1/7. The haemolytic uraemic syndrome--a family study.This study included patients, all relatives with the haemolytic uraemic syndrome, and 18 family members. The diagnosis was uncertain in one other and definite in four patients. Three of these four comprised a father and two of his children. Data are presented to emphasize the widespread nature of the disease. Other than hypertension, predisposing factors, and red cell and HL-A genetic markers, although sought, have not been found. Management is discussed with special reference to the one surviving patient. Early bilateral nephrectomy may be life-saving.- - - - - - - - - - ranking = 1keywords = nature (Clic here for more details about this article) |
2/7. Severe hemolytic uremic syndrome associated with rhabdomyolysis and insulin-dependent diabetes mellitus.Hemolytic uremic syndrome has been associated with insulin-dependent diabetes mellitus eight times in the past, while the hemolytic uremic syndrome and rhabdomyolysis association has been described once. The occurrence of both conditions in a patient with the hemolytic uremic syndrome has not been reported. In this report, we described a 28-month-old girl who presented with severe hemolytic uremic syndrome muscle weakness and elevated muscle enzymes. Later, she developed hyperglycemia and ketosis requiring initiation of insulin therapy. The current literature was reviewed, and a hypothesis for the mechanism of injury and the multisystemic nature of this syndrome is presented. In patients with the hemolytic uremic syndrome, identification of potential extrarenal involvement is important as it may determine the final outcome of this disease.- - - - - - - - - - ranking = 1keywords = nature (Clic here for more details about this article) |
3/7. The histopathology of the hemolytic uremic syndrome associated with verocytotoxin-producing escherichia coli infections.Verocytotoxin-producing Escherichia coli (VTEC) infection was present in three cases of hemolytic uremic syndrome (HUS), two fatal and one non-fatal, in which detailed histopathologic investigations were conducted. Two patients had a prodrome of bloody diarrhea, one of whom required a hemicolectomy for severe bleeding. The renal histopathology was characterized primarily by glomerular thrombotic microangiopathy (TMA) with greater than 95% of glomeruli showing changes of capillary wall thickening, endothelial cell swelling, and narrowing or thrombosis of the capillary lumen. Preglomerular arterioles were frequently thrombosed, and abnormalities of the medium-sized vessels, including endothelial cell damage and thrombosis, were also commonly observed. Gastrointestinal involvement was prominent in all three cases. The colon was most severely involved, with marked mucosal and submucosal edema and hemorrhage, in the absence of significant inflammation or widespread ulceration. Microvascular angiopathy was present in all cases, with changes ranging from endothelial cell damage to overt thrombosis. Similar pathology was seen throughout the small bowel, including the presence of TMA. In one patient, typical morphologic changes of pseudomembranous enterocolitis were found in the absence of infection with clostridium difficile. The nature of vascular involvement in the kidneys and intestinal tract supports the hypothesis that HUS is mediated by systemic toxemia, and that endothelial cells are the primary target cells for the action of verocytotoxin.- - - - - - - - - - ranking = 1keywords = nature (Clic here for more details about this article) |
4/7. Homozygous protein c deficiency: observations on the nature of the molecular abnormality and the effectiveness of warfarin therapy.An infant with severe homozygous protein c deficiency was brought to medical attention because of purpura fulminans and severe bilateral vitreous hemorrhages in the neonatal period. Infusions of fresh frozen plasma were given for 8 months. On two occasions, attempts to decrease the frequency of fresh frozen plasma infusions to less than twice a day led to episodes of microangiopathic hemolysis, fibrinolysis, and acute renal failure. infarction of skin and subcutaneous tissues did not recur. Both episodes were controlled after reinstitution of fresh frozen plasma. Complications of therapy with fresh frozen plasma included hyperproteinemia and hypertension. warfarin therapy was instituted when the baby was 8 months of age, followed by a gradual withdrawal of fresh frozen plasma therapy. The dose of warfarin required to maintain the prothrombin time in a range of 1.8 to 2.2 times normal varied considerably during short periods, a phenomenon that may have been due to several factors: hypercatabolism of the drug with prolonged administration, abnormality of liver function, variation in levels of serum albumin, fluctuations in drug dosage secondary to oral administration, and variations in dietary vitamin k. Protein C determinations by immunologic and functional assays consistently showed detectable but reduced protein C antigen levels with undetectable activity levels, suggesting that a dysproteinemia rather than a deficiency of synthesis is responsible for the child's coagulopathy.- - - - - - - - - - ranking = 4keywords = nature (Clic here for more details about this article) |
