Rare Diseases Symptoms Automatic Extraction

Immediate postoperative intensive care treatment of pediatric combined liver-kidney transplantation: outcome and prognostic factors.

[primary hyperoxaluria type 1]

Studies reporting the immediate pediatric intensive care unit (PICU) treatment after combined liver-kidney transplantation (CLKT) are scarce, although this period is pivotal for survival and long-term outcome.We retrospectively analyzed all pediatric CLKT performed in our center between 1998 and 2010.Sixteen patients underwent 17 CLKT at a median age of 5.3 years (range, 1.3-15.9 years). Median body weight at CLKT was 17.7 kg (range, 9.2-55 kg). Underlying diagnosis was primary hyperoxaluria type 1 in nine patients and autosomal recessive polycystic kidney disease in seven patients. Median time on PICU was 8.5 days (range, 3-68 days); however, patients with primary hyperoxaluria type 1 had a significantly longer stay (P=0.031). Median duration of ventilation was 1 day; however, five patients required ventilation for 25 to 52 days. Continuous veno-venous hemofiltration was applied in nine patients due to delayed kidney graft function, volume overload, or high plasma oxalate. Overall, the survival rate after CLKT was 100% and long-term outcome was very good at a mean follow-up of 3.6 years (range, 0.5-12.2 years). Waiting time, donor age, and donor-to-recipient weight ratio were found to be significant risk factors for an extended PICU stay (P=0.02, 0.0031, and 0.014, respectively).Immediate postoperative course after CLKT may be challenging and complex. However, excellent results can be achieved, even in small children.

Diseases presenting "long-term outcome" symptom

  • aniridia
  • cholangiocarcinoma
  • classical phenylketonuria
  • cystinuria
  • erythropoietic protoporphyria
  • esophageal squamous cell carcinoma
  • fabry disease
  • hirschsprung disease
  • homocystinuria without methylmalonic aciduria
  • hydrocephalus with stenosis of the aqueduct of sylvius
  • inclusion body myositis
  • kabuki syndrome
  • krabbe disease
  • oligodontia
  • papillon-lefèvre syndrome
  • primary hyperoxaluria type 1
  • trochlear dysplasia
  • von hippel-lindau disease
  • waldenström macroglobulinemia

You can validate or delete this automatically detected symptom