Postpartum hemolytic uremic syndrome in a 17-year-old Filipina primigravid

A 17-year-old Filipina primigravid developed acute renal failure secondary to hemolytic uremic syndrome (HUS) after undergoing emergency cesarean section for severe pre-eclampsia and abruptio placenta. She underwent hemodialysis with concurrent infusions of fresh-frozen plasma and packed red cells for 5 weeks. Renal biopsy revealed findings consistent with HUS with glomerular crescents. She received three doses of pulse methylprednisolone followed by oral prednisone. Renal function improved 5 weeks after the onset of HUS. The pathogenesis, differential diagnosis, and treatment options of postpartum HUS are discussed.     

A clinicopathological study of 24 children with hemolytic uremic syndrome

This study reports the pattern of renal injury in 24 North American children with hemolytic uremic syndrome (HUS), and the extent of extrarenal involvement in 9 of these children examined at autopsy. Fifteen of the 24 children were studied during the first 16 days of hospital-ization; their renal specimens demonstrated glomerular thrombotic microangiopathy (TMA) in 8 children, cortical necrosis in 1, and varying degrees of glomerular TMA and cortical necrosis in 6 children. Nine of the children were studied after 16 or more days of hospitalization; these patients had prominent renal arterial lesions. Of 9 children examined at autopsy, extrarenal microthrombi were identified in 8. In 4 children who died during the acute phase of the disease, hemorrhagic colonic necrosis (3 children) and pancreatic islet cell necrosis (2 children) were the principal extrarenal lesions encountered. Rare microthrombi were present in the brains of the 3 children who manifested seizures. Southwest Pediatric Nephrology Study Group (SPNSG Central Office, Baylor University Medical Center, Dallas, Tex. USA). Director: Ronald J. Hogg; Associate Directors: Fred G. Silva, F. Bruder Stapleton; Statistician: Joan S. Reisch; Administrative Assistant: Kaye Green. Participating Centers: Baylor College of Medicine, Houston, Tex., Phillip L. Berry, L. Leighton Hill, David Powell, Edith Hawkins; Baylor University Medical Center, Dallas, Tex., Ronald J. Hogg, Kaye Green; Tulane University Medical Center, New Orleans, La., Frank Boineau, John E. Lewy, Patrick Walker; University of Arkansas, Little Rock, Ark., Watson Arnold, Eileen Ellis, M. Melinda Sanders; University of Colorado Health Science Center, Denver, Colo., Gary M. Lum, William Hammond; University of Oklahoma Medical Center, Oklahoma City, Okla., James Wenzl, Geoffrey Altshuler, Sarah Johnson; University of Tennessee, Memphis, Tenn., F. Bruder Stapleton, Shane Roy, III, Robert J. Wyatt, Charles McKay, William Murphy; University of Texas Southwestern Medical Center, Tex., Billy S. Arant, Jr, Michel Baum, Fred G. Silva, Arthur Weinberg, Craig Argyle, Joseph Rutledge, Ed Eigenbrodt; University of Texas Medical School, Houston, Tex., Susan B. Conley, Jacques Lemire, Ron Portman, Ann Ince, Regina Verani; University of Texas Health Science Center at San Antonio, Tex., Michael foulds, Sudesh Makker, Kanwal Kher, Melanie Sweet, Victor Saldivar, Fermin Tio; University of Texas Medical Branch, Galveston, Tex., Ben H. Brouhard, Alok Kalia, Luther B. Travis, Lisa Hollander, Tito Cavallo, Srinivasan Rajaraman; University of Utah Medical Center, Salt Lake City, Utah, Eileen Brewer, Richard Siegler, Elizabeth Hammond, Theodore Pysher     

Childhood hemolytic uremic syndrome in Argentina: long-term follow-up and prognostic features

