Neuropathy associated with “benign” anti-myelin-associated glycoprotein IgM gammopathy: Clinical,immunological, neurophysiological pathological findings and response to treatment in 33 cases

We studied 33 patients presenting with a peripheral neuropathy associated with non-malignant anti-myelin-associated glycoprotein (MAG) IgM monoclonal gammopathy (MG) in an attempt to delineate their clinical, immunological, electrophysiological and pathological characteristics; we also reviewed our experience concerning long-term follow-up and therapy. Peripheral neuropathy associated with non-malignant anti-MAG IgM MG was observed mostly in males (sex ratio 7.2), and mean age at onset was 67 years (range 46–81). A predominantly sensory pattern was noted in more than 80% of cases, although some patients were affected by a predominantly motor peripheral neuropathy. Although disease progression was slow in most cases, 45% of patients suffered severe disability, and in 2 cases, the patient's death appeared to stem directly from the neuropathy. The electrophysiological findings were indicative of a demyelinating process in 90% of cases, and electron microscopic examination of nerve biopsy specimens demonstrated widening of the myelin lamellae in more than 95% of cases. Most of our patients showed a disappointing response to steroids and chemotherapy or plasma exchanges. Intravenous immune globulin, evaluated in 17 patients, had a transient, mostly subjective effect in 35% and led to a clear-cut improvement in 24% of cases. We did not observe any correlation between the severity of the clinical picture and the anti-sulphoglucuronyl paragloboside antibody titre; in individual cases, clinical improvement occurred without lowering of IgM levels. Although the severity and the rate of progression may greatly vary from patient to patient, the combination of clinical, electrophysiological and pathological features delineates a characteristic pattern in peripheral neuropathy associated with non-malignant anti-MAG IgM MG.     

Comparison of FK-506 and cyclosporine regimens in pediatric renal transplantation

Clinical aspects of FK-506 or cyclosporine immunosuppression regimens were evaluated in 48 consecutive pediatric renal transplant recipients. Tapering and discontinuation of prednisone was employed only in children receiving FK-506 who experienced minor or no rejection episodes during the 1st posttransplant month. At 1 year follow-up, 17 of 22 (77%) of all children with functioning allografts were receiving no prednisone (n=13) or a mean dosage of 0.07 mg/kg per day (n=4). During the 1st month, acute cellular rejection was more common in the FK-506 group (0.58 vs. 0.21 rejections per patient,P<0.05) but allograft survival (92%) and renal function at 1 year posttransplant were identical in both groups. Compared with the cyclosporine regimen, FK-506 immunosuppression may be associated with a higher incidence of cytomegalovirus or reversible Epstein-Barr virus-induced lymphoproliferative disease. However, the FK-506 group had less hirsutism and gingival hypertrophy and required fewer antihypertensive medications independent of steroid use. Height standard deviation scores and weight-for-height index improved only in preadolescents receiving FK-506 but no prednisone (P<0.02 andP<0.05, respectively), but did not differ between children on FK-506 plus prednisone and those in the cyclosporine group. We conclude that the major advantages of FK-506 over cyclosporine immunosuppression are a reduced severity of hypertension and an improved cosmetic appearance which may improve long-term medical compliance. When used as monotherapy, FK-506 also shows promise in relieving the growth retardation associated with cyclosporine regimens that include prednisone.     

Kammerflimmern bei einem 27-j?hrigen Patienten mit Contusio cordis

Zusammenfassung Hintergrund: Bei Patienten mit stumpfem Thoraxtrauma muss mit einer Contusio cordis gerechnet werden. Das Spektrum der Symptomatik variiert von einer leichten regionalen myokardialen Funktionsstörung bis hin zur Ruptur und zum plötzlichen Herztod. Fallbeschreibung: Ein 27-jähriger Patient wurde nach einem Fußtritt gegen die Brust bei Kammerflimmern reanimiert. Das EKG sowie das Enzymmuster entsprachen denen eines akuten Myokardinfarkts, echokardiographisch zeigte sich eine Hypokinesie apikal und anteroseptal bei mittelgradig reduzierter Pumpfuktion. Die 10 Tage nach dem Trauma durchgeführte Koronarangiographie und Lävokardiographie ergaben einen unauffälligen Befund. Schlussfolgerung: Als Ursache des primären Kammerflimmerns und der initialen elektro- und echokardiographischen Befunde muss aufgrund der Anamnese eine Contusio cordis diskutiert und in der Therapie berücksichtigt werden. Abstract Background: Patients with a blunt chest trauma often sustain myocardial contusion. The spectrum of symptoms varies from regional myocardial dysfunction to myocardial rupture of sudden cardiac death. Case Report: After a kick against his chest, a 27-year-old patient was resuscitated because of ventricular fibrillation. ECG and enzymatic pattern corresponded to an acute myocardial infarction, the echocardiogram revealed an apical and anteroseptal hypokinesia. 10 days after the acute event, coronary arteriography and ventriculography did not show any abnormalities. Conclusion: On the basis of the anamnesis, a myocardial contusion must be discussed as reason for the ventricular fibrillation and the pathologic findings in ECG and echocardiogram. This has to be considered in the therapy.     

