Mesangial proliferative glomerulonephritis in familial Mediterranean fever patient with E148Q mutation: the first case report

Familial Mediterranean fever (FMF) is an autosomal recessive hereditary disease characterized by recurrent attacks of fever, usually accompanied by sterile polyserositis. Although amyloidosis is the most common renal involvement, non-amyloid renal lesions, such as glomerulonephritis, have been described in patients with FMF. In this report, we present the first case of an FMF patient with heterozygous mutation of E148Q, mesangial proliferative glomerulonephritis, and no amyloidosis. While the association of mutation E148Q with renal involvement is still obscure, colchicine treatment is useful in mesangial proliferative glomerulonephritis with FMF.     

Simple and new technique to assess left internal mammary artery function by left ventriculography

The aim of this study was to demonstrate an assessment of left internal mammary artery (LIMA) patency and anatomy by standard left ventriculography, and to define a proposal for predicting LIMA function according to left ventriculography outcome. A total of 335 patients with an indication of coronary angiography were included. Standard coronary angiography and left ventriculography were performed initially. Visualization of LIMA occurred in the late phase of ventriculography and the LIMA visualization frame rate was counted for each patient. Then selective LIMA angiography was performed and LIMA diameter, LIMA course and anatomy, and subclavian artery anatomy were noted. Finally, the results of left ventriculography and LIMA angiography were compared by statistical analysis. During left ventriculography, LIMA was visualized in 96.4% of the patients. The mean LIMA visualization frame rate was 53.8 ± 17.7 and the mean LIMA diameter was 2.60 ± 0.36 mm. There was a strong correlation between LIMA visualization frame rate and LIMA diameter, LIMA course, and also asymptomatic subclavian artery disease (P < 0.001). Regression analysis showed that LIMA visualization frame rate is the major independent determinant for LIMA diameter prediction (P < 0.001); LIMA diameter, LIMA course, proximal LIMA side branch, and subclavian artery disease are the major predictors of LIMA visualization on left ventriculography (P < 0.001). LIMA patency and anatomy can be evaluated accurately with a simple method using left ventriculography. Besides direct visualization of LIMA, the visualization frame rate may constitute a reliable parameter for assessing LIMA function. A LIMA visualization frame rate of less than 50 is associated with a healthy and well-sized LIMA.     

Ameliorative Effects of Proanthocyanidin on Renal Ischemia/Reperfusion Injury

Introduction. Several natural products have been reported to have beneficial effects on ischemia/reperfusion (I/R) injury, particularly from a preventative perspective. Therefore, this study was designed to investigate the efficiency of proanthocyanidin (PA), a natural product derived from grape seed, on renal dysfunction and injury induced by I/R of rat kidney. Materials and Methods. Twenty-four male Sprague-Dawley rats were divided into three groups: sham-operated, I/R, I/R+PA. Rats were given PA (100 mg/kg/day peroral) 7 days prior to I/R. All rats except sham-operated underwent 60 min of bilateral renal ischemia followed by 6 h of reperfusion. After reperfusion, kidneys and blood were obtained for evaluation. Superoxide dismutase, glutathione peroxidase, malondialdehyde, protein carbonyl content, and nitrite/nitrate level (NO_x) were determined in the renal tissue. Serum creatinine (S_Cr), blood urea nitrogen (BUN), and aspartate aminotransferase (AST) were determined in the blood. Additionally, renal sections were used for histological grade of renal injury. Results. PA significantly reduced the I/R-induced increases in S_Cr, BUN, and AST. In addition, PA markedly reduced elevated oxidative stress product, restored decreased antioxidant enzymes, and attenuated histological alterations. Moreover, PA attenuated the tissue NO_x, levels indicating reduced NO production. Conclusions. The pretreatment of rats with PA reduced the renal dysfunction and morphological changes, ameliorated cellular injury, and restored renal antioxidant enzymes caused by renal I/R.     

The Relationship between the MEFV Genotype,Clinical Features,and Cytokine-Inflammatory Activities in Patients with Familial Mediterranean Fever

Familial Mediterranean Fever (FMF) is an autosomal recessive disease characterized by periodic attacks of fever and polyserositis. The effects of the MEFV genotype differences on clinical picture and inflammatory activity have not been well documented. The aim of this study was to investigate levels of conventional inflammation markers, procalcitonin, interleukin levels, TNF-alpha, and C5a levels in patients with FMF who had different MEFV genotypes and compare them with those of healthy subjects. The study consisted of 41 patients with FMF (F/M: 23/18), and 31 healthy subjects (F/M: 18/13). Tests were performed during the attack-free period.

White-blood cell count, CRP and IL-8 levels were higher in patients with FMF than in healthy subjects (p < 0.05) and also higher in M680I carriers than in the patients with M694V allele carriers. However, ESR, fibrinogen, procalcitonin, IL-6, C5a, TNF-alpha, and IgD levels were not significantly different between patients and healthy subjects (p > 0.05). Arthralgia or arthritis was significantly higher in M694V carriers than in non-M694V carriers (p < 0.05). It is concluded that the clinical features and inflammatory-cytokine activities were higher in patients with FMF during the attack-free period than in healthy subjects, and the different genotype might be related to different clinical pictures.     

