Correction to: Primary immunodeficiencies in Central and Eastern Europe-the power of networking Report on the activity of the Jeffrey Modell Foundation Centers Network in Central and Eastern Europe.

Due to authors' internal mistake they have misspelled the name of one of the co-authors in this article.

Primary immunodeficiencies in Central and Eastern Europe-the power of networking Report on the activity of the Jeffrey Modell Foundation Centers Network in Central and Eastern Europe.

Jeffrey Modell Foundation centers' network activities in Central and Eastern Europe (JMF CEE) have contributed to the development of care for patients with primary immunodeficiencies. On the data continuously collected from individual centers in participating countries since 2011, we demonstrate a steady improvement in a number of aspects concerning complex care for patients with primary immunodeficiencies. The presented data show an improvement of awareness about these rare diseases across the whole Central and Eastern European region, an increase in newly diagnosed patients as well as genetically confirmed cases, earlier establishment of diagnosis, and improved access to clinical treatment. We also present an active patient involvement that is reflected in the expansion of patient organization centers and their activities. The cooperation within the JMF CEE network has also contributed to greater international exposure of participating centers and further to the gradual development of research activities in the rapidly evolving field of primary immunodeficiencies. The improvement of all important aspects of the complex field of primary immunodeficiencies within the JMF CEE network documents the strength and advantages of the joint and coordinated networking.

High prevalence of coeliac disease: need for increasing awareness among physicians.

BACKGROUND: Coeliac disease is a wheat gluten and related prolamines-induced disease with a prevalence that may be underestimated in many geographical regions and populations. AIM: To investigate the prevalence of coeliac disease in a population of schoolchildren of Estonia using tissue transglutaminase antibodies for screening. SUBJECTS AND METHODS: The study was designed as cross-sectional. Serum samples from 1160 randomly selected schoolchildren (636 female and 564 male, aged 9 or 15 years) were studied using a novel tissue transglutaminase antibody immunoassay (EliA Celikey IgA assay). Antibody-positive subjects were investigated for coeliac disease. RESULTS: A total of five subjects had antibodies. Four of them agreed for further investigations. By small-bowel biopsy they all were confirmed to have active coeliac disease, including three subjects with symptoms that were not considered by their family doctors. The prevalence of coeliac disease is at least 1 case per 290 (0.34% with CI 0.09-0.88%) in Estonia. It is much higher than that in our previous screening studies but is comparable with data from other European countries. CONCLUSION: The prevalence of coeliac disease might have increased during the last decade in Estonia. This study clearly shows that the awareness of coeliac disease among physicians is low. Thus, there is a need for more epidemiological studies and education related to coeliac disease.

Quantitation of pentisomide and its metabolite by high-pressure liquid chromatography in human serum.

A rapid, inexpensive, and accurate high-performance liquid chromatographic method for the simultaneous determination of pentisomide and its major mono-N-dealkylated metabolite has been developed. After a simple and inexpensive solvent extraction procedure, the unchanged drug, its metabolite, and the internal standard are separated using a C18 reversed-phase column with a 5-microns particle size. The eluent is monitored with ultraviolet detection at 260 nm. Endogenous substances or a variety of drugs do not interfere with the assay. The mean recoveries of pentisomide and its metabolite are 92.6% and 92.2%, respectively. The limit of detection of the assay is 28 ng/mL for both drugs. Serum levels of pentisomide and its metabolite in patients on oral therapy for supraventricular tachycardia are reported.

Pharmacokinetic and blood pressure effects of carvedilol in patients with chronic renal failure.

The pharmacokinetic and acute systemic haemodynamic effects of a single oral dose of 50 mg carvedilol has been studied in 24 hypertensive patients with chronic renal failure. The patients were stratified into 3 groups according to the creatinine clearance: I 51-90 ml.min-1; II 26-50 ml.min-1; III 4-25 ml.min-1. The area under plasma level time curve AUC, the elimination half-life t1/2, the maximum plasma concentration Cmax, the time to peak concentration tmax were not significantly different between groups, whereas the amount of unchanged drug or metabolite excreted in urine Ae and the renal clearance CLR of carvedilol and its metabolites M2, M4, M5 were significantly decreased in Group III. Blood pressure and heart rate decreased in all 3 groups of patients after acute administration of 50 mg carvedilol. Mild adverse effects were reported in 6 patients. Despite a decrease in the renal clearance of carvedilol and of its metabolites with decreasing kidney function, its main pharmacokinetic parameters remained unchanged. The present results suggest that the dose of carvedilol need not be reduced in hypertensive patients with chronic renal failure.

