Search

Life-threatening lingual haemorrhage was successfully treated in a 3-year-old haemophiliac who had a high level of inhibitor in his plasma. Repeated plasma exchange with high-dose factor VIII concentrate led to a cessation of bleeding. Immunosuppressive treatment introduced at the time of plasmapheresis and carried out for 6 weeks seems to have prevented the return of the inhibitor. [ABSTRACT FROM AUTHOR]

In light chain amyloidosis (LA), the massive glomerular and vascular amyloid deposition leading to interstitial fibrosis/tubular atrophy (IFTA) is thought to be responsible for renal failure. The amyloid deposition in the interstitium and the tubular basement membrane (TBM) has received less attention in the study of LA. We, therefore, collected clinical and laboratory data on patients diagnosed with LA in our Nephrology Department and studied amyloid deposition in the TBM. Twelve LA patients were diagnosed by renal biopsy during a seven-year period. In 4 of the 12, amyloid deposition could also be detected in the TBM. In our first case of a patient with diabetes mellitus, non-amyloid fibrils resembling 'diabetic fibrillosis' were also seen by electron microscopy. Despite the double damage, IFTA was negligible, blood vessels were unaffected, and the glomerular deposition was segmental. In the other three cases, significant (>50%) IFTA and a severely reduced estimated glomerular filtration rate were already detected at the time of diagnosis and amyloid deposition was also observed in the blood vessels. These findings indicate the importance of TBM amyloid deposition in the progression of renal disease. This may represent a late-stage presentation of the disease with a heavy LC burden.

Összefoglaló. A jelenlegi hazai gyakorlatban sokszor indokolatlanul korlátozzák a vesebetegek kontrasztanyagos vizsgálatát, és halasztódik a metformint szedők vizsgálata is, kontrasztanyag által okozott akut vesekárosodástól (contrast-induced acute kidney injury, CI-AKI) tartva. Összefoglalónk célja az ezzel kapcsolatos újabb ismeretek áttekintése és egy szakmai javaslat ismertetése annak érdekében, hogy a betegellátás szempontjából fontos vizsgálatok ne maradjanak el, ugyanakkor azok a maximális betegbiztonság jegyében készüljenek. Az elmúlt évek tanulmányai alapján a CI-AKI előfordulása a korábbinál kevésbé gyakori, és jelentősen különböző a kontrasztanyag intravénás vagy intraarteriális alkalmazásától függően. Legfontosabb rizikótényezője a csökkent glomerulusfiltrációs ráta (GFR), mely stabil állapotú vesebetegnél, intravénás kontrasztanyag adásakor 30 ml/min/1,73 m2 alatt, intraarteriális alkalmazásakor 45 ml/min/1,73 m2 alatt képez magas rizikót. Proteinuria esetén a CI-AKI és a kontrasztanyaggal társult akut vesekárosodás (contrast-associated kidney injury, CA-AKI) kockázata is nagyobb, ezért a számított GFR mellett indokolt a vizelet albumin/kreatinin vagy fehérje/kreatinin hányados meghatározása is a vizsgálat előtt. Az instabil állapot, az akut veseelégtelenség mindenkor magas kockázatot jelent, ilyenkor a számított GFR pontatlan, nem használható. Csökkent vesefunkció mellett figyelni kell a beadott kontrasztanyag mennyiségére, a vizsgálat 48-72 órán belüli ismétlésének kerülésére, a nemszteroid gyulladásgátlók vagy más nephrotoxicus szerek lehetőség szerinti szüneteltetésére. Prevenciós intézkedés a magas rizikóval bíró betegek esetében javasolt intravénás hidrálás formájában, fiziológiás koncentrációjú nátrium-klorid vagy nátrium-bikarbonát infúziójával. Az egyéb eljárások hatástalanok, és nem indokolt a beavatkozás utáni dialízis végzése sem végstádiumú veseelégtelen betegekben. A metformint 60 ml/min/1,73 m2 feletti eGFR-rel rendelkező beteg vizsgálata kapcsán szükségtelen elhagyni, ettől rosszabb veseműködés esetén kell szüneteltetni. Amennyiben a vizsgálat indikációja sürgősségi, az a metformin egyidejű elhagyásával elvégezhető, de a gyógyszer csak 48 óra múlva, az akut vesekárosodás kizárását követően adható vissza. Orv Hetil. 2022; 163(3): 83-91. Summary. In the current clinical practice, studies with iodinated contrast agents are often limited in patients with kidney disease and delayed in those on metformin therapy due to fear of contrast-induced acute kidney injury (CI-AKI). We aim to review the most recent information about CI-AKI and provide recommendations in order to avoid cancellation of important contrast-enhanced tests, but maximize safety considerations. According to the most recent findings, CI-AKI occurs less frequently nowadays than previously, and depends significantly on the route of contrast administration (intraarterial or intravenous). The most important risk factor is the decreased GFR, which, in stable patients with intravenous contrast administration provides high risk if the eGFR is less than 30 ml/min/1.73 m2, and with intraarterial contrast is less than 45 ml/min/1.73 m2. In patients with proteinuria, the risk of both CI-AKI and CA-AKI (contrast-associated kidney injury) is increased, therefore urinary albumin/creatinine or protein/creatinine ratios are recommended to measure before the contrast material administration, beside the eGFR determination. Unstable condition, acute renal failure always mean high risk; in these cases, eGFR calculation is imprecise and useless. If renal function is decreased, the amount of contrast material needs consideration, repeated contrast-enhanced studies should be avoided in 48-72 hours, the non-steroidal anti-inflammatory agents and other nephrotoxic drugs have to be discontinued. For high risk patients, preventive intravenous hydration should be given, either by physiologic saline or sodium bicarbonate infusion. Other drugs aiming prevention have proved to be useless; dialysis treatment immediately after contrast administration in end-stage renal disease patients is unnecessary. There is no indication to discontinue metformin if eGFR is higher than 60 ml/min/1.73 m2, but if the patient has less than that value, the metformin needs to be stopped. In urgent studies with contrast agent, metformin administration has to be discontinued simultaneously with the intervention, and this drug can only be readministered after ruling out acute kidney injury in 48 hours following contrast exposure. Orv Hetil. 2022; 163(3): 83-91.

