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The hemolytic-uremic syndrome consists of microangiopathic hemolytic anemia, acute renal failure, and thrombocytopenia following a prodromal illness of gastroenteritis or upper respiratory infection. The syndrome can present in dramatic fashion with severe abdominal pain and signs of peritonitis suggesting an acute surgical crisis. In a series of 25 patients, 40% had abdominal pain, 25% had abdominal tenderness, and 20% had peritoneal signs. Clues to diagnosis in the early stages of the acute illness were mild to moderate hypertension, abnormal peripheral blood smear, anemia despite dehydration, and proteinuria. Significant abdominal pain and x-ray evidence of colitis may occur before development of typical laboratory findings, and these were evident in at least one case. Three patients underwent laparotomy for suspected bowel perforation. Colitis without perforation was found in all cases. In the absence of documented perforation, toxic megacolon, or intussusception, the decision to perform laparotomy in patients with hemolytic-uremic syndrome who have signs of peritonitis must be individualized. Failure to recognize the underlying renal problem can lead to serious errors in fluid and electrolyte management and delay of appropriate therapy.
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PMID:Hemolytic-uremic syndrome: a diagnostic and therapeutic dilemma for the surgeon. 73 58

The hemolytic-uremic syndrome (HUS) is an acute disorder, characterized by the triad of microangiopathic hemolytic anemia, nephropathy and thrombocytopenia. The great majority of patients are children, usually under 4 years of age, although adults can be affected. The onset is abrupt and usually follows gastroenteritis or upper respiratory infection. Later, clinical manifestations based on the triad, such as pallor, jaundice, edema, hypertension and purpura soon develop. The urinary output is reduced and the urine may appear dark yellow or tea-colored. Laboratory tests of peripheral blood show severe hemolytic anemia associated with fragmented red blood cells and thrombocytopenia, usually below 50,000/microliters. The blood urea nitrogen, serum creatinine and lactate dehydrogenase concentrations are elevated. Proteinuria and microscopic hematuria, which are indicative of active glomerular damage are also seen. Profound understanding of these manifestations is sufficient to permit an early diagnosis of HUS.
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PMID:[Diagnosis and clinical features of hemolytic uremic syndrome]. 843 21

We undertook a case-control study to evaluate the renal health of survivors of hemolytic-uremic syndrome (HUS) from the 1991 Arctic epidemic of Escherichia coli O157:H7 gastroenteritis 4 years after the epidemic. Eighteen children who developed HUS during the 1991 epidemic and 18 age- and sex-matched controls from the same community who had uncomplicated gastroenteritis were compared in 1995 for height, weight, blood pressure, urinalysis, and glomerular filtration rate (GFR), measured using continuous subcutaneous infusion of non-radioactive iothalamate. HUS survivors did not differ from controls in height, weight, systolic (HUS 118 mmHg, control 117 mmHg) or diastolic (HUS 64 mmHg, control 62 mmHg) blood pressures. Hematuria was detected more frequently in HUS survivors (11/18 vs. 4/18, P<0.05), but no child had proteinuria. Mean GFR did not differ between the two groups (HUS 159 ml/min per 1.73 m2, control 147 ml/min per 1.73 m2). Survivors of post-enteritic HUS from the 1991 Arctic E. coli 0157:H7 outbreak have excellent renal function 4 years after the epidemic.
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PMID:Renal function in Inuit survivors of epidemic hemolytic-uremic syndrome. 974 74