5/7. cardiac tamponade as a terminal event in the hemolytic uremic syndrome in childhood.The case of a 6-year-old Inuit female with the epidemic form of hemolytic uremic syndrome (HUS) with myocardial involvement and probable cardiac tamponade is presented. This case illustrates the multisystemic nature of the syndrome, and to our knowledge, cardiac tamponade as a probable terminal event in HUS has not been reported previously.- - - - - - - - - - ranking = 1keywords = nature (Clic here for more details about this article) |
6/7. Malignant vascular disease of the kidney: nature of the lesions, mediators of disease progression, and the case for bilateral nephrectomy.A case of thrombotic microangiopathy presenting as a hemolytic uremic syndrome complicated by untreatable hypertension and ultimately requiring bilateral nephrectomy is discussed. Severe hypertension and renal failure may complicate the course of vascular diseases of the kidney, including thrombotic microangiopathy, chronic hypertension, and scleroderma. Toxins, pressure stress, and immune material may trigger the initial injury to vascular endothelium. The malignant course of these renal vascular diseases seems linked to the severity of vascular injury. Endothelial injury manifests with swelling and detachment of endothelial cells from the basement membrane, expansion of the subendothelial space, and newly formed basement membrane-like material. In arterioles, endothelial injury precedes myointimal swelling and proliferation, leading to vascular lumina narrowing or obliteration and secondary glomerular ischemia, with glomerular tuft collapse and garland-like wrinkling and thickening of the capillary wall. Endothelial cell injury is very likely the common determinant of a cascade of events that lead to irreversible renal failure. When the initial insult (toxins, mechanical stress, antibodies) is promptly removed, lesions are self-limiting and the patient usually recovers. However, a severe insult persisting for some time can lead to chronic and irreversible vascular lesions that, through renal ischemia, trigger maximal activation of the renin angiotensin system with a brisk elevation in arterial blood pressure that may combine to further vascular injury and renal ischemia. Moreover, enhanced shear stress in the severely narrowed microcirculation, through abnormal von willebrand factor processing, can also favor endothelial injury and platelet aggregation, which may further worsen the vascular lesions and sustain the microangiopathic process. Plasma manipulation, arteriolar vasodilators, and angiotensin-converting enzyme inhibitors normally control the vicious circle, but in few severe cases bilateral nephrectomy remains the last chance to save the patient's life.- - - - - - - - - - ranking = 4keywords = nature (Clic here for more details about this article) |
7/7. quinine-induced hemolytic uremic syndrome.Although quinine use is very common, hemolytic uremic syndrome (HUS) following exposure to quinine is only a recently reported phenomenon, with the first description published in 1991. Previous reports have concentrated on the nature of the hematological process and in particular characterization of the quinine-induced antibodies involved. We present a case of HUS with a clear temporal and immunological relationship to quinine which demonstrates the pathognomonic renal features of HUS. An indirect antiglobulin test with the patient's serum agglutinated red blood cells only in the presence of quinine. Renal biopsy features included glomerular and arteriolar endothelial swelling, capillary loop thrombi, mesangiolysis, segmental sclerosis and segmental ischemia. Early empiric treatment with plasma exchange and corticosteroids was instituted and this resulted in recovery of renal function to normal.- - - - - - - - - - ranking = 1keywords = nature (Clic here for more details about this article) |