A new form of metaphyseal chondrodysplasia in two sibs: surgical treatment of tracheobronchial malacia and scoliosis.

We describe a 19-year-old male with a previously unrecognized form of disproportionate short stature, tracheobronchial malacia, and progressive scoliosis and his 28-year-old sister with the same but milder condition. The clinical characteristics were short limbs and digits and thoracolumbar scoliosis. Bone films showed progression from marked metaphyseal dysplasia of tubular bones in childhood to short and broad bones with mild dysplasia of the joints in adulthood. The vertebrae and the intervertebral plates were only mildly affected in spite of marked scoliosis. Trachea and bronchi were reinforced with surrounding acrylate mesh before surgical treatment of the scoliosis. Affected sibs of both sexes and healthy parents suggest an autosomal recessive mode of inheritance.

Intramucosal pH and endotoxin and cytokine release in severe acute pancreatitis.

It has been postulated that in severely ill patients splanchnic hypoperfusion may cause endotoxin release from the gut, and this leakage of endotoxin into the circulation can trigger the cascade of inflammatory cytokines. We tested this hypothesis in 9 patients with acute severe pancreatitis by monitoring gastric intramucosal pH (pHi) as measure of splanchnic hypoperfusion at 12-h intervals trying to correlate it to endotoxin and cytokine release. Only 3 of 59 samples, obtained from 3 patients contained circulating endotoxin. Thirteen of 15 plasma samples drawn at pHi <7.20 did not contain endotoxin. The pHi was significantly lower in patients who subsequently developed 3 or more organ failures (P = 0.0017, analysis of variance). Although endotoxemia was only occasionally found, most patients had measurable interleukin 1beta (IL-1beta), interleukin 6 (IL-6), interleukin 8 (IL-8), and interleukin 10 (IL-10) in their plasma. Concentrations of IL-6, IL-8, and IL-10 on admission correlated to degree of organ dysfunction as measured by the multiple organ system failure score (P = 0.035, r = 0.74; P = 0.010, r = 0.91; P = 0.021, r = 0.82, respectively). In conclusion, patients with acute, severe pancreatitis often have splanchnic hypoperfusion and produce a wide array of cytokines despite a rare occurrence of endotoxemia.

Zinc chloride smoke inhalation: a rare cause of severe acute respiratory distress syndrome.

Zinc chloride smoke inhalation is a rare cause of slowly progressive and often fatal acute respiratory distress syndrome (ARDS). The conventional treatment includes intravenous N-acetylcysteine, L-3, 4-dehydroproline, methylene blue, and respiratory support according to the lung protective strategy. This report presents the cases of three patients with serious zinc chloride inhalation and ARDS, the last of whom survived after prolonged intensive care, videothoracoscopic excision of emphysema bullae, and recurrent chemical pleurodesis.

Late steroid therapy in primary acute lung injury.

OBJECTIVE: To investigate the effect of steroid treatment in the late phase of primary acute lung injury (ALI) with special emphasis on pneumococcal pneumonia. DESIGN: Retrospective study. SETTING: Multidisciplinary intensive care unit (ICU) in a university hospital. PATIENTS: Of 31 patients with primary ALI requiring mechanical ventilation for more than 10 days, 16 were treated with methylprednisolone and 15 served as controls. MEASUREMENTS AND RESULTS: Steroid and control groups were comparable regarding demographic data, APACHE II score, Multiple Organ Dysfunction Score (MODS), and PaO2/FiO2-ratio on admission to ICU. The mean start of steroid therapy was 9.7 days after establishment of respiratory failure, and values for control patients were registered on day 10. The PaO2/FiO2 ratio improved significantly within 3 days after the start of steroid therapy, and MODS and C-reactive protein decreased concurrently. No differences in mortality, in length of ICU stay, or in length of mechanical ventilation were detectable. In a subgroup analysis, for patients with Streptococcus pneumoniae pneumonia, beneficial change in physiological variables was evident. CONCLUSIONS: In patients with primary ALI, steroid therapy, started 10 days after the start of mechanical ventilation, improves gas exchange and is associated with a decrease in multiorgan dysfunction.

