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.

Nationwide renal biopsy data in Lithuania 1994-2012.

BACKGROUND: Our aim was to evaluate the incidence of biopsy-proven kidney diseases in Lithuania and to compare their changes in three different time intervals. All Lithuanian kidney biopsies were performed in the National Center of Pathology, enabling analysis at the national level. METHODS: The native kidney biopsy data were reviewed, and incidence of renal disorders and patient demographics were compared during three time intervals: 1994-1999, 2000-2006, and 2007-2012. RESULTS: A total of 5,368 kidney biopsies were performed, including 3,640 native kidney and 1,728 kidney transplant biopsies; 59.5% (2,165) of the native kidney biopsies were classified as primary glomerulopathies. The most common entity was IgA nephropathy (737; 34.0%), followed by focal segmental glomerulosclerosis (285; 13.2%) and membranoproliferative glomerulonephritis (256; 11.8%). Prominent decrease in incidence of membranoproliferative glomerulonephritis (16.8 to 8.7% from the first to third time interval) and increase in (mainly, pauci-immune) crescentic glomerulonephritis (6.2 to 15.3%) were noted over the study period. In a subgroup of 427 pediatric native kidney biopsies, IgAN accounted for 24.9% of biopsies. The incidence of MCNS increased dramatically from the first to third time interval (6.3 to 25.4%), while the number of MPGN increased in the second time interval (from 7.2 to 8.9%) but decreased in the third one (to 4.4%). CONCLUSIONS: Decrease in relative incidence of membranoproliferative glomerulonephritis, most likely, reflects improvement in socioeconomic conditions, while relative increase in crescentic glomerulonephritis is interpreted as improved diagnostics of the disease.

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.

Prevalence and predictors of the sub-target Hb level in children on dialysis.

BACKGROUND: Anaemia is a common and potentially treatable co-morbidity of end-stage renal disease. We aimed to determine the prevalence of the sub-target haemoglobin (Hb) level among European children on dialysis and to identify factors associated with a low Hb level. METHODS: From the European Society for Paediatric Nephrology (ESPN)/European Renal Association-European Dialysis Transplant Association (ERA-EDTA) registry, data were available on 2351 children between 1 month and 18 years of age, totalling 5546 measurements from 19 countries. RESULTS: The mean Hb level was 10.8 g/dL (5th-95th percentiles, 7.4-13.9). Among those above 2 years of age, the mean Hb level was 10.9 g/dL (11.4% below 8.5 g/dL), while it was 10.3 g/dL among those below 2 years (11.2% below 8.0 g/dL). A total of 91.2% of the patients were on an erythropoiesis-stimulating agent (ESA). Hb levels increased with age and were higher in peritoneal dialysis compared with haemodialysis patients. Patients with congenital anomalies of the kidney and urinary tract showed the highest Hb levels, and those with cystic kidney diseases or metabolic disorders the lowest ones. Ferritin levels between 25 and 50 ng/mL were associated with the highest Hb levels. We found a weak inverse association between parathyroid hormone (PTH) and Hb. Whereas standardized blood pressure (BP) was not elevated in patients with above-target Hb, elevated systolic BP z-score was noted in those with sub-target Hb levels. CONCLUSIONS: Sub-target Hb levels remain common in children on dialysis, in spite of virtually all children being treated with ESA; although we cannot exclude under-dosing. Optimal ferritin levels seemed to be slightly lower in children (25-50 ng/mL) than those in adults. Other risk factors for sub-target Hb are dialysis modality and a high PTH level.

Acute kidney injury in pediatric patients: experience of a single center during an 11-year period.

UNLABELLED: The aim of our study was to determine the causes of acute kidney injury (AKI) in children, to compare outcomes between two periods--1998-2003 and 2004-2008--and to evaluate the influence of new methods of renal replacement therapy on mortality. MATERIAL AND METHODS: A retrospective analysis of medical record data of all children treated for AKI at the Clinic of Children Diseases, Hospital of Kaunas University of Medicine, during the period of 1998-2008 was made. Both periods were compared regarding various variables. RESULTS: Of the 179 children with AKI, 75 (41.9%) were treated during 1998-2003 and 104 (58.1%) during 2004-2008. Primary glomerular disease and sepsis were the leading causes of AKI in both the periods. AKI without involvement of other organs was diagnosed for 106 (59.2%) children: for 42 (56.0%) children in the first period and 64 (61.5%) in the second. A total of 124 (69.3%) children were treated in a pediatric intensive care unit. Multiple organ dysfunction syndrome with AKI was diagnosed for 33 (44%) patients in the first period and for 40 (38.5%) in the second. A significant decrease in mortality among patients with multiple organ dysfunction syndrome during the second period was observed (78.8% vs. 37.5%). CONCLUSIONS: More than half of patients had secondary acute kidney injury of nonrenal origin. More than two-thirds (69.3%) of patients with AKI were treated in the pediatric intensive care unit. Multiple organ dysfunction syndrome was diagnosed for 40.8% of children with AKI. Renal replacement therapy was indicated for one-third of patients with AKI. A 2.5-fold decrease in mortality was observed in the second period as compared to the first one.

