Renal involvement in malignant plasmacytoma

Malignant plasmacytoma (MP) (multiple myeloma) is a clonal B cell disease affecting slow-release plasmatic cells accompanied by monoclonal paraprotein production followed by lytic bone lesions. Nearly 50% of the newly diagnosed patients have a reduced glomerular filtration rate and about 9% require dialysis treatment due to severe renal impairment. The aim of this study is to direct nephrologists` attention to seeking and diagnosing patients with unclear fast progression of CKD and to highly sensitive methods of proving especially light chainsin urine. Early diagnosis and treatment, rapid and effective reduction in the number and removal of light paraprotein chains by aggressive chemotherapy and/or apheresis may reduce the risk of renal impairment and prevent end-stage renal failure. Rapid and timely diagnosis as well as effective treatment of renal failure combined with conservative and interventional therapy reduces severe complications and significantly prolonged survival in patients with malignant plasmacytoma, delaying the progression of renal involvement.Malignant plasmacytoma (MP) (multiple myeloma) is a clonal B cell disease affecting slow-release plasmatic cells accompanied by monoclonal paraprotein production followed by lytic bone lesions. Nearly 50% of the newly diagnosed patients have a reduced glomerular filtration rate and about 9% require dialysis treatment due to severe renal impairment. The aim of this study is to direct nephrologists` attention to seeking and diagnosing patients with unclear fast progression of CKD and to highly sensitive methods of proving especially light chainsin urine. Early diagnosis and treatment, rapid and effective reduction in the number and removal of light paraprotein chains by aggressive chemotherapy and/or apheresis may reduce the risk of renal impairment and prevent end-stage renal failure. Rapid and timely diagnosis as well as effective treatment of renal failure combined with conservative and interventional therapy reduces severe complications and significantly prolonged survival in patients with malignant plasmacytoma, delaying the progression of renal involvement

Renal involvement in hyperuricemia

Hyperuricemia is a condition characterized by elevated levels of serum uric acid (SUA) in the blood. It is a chronic metabolic disorder due to purine metabolism disorders that may be primary or induced by other pathogenetic disorders. It is an important risk factor for the development of cardiovascular (CHD) and chronic kidney disease (CKD). The meta-analysis of two studies with adjusted risk assessments indicates that hyperuricemia is independently associated with an increased risk of peripheral neuropathy in patients with type 2 diabetes.The decrease in SUA values with xanthine oxidase inhibitors has been associated with a favorable response in regard to arterial blood pressure, arterial rigidity, vascular wall thickening, oxidative stress, left ventricular hypertrophy and renal function.The development of hyperuricemia occurs by way of two main mechanisms:1. Increased purine synthesis;2. Decreased kidney elimination.The subject of this work is the early and specific manifestations of hyperuricemia, mechanisms of kidney involvement and methods of treatment depending on the degree of glomerular filtration.Together with hypertension, obesity, dyslipidemia and insulin resistance, hyperuricemia is an invariable part of the metabolic syndrome and increasing cardiovascular risk that impacts renal function. Any increase in SUA by 60 μmol/L significantly increases cardiovascular mortality and overall mortality, and this dependence remains significant after considering the impact of all potential factors. Timely and adequate treatment of hyperuricemia leads to improvement of endothelial function, decrease of elevated blood pressure, retardation of the progression of renal involvement and prevention of the worsening of glomerular filtration rate.Hyperuricemia is a condition characterized by elevated levels of serum uric acid (SUA) in the blood. It is a chronic metabolic disorder due to purine metabolism disorders that may be primary or induced by other pathogenetic disorders. It is an important risk factor for the development of cardiovascular (CHD) and chronic kidney disease (CKD). The meta-analysis of two studies with adjusted risk assessments indicates that hyperuricemia is independently associated with an increased risk of peripheral neuropathy in patients with type 2 diabetes.The decrease in SUA values with xanthine oxidase inhibitors has been associated with a favorable response in regard to arterial blood pressure, arterial rigidity, vascular wall thickening, oxidative stress, left ventricular hypertrophy and renal function.The development of hyperuricemia occurs by way of two main mechanisms:1. Increased purine synthesis;2. Decreased kidney elimination.The subject of this work is the early and specific manifestations of hyperuricemia, mechanisms of kidney involvement and methods of treatment depending on the degree of glomerular filtration.Together with hypertension, obesity, dyslipidemia and insulin resistance, hyperuricemia is an invariable part of the metabolic syndrome and increasing cardiovascular risk that impacts renal function. Any increase in SUA by 60 μmol/L significantly increases cardiovascular mortality and overall mortality, and this dependence remains significant after considering the impact of all potential factors. Timely and adequate treatment of hyperuricemia leads to improvement of endothelial function, decrease of elevated blood pressure, retardation of the progression of renal involvement and prevention of the worsening of glomerular filtration rate