The Frictional Behavior of Coated Guiding Archwires

Background: The vast range of orthodontic wires made of different alloys makes it increasingly difficult for orthodontists to judge them. Coated orthodontic wires form a group of innovative guiding archwires. Material and Methods: In the present in vitro study the frictional behavior of eight coated wires of different dimensions was investigated in archwire-guided canine retraction in the upper jaw. For this purpose five superelastic nickel titanium alloy wires (Titanol^/reg; Low Force River Finish Gold and Gold 2: Forestadent^®, Pforzheim Germany; Titanol^® Superelastic tooth Sentalloy Ionguard™: GAC, Central Islip, NY, USA; NITI Imagination™: GAC, Central Islip, NY, USA), two #-titanium wires (TMA^® Low Friction Ionguard: Ormco, Glendora, CA, USA; TMA^® Low Friction Ionguard Purple: Ormco, Glendora, CA, USA) and one steel wire (Stainless steel Imagination™: GAC, Central Islip, NY, USA) were selected. The coatings were made of Teflon^® or polyethylene, and by ion implantation. Three uncoated archwires (Rematitan^® Lite Dimple; Dentaurum, Pforzheim, German; Titanol^® Low Force River Finish: Forestadent^®, Pforzheim, Germany; BioForce Sentalloy™: GAC, Central Islip, NY, USA) were used for comparison purposes. The force losses due to friction were measured using the Orthodontic Measurement and Simulation System (OMSS). Results: The results indicated that all coatings can reduce frictional losses compared with an uncoated reference wire by the same manufacturer. Measured frictional losses ranged from 48.3-6.1% with the Teflon^® coatings reducing the frictional losses to less than 10% in some cases. Conclusion: An unequivocal correlation between the surface roughness and frictional forces of the wires could not be verified by scanning electron microscopy. Zusammenfassung Hintergrund: Die Vielzahl an orthodontischen Drähten aus diversen Legierungen macht es die Kieferorthopäden immer schwerer, sie zu beurteilen. Eine Gruppe von neu angebotenen Führungsbögen stellen die beschichteten orthodontischen Drähte dar. Material und Methode: In der vorliegenden In-vitro-Studie wurde das Reibungsverhalten von acht beschichteten Drähten unterschiedlicher Dimension bei den bogengeführten Eckzahnretraktion im Oberkiefer untersucht. Neben fünf Nickel-Titan-Drähten (Titanol^® Low Force River Finish Gold und Gold 2: Fa. Forestadent^®; Titanol^® Superelastic zahnfarben: Fa. Forestadent^®; BioForce Sentalloy Ionguard™: Fa. GAC; NiTi Imagination™: Fa. GAC) wurden zwei #-Titan- (TMA Low Friction Iongard: Fa. Ormco; TMA Low Friction Ionguard Purple: Fa. Ormco) und ein Stahldraht (Stainless Steel Imagination™: Fa. GAC) ausgewählt. Die Beschichtungen bestanden aus Teflon^®, Polyethylen oder Ionenimplantation. Als Referenz wurden drei unbeschichtete Drähte (Rematitan^® Lite Dimple: Fa. Dentaurum; Titanol^® Low Force River Finish: Fa. Forestadent^®; BioForce Sentalloy™: Fa. GAC) in die Untersuchung einbezogen. Die Reibungsverluste wurden mit dem Orthodontischen Mess- und Simulations-System (OMSS) bestimmt. Ergebnisse: Die Ergebnisse zeigten, dass alle Beschichtungen, verglichen mit einem unbeschichteten Referenzdraht desselben Herstellers, eine Reduktion der Reibungsverluste bewirken. Die gemessenen Reibungsverluste lagen zwischen 48,3% und 6,1%, wobei bei Teflon^®-Beschichtungen der Reibungsverlust zum Teil auf unter 10% sank. Schlussfolgerung: Ein eindeutiger Zusammenhang zwischen der Oberflächenrauheit und den Friktionswerten der Drähte konnte anhand von rasterelektronenmikroskopischen Aufnahmen nicht bestätigt werden.     

Different proliferative activity of the glandular and myoepithelial lineages in benign proliferative and early malignant breast diseases.

The aim of the present study was to explore cell biological characteristics of normal breast, benign proliferative breast diseases and noninvasive breast malignancies based on the recently published adult progenitor cell concept from our group. Here, we investigated the proliferative activity of CK5/14(+), CK8/18/19(+) and alpha-smooth muscle actin(+) cellular phenotypes encountered in normal mammary gland, in a series of usual ductal hyperplasias and early malignant breast diseases, such as atypical ductal and lobular hyperplasias, as well as ductal and lobular in situ carcinomas. Immunohistochemical double labeling was performed on frozen sections from diagnostic breast biopsies by using antibodies to basal cytokeratins (CK5/14), glandular cytokeratins (CK8/18/19), smooth muscle actin and the Ki-67 antigen (MIB1). Normal breast tissues and usual ductal hyperplasias were characterized by a heterogeneous cellular composition of the growth fraction. The proliferative cell compartment consisted of CK8/18/19(+) glandular and, in a variable proportion, CK5/14(+) progenitor phenotypes. In contrast, noninvasive breast malignancies were composed of a monotonous proliferation of CK 8/18/19(+) neoplastic glandular cells. These findings indicate a significant role of progenitor cells in the development of benign proliferative breast diseases and lend support to the view that malignant transformation in the human breast usually occurs in a cell committed to the glandular lineage. Our results provide cell kinetic support to the functional progenitor cell hypothesis, and we propose this concept as an operative model for understanding benign proliferative and malignant breast diseases.