PubMed HealthSearch

Biomedical subjects

P Sporn

Publications and source records attributed to P Sporn.

At least 19 recordsLinked to original sources

Muscle biopsy for diagnosis of malignant hyperthermia susceptibility in two patients with severe exercise-induced myolysis.

Muscle biopsy and in vitro contracture tests for diagnosis of susceptibility to malignant hyperthermia (MH) were performed in two patients who had developed fever and severe myolysis during exercise. MH susceptibility was confirmed in one patient, but in the other, exercise-induced heat stroke proved to be the correct diagnosis. Clinical presentation and epidemiology of exercise-induced MH and its relation to the heat stroke syndrome are discussed.

Adult

Serum vasopressin (AVP) levels in polyuric brain-dead organ donors.

Hydromineral metabolism and serum arginine-vasopressin (AVP) levels were investigated in 11 patients who sustained brain death. They showed various degrees of polyuria with low osmolality and low fractional sodium excretion. Urine osmolality was always below that of serum, and AVP levels were between 1.3 and 50.0 pg/ml vs 0.7-8.0 pg/ml in ten normal subjects. Thus central diabetes insipidus was excluded. A renal mechanism inducing water diuresis has to be assumed. The type of renal lesion, however, remains unclear.

Adolescent

Prediction of malignant hyperthermia susceptibility: statistical evaluation of clinical signs.

The anaesthetic records of 61 patients who had experienced adverse reactions thought to be malignant hyperthermia (MH) were reviewed retrospectively to evaluate the diagnostic importance of clinical symptoms. Using the in vitro contracture test, 38 (62%) patients were identified as MH susceptible (MHS), the remainder showing normal test results (MHN). Generalized rigidity, ventricular arrhythmias, cyanosis and postoperative myoglobinuria were observed significantly more often in MHS patients. Median values of body temperature and creatine kinase serum concentrations were significantly greater in the MHS group. Masseter spasm and sinus tachycardia were as common in MHS as in MHN individuals. Statistical models using generalized rigidity, ventricular arrhythmias, cyanosis and fever exceeding 38 degrees C for prediction of MH showed a maximum sensitivity and specificity of 78% and thus are not acceptable for clinical use. For definitive diagnosis of MH, the well established in vitro contracture test remains essential.

Adolescent

Anaesthesia in malignant hyperthermia-susceptible patients without dantrolene prophylaxis: a report of 30 cases.

The intra- and postoperative course of 30 anaesthetics in 24 MH-carriers verified by in vitro contracture tests is reported. None of the patients received dantrolene prophylactically and only agents known to be nontriggers were used for anaesthesia. Neither MH-related changes in perioperative heart rates, body temperatures, and CK levels nor any other symptoms of MH were observed. We conclude that the prophylactic use of dantrolene is not necessary in patients suspected to be prone to MH if triggering agents are avoided and the patients are closely monitored.

Adolescent

Anesthesia in malignant hyperthermia susceptible patients.

The intra- and postoperative course of 30 general and 3 regional anesthetics in 27 MH-carriers verified by in vitro contracture tests is reported. None of the patients received dantrolene prophylactically. Disposable tubings were used for ventilation, vaporizers and soda lime were removed. ECG, esophageal temperature, blood pressure, oxygen saturation, and end tidal pCO2 were monitored. Minor tranquilizers were offered for premedication. Fentanyl, thiopentone, nitrous oxide, non depolarizing relaxants, neuromuscular antagonists and naloxone were used. In three patients, surgery was performed during epidural or spinal anesthesia with the use of amide local anesthetics. Neither MH-related changes in perioperative heart rates, body temperatures, and CK levels nor any other symptoms of MH were observed in any patient. The anesthetic techniques used seem to be safe and reliable; the anesthetic management of known MHS patients is discussed in detail.

Adult

[Noradrenaline in the "high output-low resistance" state of patients with abdominal sepsis].

