Reduced Ia-afferent-mediated Hoffman reflex in streptozotocin-induced diabetic rats

Familial amyloidotic polyneuropathy (FAP) is a late-onset inherited disease characterized by the deposition of amyloid fibrils. FAP is associated with mutations on the transthyretin (TTR) gene. A monoclonal antibody, MAb 39-44, reacting with high molecular weight aggregates of TTR but not with tetrameric TTR has recently been generated and characterized. This antibody recognizes a cryptic epitope that is expressed in isolated recombinant amyloidogenic mutants and in ex vivo amyloid. In the present work we show that this amyloid-specific antibody specifically recognizes in a direct enzyme-linked immunoassay (ELISA) plasma TTR from carriers of various mutations associated with FAP, both in asymptomatic individuals and in patients. In contrast, it does not react with plasma TTR from healthy individuals or that from carriers of nonpathogenic mutations. Using the ELISA developed in this study we identified three different TTR mutations in Portuguese patients with neuropathy of unknown cause, later shown to have amyloid tissue deposition. This antibody recognizes conformations that express cryptic epitopes shared by amyloidogenic TTR variants associated with FAP, not present among nonpathogenic TTR molecules. This antibody will contribute to further identify and characterize intermediates of the amyloidogenic cascade. In addition, it will also be useful for screening amyloidogenic TTR mutations in patients with neuropathy of unknown cause, prior to precise molecular diagnosis using protein and/or DNA analysis.     

Diagnosis of a Bleeding Meckel''s Diverticulum Using Radiopertechnetate

A case report of bleeding from a Meckel's diverticulum diagnosed by Tc99-m pertechnetate scanning is presented. The noninvasive advantage of this method justifies its early use as a diagnostic measure when a Meckel's diverticulum is suspected in the differential diagnosis of lower gastrointestinal bleeding.     

Initial and Early Follow-up Assessment of the Clinical Efficacy of a Multiparameter-Programmable Puise Generator

Recent advances in pacemaker technology have produced noninvasively multiparameter-programmable puise generators that have the potential for resolving complications that result from the pacing system and forproviding a mechanism whereby adjustments can be made for the specific needs of a patient without corapromising reliability and longevity. These applications were assessed by analyzing the initial indications, clinical efficacy, and reliability in 100 consecufive patients who received a multiparameter-program-mable puJse generator. Data analysis showed that 78% of the 100 patients had changes in rate programmed, 16% in pufse width, and 5% in sensitivity. Program-mability was utilized for voJtage amplitude (one patient) but was not utilized for hysteresis and refractory period. Although progrtimming changes (rate and pulse width) generally could have been accomplished with simpler units, program-mability for sensitivity made it possible to solve pacemaker problems in five patients and clearly eliminated the need for reoperation in two patients. The compact size of the multiparameter-programmable pulse generator has allowed its use in patients in the pediatric age group, patients with a small frame, and patients with a major concern about cosmetic appearance. Programming sensitivity for proper sensing of the atrial signal allows use of the multiparameter-programmable pulse generator as an atrial pacemaker. This was done in one pediatric patient who also benefited from suppression of atrial overdrive. There have been no episodes of spurious programming. Reliabitity has not been sacrificed by the increased complexity of this unit. If preimplant criteria for use of a multiparameter-program-mable puise generator are too sfrict, this may limit its potential therapeutic value because the need for its flexible characleristics is unpredictable.