[Butyrylcholinesterase deficiency: how to analyse the cholinesterase activity in small children?]. / Déficit génétique de la butyrylcholinestérase: comment interpréter l'activité cholinestérasique chez le jeune enfant ?

We report the case of a prolonged neuromuscular blockade in an 18-month-old age girl following administration of a usual dose of succinylcholine. The diagnosis was highly suggested by the clinical history while cholinesterase activity was included in adult normal values but below values of a personal series of 41 small children. The familial analysis of dibucaine and fluoride number confirmed the hypothesis of an atypical variant (AA phenotype). The cholinesterase activity is higher in small children than in adult and has to be analysed according to the age.

Perilymph detection by beta 2-transferrin immunoblotting assay. Application to the diagnosis of perilymphatic fistulae.

beta 2-Transferrin, the asialotransferrin, is found in cerebrospinal fluid (CSF) and inner ear perilymph, but is absent from serum and other body fluids or secretions except the aqueous humor. The detection of this asialo-fraction of the transferrin in ear fluid microsamples with an immunoblotting technique is of great interest when a perilymphatic fistula (PLF) is suspected. beta 2-Transferrin was detected on microsamples collected by syringe or on micro-collagen sponges from 30 patients undergoing ear surgery. The problem is reviewed, the technique and sample preparation are explained and the results discussed. beta 2-Transferrin detection in the ear fluid allows the identification of perilymph, except in the CSF oto- or rhinorrheal context, and is proposed as a promising test to confirm perilymphatic fistula.

[Automatic assay of plasma and urinary calcium, inorganic phosphate, creatinine and uric acid. Comparison between continuous flow analysis on a single microsample and low speed centrifugation]. / Dosage automatique du calcium, du phosphate minéral, de la créatinine et de l'acide urique plasmatiques et urinaires. Comparaison des méthodes en flux continu sur microprélèvement unique et par centrifugation à petite vitesse

A device is described allowing the association on a single microsample of assay by means of continuous flow analysis. The preparation of standards, the evaluation citeria of the method are presented. The preparation of standards, the evaluation criteria of the method are presented. The results are compared to those obtained by low speed centrifugation, i.e. by means of quite different analytical prinicples -- both dynamics and chemicals. These results are discussed : they allow to observe, as a whole, an excellent concordance.

[Automated kinetic assay of plasmatic L-asparaginase activity undergoing therapy for acute lymphoblastic leukemia]. / Dosage automatique en cinétique de l'activité L-asparaginase plasmatique en suivi thérapeutique des leucémies aiguës lymphoblastiques.

The L-asparaginase is a critical drug for the treatment of acute lymphoblastic leukaemia, that achieves blood L-asparagin depletion. However, such a therapy is associated with a high rate of negative side effects, particularly antibody synthesis against L-asparaginase. This therefore decreases therapy efficiency requiring the monitoring of L-asparaginase activity since L-asparagin determination is not easy. We compared here the results obtained with an automated kinetic enzymatic method to those obtained with the most commonly used Nessler reagent method. The correlation coefficient, r = 0,992, obtained was very good, and the allometric regression line was y = 1,038x - 0,37 microkat/L. We also showed that the specificity and the precision were better with the enzymatic method than the Nessler one. Moreover, the enzymatic method was easier and required less time to perform. Finally, the method appears able to perform real time monitoring of the therapy.

[Decrease in plasmatic cholinesterase activity in severe bacterial infections: comparison with the decrease observed in severe liver cirrhosis]. / Diminution de l'activité de la cholinestérase plasmatique au cours des infections bactériennes sévères: comparaison avec celle observée en cas de cirrhose grave.

As the influence of sepsis on plasma cholinesterase activity is not clearly established, plasma cholinesterase activity was measured in 30 healthy surgical orthopaedic patients, 11 patients with severe postoperative infections and 18 patients with patent cirrhosis (Child-Pugh C). Plasma cholinesterase activities were significantly decreased (P < 0.001) in patients with postoperative infections (1,706 +/- 535 Ul.L-1) and in those with patent cirrhosis (1,318 +/- 538 Ul.L-1) in comparaison to healthy surgical patients (4,716 +/- 1,232 Ul.L-1). The decrease in patients with postoperative infections and in those with patent cirrhosis was similar. It remains to be assessed whether the activity of anaesthetic agents biotransformed by plasma cholinesterase is modified during severe infections.

[Prolonged apnea after suxamethonium administration during staphylococcal toxic shock]. / Apnée prolongée après administration suxaméthonium au cours d'un choc toxique staphylococcique.

A toxic shock syndrome occurred after a femoral nail removal requiring revision surgery. After administration of suxamethonium (1 mg.kg-1), an apnoea prolonged over 45 minutes was observed. The trachea was extubated 105 minutes after suxamethonium administration. For the nail removal, two days before, the anaesthetic had been given by the same anaesthesiologist, with a similar protocol. Apnoea extended over 20 minutes. The day of the revision surgery, plasma cholinesterase activity was 410 UI.L-1 and reached 910 UI.L-1, 9 months later. Dibucaine number was 20 and fluorure number 17. The apnoea was in relation with a genetic plasma cholinesterase deficiency increased by the toxic shock syndrome. Shock and hepatic insufficiency were suspected to contribute to the decrease in plasma cholinesterase. Suxamethonium should be avoided in case of toxic shock syndrome.