The introduction of Bruton's tyrosine kinase inhibitor ibrutinib has made a significant progress in the treatment of chronic lymphocytic leukemia and other B-cell malignancies. Due to the reduction of cytokine release, it is effective in chronic graft-versus-host disease, and its use has also been suggested in autoimmune diseases and in prevention of COVID-19-associated lung damage. Despite this effect on the immune response, we report a severe hypersensitivity reaction in a 76-year-old male patient diagnosed with prolymphocytic leukemia. Four weeks after the ibrutinib start, non-oliguric acute kidney injury with proteinuria and microscopic hematuria developed and that was accompanied by lower limb purpuras and paresthesia. Renal biopsy revealed acute interstitial nephritis. Employing 1 mg/kg methylprednisolone administration, serum creatinine decreased from 365 μmol/L to 125 μmol/L at 11 days and the proteinuria-hematuria as well as the purpura, paresthesia resolved. Three months later at stabile eGFR of 56 ml/min/1.73 m 2 methylprednisolone was withdrawn and a rituximab-venetoclax treatment was initiated without side effects. We conclude that despite the beneficial effect on cytokines response in Th1 direction, ibrutinib can cause acute interstitial nephritis. Early detection, discontinuation of ibrutinib, glucocorticoid administration may help to better preserve renal function, thereby lowering the risk of potential subsequent kidney injury.

Introduction: Patients with renopulmonary syndrome who have both anti-neutrophil cytoplasmic and anti-glomerular basement membrane antibodies have been described since 1989., Aim: The aim of the authors was to analyse the data of "double positive" patients diagnosed in their department, and compare these with previous studies., Method: During the last 16 years, 87 anti-neutrophil cytoplasmic antibody positive and 11 anti-glomerular basement membrane antibody positive patients were diagnosed. Four patients with anti-glomerular basement membrane antibodies (36%) had detectable anti-neutrophil cytoplasmic antibodies, 2 patients were positive for anti-myeloperoxidase and 2 patients for anti-proteinase 3., Results: In comparison with patients having anti-glomerular basement membrane antibodies, the double-positive patients were characterized by older age (median of 46 vs. 24 years), lack of male dominance (50% vs. 71%), more frequent presence of previous extrarenal symptoms (50% vs. 0%), and lower anti-glomerular basement membrane antibody levels (<100EU/ml: 100% vs. 29%). The double-positive patients had more favourable 1-year survival (100% vs. 71%), despite their older age and similar treatment regimen (immunosuppression 100% in both groups, plasmapheresis in 75% vs. 86%), but 1-year renal survival was not different (25% vs. 14%)., Conclusions: In agreement with literature data, about one third of patients with anti-glomerular basement membrane antibodies had detectable anti-neutrophil cytoplasmic antibodies, and the coexistence of the two antibodies may have clinical consequences.

The Wilms tumor 1 (WT1) gene is currently in focus by pediatric nephrologists as its mutations are associated with nephrotic syndrome, especially as part of complex clinical entities like Denys-Drash or Frasier syndrome. Renal failure may also develop in young WAGR patients, whose condition is attributed to a deletion at chromosomal region 11p13. However, only limited data exist on WT1 microdeletions. A 30-year-old male patient, with a history of genital malformations, a Wilms tumor manifested during the treatment of acute lymphoid leukemia (ALL) at the age of 4, and a cerebellar angioblastoma, was referred with proteinuria and a reduced glomerular filtration rate (GFR). Kidney biopsy revealed FSGS. Although all WT1 exons were amplified with polymerase chain reaction (PCR) and sequenced, none of them showed a mutation. However, an formalin-fixed, paraffin- embedded (FFPE) tissue sample of the patient's childhood Wilms tumor showed WT1- positivity restricted to the renal tumor cells, so the WT1 gene was investigated further. Using quantitative reverse transcription PCR (qRT-PCR), the gene was found to be present in only one copy in the patient's genomic DNA sample, while both copies were detected in both parents. In the patient's sister, the proximal region of WT1 was shown to have an extra copy. Evidence suggests that a heterozygous microdeletion of the gene WT1 is responsible for the patient's disease. It seems reasonable to assume a possible abnormality affecting meiotic crossing over at the WT1 locus in one of the parents.