Simplified, rapid and inexpensive extraction procedure for a high-performance liquid chromatographic method for determination of disopyramide and its main metabolite mono-N-dealkylated disopyramide in serum.

A simplified, rapid and inexpensive extraction procedure for the determination of the antiarrhythmic drug disopyramide and its main metabolite mono-N-desalkylated disopyramide in serum by high-performance liquid chromatography has been developed. The analysis uses ultraviolet detection at 254 nm, and a 5 micron reversed-phase column with a mobile phase of water-triethylamine-acetonitrile-PIC-B8 reagent. Serum extraction is performed with dichloromethane and 1 M sodium hydroxide. p-Chlorodisopyramide is used as internal standard. Recovery rates were 94.5% (S.D. 5.7%) for disopyramide, 96.8% (S.D. 2.2%) for mono-N-desalkylated disopyramide and 97.9% (S.D. 2.8%) for the internal standard.

Effects of beta-blockade on urinary albumin excretion in normal subjects.

Urinary albumin excretion is a marker of renal damage in diabetic or hypertensive patients and a reduction of albuminuria during beta-blockade has been demonstrated in several studies. In the present study the effects of beta-blockade with drugs known for differences in their impact on renal function (propranolol, atenolol, bopindolol) on albumin excretion in normotensive and normoalbuminuric subjects were investigated. No effect of beta-blockade on albumin excretion was found, despite decreased plasma renin activity, blood pressure and heart rate. It was concluded that studies in healthy controls are not useful to predict the outcome of treatment in microalbuminuric patients. Cross-over studies using different beta-blockers have yet to be done in micro- or macroalbuminuric patients to evaluate the potential for benefit.

Regulation of plasma aldosterone during hemodialysis.

In order to clarify the influence of serum potassium, serum sodium and plasma angiotensin II concentrations on aldosterone release during hemodialysis (HD), six chronic hemodialysis patients were studied during HD with varying dialysate sodium concentrations and different buffers. Plasma aldosterone concentrations were higher during acetate than bicarbonate HD, during low sodium compared to high sodium HD, and were correlated inversely to serum sodium concentrations. The decline in plasma aldosterone concentrations during HD paralleled the decrease in serum potassium concentrations, and plasma aldosterone concentrations were correlated with serum potassium concentrations. In addition, plasma aldosterone and plasma angiotensin II concentrations were correlated significantly. It is proposed that serum potassium and the renin-angiotensin system are the main factors of aldosterone release during hemodialysis, while serum sodium per se seems to be of less importance. The dialysate buffer employed also plays a role in aldosterone regulation (via the renin-angiotensin system).

Renal effects of the new calcium channel blocking drug isradipine.

The acute effect of a single oral dose of isradipine 5 mg on blood pressure, renal haemodynamics, electrolyte excretion and plasma renin activity was studied in 10 healthy males. Isradipine did not produce a significant change in systolic or diastolic blood pressure, and glomerular filtration rate, renal plasma flow, renal vascular resistance, and urinary albumin excretion remained constant. There was a marked natriuretic and diuretic effect about 1-3 h after isradipine. Plasma renin activity showed a slight, insignificant increase 1 h after dosing. Uric acid clearance and beta 2-microglobulin excretion showed no significant changes, despite an increase in sodium clearance, suggesting an additional mechanism of action other than the proximal tubular natriuretic effect of isradipine in normotensive volunteers.

Influence of strenuous exercise on albumin excretion.

Renal albumin excretion rate was 7.3 mg/24 h (SEM 0.5, range 0.6-21.0) in 66 healthy subjects. This rate increased markedly during and shortly after strenuous exercise on a bicycle ergometer (before: 5.5 +/- 0.6 micrograms/min; during and just after: 16.9 +/- 2.2 micrograms/min; P less than 0.001; n = 30). However, albumin excretion/24 h was not significantly higher during 24 h with a period of strenuous exercise than during 24 h without such exercise (10.3 +/- 0.9 mg/24 h vs 8.5 +/- 0.7 mg/24 h).