[Perilymph fistula: diagnosis by detection of perilymph in the middle ear by beta-2 transferrin immunofixation]. / Fistules périlymphatiques: diagnostic par la détection de la périlymphe dans l'oreille moyenne par immunofixation de la beta 2 transferrine.

beta 2-transferrin is a specific protein found in the cerebrospinal fluid and in the perilymph. Detecting beta 2-transferrin in the middle ear is of great interest when a fistula of the perilymph is suspected. This protein can be detected on microsamples with an immunofixation technique. We searched for beta 2-transferrin in pure perilymph, cerebrospinal fluid and serum in 8 patients operated by translabyrinthine approach for acoustic tumor removal. Search for beta 2-transferrin was performed in liquid from the inner ear in 3 labyrinthectomies. Samples were taken on collagen sponges or with micro syringes. beta 2-transferrin was detected in the perilymph of patients operated for neurinoma and in 2 of the 3 labyrinthectomies. This protein was found in only one of the patients for which the diagnosis of perilymphatic fistula had been retained. Detection of beta 2-transferrin in the middle ear can be proposed as a specific diagnostic test for perilymphatic fistula when the clinical situation does not suggest a fistula involving cerebrospinal fluid.

[The value of beta-2 transferrin analysis and MR cisternography for the diagnosis of cerebrospinal fluid fistulas]. / Intérêt de la recherche de beta2 transferrine et de la MR cisternographie pour le diagnostic des fistules de liquide céphalorachidien.

The diagnosis of cerebrospinal fluid (CSF) fistula may require invasive techniques. Detection of CSF and perilymph-specific beta 2 transferrin and MR cisternography which greatly enhances the CSF signal are sensitive and noninvasive techniques which allowed a precise diagnosis in seven patients with suspected CSF fistula. We review the different diagnostic techniques used for CSF fistula, beta 2 transferrin analysis and MR cisternography appear to provide accurate and noninvasive methods for investigating CSF fistulae. They should replace invasive techniques such as CT cisternography.

Cerebral ischemia complicating intracranial aneurysm: a warning sign of imminent rupture?

BACKGROUND AND PURPOSE: Patients harboring nongiant cerebral aneurysms may rarely present with an ischemic infarct distal to the aneurysm. The aim of this case series was to report clinical and radiologic characteristics of these patients, their management, and outcome. MATERIALS AND METHODS: We undertook a single-center retrospective analysis of consecutive patients admitted during an 8-year period with an acute ischemic stroke revealing an unruptured nongiant (<25 mm) sacciform intracranial aneurysm. Clinical, radiologic, therapeutic, and follow-up data were analyzed. RESULTS: Nine patients were included. The mean size of aneurysms was 9.6 ± 6 mm, and 5 were partially or totally thrombosed. Two patients had a fatal SAH within 3 days after stroke-symptom onset, whereas asymptomatic meningeal bleeding was diagnosed or suspected in 2 others. Most of the patients with unthrombosed aneurysms were successfully treated by endovascular coiling in the acute phase. Thrombosed aneurysms were usually treated with antithrombotics, and most recanalized secondarily, requiring endovascular treatment or surgical obliteration. No recurrence of an ischemic event or SAH was observed during the 31 ± 12 months of follow-up (from 4 to 53 months). CONCLUSIONS: In this single-center series, the frequency of early SAH in patients with ischemic stroke distal to an unruptured intracranial aneurysm was high. Acute management should be undertaken with care regarding antithrombotic use, and early endovascular coiling should be considered.

[Increased serum IgA levels in alcoholic polyneuropathy. Pathogenetic discussion]. / Elévation du taux des IgA sériques au cours de la polyneuropathie éthylique. Discussion pathogénique.

Increased serum IgA in patients with direct toxic alcoholic peripheral neuropathy was studied prospectively in 33 patients with an obvious clinical polyneuropathy confirmed by electrophysiological investigations. The average concentration of IgA in this group (450 mg/100 ml) was significantly higher than in a group of 24 matched alcoholic control patients without peripheral neuropathy (306 mg/100 ml), p less than 0.0001. The authors discuss possible direct and indirect modifications of the humoral immune response due to the peripheral neuropathy.

Does sevoflurane inhibit serum cholinesterase in children?

Serum cholinesterase activity was measured at induction, and following anaesthesia in 41 children aged between 4 and 30 months. The median exposure to sevoflurane was 273%.min. The results did not demonstrate any significant difference in cholinesterase activity, when expressed by gram of serum proteins following inhalation anaesthesia using sevoflurane or intravenous anaesthesia using propofol. The values (SD) obtained were 175 (42) UI.g(-1) before anaesthesia and 177 (43) UI.g(-1) following anaesthesia. The only change in cholinesterase activity detected was related to heamodilution. We conclude that plasma fluoride concentration following sevoflurane administration [13.8 (4.2) microm x l(-1)] is too low to exert an inhibiting effect on in vivo cholinesterase activity and that the previously reported decrease in mivacurium requirements during sevoflurane anaesthesia is unlikely to be due to inhibition by fluoride ions.