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BACKGROUND: Cystic fibrosis (CF) is the most common cause of exocrine pancreatic insufficiency in childhood. The aim of the present study is to evaluate the correlation between genotype and exocrine pancreatic insufficiency in CF patients. The special emphasis was put on the analysis of mild CFTR mutations. DESIGN: The study comprised 394 CF patients and 105 healthy subjects (HS). Elastase-1 concentrations were measured in all subjects. RESULTS: Severe pancreatic insufficiency was associated with the presence of two CFTR gene mutations (DeltaF508, N1303K, CFTR dele 2,3 (21kb), G542X, 1717-1G-A, R533X, W1282X, 621GT, 2183AAG, R560T, 2184insA and DeltaI507, G551D, 895T) and mild insufficiency with the presence of at least one mutation (R117H, 3171insC, A155P2, 138insL, 296 + 1G-A, E92GK, E217G, 2789 + 5G-A. 3849 + 1kbC-T/3849 + 1kbC-T) genotype resulted in high elastase-1-values. However, in case of patients with genotype DeltaF508/3849 + 10kbC-T, 1717-1GA/3849 + 10kbC-T as well as with DeltaF508/R334W, both high and low elastase-1 concentrations were found. Low E1 values were found in a patient with DeltaF508/R347P genotype. CONCLUSION: Patients who carry two 'severe' mutations develop pancreatic insufficiency, whereas those who carry at least one 'mild' usually remain pancreatic sufficient. However, the presence of one mild mutation does not exclude pancreatic insufficiency.

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Propofol provides smooth and rapid induction of anesthesia in adults and guarantees rapid recovery. The use of propofol in adults is frequently associated with pain on injection, but this can be reduced by: (1) injection into the relatively large veins in the forearm or the antecubital fossa: (2) addition of lignocaine to the propofol; or (3) injection of an opioid (alfentanil) before propofol. Compared with experience in adults, there is very little experience with propofol in pediatric anesthesia. The aim of this random prospective study was to compare the induction characteristics of propofol and thiopentone in pediatric anesthesia. Vigilance and behavior in the postoperative period were also compared. METHOD. A total of 75 healthy children aged 3-12 years who were undergoing elective operations were studied. All the children received premedication with 0.5 mg/kg midazolam with 0.02 mg/kg atropine by the rectal route. The children were divided randomly into 3 groups and received: group A, thiopentone 3-6 mg/kg; group B, propofol 1-3 mg/kg mixed with lignocaine (10/200 mg); group C, propofol 1-3 mg/kg, followed 1 min later by alfentanil 0.01 mg/kg. The induction agent was injected over 30 s, if possible into the vein in the forearm or the antecubital fossa. The immediate reaction on vein puncture and any discomfort during the injection were noted. After intubation the anesthesia was maintained with 1 vol% halothane, nitrous oxide and oxygen (2:1). Arterial pressure was measured on arrival in the induction room, immediately after the induction of anesthesia, immediately after intubation and thereafter at 5-min intervals throughout the anesthesia. Heart rate (ECG) and arterial oxygen saturation (saO2) were measured continuously. The existence of any anterograde amnesia was tested (age over 4 years) by means of one of 6 pictures shown to each before operation. The ability to recall the picture shown was recorded 2-5 h postoperatively. The completeness of recovery was assessed at 10-min intervals up to 1 h, and thereafter at 1-h intervals. In the postoperative period a recovery test (postbox test) was performed. The incidence of side effects during the induction of anesthesia and also during recovery and the postoperative period (for 5 h) was recorded. RESULTS. There was no significant difference between the children in the 3 different groups with regard to age, body weight, type of operation, or duration of anesthesia. Among the total of 75 children, 66.7% accepted the vein puncture very well, and 24% well. In some children in each group we observed obstruction of the respiratory tract (group A, 36%; group B, 48%; group C, 64%). Apnea for 20 s was observed only in groups B and C (2 and 3 children respectively). During spontaneous respiration with room air there was a significant decrease of the arterial oxygen saturation about 1 min after induction in all groups. In children ventilated with oxygen by mask, the SaO2 remained nearly constant...