Validation of air tonometric measurement of gastric regional concentrations of CO2 in critically ill septic patients.

OBJECTIVE: To evaluate the accuracy of continuous air tonometry (Tonocap, Tonometric Division, Instrumentarium, Helsinki, Finland). DESIGN: The accuracy of air tonometry was tested by comparing it with conventional saline tonometry in mechanically ventilated, critically ill septic patients and in vitro determining the partial pressure of carbon dioxide (PCO2) of humidified gases with known concentrations of CO2. SETTING: A mixed intensive care unit in a university hospital. PATIENTS: 16 mechanically ventilated patients with sepsis. MEASUREMENTS AND RESULTS: Two gastric tonometer catheters (TRIP NGS catheter, Tonometric Division, Instrumentarium, Helsinki, Finland) were introduced into the patients' stomachs. The control catheter was used as a conventional saline tonometer and the other catheter was used with the Tonocap monitoring device. A total of 153 paired measurements was made and analysed according to Bland and Altman. The mean difference between air PCO2 and saline PCO2 values (bias), the standard deviation of the differences (precision), and the Pearson correlation coefficient between air PCO2 and saline PCO2 were calculated. The data on patients were pooled and calculated for different cycle times. The mean bias (kPa) was-0.02 with a 10-min cycle time, 0.31 with 15 min, 0.56 with 30 min and 0.21 with 60-min. The precisions were 0.39, 0.54, 0.44 and 0.76, respectively. Pearson correlation coefficients were 0.93, 0.97, 0.95 and 0.82, respectively (p < 0.0001). In vitro tonometry with the Tonocap was performed in a gas chamber fully saturated with known CO2 concentrations. The clinically important 10-min cycle time was tested with 5 Tonocap monitors. Except for the first 10-min cycle time, PCO2 values determined by the Tonocap monitoring systems were comparable to known CO2 concentrations. CONCLUSIONS: The accuracy of Tonocap continuous air tonometry is close to that of conventional saline tonometry. Moreover, the clinically important 10-min cycle time with air tonometry correlated very well with saline tonometry and the time response with air tonometry was short.

Early signs of critical illness polyneuropathy in ICU patients with systemic inflammatory response syndrome or sepsis.

OBJECTIVE: To evaluate with electromyography the incidence and the time of appearance of neuromuscular abnormality in patients with systemic inflammatory response syndrome (SIRS) and/or sepsis. DESIGN: Follow-up study. SETTING: Intensive care unit of Helsinki University Hospital, Finland. PATIENTS: Nine mechanically ventilated patients with SIRS and/or sepsis. INTERVENTIONS: Electromyography and conduction velocity measurements on the 2nd-5th day after admission to the intensive care unit. MEASUREMENTS AND RESULTS: In all nine patients electromyography revealed signs of neuromuscular abnormality. The means of compound muscle action potential amplitudes of the median and ulnar nerves were decreased. Fibrillation was observed in four patients out of nine. CONCLUSION: Because neuromuscular abnormalities seem to develop earlier than previously reported, electroneuromyography should be used more frequently as a diagnostic test.

Interleukin 1 receptor antagonist and E-selectin concentrations: a comparison in patients with severe acute pancreatitis and severe sepsis.

PURPOSE: This prospective clinical study was designed to compare interleukin 1 receptor antagonist (IL-1ra) and E-selectin concentrations in patients with severe acute pancreatitis to those with severe sepsis. MATERIALS AND METHODS: Nine consecutive patients with severe acute pancreatitis and 11 consecutive patients with severe sepsis admitted to a medical/surgical intensive care unit were included in the study. Plasma concentrations of IL-1ra and E-selectin were serially measured daily for 7 days or throughout their stay in the intensive care unit if shorter. RESULTS: The concentrations of IL-1ra were significantly higher on admission in patients with severe sepsis compared with the patients with severe pancreatitis (median levels 10,500 and 2,600 pg/mL, respectively, P = .007). When the data from the first 3 days were analyzed using analysis of variance (ANOVA), the levels of IL-1ra and E-selectin were similar in both groups. The concentrations of IL-1ra and E-selectin correlated to the development of multiorgan dysfunction as assessed by sequential organ failure assessment (SOFA) score (P = .032 and .043, respectively). CONCLUSION: This study shows that IL-1ra and E-selectin are released in acute severe pancreatitis, and the levels seem to be comparable to those in patients with severe sepsis. Concentrations of IL-1ra and E-selectin correlate to the development of multiorgan failure as indicated by high SOFA scores during the first week of disease.

