CAROTID SINUS HYPERSENSITIVITY AND COMPLETE HEART BLOCK IN REITER'S SYNDROME

We describe a 42-year-old man with Reiter's syndrome who developedcomplete heart block only 5 wee after the onset of his illness.He was also noted to have carotid sinus hypersensitivity andevidence sinoatrial disease. There were no clinical signs orechocardiographic findings to suggest involvement of tl aorticroot or aortic valve. His arrhythmias were treated successfullywith a physiological pacemaker. The combination of sinoatrialand atrioventricular node disease suggests that the inflammatoryprocess Reiter's syndrome can produce widespread involvementof the conducting system. KEY WORDS: Reiter's syndrome, Arthritis, Carotid sinus hypersensitivity, Stokes-Adams attacks     

Extraction of Pacing Leads Jailed by a Stent in a Mustard Circulation

A case of successful lead extraction of atrial and ventricular pacing leads “jailed” by a stent in the superior limb of a Mustard baffle in a 40‐year‐old man with surgically corrected transposition of the great arteries and pacemaker pocket infection. Manual traction alone was sufficient to remove the jailed leads with no subsequent complications. (PACE 2010; e65–e67)     

Conversion of proendothelin-1 into endothelin-1 by aspartylproteases

The ability of cathepsin D, chymosin, pepsin and renin to produce endothelin-1 (ET-1) from proendothelin-1 (proET-1) was compared. No significant conversion was observed when proET-1 was incubated with up to 1 U of renin for 15 min at 37°C. Cathepsin D generated, as well as degraded, ET-1 rapidly. Net production of ET-1 reached a maximum when 0.003 U of cathepsin D was used, and about 16% of the initial proET-1 was detected as ET-1 by HPLC. Pepsin up to 1 U converted pro ET-1 into ET-1 dose-dependently with a maximum of 71% conversion. A further increase of the amount of pepsin in the reaction mixture produced nonspecific cleavage of ET-1. Less than 10% of ET-1 remained in the presence of 15 U of pepsin. Chymosin also generated ET-1 dose-dependently, and a complete conversion was obtained at 1 U of enzyme. Greater than 1 U of chymosin only slightly degraded ET-1; at least 80% of ET-1 was still present when 15 U of chymosin was included in the assay. Other properties associated with the conversion of proET-1 into ET-1 by chymosin were investigated. Similar to authentic ET-1, the product of chymosin treatment caused contraction of isolated rabbit aortic rings, and pre-incubation of chymosin with pepstatin A abolished this contractile response. Using fast atom bombardment mass spectrometry, the molecular weights of the two peptide fragments generated from proET-1 by chymosin were shown to be identical to those of authentic ET-1 and the C-terminal fragment of proET-1. The optimal pH of the enzyme reaction was between 3.5 and 4.5, and the K_m and V_max values for the enzyme were 140 μM and 102 nmol/(mg min), respectively. These results indicate that chymosin specifically and rapidly converts proET-1 into ET-1 in vitro.     

Cancers in patients with hemophilia: a retrospective study from the Italian Association of Hemophilia Centers: a rebuttal

See also Tagliaferri A, Franchini M. Cancers in patients with hemophilia: a retrospective study from the Italian Association of Hemophilia Centers: a reply to a rebuttal. This issue, pp 1201–2. Tagliaferri A, Di Perna C, Santoro C, Schinco P, Santoro R, Rossetti G, Coppola A, Morfini M, Franchini M, on behalf of the Italian Association of Hemophilia Centers. Cancers in patients with hemophilia: a retrospective study from the Italian Association of Hemophilia Centers. J Thromb Haemost 2012; 10 : 90–9.     

Haemophilia care in India: innovations and integrations by various chapters of Haemophilia Federation of India (HFI)

Summary.  Care of persons with haemophilia (PWH) in western countries is the responsibility of the government of those countries with or without funding from health insurers. Haemophilia societies in western countries work as pressure groups to ensure better care, and they disseminate information on the disease and some of the societies even support medical research for haemophilia care. In India, Haemophilia Federation of India (HFI) was established in 1982 with few haemophilia families and sympathizers of their cause; subsequently more than 65 chapters involving more than 12 500 PWH came up under HFI. HFI and its constituent chapters are unique in the world in the sense that they are not only trying to involve state and federal government to take responsibility for delivering haemophilia care, but they are also using various innovative and integrative techniques to deliver haemophilia care to PWH themselves, till the time federal and state governments of the country make suitable arrangement for their care. In this study, several of these approaches are discussed with the understanding that 80% of worlds' haemophilia population needs similar help, and the national haemophilia organizations (NMO) of various developing countries will find some of the approaches useful and adaptable to their own circumstances.     

Menorrhagia and reproductive health in rare bleeding disorders: a study from the Indian subcontinent

Abstract.  At this centre, 130 women with rare bleeding disorders (RBD) were investigated over the past 15 years. Fifty patients were above the age of menarche (age of menarche in India is 10 years). Of these 44 presented with menorrhagia. Other complications in these patients involved bleeding because of ruptured graffian follicle (1), severe haemorrhage following caesarean section (1), recurrent pregnancy losses (3), hysterectomy to control menorrhagia (2), laser ablation of endometrium (1) and irradiation of ovary (1). Three patients voluntarily chose to remain unmarried because of the problems associated with menorrhagia which they assumed will interfere with married life. All the 45 patients had iron deficiency anaemia. The spectrum of RBD in these patients comprised Glanzmann's thrombasthenia (17), Bernard-Soulier syndrome (2), storage pool disorder (2), factor V (FV) deficiency (3), combined FV and factor VIII deficiency (5), factor XI deficiency (3), factor XIII deficiency (1), factor X deficiency (5), factor VII deficiency (2), α₂-antiplasmin deficiency (1) and afibrinogenemia (3). RBD in women is diagnosed late and often they are not optimally managed hence suffer both iatrogenic and non-iatrogenic complications in this country.