Metformin is the first-line, widely used oral antidiabetic agent for the management of type 2 diabetes. There is increasing evidence that metformin use results in a reduction in cardiovascular morbidity and mortality, and might have anticancer activity. An extremely rare, but potentially life-threatening adverse effect of metformin is lactic acidosis, therefore, its use is traditionally contraindicated if the glomerular filtrate rate is below 60 mL/min. However, lactic acidosis is always associated with acute events, such as hypovolemia, acute cardiorespiratory illness, severe sepsis and acute renal or hepatic failure. Furthermore, administration of insulins and conventional antihyperglycemic agents increases the risk of severe hypoglycemic events when renal function is reduced. Therefore, the magnitude of the benefit of metformin use would outweigh potential risk of lactic acidosis in moderate chronic renal disease. After reviewing the literature, the authors give a proposal for the administration of metformin, according to the calculated glomerular filtrate rate.

Objectives: There have been few reports on the association of rheumatoid arthritis (RA) with anti-neutrophil cytoplasmic antigen (ANCA)-associated systemic vasculitides., Methods: Here we present four cases of RA overlapping with Wegener's granulomatosis (WG), microscopic polyangiitis (MPA) and Churg-Strauss syndrome (CSS). All these cases had both the diagnosis of RA and that of ANCA-associated vasculitis., Results: After we tried corticosteroids, multiple immunosuppressive drugs, sometimes plasmapheresis, rituximab was introduced to 3 out of 4 cases. Rituximab therapy was found to be effective in these three overlap cases. In the fourth case, rituximab was and will be considered; however, the patient has not given consent., Conclusion: To our knowledge, this is the first report on RA+CSS association. Common pathogenic background, such as genetic predisposition and ANCA may account for the development of RA+vasculitis overlaps. In DMARD-refractory cases, such as the ones presented here, biologics, especially rituximab may be highly effective.

Atherosclerosis is a systemic disease affecting the coronary, carotid, intracerebral, renal and peripherial arteries. The early morphological and functional impairments could be detected in the second or third decades of life and their progression depend on the number and severity of risk factors and individual susceptility. Although the vascular risk factors (smoking, overweight, age, unhealthy diet, lack of physical exercise, hypertension, diabetes mellitus, chronic kidney disease and dyslipidemia) are the same and common in the different vascular diseases, the present clinical routine artificially classifies the diagnosis and therapy of different vascular diseases into different subfields of medicine with the negative impact of possible polypragmasia. Recently, worldwide health surveys (e.g. REACH registry) have proven the usefulness of a holistic approach in the diagnosis and therapy of multiorgan-affected vascular patients. This review summarizes the multidisciplinary advances and future perspective of vascular diseases.

According to current clinical trials, albumin excretion is an early indicator of cardiovascular damage. While proteinuria is considered as a marker of kidney function, albuminuria indicates cardiovascular risk first of all. Sensitivity of the previous laboratory tests does not meet the clinical requirements, and the error of urine collection makes the results misleading. For that reason recent guidelines suggest to calculate albumin/creatinine (ACR) and protein/creatinine (PCR) measured from the first morning urine. For the clinical diagnosis of albuminuria the sensitive immunoturbidimetric assays are suggested. Albumin dipsticks are not recommended for the measurement of albuminuria. Wide-range urinary protein reagents are also available with high sensitivity, while serum reagents are not applicable (Biuret). The traceability of calibrator to a reference material is a critical requirement. Proteinuria and albuminuria of a patient should be monitored in the same laboratory, using a fixed method and cut-off value. Albumin/creatinine value should be reported together with gender-dependent reference range.

Nowadays chronic kidney disease has become a major public health problem due to the great increase in atherogenic nephropathies. In the absence of classic renal symptoms, chronic kidney disease is mostly diagnosed when renal failure is already advanced, although it can be revealed by laboratory tests in the earlier stages. When diagnosis is late, the progression to end-stage renal failure is unavoidable and renal replacement therapy is needed. Even early-moderate renal failure significantly increases the risks for atherosclerosis, thereby leading to the deaths of patients from cardiovascular disease before initiation of dialysis. Therefore screening for asymptomatic chronic kidney disease is urgently needed. Estimated glomerular filtration rate has the greatest importance in the screening and in the timely intervention to slow down the progression of renal failure and cardiovascular disease. In 2005, the Hungarian Society of Nephrologists and the Hungarian Society of Laboratory Medicine suggested the automatic estimation and reporting of glomerular filtration rate, each time serum creatinine measurements were made. This practice is used more frequently by laboratories in Hungary. This article aims to help facilitate the utilization and evaluation of estimated glomerular filtration rate.

