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Sara K Vesely - One of the best experts on this subject based on the ideXlab platform.
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sporadic Bloody Diarrhea associated thrombotic thrombocytopenic purpura hemolytic uremic syndrome ttp hus in adults in oklahoma comparison to adults with severe adamts13 deficiency and to children with typical hus
Blood, 2007Co-Authors: Loan Nguyen, Deirdra R Terrell, Bernhard Lammle, Sara K Vesely, Charity A Karpac, Johanna Kremer A Hovinga, James N GeorgeAbstract:The frequency, presenting features and clinical outcomes of sporadic TTP-HUS following a prodrome of Bloody Diarrhea in adults are not described. The Oklahoma TTP-HUS Registry enrolled 237 consecutive patients over age 18 years with their first episode of clinically diagnosed TTP from 11-13-1995 (the date of our initial ADAMTS13 measurement) to 12-31-2006 for whom plasma exchange treatment (PEX) was requested. ADAMTS13 activity was measured on 218 (92%) patients immediately before their first PEX. 16 (7%) of these 218 patients presented with a prodrome of acute Bloody Diarrhea. 42 (19%) patients had ADAMTS13 activity Conclusions: TTP-HUS following Bloody Diarrhea is an endemic, sporadic disorder among adults that is less common and less familiar than in children. Distinct from children, adults with Bloody Diarrhea have a higher frequency of severe neurologic abnormalities and death; distinct from adults with severe ADAMTS13 deficiency, adults with Bloody Diarrhea are primarily white, have a higher frequency of acute renal failure, and have not relapsed. Although the role of PEX in the recovery of adult patients presenting with Bloody Diarrhea is unclear, PEX may be appropriate initial treatment since the mortality is high, many patients appear to respond, and patients with severe ADAMTS13 deficiency may also present with Bloody Diarrhea apparently caused by intestinal ischemia.
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thrombotic thrombocytopenic purpura hemolytic uremic syndrome ttp hus in adults following a prodrome of Bloody Diarrhea
Blood, 2004Co-Authors: Qurana F Lewis, Deirdra R Terrell, Bernhard Lammle, Johanna Kremerhovinga, James N George, Sara K VeselyAbstract:Diarrhea, often Bloody Diarrhea caused by infection with E. coli 0157:H7, is the prodrome for typical HUS in children. In adults, HUS has been reported following epidemics of enteric infections, however the frequency and clinical features of sporadic HUS or TTP in adults following a prodrome of Bloody Diarrhea have not been characterized. In the Oklahoma TTP-HUS Registry, January 1, 1989 to December 31, 2003, 19 (6%) of 301 consecutive patients had a prodrome of Bloody Diarrhea. The 19 cases were separated by time and location, indicating no common source outbreak. 5/10 patients who were appropriately tested had positive stool cultures for E. coli O157:H7. Although more cases (12/19, 63%) occurred in warm months, April–September, a seasonal difference was not significant (p= 0.25). We compared the clinical features of these 19 patients to the 119 patients who had no apparent etiologies or associated conditions and were therefore defined as idiopathic TTP-HUS. | | Bloody Diarrhea | Idiopathic | p-value | |:---------------------------------------------------------------------------------------------------------------------- | --------------- | ----------- | ------- | | | (n=19) | (n=119) | | | Median values are presented for continuous data. Laboratory data: most abnormal value at diagnosis of TTP-HUS ± 7 days | | Age (years) | 59 | 48 | 0.109 | | Race (% Black) | 5% | 25% | 0.073 | | Gender (% female) | 79% | 74% | 0.781 | | Obesity (BMI ≥ 30 kg/m 2 ) | 21% | 40% | 0.120 | | Severe neurologic abnormalities | 63% | 49% | 0.243 | | Platelet count | 26 | 13 | 0.010 | | Hematocrit | 22 | 22 | 0.931 | | LDH | 1410 | 1305 | 0.115 | | Acute renal failure | 68% | 29% | <0.001 | | Response to PE | 84% | 82% | 1.000 | | ADAMTS13 deficiency (<5%) | 0% (0/13) | 30% (20/67) | 0.031 | | Death | 32% | 19% | 0.233 | | Relapse in 30 day survivors | 0% | 20% | 0.119 | Although women predominated in both groups, 18/19 patients with a prodrome of Bloody Diarrhea were White, consistent with the predominance of White subjects among children with Diarrhea-associated HUS, but distinct from the significantly higher incidence of Blacks among adult patients with idiopathic TTP-HUS (compared to the incidence among other races, p<0.0001). 3 patients with a prodrome of Bloody Diarrhea never had an abnormal serum creatinine, therefore the term TTP-HUS, rather than HUS, may better describe these patients. The only significant differences in presenting features and outcomes were the severity of thrombocytopenia, the relative frequency of acute renal failure, and the relative frequency of severe ADAMTS13 deficiency (<5% activity). ADAMTS13 activity was measured in 13 of the 19 patients with a prodrome of Bloody Diarrhea (median 50%, range 5–100%). We conclude that there is a continuous occurrence of TTP-HUS in adults preceded by a prodrome of Bloody Diarrhea, presumably related to Shiga toxin-producing enteric infections, even in the absence of recognized outbreaks. Plasma exchange is an appropriate treatment for adult patients with a prodrome of Bloody Diarrhea since they cannot be accurately distinguished from patients with idiopathic TTP, they have a high mortality rate, and they apparently respond to plasma exchange treatment.