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Anesthetic premedication by injection is usually poorly accepted by children, especially those under 10 years of age. Less disturbing for the child is oral premedication, but this increases the risk of aspiration and must be administered 1.5-2 h before anesthetic induction. This double-blind study was performed in children to investigate the efficacy, acceptance, and general safety of midazolam given rectally. METHOD. Rectal premedication was administered to a total of 80 healthy children between 2 and 10 years of age undergoing elective operations. The children were divided randomly into two groups: group I received 0.4 mg/kg and group II 0.5 mg/kg midazolam with the addition of 0.015-0.02 mg/kg atropine. Premedication was carried out on the pediatric ward. The calculated dose was drawn from the ampule and diluted to 8-10 ml with distilled water. This dose was instilled immediately behind the anal sphincter using a suitable plastic applicator (Stanylan). The following parameters were recorded: immediate reaction to the rectal medication, sedative-hypnotic signs, and acceptance of the anesthetic mask. Heart rate and blood pressure were measured before premedication and before the induction of anesthesia. Observations were made for 5 h post-operatively. Any unusual side effects of the treatment were also noted. The existence of any anterograde amnesia was investigated in 20 children (10 in each group) between 6 and 10 years of age. RESULTS. There was no significant difference between the children allocated to the two groups with regard to age, body weight, sex, type of operation, and duration of anesthesia (Table 2). Of the total of 80 children, 66 (82.5%) accepted the rectal instillation well, 12 (15%) moderately well, and 2 (2.5%) poorly. Signs of respiratory depression or allergic reaction to midazolam were not observed in any case. The observations made before induction of anesthesia are presented in Table 3. The children in group II exhibited significantly greater (P less than 0.05) slurred speech than those in group I. A low incidence of hiccup was seen in both groups. Most of the children (27 in group I, 67.5%; 37 in group II, 92.5%: P less than 0.05) were delivered to the operating room lying down, whereas the others were sitting up in bed but showed no desire to get up. Between 10 and 55 min after the premedication, a total of 5 children (12.5%) in group I and 2 (5%) in group II were restless or crying on arrival in the induction room. Most, however, were quiet to tired/drowsy. The optimal sedative-hypnotic action was observed after 20-30 min (Fig. 1). At this time 21.7% of the children in group I were tired/drowsy, whereas 50% in group II were tired/drowsy and 9.1% were asleep but easy to arouse. This effect was significantly greater in group II (P less than 0.01). Acceptance of the mask was comparable in both groups (Table 4) and was tolerated well to very well by 92-97% of the children. (ABSTRACT TRUNCATED AT 400 WORDS)

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The administration of vecuronium in divided doses with respect to onset of action and intubating conditions was investigated. This study was conducted in 3 parts. In part 1 the maximal precurarizing dose of 0.015 mg/kg vecuronium was found. In the second part we showed that the optimal time interval between divided doses was 3 min. In the third part we made a comparative investigation between the effect of 0.015 + 0.085 mg/kg body wt. at a 3 min interval and that of a single dose of 0.1 mg/kg body wt. vecuronium. --Neuromuscular function was assessed in semiquantitative manner, using the train of four (TOF) stimulation of ulnar nerve. Administration of vecuronium in divided doses, compared with a single dose of 0.1 mg/kg body wt., resulted in a more than 1 min earlier onset time of 1.5-2.5 min and facilitated early intubation. As soon as twitch tension had completely disappeared uniform by excellent intubating conditions were available in all cases. The administration of vecuronium in divided doses enabled better intubating conditions than the same single dose of vecuronium.

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Our previous work has shown that the addition of diphenhydramine hydrochloride to platelet-rich plasma is capable of causing disaggregation of platelet aggregates already induced by adenosine diphosphate as well as of inhibiting platelet aggregation when added prior to the aggregating agent. This led us to attempt to prevent arterial thrombosis in a canine experimental model. Ten dogs received an injection of diphenhydramine (approximately 3 mg/kg of body-weight) into the right common femoral artery 1 cm proximal to the proximal end of a 2 cm endarterectomy immediately after closure of the endarterectomy site and prior to performing an endarterectomy of the other common femoral and both common carotid arteries. The dogs which received diphenhydramine were compared with ten control dogs and found to have no protection against thrombosis at the sites of endarterectomy after 4 and 24 hours.

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