[Incipient pericardial tamponade as the first symptom of systemic lupus erythematosus in 2 children]. / Prijeteca perikardijalna tamponada kao prvi simptom sistemnog eritemskog lupusa u dvoje djece.

Two patients (a boy and a girl), with systemic lupus erythematosus (SLE), in which pericarditis with threatening tamponade was an initial symptom of disease, are presented. Pericardial tamponade is very rare initial manifestation, described in only 1-3% of all the cases of SLE. The typical clinical features of tamponade are described (tachycardia, hypotension, venous congestion) and the importance of echocardiography in early diagnosis of pericarditis and pericardial tamponade is pointed out. Therefore, the echocardiography is considered as the complementary method in diagnosis of SLE.

Clinical features of Balkan endemic nephropathy.

This paper describes the clinical symptoms and signs of Balkan endemic nephropathy (BEN). The initial asymptomatic period followed by weakness and lassitude during renal insufficiency is emphasized. Non-characteristic lumbar pain may be present and episodes of macrohaematuria may occur. There is no fever, no severe dysuria, and no general disease preceding the symptoms. No oedema of the nephrotic type is recognized. Working capacity is unaffected until the late stage of the disease. In the advanced stages pallor of the skin and xantochromia of palms and soles are noticeable. Blood pressure is normal, but in the advanced phase may be elevated. In the uraemic phase a full uraemic syndrome is found. Urothelial tumours are frequent, occurring in 2-47% of cases; tumour cells may be found in the urine. Proteinuria of tubular type may be found early; in the uraemic phase it is constant. In the urinary sediment there are scarce white and red blood cells (the latter episodically abundant). Bacteriuria is present in about 20% of women patients. Glucosuria (less than 10%) and aminoaciduria (less than 10%) have been reported. In the early stages of BEN the urine concentration capacity is impaired earlier than renal blood flow and glomerular filtration rate. Enzymuria is present in the early phases. Tamm-Horsfall protein may be increased in the urine. Normo- or hypochromic normocytic hyporegenerative anaemia is a frequent finding. Bone marrow and leucocytes are normal. Serum proteins and immunoglobulins are not altered. Complement is normal; anti-glomerular basal membrane and anti-tubular basal membrane are negative. On radiography, kidney size varies from normal to the size of a small contracted kidney. The clinical picture of the disease is that of a slowly progressing tubulo-interstitial chronic nephritis.

Tamm-Horsfall protein determination in Balkan endemic nephropathy.

Data on the excretion of Tamm-Horsfall protein (THP) in subjects living in an area of Balkan endemic nephropathy (BEN) are reported. The study subjects were divided into groups as follows: diseased, suspect, "at risk" and others, according to previously adopted criteria. The THP excretion in "at risk" subjects was found to be significantly higher as compared to control subjects. The difference between these two groups could not be registered by any other clinical or laboratory diagnostic methods. No difference in the excretion of THP was observed between the groups of others and control subjects. According to the results obtained, the excretion of THP may be considered a possibly useful additional diagnostic test for the detection of subjects with the latent, early subclinical phase of BEN. On the other hand, the data obtained shed some more light on the still obscure pathogenesis and natural history of BEN.

Relationship between tubular and Tamm-Horsfall proteinuria in Balkan endemic nephropathy.

Excretion of low molecular mass proteins derived from blood plasma and Tamm-Horsfall protein, a specific renal protein, was determined in the subjects from the area of Balkan endemic nephropathy. A significantly higher excretion of Tamm-Horsfall protein was observed in the subjects with tubular type of proteinuria compared to the subjects with glomerular selective type proteinuria and the subjects with physiological type proteinuria.

Tamm-Horsfall protein in Balkan endemic nephropathy.