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Thrombotic Thrombocytopenic Purpura-Hemolytic Uremic Syndrome (TTP-HUS) in Adults Following a Prodrome of Bloody Diarrhea.
Blood, 2004Co-Authors: Qurana F Lewis, Deirdra R Terrell, Bernhard Lammle, James N George, Johanna Kremer-hovinga, Sara K VeselyAbstract:Diarrhea, often Bloody Diarrhea caused by infection with E. coli 0157:H7, is the prodrome for typical HUS in children. In adults, HUS has been reported following epidemics of enteric infections, however the frequency and clinical features of sporadic HUS or TTP in adults following a prodrome of Bloody Diarrhea have not been characterized. In the Oklahoma TTP-HUS Registry, January 1, 1989 to December 31, 2003, 19 (6%) of 301 consecutive patients had a prodrome of Bloody Diarrhea. The 19 cases were separated by time and location, indicating no common source outbreak. 5/10 patients who were appropriately tested had positive stool cultures for E. coli O157:H7. Although more cases (12/19, 63%) occurred in warm months, April–September, a seasonal difference was not significant (p= 0.25). We compared the clinical features of these 19 patients to the 119 patients who had no apparent etiologies or associated conditions and were therefore defined as idiopathic TTP-HUS. | | Bloody Diarrhea | Idiopathic | p-value | |:---------------------------------------------------------------------------------------------------------------------- | --------------- | ----------- | ------- | | | (n=19) | (n=119) | | | Median values are presented for continuous data. Laboratory data: most abnormal value at diagnosis of TTP-HUS ± 7 days | | Age (years) | 59 | 48 | 0.109 | | Race (% Black) | 5% | 25% | 0.073 | | Gender (% female) | 79% | 74% | 0.781 | | Obesity (BMI ≥ 30 kg/m 2 ) | 21% | 40% | 0.120 | | Severe neurologic abnormalities | 63% | 49% | 0.243 | | Platelet count | 26 | 13 | 0.010 | | Hematocrit | 22 | 22 | 0.931 | | LDH | 1410 | 1305 | 0.115 | | Acute renal failure | 68% | 29% |
James N George - One of the best experts on this subject based on the ideXlab platform.
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sporadic Bloody Diarrhea associated thrombotic thrombocytopenic purpura hemolytic uremic syndrome ttp hus in adults in oklahoma comparison to adults with severe adamts13 deficiency and to children with typical hus
Blood, 2007Co-Authors: Loan Nguyen, Deirdra R Terrell, Bernhard Lammle, Sara K Vesely, Charity A Karpac, Johanna Kremer A Hovinga, James N GeorgeAbstract:The frequency, presenting features and clinical outcomes of sporadic TTP-HUS following a prodrome of Bloody Diarrhea in adults are not described. The Oklahoma TTP-HUS Registry enrolled 237 consecutive patients over age 18 years with their first episode of clinically diagnosed TTP from 11-13-1995 (the date of our initial ADAMTS13 measurement) to 12-31-2006 for whom plasma exchange treatment (PEX) was requested. ADAMTS13 activity was measured on 218 (92%) patients immediately before their first PEX. 16 (7%) of these 218 patients presented with a prodrome of acute Bloody Diarrhea. 42 (19%) patients had ADAMTS13 activity Conclusions: TTP-HUS following Bloody Diarrhea is an endemic, sporadic disorder among adults that is less common and less familiar than in children. Distinct from children, adults with Bloody Diarrhea have a higher frequency of severe neurologic abnormalities and death; distinct from adults with severe ADAMTS13 deficiency, adults with Bloody Diarrhea are primarily white, have a higher frequency of acute renal failure, and have not relapsed. Although the role of PEX in the recovery of adult patients presenting with Bloody Diarrhea is unclear, PEX may be appropriate initial treatment since the mortality is high, many patients appear to respond, and patients with severe ADAMTS13 deficiency may also present with Bloody Diarrhea apparently caused by intestinal ischemia.
