RESUMO
RESULTS: Out of 34 patients who underwent gastrostomy tube insertion, 30 patients had their long tube replaced by a button gastrostomy. Majority were males (N = 18, 60%). Prolonged nasogastric tube feeding was the main indication of referral (N = 17, 56%) followed by feed intolerance (N = 6, 17%) and gastroesophageal reflux disease (N = 5, 16%). The main underlying diseases at referral were neurological impairment (N = 19, 63%) and metabolic disorders (N = 4, 13%). There was no significant difference between patients with neurological disorders and other diseases in terms of gender, nationality, or age. Laparotomy with gastrostomy is the main approach used (N = 18, 60%). No reported complications of button tubes in 50% of the patients (N = 15). CONCLUSIONS: Prolonged nasogastric tube feeding is the main indication of referral for gastrostomy tube insertion. Neurological disorders are the main diagnosis for the cases operated upon. Laparotomy with gastrostomy is the procedure of choice at our center. Majority of patients had no reported complications of button tube replacement. These children are likely to benefit from the button tube with fewer complications.
RESUMO
PURPOSE: Auxiliary partial orthotopic liver transplantation (APOLT) in metabolic liver disease (MLD) has the advantage of correcting the metabolic defect, preserving the native liver for gene therapy in the future with the possibility of withdrawal of immunosuppression. METHODS: Retrospective analysis of safety and efficacy of APOLT in correcting the underlying defect and its impact on neurological status of children with MLD. RESULTS: A total of 13 APOLT procedures were performed for MLD during the study period. The underlying aetiologies being propionic acidemia (PA)-5, citrullinemia type 1 (CIT1)-3 and Crigler-Najjar syndrome type 1 (CN1)-5 cases respectively. Children with PA and CIT1 had a median of 8 and 4 episodes of decompensation per year, respectively, before APOLT and had a mean social developmental quotient (DQ) of 49 (<3 standard deviations) as assessed by Vineland Social Maturity Scale prior to liver transplantation. No metabolic decompensation occurred in patients with PA and CIT1 intraoperatively or in the immediate post-transplant period on protein-unrestricted diet. Patients with CN1 were receiving an average 8-15 h of phototherapy per day before APOLT and had normal bilirubin levels without phototherapy on follow-up. We have 100% graft and patient survival at a median follow-up of 32 months. Progressive improvement in neurodevelopment was seen in children within 6 months of therapy with a median social DQ of 90. CONCLUSIONS: APOLT is a safe procedure, which provides good metabolic control and improves the neurodevelopment in children with selected MLD.
