Lower motor neuron disease associated with myelofibrosis

We present a patient who has signs pointing to the involvement of lower motor neurons and myelofibrosis. To our knowledge, unlike lymphoproliferative disorders, co-occurrence of myelofibrosis and lower motor neuron disease (MND) has not been reported so far. A 64-year-old male patient was admitted to our hospital with the complaint of painful cramps in his neck and forearms. On physical examination marked hepatomegaly and splenomegaly were found. On neurologic examination nasal quality of the voice and slight palatal weakness were detected. There were generalised slight weakness and atrophy in both proximal and distal muscle groups. Fasciculations were observed especially in forearm muscles and it was observed that he had been avoiding head movements because of painful muscle cramps. Deep tendon reflexes were hypoactive. Nerve conduction studies were normal. By needle electromyography, giant motor unit action potentials (amplitudes up to 8 mV), fibrillation potentials, positive sharp waves and fasciculation potentials were detected in all muscles which were investigated. A hypercellular bone marrow (100%) was determined by bone marrow biopsy. In addition to increased production of the myeloid and megakaryocytic lines, abnormal aggregation and grouping of megakaryocytes were seen. Reticular fibers were increased. He had some benefit of dyphenilhydantoin treatment given for the painful cramps in his neck and forearm muscles. Hydroxyurea treatment was started for myelofibrosis. Six months later, his general condition was better, and the painful cramps were completely resolved. No marked deterioration has been detected in neurologic examination and electromyography for 1 year.     

Early-mid term follow-up results of percutaneous closure of the interatrial septal defects with occlutech figulla devices: a single center experience

Background: Percutaneous closure of secundum type atrial septal defect (ASD) and patent foramen ovale (PFO) has gained widespread use in recent years. Herein, we evaluated the safety and efficacy of the Occlutech® Figulla devices for PFO and ASD closure in a reference tertiary center. Methods: All 143 patients (46.9% male, mean age 39.3 ± 12.2 years) who underwent transcatheter PFO (n = 85) and ASD (n = 58) closure with Occlutech® Figulla devices between February 2009 and October 2011 were included in this study. An echocardiographic follow‐up examination was performed at the 1st, 6th, and 12th month visits. Results: The devices were successfully implanted in all 143 patients (100%). In‐hospital periprocedural complications were device embolization (0.7%; 1 ASD patient), atrial fibrillation (1.4%; 1 ASD and 1 PFO patients), supraventricular tachycardia (0.7%; 1 PFO patient), and vascular access hematoma (0.7%; 1 ASD patient). Among ASD patients, 2 patients had trivial (jet width <1 mm in diameter) and 1 patient had small (1–2 mm) residual shunts before hospital discharge, which disappeared after the 6‐month visit. During the mean 15.4 ± 9.6 months follow‐up, all patients were asymptomatic and no ischemic stroke, cardiac perforation, device erosion, embolization, thrombus formation, or malposition of the device was observed. Conclusions: Percutaneous PFO and secundum type ASD closure with the novel Occlutech® Figulla Occluder devices without left atrial central pin and with significantly reduced meshwork was safe, feasible, and effective. (J Interven Cardiol 2012;25:375–381)     

Circulating irisin levels reflect visceral adiposity in non-diabetic patients undergoing hemodialysis

Background: Recent evidence suggests that increased visceral adiposity is a strong independent risk factor for cardiovascular death and all-cause mortality in hemodialysis (HD) patients. Irisin, which is a novel myokine, can play critical roles in diabetes and adiposity. The purpose of our study was to investigate whether serum irisin levels are associated with body mass index, waist circumference (WC), and total fat mass in non-diabetic patients undergoing maintenance HD.

Methods: This cross-sectional study included 108 non-diabetic HD patients and 40 age- and sex-matched apparently healthy subjects. Serum irisin concentrations were determined using an enzyme-linked immunosorbent assay. Body fat composition (TBF-410 Tanita Body Composition Analyzer) was measured and calculated.

Results: Serum irisin levels did not differ between HD patients and the healthy controls (523.50?±?229.32 vs. 511.28?±?259.74, p?=?0.782). Serum irisin levels were associated with age (r?=?0.314; p?=0.006), HOMA-IR (r?=?0.472; p?=?0.003), WC (r?=?0.862; p?r?=?0.614; p?β?=?1.240, p?β?=?0.792, p?=?0.015) were the variables that were significantly associated with irisin concentrations (R^2?=^?0.684, p?Conclusions: These results suggest that serum irisin levels are related to visceral adiposity in non-diabetic HD patients.