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thrombotic thrombocytopenic purpura hemolytic uremic syndrome ttp hus in adults following a prodrome of Bloody Diarrhea
Blood, 2004Co-Authors: Qurana F Lewis, Deirdra R Terrell, Bernhard Lammle, Johanna Kremerhovinga, James N George, Sara K VeselyAbstract:Diarrhea, often Bloody Diarrhea caused by infection with E. coli 0157:H7, is the prodrome for typical HUS in children. In adults, HUS has been reported following epidemics of enteric infections, however the frequency and clinical features of sporadic HUS or TTP in adults following a prodrome of Bloody Diarrhea have not been characterized. In the Oklahoma TTP-HUS Registry, January 1, 1989 to December 31, 2003, 19 (6%) of 301 consecutive patients had a prodrome of Bloody Diarrhea. The 19 cases were separated by time and location, indicating no common source outbreak. 5/10 patients who were appropriately tested had positive stool cultures for E. coli O157:H7. Although more cases (12/19, 63%) occurred in warm months, April–September, a seasonal difference was not significant (p= 0.25). We compared the clinical features of these 19 patients to the 119 patients who had no apparent etiologies or associated conditions and were therefore defined as idiopathic TTP-HUS. | | Bloody Diarrhea | Idiopathic | p-value | |:---------------------------------------------------------------------------------------------------------------------- | --------------- | ----------- | ------- | | | (n=19) | (n=119) | | | Median values are presented for continuous data. Laboratory data: most abnormal value at diagnosis of TTP-HUS ± 7 days | | Age (years) | 59 | 48 | 0.109 | | Race (% Black) | 5% | 25% | 0.073 | | Gender (% female) | 79% | 74% | 0.781 | | Obesity (BMI ≥ 30 kg/m 2 ) | 21% | 40% | 0.120 | | Severe neurologic abnormalities | 63% | 49% | 0.243 | | Platelet count | 26 | 13 | 0.010 | | Hematocrit | 22 | 22 | 0.931 | | LDH | 1410 | 1305 | 0.115 | | Acute renal failure | 68% | 29% | <0.001 | | Response to PE | 84% | 82% | 1.000 | | ADAMTS13 deficiency (<5%) | 0% (0/13) | 30% (20/67) | 0.031 | | Death | 32% | 19% | 0.233 | | Relapse in 30 day survivors | 0% | 20% | 0.119 | Although women predominated in both groups, 18/19 patients with a prodrome of Bloody Diarrhea were White, consistent with the predominance of White subjects among children with Diarrhea-associated HUS, but distinct from the significantly higher incidence of Blacks among adult patients with idiopathic TTP-HUS (compared to the incidence among other races, p<0.0001). 3 patients with a prodrome of Bloody Diarrhea never had an abnormal serum creatinine, therefore the term TTP-HUS, rather than HUS, may better describe these patients. The only significant differences in presenting features and outcomes were the severity of thrombocytopenia, the relative frequency of acute renal failure, and the relative frequency of severe ADAMTS13 deficiency (<5% activity). ADAMTS13 activity was measured in 13 of the 19 patients with a prodrome of Bloody Diarrhea (median 50%, range 5–100%). We conclude that there is a continuous occurrence of TTP-HUS in adults preceded by a prodrome of Bloody Diarrhea, presumably related to Shiga toxin-producing enteric infections, even in the absence of recognized outbreaks. Plasma exchange is an appropriate treatment for adult patients with a prodrome of Bloody Diarrhea since they cannot be accurately distinguished from patients with idiopathic TTP, they have a high mortality rate, and they apparently respond to plasma exchange treatment.
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Thrombotic Thrombocytopenic Purpura-Hemolytic Uremic Syndrome (TTP-HUS) in Adults Following a Prodrome of Bloody Diarrhea.
