The treatment of ANCA-associated rapidly-progressive glomerulonephritis and Goodpasture syndrome with therapeutic apheresis

Therapeutic plasma aphresis (plasmapheresis) is one form of treatment that is frequently used in practice of Nephrology. Plasmapheresis is the most important part of the therapies for Goodpasture's syndrome and anti-neutrophil cytoplasmic antibody (ANCA) associated vasculitis which are causes of rapidly progressive glomerulonephritis. The reason why the effectiveness of plasmapheresis therapy cannot be clearly demonstrated in renal involvement in these diseases is that it does not appear to be possible to recruit an adequate number of patients and plasmapheresis is not effective in advanced disease if early treatment is not initiated.     

Life saving carotid artery stenting in Takayasu arteritis

We present two patients who had high grade unilateral carotid artery stenosis and controlateral carotid artery occlusion secondary to active stage of Takayasu arteritis. The patients were presented with hemiplegia and history of transient ischemic attacks and visual disturbances. Despite the administration of high dose systemic corticosteroids, both patients deteriorated clinically and surgery was not preferred. Angiogram of the first patient revealed the occlusion of the right common carotid, right subclavian and left subclavian artery and high grade stenosis (>95%) of the left common carotid and right vertebral artery. These two stenotic arteries were stented. Angiogram of the second patient revealed the occlusion of the left common carotid and subclavian artery and high grade stenosis (>90%) of the right common carotid artery. PTA + stenting of the right carotid artery was performed. There were no complications during a follow-up period of 10.5±7 months and control angiograms revealed that all stented vessels were free of restenosis. In conclusion, stent-supported PTA to carotid arteries provides immediate symptomatic relief for patients in the active phase of the disease. Its efficacy in the long term should be investigated.     

The potential effect of anatomic relationship between the femur and the tibia on medial meniscus tears

Background

The anatomic and the kinematical relationships between the femur and the tibia have been previously examined in both normal and diseased knees. However, less attention has been directed to the effect of these relationships on the meniscal diseases. Therefore, we aimed to investigate the impact of femorotibial incongruence on both lateral and medial meniscal tears.

Materials and methods

A total of 100 images obtained from MRI of 100 patients (39 males and 61 females) were included in the study. Diameters of the medial and the lateral femoral condyles, thicknesses of the menisci, and diameters of the medial and the lateral tibial articular surfaces were measured.

Results

The medial meniscus tear was detected in 40 (40 %) patients. However, no lateral meniscus tear was found. Significant relationships were found between the diameters of the posterior medial femoral condyle and the medial tibial superior articular surface and between the diameters of the posterior lateral femoral condyle and the lateral tibial superior articular surface. The mean values for the diameter of the medial condyle of the femur, the lateral condyle of the femur, the medial superior articular surface of the tibia, and the lateral superior articular surface of the tibia were found to be significantly higher in cases with meniscus tear compared to cases without meniscus tear. However, no significant difference was present regarding the thicknesses of the medial and the lateral menisci. A positive relationship between the diameter of the posterior medial femoral condyle and the tibial medial superior articular surface was found in cases with (n = 40) (r ² = 0.208, p = 0.003) and without tear (n = 60) (r ² = 0.182, p = 0.001). In addition, a significant positive relationship was found between the diameter of the posterior medial femoral condyle and the medial tibial superior articular surface in cases with and without tear.

Conclusion

The impact of femorotibial incongruence on the medial meniscus tear is important for the understanding of the lesions.     

Tuberculin skin test screening in a military school in Istanbul city center

To investigate the reason of high incidence of annual patients with tuberculosis (TB) in a military school previously known by screening tuberculin skin test (TST) and finding out the proportion of annual infection risk (PAIR), the prevalance of TB infection and the distribution for each grades. Our study is a cross-sectional epidemiologic study made about TB infection. TST were screened for all students in the school. 5 TU PPD was injected to every student and after 72 hours, the results were evaluated by measuring the diameter of enduration. Test was repeated after 10 days for negative reactions. Age, sex, the number of BCG wound, smoking and dwelling for last 5 years were asked from the students and their answers were recorded. More than 10 mm enduration for cases who had no BCG and 15 mm enduration for cases who had BCG were accepted positive. Chest roentgenogram was taken for each student enrolled into the study. Infection prevalance and PAIR were calculated after tests and measurements. The total number of students was 948. Of 917 (96.7%) were male and 31 (3.3%) were female. The mean age was 19.72 +/- 1.25. The mean of TST was 12.79 +/- 5.96 mm for all students. According to the number of BCG scar, the numbers of students, percentage and the mean of TST were like that 70 (7.3%) cases no BCG scar 8.41 +/- 7.87 mm, 393 (41.4%) students one BCG scar, 11.94 +/- 6.26 mm, 343 (36.1%) cases two BCG scars, 13.74 +/- 5.12 mm, 142 (14.9%) students three or more then three scars, 14.97 +/- 4.11 mm. In the students who had no BCG, TST positivity was 50%. TB infection prevalance of entire school and PAIR were 46% and 3.44% (respectively). In this study, we found that increased number of BCG wound associated with the increased diameter of TST enduration. The proportion of unvaccinated students was similar to the same age population in our country but it showed differences in the distribution of regions. The students who started first grade had serious TB infection risk in their first school year. We think that PAIR values derived from TST conversions done in high risky community by screening annual TST could show all aspects of TB infection risk in those community.     