The prevalence of hepatitis C virus infection among patients on hemodialysis is about ten times higher than in the normal population. The infection can induce chronic glomerulonephritis, as an extrahepatic manifestation, which can lead to end stage renal disease. However, in the majority of the patients hepatitis C virus is acquired as a nosocomial infection during the hemodialysis. In most of the infected patients the liver enzymes are usually normal and need regular screening of the hepatitis C antibody to detect the infection. Despite of the normal liver enzymes, the liver disease may progress to cirrhosis. A part of the patients wait for renal transplantation. The immunosuppressive treatment after the renal transplantation results in a significantly increased viral replication which might induce further progression of the liver disease. Interferon treatment given after the transplantation can induce rejection and graft failure. Therefore the antiviral treatment should be administered during the hemodialysis or earlier. Only limited data are available with the treatment of patients with impaired renal function. Mostly alfa-interferon was used in these patients. Due to the impaired renal clearance and higher serum concentration interferon seems to be more effective, but less tolerable in patients with end stage renal disease than in normal patients. Ribavirin is also excreted exclusively by the kidney and the anemia is even more pronounced in these patients, therefore it is contraindicated in patients on hemodialysis. The pharmacokinetics of the pegylated interferon alfa-2a is very advantageous for the patients with end stage renal disease. The safety and efficacy of peginterferon alfa-2a is now being confirmed in many publications.

Sulfhemoglobinemia (SHb) is an uncommon cause of cyanosis that is predominantly drug-induced in adults. We report an unusual case of sodium sulfate-induced sulfhemoglobinemia in a 61-year-old woman after surgical polypectomy. Fractional hemoglobin derivates were assayed by spectrophotometry and high-performance liquid chromatography. The SHb ratio was 8.6% in the first sample and 3.77% a month later measured by spectrophotometry. In the blood hemolysate, a new peak was identified as SHb with high-performance liquid chromatography (HPLC). HPLC showed the presence of 9.37% SHb in the first sample and 4.88% a month later. After removing the suspected toxic agent the cyanosis decreased significantly. The findings underline the importance of routine SHb detection in cyanosis of unknown origin especially in emergency cases.

Background: The diagnosis of analgesic nephropathy has improved significantly with modern imaging techniques. We reviewed a large portion of the Hungarian dialysis population to obtain additional insight into the problem., Methods: Twenty-two participating dialysis units enrolled 1400 patients on renal replacement therapy between 1 January 1995 and 1 January 1998. Patients with no known aetiology (n = 284) were interviewed and studied with renal imaging. We assessed the presence of decreased renal mass combined with either bumpy contours, papillary calcification, or both. The subjects studied were interrogated extensively., Results: Our survey suggested analgesic nephropathy in 47 of 1400 patients (3.3%), 3-fold higher than the EDTA database estimate for Hungary. The analgesics most commonly abused were phenacetin-containing mixtures. The driving symptoms were mainly headache and joint pain. Cardiovascular complications were more common than in the rest of the dialysis population, independent of smoking and lipid values (P<0.01)., Conclusions: Phenacetin should be banned. Our study results support the need for longitudinal cohort and case-control studies in Hungary.

The authors report a case of 47 years patient, who underwent successful combined liver-kidney transplantation. The primary cause of cirrhosis was HCV infection combined with membranoproliferative glomerulonephritis, witch resulted end stage kidney and liver disease. One and half year after operation the patient has full rehabilitation. By the case report the authors overview the indications and techniques of combined kidney-liver transplantation.

Background: Analysing a 12-lead surface electrocardiogram (ECG), the inter-lead variability of the P wave interval, i.e. P wave dispersion, is defined as the difference between the maximum and the minimum P wave duration. Our aim was to assess the effect of haemodialysis on P wave duration and dispersion in non-diabetic patients with end-stage renal failure on chronic haemodialysis., Methods: Twenty-eight patients (14 men and 14 women, mean age 58+/-16 years, average duration of dialysis 4.5+/-2.8 years) were examined. Prior to haemodialysis, echocardiography (M-mode and two-dimensional) was performed. Haemodialysis sessions were carried out with polysulfone dialysers and bicarbonate dialysate fluids. Twelve-lead ECGs were recorded at the beginning, 15 and 30 min after starting dialysis, at the end, and 2 h after completion of each session. Ionic parameters were checked during the study. P wave durations were measured with calipers in three consecutive complexes of each lead by one observer., Results: P maximum was 58+/-16 ms at the beginning, and showed an increase by the end of dialysis to 98+/-8.9 ms (P<0.0001). Pre-dialysis P dispersion was 23+/-10 ms and increased to 41+/-16 ms by the end of the sessions (P<0.0001). In patients with a left atrial diameter larger than 45 mm, P dispersion increased from 23+/-11 to 53+/-10 ms (P<0.0003) by the end of the sessions., Conclusions: According to our results, ionic imbalance and dialysis itself may cause changes in P duration and dispersion simultaneously.

