Marius Miglinas

Tumour-induced osteomalacia: a literature review and a case report.

Tumour-induced osteomalacia (TIO) is a rare paraneoplastic syndrome characterised by severe hypophosphataemia and osteomalacia, with renal phosphate wasting that occurs in association with tumour. The epidemiology likewise aetiology is not known. The clinical presentation of TIO includes bone fractures, bone and muscular pains, and sometimes height and weight loss. TIO may be associated with mesenchymal tumours which may be benign or malignant in rare cases. Mesenchymal tumour itself may be related to fibroblast growth factor 23 (FGF23), which is responsible for hypophosphataemia and phosphaturia occurring in this paraneoplastic syndrome. Hypophosphataemia, phosphaturia and elevated alkaline phosphatase are the main laboratory readings that may lead to more precise investigations and better diagnosis. Finding the tumour can be a major diagnostic challenge and may involve total body magnetic resonance imaging, computed tomography and scintigraphy using radiolabelled somatostatin analogue. The treatment of choice for TIO is resection of a tumour with a wide margin to insure complete tumour removal, as recurrences of these tumours have been reported. We provide here an overview on the current available TIO case reports and review the best practices that may lead to earlier recognition of TIO and the subsequent treatment thereof, even though biochemical background and the long-term prognosis of the disease are not well understood. This review also includes a 4-year-long history of a patient that featured muscular pains, weakness and multiple stress fractures localised in the hips and vertebra with subsequent recovery after tumour resection. Because the occurrence of such a condition is rare, it may take years to correctly diagnose the disease, as is reported in this case report.

Malignancy after Renal Transplantation: A Single-Center Experience

BACKGROUND The aim of this study was to evaluate the incidence and characteristics of malignant tumors in kidney transplant recipients (KTR) in Lithuania and to access the changes in KTR survival after developing cancer. We also analyzed and compared results with data from other centers worldwide. MATERIAL AND METHODS We performed a retrospective cohort study of all 395 patients transplanted at Renal Transplantation Center of Vilnius University Hospital Santariškių Klinikos (RTC of VUHSK) between 1 January 2000 and 31 December 2010. RESULTS Mean age at transplantation was 40.33 ± 11.46 years; 54.9% of recipients were male, 45.1% female; 23 (5.8%) recipients developed 25 malignancies, of which 1.5% had urinary system cancer, 0.8% had non-melanoma skin cancer, hematolymphopoetic cancer, or cancer of gastrointestinal tract, and 0.5% developed cancers of female reproductive system, breast, central nervous system cancer, or had more than 1 malignancy. Average time to first malignancy was 46.7 months. Cumulative incidence of malignancy was 1.8%, after 1 year, 4% after 5 years, and 14.2% after 10 years. There were 32 patients (8.1%) with pre-malignant lesions. Recipients older than 45 years had higher frequency of malignancies (p = 0.005). KTR who developed gastrointestinal cancer had significantly shorter survival time than patients without malignancy (p = 0.01). Recipients who had been on dialysis for more than 35 months also had a significantly shorter survival (p=0.001). CONCLUSIONS Older patients had higher risk for developing malignancies, and recipients with gastrointestinal cancer had the worst survival. That suggests we need better screening programs for this type of cancer and for older patients at RTC of VUSHK.

Utility of renal biopsy in the clinical management of renal disease: hematuria should not be missed

To the Editor: In a recent issue, Dhaun et al.1 reviewed the utility of renal biopsy in the clinical management of renal disease. The article describes standard and expanded indications for renal biopsy, such as renal biopsy in the elderly, in those with diabetes or ‘hypertensive nephropathy’ and even renal biopsy in advanced chronic kidney disease (CKD). However, the isolated hematuria should also not be missed. Typically, nephrologists tend to diagnose, monitor, and treat another fundamental manifestation of glomerular injury, proteinuria. Persistent glomerular hematuria is very common in daily clinical practice and is typically due to thin basement membrane nephropathy (TBMN) or less often from Alport syndrome (AS).2 AS and TBMN may be clinically indistinguishable. The differentiation is, however, of great importance due to different risks of CKD for the individual and their family members. A group of European, American, and Australian experts in their recent guidelines recommend renal biopsy in suspected AS for glomerular basement membrane (GBM) ‘ultrastructure, collagen IV composition, and an assessment of damage’.3 The tissue distribution and trimer composition of the different collagen IV molecules are well known. The diagnosis of AS is highly likely if GBM lacks the collagen IV a5 chain, and in most cases, immunohistochemical staining for the alpha-3 and alpha-5 chains will be pathognomonic. The same guidelines state that ‘individuals suspected of having TBMN should undergo renal biopsy if they have atypical features … or if X-linked Alport syndrome … cannot be excluded’.3

