L. Ujhelyi

The Serum Paraoxonase Activity in Patients with Chronic Renal Failure and Hyperlipidemia

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.

The epidemic of chronic kidney disease requires the estimation of glomerular filtration rate

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.

Relapsing polychondritis with p-ANCA associated vasculitis: Which triggers the other?

Relapsing polychondritis (RP) is a rare autoimmune disease with chronic inflammatory/destructive lesions of the cartilaginous tissues. In one third of the cases it is associated with other autoimmune disorders, mostly with anti-neutrophil cytoplasmic antibody (ANCA) associated vasculitis (AAV). We report three cases of RP with p-ANCA positive AAV. In the first patient RP developed 1.5 years after the onset of AAV. In the others the signs of RP were present before the onset of severe crescent glomerulonephritis. Patients responded well on steroid and cyclophosphamide. In dialysis dependent cases plasmapheresis was also used successfully. During the 2 and 1.5 years of follow up, they were symptom-free, and had stable glomerular filtration rate. The first patient died after four years of follow-up due to the complications of sudden unset pancytopenia, which raises the possibility of associated hemophagocytic syndrome. In the setting of RP or AAV physicians should always be aware of the possibility of sudden or insidious appearance of the other disease.

Coexistence of thyroid carcinoma and secondary hyperparathyroidism

SummaryBackgroundReports on the coexistence of secondary hyperparathyroidism (sHPT) and thyroid carcinoma are very rare.MethodsA case of secondary hyperparathyroidism and concomitant papillary thyroid carcinoma (follicular variant) is described, and compared to data from the literature.Results1 out of 26 patients undergoing parathyroidectomy for secondary hyperparathyroidism had papillary thyroid microcarcinoma (follicular variant) associated with regional lymph node metastases. Head and neck were not irradiated during the patient’s case history. Dialysis had been performed for 23 years prior to simultaneous near-total thyroidectomy, total parathyroidectomy and autotransplantation. The prolonged high parathyroid hormone levels, or massive hemosiderin deposits may have caused the development of carcinoma. The incidence rate of occult thyroid microcarcinoma in all autopsies of our geographical region and period was 4.5 per cent.ConclusionsCareful exploration of the thyroid gland, and liberal indication for biopsy of suspicious thyroid areas is mandatory in each sHPT patient who is operated on, in order to exclude possible thyroid carcinomas.ZusammenfassungGrundlagenIn der Literatur gibt es nur wenige Berichte über das gemeinsame Vorkommen von sekundärem Hyperparathyreoidismus (sHPT) und Schilddrüsenkarzinom.MethodikEin Fall von sekundärem Hyperparathyreoidismus mit gleichzeitigem papillärem Schilddrüsenkarzinom (follikuläre Variante) wird beschrieben und mit Literaturdaten verglichen.ErgebnisseUnter 26 Patienten, die wegen sHPT operiert wurden, fand sich eine Patientin mit papillärem Mikrokarzinom (follikuläre Variante) und Lymphknotenmetastasen. Anamnestisch wurde keine Bestrahlung der Kopf-Halsregion durchgeführt. Die erhöhten Parathormonwerte und die massiven Hämosiderinablagerungen während der 23 Jahre langen Hämodialyse könnten die Ursache für die Karzinomentstehung sein. Neben einer totalen Parathyroidektomie mit simultaner Autotransplantation wurde eine „near-total” Thyroidektomie durchgeführt. In derselben geographischen Region wurden im gleichen Zeitraum bei 4,5% aller Autopsien Mikrokarzinome der Schilddrüse beobachtet.SchlußfolgerungenBei jeder Halsexploration wegen sHPT ist immer die Schilddrüse sorgfältig nach einem Schilddrüsenkarzinom zu untersuchen und suspekte Schilddrüsenareale sind gegebenenfalls zu biopsieren.

Use of Hemadsorption in a Case of Pediatric Toxic Shock Syndrome

Background Toxic shock syndrome is a potentially fatal toxin-mediated disease. The role of toxins in this clinical entity made us hypothesize that extracorporeal blood purification with CytoSorb® could play a beneficial role in the clinical management of toxic shock syndrome. This case report describes the successful treatment of toxic shock syndrome using a combination of renal replacement therapy and hemadsorption in a pediatric patient. Case Presentation A 5-year-old girl with Downs syndrome presented with an inflamed area surrounding an insect bite, signs of systemic inflammation, and multiple organ failure. As previous attempts of immune modulation therapy were unsuccessful, renal replacement therapy was supplemented by the cytokine absorber CytoSorb. Treatment using this combination was associated with a rapid and significant stabilization in the hemodynamic situation and a decrease in inflammatory mediators within hours after the initiation of therapy. The application of CytoSorb therapy was simple and safe. Conclusion The use of extracorporeal blood purification with CytoSorb proved potentially beneficial by removing toxins and inflammatory mediators in this case and could therefore play a role in the clinical management of toxic shock syndrome. Whether CytoSorb has the potential to even positively influence mortality in patients with toxic shock syndrome still needs to be confirmed.

Effect of Creatinine Methods on the eGFR: the New and Old Formulae

Background: Recently several estimated GFR (eGFR) formulae have been developed and new creatinine methods were introduced to compensate for the effect of “pseudocreatinines”. These methods resulted in lower creatinine and higher eGFRs. Methods: Original 6-v MDRD (Modification of Diet in Renal Disease), 4-v MDRD, Cockcroft-Gault (C-G), Quadratic and the new MDRD-175 formulae were tested in 31 patients. The eGFR based on both Jaffe and compensated-Jaffe creatinine were compared to Tc-99m labeled diethylenetriamine pentaacetic acid clearance (DTPA-GFR). Results: All eGFRs correlated with DTPA-GFR (range 16–104 mL/min/1.73 m 2 ). Original MDRD and C-G showed high correlation coefficients (0.88 and 0.78), but original and 4-v MDRD tended to overestimate GFR below 60 ml/min/1.73 m 2 and underestimate it above 60 mL/min/1.73 m 2 . Using compensated-Jaffe creatinine reduced the underestimation of the original MDRD, but resulted in overestimation of the 4-v MDRD, C-G and quadratic GFR. With the exception of the or...