From January 1968 to December 1984, 312 infants and children with hemolytic uremic syndrome were admitted to our unit; 8 patients died (2.5%) during the acute phase; 118 children were followed as outpatients at yearly intervals for at least 10 years (mean follow-up 13 years, range 10 – 19.8 years). Four evolution patterns at the end of the follow-up were defined: group 1, complete recovery, 74 (62.7%); group 2, proteinuria with/without hypertension, 21 (17.7%); group 3, reduced creatinine clearance, often in conjunction with proteinuria and hypertension, 19 (16.1%); group 4, end-stage renal failure, 4 (3.4%). We investigated the association between several variables of the acute stage and the long-term evolution. Most non-anuric patients recovered completely (92.5%), while 38.4% of those with 1 – 10 days and 69.2% of those with 11 or more days of anuria had chronic renal sequelae. Similar results were found when analyzing the requirement for peritoneal dialysis. Of the patients with proteinuria at the 1-year control, 86% had renal abnormalities at the end of the follow-up. In our experience, although the final outcome was not predictable in every instance, the severity of acute renal failure – as determined by the days of anuria – and the presence of proteinuria 1 year after the acute phase were the most useful prognostic indicators. Received January 9, 1996; received in revised form April 16, 1996; accepted May 10, 1996     

Low levels of serum erythropoietin in children with endemic hemolytic uremic syndrome

Serum erythropoietin (EPO) levels were measured in ten previously non-transfused children with hemolytic uremic syndrome (HUS). Complete blood cell count, serum EPO, and renal function tests were carried out upon admission and weekly thereafter. Blood samples were obtained: (1) prior to the first transfusion; (2) after the first transfusion but before recovery from renal failure; (3) during the recovery stage. All patients required transfusions (mean 1.8±0.8 per child). Absolute values of EPO correlated positively with the hematocrit during the three stages (r = 0.53, 0.36, and 0.12, respectively) which is opposite to expected results. The observed EPO logarithm/predicted EPO logarithm upon admission was low (0.70±0.08), falling further during stage 2 (0.57±0.03), but increasing thereafter (0.78±0.07) without reaching normal values. The reticulocyte production rate followed a parallel course (0.74±0.14, 0.54±0.11, and 0.60±0.10, respectively). On comparing the observed serum EPO levels with those expected, 9 of 11 pre-transfusion samples showed low values; in stage 2, all samples were below normal; in the recovery phase most (77.8%) were still low. Our results show an inadequate EPO synthesis in children with HUS, which could play an important pathogenic role, since it aggravates the severity of the existing hemolytic anemia; the secondary inhibitory effect of repeated transfusions exacerbates this inadequate synthesis. Received March 22, 1997; received in revised form and accepted October 1, 1997     

Hypocomplementemic autosomal recessive hemolytic uremic syndrome with decreased factor H

We describe the clinical course, complement components, and pathological findings of 10 infants with autosomal recessive hemolytic uremic syndrome (HUS). All patients were members of one extended highly inbred Bedouin kindred. The median age of presentation was 2 weeks (range 1–20 weeks). Eight patients died, 2 patients are alive, on dialysis. Renal biopsies revealed thrombotic microangiopathy with a predominant early arteriolar involvement and subsequent development of ischemic glomerular changes. Immunofluorescence was positive for C3 in glomeruli. All patients had low complement components levels during and between relapses, and in some this was evident soon after birth and prior to the onset of symptoms. This deficiency could not be normalized by repeated plasma transfusions. Biosynthetic labelling of patients’ fibroblasts demonstrated normal rates of C3 protein synthesis. Serum factor H levels were greatly decreased or absent in 4 patients tested and moderately decreased in 15 of 23 healthy unaffected siblings and patients. This defect may cause complement activation and consumption, possibly at the endothelial cell level. Received April 18,1997; received in revised form and accepted January 27, 1998     

Development of an experimental hemolytic uremic syndrome in rats

Escherichia coli strains producing Shiga toxins (Stxs) colonize the lower gastrointestinal tract and cause watery diarrhea, hemorrhagic colitis, and hemolytic-uremic syndrome (HUS). HUS is characterized by hemolytic anemia, thrombocytopenia, and acute renal failure. Oliguria associated with acute tubular necrosis and microangiopathic thrombosis has been reported as the most common cause of renal failure in Argentinean children. Our study was undertaken to obtain a model of HUS in rats that was similar to the clinical and renal histopathology findings described in humans. Rats were intraperitoneally inoculated with culture supernatant from recombinant E. coli expressing Stx2. Glomerular filtrate volume evaluated from clearance of creatinine resulted in a progressive reduction (from 53% at 24 h to 90% at 48 h). Urine volume increased significantly at 24 h but returned to normal levels at 48 h. Evidence of thrombocytopenia, anemia and leukocytosis was documented. Macroscopic analysis revealed a hyperemic peritoneal face with intestinal water accumulation. The kidneys were friable and congestive. Histopathological analysis showed glomerular and tubular necrosis as well as microangiopathic thrombosis. Our findings indicated vascular damage and kidney lesions similar to those described in humans with HUS.     

