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     

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     

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     

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.     

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。     

溶血性尿毒症性综合征14例临床分析

目的探讨溶血性尿毒症性综合征（HUS）的病因及临床诊疗。方法分析我院近10年来14例HUS患者的临床资料。结果13例患者具备HUS的典型临床表现,包括溶血性贫血、血小板减少和急性肾衰竭。1例患者虽不具有HUS的典型临床表现,但肾活检病理显示为典型的HUS肾脏微血管病变。经过包括血液净化在内的综合治疗,9例患者获得临床治愈,5例患者死亡。结论该病诊断主要依据病史、临床表现及病理检查进行综合分析,早期改善肾衰竭、纠正重度贫血并积极进行抗凝是治疗关键。     

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.     

Early erythropoietin reduced the need for red blood cell transfusion in childhood hemolytic uremic syndrome—a randomized prospective pilot trial

Childhood hemolytic uremic syndrome (HUS) is most often caused by enterohemorrhagic Escherichia coli (EHEC). Due to severe hemolysis, red blood cell (RBC) transfusions are often necessary, and anemia is aggravated by low erythropoietin (EPO) levels caused by acute renal failure. In a single center, prospective study, we randomized ten children with EHEC-positive HUS into two therapeutic groups: one receiving EPO treatment (median age 2 years, age range 1–3 years) and the other receiving standard therapy (median age 2 years, age range 1–6 years). Red blood cell transfusions were performed when the hemoglobin level (Hb) fell below 5 mg/dl. The number of RBC transfusions was compared in both groups. The Hb level at admission was comparable between both groups (6.4 vs. 8.1 mg/dl, P > 0.05, t-test). However, children in the EPO group required a significantly lower mean number of RBCs than those in the non-EPO group (0.2 vs. 1.4, P < 0.04, t-test). Based on these results, we suggest that the early administration of EPO at the time of hemolytic anemia and beginning renal failure may attenuate renal anemia in children with EHEC-induced HUS and thereby reduce the number of RBC transfusions required. The results of this pilot study will have to be confirmed in a larger multicenter trial.     

FK 506 as an alternative in cyclosporin-induced hemolytic uremic syndrome in a kidney transplant recipient

We describe a patient who received a living related kidney transplant that worked very well initially but developed oliguria and renal failure within 1 week and required dialysis. Clinical and hemological changes, as well as renal biopsy, confirmed the diagnosis of cyclosporin-induced hemolytic uremic syndrome. The patient did not respond to antirejection therapy or plasma exchange but did respond to the withdrawal of cyclosporin A and the commencement of FK 506.     

Childhood hemolytic uremic syndrome is associated with adolescent-onset diabetes mellitus

Extra-renal manifestations of the hemolytic uremic syndrome in children are well described. Pancreatic involvement may manifest as transient hyperglycemia and permanent diabetes mellitus. Two previous case reports demonstrate short periods of “remission” between initial hyperglycemia and the development of permanent diabetes mellitus. We report an unusual case of a two-year-old Caucasian boy whose HUS-associated hyperglycemia resolved shortly after the acute phase of his illness only to recur as permanent diabetes mellitus at puberty. To our knowledge no other case is reported that demonstrates such a long interval between initial presentation and the development of permanent diabetes mellitus.     

The use of intravenous gammaglobulin in the treatment of typical hemolytic uremic syndrome

Nine children with acute typical post-diarrhea hemolytic uremic syndrome (HUS) were treated with intravenous gammaglobulin (IVIG). These children were compared to nine children with HUS who did not receive IVIG. The use of IVIG did not appear to have a beneficial effect on eight of the nine treated children. There were no significant differences found in the duration of hemorrhagic colitis, thrombocytopenia, elevation of the white blood count (WBC), anuria, dialysis, or hospitalization, or the presence of a central nervous system complication or pancreatitis. Although no significant difference was found in the duration of thrombocytopenia, there was a trend towards a longer duration of thrombocytopenia in children treated with IVIG (P=0.13). One child demonstrated both an increase in her platelet count and a decrease in her WBC count within 24 h of receiving her first dose of IVIG.     

