Mechanism of cystogenesis in nephrotic kidneys: a histopathological study.

Background: Nephrotic syndrome (NS) is pathological condition characterized by heavy proteinuria. Our study investigates hypothesis that change in cell proliferation of proximal tubules influences primary cilia structure and function and promotes cystogenesis in congenital nephrotic syndrome of the Finnish type (CNF) and focal segmental glomerulosclerosis (FSGS).

Methods: CNF kidneys were analyzed genetically.

Impact of graft loss among kidney diseases with a high risk of post-transplant recurrence in the paediatric population.

Background: Some kidney diseases tend to recur in the renal allograft after transplantation. We studied the risk of graft loss among primary renal diseases known for their high risk of recurrence and compared it with that of patients with hypoplasia and/or dysplasia.

Methods: Within the European Society of Paediatric Nephrology and European Renal Association and European Dialysis and Transplant Association (ESPN/ERA-EDTA) registry, we studied children from 33 countries who received a kidney transplant before the age of 20 between 1990 and 2009.

Treatment of steroid unresponsive relapse with plasma exchange in aggressive multiple sclerosis.

The options for treating steroid unresponsive relapses in relapsing remitting multiple sclerosis (RRMS) are modest. We present a small series of patients with an aggressive course of RRMS whose steroid unresponsive relapses were treated with plasma exchange. In the period from January 2007 until February 2009 we identified four patients with steroid unresponsive relapses.

[Characteristics of anaemia treatment in children with chronic kidney disease].

In children with chronic kidney disease anaemia is seen very often. Depending of etiology and the degree of renal insufficiency is found up to 80%. The cause is mainly because of lowered production of erythropoietin in chronically damaged kidney, but also because of insufficient intake of iron and folic acid, or intake of some drugs.

The life-threatening hemodialysis catheter heparin lock caused bleeding in a child after peritoneal catheter removal.

Hemodialysis catheter patency is regularly maintained by high-concentration heparin filling, according to manufacturer's recommendation. Surprisingly, there are only few reports on serious bleeding complications in children on dialysis. A case of serious, life-threatening hemorrhage in a child after tunneled peritoneal catheter removal because of hemodialysis catheter heparin lock flushing is described.

[Clinical and histopathological characteristics of biopsy-proven renal diseases in Croatia].

There is little data on the spectrum of renal diseases in children in Croatia. The Croatian Society for Pediatric Nephrology has established the Registry of Biopsy-Proven Renal Diseases in an attempt to address this issue nationwide. Here we report preliminary results of a retrospective analysis of clinical and histopathological data of 565 children aged < or =17 years presenting to 9 hospitals in Croatia from 1991 to 2004, in whom kidney biopsy was performed.

[Three-year-old boy--a homozygote for familiar hypercholesterolemia].

Homozygous familial hypercholesterolemia (FH) is a rare autosomal dominant disorder caused by mutations in the low-density-lipoprotein (LDL) receptor gene. It occurs with a frequency of approximately 1 per million persons world-wide. Clinically, homozygous FH is associated with extremely elevated levels of LDL cholesterol and cutaneous xanthomas that develop in early childhood.

Complications of therapeutic plasma exchange: experience with 4857 treatments.

Plasma exchange (PE) is a technique of extracorporeal blood purification which removes large molecular weight substances from plasma. The Department of Dialysis, Zagreb University Hospital Center's database, which includes data on 509 patients, or 4857 PE treatments, was retrospectively analyzed to test the safety of PE. A total of 231 adverse reactions were recorded (4.

[Primary cytomegalovirus infection after combined cadaveric transplantation of the liver and kidney].

Seronegative transplant recipients are at a high risk of developing primary cytomegalovirus (CMV) infection. The D+/R--constellation produces a 60%-80% probability of CMV disease. In such cases CMV prophylaxis is justified.

[Long-term monitoring of children with focal segmental glomerulosclerosis and a transplanted kidney].

Introduction: Focal segmental glomerulosclerosis (FSGS) is an important cause of nephrotic syndrome and end-stage renal disease. Children with FSGS are at a risk of recurrence of disease following renal transplantation. The rate of recurrence has been estimated to range from 15% to 50%, having a potentially detrimental course towards the loss of renal function.