pressure/drug effectsABSTRACT
Actualización de Hipertensión Arterial. Guía de diagnóstico y tratamiento
Subject(s)
Humans , Child , Diagnosis , Hypertension/diagnosis , Blood PressureABSTRACT
Introducción. Las complicaciones tromboembólicas, en niños con síndrome nefrótico, aunque infrecuentes, se cuentan entre las intercurrencias más graves. Objetivo. Descripción de nuestra experiencia en pacientes con síndrome nefrótico que han padecido episodios de complicaciones tromboembólicas, su incidencia, presentación clínica, anomalías de laboratorio, métodos auxiliares de diagnóstico empleados, tratamiento y evolución. Población, material y métodos. Se estudiaron en forma retrospectiva 610 niños con síndrome nefrótico primario asistidos en el Servicio de Nefrología del Hospital de Niños Superiora Sor María Ludovica de La Plata (Argentina), en un período de 32 años (1969-2001). Se analizaron 9 pacientes en los cuales se determinó la presencia de complicaciones tromboembólicas, su presentación clínica, localización, factores condicionantes, laboratorio, tratamiento y evolución. Resultados. En 9 de 610 pacientes con síndrome nefrótico (incidencia: 1,47 por ciento) se detectaron 11 episodiosde trombosis. Cinco de ellos presentaron un síndrome nefrótico corticosensible (2 corticodependientes) y 4 corticorresistentes. Se efectuaron biopsias renales percutáneas en 5 (3 cambios mínimos y 2 glomerulonefritis membrano proliferativa tipo II). Las localizaciones de las complicaciones tromboembólicas fueron variadas: cerebro (n igual 6 ), venas profundas de miembros inferiores (n igual 5), senos intracraneanos (n igual 2), venas ilíacas, venas renales, vena cava, un trombo intracardíaco y tromboembolismo pulmonar (estas 5 últimas como parte de un evento trombótico generalizado en un mismo paciente). Todos los episodios se desarrollaron durante una recaída del síndrome nefrótico, bajo tratamiento esteroideo. Según la localización, la complicación tromboembólica se confirmó mediante ecografía Doppler color, tomografía axial computada, angiografía radiológica y tomográfica o angiorresonancia magnética.
Subject(s)
Child , Embolism , Nephrotic Syndrome/complications , Nephrotic Syndrome/diagnosis , Nephrotic Syndrome/therapy , Thrombosis/complications , Retrospective StudiesABSTRACT
Introducción. Las complicaciones tromboembólicas, en niños con síndrome nefrótico, aunque infrecuentes, se cuentan entre las intercurrencias más graves. Objetivo. Descripción de nuestra experiencia en pacientes con síndrome nefrótico que han padecido episodios de complicaciones tromboembólicas, su incidencia, presentación clínica, anomalías de laboratorio, métodos auxiliares de diagnóstico empleados, tratamiento y evolución. Población, material y métodos. Se estudiaron en forma retrospectiva 610 niños con síndrome nefrótico primario asistidos en el Servicio de Nefrología del Hospital de Niños ¶Superiora Sor María Ludovica÷ de La Plata (Argentina), en un período de 32 años (1969-2001). Se analizaron 9 pacientes en los cuales se determinó la presencia de complicaciones tromboembólicas, su presentación clínica, localización, factores condicionantes, laboratorio, tratamiento y evolución. Resultados. En 9 de 610 pacientes con síndrome nefrótico (incidencia: 1,47 por ciento) se detectaron 11 episodiosde trombosis. Cinco de ellos presentaron un síndrome nefrótico corticosensible (2 corticodependientes) y 4 corticorresistentes. Se efectuaron biopsias renales percutáneas en 5 (3 cambios mínimos y 2 glomerulonefritis membrano proliferativa tipo II). Las localizaciones de las complicaciones tromboembólicas fueron variadas: cerebro (n igual 6 ), venas profundas de miembros inferiores (n igual 5), senos intracraneanos (n igual 2), venas ilíacas, venas renales, vena cava, un trombo intracardíaco y tromboembolismo pulmonar (estas 5 últimas como parte de un evento trombótico generalizado en un mismo paciente). Todos los episodios se desarrollaron durante una recaída del síndrome nefrótico, bajo tratamiento esteroideo. Según la localización, la complicación tromboembólica se confirmó mediante ecografía Doppler color, tomografía axial computada, angiografía radiológica y tomográfica o angiorresonancia magnética.(AU)
Subject(s)
Child , Thrombosis/complications , Nephrotic Syndrome/complications , Nephrotic Syndrome/diagnosis , Nephrotic Syndrome/therapy , Embolism , Retrospective StudiesABSTRACT
Introducción.El objetivo del presente estudio fue analizar el comportamiento evolutivo de pacientes con glomerulonefritis rápidamente progresiva(GNRP)e identificar variables con implicancias pronósticas y terapéuticas.Material y métodos.Se efectuó un estudio retospectivo de 26 niños con GNRP(> 50 por ciento de los glomérulos con semilunas que ocuparan más del 5 0 por ciento de la circunferencia glomerular)asistidos en el Hospital de Niños de La Plata en los últimos 22 años.Se analizaron el porcentaje y tipo de semilunas,el intervalo entre el comienzo de la enfermedad y el tratamiento,así como la necesidad de diálisis y las condiciones subyacentes(variables pronósticas)23 pacientes fueron tratados con" pulsos"de metilprednisolona IV(15-30 mg/kg nº3-12)seguidos de prednisona y ciclofosfamida.Conclusiones.Más de 2/3 de los pacientes mejoraron la función renal.El intervalo mayor de un mes entre el comienzo de la enfermedad y el tratamiento,el predominio de las semilunas estuvieron asociados con un peor pronóstico.Los resultados sugieren que la administración precoz de alta dosis de metilprednisola intravenosa seguida de tratamiento inmunosupresor puede ser de beneficio en casos de GNRP
Subject(s)
Child, Preschool , Child , Glomerulonephritis/pathologyABSTRACT
Introducción.El objetivo del presente estudio fue analizar el comportamiento evolutivo de pacientes con glomerulonefritis rápidamente progresiva(GNRP)e identificar variables con implicancias pronósticas y terapéuticas.Material y métodos.Se efectuó un estudio retospectivo de 26 niños con GNRP(> 50 por ciento de los glomérulos con semilunas que ocuparan más del 5 0 por ciento de la circunferencia glomerular)asistidos en el Hospital de Niños de La Plata en los últimos 22 años.Se analizaron el porcentaje y tipo de semilunas,el intervalo entre el comienzo de la enfermedad y el tratamiento,así como la necesidad de diálisis y las condiciones subyacentes(variables pronósticas)23 pacientes fueron tratados con" pulsos"de metilprednisolona IV(15-30 mg/kg nº3-12)seguidos de prednisona y ciclofosfamida.Conclusiones.Más de 2/3 de los pacientes mejoraron la función renal.El intervalo mayor de un mes entre el comienzo de la enfermedad y el tratamiento,el predominio de las semilunas estuvieron asociados con un pe
Subject(s)
Child, Preschool , Child , Glomerulonephritis/pathologyABSTRACT
Se relata la historia clínica de una niña de 13 años con síndrome de Sj÷gren primario que se presentó como una acidosis tubular renal con nefrocalcinosis. La ecografía de parótida que evidenciaba áreas ecolúcidas, una biopsia de glándula salival con infiltrados linfocitarios y atrofia acinar y la existencia de una hermana que padece dicho síndrome, contribuyeron al diagnóstico (AU)