Cisplatin increases urinary sodium excretion in rats: gender-related differences.

OBJECTIVE. There are well-documented reports of cisplatin-associated hyponatremia in the literature, but there are no data on gender-dependent differences. The aim of the present study was to define characteristics of 24-hour urinary sodium excretion in young adult Wistar rats of both genders and to evaluate the gender-related effect of cisplatin. MATERIALS AND METHODS. Twelve control Wistar rats (6 males and 6 females) and 12 cisplatin-treated Wistar rats (6 males and 6 females) after a single and repeated injection of cisplatin (once a day for 3 days) at a dose of 2.5 mg/kg body weight into the caudal vein were examined. The experiment was carried out by measuring 24-h urinary sodium, potassium, chloride, magnesium, creatinine excretion and pH in the urine of age-matched male and female rats. RESULTS. The 24-h urinary sodium excretion, sodium/chloride ratio, and diuresis showed no gender-related differences in control rats. After a single administration of 2.5 mg/kg cisplatin, 24-h urinary sodium excretion was not significantly higher in cisplatin-treated rats than in gender-matched controls. After repeated cisplatin administration, 24-h urinary sodium excretion was significantly higher in cisplatin-treated male rats as compared to matched controls (P<0.05). No such effect was found in cisplatin-treated female rats. CONCLUSION. The study data show that cisplatin enhances urinary sodium excretion in male but not in female rats. The mechanism of such a gender-related effect is not yet clear. Further investigations are necessary to elucidate the mechanism of this pharmacological effect of cisplatin.

Recurrent urinary tract infection in girls: do urodynamic, behavioral and functional abnormalities play a role?

BACKGROUND: To determine urodynamic, behavioral and functional abnormalities predisposing to recurrent urinary tract infection in 5- to 17-year-old girls. METHODS: A prospective case-control study was carried out. A total of 148 girls met inclusion criteria. They received a careful evaluation including complete history, voiding-drinking diary, bowel questionnaire, physical investigation, sonography, voiding cystourethrogram and urodynamic investigation. RESULTS: In a multivariate model, independent risk factors for recurrent urinary tract infection included age 20 mL (OR=1.1; 95% CI, 1.0-1.1). CONCLUSIONS: Independent risk factors for recurrent urinary tract infection were age

Characteristics of gender-related circadian arterial blood pressure in healthy adolescents.

SUMMARY OBJECTIVE: To define 24-h characteristics of arterial blood pressure in healthy adolescent girls and boys; to determine gender-related differences of blood pressure, its circadian pattern. MATERIAL AND METHODS: The 24-h blood pressure was monitored hourly in healthy girls (n=22, without no account for the menstrual cycle phase) and boys (n=22). Additionally, blood pressure of adolescent girls (n=15) was examined during different phases of their menstrual cycle (follicular, ovulation, and luteal). Blood pressure was monitored with an auto-cuff automatic outpatient blood pressure monitor. RESULTS: Investigation showed gender-related differences in 24-h blood pressure. Study results revealed the circadian blood pressure rhythm characterized by a period of low values during nighttime and an early morning increase in both adolescent groups. Nocturnal systolic blood pressure was higher (P<0.05) in boys than in girls in all phases of their menstrual cycle. Diurnal systolic blood pressure in boys was higher than in girls in their follicular phase (P<0.05). The day and night blood pressure differed between boys and girls (P<0.05). A dipping blood pressure pattern as a decrease in mean nighttime blood pressure as compared with mean daytime blood pressure was defined: 10.02+/-6.7% in girls (n=22) and 13+/-6.3% in boys (n=22), without gender-related differences (P>0.05). There were no differences in blood pressure dipping among girls' groups in different menstrual cycle phases (P>0.05). Adolescent boys showed a significant positive correlation between their mean diurnal blood pressure and height (P<0.05). CONCLUSION: The study proved gender-related arterial blood pressure differences in healthy adolescents. The results demonstrate the gender-specific circadian blood pressure rhythm pattern in both gender groups.