The effects of fetal allogeneic umbilical cord tissue transplant following experimental spinal cord injury on urinary bladder morphology

Background and purposeIn continuation of our previous experimental study on spinal cord injury (SCI) using fetal stem cells, we investigated here the effects of fetal allogeneic umbilical cord tissue transplant on the urinary bladder morphology in a rat SCI model.Material and methodsFive pregnant albino Wistar rats at 12 days of gestation were used to obtain the umbilical cord cell graft. In Group 1 (n = 5), Th8-Th9 laminectomy was performed. Group 2 (n = 5) received spinal cord injury. In Group 3 (n = 5), the cultured fetal umbilical cord cells coated with alginate gel were placed into the lesion cavity. In Group 4 (n = 5), only alginate sponges without umbilical cord cells were placed into the injury cavity. The bladders of animals were analyzed pathologically at 21 days after surgery.ResultsThe thickness of the epithelium and the lamina propria did not differ among studied groups (p > 0.05). The lamina muscularis thickness was significantly higher in Group 2 and Group 4 than the others (p < 0.05). The bladder weight was similar among Groups 1, 2, and 3 (p > 0.05). Fibrosis was significantly increased in Group 2 (p < 0.05); it was greater in Group 2 than in Group 3 (p < 0.05) but did not differ between Groups 1 and 3 (p > 0.05).ConclusionsThis study suggests that allogeneic umbilical cord tissue transplantation after SCI may prevent bladder wall hypertrophy and fibrosis in the rat SCI model.     

The intracranial bridging veins: a comprehensive review of their history, anatomy, histology, pathology, and neurosurgical implications

Introduction

The intracranial bridging veins are pathways crucial for venous drainage of the brain. They are not only involved in pathological conditions but also serve as important landmarks within neurological surgery.

Methods

The medical literature on bridging veins was reviewed in regard to their historical aspects, embryology, histology, anatomy, and surgery.

Conclusion

Knowledge on the intracranial bridging veins and their dynamics has evolved over time and is of great significance to the neurosurgeon.     

Kidney transplantation for end‐stage renal disease secondary to familial Mediterranean fever

Although kidney transplantation (KT) is widely used for treating renal amyloidosis secondary to familial Mediterranean fever (FMF), data concerning transplant outcome are limited and inconsistent. The aim of this study was to determine the long‐term outcome of KT in patients with amyloidosis secondary to FMF. Kidney transplantation outcome in 24 patients with FMF was compared to that in 72 controls matched for age, gender of recipient, and type of the donor that underwent KT due to end‐stage renal disease (ESRD) not caused by FMF. Mean follow‐up time was 80.3 ± 55.1 months in the FMF group, vs. 86.5 ± 47.6 months in the control group. Death‐censored graft survival at five and 10 yr in the FMF group was 95.8% and 78.4%, respectively, and was comparable to that in the control group. In the FMF group, five‐ and 10‐yr patient survival (87.5 and 65.6%) was shorter than in the control group, but the difference was not statistically significant. The findings show that long‐term outcome of KT in the patients with amyloidosis secondary to FMF was comparable to that in patients with ESRD not caused by FMF. Recurrence of amyloidosis in the allograft, gastrointestinal intolerance, and fatal infections remain as major complications during the post‐transplant period.     

Two level pedicle substraction osteotomies for the treatment of severe fixed sagittal plane deformity: computer software-assisted preoperative planning and assessing

Purpose

To evaluate the efficacy of two level pedicle substraction osteotomies (PSOs) planned preoperatively with a computer software, in the patients with severe fixed sagittal plane deformities.

Methods

In the literature, there are studies indicating that two level PSOs may be required in severe cases. However, the results of two level PSOs preoperatively planned with computer software-assistance have not yet been reported in the English literature. Severe fixed sagittal plane deformities of 11 patients are described. Preoperative surgical planning was done with the aid of a computer software. Two level PSOs were indicated after the process. After the application of the indicated surgical technique, clinical and radiological results were evaluated in the preoperative, the early postoperative periods and during the last follow-up.

Results

The mean sagittal vertical axis was found as 190.5 (range 161–220) mm in the preoperative period, 23.5 (range ?27 to 61) mm in the early postoperative period (P < 0.001) (87.7 % correction) and 34.5 (range ?3 to 55) mm during the last follow-up (P < 0.001). The mean pelvic tilt (PT) significantly decreased from 38.3° (range 21°–63°) preoperatively to 23.8° (range 18°–42°) postoperatively (P = 0.008) and to 27.5° (range 17°–42°) during the last follow-up (P = 0.042). The mean lumbar lordosis (LL) was 2.8° (range ?29° to 20°) preoperatively, ?35.6° (range ?54° to 23°) early postoperatively (P < 0.001) and ?33.6° (range ?52° to 20°) during the last follow-up (P < 0.001). The average amount of bleeding was 5345 (range 2600–7415) ml.

Conclusion

Although a statistically significant correction was obtained, the mean PT and PI–LL value could not be restored in physiological limits during the last follow-up. Thus, two level PSOs performed after computer software (surgimap) assisted preoperative planning failed to correct severe fixed sagittal plane deformities. Besides, this procedure is of possible risks for major complications such as a result of excessive bleeding. We recommend that two level PSOs should be rarely indicated, but preferred as an alternative technique only in the most severe cases.