Introduction: There's a fact, that Hungary has held the first places in suicidal statstics., Methods: The authors studied toxicological cases between 1989 and 1998 at the 1st Department of Medicine of the Medical and Health Science Centre, at the University of Debrecen, paying special attention to suicidal poisoning cases., Results: 2% of the patient turnover accounted for acute poisoning cases, the number of which increased during the 10 years in question. 70% of the cases were of suicidal intentions, 20% were unintentional, these poisonings were not committed on purpose, while the proportion of iatrogenic intoxication cases was 10%. Amongst the failed suicide cases there was a higher proportion of women, whereas a higher percentage of men accounted for "successful" suicide cases. When examining auto-intoxication cases it turned out that the medicine most frequently used was meprobamate, besides benzodiazepines. Mortality rate was highest in the glutethimide intoxication cases. Most poisonings with suicidal intentions took place in the 2nd quarter of the year. Most completed suicides were committed on Wednesdays and Thursdays. 81% of the iatrogenic intoxication cases happened to be with digitalis and coumarin overdose. Nearly 50% of the cases turned out to be combined intoxications. 40% of the men took alcoholic drinks during the auto-intoxications. In the case of 135 patients extracorporeal detoxification therapy was applied, which consisted mostly of hemoperfusion. Three quarters of the patients needed psychiatric care and every fourth patient was admitted to the Department of Psychiatry. 6.9% of the poisonings were fatal., Conclusions: The growing number of toxicological cases--amongst these suicidal poisonings--compels us to pay more attention to the setting up of interdisciplinary based prevention as well as running effective toxicological centres. All physicians have a responsibility to recommend psychiatric care for people suffering from mental problems or depression and for the unsuccessful or potential suicide seeking help for the first time. Family doctors in primary medical care and who meet patients first have an important role in this job.

Retrospective study was performed to measure the results of parathyroidectomy in patients with secondary hyperparathyroidism. From 1987 to 2000, 48 patients underwent surgery for secondary hyperparathyroidism. There were 30 of 48 patients on haemodialysis treatment, and 11 patients were in pre-dialysis stage. Parathyroidectomy was performed after successful kidney transplantation in 4 cases. Indication of the surgery was extremely elevated serum level of parathyroid hormone (at least 10 fold elevation), which was resistant for the conservative medical therapy. Subtotal parathyroidectomy (3 1/2) was performed in 30 patients. Five patients underwent total parathyroidectomy and autotransplantation. Only 2 or 3 parathyroid glands have been removed in 13 patients. Haematoma occurred in 3 cases after parathyroidectomy. Recurrent nerve injury or septic complication did not occur. Two patients died in the early postoperative period due to cardiac failure. Tetania was noted in 2 patients after surgery. Permanent postoperative hypocalcaemia (over 6 months) occurred in 3 cases. Persistent hyperparathyroidism was diagnosed in 5 patients. In these patients 2 parathyroid glands were removed during the primary operation. Recurrent hyperparathyroidism was detected in 2 patients. Subtotal parathyroidectomy was carried out in these cases previously. At the reoperation for persistent and recurrent hyperparathyroidism, total parathyroidectomy and autotransplantation was performed. Serum alkaline phosphatase level and serum parathyroid hormone value decreased after surgery, except those patients with persistent hyperparathyroidism. Bone pain decreased in 96% of the cases and pruritus decreased in 92% of the patients after parathyroidectomy. Soft tissue calcification showed improvement in 45% of cases. In conclusion, the subtotal parathyroidectomy or total parathyroidectomy with autotransplantation cause a rapid decrease of PTH level and the improvement of the clinical symptoms in patients with medical treatment resistant secondary hyperparathyroidism. Persistent hyperparathyroidism occurs in those cases when inadequate parathyroidectomy was performed.

In recent years elaboration of the diagnosis of analgesic-nephropathy (ANP) with the help of imaging techniques significantly increased the possibility of diagnosing the disease. Therefore, evaluating the prevalence of ANP has become more accurate in our country as well. The prevalence of ANP has been investigated in patients who have newly been taken into the dialysis program due to renal disease of unknown aetiology in 22 dialysis centers between December 1994-December 1997. The diagnosis of ANP has been based on revealing chronic drug abuse in the history and positive results of renal imaging (decrease in length of both kidneys combined with either bumpy contours and/or papillary calcification). Among 284 patients dialysed with unknown diagnosis 42 (14.8% of all cases) proved to have ANP. All patients except 2 took analgesic mixtures containing phenacetin/paracetamol, phenason derivatives, acetilsalysilic acid, caffeine and/or codeine. According to their investigations, ANP is a common disease resulting in end-stage renal failure in Hungary as well.

The first case of oncogen osteomalacia in Hungary is reported, to draw the attention of the medical profession to it and to present the new data about its pathomechanism. Pathological hip fracture caused by hypophosphataemic osteomalacia due to isolated renal phosphate wasting was found in a previously healthy 19 years old sportsman. In spite of daily 1.5 micrograms calcitriol treatment and phosphate supplementation, hypophosphataemia persisted for 13 years and he needed regular indometacin medication for his bone pain. During that time an 1.5 cm gingival tumour was found and radically removed. The serum phosphate level returned to normal in a few hours after the operation (preoperative 0.51, after 2, 4 and 8 hours 0.61, 0.68 and 0.79 mmol/l respectively), and remained normal without calcitriol. The histological examination showed epulis with fibroblast and vascular cell proliferation, which has never been previously reported in connection with oncogenic osteomalacia. The pain resolved after 3 months and the bone density became normal in one year. Oncogenic osteomalacia must be considered in every case presenting with atypical hypophosphataemic osteomalacia. Careful dental examination is needed also in the course of search for the underlying tumour. Every tumour-like growth, even the common epulis, has to be operated radically and serum phosphate monitored in the postoperative period in all such cases.