Post-dysenteric hemolytic uremic syndrome in children during an epidemic of Shigella dysentery in Kwazulu/Natal

We report 81 of 107 cases of hemolytic uremic syndrome (HUS), admitted between July 1994 and February 1996, following an outbreak of Shigella dysenteriae type 1 dysentery in Kwazulu/Natal. All patients, excluding 1, were black with a mean age of 38 months (range 1 – 121); 50 (61.7%) were males. The mean duration of dysentery was 11.3 days (range 1 – 41) and HUS 15 days (range 1 – 91). Most patients had acute oliguric renal failure (90.1%); 42 (51.6%) required peritoneal dialysis. Complications included encephalopathy 30 (37.0%), convulsions 12 (14.8%) and hemiplegia 2 (2.3%), gastrointestinal perforation 8 (9.9%), protein losing enteropathy 26 (32.1%), toxic megacolon 4 (4.9%), rectal prolapse 5 (6.2%), hepatitis 11 (13.6%), myocarditis 5 (6.2%), congestive cardiac failure 3 (3.7%), cardiomyopathy 3 (3.7%), infective endocarditis 1 (1.2%), septicemia 15 (18.5%), disseminated intravascular coagulation 17 (21%). Leukemoid reactions were found in 74 (91.3%) patients, hyponatremia in 56 (69.1%), and hypoalbuminemia in 67 (82.7%). Stool culture for Shigella dysenteriae type I was positive in only 7 (8.6%) patients; Shiga toxin assays were not performed. Outcome was as follows: recovery 32 (39.5%), impaired renal function 8 (9.9%), chronic renal failure 26 (32.1%), end-stage renal disease 1 (1.2%), and death 14 (17.3%) patients. Received November 26, 1996; received in revised form and accepted April 15, 1997     

Cardiomyopathy: a late complication of hemolytic uremic syndrome

This report describes a child who presented with classic hemolytic uremic syndrome (HUS) and 4 months later developed a life-threatening but reversible cardiomyopathy with global cardiac dysfunction and a left ventricular ejection fraction of 14%. There was no evidence of electrolyte abnormalities, anemia, hypertension, severe fluid overload, or viral infection. Endomyocardial biopsies were consistent with a dilated cardiomyopathy. This paper highlights the importance of considering the diagnosis of associated cardiomyopathy when presenting with late-onset edema following HUS. Received February 12, 1996; received in revised form and accepted August 22, 1996     

Steroids in the hemolytic uremic syndrome

To determine whether steroids could be of clinical benefit in the treatment of the hemolytic uremic syndrome (HUS), we conducted a randomized, double-blinded, placebo-controlled trial of methylprednisone (5 mg/kg per day in four oral doses over 7 days), in children with HUS during the acute phase. Ninety-two patients with typical HUS (47 receiving placebo and 45 receiving steroids) were investigated for neurological, hematological, and nephrological variables. There were no differences between groups in the number of convulsive episodes or transfusion requirements during the hospital stay. Serum creatinine levels were slightly increased on day 10 in the placebo group compared with the steroid group (P = 0.06) and declined significantly between days 1 and 10 only in the steroid group (P = 0.001). In the 51 patients with anuria (24 placebo, 27 steroids), median serum creatinine levels were reduced in the steroid group compared with the placebo group on the 10th day (P = 0.01). Differences in median days of oliguria [11.5 versus 8 (P = 0.28)], anuria [5 versus 7 (P = 0.20)], and dialysis [12 versus 10 (P = 0.26)] for the placebo and the steroid group respectively were not significant. Our data suggest that oral steroids are not able to modify hematological, neurological, or nephrological clinical parameters during the acute phase of childhood HUS, even though they do seem to be associated with a more rapid decline in serum creatinine levels. Received April 9, 1997; received in revised form September 8, 1997, accepted September 10, 1997     

