automated (centrifugal) therapeutic plasma exchange option for Guillain‑Barre syndrome: A report from Calabar, Nigeria

Therapeutic plasma exchange (TPE) is performed frequently and effectively in developed countries, whereas the reverse is the case in developing countries. Guillain‑Barre syndrome (GBS), synonymous with acute inflammatory demyelinating polyneuropathy, is an important indication for TPE, but this is rarely administered in the treatment of such patients in Nigeria due to lack of such automated facility, limited expertise, and high cost. This report therefore presents an uncommon case of GBS in which automated TPE was utilized in the management, with the aims of highlighting the current status and challenges of therapeutic apheresis services in Nigeria. A 42‑year‑old male presented with rapidly progressive (in an ascending fashion) paralysis of all four limbs within 24 h without any preceding history of fever or other symptoms. Clinical examination revealed a young man, afebrile, not pale, and also not dehydrated. Central nervoussystem examination showed a conscious man, alert, and oriented in time, person, and place. There were no signs of meningeal irritation and the cranial nerves were grossly intact. There was no power in the limbs: global hypotonia and areflexia were noted on examination. However, he had intact sensory perceptions to touch and pain. Following a diagnosis of GBS, he was treated with four sessions of plasmapheresis and TPE. The TPE session was done using a discontinuous flow apheresis machine which exchanged one plasma  volume (3 L of plasma) and 5% albumin used for replacement. The patient made gradual but steady recovery as return of power to the upper limbs and trunk started by the 2nd week of treatment. TPE is an important  treatment modality in the management of GBS as well as several other conditions, and it is becoming   increasingly available in Nigeria. However, it is still grossly underutilized, thus the need for more therapeuticapheresis facilities and trained personnel, in addition to concerted efforts to subsidize the cost of accessing the  treatment.Keywords: Guillain‑Barre syndrome, therapeutic apheresis, therapeutic plasma exchang

The lipid-lowering effects of lomitapide are unaffected by adjunctive apheresis in patients with homozygous familial hypercholesterolaemia - A post-hoc analysis of a Phase 3, single-arm, open-label trial

Objectives: Lomitapide (a microsomal triglyceride transfer protein inhibitor) is an adjunctive treatment for homozygous familial hypercholesterolaemia (HoFH), a rare genetic condition characterised by elevated low-density lipoprotein-cholesterol (LDL-C), and premature, severe, accelerated atherosclerosis. Standard of care for HoFH includes lipid-lowering drugs and lipoprotein apheresis. We conducted a post-hoc analysis using data from a Phase 3 study to assess whether concomitant apheresis affected the lipid-lowering efficacy of lomitapide. Methods: Existing lipid-lowering therapy, including apheresis, was to remain stable from Week-6 to Week 26. Lomitapide dose was escalated on the basis of individual safety/tolerability from 5mg to 60mg a day (maximum). The primary endpoint was mean percent change in LDL-C from baseline to Week 26 (efficacy phase), after which patients remained on lomitapide through Week 78 for safety assessment and further evaluation of efficacy. During this latter period, apheresis could be adjusted. We analysed the impact of apheresis on LDL-C reductions in patients receiving lomitapide. Results: Of the 29 patients that entered the efficacy phase, 18 (62%) were receiving apheresis at baseline. Twenty-three patients (13 receiving apheresis) completed the Week 26 evaluation. Of the six patients who discontinued in the first 26 weeks, five were receiving apheresis. There were no significant differences in percent change from baseline of LDL-C at Week 26 in patients treated (-48%) and not treated (-55%) with apheresis (p=0.545). Changes in Lp(a) levels were modest and not different between groups (p=0.436). Conclusion: The LDL-C lowering efficacy of lomitapide is unaffected by lipoprotein apheresis

Molecular characterization of autonomic and neuropeptide receptors

© 1994 by Marcel Dekker, Inc. All Rights Reserved. For many years, it was believed that the control of airway function was dependent on the balance between the cholinergic (parasympathetic) and adrenergic (sympathetic) nervous systems. The cholinergic system is considered excitatory because it plays a role in maintaining airway tone and in mediating acute bronchospastic responses (Casale, 1993). The effect of acetylcholine to produce narrowing of the airways is blocked by atropine, indicating that this effect is mediated by muscarinic acetylcholine receptors (Colebatch and Halmagyi, 1963; Olsen et al., 1965). In contrast, the adrenergic system in the lung is considered inhibitory because stimulation of β-adrenergic receptors produces relaxation of bronchial smooth muscle. The beta-blockade theory of the pathogenesis of asthma from Szentivanyi (1968) proposed that asthma was related to an imbalance in the autonomic control of airway diameter due to a decrease in β-adrenergic sensitivity in bronchial smooth muscle, mucus glands, and mucosal blood vessels