We investigated the impact of norepinephrine administration on hemodynamics, oxygen metabolism and renal function in patients in severe septic shock. PATIENTS AND METHODS. Twenty-six patients with extremely low resistance who were between 24 and 87 years of age were included in the study. In 7 patients, acute necrotizing pancreatitis and superinfection was diagnosed; 19 patients suffered from diffuse peritonitis. The entrance criteria for the study were: a mean arterial pressure (MAP) of below 60 torr or, in chronic hypertensive patients, a decrease in systolic pressure of more than 50 torr compared to previous values, despite volume optimization, and dopamine greater than 20 micrograms/kg per min and cumulative doses of dopamine/dobutamine greater than 30 micrograms/kg per min, respectively. Cases with tachycardia greater than 140/min were also included in the study even when the inotropic medication dose was lower. After registration of baseline values, dopamine was reduced to 2.5 micrograms/kg per min, and norepinephrine was administered starting at a dose rate of 0.05 micrograms/kg per min, until a MAP of greater than 60 torr could be maintained. RESULTS. Of the 26 patients investigated, 16 survived; 10 patients with persisting sepsis died due to multiple organ failure (mortality: 38.5%). During the study period, a norepinephrine dosage ranging between 0.1 and 2 micrograms/kg per min was necessary to stabilize the arterial pressure. The mean dose rate was 0.3 micrograms/kg per min. The mean arterial pressure and systemic vascular resistance index showed a statistically significant increase of 30 and 20%, respectively, just after 1 h and distinctly remained above the initial values in the further course. The cardiac index remained constant or increased slightly. After 24 h a statistically significant increase in stroke volume and a decrease in heart rate could be observed. Creatinine clearance increased significantly from the control value of 73 +/- 48 ml/min to 114 +/- 37 ml/min after 48 h under norepinephrine treatment. O2-delivery and O2-consumption did not change significantly, although they showed a slight tendency to increase. CONCLUSION. When patients are in a septic high output-low resistance condition, particular attention must be paid to maintaining sufficient mean arterial pressures. Our results suggest that this essential goal can be achieved by norepinephrine. The mean arterial pressure and glomerular filtration rate improved markedly, and there was no evidence of bad effects such as an increased afterload on critical parameters like cardiac index, O2-delivery and O2-consumption.

Acute Disease

Effect of large volume replacement with balanced electrolyte solutions on extravascular lung water in surgical patients with sepsis syndrome.

We investigated the effect of large volume replacement with balanced electrolyte solutions on extravascular lung water (EVLW) in 16 adult surgical patients with sepsis syndrome. Patients entered the study within the 24 h period following surgical interventions for acute necrotizing pancreatitis, intra-abdominal abscesses, and/or peritonitis. Sequential measurements (n = 108) were made at intervals of 6-12 h over a 48 h period. There were no significant differences between initial and final values of thermal-dye EVLW (5.0 +/- 1.1 vs. 5.7 +/- 1.1 ml/kg), plasma colloid osmotic pressure (COP, 13.3 +/- 2.5 vs. 13.2 +/- 2.9 mmHg), pulmonary artery wedge pressure (PAWP, 9.2 +/- 3.0 vs. 10.8 +/- 3.0 mmHg), and COP-PAWP gradient (4.0 +/- 3.5 vs. 2.4 +/- 3.9 mmHg). All results expressed as (mean +/- SD). The EVLW did not correlate with plasma COP, PAWP, or COP-PAWP gradient. We conclude that large volume replacement with balanced electrolyte solutions with the secondary decrease in plasma COP and COP-PAWP gradient do not necessarily contribute to a substantial increase in EVLW. This study fails to show any causal relationship between decrease in plasma COP or COP-PAWP gradient and oedema formation in the lung.

Adult

[Preoperative plasma exchange in treatment of plasma-related coagulation disorders before liver transplantation].