Blood, 2004Co-Authors: Qurana F Lewis, Deirdra R Terrell, Bernhard Lammle, James N George, Johanna Kremer-hovinga, Sara K VeselyAbstract:Diarrhea, often Bloody Diarrhea caused by infection with E. coli 0157:H7, is the prodrome for typical HUS in children. In adults, HUS has been reported following epidemics of enteric infections, however the frequency and clinical features of sporadic HUS or TTP in adults following a prodrome of Bloody Diarrhea have not been characterized. In the Oklahoma TTP-HUS Registry, January 1, 1989 to December 31, 2003, 19 (6%) of 301 consecutive patients had a prodrome of Bloody Diarrhea. The 19 cases were separated by time and location, indicating no common source outbreak. 5/10 patients who were appropriately tested had positive stool cultures for E. coli O157:H7. Although more cases (12/19, 63%) occurred in warm months, April–September, a seasonal difference was not significant (p= 0.25). We compared the clinical features of these 19 patients to the 119 patients who had no apparent etiologies or associated conditions and were therefore defined as idiopathic TTP-HUS. | | Bloody Diarrhea | Idiopathic | p-value | |:---------------------------------------------------------------------------------------------------------------------- | --------------- | ----------- | ------- | | | (n=19) | (n=119) | | | Median values are presented for continuous data. Laboratory data: most abnormal value at diagnosis of TTP-HUS ± 7 days | | Age (years) | 59 | 48 | 0.109 | | Race (% Black) | 5% | 25% | 0.073 | | Gender (% female) | 79% | 74% | 0.781 | | Obesity (BMI ≥ 30 kg/m 2 ) | 21% | 40% | 0.120 | | Severe neurologic abnormalities | 63% | 49% | 0.243 | | Platelet count | 26 | 13 | 0.010 | | Hematocrit | 22 | 22 | 0.931 | | LDH | 1410 | 1305 | 0.115 | | Acute renal failure | 68% | 29% |
Bernhard Lammle - One of the best experts on this subject based on the ideXlab platform.
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sporadic Bloody Diarrhea associated thrombotic thrombocytopenic purpura hemolytic uremic syndrome ttp hus in adults in oklahoma comparison to adults with severe adamts13 deficiency and to children with typical hus
Blood, 2007Co-Authors: Loan Nguyen, Deirdra R Terrell, Bernhard Lammle, Sara K Vesely, Charity A Karpac, Johanna Kremer A Hovinga, James N GeorgeAbstract:The frequency, presenting features and clinical outcomes of sporadic TTP-HUS following a prodrome of Bloody Diarrhea in adults are not described. The Oklahoma TTP-HUS Registry enrolled 237 consecutive patients over age 18 years with their first episode of clinically diagnosed TTP from 11-13-1995 (the date of our initial ADAMTS13 measurement) to 12-31-2006 for whom plasma exchange treatment (PEX) was requested. ADAMTS13 activity was measured on 218 (92%) patients immediately before their first PEX. 16 (7%) of these 218 patients presented with a prodrome of acute Bloody Diarrhea. 42 (19%) patients had ADAMTS13 activity Conclusions: TTP-HUS following Bloody Diarrhea is an endemic, sporadic disorder among adults that is less common and less familiar than in children. Distinct from children, adults with Bloody Diarrhea have a higher frequency of severe neurologic abnormalities and death; distinct from adults with severe ADAMTS13 deficiency, adults with Bloody Diarrhea are primarily white, have a higher frequency of acute renal failure, and have not relapsed. Although the role of PEX in the recovery of adult patients presenting with Bloody Diarrhea is unclear, PEX may be appropriate initial treatment since the mortality is high, many patients appear to respond, and patients with severe ADAMTS13 deficiency may also present with Bloody Diarrhea apparently caused by intestinal ischemia.
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thrombotic thrombocytopenic purpura hemolytic uremic syndrome ttp hus in adults following a prodrome of Bloody Diarrhea
Blood, 2004Co-Authors: Qurana F Lewis, Deirdra R Terrell, Bernhard Lammle, Johanna Kremerhovinga, James N George, Sara K VeselyAbstract:Diarrhea, often Bloody Diarrhea caused by infection with E. coli 0157:H7, is the prodrome for typical HUS in children. In adults, HUS has been reported following epidemics of enteric infections, however the frequency and clinical features of sporadic HUS or TTP in adults following a prodrome of Bloody Diarrhea have not been characterized. In the Oklahoma TTP-HUS Registry, January 1, 1989 to December 31, 2003, 19 (6%) of 301 consecutive patients had a prodrome of Bloody Diarrhea. The 19 cases were separated by time and location, indicating no common source outbreak. 