[Anesthetic management of a patient with Coffin-Lowry syndrome].

Coffin-Lowry syndrome (CLS) is characterized by mental retardation, a peculiar face and deformities of the thorax and spine. A 33-year-old female with Coffin-Lowry syndrome (CLS), further complicated with atrial septal defect and ventricular tachycardia, underwent elective surgery for anterior cervical cyst. As difficult intubation had been anticipated, anesthesia was induced with continuous administration of propofol. After confirming that she could be ventilated by mask, vecuronium bromide, midazolam and fentanyl were given. The operation and anesthesia were conducted uneventfully. No complications occurred postoperatively. The use of propofol for slow induction of anesthesia was advantageous for hemodynamic stability in this case.

[Anesthetic management of a pediatric patient with non-Fukuyama type congenital muscular dystrophy].

Non-Fukuyama type congenital muscular dystrophy (n-FCMD), a subtype of progressive muscular dystrophy (PMD), is a very rare autosomal recessive disorder. N-FCMD is characterized by severe and progressive motor weakness and atrophies of proximal muscles during the infant period. A 9-year-old boy with n-FCMD underwent elective surgery for muscle release around the hip joints bilaterally. As many perioperative complications related with volatile anesthetics and muscle relaxants had been reported in the anesthetic management of PMD, these drugs were thought to be contraindicated in patients with n-FCMD. Because n-FCMD seemed to have very similar pathogenesis with PMD, caudal epidural block was chosen, supplemented with the administration of diazepam, pentazocine and nitrous oxide. The operation and anesthesia were conducted uneventfully. No complications occurred postoperatively.

[Anesthesia for a patient with severe aplastic anemia].

A 48-year-old female with severe aplastic anemia was scheduled for transurethral lithotomy because of pyelonephritis and urethral stone. Laboratory studies showed anemia (168 x 10(4).mm-3), leukopenia (2300.mm-3) and thrombocytopenia (5000.mm-3). Bleeding time exceeded 30 min, but the transfusion of fresh platelet concentrate was not effective for bleeding tendency. Anesthesia was induced with midazolam 0.5 mg and fentanyl 100 micrograms, and maintained with N2O-O2-sevoflurane through a mask. The operation, which lasted for 40 min, was uneventful without marked hemodynamic changes, bucking or massive bleeding. Although 100 units of fresh platelet and 13 units of leucocyte poor red cells were infused during hospitalization, macrohematuria continued for about 3 weeks after this operation.

Perioperative plasma concentrations of endothelin and natriuretic peptides in children undergoing living-related liver transplantation.

To investigate the clinical significance of endothelin (ET), natriuretic peptides, and the renin-angiotensin-aldosterone system in pediatric liver transplantation, we measured plasma levels of ET, atrial and brain natriuretic peptides (ANP, BNP), aldosterone, and plasma renin activity in 18 patients (aged 0.5-12 yr; median 1 yr) undergoing living-related liver transplantation due to congenital biliary atresia and severe liver cirrhosis. Before transplantation, the plasma ET level (28.9 +/- 2.5 [mean +/- SEM] pg/mL) was increased compared with that of healthy children (10-18 pg/mL), but decreased during the anhepatic phase (22.5 +/- 1.6 pg/mL). It increased again after reperfusion and remained at high levels in the early postoperative period (postoperative day 3, 27.8 +/- 3.0 pg/mL). Plasma levels of ANP and BNP and aldosterone and plasma renin activity were also high before surgery. Plasma ANP and BNP did not change significantly during surgery. After transplantation, plasma BNP significantly increased, and plasma ANP tended to increase. Plasma aldosterone increased markedly during the anhepatic phase, although plasma renin activity decreased. After transplantation, plasma aldosterone and plasma renin activity both decreased to within normal levels. Mean arterial blood pressure increased gradually after reperfusion and surgery (postoperative day 3, 35.7 +/- 5.2% increase). No substantial differences in these variables occurred between the younger (< or = 1.0 yr, n = 9) and older patients (> 1.0 yr, n = 9). These results suggest that ET production in the cirrhotic liver is augmented and ET, natriuretic peptides, and the renin-angiotensin-aldosterone system all play some role in the circulatory regulation during perioperative periods of pediatric liver transplantation.

[Spinal anesthesia for patients with progressive muscular dystrophy].

Spinal anesthesia was applied on 8 occasions to 7 patients with progressive muscular dystrophy (PMD) undergoing orthopedic lower limb surgery. No postoperative complication occurred in all patients. During the operations, however, two patients were subjected to high spinal anesthesia, which caused ventilatory suppression in one patient and bronchial asthma attack in another. Both respiratory complications were easily managed by ventilatory assistance with endotracheal intubation or by administration of bronchodilator, respectively. High spinal anesthesia should be avoided in applying spinal anesthesia to patients with PMD.

Effects of anesthesia and surgery on plasma endothelin levels.

To investigate the clinical significance of endothelin (ET), a potent and long-acting vasoconstrictor peptide in anesthesia and surgery, we measured plasma ET-like immunoreactivity (ET-LI) levels by using radioimmunoassay in patients undergoing various kinds of surgery under general anesthesia. No significant changes in plasma ET-LI levels were observed in patients undergoing relatively minor surgery under general anesthesia with nitrous oxide and halothane (n = 6), enflurane (n = 6), or isoflurane (n = 5). Although plasma ET-LI levels after surgery in patients undergoing total knee replacement (12.4 +/- 0.9 [mean +/- SEM] pg/mL, n = 7), hysterectomy (11.4 +/- 0.6 pg/mL, n = 8) or cholecystectomy (14.8 +/- 1.2 pg/mL, n = 9) were no different from those before surgery, plasma ET-LI levels after surgery in patients undergoing gastrectomy (20.4 +/- 1.9 pg/mL, n = 15), esophagectomy (24.7 +/- 2.5 pg/mL, n = 12), hepatectomy (27.5 +/- 3.4 pg/mL, n = 12), or heart surgery (43.1 +/- 4.1 pg/mL, n = 18) were higher than those before surgery (P < 0.05). Changes in plasma ET-LI levels during surgery had positive correlations with the duration of the operation (n = 100, r = 0.51, P < 0.01) and intraoperative blood loss (n = 100, r = 0.30, P < 0.01). In patients undergoing subtotal esophagectomy, the plasma ET-LI level did not increase during the initial 2 h, but increased gradually during surgery, reached a peak within a few hours after surgery, and declined slowly thereafter.(ABSTRACT TRUNCATED AT 250 WORDS)