Pediatric case study: hemolytic uremic syndrome

The nursing interventions necessary to care for this critically ill child were professionally challenging. Meeting the psychosocial, educational, and supportive needs of the family during this period of uncertainty, as well as, providing the dialysis therapy and support for this fragile child demonstrated the unique contributions of professional nursing. All outcomes were met, but the patient's extremely poor myocardial function made dialysis treatments and volume control difficult. The use of CVVHD was instrumental to providing dialysis in a safe, effective manner. The nursing care for S.B.K. and her family was physically and emotionally draining, but seeing her neurological status return was the great reward for all of our efforts.     

Coombs-positive hemolytic uremic syndrome

Two children presenting typical clinical features of the hemolytic-uremic syndrome had a positive direct Coombs test that remained positive throughout the illness. It is probable that an upper respiratory tract infection precipitated an antigen-antibody reaction that caused the autoimmune hemolytic anemia and immune-complex glomerulonephritis.     

Treatment of hemolytic uremic syndrome

Since the pathogenesis of hemolytic uremic syndrome (HUS) has not been fully understood, it is difficult to establish a therapy protocol for HUS. Until now, there have been no effective treatments of HUS, evaluated by prospective controlled studies. Progress in the management of acute renal failure has improved the prognosis of HUS. Recently, the effectiveness of plasma infusion has been considered to be doubtful. Antiplatelet therapy and vitamin E administration may be recommended to treat typical HUS without severe complications. In addition to these treatments, plasma exchange using fresh plasma or fresh frozen plasma is recommended to treat HUS in the adult, atypical HUS and typical HUS with severe complications in childhood, considering the poor prognosis in these conditions.     

Clostridium septicum infection and hemolytic uremic syndrome

Ultrasonography of the hip was performed sequentially by two different examiners in 75 infants. The ultrasound strips were reviewed twice by three paediatric orthopaedic surgeons and classified by the Graf method. The intraobserver and interobserver agreement between the interpretations was analysed using simple and weighted kappa coefficients calculated for agreement on the Graf classification and for grouping as normal (types 1A to 2A), and abnormal requiring treatment (types 2B to 4). When examining the same ultrasound strip, intraobserver agreement for the Graf classification was substantial (mean kappa 0.61), but interobserver agreement was only moderate (kappa 0.50). For the grouping into normal and abnormal, the mean kappa value for intraobserver agreement was 0.67 and for interobserver agreement 0.57. Because of the significant differences in agreement between normal and abnormal hips, we analysed a subgroup of those with at least one abnormal interpretation. Intraobserver agreement within this subgroup showed moderate reliability (kappa 0.41), but interobserver agreement was only fair (kappa 0.28). Interpretations of two different strips performed sequentially showed significantly lower agreement with an intraobserver kappa value of 0.29 and an interobserver value of 0.28. In the subgroup with at least one abnormal reading, the intraobserver kappa was 0.09 and the interobserver 0.1. Our findings suggest that both the technique of performing ultrasonography and the interpretation of the image may influence the result.     

Clinical aspects of the hemolytic uremic syndrome

Multiple organ dysfunction syndrome (MODS) appears to be the result of a complex program influenced by multiple factors, including environmental, physiological, and immunological conditions. Thus, an uncontrolled inflammatory response following a stochastic event, the initial injury, is believed to be the cause for the development of this syndrome. Several lines of evidence suggest that a genetic component could contribute to the regulation of the inflammatory response, as well, but no direct evidence demonstrates a heritable predisposition to MODS. In the present study, a genetic contribution was demonstrated for the inflammatory response induced by the administration of bacterial lipopolysaccharide (LPS) in different, genetically distinct strains of inbred mice. A survey of five inbred strains showed that mortality following administration of Escherichia coli LPS (20 mg/kg) was highest in C57BL/6J (B6) mice, while A/J mice were the most resistant. Accordingly, B6 and A/J mice were examined further for differences in the inflammatory response elicited by LPS. B6 mice showed higher levels of circulating interleukin-1beta and interleukin-6, as well as higher mRNA levels of hepatic beta-fibrinogen (an acute-phase gene) and metallothionein. Surprisingly, the circulating levels of tumor necrosis factor-alpha were significantly higher in A/J than in B6 mice after LPS administration. Since B6 and A/J mice were bred and raised in identical environments and received the same LPS challenge, the contrasting inflammatory response that was observed is largely attributable to genetic differences between these two strains. These data illustrate that the response to injury could be modulated by the genetic background of the individual. This information may be pertinent for the care of critically ill patients.     

The pathogenesis and treatment of hemolytic uremic syndrome

BACKGROUND: This study evaluated the cost-effectiveness of posttreatment surveillance after radiation therapy for early stage seminoma. METHODS: From 1988-1995, 47 patients with Stage I, and 11 patients with Stage II seminoma (based on the Royal Marsden staging system) received paraaortic and pelvic lymph node radiation after radical orchiectomy. Patient records were reviewed and patients surveyed to determine the tests ordered for posttreatment surveillance. RESULTS: With a median follow-up of 55 months, there were 2 recurrences among the 58 patients. Eight-year actuarial disease free survival was 93%, with 100% overall survival. Information concerning follow-up screening was available for 56 patients. The follow-up tests ordered included 842 physical examinations, 815 chest X-rays, 839 serum markers, 250 computerized tomography scans, and 112 abdominal plain films. The total cost of these examinations according to 1996 private sector charges and 1996 Medicare reimbursement rates, respectively, was $602,673.01 (average $10,762.02 per patient) and $282,746.52 (average $5049.05 per patient). The two patients who experienced recurrence were diagnosed independently of their posttreatment screening program. One patient recurred 7.5 months after his original diagnosis with an isolated spinal cord compression. The second patient had a mediastinum recurrence > 6 years after treatment. At last follow-up, both patients were disease free after salvage treatment. CONCLUSIONS: Patients with early stage seminoma treated with orchiectomy and radiation have excellent disease free survival rates. The cost of the surveillance program studied does not appear to be justifiable.     