[Etiology, prevalence of chronic renal failure and growth retardation in Lithuanian children]. / Lietuvos vaiku letinio inkstu nepakankamumo paplitimas, priezastys ir augimo atsilikimas.

The aim of the study was to evaluate the causes, prevalence, and grades of chronic renal failure in Lithuanian children and to assess its influence on children's growth. The study was performed in Vilnius University Children's Hospital and Clinic of Children's Diseases, Kaunas University of Medicine. By March 31, 2006, 65 children with chronic renal failure had been registered. The prevalence was 88.3 cases per million children. The mean age was 10.8+/-4.9 years. The leading causes of chronic renal failure were congenital diseases (37%) and obstruction with interstitial nephritis (33.8%). At presentation, 23 (35.40%) children had mild, 17 (26.15%) had moderate, 9 (23.85%) had severe renal failure, and 16 (24.6%) had end-stage renal disease. Fourteen (21.54%) children were below the third percentile in height for their age. Growth failure was observed in one (4.35%) child with Grade 1 renal failure. The deterioration of renal function had a significant influence on growth impairment, and 13 (30.95%) children with glomerular filtration rate of less than 60 mL/min/1.73 m2 and 7 (43.75%) with end-stage renal disease had a height below the third percentile. Growth retardation as one of the symptoms of impairment of children's physical development depends on the severity of renal function.

[Postinfectious glomerulonephritis in children in Lithuania during 1995-2004: prevalence and clinical features]. / Vaiku poinfekcinis glomerulonefritas Lietuvoje 1995-2004 m.: sergamumas ir klinikiniai pozymiai.

During the period of 1995-2004, acute postinfectious glomerulonephritis was diagnosed in 322 children aged 1-16 years. An outbreak of acute postinfectious glomerulonephritis was registered in 1995, with a prevalence of 8.3 cases per 100,000 children; during 2003-2004 prevalence decreased and it was 2.2-2.4 cases per 100,000 children. Acute postinfectious glomerulonephritis was most frequently caused by upper respiratory tract infection (28.3%), following by tonsillitis (24%) and skin infection (24%). The mean interval between diagnosis of primary infection and onset of acute postinfectious glomerulonephritis was 14 days and did not differ by infection. The disease was more common in autumn (96 of the 322 cases, P<0.05). There were 4.3% of familial cases; no relapses occurred. Edema was observed in 70.3% of patients; 55.5% had elevated blood pressure according to their age. There were no proteinuria and hematuria in 17.6% and 1.7% of patients, respectively. Short-term renal failure was diagnosed in 32.9% of patients; in two cases, acute renal failure was severe and hemodialysis was started. No patient died.

[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.

[Hemolytic-uremic syndrome in Lithuania over the period 1990-2004]. / Vaiku sergamumas hemoliziniu-ureminiu sindromu Lietuvoje (1990-2004).

More than 80 cases of the hemolytic-uremic syndrome were registered in Lithuania over the period 1990-2004. The incidence of this syndrome fluctuated annually; higher incidence rates were observed in 1990-1993, 1998-1999 and 2003-2004, however, there was no overall increase in morbidity over the 15-year period. Most cases occurred in the first two years of life; besides, girls suffered slightly more often than boys. At least in 80% of cases the illness was typical, i.e. was associated with diarrhea. In general, the data correspond to the findings of other scientists.

[Etiology and outcomes of acute renal failure in childhood]. / Vaiku uminio inkstu nepakankamumo priezastys ir baigtys.

The aim of the research was to determine causes of acute renal failure in children, their outcome and to define risk factors associated with mortality. 75 children with acute renal failure, who were treated at the Clinic of Children's Diseases of Kaunas University of Medicine between 1998-2003 years, were included in the study. The age range of patients was 1 month to 16 years. They were divided into two groups. Acute renal failure was diagnosed in 42 (56%) patients (the first study group) and in 33 (44%) patients acute renal failure was together with multiple organ failure (the second study group). In the first study group 69% of cases of acute renal failure were found to be due to renal diseases and in the second study group 97% were because of extrarenal diseases. Sepsis was the most frequent cause of acute renal failure in the second group (p<0.02). Dialysis was made for 28% patients. Hypertension was diagnosed more often in the first patients group (p<0.05). Hypertension persisted in 9 (36%) patients after recovery. Chronic renal failure developed in two patients. 28 (37.3%) patients of the original study group died. Mortality rate for children with multiple organ failure was higher than for the children, who had renal insufficiency only (78.8% vs 4.8%; p<0.001). Mortality rate of infants in the first study group was higher than for children of the same age in the second group (p<0.001). Mortality rate for children, who had oliguria or anuria, was higher in the second group, too (p<0.001).

