Changes in osteoporosis therapy in postmenopausal women from the RAC­OST­POL study: a 10­year follow­up.

INTRODUCTION: Therapeutic regimens for osteoporosis are the key elements in the management of osteoporotic patients. OBJECTIVE: The aim of the study was to present data on changes in osteoporosis therapy in women during a 10­year follow­up. PATIENTS AND METHODS: We analyzed a population­based sample recruited in the RAC­OST­POL study. At baseline, the cohort included 978 women, of whom 541 remained in the follow­up. Mean (SD) age of the patients was 74.7 (6.6) years. RESULTS: The number of untreated women and those on alendronate decreased, while the number of patients receiving other forms of the antiresorptive therapy or calcium / vitamin D supplementation increased during the study. The percentage of untreated women in the rural areas and the urban areas was, respectively, 85% and 74.1% at baseline, and 74.3% and 63.3% at the end of the follow­up. The percentage of untreated women decreased significantly, both in rural and urban cohorts. It was accompanied by a significant increase in calcium / vitamin D supplementation in both subgroups. Regarding the antiresorptive therapy, there was a significant increase in the frequency of its use only in the rural cohort. The use of therapeutic strategies was determined by the level of education. The frequency of calcium and / or vitamin D supplementation increased significantly in all education-related categories during the follow­up. CONCLUSION: Changes in the therapy of postmenopausal women with osteoporosis, observed in a 10­year follow­up of the RAC­OST­POL study, indicated that the level of care did not improve in this group of patients.

Dent disease in Poland: what we have learned so far?

PURPOSE: Dent disease (DD) is a rare tubulopathy characterized by proximal tubular dysfunction leading to chronic kidney disease (CKD). The aim of the study was to characterize patients with DD in Poland. METHODS: A retrospective analysis of a national cohort with genetically confirmed diagnosis. RESULTS: Of 24 males, all patients except one carried mutations in the CLCN5 gene; in one patient a mutation in the OCRL gene was disclosed. Molecular diagnosis was delayed 1 year on average (range 0-21 years). The most common features were tubular proteinuria (100%), hypercalciuria (87%), and nephrocalcinosis (56%). CKD (≤stage II) and growth deficiency were found in 45 and 22% of patients, respectively. Over time, a progression of CKD and persistence of growth impairment was noted. Subnephrotic and nephrotic proteinuria (20%) was found in most patients, but tubular proteinuria was assessed in only 67% of patients. In one family steroid-resistant nephrotic syndrome prompted a genetic testing, and reverse phenotyping. Five children (20%) underwent kidney biopsy, and two of them were treated with immunosuppressants. Hydrochlorothiazide and angiotensin-converting enzyme inhibitors were prescribed for a significant proportion of patients (42 and 37.5%, respectively), while supplemental therapy with phosphate, potassium, vitamin D (12.5% each), and alkali (4.2%) was insufficient, when compared to the percentages of patients requiring repletion. CONCLUSIONS: We found CLCN5 mutations in the vast majority of Polish patients with DD. Proteinuria was the most constant finding; however, tubular proteins were not assessed commonly, likely leading to delayed molecular diagnosis and misdiagnosis in some patients. More consideration should be given to optimize the therapy.

Complications of permanent cardiac pacing in patients with persistent left superior vena cava.