Interlead variability of the QT interval in surface electrocardiogram (ECG), i.e., QT dispersion, reflects regional differences in ventricular recovery time, and it has been linked to the occurrence of malignant arrhythmias in different cardiac diseases. The purpose of the study was to assess the effect of hemodialysis on QT and corrected QT (QTc) interval and dispersion in chronic hemodialyzed patients. Data of 34 nondiabetic patients (male/female = 21/13; mean age, 54 +/- 15 yr) on chronic hemodialysis were studied. Polysulfone capillaries and bicarbonate dialysate containing (in mEq/L) 135 Na+, 2.0 K+, 1.5 Ca2+, and 1.0 Mg2+ were used. Simultaneous 12-lead ECG were recorded before and after hemodialysis in a standard setting. The QT intervals for each lead were measured manually on enlarged (x3) ECG by one observer using calipers. Each QT interval was corrected for patient heart rate: QTc = QT/square root of RR (in milliseconds [ms]). The average cycle intervals were 853 +/- 152 ms predialysis and 830 +/- 173 ms postdialysis; the difference was not significant. The maximal QT interval changed significantly from 449 +/- 43 to 469 +/- 41 ms (P < 0.01). The corrected maximal QT interval increased significantly from 482 +/- 42 to 519 +/- 33 ms (P < 0.01). The QT dispersion changed from 56 +/- 15 to 85 +/- 12 ms (P < 0.001) and the corrected QT interval dispersion from 62 +/- 18 to 95 +/- 17 ms (P < 0.001). During hemodialysis, the serum potassium and phosphate levels decreased from 5.5 +/- 0.8 to 3.9 +/- 0.5 (mM) and from 2.3 +/- 0.5 to 1.6 +/- 0.4 (mM), respectively, whereas calcium increased from 2.2 +/- 0.23 to 2.5 +/- 0.22 (mM). It is concluded that hemodialysis increases the QT and QTc interval and QT and QTc dispersion in patients with end-stage renal failure. Thus, it may be stated that the nonhomogeneity of regional ventricular repolarization increases during hemodialysis. Measurement of QT and QTc dispersion is a simple bedside method that can be used for analyzing ventricular repolarization during hemodialysis.

Interlead variability of the QT interval in surface 12-lead ECG (i.e. QT dispersion) reflects regional differences in ventricular recovery time and it has been linked to the occurrence of malignant arrhythmias in different cardiac diseases. The purpose of the study was to assess the effect of hemodialysis on QT dispersion in chronic hemodialyzed patients. The data of 34 patients (Male/Female = 21/13, mean age 54 +/- 15 years) on chronic hemodialysis were studied. Simultaneous 12 lead ECGs were recorded pre- and post-hemodialysis in a standard setting. The QT intervals for each lead were measured manually by one observer. Each QT interval was corrected for patient's heart rate: QTc = QT/square route of RR (sec). The maximal QT interval changed from 449 +/- 43 to 469 +/- 41 ms (p < 0.01). The maximal QTc interval increased from 482 +/- 42 to 519 +/- 33 ms (p < 0.01). The QT dispersion changed rom 56 +/- 15 to 85 +/- 12 ms (p < 0.001), and the QTc interval from 62 +/- 18 to 95 +/- 17 ms (p < 0.001). During hemodialysis the serum potassium and phosphate decreased from 5.5 +/- 0.8 to 3.9 +/- 0.5 (p < 0.001), and from 2.3 +/- 0.5 to 1.6 +/- 0.4, respectively, whereas calcium level increased from 2.2 +/- 0.23 to 2.5 +/- 0.22 (p < 0.001). It can be concluded that the hemodialysis increased the inhomogeneity of regional ventricular repolarization. Measurement of QT and QTc dispersion by a cheap and simple bedside method could predict the increased myocardial inhomogeneity in dialyzed patients.

Serum paraoxonase (PON) is a high-density lipoprotein (HDL)-associated hydrolase, which inhibits low-density lipoprotein oxidation. Uremic and kidney-transplanted patients have an increased risk of atherosclerosis, to which an increased lipoprotein oxidation may contribute. The aim of our study was to determine whether the PON activity or phenotype is altered in uremic and kidney-transplanted patients, and to compare the values with those of healthy controls. 117 uremic patients on long-term hemodialysis treatment, 115 renal-transplanted patients, and 110 healthy controls were involved in the study. The PON activity was significantly reduced in the uremic patients compared to controls (PON 101.36+/-30. 12 vs. control 188.05+/-58.96 U/ml; p < 0.001), while in kidney-transplanted patients the values were almost identical to those of controls (PON 161.5+/-35.39 U/ml). The different immunosuppressive drug combinations did not influence PON activity. To assess whether the altered PON activity was due to a decrease HDL level, we standardized the enzyme activity for the HDL concentration (PON/HDL ratio). We found that the standardized enzyme activity was lower in the uremic (102.7+/-54.8) and kidney-transplanted patients (144.5+/-32.7) when compared to controls (194.5+/-94.5; p < 0.001). The phenotypic distribution of PON in uremic, renal transplant and control patients are as follows: AA 66.67, 56.48 and 66.67%; AB 31. 62, 33.3 and 26.67%; BB 1.71, 10.19 and 6.67%. We conclude that the decreased PON/HDL and PON/apoA-1 ratios may lead to a reduction in the antioxidant capacity of HDL, which might contribute to the accelerated development of atherosclerosis in uremic and kidney-transplanted patients.