[Dysfunction of lower urinary tract in 5-18 years old children with recurrent urinary tract infections]. / Apatiniu slapimo organu funkcijos pokyciai 5-18 metu vaikams, sergantiems pasikartojancia slapimo organu infekcija.

The purpose of this article was to evaluate function of lower urinary tract for 5-18 years old children with recurrent urinary tract infections and to compare urodynamic changes in two groups: with recurrent lower urinary tract infections and with recurrent upper urinary tract infections. 35 urodynamic studies in 5-18 years old children (3 boys and 32 girls) with recurrent urinary tract infection were performed at the Clinic of Children's Diseases of Kaunas University of Medicine in 2004. 21 of these children had recurrent lower urinary tract infections and 14 recurrent upper urinary tract infections. Changes in urodynamics were present in 91.4% of children: in all children with recurrent lower urinary tract infections and in 11 children with upper recurrent urinary tract infections (78.6%). Detrusor instability was found in 20 (57.1%) children: in 14 (66,7%) with recurrent lower urinary tract infections and in 6 (42.9%) with recurrent upper urinary tract infections. In 32 (85%) children detrusor instability was accompanied by changes in bladder volume. Changes of bladder volume were present for 26 (74.3%) children with recurrent urinary tract infections. Detrusor after contraction was diagnosed in 57.1% of children with recurrent lower urinary tract infections and in 28.6% with recurrent upper urinary tract infections. For children with recurrent urinary tract infections attention for urinary tract dysfunction must be paid.

Denys-Drash syndrome.

Constitutional missense mutations in the WT1 gene are usually associated with Denys-Drash syndrome. This rare syndrome is characterized by a rapid progressive nephropathy, male pseudohermaphroditism, and an increased risk for Wilms tumor. We report on a patient with incomplete Denys-Drash syndrome, which was evident by the clinical data and proved by molecular genetics methods. The patient has the mutation p.R394W in the WT1 gene and clinical symptoms of Denys-Drash syndrome.

[Changes of etiology and management of childhood urinary tract infection during the period of 1994-2002 (data of Kaunas University of Medicine Hospital)]. / Vaiku slapimo organu infekcijos etiologiniu faktoriu kitimas ir diagnostikos bei gydymo taktikos pokyciai (Kauno medicinos universiteto kliniku 1994-2002 m. duomenys).

The purpose of this article was to establish changes of age, etiology, diagnostics and treatment for children, who were treated for urinary tract infection in Clinic of Children Diseases, Kaunas University of Medicine Hospital in 1994-1998 and 1999-2002. Seven hundred fifty children urinary tract infections were treated during the period of 1994-1998, and 673--during the period of 1999-2002. Female patients had urinary tract infection nearly five times more than male patients in other age groups. Rate of urinary tract infection in male patients under one year age was greater than in female in this age group (p=0.0001). Greater incidence of urinary tract infection in age group under one year age was established. The incidence among male patients with urinary tract infection grew from 37.8% to 64.0% (p=0.0001) and among female patients--from 9.6% to 18.1% (p<0.0001). More vesicoureteral refluxes (VUR) were diagnosed: 44 (7.8%) in period 1994-1998 and 52 (9.0%) in the period 1999-2002. More than one third of all VUR (36.5%) were diagnosed in age group under one year. The incidence of VUR in this group was greater for male than for female patients (p=0.015). More positive urinary culture for patients with pyelonephritis were in period 1999-2002 (74.6%) than in 1994-1998 (66.8%). The incidence of microorganisms was nearly the same. E. coli was found in 83.4% and in 83.9% of all positive urinary cultures. Sensitivity to the commonly used antibiotics was better (p<0.0001). Pyelonephritis mostly was treated with gentamycin.

[The value and adaptability of glomerular filtration rate and reserve test (review of the literature)]. / Glomerulu filtracijos greicio ir rezervo tyrimu verte bei pritaikymas (literaturos apzvalga).

In normal conditions only 75% of kidney function capability are in use. Resting part of kidney function is called renal functional reserve. It is a very important indicator for prediction of renal insufficiency in chronic renal diseases, morphological changes or reduced mass of kidney parenchyma. More common test to estimate renal functional reserve is a method of measurement of glomerular filtration rate (clearance) before and after provocation of renal filtration by acute peroral protein load. The value and the possibilities to put in practice this test as well the factors influencing the results of glomerular filtration rate were estimated in this review.