A Perspective From the Baltics Regarding the Canadian Society of Nephrology Commentary on the KDIGO Glomerulonephritis Guideline

To the Editor: In their commentary on the 2012 KDIGO (Kidney Disease: Improving Global Outcomes) clinical practice guideline recommendations for glomerulonephritis (GN) in adults, Cybulsky et al correctly identify gaps in current knowledge regarding GN management. However, their statement that “GNs collectively account for approximately one-quarter of end-stage renal disease (ESRD)” is an underestimation. GN-associated ESRD is much more common in sub-Saharan Africa and Asia. Further, community-wide incidence or prevalence of GN can be estimated only from kidney biopsy series, which are subject to bias because of differing criteria for biopsy. Moreover, the authors state that levels of a soluble form of urokinase-type plasminogen activator receptor (suPAR) are elevated in FSGS [focal segmental glomerulosclerosis] and that suPAR and other “novel biomarkers may provide insights into the pathogenesis of the disease and its activity, predict remission more accurately, and may lead to mechanism-based therapeutics.” However, recent data show that serum suPAR levels are not reliable in distinguishing FSGS from other proteinuric glomerular diseases. Reduced estimated glomerular filtration rate makes suPAR an unreliable diagnostic biomarker in FSGS, and serum suPAR is influenced markedly by systemic inflammation, acute illnesses, cancer, and diabetes. Thus, serum suPAR cannot be considered a valid biomarker for either primary or secondary FSGS. Despite these concerns, I generally agree with Cybulsky et al, especially in the section on pauci-immune focal and segmental necrotizing GN, in which the authors point out that the guideline does not discuss glucocorticoids for maintenance therapy or the relative cost-effectiveness of rituximab. I concur that cost should be heeded when considering mycophenolate mofetil treatment for resistant disease. And, the authors’ observation that “[t]he challenge will be how to incorporate.management suggestions into.everyday practice” holds true for Lithuanian clinicians as well.

A Successful Implementation of Laparoscopic Living Donor Nephrectomy: First Experience in Lithuania

Introduction Almost two decades ago laparoscopic living donor nephrectomy (LLDN) technique was developed to reduce living donor complication rate. However, the requirement of experienced surgeons, the use of mainly left donor kidney and technical challenges for multiple arteries are the main limitations that inhibit the application of LLDN technique in some centers. We aimed to share the initial results of LLDN in high volume university center, which is performing laparoscopic nephrectomies for other indications. Materials and Methods During year 2017, we performed 4 LLDN. Transperitoneal approach was used in all cases and kidney was removed using suprapubic incision. All donors and recipients were prospectively analyzed within 6 months follow-up. Patient clinical, laboratory, operation-related data were collected from directly interviewing them and from medical records. All patients have their written informed consent. Results and Discussion 1 male and 3 female donated left kidney by using LLDN technique. Mean age 58 ∓9 y, 2 of them with history of previous cholecystectomy. All donated kidney had a single renal artery and renal vein. Pre-operative average eGFR was 94.2 ∓7.1 ml/min/1.73m2, immediately after LLDN – 57.5 ∓10.3 ml/min/1.73m2, after 1 month – 56.0 ∓9.1 ml/min/1.73m2. There were no intraoperative complications, surgery duration 223.75 ∓21.74min, cold ischemia time was 77.5 ∓28.77 min, warm ischemia time – 6.37 ∓3.14 min. The donors were discharged within 5-6 days after LLDN. Postoperative donor complications: acute kidney injury – 1; prolonged postoperative abdominal pain – 1. Posttransplant recipient complication: acute kidney rejection – 1 case of atypical hemolytic syndrome; no cases of delayed graft function. Conclusion Our initial experience confirms that LLDN is an approach which is easy to learn, especially in a high volume university hospital with expertise in performing laparoscopic nephrectomies for other indications. Therefore, we would like to encourage centers, which are still performing open living donor nephrectomies, to introduce laparoscopic method into everyday practice. We believe, that changing the nephrectomy technique could increase living donation numbers in the center.