The course of the inferior gluteal nerve and surgical landmarks for its localization during posterior approaches to hip

The position of the inferior gluteal nerve (IGN) makes it vulnerable to iatrogenic injury during posterior and posterolateral approaches to the hip. Although the posterior approach has been reported to be the most frequently used technique, it is most likely to be associated with damage to the IGN. As there is scant information in the literature regarding the course and the anatomic relationships of the IGN, we aimed to investigate the anatomic course of the IGN and define the anatomical landmarks that can be used by surgeons during posterior approaches to the hip. Thirty-six gluteal regions from adult fixed cadavers were used for this study. A triangular-shaped anatomic area that contains the IGN was defined. This geometric area was formed by connecting the following points: posterior inferior iliac spine (PIIS) (apex), ischial tuberosity (IT) and greater trochanter (GT). This triangle can further be divided into two, the upper triangle being the “danger zone” since it contains the IGN and its branches. The closest mean distance between the point of IGN origin and the PIIS, IT and the GT was 3.2, 4.8 and 5.4 cm, respectively. In all specimens, the nerve entered the deep surface of the gluteus maximus approximately 5.4 cm from the apex of the GT and approached the GT as close as 0.8 cm, on average. Based on our study, dividing the gluteus maximus with standard techniques may damage the IGN. Posterior minimally invasive approaches to the hip should take into account the point of entry of the IGN into the gluteus maximus. Localization of the IGN by using the anatomic triangle defined in this study may decrease surgical morbidity.     

Serum prolactin levels in Behçet's disease

PURPOSE: Evaluation of serum prolactin levels in Beh?et's disease patients in Turkey. METHODS: Serum prolactin levels were measured by radioimmunoassay (RIA) in 17 patients with ocular findings, and 20 patients without ocular findings of Beh?et's disease, and in 17 healthy volunteers. RESULTS: The average prolactin levels were measured as 9.53 ng/mL in patients with ocular findings, 8.84 ng/mL in patients without ocular findings, and 9.59 ng/mL in healthy controls. There was no statistical significance among these three groups. Also, the average levels were 9.84 ng/mL in remission periods and 7.54 ng/mL in attacks. CONCLUSIONS: In some studies, it has been suggested there is a correlation between high serum prolactin levels and activation of some autoimmune diseases, such as systemic lupus erythematosus and rheumatoid arthritis. However, we found no such correlation in Beh?et's disease. On the contrary, prolactin levels were lower in attacks than in remissions.     

Perigraft to right atrial shunt by using autologous pericardium for control of bleeding in acute type A dissections

BACKGROUND: We report our experience with creating a perigraft to right atrial fistula by using autologous pericardium to control the inaccessible bleeding after aortic root repair in patients with acute type A aortic dissection. METHODS: Between 1994 and 2001, perigraft to right atrial fistula was used in 7 of 109 patients (mean age; 55 years) who underwent emergency operation for acute type A dissections. A chamber around the aortic graft was created by suturing a patch of pericardium to the right ventricular wall inferiorly, to the pulmonary artery medially, to the Teflon felt at the distal aortic anastomosis or innominate vein superiorly, and to the superior vena cava and right atrium laterally. A large stab wound was created on the medial aspect of the right atrium. The perigraft space was then closed expeditiously. RESULTS: None of these patients required reexploration for bleeding and they were discharged from the hospital without complications. The average blood and fresh frozen plasma requirement was 3.4 +/- 0.9 and 2.7 +/- 0.7, respectively. All underwent echocardiographic examination before discharge and no perigraft to right atrial shunt was detected. CONCLUSIONS: If intractable bleeding is encountered after the administration of protamine and thrombotic agents and a discrete bleeding site can not be found, then a perigraft to right atrial fistula using autologous pericardium can be created as a last resort. It provides primary and definite sternal closure and avoids the detrimental effects of a second pump run and continued bleeding.     

Assessment of movement time in patients with essential tremor

Background We have observed mild bradykinesia in essential tremor (ET) patients, which do not satisfy the criteria of Parkinson’s disease (PD). Objective To compare the mean movement time for repetitive movements around distal and proximal joints in ET patients with normal controls by using a simple test paradigm. Patients and methods Seventeen patients with ET and 14 control subjects were instructed to tap with the index finger sequentially on push–button microswitches. Movement times around metacarpophalangeal, wrist, elbow, and shoulder joints of the right side were tested. The data collected were stored on a computer and the time elapsed between sequential taps on two keys (ms) and number of taps on the left key for 15 seconds were evaluated offline. Results Movement times of the patients with ET were not found to be significantly different from those of the controls at all joints tested despite slight prolongation for movements around the shoulder joint. Conclusion The simple test paradigm we have used showed that there is no difference in the movement time for repetitive movements around four joints of the upper extremity between patients with ET and normal control subjects. The slightly prolonged movement time around the shoulder joint noted in patients with ET may be ascribed to tremor, not bradykinesia. Tremor may cause these patients to pay more attention to the performance of goal–directed finger movements and consequently prolong movement time slightly or it may simply delay the time elapsed to reach the goal in the absence of overt intention tremor.     

Waardenburg-Syndrom

BACKGROUND: Waardenburg syndrome (WS) is an autosomal dominant disorder characterised by pigmentary anomalies of the skin, hairs, eyes and various defects of other neural crest derived tissues. It accounts for over 2% of congenital hearing impairment. At least four types are recognized on the basis of clinical and genetic criteria. PATIENTS AND METHODS: Based on a screening of congenitally hearing impaired children, 12 families with WS type II were detected. Of special interest was the phenotype of these families, in particular the reduced penetrance of hearing impairment within the families. RESULTS AND CONCLUSION: In all cases a high variability of the disease phenotype was detected and the penetrance of the clinical traits varied accordingly. Therefore, it is not possible to predict the clinical phenotype even in a single family. Based on these studies, we plan to identify the pathogenetic cause of the disease in order to perform a detailed genotype/phenotype analysis.