It is suggested that Tamm-Horsfall protein, a specific renal glycoprotein, may be involved in the pathogenesis of some renal diseases. In cadmium nephropathy and Fanconi syndrome (primary tubular diseases of the kidney) an increased excretion rate of Tamm-Horsfall protein has been observed. Balkan endemic nephropathy is a chronic tubulointerstitial disease of unknown etiology, most probably a primary disease of the kidney tubules with secondary reaction of the interstitial tissue. Investigation of Tamm-Horsfall proteinuria in Balkan endemic nephropathy has shown that subjects living in the area where this condition is prevalent have a significantly higher Tamm-Horsfall protein /creatinine ratio than those living in the control area where the condition has not been observed. Differences in this ratio among diseased, suspect and subjects "at risk" were not observed, despite differences in their glomerular filtration rates. But excretion of Tamm-Horsfall protein per litre of glomerular filtrate was significantly different among diseased, suspect and subjects "at risk" and significantly higher compared to control subjects. a relatively significant correlation was obtained between Tamm-Horsfall protein excretion rate and glomerular filtration rate as measured by creatinine clearance in both control and subjects living in the area of Balkan endemic nephropathy. Determination of Tamm-Horsfall protein in urine together with determination of proteinuria by electrophoresis on cellulose acetate membranes as a screening procedure, and by SDS -electrophoresis in polyacrylamide gell may be useful laboratory tests in detecting this nephropathy.

An etiological approach to Balkan endemic nephropathy based on the investigation of two genetically different populations.

The direct inheritance as a possible etiologic factor of Balkan endemic nephropathy (BEN) has been studied in an endemic area in the district of Slavonski Brod (Croatia-Yugoslavia). The basis for the investigation was given by a natural experiment induced by immigration of people from Ukraina to the endemic and nonendemic areas near Slavonski Brod. 1,536 persons in endemic villages and 733 inhabitants from the villages, where BEN has not been found, were studied with the aim of establishing diagnosis or excluding BEN. The generally adopted criteria enabling to classify the examined persons into the following 3 groups have been used: (a) diseased of BEN; (b) suspect to be diseased of BEN, and (c) individuals without signs of a renal disease. In the endemic villages among the population of Croatian aborigines, 12,1% diseased and suspects have been revealed. Among the Ukrainian immigrants and their offsprings, 10.5% diseased and suspects have been found. The difference is not significant (t = 0.7, p greater than 0.05). On the other hand, in nonendemic villages there were no diseased nor suspect BEN patients among the indigenous people as well as among the Ukrainians. The inference is suggested that the factors of the direct inheritance do not play a decisive role in the etiopathogenesis of BEN.

Characteristics of proteinuria in endemic nephropathy.

Subjects living in the endemic area of Posavina round Slavonski Brod have a significantly greater incidence of proteinuria (13.6%) than those living under the same conditions in the area where endemic nephropathy has not been observed (5.5%). Following determination of the grade and type of proteinuria, physiologic proteinuria was found in 36% of subjects from the endemic area. In nonphysiologic proteinuria the incidence of tubular proteinuria was 41%. A slight selective glomerular proteinuria was found in 51%, and other types of proteinuria in 8% of subjects. Tubular proteinuria was significantly higher in the endemic area than in the control area. The subjects with tubular proteinuria from the endemic area excrete significantly greater amounts of beta 2-microglobulin and light immunoglobulin chains.

beta2-Microglobulin in Balkan endemic nephropathy.

The results of low molecular weight (LMW) proteinuria test in urines of patients and suspect subjects in an endemic area of Balkan endemic nephropathy (BEN) in Yugoslavia are reported. An accordance between the positive LMW test and the clinical diagnosis of BEN has been found in more than 70%. The authors claim that LMW immunodiffusion test is a very useful procedure for detecting BEN and a great aid in the epidemiological work in the endemic field. The results of determining beta2-microglobuline (beta2m) in serums and urines by radioimmunoassay technique as well as counting of clearances of endogenous creatinine in healthy persons, "endangered" subjects in endemic area, and BEN patients without azotemia, with azotemia and uremia are reported. The group of 17 patients suffering of BEN without azotemia having slightly or more markedly lowered creatinine clearances showed higher serum and much higher urinary concentration of beta2m which are not in proportion with creatinine clearance. The results are interpreted so far as a consequence of the predominant tubular lesion in BEN. In groups of patients with azotemia and uremia no differences in beta2m handling have been found. The report has a preliminary character and requires further study.