Usefulness of pretransplant aortic arch calcification evaluation for kidney transplant outcome prediction in one year follow-up

Abstract Vascular calcification (VC) is linked to post-transplant cardiovascular events and hypercalcemia which may influence kidney graft function in the long term. We aimed to evaluate whether pretransplant aortic arch calcification (AoAC) can predict post-transplant cardiovascular or cerebrovascular events (CVEs), and to assess its association with post-transplant plasma calcium levels and renal function in one-year follow-up. Our single-center observational prospective study enrolled 37 kidney transplant recipients (KTR) without previous history of vascular events. Two radiologists evaluated pretransplant AoAC on chest X-ray as suggested by Ogawa et al. in 2009. Cohen’s kappa coefficient was 0.71. The mismatching results were repeatedly reviewed and resulted in consensus. Carotid-femoral (cfPWV) and carotid-radial pulse wave velocity (crPWV) was measured using applanation tonometry before and one year after transplantation. Patient clinical, biochemical data, and cardiovascular/CVE rate were monitored within 1 year. We found out that eGFR1year correlated with eGFRdischarge and calcium based on hospital discharge data (β = 0.563, p = .004 and β = 51.360, p = .026, respectively). Multivariate linear regression revealed that donor age, donor gender, and recipient eGFRdischarge (R-squared 0.65, p = .002) better predict eGFR1year than AoAC combined with recipient eGFRdischarge (R-squared 0.35, p = .006). During 1-year follow-up, four (10.81%) patients experienced cardiovascular events, which were predicted by PWV ratio (HR 7.549, p = .045), but not related to AoAC score (HR 1.044, p = .158). In conclusion, KTR without previous vascular events have quite low cardiovascular/CVE rate within 1-year follow-up. VC evaluated as AoAC on pretransplant chest X-ray together with recipient eGFRdischarge could be related to kidney function in one-year follow-up.

A case of successfully treated relapsing peritoneal dialysis-associated peritonitis caused by Gordonia bronchialis in a farmer

Gordonia species are aerobic, weakly acid-fast, Gram-positive pathogens that rarely cause human infections, usually in immunocompromised patients. It is uncommon bacilli in cases of peritoneal dialysis-related peritonitis. The small number of infections with Gordonia species reported for humans may be stipulated by the difficulty in identifying the organism using conventional techniques. Careful review of Gram stains and modified-acid-fast stains should be done, so that confusion with other actinomycetes is minimized, pending the genotypic identification. Here we report a case that was caused by Gordonia bronchialis and thus required different considerations of treatment.

[LB.01.16] FACTORS RELATED TO LEFT VENTRICULAR HYPERTROPHY IN ONE YEAR FOLLOW-UP OF HEMODIALYSIS PATIENTS

Objective: The phenomenon of cardiac remodeling in dialysis population which leads to high prevalence of cardiovascular disease is every day burden for nephrologists. We aimed to analyze which factors could better indicate the presence of left ventricular hypertrophy in dialysis population. Design and method: 60 stable hemodialysis patients were screened for a prospective study. 45 patients had all data and met the inclusion criteria (no previous cardiovascular events, no cerebrovascular events). The calculated effect size for this population is 0.4052 (&agr; 0.05, &bgr; 0.90). Blood tests (including beta2-microglobulin, cystatin C), carotid femoral pulse wave velocity (cfPWV), left ventricular mass (LVM) and index, left ventricular end diastolic diameter, relative wall thickness, mean wall thickness and left ventricular ejection fraction were evaluated twice, average 1 year apart was also tested. Results: LVM and LVM index correlated with cystatin C level (r = 0.5141, p = 0.0085 and r = 0.4381, p = 0.0284 respectively), beta2-microglobulin concentration (r = 0.5759, p = 0.0025 and r = 0.5136, p = 0.0086 respectively). Change in LVM and LVM index within one year follow-up correlated with change in cfPWV (r = 0.5201, p = 0.0468 and r = 0.5518, p = 0.0266 respectively). Left ventricular end diastolic diameter, and MWT also strongly correlated with beta2-microglobulin and cystatinC. The strongest relationship revealed by linear regression indicated that for an increase of 1 m/s in cfPWV the expected increase in LVM index is 5.505 g/m2 (r-squared 0.3695, p = 0.3244, 95%CI [1.38;9.62]). During one year follow up the reverse cardiac remodeling was observed in some patients. Conclusions: Change in carotid-femoral pulse wave velocity within 1 year in dialysis population without previous cardiovascular or cerebrovascular events is associated with change in LVM index and has better descriptive value than cystatin C or beta2-microglobulin.