Selective Adsorption Plasma Therapy in Some Autoimmune Diseases with Kidney Lesions /// Селективна адсорбционна плазма-терапия при някои автоимунни заболявалия с бъбречни лезии

[EN] Approximately half of patients with terminal kidney failure suffer from one or another form of glomerulonephritis. To a significant extent, glomerular diseases result from primary or secondary glomerular involvement in a number of systemic autoimmune diseases. Most often is mediated by an immune mechanism involving the deposition of antibodies and / or immune complexes in glomeruli. Tissue deposition of autoantibodies and immune complexes is most often followed by rapid deterioration of renal, pulmonary or other organ function and is the beginning of a series of serious immune disorders. Typical clinical manifestation in such cases are the lesions induced by rapidly progressive glomerulonephritis in systemic autoimmune diseases and in some types of vasculitis. Treatments for autoimmune disease still vary widely: from Therapeutic nihilism to intense pathogenetic therapy. Isolation of pathogenic autoantibodies has become a basic principle in the combined therapeutic strategy in a significant number of nephrological diseases with proven or suspected humoral mediated immune pathogenesis. Purpose of the study: To evaluate selective adsorption plasma therapy in the treatment of autoimmune diseases with kidney lesions. Comparative study on the effect of application of three different methods - combination of drug pathogenetic treatment with selective adsorption plasma therapy, plasmapheresis and self-administration of drugs pathogenetic treatment for a period of six months was carried out for the first time. The advantages of selective adsorption plasma therapy in combination with the drug pathogenetic treatment before the self-administered drug pathogenetic treatment have been established.[BG] Приблизително половината от пациентите с терминална бъбречна недостатъчност страдат от една или друга форма на гломерулонефрит. В значителна степен гломерулните заболявания са резултат на първично или вторично гломерулно засягане при някое от многобройните системни автоимунни заболявания. Повечето гломерулонефрити са медиирани от имунен механизъм включващ отлагане на антитела и/или имунни комплексни формации в гломерулите. Тъканното отлагане на автоантителата и имунните комплекси най-често е последвано от бързо влошаване на бъбречната, белодробната или функцията на други органи и е начало на поредица от сериозни имунни разстройства. Типично клинично проявление в такива случаи са тъканните лезии предизвикани от бързопрогерсиращия гломерулонефрит при системни автоимунни забоявания и при някои видове васкулити. Методите за лечение на автоимунните заболявания все още варират в твърде широки граници-от терапевтичен нихилизъм до интензивно патогенетично поведение. Извличането на патогенните автоантитела е станало вече основен принцип в комбинираната терапевтичната стратегия при значителен брой нефрологични заболявания с доказана или подозирана хуморално медиирана имунна патогенеза. Цел на изследването: Да се оцени мястото на селективната адсорбционна плазма-терапия при лечението на автоимунни заболявания с бъбречни лезии. Проведено е за първи път сравняване на ефекта от приложението на три различни метода - комбинация от медикаментозно патогенетично лечение със селективна адсорбционна плазма-терапия, с плазмафереза и самостоятелно приложение на медикаментозно патогенетично лечение за период от шест месеца. Установени са предимствата на селективната адсорбционна плазма-терапия в комбинация с медикаментозното патогенетично лечение пред самостоятелно приложеното медикаментозно патогенетично лечение

Calciphylaxis

Calciphylaxis is a rare, often fatal, systemic disorder characterized by deposition (precipitation) of calcium hosphate salts (calcification) in the medial layer of the arteries and soft tissues. Calcification of the media is followed by fibrous hyperplasia of the intima with obliteration of the lumen and tissue ischemia, necrosis, and gangrene. The first description of calciphylaxis was published in 1962. Calciphylaxis occurs in patients with end stage renal disease. Almost all reported patients were on dialysis.Scripta Scientifica Medica 2007;39(1):49-5