PATIENTS AND METHODS: Seventy-two consecutive patients undergoing orthotopic liver transplantation at the Department of Surgery I, University of Vienna Medical School (OLT nos. 1 to 72), were evaluated. Their mean age was 47 years (range: 18-63 years). The indications for liver transplantation are listed in Table 1. All transplant procedures were performed without using a bypass technique. The intraoperative management and surgical procedure have been described elsewhere [7]. Patients were categorized in two groups, each of which was divided in two subgroups. Group I consisted of 18 patients transplanted before the introduction of preoperative plasma exchange. These were retrospectively allocated to two subgroups on basis of their preoperative prothrombin times (PT): A (n = 9): preoperative PT less than 40%; B (n = 9): preoperative PT greater than 40%. The two subgroups of group 2, which contained 54 patients, were compared on a prospective basis: C (n = 32): preoperative PT above 40%; D (n = 22): PT on admission below 40%, preoperative plasma exchange. Comparison of the two subgroups was based on the following parameters: (1) pre-exchange PT (subgroup D); (2) preoperative PT (= PT post-plasma exchange in subgroup D; (3) intraoperative infusion volumes (balanced electrolyte solutions and human albumin to maintain an intravascular colloid osmotic pressure greater than 16 mm Hg); (4) transfusion volumes (whole blood stored for no more than 72 h or packed red cells and fresh plasma, as available; and (5) intraoperative sodium bicarbonate requirements to maintain an arterial pH greater than 7.20. RESULTS: (Table 2) . Prothrombin time (PT): Group 1: Patients in subgroup A had a mean preoperative PT of 34% (range: 15%-40%). This was significantly lower than in subgroup B (74%; 52%-100%; P less than 0.001). Group 2: The pre-exchange mean PT in subgroup D was 27% (12%-39%) vs. 68% in subgroup C (45%-104%), the difference being highly significant (P less than 0.0001). In patients in subgroup D a mean plasma volume of 3638 ml was exchanged by plasmapheresis. This resulted in a significant increase in PT to 55% (Table 3). As a result, the preoperative post-exchange PT in subgroup D was slightly but significantly (P less than 0.005) less than in subgroup C. Transfusion volumes: Group 1: Patients in subgroup A needed significantly more blood units than those in subgroup B (55.3 units [19-110] vs. 18.7 [3-33]).(ABSTRACT TRUNCATED AT 400 WORDS)

Adolescent

[Liver transplantation--anesthesiology aspects].

Based on our experience in 82 liver transplant procedures performed without using a bypass-technique, we report on our current perioperative anaesthetic management. Preoperative plasma exchange with fresh frozen plasma proved very effective in 16 patients with severe coagulation defects, leading to a significant increase in prothrombin time from 27 to 55%. Intra- and postoperative haemodynamic profiles of 48 patients are presented. Baseline measurements revealed a hyperdynamic circulatory state with elevated cardiac index (CI) and heart rate in all patients. During the anhepatic phase CI and mean arterial pressure (MAP) were persistently lowered. Immediately after reperfusion of the graft, reflux of the stagnant venous content induced a significant increase in CI and led to a moderate elevation of pulmonary artery pressure. MAP showed a rather slow recovery, but was within the normal range at the end of surgery. Oxygen consumption index, which was significantly lowered during the anhepatic period, excessively increased after reperfusion of the graft.

Adult

[Evoked potentials in the follow-up and prognosis of patients with craniocerebral trauma].

The aims of this study were to find a reliable way of establishing the prognosis for the final outcome in the first week after head injury, to show the correlation between abnormalities in evoked potentials (EP) and clinical coma score, and finally, to document EP results in patients with the clinical diagnosis of brain death. We examined 46 patients, 23 in different states of coma and 23 with bulbar syndrome (complete absence of cortical and brain stem function). In the group of comatose patients brain stem auditory EP (BAEP) and somatosensory EP (SEP) were recorded in the first 48 h, 3-5 days, 1 week and 4 weeks after the head injury. The depth of coma was scaled with a scoring system devised by the authors and with the Innsbruck coma scale. Outcome was evaluated with the Glasgow outcome scale after 3, 6, and 9 months. BAEP were recorded bilaterally after stimulation with clicks; SEP were recorded from the neck (C2) and the contralateral cortex (C3', C4') after electrical stimulation of the median nerve. Evoked potentials were scored according to a four-point scale from grade 1 (normal) to grade 4 (only component I present in BAEP or absence of cortical responses on both sides in SEP). We found a significant correlation between the mean SEP score of the first week and the Glasgow outcome of the 3rd month, but no significant correlation between the BAEP score of the first week and the Glasgow outcome. There was a significant correlation between SEP (BAEP) scores and the corresponding clinical score.(ABSTRACT TRUNCATED AT 250 WORDS)

Adolescent

Malignant hyperthermia susceptibility confirmed in both parents of probands. A report of three Austrian families.

