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.
Similar content being viewed by others
References
Heya RM, Hamoudi A, Newton A, Newton Jr. WA. Pretreatment liver biopsy in 20 children with histiocytosis X: A clinicopathologic correlation.Medical and Pediatric Oncology, 1990; 18: 110–118.
Di Bisceglie AM, Ishak, AG, Robin L.et al. Cholesteryl ester storage disease: hepatopathology and effect of therapy with lovastatin.Hepatology, 1990; 11: 764–772.
Brady RO, Barranger JA. Glucosylceramide lipidosis. In: Stanbury JB, Wyngaarden JB, Fredrickson DS, Goldstein JL, Brown MS, eds.The Metabolic Basis of Inherited Disease N.Y. McGraw-Hill, 1983; 842–856.
Blanchette-Makie J, Kruth HSet al. Type C Niemann-Pick Disease.J Biol Chem, 1990; 7: 3411–3417.
Sherlock, S. Needle biopsy of the liver. In:Diseases of the Liver and Biliary System. 8th ed. Oxford: Blackwell Scientific Publication, 1990.
Mailer, ES, Watkins JB. Liver biopsy. In: Dinari G, Rozen P, Bujanover Y, Lebenthal E, eds.Newer Tests and Procedures in Pediatric Gastroenterology. Front Gastrintest Res. Basel, Karger, 1989: 254–267.
Rail TW in Gilman GG, Rall TW, Nies ASet al. eds.The Pharmacological Basis of Therapeutics, 8th ed. N.Y. Macmillan, 1990; 362.
Fink JK, Filling-Katz MR, Sokol Jet al. Clinical spectrum of Niemann-Pick disease type C.Neurology, 1989; 39: 1040–1049.
Sharma P, McDonald GB, Banarji M. The risk of bleeding after percutaneous liver biopsy: relation to Platelet Count.J. Clin Gastroenterol, 1982; 4: 451–453.
Parker RI, Grewal RP, Barton NW. The effect of platelet count on DDAVP-induced shortening of the bleeding time in Gauchers disease.Clinical Research, 1990; 38: 539A.
Author information
Authors and Affiliations
Corresponding author
Rights and permissions
About this article
Cite this article
Grewal, R.P., Yu, K.T., Barton, N.W. et al. Liver biopsies in patients with lysosomal storage disease: Experience with effective sedation. Indian J Pediatr 64, 887–891 (1997). https://doi.org/10.1007/BF02725518
Issue Date:
DOI: https://doi.org/10.1007/BF02725518