Resistance to rocuronium in a child with Schwartz-Jampel syndrome type 1 B.

In Schwartz-Jampel syndrome micrognathia and jaw muscle rigidity may result in difficult or impossible tracheal intubation. Since the dose-response relationship to muscle relaxants is unknown in this rare disease we assessed by mechanomyography the neuromuscular response to the rocuronium in a two-year-old child with Schwartz-Jampel syndrome (SJS) Type 1 B. Rocuronium's dose-response curve was markedly shifted (3.5-fold dose) to the right when compared to healthy children and intubation conditions were improved. This resistance to NDMR may result from a lower acetylcholine degradation rate suggested as being the consequence of mutation of the gene encoding perlecan (HSPG2) in SJS. Thus, considerably higher doses of NDMR than usual may be required for facilitation of tracheal intubation in patients with SJS. Since evidence for genetic heterogeneity of SJS exists we also recommend incremental doses of a rapidly acting NDMR with continuous monitoring of neuromuscular function so as to assess the optimum relaxant dose.

Intubating conditions and onset of action after rocuronium, vecuronium, and atracurium in young children.

To evaluate muscle relaxant onset times and tracheal intubating conditions, 60 children (ASA physical status I or II) aged 18 to 72 mo were randomly assigned to receive a bolus of either rocuronium 0.6 mg/kg, vecuronium 0.1 mg/kg, or atracurium 0.5 mg/kg. After induction of anesthesia with etomidate 0.2-0.4 mg/kg and fentanyl 1-3 mg/kg, lungs were ventilated with 50% nitrous oxide in oxygen via a face mask. The evoked electromyogram of the adductor pollicis to a train-of-four stimulation every 20 s was monitored. After administration of the muscle relaxant, endotracheal intubation was attempted every 30 s, beginning 30 s after drug administration, until intubation could be achieved with good or excellent conditions. Rocuronium produced acceptable intubating conditions significantly faster (all tracheas intubated within 60 s) compared with vecuronium (120 s) and atracurium (180 s). The quality of intubating conditions at the time of completed intubation was rated significantly better with rocuronium than with vecuronium or atracurium. However, onset to 95% block at the adductor pollicis muscle was not significantly different after rocuronium (92 +/- 46.9 s), vecuronium (112 +/- 33.3 s), or atracurium (134 +/- 57.1 s), and mean neuromuscular block achieved at the point of successful intubation was not complete in all groups. We conclude that clinically acceptable intubating conditions are produced more rapidly with rocuronium than with atracurium or vecuronium.

Lymphocyte subsets in bronchoalveolar lavage fluid of children without bronchopulmonary disease.

Bronchoalveolar lavage (BAL) is increasingly used in the pediatric age group. However, normal values for BAL fluid (BALF) constituents are lacking. As part of an investigation to define reference values in children, we studied lymphocyte surface markers of BALF in 28 children 3 to 16 yr of age without bronchopulmonary disease. All of them were undergoing elective surgery for nonpulmonary illnesses. BAL was performed under general anaesthesia with tracheal intubation. A flexible bronchoscope (Pentax 3.5 or 4.9 mm) was wedged into the middle lobe or into one of its segments, and 3 x 1 ml/kg body weight normal saline warmed to body temperature were instilled and immediately withdrawn. The first sample was studied separately; subsequent samples were pooled for analysis. Studies on lymphocyte surface markers were performed on the pooled sample only. The distribution of B-cells, pan T-cells, and CD57 positive cells was within the range reported for adult subjects. However, CD4/CD8 ratios were lower than in adults (0.7 +/- 0.4, mean +/- SD). This decrease in the CD4/CD8 ratio was caused by an increase in CD8 cells. Comparative analysis of blood and BALF lymphocytes in a subgroup of children showed that CD4/CD8 ratios in blood were within the normal range reported for this age group and significantly higher when compared with BALF. The lower CD4/CD8 ratios in normal children have to be considered in the interpretation of BALF in children with pulmonary diseases.

Differential cytology of bronchoalveolar lavage fluid in normal children.

Bronchoalveolar lavage (BAL) is increasingly used in the assessment of pulmonary diseases in children. However, reference values for cellular and non-cellular constituents of BAL fluid in children are lacking. We have studied the differential cytology of BAL fluid in 48 children aged 3-16 years (mean age +/- SD 7.9 +/- 3.5 yrs) undergoing elective surgery for nonpulmonary illnesses. A flexible bronchoscope (Pentax 3.5 or 4.9 mm) was wedged in the middle lobe or one of its segments. BAL was performed with 3x1 ml-kg-1 body weight of normal saline warmed to body temperature. The first sample was studied separately; subsequent samples were pooled for analysis. The mean recovery was 58 +/- 15%. Significantly more granulocytes and less lymphocytes were found in the first, as compared to the pooled, sample. Total cell counts were highly variable and ranged from 0.5-57.1 x 10(4) ml-1. Macrophages were the predominant cell type, with a mean percentage of 81.2 +/- 12.7%. The relative proportion of lymphocytes was higher than that reported in most studies of adult volunteers (16.1 +/- 2.4%). No age dependency was observed for either cell type. The mean percentage of granulocytes was 2.5 +/- 3.3%. Absolute granulocyte counts were significantly higher in children under 8 yrs of age. This study provides the first reference data on BAL differential cytology in children without pulmonary disease and will be the basis for future investigations of BAL in paediatric lung diseases.

[Porto-femoro-subclavian bypass and the rapid infusion technique in orthotopic liver transplantation]. / Portofemoro-Subclavia Bypass und "Rapid Infusion"-Technik bei orthotoper Lebertransplantation.

Veno-venous bypass during orthotopic liver transplantation is commonly performed as a porto-femoro-axillary bypass. Right-angle positioning of one patient's arm and surgical preparation of the femoral and axillary veins are necessary. In 17 patients and 20 consecutive orthotopic liver transplant procedures the common porto-femoro-axillary veno-venous bypass was substituted by porto-femoro-subclavian bypass with a percutaneous 20 F cannulation set (LAUB catheter, Cook). The 20 F catheter was introduced into the left subclavian vein by Seldinger's technique before the operation and was connected intraoperatively to the outflow tube of the biopump. Surgical preparation of the axillary vein was not performed. In 10 patients a Y-connector was used to connect up the Rapid Infusion System in addition. Postoperatively the catheter was left in place for 2-4 days. Introduction and removal of the catheter were uneventful in all cases. High blood flow through the catheter could be maintained by a low driving pressure of the pump (4000 ml/min; 100 mmHg). No intraoperative complications were observed. Shunt flows remained stable throughout the surgical procedure during the anhepatic stage. There was no bleeding from the puncture site, especially after removal of the catheter, though several patients had a poor coagulatory status in the early postoperative period. Two postoperative complications were observed: air embolism due to disconnection and formation of a thrombus at the catheter tip, which it was possible to remove together with the catheter itself. Installation of an irrigation infusion in the postoperative period and well-tightened connections help avoid such complications as thrombus formation, bleeding or air embolism.(ABSTRACT TRUNCATED AT 250 WORDS)