Eltrombopag (thrombopoietin-receptor agonist) and plasmapheresis as rescue therapy of acute post-renal transplant immune thrombocytopenia in a child with Schimke immuno-osseous dysplasia-case report.

SIOD is rare disorder related to SMARCAL1 or SMARCAL2 gene mutation, including (among other comorbidities) T-cell immunodeficiency, nephrotic syndrome, and renal failure. Up to 22% of primary patients may develop various autoimmune disorders. We report the case of 11-year-old male with SIOD, who presented ITP at 2 years after renal transplantation with decrease in platelet count (from normal) to 56 000/µL and then (gradually) to 2000/µL. There was no effect of iv. methylprednisolone/dexamethasone. As the presence of antibodies against GPIIb/IIIa, GPIb, and GPIaIIa platelet glycoproteins was confirmed, patient was given 50 g of IVIG and then was put on plasmapheresis; however, both showed poor direct effect. As we were afraid to give rituximab (due to expected overimmunosuppression), we prescribed the oral TPO-receptor agonist (eltrombopag). Patient responded after 17 days of therapy, to the final dose of 50 mg/d (approx. 2 mg/kg). The antiplatelet antibodies disappeared after four plasmapheresis. Overall, the therapy was continued for 7 weeks and was stopped at platelet count of 433 000/µL. Platelet count remained stable in 8-month follow-up. Combination of plasmapheresis and TPO-receptor agonist was effective in post-renal transplant acute ITP in patient with SIOD.

Successes and pitfalls of chronic peritoneal dialysis in infants - a Polish nationwide outcome study.

INTRODUCTION: Peritoneal dialysis (PD) is a preferred method of renal replacement therapy for end-stage renal disease in children. Recent advances have allowed chronic PD to be provided to children of all ages and sizes. MATERIAL AND METHODS: The study was designed as a national (10 dialysis centres), multicentre retrospective analysis of the medical history of 33 children who started chronic peritoneal dialysis in their infancy between 1993 and 2005, with a follow-up period of at least 24 months. RESULTS: The nutritional status of the infants was unsatisfactory. The mean SDS of body weight at the start was -2.0, at 1 year of age -1.7. Only 40% of infants were adequately nourished at 1 year of age. Long-term follow-up analysis showed that 12 children received a kidney transplant, 13 were still on dialysis (4 changed method) and 6 died (mortality rate in the first year of life of 9%). In 2 children we observed an improvement of renal function. We observed a relatively high (1/8.8 patient-months) peritonitis rate in the analysed children when compared to 1 : 22 patient-months in all children undergoing PD in Poland. CONCLUSIONS: The results of our survey have shown that the management of dialysed infants is still a challenge for the medical team and families, but long-term results of the therapy are encouraging.

[Chronic peritoneal dialysis in infants--preliminary results of the multicenter survey]. / Przewlekla dializa otrzewnowa u niemowlat--wstepna analiza ankiety wieloosrodkowej.

We retrospectively analysed peritoneal dialysis treatment in 29 infants dialysed in 9 paediatric centres in Poland in the years 1993-2004. The mean age at the start of dialysis was 4.9 +/- 3.5 months (range 2 days to 11 months), mean body mass 5.6 +/- 2.5 kg (range 2.5 to 11 kg). The mean duration of PD was 6.8 +/- 3.9 in the first year of life and total duration of the therapy 34 +/- 27 months. Of the 29 infants 4 died (2 in infancy), 11 underwent renal transplantation, in 2 children PD was stopped (they received a conventional treatment) and 12 were still dialysed at the date of data collection. The peritonitis rate was 1/9.5 patient-month and exit site infection rate 1/16 patient-month up to 1 year of life. 9 children (31%) required hernia repairs and in 9 catheters were replaced. Chronic peritoneal dialysis in infants is associated with high risk of infections and surgical complications and remains a challenge for paediatric nephrologists.

Experience using presternal catheter for peritoneal dialysis in Poland: a multicenter pediatric survey.

