Gentamicin administration via peritoneal dialysis fluid: the risk of ototoxicity.

In a prospective study on 47 patients, 16 mg of gentamicin per two litres dialysate was administered intraperitoneally at every cycle of intermittent peritoneal dialysis, carried out over the course of several days. Serum gentamicin sampling, pure tone audiometry and caloric tests were performed before and during the treatment. The gentamicin levels reached at the end of the thirtieth cycle were observed to be low. In view of this, the risk of acute ototoxicity was considered to be minimal. This was confirmed by the absence of clinical audiometric or vestibulometric evidence of toxicity.     

Executive cognitive deficits in primary dystonia.

Primary dystonia is a disorder of movement for which no consistent pathophysiology has been identified; in the absence of evidence to the contrary, it is assumed to be cognitively benign. We have studied a clinically heterogeneous group of 14 patients with primary dystonia on a battery of neuropsychological tests. Despite well-preserved speed of information processing, language, spatial, memory and general intellectual skills relative to normal controls, we have identified a constellation of attentional-executive cognitive deficits on the Cambridge Neuropsychological Test Automated Battery (CANTAB). Specifically, patients demonstrated significant difficulties negotiating the extra-dimensional set-shifting phase of the IED task. The implications of these findings for the pathophysiology of primary dystonia are discussed. This is, to the best of our knowledge, the first report of a significant cognitive deficit in patients with primary dystonia.     

Post-operative progress of dystonia patients following globus pallidus internus deep brain stimulation

In the current era of functional surgery for movement disorders, deep brain stimulation (DBS) of the globus pallidus internus (GPi) is emerging as the favoured intervention for patients with dystonia. Here we report our results in 20 patients with medically intractable dystonia treated with GPi stimulation. The series comprised 14 patients with generalized dystonia and six with spasmodic torticollis. Although comparisons were limited by differences in their respective neurological rating scales, chronic DBS clearly benefited both patient groups. Data conveying the rate of change in neurological function following intervention are also presented, demonstrating the gradual but progressive and sustained nature of improvement following stimulation of the GPi in dystonic patients.     

Mechanism of electrolyte and water transport in the first 2 minutes of hypotonic water resorption

1.2 ml of water or 1.2-2.4 ml of 1/2-isotonic saline were injected into the duodenum of the unanaesthetized rat. Continuous measurements of conductivity and sodium concentration in whole blood, the last by means of ion-selective electrodes, and of protein and electrolyte concentration in a filtrate of portal vein blood were made. Whereas conductivity and sodium or electrolyte concentration dropped promptly and steeply, the drop of protein concentration (Cprot) was registered with a delay of 1.9 min. A quantitative comparison of the conductivity and sodium concentration curves, showing strictly synchronous courses, suggested that no hematocrit changes (these would have influenced conductivity) had taken place during the period of constancy of Cprot. In rats having thirsted for 36 h, Cprot did not fall during a period of 5-8 min in which they drank up to 5.5 ml of water, despite a great drop of electrolyte concentration. It is concluded that at the beginning of water absorption all the fluid absorbed is transported via the lymph ducts. A gradient of negative pressure from the interstitial tissues to the lymphatic system in the non-absorbing gut is proposed, an additional electrolyte secretory process being taken into consideration.     

Effort-related axillosubclavian vein thrombosis. A new theory of pathogenesis and a plea for direct surgical intervention

Four cases of effort-related axillosubclavian thrombosis are described. The pathogenesis of this disorder is related to an anatomic constriction of the vein by the clavicle and first rib complex associated with repetitive trauma to the vein and resultant changes in the vein wall itself. Therapy must, therefore, not only address the superimposed thrombosis but must also provide a reliable correction of the constricting mechanism and the resultant lesion in the proximal subclavian vein. Our operative approach resulted in a full return to daily activities and no recurrence or persistence of symptoms in our series. Although early intervention is advisable, patients who present with long-standing venous occlusion should also be considered for operation. Medical therapy, consisting either of anticoagulation or thrombolytic therapy, results in an unsatisfactory clinical outcome because it does not correct the underlying mechanical abnormality.     

Cystic fibrosis detection in high-risk Egyptian children and CFTR mutation analysis.

BACKGROUND: Knowledge about Cystic Fibrosis (CF) in Egypt is very limited. The objective of this study was to screen for CF in Egyptian children with suggestive clinical features and to identify causative genetic mutations. METHODS: Sixty-one patients from the Chest Unit, Cairo University Children's Hospital, Egypt, were included. Subjects presented with persistent or recurrent respiratory symptoms, failure to thrive, diarrhea and/or steatorrhea and unexplained persistent jaundice. Patients were screened using the CF Indicatortrade mark sweat test system (PolyChrome Medical, Inc., Brooklyn Center, MN). A quantitative sweat testing was conducted on 10 of the 12 positive patients. Seven probands and one sibling underwent molecular analysis by direct DNA sequencing of the coding region and of the intronic sequences adjacent to the 27 exons of the CFTR gene. RESULTS: Of 61 patients, 12 (20%) had positive sweat chloride screening. Ten of the 12 patients underwent quantitative sweat testing and were positive. Eight CFTR sequence changes were identified in seven affected probands and two were confirmed in one sibling by direct DNA sequencing. CONCLUSION: The study results suggest that CF is more common in Egypt than previously anticipated. Larger studies are warranted to identify the incidence, molecular basis and clinical pattern of CF in the Egyptian population.     

Blastomycosis: a case study of a dimorphic fungal disease.

A 17-year-old male presented to a local hospital with symptoms of pneumonia and a hacking cough productive of yellow sputum. Direct examination of a bronchial lavage sample revealed organisms morphologically consistent with Blastomyces dermatitidis, which was confirmed by culture. The patient was placed on intravenous antifungal therapy and his condition improved dramatically. OBJECTIVES: to describe the gross and microscopic morphologies of both yeast and mold forms of B. dermatitidis; to identify B. dermatitidis given patient history, and microscopic and colony morphology; to describe the symptoms of primary pulmonary infections caused by B. dermatitidis, and to name additional tissues typically affected by the systemic pathogen.     

A clinico-pathological study of laryngeal and hypopharyngeal carcinoma: correlation of cord-arytenoid mobility with histopathologic involvement.

OBJECTIVE: This study evaluates the vocal cord and arytenoid mobility in relation to the tumoral involvement of the cricoarytenoid joint (CAJ), thyroarytenoid (TA) muscle, and posterior cricoarytenoid (PCA) muscle in whole-organ sections of total laryngectomy specimens. STUDY DESIGN AND SETTING: The study was prospectively carried out between 1998 and 2003 and involved 133 primary squamous cell carcinoma cases of larynx or pyriform sinus that were treated with total laryngectomy. Preoperative mobility of vocal cords and arytenoids were evaluated separately and correlated with histopathologic findings. RESULTS: In cases with fixed vocal cord CAJ, TA muscle, and PCA muscle were involved in 42.4%, 72.9%, and 27.1% of the cases, respectively, while in cases with fixed arytenoid these structures were affected in 40%, 66.3%, and 25.3% of the cases. Weight effect seemed to play a role in 60% and 50% of the cases of supraglottic and pyriform sinus carcinoma, respectively. CONCLUSION: Careful evaluation of the mobility of the cord arytenoid unit separately helps the surgeon to better understand the patterns of tumoral extension guiding for the appropriate surgery which should be undertaken.