5/10 patients who were appropriately tested had positive stool cultures for E. coli O157:H7. Although more cases (12/19, 63%) occurred in warm months, April–September, a seasonal difference was not significant (p= 0.25). We compared the clinical features of these 19 patients to the 119 patients who had no apparent etiologies or associated conditions and were therefore defined as idiopathic TTP-HUS. | | Bloody Diarrhea | Idiopathic | p-value | |:---------------------------------------------------------------------------------------------------------------------- | --------------- | ----------- | ------- | | | (n=19) | (n=119) | | | Median values are presented for continuous data. Laboratory data: most abnormal value at diagnosis of TTP-HUS ± 7 days | | Age (years) | 59 | 48 | 0.109 | | Race (% Black) | 5% | 25% | 0.073 | | Gender (% female) | 79% | 74% | 0.781 | | Obesity (BMI ≥ 30 kg/m 2 ) | 21% | 40% | 0.120 | | Severe neurologic abnormalities | 63% | 49% | 0.243 | | Platelet count | 26 | 13 | 0.010 | | Hematocrit | 22 | 22 | 0.931 | | LDH | 1410 | 1305 | 0.115 | | Acute renal failure | 68% | 29% | <0.001 | | Response to PE | 84% | 82% | 1.000 | | ADAMTS13 deficiency (<5%) | 0% (0/13) | 30% (20/67) | 0.031 | | Death | 32% | 19% | 0.233 | | Relapse in 30 day survivors | 0% | 20% | 0.119 | Although women predominated in both groups, 18/19 patients with a prodrome of Bloody Diarrhea were White, consistent with the predominance of White subjects among children with Diarrhea-associated HUS, but distinct from the significantly higher incidence of Blacks among adult patients with idiopathic TTP-HUS (compared to the incidence among other races, p<0.0001). 3 patients with a prodrome of Bloody Diarrhea never had an abnormal serum creatinine, therefore the term TTP-HUS, rather than HUS, may better describe these patients. The only significant differences in presenting features and outcomes were the severity of thrombocytopenia, the relative frequency of acute renal failure, and the relative frequency of severe ADAMTS13 deficiency (<5% activity). ADAMTS13 activity was measured in 13 of the 19 patients with a prodrome of Bloody Diarrhea (median 50%, range 5–100%). We conclude that there is a continuous occurrence of TTP-HUS in adults preceded by a prodrome of Bloody Diarrhea, presumably related to Shiga toxin-producing enteric infections, even in the absence of recognized outbreaks. Plasma exchange is an appropriate treatment for adult patients with a prodrome of Bloody Diarrhea since they cannot be accurately distinguished from patients with idiopathic TTP, they have a high mortality rate, and they apparently respond to plasma exchange treatment.
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Thrombotic Thrombocytopenic Purpura-Hemolytic Uremic Syndrome (TTP-HUS) in Adults Following a Prodrome of Bloody Diarrhea.
Blood, 2004Co-Authors: Qurana F Lewis, Deirdra R Terrell, Bernhard Lammle, James N George, Johanna Kremer-hovinga, Sara K VeselyAbstract:Diarrhea, often Bloody Diarrhea caused by infection with E. coli 0157:H7, is the prodrome for typical HUS in children. In adults, HUS has been reported following epidemics of enteric infections, however the frequency and clinical features of sporadic HUS or TTP in adults following a prodrome of Bloody Diarrhea have not been characterized. In the Oklahoma TTP-HUS Registry, January 1, 1989 to December 31, 2003, 19 (6%) of 301 consecutive patients had a prodrome of Bloody Diarrhea. The 19 cases were separated by time and location, indicating no common source outbreak. 5/10 patients who were appropriately tested had positive stool cultures for E. coli O157:H7. Although more cases (12/19, 63%) occurred in warm months, April–September, a seasonal difference was not significant (p= 0.25). We compared the clinical features of these 19 patients to the 119 patients who had no apparent etiologies or associated conditions and were therefore defined as idiopathic TTP-HUS. | | Bloody Diarrhea | Idiopathic | p-value | |:---------------------------------------------------------------------------------------------------------------------- | --------------- | ----------- | ------- | | | (n=19) | (n=119) | | | Median values are presented for continuous data. Laboratory data: most abnormal value at diagnosis of TTP-HUS ± 7 days | | Age (years) | 59 | 48 | 0.109 | | Race (% Black) | 5% | 25% | 0.073 | | Gender (% female) | 79% | 74% | 0.781 | | Obesity (BMI ≥ 30 kg/m 2 ) | 21% | 40% | 0.120 | | Severe neurologic abnormalities | 63% | 49% | 0.243 | | Platelet count | 26 | 13 | 0.010 | | Hematocrit | 22 | 22 | 0.931 | | LDH | 1410 | 1305 | 0.115 | | Acute renal failure | 68% | 29% |
Deirdra R Terrell - One of the best experts on this subject based on the ideXlab platform.