Every year in France, about 100 children, most of them less than 3 years old, have typical diarrhea-associated HUS (D + HUS). Evidence of exposure to verotoxin producing E. coli (VTEC), mostly the O157: H7 serotype, is demonstrated in about 85% of cases. A prodromal illness of acute gastroenteritis with diarrhea, often bloody, precedes the HUS by 1 to 15 days. HUS onset is sudden, with the typical association of hemolytic anemia with fragmented red blood cells, thrombocytopenia and acute renal insufficiency. Involvement of other organs than the kidneys may occur, such as severe hemorrhagic colitis with rectal prolapse, bowel wall necrosis or secondary stenosis, acute pancreatitis, central nervous system involvement which determines the vital outcome. Early accurate supportive treatment allows a current mortality rate below 5%, with most deaths due to central nervous system involvement. Five to 10% of children develop end stage renal disease, rarely directly, more often after having recovered some renal function with chronic renal insufficiency during a few years. After 15 or more years follow-up, at least one third of patients have some degree of proteinuria and/or hypertension, and eventually chronic or end stage renal failure. Predictive features of poor renal outcome at the acute phase are severe gastrointestinal involvement, severe CNS involvement, polyncleosis over 20,000/mm3, and duration of initial anuria longer than one week. The role of VTEC in D + HUS makes the disease a public health problem. Preventive measures are essential.
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PMID:[Post-diarrhea hemolytic-uremic syndrome: clinical aspects]. 1158 27

Amyloidosis is the most common and devastating complication of familial Mediterranean fever (FMF). Renal transplantation is the choice of treatment of in most end-stage renal disease (ESRD). We report our experience on the outcomes in eight patients who underwent renal transplantation for ESRD due to FMF secondary to amyloidosis, and we provide a discussion on the current evidence on this topic of study. The clinical charts of eight renal transplant patients (seven male, one female) who underwent ESRD due to FMF-related amyloidosis were investigated. Five patients underwent living-donor renal transplantation and three patients underwent deceased-donor renal transplant. The mean follow-up period was 35 months (range 3-72). All patients were on triple immunosuppressive treatment and received colchicine. All allografts are currently functioning well with a mean serum creatinine level of 1.4 (range 0.7-2.6) mg/dL. Posttransplantation complications included acute rejection (n = 4), chronic rejection (n = 1), severe gastroenteritis (n = 2), and erythrocytosis (n = 5). None of the patients had proteinuria. During follow-up, we did not observe clinically severe FMF attack, septicemia, rhabdomylosis, symptoms related to vasculitis, and clinical neuropathy. The clinical outcome of the patients in this cohort was similar to that of other renal transplant patients with ESRD due to other causes. This study shows favorable prognosis of eight ESRD patients due to amyloidosis caused by FMF after renal transplantation. Renal transplantation is a safe procedure for ESRD patients having amyloidosis due to FMF. Regular use of colchicine after transplantation should be mentioned.
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PMID:Renal transplantation in patients with familial Mediterranean fever. 2256 68

Antiphospholipid syndrome (APS) is a distinct autoimmune prothrombotic disorder due to pathogenic autoantibodies directed against proteins that bind to phospholipids. APS is characterized by arterial and venous thrombosis and their clinical sequelae. Catastrophic antiphospholipid syndrome (CAPS) is a rare and often fatal form of APS characterized by disseminated intravascular thrombosis and ischemic injury resulting in multiorgan failure. Rarely, intravascular thrombosis in CAPS is accompanied by hemorrhagic manifestations such as diffuse alveolar hemorrhage. Here, we report a 43-year-old woman who presented with anemia, acute gastroenteritis, abnormal liver function tests, bilateral pulmonary infiltrates, and a systemic inflammatory response syndrome. The patient developed respiratory failure as a result of diffuse alveolar hemorrhage followed by acute renal failure. Laboratory tests disclosed hematuria, proteinuria, and reduced platelet count. Microbiologic tests were negative. A renal biopsy demonstrated acute thrombotic microangiopathy and extensive interstitial hemorrhage. Serologic tests disclosed antinuclear antibodies and reduced serum complement C4 concentration. Coagulation studies revealed the lupus anticoagulant and autoantibodies against cardiolipin, beta 2-glycoprotein I, and prothrombin. High-dose glucocorticoids and plasma exchange resulted in rapid resolution of pulmonary, renal, and hematological manifestations. This rare case emphasizes that CAPS can present with concurrent thrombotic and hemorrhagic manifestations. Rapid diagnosis and treatment may result in complete recovery.
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PMID:Catastrophic antiphospholipid syndrome with concurrent thrombotic and hemorrhagic manifestations. 2372 30