ABSTRACT
Laboratory confirmation of a clinical suspicion of malignant hyperthermia (MH) susceptibility by the standard in vitro contracture test remains inconclusive in patients reacting only to caffeine or halothane (called 'Equivocal') or in patients with concomitant neuromuscular disease. The detection of point mutations in the ryanodine receptor gene potentially provides additional information in these cases. The diagnostic value of the Gly341 Arg mutation in a patient reacting in vitro only to caffeine was reported previously by Quane et al. (1994). The present report describes a patient with motor neuron disease carrying the Gly341 Arg mutation, expanding the diagnostic value of this mutation to the group of patients with neuromuscular diseases.
Subject(s)
Malignant Hyperthermia/complications , Malignant Hyperthermia/genetics , Motor Neuron Disease/complications , Motor Neuron Disease/genetics , Adolescent , Calcium Channels/genetics , Calmodulin-Binding Proteins/genetics , Female , Humans , Male , Malignant Hyperthermia/diagnosis , Motor Neuron Disease/diagnosis , Muscle Proteins/genetics , Pedigree , Point Mutation/genetics , Ryanodine Receptor Calcium Release ChannelABSTRACT
A four year old boy suffered from a prolonged cardiac arrest during anesthesia. Laboratory examination subsequently demonstrated extensive rhabdomyolysis. The possibility of an underlying muscle disease in this family was investigated by muscle biopsy, histological examination and in-vitro contracture testing. The latter test result was consistent with malignant hyperthermia susceptibility. This case history illustrates the differential diagnosis of anesthesia induced rhabdomyolysis.
Subject(s)
Anesthesia, Inhalation/adverse effects , Malignant Hyperthermia/diagnosis , Rhabdomyolysis/etiology , Adult , Brain Edema/etiology , Child, Preschool , Disease Susceptibility , Female , Humans , MaleABSTRACT
Two patients are described with severe coagulation disturbances, in one instance leading to extensive skin bleeding, secondary to the use of cefamandole. This cefalosporin antibiotic carries the same N-methylthiotetrazole side chain as moxalactam. Pathogenetic mechanisms leading to hypoprothrombinemia, its prevention and treatment are discussed.
Subject(s)
Cefamandole/adverse effects , Hemorrhagic Disorders/chemically induced , Hypoprothrombinemias/chemically induced , Aged , Amputation, Surgical , Cefamandole/therapeutic use , Diabetic Angiopathies/surgery , Female , Humans , Kidney Transplantation , Male , Middle Aged , Pneumonia/drug therapy , Postoperative Complications/drug therapyABSTRACT
The arterial oxygen and carbon dioxide tensions, pulmonary and systemic haemodynamics and pulmonary shunting and mechanics were measured during the first 30 min after intravenous labetalol administration. Thirty patients, recovering in the intensive care unit after neurosurgical interventions were randomly divided in 2 groups of 15 patients, receiving either labetalol or placebo. In the labetalol treated group the arterial oxygen tension decreased from 553.6 +/- 16.8 to 529.3 +/- 19.8 mmHg 5 min after the injection of labetalol. A concomitant increase in arterial carbon dioxide tension from 40.1 +/- 1.1 to 45.5 +/- 1.3 mmHg was noticed. Pulmonary vascular resistance decreased from 159.6 +/- 14.7 to 116.7 +/- 11.7 dynes.sec.cm-5 and pulmonary shunting increased from 4.8% +/- 1.4% to 8.1% +/- 2.4% 5 min following injection. All these changes were statistically significant for p less than 0.01. After 30 min all values had returned to their initial level. No changes were registered in the control group. As airway resistance appeared not to be affected by the labetalol administration it may be concluded that the observed changes in blood gas data are most likely due to a transient decrease of the pulmonary vascular resistance with a concomitant increase in pulmonary shunting.