Appearance of esophageal peristalsis in treated idiopathic achalasia

The authors studied 22 patients with severe dysphagia, and follow-up studies showed the appearance of some esophageal peristalsis for the first time in three of these patients after Heller myotomy. Comparison of pre- and postoperative clinical, radiological, and manometric data of these three subjects and the data of the other 19 patients showed no statistically significant differences between the two groups of patients either before or after cardiomyotomy except for the unusual appearance of esophageal peristalsis after surgery. According to these results it is not possible to predict which patients will develop peristalsis after cardiomyotomy. The different motor pattern observed after surgery might suggest the existence of a different pathogenesis underlying the esophageal motor disorders in these two groups of patients.     

Myoclonic encephalopathy in the CDKL5 gene mutation.

OBJECTIVE: Epilepsy with mutation of the CDKL5 gene causes early seizures and is a variant of Rett syndrome (MIM (312750), which is reported typically as infantile spasms. The purpose of this study was to analyze the epileptic histories and EEGs of patients with the CDKL5 mutation. METHODS: We reviewed the epilepsy histories and electroclinical analyses of three girls aged 9.5, 7.4, and 9.4 years, each with a mutation of the CDKL5 gene. RESULTS: We revealed the presence of an encephalopathy that started by 1.5 months of age. At first, seizures involved tonic spasms or complex partial seizures, and were complicated by the later appearance of complex partial, tonic, and unexpectedly, myoclonic seizures. This form of epilepsy was drug resistant. Routine and prolonged video EEGs both displayed a homogeneous electroclinical pattern consisting of (a) unique background with diffuse high voltage sharp waves of 6-7 Hz, and absence of the typical rhythmic frontal-central theta activity present in Rett syndrome; (b) unique awake and sleep background, with diffuse, high voltage, continuous sharp waves with multifocal and diffuse spikes; (c) rhythmic, diffuse, 15 Hz activity accompanied clinically by tonic seizures; (d) intercritical pattern with pseudoperiodic, diffuse, sharp waves or pseudoperiodic, diffuse spike and polyspike or wave discharges; and (e) diffuse, spike, polyspike and wave discharges accompanied by massive or focal myoclonias or both. CONCLUSIONS: Patients with the CDKL5 mutation have an early onset, epileptic encephalopathy in infancy that evolves into myoclonic seizures in childhood with a unique EEG pattern. SIGNIFICANCE: Recognizing this type of encephalopathy could be useful in prompting clinicians to proceed further with their diagnostic work in patients not fitting the criteria of classical Rett syndrome.     

Central nervous system and cardiac manifestations of hydrochlorothiazide overdosage; treatment with hemodialysis

A patient with end-stage renal failure inadvertently received high-dose hydrochlorothiazide as treatment for hypertension, resulting in CNS and cardiac toxicity. These toxic manifestations were successfully treated with hemodialysis. A hydrochlorothiazide dialysance of 62.5 mL/min was demonstrated. The possibility of hydrochlorothiazide toxicity should be considered in any patient with renal insufficiency who exhibits unexplained arrhythmias or symptoms related to the CNS.     

Amiodarone-induced experimental acute neuropathy in rats.

Amiodarone was injected endoneurially at increasing doses into the exposed tibial nerve of rats to study its electrophysiologic and pathologic effects on peripheral nerve fibers. Forty-five male Wistar rats were used, and each of the following concentrations was injected into 15 nerves: 25 micrograms/mL, 50 micrograms/mL, and 100 micrograms/mL. Microinjection of a 25 micrograms/mL concentration of amiodarone resulted in a subacute, incomplete conduction block evident at day 3 postinjection. This conduction block remained stable until day 10 and recovery was complete at day 35. Microinjection of a 50 micrograms/mL concentration of amiodarone produced a faster evolving conduction block, and significant axon degeneration (approximately 40% of fibers). Injection of a 100 micrograms/mL concentration resulted in severe acute motor axon degeneration followed by complete but delayed regeneration. Results of morphological studies closely correlated with electrophysiological findings. Amiodarone thus seems to have a direct toxic effect on axons at high concentrations in the peripheral nerve, and we suggest that different pathological changes described in human amiodarone neuropathy could be related to different concentrations of the drug in the nerve, perhaps due to variability of blood-nerve barrier efficacy.     

Intravenous paracetamol as adjunctive treatment for postoperative pain after cardiac surgery: a double blind randomized controlled trial.