Human serum paraoxonase is physically associated with an apolipoprotein (Apo-A1) and clusterin-containing high-density lipoprotein (HDL) and prevents low-density lipoprotein from lipid peroxidation. The aim of our study was to determine whether paraoxonase activity or phenotype is altered in patients with chronic renal failure and in hyperlipidemic subjects without renal insufficiency and to compare the values with those of healthy controls. We investigated the serum paraoxonase activity and polymorphism in 119 hemodialyzed uremic patients, 107 patients with primary hyperlipoproteinemia, and in 110 healthy control subjects. The serum paraoxonase activity was significantly decreased both in hyperlipidemic (p < 0.01) and uremic patients (p < 0.001) as compared with controls. On comparison, the serum paraoxonase activity was significantly lower (p < 0.001) in uremic than in hyperlipoproteinemic patients. The HDL and Apo-A1 levels were as follows: uremic < hyperlipidemic < control. To assess whether the observed reduction in paraoxonase activity was due to HDL and Apo-A1 level decreases, we standardized the enzyme activity for HDL and Apo-A1 concentrations. We found that the standardized paraoxonase activity (paraoxonase/HDL ratio) was also lower in the uremic patients (103.3 +/- 69.5) as compared with hyperlipidemic patients (137.64 +/- 81.0) and controls (194.45 +/- 94.45). The standardized values for Apo-A1 showed a similar tendency: paraoxonase/Apo-A1 ratio in uremic patients 89.64 +/- 47.8, in hyperlipidemic patients 128.12 +/- 69.83, and in controls 161.40 +/- 47.35. The phenotypic distribution of paraoxonase (AA, AB, BB) did not change significantly in the patient groups. These results suggest that HDL concentration and phenotypic distribution of paraoxonase may not be the only determining factors, but that other as yet undetermined factors could be involved in the enzyme activity changes.

Recently a growing number of case reports has been published about successful pregnancy outcome of dialysed women on recombinant human erythropoietin therapy. During pregnancy the maternal demand for erythropoietin may undergo changes, with consideration of recombinant human erythropoietin therapy in the early stage of renal insufficiency, as is shown by our two reported cases. The use of recombinant human erythropoietin seems to be safe for the foetus: it does not cross the placental barrier, and therefore lacks any direct foetal effect. The treatment of anaemia with recombinant human erythropoietin carries benefits for both the mother and foetus. One of the most important preconditions for successful recombinant human erythropoietin therapy is adequate iron supplementation. Due to the increased risk of pregnancy induced hypertension or preeclampsia, there is a need for slow and gradual correction of anaemia, and an individually tailored target hematocrit. A close follow up of he patient by the obstetrical-nephrological team is essential, with the intensive monitoring of the fetuses. In some cases with normal renal function the stimulation of erythropoiesis with recombinant human erythropoietin may also be needed during the pregnancy.

The authors report the case of a 38 year old man with horseshoe kidney who developed a severe nephroso-nephritis syndrome, caused by cryoglobulinemic membranoproliferative glomerulo-nephritis. A combination of steroid and cyclophosphamide treatment resulted in partial improvement, but was discontinued after 12 weeks due to adverse reactions, with a consequent early relapse. The 4 week course of cyclosporine monotherapy proved ineffective and signs of cryoglobulinemia appeared. The elevation of transaminase, manifested during the immunosuppressive therapy demonstrated the presence of underlying chronic C hepatitis. In the light of the liver biopsy result, interferon treatment was commenced at a dose of 3 million unit thrice weekly. After 4 months of interferon treatment the persistent nephrotic range proteinuria decreased to below 0.5 g/day. Four months later clinical signs of cryoglobulinemia disappeared, and after the 10th month of interferon treatment no cryoglobulin could be detected in the patient's sera. After one year, the interferon treatment was discontinued following a negative PCR result for HCV. However, one month later the proteinuria increased and the quantitative hepatitis C virus nucleic acid test in sera became positive again. Our case demonstrates that interferon therapy may be effective in the treatment of cryoglobulinemic glomerulonephritis responding poorly to the immunosuppressive therapy, though larger doses or longer periods of treatment may be required to prevent relapses.