[LB.02.18] DIFFERENCE BETWEEN CAROTID-FEMORAL AND CAROTID-RADIAL PULSE WAVE VELOCITY CAN INDICATE THE EXTENT OF AORTIC ARCH CALCIFICATION IN DIALYSIS PATIENTS

Objective: Aortic arch calcification (AoAC) score evaluated on plain chest X-ray was proven to be associated with increased cardiovascular risk. We aimed to determine whether the difference between carotid-femoral pulse wave velocity (CFPWV) and carotid-radial pulse wave velocity (CRPWV) could describe the extent of AoAC in dialysis patients. Design and method: This cross-sectional study included 61 dialysis patients without previous vascular events. They all underwent chest X-ray. Two radiologists blinded to patient medical records graded aortic arch calcification (AoAC) by using a scale from 0 to 3 (grade0–no visible calcification, grade1 - < 50% calcification in the arch, grade2 - >50% calcification, grade3–circumferential calcification). These patients had their CFPWV and CRPW measured and the difference (&dgr;PWV=CFPWV-CRPWV) calculated. Biochemical blood tests included creatinine, urea, uric acid, cystatin C, ferritin, protein, albumin, C-reactive protein (CRP), calcium, phosphate, parathormone, total cholesterol, haemoglobin, platelets, white blood cells. Corrected total calcium and calcium phosphate products were calculated. According to AoAC grade patients were divided into 3 groups: group A–AoAC grade0, group B–grade1, group C–grade2, 3. Results: Patients with AoAC were significantly older, shorter in height, had higher than 25 kg/m2 body mass index (BMI), lower peripheral and central diastolic BP, lower end systolic BP, higher CRP levels. Group B had significantly lower phosphate and calcium phosphate product levels. We observed that patients with AoAC had higher CFPWV, but lower CRPWV values. &dgr;PWV was as follows: A -0,05, B 1,72, C 4,23, p = 0,115. On multivariate logistic regression models group A was significantly associated with &dgr;PWV (OR 0.48), albumin level (OR 0.69), diastolic BP (OR 1.13), BMI (OR 0.68) and time on dialysis (OR 0.99); group C–with &dgr;PWV (OR 1.73) and phosphate (OR 1.25). In group B the association was only with CRPWV (OR 0.50), BMI (OR 1.17), &dgr;PWV had no significant influence. Conclusions: The discrepancy between CFPWV and CRPWV measurement confirms different pattern of elastic vessels (Aorta) and muscular conduit arteries (radial arteries) and can describe the extent of aortic arch calcification in dialysis patients.

Successful adsorption of anti-A/B antibodies with multiple personal use columns in AB0 incompatible kidney recipients: A single centre experience.

Laurynas Rimsevicius,* Roberta Bagarauskyte, Antanas Griskevicius, Judita Audzijoniene, Laimonas Griskevicius, and Marius Miglinas Nephrology Center, Vilnius University Hospital Santariskiu Klinikos, Vilnius 08661, Lithuania Clinic of Gastroenterology, Nephrourology and Surgery, Faculty of Medicine, Vilnius University, Vilnius 03101, Lithuania Hematology, Oncology and Transfusion Medicine Center, Vilnius University Hospital Santariskiu Klinikos, Vilnius 08661, Lithuania Clinics of Internal, Family Medicine and Oncology, Faculty of Medicine, Vilnius University, Vilnius 03101, Lithuania