Successful living-related renal transplantation in a patient with factor H antibody-associated atypical hemolytic uremic syndrome.

CFH-Ab-associated aHUS requires different diagnostic and therapeutic approaches and then the genetically defined aHUS forms. The risk of post-transplant recurrence with graft dysfunction in CFH-Ab aHUS is not well documented. It is suggested that recurrence can be expected if a significant CFH-Ab load persists at the time of transplantation. A pretransplant procedure to reduce CFH-Ab titer seems reasonable, but accurate recommendations are lacking. Whether further prophylactic interventions after transplantation are necessary has to be decided on an individual basis. We report the case of a late diagnosed CFH-Ab HUS with initial ESRD and a successful living-related renal transplantation over a post-transplant period of four and a half years on the basis of a prophylactic pretransplant IVIG admission.

Long-term follow-up of children with typical hemolytic uremic syndrome.

OBJECTIVE: The aim of the study was to determine the associations of the acute period course with late-emerging sequelae in children with typical hemolytic uremic syndrome (HUS). MATERIALS AND METHODS: The data of 62 children with typical HUS during the acute phase were retrospectively analyzed by age, sex, duration of anuria/oliguria, method and duration of renal replacement therapy, proteinuria, hypertension, and renal function. The data of 33 children at 10-year follow-up after the onset of the disease were evaluated for changes in hypertension, proteinuria, and renal function. RESULTS: In the acute phase of the disease (n=62), hypertension was documented in 75.8% of the children; proteinuria, in 85.5%; and renal dysfunction, in 100%. At 10 years after the onset of the disease (n=33), hypertension was documented in 12.1%, 6.1%, and 24.2% at 1-, 5-, and ≥10-year follow-ups, respectively, and more often in children aged <1 year at the onset of the disease. Proteinuria was found in 15.2%, 9.1%, and 33.3% of the patients, respectively. After ≥10 years, hypertension developed for the first time in 6.1% of the patients. Renal injury of varying degrees was seen in 15.2% of the children at the 1-year follow-up, and after ≥10 years the proportion increased to 33.3%. CONCLUSIONS: At 10 years after the acute phase of typical HUS in children, the prevalence of hypertension and proteinuria at 1- and 5-year follow-ups decreased, but after 10 years it started to increase. As much as 6.1% of the children developed hypertension or proteinuria for the first time at 10 years. Hypertension was documented more frequently in children who were younger than <1 year at the onset of the disease. Renal dysfunction after 5 and 10 years remained in more than one-third of cases, and it was observed more often if hypertension was documented at the acute period.

[The clinical course and long-term outcome of hemolytic-uremic syndrome in children]. / Vaiku hemolizinio ureminio sindromo klinikine eiga ir velyvosios baigtys.

The aim of this study was to evaluate the long-term outcome of hemolytic-uremic syndrome in children and the dependence of outcome on severity of the acute phase of the illness. We analyzed data of 20 children who were hospitalized and treated at the Clinic of Children's Diseases, Kaunas University of Medicine Hospital, during 1995-2006. Data were obtained from case histories and outpatient case records with the help of prepared questionnaire. The course of acute disease and health status were evaluated at discharge from hospital and at 6-month, 1-year, and 3-year follow-ups. There were 8 boys and 12 girls in the study group; their age ranged from 3 months to 12 years. According to the clinical course of the acute phase of the illness, children were divided into two groups. Group A (severe course) consisted of 15 patients with blood leucocytosis (more than 20x10(9)/L) and signs of CNS involvement, who required renal replacement therapy. Group B (mild course) consisted of five children who did not have such symptoms. Twelve (60%) children underwent dialysis during acute illness; two patients died (10%). One (20%) patient in Group B had proteinuria, four (80%) had renal insufficiency, and three (60%) had arterial hypertension at discharge from hospital. Subsequently these changes disappeared, and 3 years later arterial hypertension was detected in 1 (25%) patient in Group B. Eight (61.5%) patients from Group A had renal insufficiency, nine (69.2%) had proteinuria, and two (15.4 %) had arterial hypertension at discharge from hospital. Three years later from the onset of the disease, two (20%) patients had arterial hypertension, proteinuria was detected in two (20%) patients, and renal insufficiency remained in six (60%) children. Our data revealed that the outcomes of the disease are strongly influenced by the severity of the acute phase of the illness.