Predictive value of interleukins 6, 8 and 10, and low HLA-DR expression in acute renal failure.

AIMS: HLA-DR expression and plasma levels of pro- and anti-inflammatory cytokines (IL-6, IL-8 and IL-10) and their predictive value concerning survival of critically ill systemic inflammatory response syndrome (SIRS) patients with and without acute renal failure (ARF) were evaluated. MATERIAL: A total of 103 consecutive adult patients with SIRS from 2 university hospital intensive care units participated in the study. METHOD: Laboratory data for all patients were prospectively collected on the day of admission and 2 days thereafter. Patients with acute renal failure (ARF) and non-ARF patients were compared by Mann-Whitney U-test. Independent predictors of mortality were tested using forward stepwise logistic multiple regression analysis. The discriminative power of different variables was tested using receiver operating characteristic (ROC) curve analysis. RESULTS: ARF developed in 36 patients (35%). ARF patients showed significantly lower HLA-DR expression and higher plasma levels of IL-6, IL-8 and IL-10 than non-ARF patients. In ARF, moderate discriminative power in predicting survival was observed for day 2 IL-6 and IL-10 plasma levels (AUCs 0.703 and 0.749, respectively). CONCLUSIONS: We found no clinically significant discriminative power in predicting survival of ARF patients for monocyte HLA-DR expression or cytokine plasma levels. Therefore, our results do not support the use of HLA-DR expression or cytokine plasma levels for that purpose.

Comparison of long-term survival after repair of ruptured and non-ruptured abdominal aortic aneurysm.

Late survival rates were compared and analysed for 1070 patients undergoing repair of ruptured infrarenal abdominal aortic aneurysm (RAAA, n = 364, mean age 70.0 years, male:female ratio 5.6:1) and non-ruptured abdominal aortic aneurysm (AAA, n = 706, mean age 66.6 years, male: female ratio 5.4:1) between January 1970 and July 1992 at the Department of Thoracic and Cardiovascular Surgery of Helsinki University Central Hospital, Finland. There was a statistically significant difference in survival rates between the RAAA and AAA groups during the first three months after repair of abdominal aortic aneurysm. Operative mortality rates were 7.4% for electively repaired abdominal aortic aneurysms and 48.7% for ruptured abdominal aortic aneurysms. For 3-month postoperative survivors there existed no statistically significant difference in late survival rates, nor did these rates differ from those of an age- and sex-matched population. Five-year survival rates for 3-month postoperative survivors were 60% in the RAAA group and 67% in the AAA group. Median survival time was 5.7 years and 7.5 years, respectively. Coronary artery disease, hypertension, chronic obstructive pulmonary disease and renal insufficiency statistically significantly reduced late survival rates after 3 months post-surgery for non-ruptured abdominal aortic aneurysm, whereas these risk factors did not alter late prognosis after successful repair of ruptured abdominal aortic aneurysm. Cerebrovascular disease reduced late survival rates both in AAA (median survival time 6.3 years) and RAAA group (median survival time 4.9 years). Of late deaths 41% were caused by coronary artery disease in the AAA group and 38% in the RAAA group.(ABSTRACT TRUNCATED AT 250 WORDS)

Tracheobronchomalacia in an adult with metaphyseal chondrodysplasia.

