Surgery in patients with haemophilia and high responding inhibitors: Izmir experience

Summary. This report evaluates the haemostatic efficacy of recombinant factor VIIa (rFVIIa) and activated prothrombin complex concentrate (APCC) in patients with haemophilia and high responding inhibitors who underwent major and minor surgery. Data pertaining to surgeries from 2001 to 2009 at a single centre were retrospectively analysed. During this period, 53 surgical procedures were performed in 30 haemophiliacs with high responding inhibitors. Mean age was 16.2 ± 9.4 years. Eleven major surgeries in 4 patients, 41 radioisotope synovectomies (RS) and one circumcision classified as minor surgery in 28 patients were performed. Among the major surgery procedures, four were treated with rFVIIa, five with APCC and two with sequential use of APCC and rFVIIa. We used rFVIIa at the dosage of 80–120 μg kg^?1 every 2 h and APCC 100 IU kg^?1 every 12 h for the major surgery. When performing RS, we used rFVIIa in 18 patients with 26 target joints and APCC in 9 patients with 15 target joints. Three consecutive doses of rFVIIa (90 μg kg^?1) were used at 2‐h intervals followed by additional three doses at 6‐h intervals. The initial dose of APCC was 75 IU kg^?1 followed by a second and third dose of 50 IU kg^?1 at 12‐h intervals. APCC and rFVIIa demonstrated excellent efficacy in our major and minor surgical interventions [100% (22/22) and 94% (31/33), respectively]. We had only two bleeding complications with rFVIIa. There were no thromboembolic complications. APCC and rFVIIa provide an effective and safe first line haemostatic therapy for inhibitor‐positive haemophiliacs, allowing both major and minor surgery to be successfully performed.     

Patient resources in the therapeutic education of haemophiliacs in France: their skills and roles as defined by consensus of a working group

Summary. The activities of ‘expert patients’ or ‘patient tutors’, who help educate their peers, are gaining recognition in the health care system. This study investigates the role played by such patients in therapeutic education programmes organized by caregivers to validate the role of patients in implementing the therapeutic education of haemophilic patients and to define the skills required for such activities. This study employs the consensus methodology recommended by France’s National Authority for Health. The working group includes seven caregivers from Hemophiliac Treatment Centers (HTCs) and three patients from the French Association of Hemophiliacs (FAH). The role of patients in haemophilia education is recognized. Patients participating in the education of their peers are referred to as ‘patient resources’. A patient resource should be an adult, a volunteer and live in the same region as his peers. Candidates are chosen by the FAH and the HTCs to serve based on their motivation to facilitate the education of other patients as well as on their psychological and pedagogical aptitudes. A patient resource participates in the conception and administration of therapeutic education programmes. He also mediates between the caregivers and the patients. He ensures that the patients understand the material and are able to apply their knowledge in daily life. His activities are governed by professional ethics. Seven categories of skills were defined, permitting the group to determine precisely which skills are required to function as a patient resource. Supervision of the patients is planned to reinforce reflexive practices in the patients. Evolution of the health care system has led patients to become involved in therapeutic education. This phenomenon calls for a framework to be developed and an evaluation of its eventual effects.     

Organ of vision in preterm infant]

Examinations of the organ of vision in 79 preterm infants showed lack of sensitivity of the conjunctiva and cornea, unstable precorneal membrane, and insufficient lacrimal production; these features may promote the development of diseases of the anterior segment of the eyeball. Decreased reaction to a light stimulus and lack of the pupil reaction to light are characteristic features of a preterm infant. The position of the baby lying in an incubator and poor transparency of ocular media impedes the ophthalmological examination and require special training of the physician. Pathological changes in the fundus oculi of a preterm baby should be regularly checked up by an oculist during the postnatal period.     

Increased protein oxidation and decreased creatine kinase BB expression and activity after spinal cord contusion injury

Traumatic injury to the spinal cord triggers several secondary effects, including oxidative stress and compromised energy metabolism, which play a major role in biochemical and pathological changes in spinal cord tissue. Free radical generation and lipid peroxidation have been shown to be early events subsequent to spinal cord injury. In the present study, we demonstrated that protein oxidation increases in rat spinal cord tissue after experimental injury. As early as h after injury, the level of protein carbonyls at the injury epicenter was significantly higher than in control (169%, p < 0.05) and increased gradually over the next 4 weeks to 1260% of control level. Both caudal and rostral parts of the injured spinal cord demonstrated a mild increase of protein carbonyls by 4 weeks postinjury (135-138%, p < 0.05). Immunocytochemical analysis of protein carbonyls in the spinal cord cross-sections showed increased protein carbonyl immunoreactivity in the epicenter section compared to rostral and caudal sections of the same animal or control laminectomy animals. Increased protein carbonyl formation in damaged spinal cord tissue was associated with changes in activity and expression of an oxidative sensitive enzyme, creatine kinase BB, which plays an important role in the maintenance of ATP level in the CNS tissue. Damage to CK function in the CNS may severely aggravate the impairment of energy metabolism. The results of our study indicate that events associated with oxidative damage are triggered immediately after spinal cord trauma but continue to occur over the subsequent 4 weeks. These results suggest that antioxidant therapeutic strategies may be beneficial to lessen the consequences of the injury and potentially improve the restoration of neurological function.     

Pain in the lower part of the spine in spinal osteochondrosis: experience in using chondroprotective drugs

AIM: To assess the response to chondroitin sulfate (structum) in low back pain (LBP) due to spinal osteochondrosis. MATERIAL AND METHODS: 30 patients (mean age 51.4 years) with a definite primary LBP took structum in a dose 1 g/day for 24 weeks. The diagnosis was made according to WHO recommendations (2000). The response was assessed with uniform international questionnaires and visual analogue scale. RESULTS: The treatment reduced pain syndrome and improved spinal function in 73.3% patients with LBP studied. CONCLUSION: It would be valid to include long-acting chondroprotective drugs in the program of LBP in spinal osteochondrosis.     

Electrophysiological methods for examining preterm children and diagnosis of retinopathy of prematurity

The first stage of the study was devoted to registration of visual evoked potentials (VEP) in 14 full-term and 27 preterm children without signs of neonatal retinopathy (NR) at the age of 6-7 months. The authors conclude that VEP in preterm children without signs of NR, born at different terms of gestation, reach the values of full-term children by the age of 6 months. These data should be taken account of when examining preterm babies for timely diagnosis of pathological changes in the visual analyzer and treatment. The second stage of the study was developed to registration of VEP and electroretinogram (ERG) in 36 children aged 4-12 years with cicatricial forms of NR. Changes in the central compartments of the retina were responsible for low visual functions in children with the cicatricial stages of NR. This abnormality is caused by displacement of the macular zone, impairment of its electrogenesis and topographic anatomic relationships. Examination of the marginal retinal periphery (superior external quadrant) once a year is obligatory for patients with cicatricial stages of NR in order to timely detect inapparent local fixed detachments or prevent them by timely prophylactic laser coagulation of the retina.