Hypoalbuminemia: a risk factor in patients with STEC-associated hemolytic uremic syndrome.

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.

Clinical parameters, LysoGb3, podocyturia, and kidney biopsy in children with Fabry disease: is a correlation possible?

BACKGROUND: Fabry disease is an X-linked lysosomal storage disorder caused by α-galactosidase enzyme deficiency. We present clinical, biochemical, and histologic findings in children with classical phenotypic presentation of Fabry disease. METHODS: A retrospective analysis was performed using charts from 14 children with confirmed diagnosis. Clinical parameters were evaluated. Globotriaosylsphingosine -lysoGb3- detection in plasma, podocyturia, and kidney biopsy were carried out in all cases. RESULTS: All patients except one demonstrated at least one symptom of Fabry disease. LysoGb3 levels were above the normal range in all patients. Podocyturia was documented in all patients. Kidney biopsy revealed glomerular, interstitial, vascular, and tubular changes on light microscopy in nearly all patients. Electron microscopy showed podocyte inclusions in all patients. CONCLUSIONS: No difference in symptomatology was discernible between boys and girls. Podocyturia was detectable in children serving as a possible early marker of kidney injury. LysoGb3 was elevated in all cases, emphasizing the importance for diagnosis especially in female patients with normal αGal A activity. A possible association between lysoGb3 and symptom severity and histological involvement in kidney biopsy should be assessed in prospective studies with enough statistical power to determine if lysoGb3 can be used to predict nephropathy in children with Fabry disease.

Relationship between red blood cell transfusion requirements and severity of renal disease during the acute stage of hemolytic uremic syndrome.

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.

Hemorrhagic colitis in postdiarrheal hemolytic uremic syndrome: retrospective analysis of 54 children.

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.

Síndorme Nefrítico / Nephritic Syndrome

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

Síndorme nefrítico / Nephritic syndrome

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.

Síndrome Urémico Hemolítico Clásico / Syndrome Hemolytic Uremic

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

Infección Urinaria / Urinary Infection

La infección urinaria (IU) constituye el conjunto de signos y síntomas resultantes de la multiplicación microbiana dentro del tracto urinario

Síndrome Urémico Hemolítico Clásico / Syndrome Hemolytic Uremic

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

Infección Urinaria / Urinary Infection

La infección urinaria (IU) constituye el conjunto de signos y síntomas resultantes de la multiplicación microbiana dentro del tracto urinario

Válvulas de uretra posterior: seguimiento a largo plazo de la función renal / Long-term follow-up of renal function in children with posterior urethral valves

Introducción.Las válvulas de uretra posterior(VUP)lauropatía obstructiva de mayor severidad en la infancia,pueden conducir a la insuficiencia renal crónica terminal(IRCT)Se describe la evolución alejada de la función renal en niños con VUP y su relación con la precocidad del diagnóstico,la función renal inicial y tratamiento quirúrgico.Material y métodos.Se estudiaron retrospectivamente 38 niños con VUP asistidos entre 1969-96.La mediana de la edad fue de 9 meses(20 menores de 1 año)El seguimiento promedio fue de 7,08 años.Conclusión.El seguimiento alejado de nuestros pacientes portadores de VUP demuestra la gravedad de esta patología.Se corroboró que el pronóstico alejado de la función renal está determinado en gran medida por el grado del filtrado glomerulal en el momento del diagnóstico.No pudimos hallar relación entre diagnóstico precoz y un peor pronóstico alejado.Tampoco hallamos diferencias significativas en la evolución alejada de la función renal en pacientes sometidos a resección valvular primaria en comparación con otras técnicas de derivación urinaria

Válvulas de uretra posterior: seguimiento a largo plazo de la función renal / Long-term follow-up of renal function in children with posterior urethral valves

Introducción.Las válvulas de uretra posterior(VUP)lauropatía obstructiva de mayor severidad en la infancia,pueden conducir a la insuficiencia renal crónica terminal(IRCT)Se describe la evolución alejada de la función renal en niños con VUP y su relación con la precocidad del diagnóstico,la función renal inicial y tratamiento quirúrgico.Material y métodos.Se estudiaron retrospectivamente 38 niños con VUP asistidos entre 1969-96.La mediana de la edad fue de 9 meses(20 menores de 1 año)El seguimiento promedio fue de 7,08 años.Conclusión.El seguimiento alejado de nuestros pacientes portadores de VUP demuestra la gravedad de esta patología.Se corroboró que el pronóstico alejado de la función renal está determinado en gran medida por el grado del filtrado glomerulal en el momento del diagnóstico.No pudimos hallar relación entre diagnóstico precoz y un peor pronóstico alejado.Tampoco hallamos diferencias significativas en la evolución alejada de la función renal en pacientes sometidos a resección valvular primaria en comparación con otras técnicas de derivación urinaria