溶血尿毒综合征的诊治进展

溶血尿毒综合征(HUS)属于血栓性微血管性疾病。以微血管性溶血性贫血(可找到红细胞碎片)、血小板减少、肾功能损伤为特点。微血栓主要分布于肾脏。感染、多种毒素、抗内皮细胞抗体、药物等因素使内皮损伤是发病的关键。近年来随着对HUS的病因和发病机制有了新的认识,输注血浆和血浆置换是目前治疗HUS最有效的方法,已大大提高了HUS患者的生存率。     

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     

Isolated abducens nerve palsy in hemolytic uremic syndrome

Isolated abducens nerve (VI cranial nerve) palsies are reported in a dialyzed child with Escherichia coli 0157:H7-associated hemolytic uremic syndrome (HUS). There were no other neurological manifestations and he made a complete recovery, suggesting that isolated abducens nerve palsy in HUS may represent a minor neurological complication.     

Recovery of renal function after long-term dialysis in hemolytic uremic syndrome

Long-lasting recovery of renal function of the native kidneys after prolonged renal replacement therapy is rare. An 8-year-old girl and a 3-year-old boy had suffered from acute atypical and diarrhea-associated hemolytic uremic syndrome (HUS), respectively, with subsequent apparent end-stage renal failure. Both recovered renal function after long-lasting anuria and dialysis of 8 and 16 months, respectively. After prolonged follow-up, i.e., 7 and 5 years after cessation of dialysis, they attained normal or slightly reduced renal function (plasma creatinine 84 and 90 µmol/l, respectively). In addition, growth and cognitive development were normal. We conclude that caution is appropriate before offering early renal transplantation to pediatric patients with presumed end-stage kidney disease secondary to HUS.     

Acquired glomerulocystic kidney disease following hemolytic uremic syndrome

Glomerulocystic kidney disease (GCKD) is a rare congenital condition that is usually reported in infants and young children. Only five cases of acquired GCKD after an acquired renal disease have been reported. Among these, two patients have developed cystic glomerular lesions following hemolytic uremic syndrome (HUS). We report a third case in a 2-year-old patient with this association. Common features between these three cases include atypical HUS, development of GCKD after prolonged peritoneal dialysis treatment, severe hypertension, and normal-sized kidneys without development of macroscopic cysts. Pathology findings in our patient include heavy expression of epidermal growth factor receptor in proximal tubules and evidence of obstruction of the glomerular outflow. We speculate that cystic dilatation of the Bowman’s capsule may be secondary to ischemic lesions leading to proximal tubular obstruction. Received: 6 October 2000 / Revised: 1 February 2001 / Accepted: 13 February 2001     

Blood pressure in the long-term follow-up of children with hemolytic uremic syndrome

The hemolytic uremic syndrome (HUS) is the most common cause of acute renal failure (ARF) in young children. Most patients recover from the acute phase of the illness but they may develop arterial hypertension (AH) after many years, even in the absence of signs of renal impairment during short-term follow-up. In this study, we performed casual blood pressure (BP) measurement, 24-h blood pressure monitoring (ABPM), and a Bruce walking treadmill study (ET) in 24 children (aged 5–15 years, 13 males, 11 females) with a history of HUS and normal renal function during follow-up (median 5.8 years, range 1.8–12.4 years). There were 22 children (91%) with prodromal diarrhea associated with HUS and 20 (83%) underwent dialysis during the acute illness. All children had normal casual BP measurement. Of 13 children (54%) with normal ABPM, 5 patients (38%) had an abnormal BP response during the ET study. There were 4 (58%) of the 7 patients with AH by ABPM (29%) and an abnormal BP response during ET. These findings suggest that ET could be a useful means of identifying children with a history of HUS that could be at risk of future AH even if they had normal renal function, casual BP, and ABPM during long-term follow-up. These results should be confirmed with a large prospective clinical study.