Primary Sj?gren''s syndrome complicated by hemolytic uremic syndrome

A primary plasmacytoma of the testis is an extremely rare tumor. To date less than 10 cases have been published. Typically a firm mass of the testis or also of the epididymis is found. Histologically, infiltration can be seen with atypical plasma cells compressing the seminiferous tubuli. Immunohistochemical examination shows a PAS-positive reaction. Monoclonality can be confirmed by identification of the IgA lambda chain. Therapy of choice is the radical orchiectomy. The prognosis of primary plasmacytoma of the testis seems to be good. Nevertheless, a follow-up of some years is necessary to detect possible intramedullar manifestations.     

Hepatic veno-occlusive disease in a case of polymyositis associated with thrombotic thrombocytopenic purpura/hemolytic uremic syndrome

A 50-year-old woman was treated with prednisolone for polymyositis. During the therapy, thrombotic thrombocytopenic purpura/hemolytic uremic syndrome (TTP/HUS) occurred. Neither plasma infusion nor plasma exchange could relieve the clinical manifestations of TTP/HUS. Moreover, massive ascites appeared and worsened her condition. She died approximately one year after the diagnosis of polymyositis. The autopsy revealed centri-lobular hepatic necrosis and nonthrombotic obliteration of hepatic small veins. The diagnosis of hepatic veno-occlusive disease (VOD) was made. It was suspected that common factors other than cytoreductive therapy had damaged the endothelium and caused TTP/HUS and VOD in a case of polymyositis.     

Secondary membranoproliferative glomerulonephritis due to hemolytic uremic syndrome: an unusual presentation

In vitro selection can be used to generate nucleic acid ligands (aptamers) to target molecules ranging in size and structure from cations to cells. However, the selection process is repetitive and time-consuming. We have automated a protocol for in vitro selection using an augmented Beckman Biomek 2000 pipetting robot. The automated selection procedure requires the integration of four devices and the optimization of four molecular biology methods, and is one of the most complex automated protocols attempted to date. Initial attempts at selection yielded robust replication parasites, but optimization of the automated selection procedure suppressed the emergence of these parasites and led to the selection of true nucleic acid ligands. Automated selection can now be used to generate nucleic acid aptamers in days rather than weeks or months.     

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.     

Post transfusion non-cardiogenic pulmonary edema in a patient with chemotherapy-related hemolytic uremic syndrome

Recent experimental studies performed at the Dept of Orthopaedics at Gothenburg University have systematically assessed the basic pathophysiological mechanisms behind sciatica due to disc herniation. It has hereby been seen that the epidural presence of nucleus pulposus may induce structural and functional injury of the adjacent nerve roots similar in magnitude to those induced by mechanical deformation. By increasing the knowledge of the substances and mechanisms leading to this nucleus pulposus-induced nerve injury it may be assumed that sciatica to a certain extent may be treated by specific pharmacologic agents in the future, in combination with existing treatment modalities.     

Cytokines in childhood hemolytic uremic syndrome and thrombotic thrombocytopenic purpura

Serum and urine cytokines were analyzed in children with hemolytic uremic syndrome (HUS) and thrombotic thrombocytopenic purpura (TTP). Interleukin-6 (IL-6) was elevated in the serum of 33 of 35 children with HUS (94%) and in 2 of 2 children with recurrent TTP. Serum IL-6 was higher in children with HUS who developed anuria, extrarenal manifestations during the acute phase of illness and/or chronic renal sequelae. Tumor necrosis factor-alpha (TNF-alpha) was detected in the serum of 7 patients with HUS (20%) and 1 patient with TTP. IL-6 and TNF-alpha were elevated in the urine of 4 of 4 children with HUS and 2 of 2 children with TTP. Urinary levels were higher than serum levels, suggesting local production of cytokines in the urinary tract. Sequential serum and urine samples showed that IL-6 levels varied with disease activity. IL-6 and TNF-alpha were not detected in the serum (n = 25) and urine (n = 15) of healthy children. We conclude that IL-6 in urine may be used to monitor disease activity in HUS and TTP.     

Reversible MR findings of hemolytic uremic syndrome with mild encephalopathy

We report the reversible MR findings in a 7-year-old girl with hemolytic uremic syndrome and mild encephalopathy. The splenium of the corpus callosum showed isointense to low signal intensity on T1-weighted images and high signal intensity on T2-weighted images, representing local edema. These findings returned to near normal on MR images obtained 1 week later. The patient recovered without CNS impairment.     

Tacrolimus (FK 506)-induced hemolytic uremic syndrome after heart transplantation

A case of deliberate ingestion of clothes washing liquid and fabric conditioner is described. Tracheal intubation and controlled ventilation became necessary because of respiratory muscle dysfunction associated with hypocalcaemia. We suggest that the phosphate component of the clothes washing liquid was responsible for the dramatic metabolic upset and subsequent need for admission to the Intensive Care Unit.