OBJECTIVES: Permanent and adequate access to the peritoneal cavity is the key to successful chronic peritoneal dialysis (PD). A variety of catheter designs and implantation techniques have been developed to achieve optimal peritoneal access. One such new and modified PD catheter is the presternal catheter [swan neck presternal catheter (SNPC)], with the exit site located on the chest wall. DESIGN: A multicenter survey was undertaken to summarize 10 years of experience with the presternal catheter in children in Poland. SETTING: Four pediatric institutions using the SNPC in children: (1) Medical University of Warsaw, Warsaw; (2) Children's Memorial Health Institute, Warsaw; (3) District Children's Hospital, Szczecin; (4) University of Medical Sciences, Poznan. PATIENTS: During the past 10 years, 20 presternal catheters were implanted in 19 children, aged 0.2-17.7 years (mean 8 +/- 5.8 years), with end-stage renal failure.The main indications for the SNPC include urinary diversion (ureterocutaneostomy or vesicostomy), use of diapers, young age, obesity, abdominal wall weakness, and recurrent exit-site infections (ESI) with previous abdominal PD catheters. INTERVENTION: In all children the presternal catheter was implanted surgically under general anesthesia by one surgeon. Uniform operative technique and uniform perioperative management were used. RESULTS: The mean observation time for the 20 presternal catheters was 24.8 +/- 25 months (range 1-83 months). The ESI rate was 1/70.9 patient-months (0.17 episodes per year), tunnel infection rate was 1/248 patient-months (0.05 episodes per year), and the overall peritonitis rate was 1/26.6 patient-months (0.51 episodes per year). Non-infectious complications associated with the SNPC included disconnection of both sections (2 children) and trauma to the exit site located on the chest wall (4 children). Mean survival time of the presternal catheter, as calculated by the Kaplan-Meier method, was 57.5 +/- 8.5 months; 50% catheter survival reached 72 months. CONCLUSIONS: The good outcome in patients with a SNPC validates the rationale for the presternal catheter design and should encourage its more widespread use. The SNPC seems to be suitable for any patient on PD; however, this catheter is particularly useful in patients with specific indications (ie., higher tendency to ESI). The SNPC allows safe and long-term chronic PD in very young children using diapers and in patients with urinary diversion.

[Clinical aspects of plasmapheresis therapy in children: single center experience]. / Kliniczne aspekty leczenia dzieci plazmafereza--doswiadczenie jednego osrodka.

Data concerning 576 plasmapheresis sessions (indications, anticoagulation applied, supplement type and acute complications) in children (mean body weight = 35 kg +/- 15; min 5 kg, max 75 kg) performed between 1990 and 2001 were analysed. INDICATIONS: Glomerulonephritis (GN)--185 (32%) (including recurrence after kidney transplantation--108, rapidly progressive GN--63, other GN--14), other immunological diseases--110 (19%) (systemic lupus erythematosus, Wegener's granulomatosis, myasthenia, Guillain-Barré syndrome, other), haemolytic-uraemic syndrome--104 (18%) (after kidney transplantation--50, atypical--54), Amanita poisoning 100 (17%), acute hepatic encephalopathy--41 (7%) (after liver transplantation--9), poisoning with drugs bound by plasma albumin--22 (4%) and complications in kidney graft recipients--14 (2%) (acute vascular rejection, parathormone toxicity). ANTICOAGULATION: Until the end of 1999--unfractionated heparin (in divided doses every 30 min--100 IU/kg/session on the average), from 2000 on--single dose of Fraxiparine (mean 70 IU anty-Xa/kg/session). SUPPLEMENT: Until 1995--Ringer solution + fresh frozen plasma, from 1996 on--Ringer solution + fresh frozen plasma or 4% albumin solution diluted in 5% dextrose (depending on indications, contraindications and fibrinogen concentration). COMPLICATIONS: Deaths in the course of plasmapheresis--0, others (rash, abdominal pain, headache, nausea and vomiting, blepharoedema, drop of arterial pressure, pruritus)--19 (3%), necessity of session termination because of patient condition (dyspnoea, urticaria, hypotension)--3 (0.5%) and technical problems--23 (4.5%) (filter damage--8, filter clotting--5, haemolysis--3, machine malfunction--5, problems with vascular access--2).