Hemolytic uremic syndrome in children in northern India

We observed 73 patients with the hemolytic uremic syndrome (HUS) in 9 years (1980–1988), comprising 34% of patients with acute renal failure treated over the same period. There were 53 boys and 20 girls; 59% were below the age of 2 years and 33% between 2 and 5 years. Acute, usually severe dysentery, responding poorly to various antibiotics, was the prodromal illness in 80%, whereas 12% had watery diarrhea. Most patients had severe renal involvement with anuria in 56% and oliguria in 30%. A polymorphonuclear leukocytosis was present in 85% of cases, but had no correlation with the highest levels of blood urea. Coagulation abnormalities suggesting consumption coagulopathy were found in 24 of 30 cases. The results of stool culture showedShigella species in 7 cases and nontyphoidalSalmonella in 9.Escherichia coli were isolated in 11 cases, but were not further characterized. Renal biopsy showed total or patchy cortical necrosis in 20 of 50 cases. The patients were managed with supportive care, including transfusion of fresh blood or plasma and dialysis as required. The mortality was 60%, being chiefly related to the duration of renal failure and presence of renal cortical necrosis, whereas persistent dysentery and infections were complicating factors. The presence of convulsions and coagulation defects had no relation to the outcome. Our observations indicate that HUS in children in northern India is mostly related to dysentery, likely to be shigellosis, and is usually associated with severe renal damage and a high death rate.     

Racial incidence of hemolytic uremic syndrome

Hemolytic uremic syndrome (HUS) is the most common cause of acute renal failure in children and is caused by infection with verotoxin-productingEscherichai coli. There is no consensus on the relative incidence of HUS in blacks and whites. An equal racial incidence has been reported by two centers with small black populations. A series from Washington D. C. reported a low incidence in blacks. The population of Alabama is 32% black and 66% white. The Children's Hospital of Alabama admission rate has a similar racial distribution (35% black, 65% white). A record review from 1980–1992 identified 45 patients with HUS; 43 (96%) were white and only 2 (4%) were black. Based on census data for Alabama in 1980 and 1990, this gives an average annual incidence of HUS of 0.45 per 100,000 in whites and of 0.043 per 100,000 in blacks (P<0.001, Fischer's exact test). Similar results were found in the group of patients with HUS and a history of diarrhea; whites 0.39 and blacks 0.02 (P<0.001). However, in those with no history of diarrhea there was no significant racial difference: whites 0.05 and blacks 0.02. There were too few blacks to compare clinical course and outcome. We conclude that typical diarrhea-associated HUS is a relatively rare disease in blacks compared with whites. The reasons are unclear.     

Post-dysenteric hemolytic uremic syndrome in Bulawayo, Zimbabwe

An outbreak of dysentery in Zimbabwe was associated with a high mortality, especially in children who developed hemolytic uremic syndrome (HUS). To examine the causes of high mortality from HUS and to suggest measures that could reduce the case fatality rate, clinical and laboratory features of 91 children with dysentery were reviewed. Of these, 14 developed HUS; their findings were compared with age-matched controls with dysentery only. Persistent alteration of consciousness after rehydration, pallor, and oliguria were early clinical indicators of HUS. Leukocytosis and leukemoid reaction, microhematuria, and non-resolving hyponatremia distinguished children with HUS from those with dysentery. While Shigella dysenteriae type I was responsible for the dysentery outbreak in the community, most stool cultures of children with HUS were negative. Mortality from HUS was high. Late recognition of HUS and a lack of peritoneal dialysis could have contributed to the fatal outcome in some cases. Early recognition of HUS through close observation of children with dysentery and appropriate laboratory investigations with referral to a hospital, where peritoneal dialysis is available, should improve the outcome. Received: 24 August 2000 / Revised: 30 July 2001 / Accepted: 31 July 2001     

Prognosis of Streptococcus pneumoniae-induced hemolytic uremic syndrome

Streptococcus pneumoniae-induced hemolytic uremic syndrome (HUS) is known to be a severe acute disease leading to death in one-third of cases, but data regarding the long-term follow-up are lacking. A new series of 11 patients with Streptococcus pneumoniae-induced HUS associated with meningitis and pneumonia constituted a multi-center review. Among 9 patients with a severe acute infectious disease, 3 died from meningitis and 1 from neurological sequelae after a partial recovery of renal function. The mean duration of dialysis was 32 days in patients with acute renal failure who survived the acute infectious period. Cortical necrosis was documented in five of six kidney specimens. Among the 7 surviving patients, 5 developed end-stage renal failure 4–17 years later. Received: 27 July 2000 / Revised: 18 December 2000 / Accepted: 19 December 2000     

De novo hemolytic uremic syndrome following renal transplantation

We report a case of complete recovery of renal function in a patient with de novo hemolytic uremic syndrome (HUS) following renal transplantation. This 3-year-old girl had none of the factors known to contribute to the development of HUS in transplant recipients. This case illustrates the usefulness of renal biopsy in the accurate diagnosis and management of dysfunction in the allograft following renal transplantation.     

