T. Rifki Evrenkaya

The Role of Prolidase Activity in the Diagnosis of Uremic Bone Disease

The derangements in bone metabolism in patients with chronic renal failure (CRF) are summarized as uremic bone disease (UBD). In this study, we planned to determine the serum prolidase to compare it with the other biochemical markers. This study was performed on 44 patients (19 females, 25 males, mean age = 56.8 ± 15.6 years) with end-stage renal disease (ESRD). The patients were divided into three groups according to serum bone alkaline phosphatase (bAP) levels. The patients whose bAP was ≥77 U/L were accepted as having high-turnover UBD (n = 18), the patients whose bAP was ≤50 U/L were accepted as having low-turnover UBD (n = 14), and the patients whose bAP levels were between these two values were accepted as having bone disease with normal turnover (n = 12). The serum prolidase levels did not increase in patients with ESRD. There were no significant differences between the serum prolidase levels of patients according to types of the UBD (p > 0.05). Kidney is the most prolidase-rich tissue of the human body. The serum prolidase activity is low in all patients with ESRD, irrespective of the type of UBD. Therefore, we concluded that prolidase had no value in the diagnosis of UBD.

A rare complication of idiopathic membranous nephropathy: crescentic transformation.

Introduction. In this study, the cause of rapidly deteriorating renal functions in a follow-up period of a 65-year-old female patient, who applied with nephrotic syndrome findings and diagnosed as membranous nephropathy, is presented. Case report. A 65-year-old patient with findings of nephrotic syndrome had normal kidney size and serum complement level, and was negative for autoantibodies and viral serology. In histopathologic examination, 20 glomeruli were consistent with membranous glomerulonephritis. The patient, evaluated for idiopathic membranous nephropathy, was followed-up monthly with supportive treatment. In the second month of follow-up, a re-evaluation of the patient due to nausea and urine discoloration revealed 144 mg/dL urea, 6.3 mg/dL creatinine, and 2.5 g/dL albumin. Urine sediment revealed dysmorphic erythrocytes and granular silenders. Renal re-biopsy was done. Of 11 glomeruli, three global sclerosis and eight crescentic glomeruli with fibrosis and scarce cellular component were seen. The case was accepted as crescentic glomerulonephritis, a rare complication of idiopathic MN. Before the treatment, antiGBM, pANCA, cANCA, and ANA were negative. Pulse metil prednisolone and pulse cyclophosphamide treatment protocol was administered. Hemodialysis was needed nine times. At the end of first month of the treatment, hemodialysis was no longer needed. Conclusion. Due to a risk of spontaneous remission up to 30% of membranous nephropathy, there is no consensus on specific treatment applicable to all cases. However, crescentic GN should be investigated immediately when sudden and rapid deterioration of renal functions appeared.

Missed Diagnosis of Aspergillus Niger Peritonitis in a Peritoneal Dialysis Patient with Standard Culture: Might Enriched Blood Culture Materials have an Advantage?

1 Trakya University Faculty of Medicine, Department of Nephrology, Edirne, Turkey 2 Pamukkale University Faculty of Medicine, Department of Nephrology, Denizli, Turkey 3 Haydarpaşa GATA Training Hospital, Department of Infectious Diseases, İstanbul, Turkey 4 Haydarpaşa GATA Training Hospital, Department of Nephrology, İstanbul, Turkey 5 Ankara Training and Research Hospital, Department of Infectious Diseases, Ankara, Turkey doi: 10.5262/tndt.2016.35

An Unusual Cause of HD Catheter Dysfunction

A 78-year-old woman with end-stage renal disease was referred to our clinic with a vascular access problem. She had been receiving HD treatment via a right brachiosephalic arteriovenous fistula (AVF) for 4 years which became silent 2 days before admission. A standard nontunneled double-lumen 14-Fr/15-cm catheter was inserted for HD treatment to the right IJV using the Seldinger technique. At the visit on the 10th day of intervention, there was no problem with HD therapy, but the pressure of the venous line had risDear Editor, Internal jugular vein (IJV) catheterization is the most preferable intervention for acute hemodialysis (HD) treatment, particularly in the last decade [1] . Although it is a simple application in experienced hands it can be associated with many complications, one of which is carotid arteryIJV fistula, a rare complication that has been described in the literature [2, 3] . Herein, we report a case with carotid artery-IJV fistula who presented with catheter dysfunction and interruption of HD treatment. Published online: January 9, 2013