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sporadic Bloody Diarrhea associated thrombotic thrombocytopenic purpura hemolytic uremic syndrome ttp hus in adults in oklahoma comparison to adults with severe adamts13 deficiency and to children with typical hus
Blood, 2007Co-Authors: Loan Nguyen, Deirdra R Terrell, Bernhard Lammle, Sara K Vesely, Charity A Karpac, Johanna Kremer A Hovinga, James N GeorgeAbstract:The frequency, presenting features and clinical outcomes of sporadic TTP-HUS following a prodrome of Bloody Diarrhea in adults are not described. The Oklahoma TTP-HUS Registry enrolled 237 consecutive patients over age 18 years with their first episode of clinically diagnosed TTP from 11-13-1995 (the date of our initial ADAMTS13 measurement) to 12-31-2006 for whom plasma exchange treatment (PEX) was requested. ADAMTS13 activity was measured on 218 (92%) patients immediately before their first PEX. 16 (7%) of these 218 patients presented with a prodrome of acute Bloody Diarrhea. 42 (19%) patients had ADAMTS13 activity Conclusions: TTP-HUS following Bloody Diarrhea is an endemic, sporadic disorder among adults that is less common and less familiar than in children. Distinct from children, adults with Bloody Diarrhea have a higher frequency of severe neurologic abnormalities and death; distinct from adults with severe ADAMTS13 deficiency, adults with Bloody Diarrhea are primarily white, have a higher frequency of acute renal failure, and have not relapsed. Although the role of PEX in the recovery of adult patients presenting with Bloody Diarrhea is unclear, PEX may be appropriate initial treatment since the mortality is high, many patients appear to respond, and patients with severe ADAMTS13 deficiency may also present with Bloody Diarrhea apparently caused by intestinal ischemia.
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thrombotic thrombocytopenic purpura hemolytic uremic syndrome ttp hus in adults following a prodrome of Bloody Diarrhea
Blood, 2004Co-Authors: Qurana F Lewis, Deirdra R Terrell, Bernhard Lammle, Johanna Kremerhovinga, James N George, Sara K VeselyAbstract:Diarrhea, often Bloody Diarrhea caused by infection with E. coli 0157:H7, is the prodrome for typical HUS in children. In adults, HUS has been reported following epidemics of enteric infections, however the frequency and clinical features of sporadic HUS or TTP in adults following a prodrome of Bloody Diarrhea have not been characterized. In the Oklahoma TTP-HUS Registry, January 1, 1989 to December 31, 2003, 19 (6%) of 301 consecutive patients had a prodrome of Bloody Diarrhea. The 19 cases were separated by time and location, indicating no common source outbreak. 5/10 patients who were appropriately tested had positive stool cultures for E. coli O157:H7. Although more cases (12/19, 63%) occurred in warm months, April–September, a seasonal difference was not significant (p= 0.25). We compared the clinical features of these 19 patients to the 119 patients who had no apparent etiologies or associated conditions and were therefore defined as idiopathic TTP-HUS. | | Bloody Diarrhea | Idiopathic | p-value | |:---------------------------------------------------------------------------------------------------------------------- | --------------- | ----------- | ------- | | | (n=19) | (n=119) | | | Median values are presented for continuous data. Laboratory data: most abnormal value at diagnosis of TTP-HUS ± 7 days | | Age (years) | 59 | 48 | 0.109 | | Race (% Black) | 5% | 25% | 0.073 | | Gender (% female) | 79% | 74% | 0.781 | | Obesity (BMI ≥ 30 kg/m 2 ) | 21% | 40% | 0.120 | | Severe neurologic abnormalities | 63% | 49% | 0.243 | | Platelet count | 26 | 13 | 0.010 | | Hematocrit | 22 | 22 | 0.931 | | LDH | 1410 | 1305 | 0.115 | | Acute renal failure | 68% | 29% | <0.001 | | Response to PE | 84% | 82% | 1.000 | | ADAMTS13 deficiency (<5%) | 0% (0/13) | 30% (20/67) | 0.031 | | Death | 32% | 19% | 0.233 | | Relapse in 30 day survivors | 0% | 20% | 0.119 | Although women predominated in both groups, 18/19 patients with a prodrome of Bloody Diarrhea were White, consistent with the predominance of White subjects among children with Diarrhea-associated HUS, but distinct from the significantly higher incidence of Blacks among adult patients with idiopathic TTP-HUS (compared to the incidence among other races, p<0.0001). 3 patients with a prodrome of Bloody Diarrhea never had an abnormal serum creatinine, therefore the term TTP-HUS, rather than HUS, may better describe these patients. The only significant differences in presenting features and outcomes were the severity of thrombocytopenia, the relative frequency of acute renal failure, and the relative frequency of severe ADAMTS13 deficiency (<5% activity). ADAMTS13 activity was measured in 13 of the 19 patients with a prodrome of Bloody Diarrhea (median 50%, range 5–100%). We conclude that there is a continuous occurrence of TTP-HUS in adults preceded by a prodrome of Bloody Diarrhea, presumably related to Shiga toxin-producing enteric infections, even in the absence of recognized outbreaks. Plasma exchange is an appropriate treatment for adult patients with a prodrome of Bloody Diarrhea since they cannot be accurately distinguished from patients with idiopathic TTP, they have a high mortality rate, and they apparently respond to plasma exchange treatment.
