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Liver biopsies in patients with lysosomal storage disease: experience with effective sedation
R P Grewal1, K T Yu, N W Barton
1Developmental and Metabolic Neurology Branch, National Institute of Neurological Disorders and Stroke, NIH, Bethesda, MD, USA. grewal@molbio.usc.edu
Insights
This study evaluated a sedation protocol for pediatric liver biopsies. The protocol safely enabled biopsies in children, with minimal complications, suggesting its utility for this procedure.
Area of Science:
- Pediatric Gastroenterology
- Pediatric Anesthesiology
- Medical Genetics
Background:
- Liver biopsies are crucial for diagnosing pediatric liver diseases.
- Sedation is often necessary for pediatric procedures like liver biopsy.
- Existing sedation protocols may have limitations in pediatric patients.
Purpose of the Study:
- To assess the safety and efficacy of a specific sedation protocol for pediatric liver biopsies.
- To evaluate the complication rates associated with the sedation protocol during liver biopsies.
- To determine the feasibility of using this protocol in children with specific genetic disorders.
Main Methods:
- Prospective application of a sedation regimen including chlorpromazine, meperidine, and pentobarbital.
- Sixty liver biopsies were performed on thirty pediatric patients.
- Patients with Gaucher's disease and Niemann-Pick disease type C were included, with specific attention to bleeding times and cognitive status.
Main Results:
- The sedation protocol allowed for safe liver biopsy acquisition in all pediatric patients.
- A low rate of serious complications (2%) was observed, with one case of airway obstruction successfully managed.
- Minor transient complications occurred in 12% of patients, with no long-term sequelae.
Conclusions:
- The evaluated sedation protocol is safe and effective for pediatric liver biopsies.
- Appropriate patient selection is key to the successful implementation of this protocol.
- This protocol may be a valuable option for sedating children undergoing liver biopsy.
Abstract:
We prospectively applied a protocol used to sedate children who required a liver biopsy. Sixty liver biopsies were performed on thirty pediatric patients to assess the effects of treatment. Sixteen patients had Type 1 Gaucher's disease of which seven had a platelet count between 50-100,000/mm3. All seven had bleeding time performed and when indicated, intravenous DDAVP (1-deamino-8-D-arginine vasopressin) was used to improve hemostasis. Fourteen patients had Niemann-Pick disease type C of which eight were significantly demented and uncooperative. Before liver biopsy, all patients were sedated with the following regimen: oral chlorpromazine (1 mg/kg) followed one hour later by intravenous meperidine (1 mg/kg) and pentobarbital (maximum dosage 6 mg/kg) administered by slow intravenous injection. Liver biopsies were obtained safely on all patients. Only 1 patient (2%) developed a potentially serious complication: an obstructed airway which was readily corrected by simple repositioning. Transient less serious complications occurred in another 7 patients (12%). There was no long term sequalae of the biopsy procedures. Our study indicates that with appropriate patient selection, this sedation protocol may be useful in pediatric patients requiring a liver biopsy.