Liver Biopsies in Patients with Lysosomal Storage Disease : Experience with Effective Sedation

被引:3
作者
Grewal R.P. [1 ,2 ,6 ]
Yu K.T. [1 ,3 ]
Barton N.W. [1 ]
Parker R.I. [4 ]
Di Bisceglie A.M. [5 ]
机构
[1] Devmtl. and Metab. Neurology Branch, Natl. Inst. Neurol. Disord. Stroke, NIH, Bethesda, MD
[2] Department of Neurology, USC School of Medicine, Los Angeles, CA
[3] Depts. of Pediatrics and Neurology, Harbor-UCLA Medical Center, Torrance, CA
[4] Hematology Service, CPD, NIH, Bethesda, MD
[5] Liver Disease Section, Natl. Inst. Diabet. Digest. K., NIH, Bethesda, MD
[6] SHS 172 MC, University Park Campus, USC, Los Angeles, CA 90089
关键词
Liver biopsy; Sedation; Type 1 Gaucher's disease;
D O I
10.1007/BF02725518
中图分类号
学科分类号
摘要
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.
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页码:887 / 891
页数:4
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