Anaesthetic complications such as obstruction of airways by submucosal cartilage-bone protuberances, immobility of the neck or instability of the atlanto-axial joint have been described earlier in paediatric patients with congenital osteochondral disorders. This report concerns a case in which tracheal collapse due to tracheobronchial malacia in an adult patient with metaphyseal chondrodysplasia evidently caused severe ventilatory difficulties in the induction of anaesthesia. The management of the patient on three subsequent occasions is described. During the first operation, support of the upper respiratory tract was performed. For this procedure, awake tracheal intubation with local anaesthesia applied to the larynx, vocal cords and trachea was used. After surgical correction of the bronchus of the right upper lobe and the stem bronchus, subsequent anaesthesias for surgical treatment of scoliosis could be conducted safely. The possibility of co-existing tracheobronchial malacia in patients with osteochondrodysplasias should be considered and tracheal intubation under local anaesthesia is recommended.

Granulocyte colony-stimulating factor therapy and systemic inflammation in critically ill patients.

OBJECTIVE AND DESIGN: The effect of the granulocyte colony-stimulating factor filgrastim on systemic inflammation was investigated in a prospective, randomized, placebo-controlled, double-blind study in critically ill patients. SUBJECTS: 59 critically ill patients were recruited within 48 h of intubation due to ventilatory insufficiency. TREATMENT: Subcutaneous dosage of placebo or 300 microg filgrastim once daily. METHODS: Serum samples were collected at study entry, and 1 and 3 days after the start (Day1 and Day3, respectively). Levels of soluble E-selectin (sE-selectin) and interleukin (IL)-10 were determined by ELISA, and those of IL-6, and soluble IL-2 receptor (sIL-2R) by Immulite chemiluminescence immunoassay. RESULTS: The median sE-selectin level decreased by day 3 significantly in the control group but not in the filgrastim group. The difference in the change between the study groups was significant (p = 0.049). IL-10 levels decreased significantly in the filgrastim group, tended to decrease in controls (p = 0.052), and the difference in the change tended to be significant (p = 0.058). IL-6 levels decreased in both groups comparably. sIL-2R levels were elevated and stable. CONCLUSIONS: Filgrastim prolongs endothelial activation and possibly inhibits development of immune suppression mediated by IL-10.

The effect of different modes of artificial ventilation and of some prophylactic means on the incidence of postoperative deep vein thrombosis.

The occurrence of postoperative deep vein thrombosis (DVT) was studied in 209 elective surgery patients aged 40 years or over. Most of the operations were cholecystectomies or other major abdominal operations. In the detection of thrombosis the 125I-fibrinogen method was used. Of 209 patients, 51 (24.4%) developed postoperative DVT and of these 10 developed thrombosis in both legs. In 36% of the DVT cases the process started during the operation or on the first postoperative day. Clinical signs of DVT did not agree with the 125I-fibrinogen test very well, whereas the correlation of the 125I-fibrinogen test with phlebography was good: of the 17 patients with a postive 125I-fibrinogen test in whom a phlebography was done, the latter method revealed thrombosis in 14 patients (82.4%). The main purpose of the study was to determine whether the mode of mechanical ventilation used during anaesthesia has any effect on the occurrence of postoperative DVT. One hundred and nine patients received intermittent positive pressure ventilation (IPPV), whereas in 100 patients the intrathoracic pressure was decreased by applying intermittent positive-negative (--5 cmH2O) pressure ventilation (IPNPV). Ventilation was standardized in both groups by keeping the end-tidal CO2% at a constant level. Inspired oxygen concentration was the same in every patient. The decreasing effect of IPNPV on intrathoracic pressure and central venous pressure as compared with IPPV was confirmed in preliminary studies.

Effects of heparin on serum ionized calcium and haemodynamics during coronary artery surgery.

Heparin has been reported to possess hypotensive and vasodilating properties when given in 3 mg/kg bolus injection. The haemodynamic effect has been thought to be related to an acute lowering of serum ionized calcium levels. The effects of heparin on haemodynamics and ionized calcium were compared with saline in 30 patients during coronary artery surgery before the commencement of cardiopulmonary bypass. There were no significant differences in haemodynamics or ionized calcium levels between heparin or saline during the observation period of 10 min. The results are at variance with some previous findings, and suggest that the hypotensive reaction occasionally seen after heparin is given is not mediated by the reduction of serum ionized calcium.