Development of focal segmental sclerosis and hyalinosis in hemolytic uremic syndrome

Renal biopsies from 19 boys and 11 girls, most with moderate or severe forms of hemolytic uremic syndrome (HUS) of the classic diarrhea-associated type, were analyzed as part of their long-term follow-up. Patients were biopsied because of late or persistent proteinuria, hypertension, and prolonged renal failure. The median length of follow-up was 11.2 years (range 0.9 – 22.0 years). Four histological groups were identified: focal segmental glomerulosclerosis and hyalinosis (FSGSH) (17 patients), diffuse mesangial proliferative glomerulonephritis (DMPGN) (9 patients), diffuse glomerulosclerosis (2 patients), and minimal glomerular changes (2 patients). The median interval between the onset of disease and renal biopsy was significantly shorter in DMPGN than in FSGSH (P <0.001). The pathological findings may be the expression of two different stages of the same dynamic process: a regular sequence of glomerular lesions consisting of early DMPGN, followed by FSGSH. This lesion would ultimately lead to the final stage of global glomerulosclerosis. At the last examination, only a quarter of the patients had normal renal function. These observations also confirm that prolonged oligoanuria during the acute stage of HUS frequently results in an unfavorable long-term prognosis. Received December 16, 1994; received in revised form and accepted February 12, 1996     

Hemolytic uremic syndrome due to Capnocytophaga canimorsus bacteremia after a dog bite

The hemolytic uremic syndrome (HUS) is known to have several causes, including infectious diseases, drugs, pregnancy, and malignant disease. We report a patient who developed acute renal failure attributable to HUS in the course of Capnocytophaga canimorsus bacteremia. Acute tubular necrosis as well as HUS should be considered as a cause of acute renal failure in the setting of Capnocytophaga canimorsus bacteremia.     

Hemolytic uremic syndrome in a child with leukemia and cytomegalovirus infection

A 2-year-old boy had a severe cytomegalovirus (CMV) infection with multi-organ involvement, while on maintenance therapy for acute lymphoblastic leukemia. The patient was treated with intravenous gancyclovir, with a marked improvement in his clinical status, with the exception of a progressive deterioration of the renal function. He also developed hemolytic anemia and thrombocytopenia, suggesting a diagnosis of atypical hemolytic uremic syndrome (HUS). A percutaneous renal biopsy showed lesions consistent with HUS, but no evidence of CMV infection. The patient had a good clinical outcome with no evidence of renal sequelae. We report a rare association of CMV infection and HUS in the pediatric age-group, which suggests a possible cause-effect relationship that deserves further evaluation. Received: 28 October 1999 / Revised: 4 April 2000 / Accepted: 4 April 2000     

8例溶血性尿毒症综合征临床分析

目的探讨溶血性尿毒症综合征（HUS）的临床特点和治疗方法。方法回顾性分析自1997年12月至2007年12月收治的8例HUS患者的临床特点及治疗方法。结果成人7例,儿童1例。诱因为产后3例,呼吸道感染2例,肠道感染1例,呼吸道合并肠道感染1例,无明显诱因1例。8例均表现为溶血性贫血、血小板减少和急性肾衰竭。病情属重型5例,轻型3例。肾损害表现为少尿1例,无尿2例,肉眼血尿3例,高血压8例,水肿6例。8例均采用综合治疗：抗感染、降压等一般治疗;血液透析、改善肾循环、血液灌流、血浆置换、新鲜冰冻血浆输注以及肾上腺皮质激素等。2例治愈,4例好转,2例病情恶化放弃治疗。治疗后血液系统及生化指标均有改善,血红蛋白（Hb）、血小板（PLT）、血肌酐（SCr）、总胆红素（TBIL）、血乳酸脱氢酶（LDH）与治疗前比较,差异有统计学意义（P〈0．05）。结论HUS较罕见,属临床急症,早期诊断、及时正确治疗有助于改善短期预后。