From 1976 to 1986, 79 cases of suspected malignant hyperthermia (MH) were reported to the authors' institution. A total of 233 muscle biopsies was done in 66 families. The mother and father of the proband were investigated in 44 families; in three of these, both parents of probands gave positive test results. These three families, all from Vorarlberg, a secluded mountain area, represent 4.5% of all our MH-positive families (n = 61) and 7.3% of all MH-positive families where both parents had been tested (n = 41). This observation might be explained by: 1) a much higher incidence of MH in this part of Austria, and 2) a different test policy, as at the authors' institution, if possible, both parents are investigated, even if there is a positive test result in one of them. It is concluded that family investigations of MH susceptibility should always include both parents of a proband.

Adult

[Hemodynamic and metabolic changes during orthotopic liver transplantation].

Hemodynamic and metabolic profiles were obtained on 48 adult patients (mean age 46 years) undergoing orthotopic liver transplantation without using a bypass technique during the anhepatic period. Baseline measurements after induction of anesthesia (A) revealed a high-output circulatory state. During hepatic dissection (B, preclamping control), mean arterial pressure (MAP), mean pulmonary artery pressure (MPAP), pulmonary capillary wedge pressure (PCWP), right atrial pressure (RAP), and elevated cardiac index (CI) were well maintained. Preoperative plasma exchange with fresh frozen plasma in the presence of severe coagulation defects and liberal use of platelet concentrates limited the need for massive blood transfusion, and thus contributed to stable hemodynamics during this stage. Significant cardiovascular changes occurred immediately after clamping of the inferior vena cava and the portal vein (C): there was a marked fall in MAP (-30%), MPAP (-45%), PCWP (-48%), RAP (-40%), and CI (-60%) reflecting the hemodynamic adaptation to the impeded venous return. At the end of the anhepatic period (D), MAP (-21%), MPAP (-38%), PCWP (-39%), RAP (-24%), and CI (-56%) were persistently lowered because no attempts were made to attenuate the clamping response by vigorous volume expansion or infusion of inotropic drugs. The reduction in oxygen availability index (O2AVI) was compensated by enhanced oxygen extraction. Oxygen consumption index (VO2I) fell secondary to the removal of the liver and the decrease in body temperature (BT). Potassium levels and acid-base balance were well controlled; no hypoglycemic episode was observed during the anhepatic period.(ABSTRACT TRUNCATED AT 250 WORDS)

Acid-Base Equilibrium

[Malignant hyperthermia in Austria. III. Anesthesia in susceptible patients].

Anesthesia in patients susceptible to malignant hyperthermia (MH) is generally considered to be very risky, although - with one notable exception - there are no prospective studies about anesthetic management in a large number of such patients. The prophylactic use of dantrolene has been recommended in MH patients, although there is no strong evidence supporting this - despite the fact that dantrolene may have serious side effects. We therefore decided to report the results of our own anesthetic technique for MH patients, as our technique does not include the prophylactic use of dantrolene. From 1981 to 1988, 19 operations on 16 MH-susceptible patients were performed. Patients 1-4 were pediatric survivors of an MH episode, where MH susceptibility was confirmed by muscle biopsy and in vitro contracture tests in at least one parent; patients 5-7 were survivors of an MH crisis, and they later underwent diagnostic muscle biopsies themselves; all other patients (nos. 8-16) were relatives of MH survivors with positive in vitro contracture tests. Diazepam, pentobarbital, pethidine, and chlorprothixene were used for premedication; no prophylactic dantrolene was given. Anesthesia was induced by thiopentone and was continued by nitrous oxide/oxygen, fentanyl, and droperidol; alcuronium, atracurium, and vecuronium were administered as necessary. Pyridostigmine, atropine, and naloxone were used if appropriate. New or disposable tubings were used for ventilation, and the vaporizers were removed from the anesthesia machines. ECG and body temperature were recorded in all patients; blood pressure was monitored invasively if indicated; end tidal CO2 was monitored whenever possible.(ABSTRACT TRUNCATED AT 250 WORDS)

Adult