BACKGROUND: Persistent left superior vena cava (PLSVC) is present in about 0.3-0.5% of the general population and in about 12% of patients with other abnormalities. This congenital anomaly is usually asymptomatic and does not cause any physiological problems. However, it may become a significant problem in multiple clinical situations. Various complications related to PLVSC are encountered in anesthesiological, nephrological, oncological and cardiological procedures. The presence of PLSVC is usually incidentally detected during placement of pacemaker (PM), implantable cardioverter-defibrillator (ICD) and cardiac resynchronization therapy (CRT) leads. Technical difficulties during lead positioning (especially ventricular leads) are commonly known and often described in the literature. The purpose of the present study was to evaluate the specific methods used for implantation of increasingly complicated pacing systems, finding an optimal strategy in patients with PLSVC, especially with electrotherapy complications. METHODS: We performed a single-center retrospective analysis of 11 patients (7 women and 4 men, mean age 60.4 ± 13 years) with PLSVC hospitalized in single Cardiology Department between 2000 and 2012. The clinical characteristic, indications for PM/ICD/CRT implantation, technique of implantation and complications were evaluated. RESULTS: In PLSVC patients, different indications for pacing or resynchronization therapy were represented: sick sinus syndrome (SSS) in 4 patients, 3rd degree atrio-ventricular (AV) block in 4 patients, dilated cardiomyopathy with left bundle branch block in 2 patients, dilated cardiomyopathy and non-sustained ventricular tachycardia episodes in 1 patient. In patients no. 1, 3, 4 and 10 the complications necessitated the change of leads or type of pacing. Transvenous lead extraction was successfully performed in patient no. 1 and 10 with re-implantation of new leads via PLSVC in patient no. 1 and via right superior vena cava in patient no. 10. Patient no. 3 received an additional ventricular lead via PLSVC because of 2nd degree AV block (formerly atrial lead implanted due to SSS). In patient no. 4 with left atrial pacing (lead in coronary sinus), prosthetic mitral valve replacement was combined with epicardial ventricular lead placement. Patients no. 2 and 7 received a CRT device, without technical problems in patient no. 7, whereas in patient no. 2 due to difficulties with left ventricular lead positioning a hybrid approach to epicardial lead pacing was used. In patient no. 8 an ICD was implanted with difficulty in placing defibrillator lead. Patient no. 5 received 2 atrial leads via PLSVC with successful biatrial pacing; patient no. 6 with the necessity of DDD pacing had a (ventricular) lead for left atrial pacing and a typical right ventricular lead. In patients no. 9 and 11 typical DDD pacing was used with contralateral placement of the leads due to anatomical and technical differences. After 12 years of follow-up the survival is 90.9%. Late electrotherapy complications have developed only in patient no. 8 (problems with the defibrillator lead). CONCLUSIONS: Patients with PLSVC are a very heterogeneous group with different indications for pacing, therefore individualization of therapy is required. Technical complications connected with pacing of the right heart chambers are commonly known, hence transvenous left atrial or left ventricular lead implantation should be attempted. In case of difficulties in transvenous positioning of the lead, a hybrid or isolated cardiac surgery technique should be considered. Because of the increasing number of electrotherapy complications, these problems are also present in PLSVC patients. Transvenous lead extraction with re-implantation of a pacing system has not been reported yet. For this reason a thorough evaluation of the venous system is required in PLSVC patients before intervention.

Surgical treatment of insular tumours with tractography, functional magnetic resonance imaging, transcranial electrical stimulation and direct subcortical stimulation support.

BACKGROUND AND PURPOSE: Surgical treatment of insular tumours carries significant risks of limb paresis or speech disturbances due to their localization. The development of intraoperative neuromonitoring techniques that involve evoked motor potentials induced via both direct and transcranial cortical electrical stimulation as well as direct subcortical white matter stimulation, intraoperative application of preoperative tractography and functional magnetic resonance imaging (fMRI) in conjunction with neuronavigation resulted in significant reduction of postoperative disabilities that enabled widening of indications for surgical treatment. The aim of this study was to present the authors' own experience with surgical treatment of insular gliomas. MATERIAL AND METHODS: Our cohort comprises 30 patients with insular gliomas treated at the Department of Neurosurgery in Sosnowiec. Clinical symptoms included sensorimotor partial seizures in 86.6%; generalized seizures in 23.3%; persistent headaches in 16.6% and hemiparesis in 6.6%. All the patients were operated on with intraoperative neuromonitoring that included transcranial cortical stimulation, direct subcortical white matter stimulation as well as tractography and fMRI concurrently with neuronavigation. The analysis in-cluded postoperative neurological evaluation along with the assessment of the radicalism of resection evaluated based on postoperative MRI. RESULTS: Postoperatively, four patients had permanent hemiparesis (13.3%); importantly, two out of those patients had preoperative deficits (6.6%). Persistent speech disturbances were present in four patients (13.3%). Partial sensorimotor seizures were noted in two patients (6.6%). Seizures in the other patients receded. Intraoperative transcranial electrical stimulation as well as direct subcortical white matter stimulation along with tractography (DTI) and fMRI facilitated gross total resection of insular gliomas in 53.5%, subtotal in 13.3% and partial resection in 33.1%. CONCLUSIONS: Implementation of TES, direct subcortical white master stimulation, DTI and fMRI into the management protocol of the surgical treatment of insular tumours resulted in total and subtotal resections in 66% of cases with permanent motor disability in 6.6% of patients. Poor prognosis for independent living after surgery mainly affects patients with WHO grade III or IV.

Skeletal status assessed by quantitative ultrasound at the hand phalanges in karate training males.

The aim of the study was to assess the influence of regularly exercised karate on the skeletal status. The study comprised a group of 226 males (the mean age: 25.64 ± 12.3 years, range 7-61 years), exercising for 61.9 ± 68.4 months, with the mean frequency of 3.12 ± 1.4 times per week, and 502 controls, matched for age and body size. The skeletal status was assessed by quantitative ultrasound, using a DBM Sonic 1200 (IGEA, Italy) sonographic device, which measures amplitude-dependent speed of sound (Ad-SoS [m/s]) at hand phalanges. Ad-SoS, T-score, Z-score were significantly higher in the examined karatekas than in controls. Up to age 18, there had been no difference between the study subjects and controls, while afterwards, up to age 35, the difference increased to stabilize again after age 35. Longer duration, higher frequency and earlier start of physical training positively influenced the skeletal status. In conclusion, karate is a sport with a positive influence on the skeletal status with the most significant benefits occurring in adults.