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Thrombotic Thrombocytopenic Purpura-Hemolytic Uremic Syndrome (TTP-HUS) in Adults Following a Prodrome of Bloody Diarrhea.
Blood, 2004Co-Authors: Qurana F Lewis, Deirdra R Terrell, Bernhard Lammle, James N George, Johanna Kremer-hovinga, Sara K VeselyAbstract:Diarrhea, often Bloody Diarrhea caused by infection with E. coli 0157:H7, is the prodrome for typical HUS in children. In adults, HUS has been reported following epidemics of enteric infections, however the frequency and clinical features of sporadic HUS or TTP in adults following a prodrome of Bloody Diarrhea have not been characterized. In the Oklahoma TTP-HUS Registry, January 1, 1989 to December 31, 2003, 19 (6%) of 301 consecutive patients had a prodrome of Bloody Diarrhea. The 19 cases were separated by time and location, indicating no common source outbreak. 5/10 patients who were appropriately tested had positive stool cultures for E. coli O157:H7. Although more cases (12/19, 63%) occurred in warm months, April–September, a seasonal difference was not significant (p= 0.25). We compared the clinical features of these 19 patients to the 119 patients who had no apparent etiologies or associated conditions and were therefore defined as idiopathic TTP-HUS. | | Bloody Diarrhea | Idiopathic | p-value | |:---------------------------------------------------------------------------------------------------------------------- | --------------- | ----------- | ------- | | | (n=19) | (n=119) | | | Median values are presented for continuous data. Laboratory data: most abnormal value at diagnosis of TTP-HUS ± 7 days | | Age (years) | 59 | 48 | 0.109 | | Race (% Black) | 5% | 25% | 0.073 | | Gender (% female) | 79% | 74% | 0.781 | | Obesity (BMI ≥ 30 kg/m 2 ) | 21% | 40% | 0.120 | | Severe neurologic abnormalities | 63% | 49% | 0.243 | | Platelet count | 26 | 13 | 0.010 | | Hematocrit | 22 | 22 | 0.931 | | LDH | 1410 | 1305 | 0.115 | | Acute renal failure | 68% | 29% |
Gaia Scavia - One of the best experts on this subject based on the ideXlab platform.
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Epidemiology of Shiga Toxin-Producing Escherichia coli Infections in Southern Italy after Implementation of Symptom-Based Surveillance of Bloody Diarrhea in the Pediatric Population.
International Journal of Environmental Research and Public Health, 2020Co-Authors: Daniela Loconsole, Mario Giordano, Antonio Parisi, Michele Quarto, Francesca Centrone, Marisa Accogli, Daniele Casulli, Anna Lisa De Robertis, Anna Morea, Gaia ScaviaAbstract:Shiga toxin-producing Escherichia coli (STEC) infections result in a significant public health impact because of the severity of the disease that, in young children especially, can lead to hemolytic–uremic syndrome (HUS). A rise in the number of HUS cases was observed in the Apulia region of Italy from 2013 to 2017, and so, in 2018, a symptom-based surveillance system for children with Bloody Diarrhea (BD) was initiated in order to detect and manage STEC infections. The objective of the study was to describe the epidemiology of STEC infections in children from June 2018 to August 2019. Children
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epidemiology of shiga toxin producing escherichia coli infections in southern italy after implementation of symptom based surveillance of Bloody Diarrhea in the pediatric population
International Journal of Environmental Research and Public Health, 2020Co-Authors: Daniela Loconsole, Mario Giordano, Antonio Parisi, Michele Quarto, Francesca Centrone, Marisa Accogli, Daniele Casulli, Anna Lisa De Robertis, Anna Morea, Gaia ScaviaAbstract:Shiga toxin-producing Escherichia coli (STEC) infections result in a significant public health impact because of the severity of the disease that, in young children especially, can lead to hemolytic–uremic syndrome (HUS). A rise in the number of HUS cases was observed in the Apulia region of Italy from 2013 to 2017, and so, in 2018, a symptom-based surveillance system for children with Bloody Diarrhea (BD) was initiated in order to detect and manage STEC infections. The objective of the study was to describe the epidemiology of STEC infections in children from June 2018 to August 2019. Children <15 years old with BD were hospitalized and tested for STEC. Real-time PCR for virulence genes (stx1, stx2, eae) and serogroup identification tests were performed on stool samples/rectal swabs of cases. STEC infection was detected in 87 (10.6%) BD cases. The median age of STEC cases was 2.7 years, and 60 (68.9%) were <4. Of these 87 cases, 12 (13.8%) came from households with Diarrhea. The reporting rate was 14.2/100,000, with the highest incidence in cases from the province of Bari (24.2/100,000). Serogroups O26 and O111 were both detected in 22/87 (25.3%) cases. Co-infections occurred in 12.6% of cases (11/87). Twenty-nine STEC were positive for stx1, stx2, and eae. Five cases (5.7%) caused by O26 (n = 2), O111 (n = 2), and O45 (n = 1) developed into HUS. A risk-oriented approach based on the testing of children with BD during the summer may represent a potentially beneficial option to improve the sensitivity of STEC surveillance, not only in Italy but also in the context of Europe as a whole.