Urinary Glycosaminoglycan Levels as a Marker of Renal Amyloidosis in Patients with Familial Mediterranean Fever

Introduction and aim. Familial Mediterranean Fever (FMF) is an autosomal recessive disease with a defect in the pyrine gene and is manifested with short attacks of inflammatory serositis, fever, and erysipelas-like skin lesions. Secondary amyloidosis is the most serious complication of the disease, in which extracellular deposits of amyloid (an amorphous and eosinophilic protein) are seen in tissues. Glycosaminoglycans are mucopolysaccharide molecules that take place in amyloid deposits with fibrillar links to amyloid. They form glycoproteins by linking to proteins, and their free forms are excreted in the urine in the form of polysaccharides. The aims of this study were to evaluate if the urinary levels of glycosaminoglycans have a predictive value in the diagnosis of amyloidosis secondary to FMF and if these levels are affected by treatment with colchicine. Materials and methods. The study included 55 volunteer patients (age range: 18–36 years) with FMF (15 with amyloidosis) of the same socio-economic circumstances without other concomitant inflammatory, malignant, or chronic diseases, along with 20 healthy subjects as control. Urinary glycosaminoglycan levels were determined twice, once when the patients were on medication and once after they have stopped treatment for two weeks. Results. Initial mean urinary GAG levels were significantly lower in amyloidosis patients. Mean urinary GAG levels determined two weeks after the cessation of colchicine was also significantly lower than controls in both amyloidosis and non-amyloidosis FMF patients. Likewise, in patients with a disease duration longer than ten years, urinary GAG levels were also lower than those with a disease duration of less than three years. Conclusion. Urinary GAG level can have a predictive value for amyloidosis in patients with FMF, and it can also be used as a non-invasive marker for screening the effects of colchicine on fibrillogenesis as well as for the follow-up of the patients.

Dissection of the Thoracic Aorta in a Patient with Autosomal Dominant Polycystic Kidney Disease

Internal Medicine,Gulhane Military Medical Academy, Haydarpasa Training Hospital, Istanbul, Turkey.Autosomal dominant polycystic kidney disease(ADPKD) is a systemic illness with a number of extra-renal manifestations. A 61-year-old male patient withknown ADPKD was admitted to the emergency roomof our hospital in May 2003 with severe chest painradiating to his neck and back, and a sense ofbreathlessness. There was no history of trauma.On admission, he was hypotensive (90/50 mmHg)and his heart rate was 110 beats/min. He had a mid-systolic murmur, which was most marked at the leftsternal edge. His peripheral pulses were weak. Bilater-al basal crepitations were found on pulmonaryexamination. Abdominal examination was normalexcept for large palpable polycystic kidneys. He hadno focal neurologic signs. Laboratory examinationshowed no significant abnormalities except for raisedserum urea (121 mg/dL) and creatinine (4.1 mg/dL).An electrocardiogram showed depression of ST seg-ments in the anterior leads. Emergency computerizedtomography scanning of the thorax and abdomen de-monstrated dissection and intramural thrombus withinthe descending thoracic aorta and bilateral polycystickidneys (Panels A and B).He was transferred immediately for aortic surgery.During the operation, a type II thoracic aortic dissectionwas observed. His recovery was complicated by theexpansion of the dissection and he died on the thirdpostoperative day.Numerous cardiovascular abnormalities have beenreported in ADPKD patients, including heart valvelesion, cerebral aneurysm, splenic artery aneurysm,aortic root dilatation, abdominal aortic aneurysm,thoracic aortic dissection, and cervical artery dissection[1–3]. The prevalence of intracranial aneurysm andrupture are five-fold higher in ADPKD patients than inthe general population [4]. However, the prevalence ofextracerebral aneurysms is not clear. One autopsy seriesdescribed aortic dissection as being seven times morecommon in patients with ADPKD than in the generalpopulation [2]. Spontaneous artery dissection wasreported as a major complication in five unrelatedADPKD patients. All underwent spontaneous dissect-ion and none had phenotype of elastic tissue disorderor thoracic aortic dissection. Torra et al, in their case-control study, found a similar distribution of aorticdiameters in 139 ADPKD patients and their 149unaffected relatives [5]. The case reported by Paynteret al appears to be very similar to our case [3]. Arterialdissection may belong to the spectrum of extrarenalmanifestations of ADPKD. The association of ADPKD