BACKGROUND: Nonsteroidal anti-inflammatory drugs and opioids are routinely used after cardiac surgery in order to mitigate postoperative pain; however, these drugs are burdened by side effects. Tramadol and paracetamol are believed to be lacking in such side effects. The aim of this study was to examine the efficacy of intravenous paracetamol as an adjunctive analgesic to a tramadol-based background analgesia after cardiac surgery. METHODS: A total of 113 patients participated in this single center, placebo-controlled, double-blind, randomized trial. Fifty-six patients were randomized to receive paracetamol and 57 to placebo. Intravenous study drug (1 g) was administered 15 min before the end of surgery and every 6h for 72 h. Standard analgesia (tramadol) and anti-emetic prophylactic regimen (ondansetron) were available to both patient groups. Postoperative pain was evaluated by visual analog scale, and it was measured at rest and during a deep breath. A rescue dose of 2-5 mg of intravenous morphine was administered whenever the VAS score was greater than 3. RESULTS: Baseline characteristics were equivalent between the two groups. At 12, 18, 24 h after the end of operation, patients who received paracetamol had significantly less pain at rest (p=0.0041, 0.0039, 0.0044, respectively); after this time the two groups did not differ. During a deep breath the difference was significant only at 12 h (p=0.0040). Paracetamol group required less cumulative morphine than placebo group (48 mg vs 97 mg) even if the difference did not reach statistical significance (p=0.274). CONCLUSIONS: In patients undergoing cardiac surgery, intravenous paracetamol in combination with tramadol provides effective pain control.     

Fragrance material review on linalyl isobutyrate.

A toxicologic and dermatologic review of linalyl isobutyrate when used as a fragrance ingredient is presented.     

Six cases of travel-associated legionnaires' disease in Ischia involving four countries

Summary The detection of travel-associated legionellosis can be extremely difficult; hence, an extensive case investigation is recommended in pneumonia-striken travellers and tourists, who are particularly at risk of acquiring the disease. On the Island of Ischia (Isola d'Ischia, Naples, Italy) a total of six cases of Legionnaires' disease occurred from 1986 to 1990. All patients (one man and two women from Germany, one Austrian woman, one Swiss man, and one Italian woman) had taken thermal baths and stayed in local hotels; they all experienced severe pneumonia, and three of them died. These cases were associated with hotels, and the hot-water supply was presumed to have transmitted the infection. Remedial procedures were applied to the hot-water plumbing of the hotels according to the WHO recommendations and were proved to be effective. The occurrences described in this paper stress the importance of rapid and accurate reporting of diagnosed cases to the country where the infection was probably acquired, in order to ensure early detection of endemic foci and emerging clusters of legionellosis.

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Sechs Fälle von reiseassoziierter Legionärskrankheit in Ischia unter Beteiligung von vier Ländern

Zusammenfassung Der Nachweis reiseassoziierter Legionärskrankheit gestaltet sich häufig schwierig. Eine Überwachung von Touristen und Reisenden, die ein erhöhtes Legionellose-Risiko haben, ist daher zu empfehlen. Zwischen 1986 und 1990 traten auf der Insel Ischia (Neapel, Italien) insgesamt sechs Fälle von Legionärskrankheit auf. Alle Patienten (ein Mann und zwei Frauen aus Deutschland, eine Österreicherin, ein Schweizer und eine Italienerin), die in Hotels auf der Insel wohnten, hatten Thermalbäder besucht. Sie erkrankten an schweren Pneumonien, wobei drei Todesfälle auftraten. Die Ansteckungsquelle konnte mit Hotels in Verbindung gebracht werden, wobei die Übertragung der Infektion über die Warmwasserleitungsnetze als gesichert anzunehmen war. Desinfektionsmaßnahmen in den betreffenden Hotels, die nach den Empfehlungen der WHO ausgeführt wurden, erwiesen sich als wirksam. Das genannte Infektionsgeschehen weist auf die Notwendigkeit hin, Fälle von Legionärskrankheit so rasch wie möglich dem Ursprungsland unter Angabe der vermuteten Infektionsquelle zu melden, um sicherzustellen, daß eine Früherkennung endemischer Herde bzw. assoziierter Legionellosen bekannt gemacht wird.

     

Intestinal permeability after single dose gluten challenge in coeliac disease.

The changes of intestinal permeability before and after a gluten load were studied. The study group comprised 27 patients with coeliac disease (mean age 12.3 years) and 19 healthy controls matched by sex and age. Intestinal permeability was studied by measuring the urinary excretion of two sugars, lactulose and L-rhamnose, before and six hours after the ingestion of five palatable biscuits made with 50 g of gluten powder. The patients with coeliac disease had been on a gluten free diet during the previous two years. After the gluten load lactulose and L-rhamnose urinary excretion changed significantly in patients, and a significant increase in the lactulose: L-rhamnose ratio was also observed. No significant changes were observed in the controls. In view of the modification of the three biopsies diagnostic protocol made by the European Society for Paediatric Gastroenterology and Nutrition, permeability tests associated with single gluten challenges may be an added contribution to the accuracy of the diagnosis in childhood.