MONITORING OF CMV INFECTION IN KIDNEY TRANSPLANT RECIPIENTS

Human cytomegalovirus is a ubiquitous herpesvirus that establishes lifelong latency after primary infection, but can cause life-threatening disease in immunosuppressed patients. CMV invasive disease leads to significant morbidity and mortality following kidney transplantation. We tested 2 groups of patients - Group A included 20 potential kidney recipients and 29 potential donors investigated by ELISA and Group B included 53 adult kidney transplant recipients all of them tested in ELISA and 24 of them tested in QRT-PCR for CMV-DNA from plasma samples. In group A 16 (80%) of 20 potential kidney recipients were anti-CMV IgG positive and 4 (20%) were anti-CMV IgG negative. Twenty eight of 29 potential donors were found seropositive, and only one was not infected. In group B overall 119 ELISA tests for specific anti-CMV antibodies were performed. Anti-CMV IgM negative was 68 (57%) of the tested samples, twelve (10%) showed anti-CMV IgM equivocal results and 39 samples (33%) were with anti-CMV IgM positive. Seven of them (13,2%) showed repeatedly anti CMV IgM positive results. All 119 (100%) displayed аnti-CMV IgG positive results. Overall 41 PCR analyses from plasma samples of 24 kidney transplant recipients (group B) were performed. CMV-DNA replication was detected in 5 plasma samples obtained from 3 patients (12.5%) at a different time - from 20 days till almost 8 years after the transplantation. Despite the high seroprevalence to CMV 20% of the potential recipients were at high risk of primary infection when receiving a kidney from a seropositive donor. Positive serological results during the regular post-transplantation monitoring complemented with or without clinical data are indicative and require further QRT-PCR analysis

INFLAMMATORY MYOFIBROBLASTIC TUMOUR OF THE KIDNEYS - CASE REPORT

Introduction: Inflammatory myofibroblastic tumour (IMT) is a rare condition which includes proliferation of myofibroblasts accompanied with inflammatory infiltrates. It is known to have a wide age range and to affect both males and females. Although its most common site is the lungs, IMT also has extrapulmonary locations which most often include the bladder and rarely the kidneys. Its biological nature varies from a completely benign to a malignant tumour with fatal outcome.Material and Methods: We report a case of a 61-year-old woman, admitted to a hospital with high temperature and anaemia, five months after left nephrectomy due to a myofibroblastic tumour of the kidney. The performed laboratory tests revealed a significant impairment of the GFR and anaemic syndrome. The conducted MRI-scan found multiple hypodense lesions in the right kidney with enlarged paraaortical lymph nodes. PET-scan and CT-scan confirmed the findings. The results of the examinations illustrated a slow progressive chronic failure of the solitary kidney, which suggested a paraneoplastic process due to recurrence of the IMT.Results: Because of the high CRP rates, the patient underwent an antibiotic therapy with Meropenem, without any clinical and laboratory improvement and deterioration of the signs of inflammation. The findings of hypodense lesions and chronic failure combined with the contraindication of nephrectomy, because of a solitary kidney, suggested the admission of Prednisolone, as this type of tumour is cortisol-sensitive. In connection with the glucocorticoids usage, the patient is scheduled for bone density test. She remains under constant observation.Conclusions: The case presents an uncommon occurrence of inflammatory myofibroblastic tumour with the kidneys as a site of progression. It illustrates the recurrent nature of this type of neoplasm and the complications that it brings, according to the organs, which affects

Messung der Parameter der Stimmbelastung für Sing- und Sprechstimme

Hintergrund: Die Stimme als Kommunikations- und Ausdrucksmittel hat im heutigen Informationszeitalter große Bedeutung: Immer mehr Menschen sind von einer uneingeschränkten und belastbaren Stimme abhängig. Die Einschätzung des Grades der stimmlichen Belastung in verschiedenen Expositionsszenarien ist demnach zunehmend von Interesse für die Diagnostik und damit einhergehend die Prävention von Stimmstörungen, die aufgrund einer zu hohen stimmlichen Belastung entstehen können.Material und Methoden: Mit einem Messgerät zur Quantifizierung der stimmlichen Belastbarkeit - VoxLog® - wurde die Stimmbelastung bei zehn professionellen Sängerinnen und Sängern eines Chores beim Singen desselben Arrangements gemessen. Ermittelt wurden neben der Grundfrequenz (f0), dem Schalldruckpegel (SPL, sowohl für das Stimmsignal als auch für Umgebungsgeräusche) und der Phonationszeit auch die gängigsten Stimmdosen (Voice Dosimetry).Ergebnisse: Die Analyse des Notentextes lieferte Ergebnisse, die durch die Messungen bestätigt werden konnten. Im Direktvergleich ergaben die Messungen mit Blick von höheren zu tieferen Stimmen abfallende Grundfrequenzen bei sich weitestgehend entsprechenden Schalldruckpegeln und gleicher Phonationszeit. Dies spricht für eine höhere stimmliche Belastung der Frauenstimmen im Vergleich zu den männlichen Sängern.Fazit: Die Untersuchungen lieferten wichtige Daten, mit denen die Ergebnisse späterer Studien verglichen werden können. Die Berechnung der Stimmdosen lieferte nachvollziehbare, fassbare Werte, die unterschiedliche Parameter miteinander verbinden und die einwirkende Stimmbelastung realitätsnah abbilden. Hier ist für die Zukunft weiterer Forschungsbedarf anzumerken: Das Ziel zukünftiger Forschung ist, die stimmliche Belastung in unterschiedlichen Situationen zu untersuchen und die Ergebnisse miteinander in Relation zu setzen

