Survival and causes of death in children dialyzed in a single center were analyzed. During the last 12 years a chronic dialysis program was introduced in 146 children in our center and 125 of them, eligible for observation, were included in this analysis; 58 patients were on hemodialysis (HD) and 67 on peritoneal dialysis [continuous ambulatory peritoneal dialysis/automated peritoneal dialysis (CAPD/APD)]. Mean age at the start of dialysis was 13.1 years in HD and 9.8 years in CAPD/APD patients. Overall, 16 patients died (12.5%); 6 (10.3%) on HD and 10 (14.9%) on CAPD/APD; 4 HD patients died of hemorrhagic stroke and 2 were killed in road traffic accidents. Of 10 CAPD/APD patients, 7 died of heart failure, ischemic stroke, and/or disseminated thromboembolic disease. Another was killed in a road traffic accident and 2 died during the course of severe infections. The 1-year patient survival rate was 96.6% in HD patients and 95% in CAPD/APD patients, 2-year survival 94% and 93% and 5-year survival 91% and 78%, respectively (P=0.2, NS). In conclusion, the survival rate for HD and CAPD patients is similar, although after 2 years of therapy, it is lower in CAPD patients. The main causes of death are cardiovascular. However, in CAPD/APD patients, heart failure with low cardiac output and thromboembolic complications are major causes of death, and in HD patients the main cause is hemorrhagic stroke.
FLF is a life-threatening disease. Hepatic coma exerts dramatic impact on patient survival. At present, LTx is the treatment modality of choice that provides significant improvement in outcome of most patients with FLF. Multiple attempts have been made to reduce mortality and improve the patient's condition. One of the new options is AD - MARS. We present the case of a 11-yr-old boy with FLF and hepatic coma who avoided the scheduled LTx because of rapid neurological and biochemical improvement immediately after three MARS sessions.
A total of 192 children and adolescents undergoing renal transplantation were randomly chosen to receive tacrolimus, azathioprine and corticosteroids (TAS, n = 93) or tacrolimus, azathioprine, corticosteroids and two doses of basiliximab (TAS + B, n = 99). Six-month outcome data have previously been reported; this manuscript reports the 2-year data. Complete 2-year data were available on 164 (85.4%) of the original 192 patients. There was a single death in the TAS arm. Kaplan-Meier estimates of survival free of graft loss at 2 years were 94.9% in the TAS + B arm and 89.6% in the TAS arm [hazard ratio (HR) 0.52; 95% confidence interval (CI) 0.17 to 1.54, P = 0.23]. Estimates of survival free from rejection at 2 years were 75.2% in the TAS + B arm and 68.7% in the TAS arm (HR 0.81; 95% CI 0.46 to 1.40, P = 0.44). The mean estimated glomerular filtration rate (GFR) at 2 years, was 65.8 ml/min per 1.73 m(2) body surface area in the TAS arm and 66.7 ml/min per 1.73 m(2) in the TAS + B arm (P = 0.78). Blood pressure and cholesterol levels were similar in the two arms, and there was no evidence of a difference in the incidence of infection or malignancy. These data provide further evidence of a lack of benefit associated with the addition of basiliximab to a TAS regimen for European paediatric renal transplant recipients at low immunological risk.
Presented recommendations are an extended and actualized version of paediatric recommendations of Polish Society of Hypertension published in 2015. Since then, new studies have appeared on this subject, introducing a new classification of hypertension, newly described principles of management in risk groups and methods of assessing hypertensive target organ damage. The presented updated recommendations included changes introduced in the 2016 European Society of Hypertension recommendations and Recommendations of Children's Memorial Health Institute in Warsaw. Recently published guidelines of American Academy of Pediatrics have been discussed. In the presented issue of guidelines, epidemiological information on the occurrence and incidence of hypertension in developmental age was added and classification of blood pressure values was updated. Subchapters on diagnosis and organ damage assessment, principles of diagnosis and treatment of hypertension in children with diabetes, chronic kidney disease and a subsection discussing diagnostic and therapeutic difficulties with setting blood pressure targets were revised. In addition, the principles of early diagnosis of arterial hypertension in post-hospital care in children born before 33 weeks of gestation published in 2018 as recommendations of the Polish Neonatal Society have been also taken into account.
Pediatric patients with end-stage renal failure due to severe drug-resistant nephrotic syndrome are at risk of rapid recurrence after renal transplantation. Treatment options include plasmapheresis, high-dose of cyclosporine A/methylprednisolone and more recently—rituximab (anti-B CD20 monoclonal depleting antibody). We report five patients with immediate (1–2 days) post-transplant recurrence of nephrotic syndrome, treated with this kind of combined therapy including 2–4 weekly doses of 375 mg/m2 of rituximab. Only two (of five) patients have showed full long-term remission, while the partial remission was seen in two cases, and no clinical effect at all was achieved in one patient. The correlation between B CD19 cells depletion and clinical effect was present in two cases only. Severe adverse events were present in two patients, including one fatal rituximab-related acute lung injury.Conclusion: The anti-CD20 monoclonal antibody may be not effective in all pediatric cases of rapid post-transplant recurrence of nephrotic syndrome, and benefit/risk ratio must be carefully balanced on individual basis before taking the decision to use this protocol. What is Known: • nephrotic syndrome may recur immediately after renal transplantation • plasmapheresis combined with pharmacotherapy is used as rescue management • rituximab was reported as effective drug both in primary and post-transplant nephrotic syndrome What is New: • rituximab may not be effective is several cases of post-transplant nephrotic syndrome due to variety of underlying mechanisms of the disease, which may be or not be responsive to this drug • there may be no correlation between drug-induced depletion of specific B cells and clinical effect; this might suggest B-cell independent manner of rituximab action
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