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Case-management protocol for Bloody Diarrhea as a model to reduce the clinical impact of Shiga toxin-producing Escherichia coli infections. Experience from Southern Italy
European Journal of Clinical Microbiology & Infectious Diseases, 2019Co-Authors: Daniela Loconsole, Mario Giordano, Nicola Laforgia, Diletta Torres, Luisa Santangelo, Vincenza Carbone, Antonio Parisi, Michele Quarto, Gaia Scavia, Maria ChironnaAbstract:To describe an operating protocol for Bloody Diarrhea (BD) in a pediatric population as a rapid response to a public health threat represented by an excess of pediatric HUS cases in the Apulia region (Southern Italy) starting from 2013. The protocol was set up with the goal of correct clinical management of Shiga toxin-producing Escherichia coli (STEC) infections, reductions in subsequent cases of hemolytic uremic syndrome (HUS), and improved short- and long-term disease outcomes. The protocol consisted of rapid hospitalization of children with Bloody Diarrhea (BD), hematochemical laboratory tests every 12–24 hours, and prompt laboratory diagnosis of STEC. No antibiotics were recommended until diagnosis. Children positive for STEC infections underwent early vigorous volume expansion. In June–December 2018, 438 children with BD were hospitalized, of which 53 (12.1%) had a STEC infection. The most common serogroups were O26 (36.1%), O111 (23.0%), and O157 (14.8%). Thirty-one samples carried the stx2 gene. Four cases evolved into HUS (7.5%), all with favorable outcome despite neurological involvement in two cases. Prompt and accurate laboratory diagnosis of STEC infections is of the utmost importance in patients with BD for correct clinical management. The strict adherence to the protocol could reduce the progression rate of STEC infections to HUS and prevents complications. Enhanced BD surveillance may help reduce cases of pediatric HUS in Southern Italy.
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Correction to: Case-management protocol for Bloody Diarrhea as a model to reduce the clinical impact of Shiga toxin-producing Escherichia coli infections. Experience from Southern Italy.
European Journal of Clinical Microbiology & Infectious Diseases, 2019Co-Authors: Daniela Loconsole, Mario Giordano, Nicola Laforgia, Luisa Santangelo, Vincenza Carbone, Antonio Parisi, Michele Quarto, Gaia Scavia, Diletta Domenica Torres, Maria ChironnaAbstract:To describe an operating protocol for Bloody Diarrhea (BD) in a pediatric population as a rapid response to a public health threat represented by an excess of pediatric HUS cases in the Apulia region (Southern Italy) starting from 2013. The protocol was set up with the goal of correct clinical management of Shiga toxin-producing Escherichia coli (STEC) infections, reductions in subsequent cases of hemolytic uremic syndrome (HUS), and improved short- and long-term disease outcomes. The protocol consisted of rapid hospitalization of children with Bloody Diarrhea (BD), hematochemical laboratory tests every 12–24 hours, and prompt laboratory diagnosis of STEC. No antibiotics were recommended until diagnosis. Children positive for STEC infections underwent early vigorous volume expansion. In June–December 2018, 438 children with BD were hospitalized, of which 53 (12.1%) had a STEC infection. The most common serogroups were O26 (36.1%), O111 (23.0%), and O157 (14.8%). Thirty-one samples carried the stx2 gene. Four cases evolved into HUS (7.5%), all with favorable outcome despite neurological involvement in two cases. Prompt and accurate laboratory diagnosis of STEC infections is of the utmost importance in patients with BD for correct clinical management. The strict adherence to the protocol could reduce the progression rate of STEC infections to HUS and prevents complications. Enhanced BD surveillance may help reduce cases of pediatric HUS in Southern Italy.