Antibodies recognizing globular domain of C1q - current view on the association between lupus nephritis manifestation and anti-gC1q autoantibodies

Introduction: Lupus nephritis (LN) is a serious complication of the systemic lupus erythematosus (SLE). Anti-C1q antibodies correlate with the occurrence and high clinical activity of LN, especially proliferative LN. The first reported anti-C1q antibodies recognized autoepitopes within collagen-like region (CLR) of C1q. Recently we have found autoantibodies against globular C1q domain (gC1q antibodies) in LN patients.The aim of the present study was to evaluate the potential pathological consequences of the presence of anti-gC1q antibodies in LN.Material and Methods: The recombinant globular head region of the three chains of C1q -A, -B and -C were expressed in E. coli BL21 and purified. Anti-C1q, anti-gC1q autoantibodies, complement proteins - C1q, C4, C3 and IgG-, IgM-CICs levels were screened by ELISA in 53 sera from LN patients. Sera from 196 normal controls served as controls.Results: We found that patients positive for anti-B-gC1q antibodies presented with significantly lower serum C4 levels than patients positive for anti-A and anti-C-gC1q antibodies (p = 0.014) and with significantly lower levels of C3 than patients positive for anti-A and anti-C-gC1q antibodies and patients without anti-C1q antibodies (p = 0.005; p = 0.018). Significant correlations to IgG CICs were detected for anti-C1q (r = 0.371, p = 0.001) and anti-B-gC1q antibodies (r = 0.431, p = 0.003).Conclusions: These findings suggest that the binding of anti-B-gC1q autoantibodies with C1q may possibly trigger mechanical stress and induce a structural change within the CLR domain of C1q, compatible with C1r-C1s complement activation in the fluid phase

PREVALENCE OF VITAMIN D DEFICIENCY IN DIFFERENT GROUPS OF CHRONIC RENAL FAILURE PATIENTS.

Purpose: To determine and compare the vitamin D status of different groups CKD patients on hemodialysis, peritoneal dialysis, or no renal replacement therapy and to evaluate the effect of vitamin D therapy. Patients and Methods: This pilot study enrolled 40 consecutive CKD patients (21 men, 19 women) divided into three groups: 15 CKD patients in 1,2,3,4 stage of the disease without renal replacement therapy (RRT); 10CKD patients on hemodialysis (HD) and 15 CKD patients on peritoneal dialysis (PD), ten of which were on vitamin D therapy. Vitamin D status was determined by serum 25-xydroxyvitamin D (25OHD). Results: Ninety percent of patients were in vitamin D deficiency/insufficiency; and only 4 patients (10.0%) reached 25OHD levels above 75nmol/L. The median 25OHD level was 31.15nmol/L (interquartile range: 16.67-48.33nmol/L).Tendency of worse vitamin D status in women than in men was observed. Higher 25OHD levels were found in pre-dialysis patients (median 44.81nmol/L, 25%-75% percentile 16.24-52.21nmol/L) and lower in HD (median 31.15nmol/L, 25%-75% percentile 13.04-64.45nmol/L) and PD patients (median 33.38nmol/L, 25%-75% percentile 23.15-48.49nmol/L), but the difference did not reach statistical significance. Better vitamin D status was found in the PD group of patients receiving vitamin D preparations (p<0.05). Conclusions: 25OHD deficiency/insufficiency is prevalent in renal failure patients with or without renal replacement therapy. It seems that vitamin D therapy improves the vitamin D status of PD patients. Further larger studies are needed to clarify the effect of specific type vitamin D therapy on serum 25OHD levels and clinical outcome in different groups of CKD patients