Effects of membrane plasma exchange on the hemostatic system and on thyroid hormones in critically ill patients.

The present study examined the effects of repeated plasma exchanges with membrane filtration (Plasmaflux P2 membrane, 4.3 I human albumin solution) on the hemostatic system and on thyroid hormones in critically ill patients. Clotting factors V, VII, VIII, IX, X, XI, XII, XIII, fibrinogen, antithrombin III (ATIII), prothrombin time (PT), activated partial thromboplastin time (PTT), total (TT3) and free tri-iodothyronine (FT3), total (TT4) and free thyroxine (FT4), and thyroxine binding globulin (TBG) were determined before, immediately after, 1, 3, 6 and 24 h after plasma exchange in 6 patients (3 with glomerulonephritis, 2 with IgG myeloma, 1 with chronic polyneuritis) during 18 plasma exchanges. After plasma exchange levels of clotting factors and ATIII were markedly lowered but except for fibrinogen and factor XIII, they were not reduced by repeated exchange procedures. Thyroid hormones and TBG were reconstituted after 24 h. Pre-exchange levels, except for TT4 and TBG, were not lowered by repeated exchange procedures. Risk of bleeding or thrombosis is small and there is no appreciable risk of loss of thyroid hormones during repeated plasma exchanges.

Enzyme-linked immunosorbent assay for urinary albumin at low concentrations.

We describe an enzyme-linked immunosorbent assay (ELISA) for urinary albumin. It requires only commercially available reagents, can detect as little as 16 micrograms of albumin per liter, and analytical recovery ranges from 92 to 116%. The assay is simple, rapid, and inexpensive. Albumin excretion was 6.2 (SD 4.1) mg/24 h in healthy subjects (n = 40), 14.7 (SD 7.2) mg/24 h in albumin-test-strip-negative Type I diabetics (n = 11), and 19.7 (SD 16.2) mg/24 h in patients with essential hypertension (n = 12).

[Digitalis therapy in medical practice. Research on indications and dosage criteria in general practice]. / Digitalistherapie in der ärztlichen Praxis. Untersuchungen über Indikationen und Dosierungskriterien im niedergelassenen Bereich.

Two hundred digitalized patients under nine freely practising physicians were investigated. One hundred and ninety-six patients received digoxin or one of its derivatives. Of these, 50% did not have therapeutic serum glycoside concentrations, 48% were in the mostly subtherapeutic range and 2% were in the potentially toxic range. Signs of glycoside intoxication were not found. A substantiated indication for glycoside therapy was found in the final analysis in 55% of the patients. In 128 patients, the methyldigoxin dose calculated (0.16 +/- 0.030 mg/d) was markedly in excess of that actually prescribed (0.13 +/- 0.050 mg/d; p less than 0.001), so that there were indications of a general underdigitalization. In addition, it was not possible to anchor the restrictive kidney function as a reason for reduction of digoxin dosage in the prescription behavior. In the long run, only 36% of the patients with justified indication and therapeutic serum glycoside concentration as well as (with reservations) the 3% with potentially toxic serum glycoside concentration profited from the glycoside therapy.

The renin-angiotensin-aldosterone system during haemodialysis with acetate or bicarbonate at different dialysate sodium concentrations.

The hormones of the renin angiotensin aldosterone system were measured during regular haemodialysis with acetate or bicarbonate at dialysate sodium concentrations of 135, 140, 145, and 150 mmol/l. Plasma renin activity and aldosterone concentration were higher during acetate haemodialysis than during bicarbonate haemodialysis. At lower dialysate sodium concentrations, plasma renin activity (acetate dialysis and bicarbonate dialysis) and aldosterone concentration (only acetate dialysis) were higher than they were at higher dialysate sodium concentrations. Plasma renin activity increased during acetate dialysis, but did not change during bicarbonate dialysis. Aldosterone and potassium concentrations were positively correlated. Aldosterone decreased during haemodialysis (increase to predialysis values at the end of haemodialysis (4 h) at lower dialysate sodium concentrations). It is concluded that the renin angiotensin aldosterone system is activated more during acetate dialysis than during bicarbonate dialysis. Aldosterone concentrations seem to be related more closely to serum potassium than to renin-angiotensin-aldosterone system and to serum sodium intradialytically.