Recently dialysis related amyloidosis has become a major complication in patients treated with long-term dialysis therapy. The serum level of the amyloid precursor beta 2-microglobulin is significantly elevated in uraemia, mostly due to the retention. The bioincompatibility of dialysis membranes and the endotoxin content of the dialysate may contribute to the synthesis and tissue deposition of beta 2-microglobulin, but the details of pathogenesis are not yet cleared. At first periarticular and perineural structures are involved in the deposition of amyloid. The carpal tunnel syndrome is of great differential diagnostic value, it appears frequently together with the beginning of the joint pain. The main target of arthropathy are the large and medium-sized joints symmetrically. Deposition of the amyloid to the subchondral bone cysts might lead to pathological fractures, mainly in the hips and destructive spondylarthrophathy might involve severe neurologic complications. Visceral organs (gastrointestinal and urogeniteal tract, heart etc.) are involved rarely and later. Ultrasonography and isotope methods in addition to the conventional radiologic examinations are also used to differentiate the joint complaints nowadays. The definitive diagnosis is based on immunohistology. The alteration of dialysis strategy first of all the usage of high permeable, biocompatibile membranes and pure dialysis water has a role in the prevention of disease and decreasing its progression. In the case of developed lesions timely surgical-orthopedic interventions are required in addition to drug therapy. Todays' renal transplantation is a successful treatment, but the consequences of amyloid depositions already formed can't be left out of considerations even after transplantation.

A 46-year-old patient with chronic renal failure receiving maintenance haemodialysis for 3 years had a few months history of blister formation and skin fragility involving the face, arms and dorsa of the hands. In this case clinically mimicking porphyria cutanea tarda (PCT) no demonstrable abnormality in the porphyrin metabolism excretion was detected.

The authors reported on a three month long EPREX (human recombinant erythropoietin) therapy of 5 hemodialysis patients for the treatment of their anemia. The drug was administered in bolus form 2 or 3 times a week after dialysis in a dose of 50 to 150 IU/bodyweight increased gradually in every (or every second) week. Hgb ad Htk values were determined once a week while erythrocyte, leukocyte, thrombocyte and reticulocyte count once a month. Serum iron, TIBC, serum ferritin, BUN, serum creatinine, urea, serum ions, liver function assays, serum lipids and amylase were also established. Hgb, Htk levels and reticulocyte count have significantly increased in the 4th week of treatment already, severe anemia ceased with improved appetite, general condition and physical strength. Serum urea and LDH levels significantly increased while SGOT decreased. No significant change in leukocyte and thrombocyte count, serum Na, K, Ca, P, Cl, BUN, creatinine, total protein level, serum albumin, bilirubin, alkaline phosphatase, GGT, GPT, amylase and blood sugar as well as serum lipid level were observed. No adverse reactions occurred during the treatment. After the three gradually decreased and within 6 weeks they had to be transfused again. In three patients the need for transfusion has significantly grown after the treatment. The authors consider EPREX a highly efficient drug in the treatment of anemia in dialysis patients.

The observations of a 16-week Tensiomin therapy of 10 hypertensive patients treated with hemodialysis have been discussed. The patients have been treated for about 5 years with hemodialysis, suffered from anuria and required besides systhematical ultrafiltration a combination antihypertensive therapy. Tensiomin was combined with Minipress, Trasicor, Depressan, Estulic and Corinfar by using three- or four-drug combinations. In the course of the administration of Tensiomin the doses of the other antihypertensive drugs could be decreased by 50% on average, while the blood pressure of the patients was normalized. By controlling the patients on weeks 1, 4, 12 and 16 of therapy toxic side-effects or notable pathological changes of the examined laboratory parameters (WBC, serum total protein, Na, K, Ca, P, bilirubin, blood sugar and SGOT values) were not seen. It has been concluded that Tensiomin is an effective drug in combination therapy applied for normalizing the hypertension of dialysed patients.

Hutchinson-Gilford progeria was observed in two brothers. Their parents, sister and other relatives did not show any signs of this illness. Serum total cholesterol and total triglyceride levels were normal in the whole family. The serum high density lipoprotein cholesterol (HDL-C) level of parents was low and that of boys was extremely low. The serum HDL-C level of the healthy sister and other relatives was normal. These findings in homozygous children and heterozygous parents may explain the development of the very early fatal arteriosclerosis described in this disease. The connection between the disorder of the lipid metabolism and progeria can serve as a useful model in the study of the role of lipid metabolism in normal ageing.

99mTc-HEDP bone scan was carried out on 12 long-time haemodialysed patients, suffering from bone pains. X-ray examinations of the bone and laboratory tests (serum calcium, -phosphor, -alkaline phosphatase, -parathormone, -aluminium, -ferritin) were also performed. The scintigrams were evaluated by two semiquantitative scores. Based on diffuse, increased radiopharmacon uptake of the bones and more than five points in the Fogelman score 5 patients most likely had serious and 3 had moderate hyperparathyroidism. In two patients osteomalacy was presumed based on decreased radiopharmacon uptake of the bones, increased uptake of the soft tissues and zero Fogelman score. Mixed or other bone disease was suggested in two other patients. Good correlation was found between the results of bone scans, the parathormone values and the results of histology obtained after parathyreoidectomy of 4 patients and autopsy of two others. This non-invasive examination (ie. bone scan) is helpful in differential diagnosis of uraemic osteodystrophy and its wide use is proposed in domestic nephrological practice.