Bradycardia during antagonism of pancuronium-induced neuromuscular block.

Heart rate was compared in matched patients during antagonism of neuromuscular block induced by tubocurarine, pancuronium or alcuronium with neostigmine 0.03 mg kg-1 preceded by atropine 0.015 mg kg-1. The frequency of bradycardia was greater during antagonism of pancuronium compared with alcuronium. Although there was a difference between the group receiving pancuronium and that receiving tubocurarine, it was not statistically significant. The decrease in heart rate was more rapid and profound in the pancuronium group; seven of the 15 patients who received pancuronium required an additional dose of atropine as compared with only one patient who received tubocurarine. However, the difference in heart rate between those who received pancuronium and those receiving tubocurarine was short-lasting, whereas the heart rate of those who received alcuronium was higher than that in the other groups during the entire 60-min period of observation. The findings with pancuronium may be a result of its inhibitory effect on serum cholinesterase.

Catheter-induced pulmonary artery perforation associated with an unusual wedge pressure tracing.

Pulmonary artery perforation is one of the most serious complications of pulmonary artery catheterization. We report a case of a pulmonary artery perforation which occurred during the placement of a Swan-Ganz catheter in a patient with mitral valve regurgitation. During the insertion of the catheter a deep negative pressure wave was seen in the pulmonary artery tracing, with concomitant haemoptysis. The postoperative chest radiograph revealed an infiltration distal to the tip of the pulmonary artery catheter. This type of unusual capillary wedge pressure tracing during pulmonary artery catheterization has not previously been reported.

Thiopentone reduces the haemodynamic response to induction of high-dose fentanyl-pancuronium anaesthesia in coronary artery surgical patients.

Thirty-three coronary artery bypass graft patients anaesthetized with high-dose fentanyl (50 micrograms/kg)-pancuronium-oxygen were divided into one control group receiving additional saline and two groups receiving additional 1 mg/kg or 2.5 mg/kg of thiopentone before laryngoscopy and intubation. During laryngoscopy and intubation, systemic arterial pressures, heart rate and rate-pressure-product remained at considerably elevated levels caused by pancuronium in the control group. Both doses of thiopentone reduced these haemodynamic values close to their initial levels. Cardiac index and left ventricular stroke work index were significantly decreased, especially by the higher thiopentone dose, as compared with the control group. However, there were no statistical differences between the haemodynamic changes produced by the two doses of thiopentone. Sedative or hypnotic supplementation of high-dose fentanyl anaesthesia seems to be necessary if pancuronium is used as a muscle relaxant. A small increment of thiopentone, 1 mg/kg, was enough to return haemodynamic parameters almost to their initial levels, whereas the effect of 2.5 mg/kg of thiopentone was unnecessarily strong.

Advantages of glycopyrrolate over atropine during reversal of pancuronium block.

Atropine 0.015 mg kg-1 and glycopyrrolate 0.0075 mg kg-1 were compared as antimuscarinic agents during reversal of pancuronium block with neostigmine 0.03 mg kg-1 in 30 patients anaesthetized with thiopental - N2O- fentanyl and undergoing minor surgery. The decrease of heart rate was more pronounced in patients who received atropine-neostigmine. The mean of the lowest heart rate was 44.3 beats min-1 in the atropine group compared with 54.3 beats min-1 in the glycopyrrolate group. Five patients with atropine-neostigmine developed a transient nodal rhythm as compared with two of those receiving glycopyrrole-neostigmine (non-significant difference). Recovery from anaesthesia, as assessed by the awakening after the discontinuation of N2O administration, was more rapid in patients given glycopyrrolate. In conclusion, glycopyrrolate seems to have advantages over atropine when used during reversal of pancuronium block with neostigmine.