[Waldenström's macroglobulinemia: clinical aspects, diagnosis and therapy].
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Biomedical subjects
Publications and source records attributed to H Brass.
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24-hour ambulatory blood-pressure measurements were obtained according to criteria of the German Hypertension League in 61 non-insulin-dependent diabetic patients after admission to hospital under clinical routine conditions. 30 patients had no signs of nephropathy; 15 patients showed signs of proteinuria of more than 0.5 g/d and/or renal insufficiency, and 16 patients were on chronic hemodialysis renal replacement therapy. Despite antihypertensive therapy, the majority of NIDDM patients with nephropathy and/or dialysis therapy were hypertensive. Hypertension of non-nephropathic patients showed a better response to therapy. About 50% of all patients with nephropathy had a higher mean arterial blood pressure at night than during the daytime. In about 25% of all diabetics with nephropathy, we found, during night time, an especially pronounced increase of both systolic and diastolic blood pressure of more than 5% above the daytime values. Diabetic patients without nephropathy already show a reduced night/daytime variation of blood pressure, however, inverse circadian rhythm as a sign of prognostically non-favorable autonomic neuropathy was found almost exclusively in the nephropathic diabetic patients.
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A 53-year-old man developed a septic fever up to 40 degrees C, pancytopenia and hepatosplenomegaly after a holiday in Spain. Administration of piperacillin and amikacin was ineffective, but the fever subsided and partial haematological remission occurred when 1 mg/kg methylprednisolone daily was added. After six months his general condition worsened and pancytopenia with typical inclusion bodies in bone-marrow macrophages was noted, leading to the diagnosis of visceral leishmaniasis (Kala-Azar). The diagnosis was confirmed by serological tests. The causative organism was eliminated and the abnormal findings regressed during treatment with sodium stibogluconate, at first 600 mg/d for two weeks, then 850 mg/d over 16 days, interrupted for 14 days because of side effects.
Eighty-eight dialysis patients were vaccinated with recombinant hepatitis B vaccine prepared in yeast. Fourty-nine patients were immunized 3 times (months 0, 1, 6) intragluteally with 40 micrograms hepatitis B surface antigen (HBsAg) per dose. Only 32 of them (65.3%) showed anti-HBs concentrations above 10 IU/l with a geometric mean titer (GMT) of 180.7 IU/l after 3 vaccinations, whereas all of the 16 healthy controls, vaccinated 3 times with a 10-micrograms dose of the same vaccine batch, had specific antibodies higher than 10 IU/l (GMT 897.4 IU/l). Responses of patients were slightly higher than those of dialysis patients vaccinated in an earlier study with plasma-derived vaccine according to the same schedule. Results in 20 patients immunized 6 times intragluteally with 40 micrograms HBsAg/dose in monthly intervals were not better (at month 7, 65% showed anti-HBs concentrations greater than 10 IU/l; GMT = 126.6 IU/l), and 19 patients receiving 6 times 20 micrograms HBsAg monthly showed significantly lower responses (anti-HBs greater than 10 IU/l in 42% of vaccinees, GMT = 89.5 IU/l). The vaccine was tolerated well; side-effects were slight, and no serious adverse reactions were observed. In conclusion, recombinant hepatitis B vaccine is comparable to plasma-derived vaccine also in the case of dialysis patients; a 6-dose schedule does not seem to have much advantage compared to the conventional 3-dose regimen.
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Familial Mediterranean fever was diagnosed in a 34-year-old Turkish patient with severe nephrotic oedema. Immunohistochemical classification of a biopsy specimen showed amyloidosis of the AA-type. There was a definite increase of serum amyloid-A-protein (SAA). The typical recurrent fever, attacks of abdominal pain with symptoms of subileus and joint swelling could be treated successfully with colchicine, the oedema with diuretics. The progression of renal failure and proteinuria as indicator of the degree of amyloid-induced renal damage remained unaffected by this treatment. With dimethyl-sulfoxide (DMSO) a marked improvement in renal function and a lowering of the SAA level could be achieved. Thus this treatment inhibits the progression of amyloidosis of the AA-type in Mediterranean fever and may be considered for other forms of AA-type amyloidoses. It is possible that the lowering of the SAA-serum concentration and the improvement of renal function is due to an antiphlogistic effect of DMSO, the mechanism of action of which is so far unknown.
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Starting from toxic concentrations of the new scilla glycoside 14-hydroxy-3beta-[(4-O-methyl-alpha-L-rhamnopyranosyl)-oxy]-14beta-bufa-4,20,22-trienolide (meproscillarin, Clift) 5 in vitro hemoperfusions with the hemoperfusion system Haemocol are described. The test showed that meproscillarin may be rapidly eliminated from the blood by adsorption to activated charcoal (hemoperfusion). The text provides basic information for possible intoxications.
The symptoms of hematuria are easily and accurately detected through careful sediment diagnosis. The path from initial indications to diagnosis often means a major expenditure of laboratory methods, of bioptic excisions, and of radiologic procedures for the examination. Above all, vascular and infected lesions of the kidneys and of the eliminative urinary tracts can cause hematuria, which in every case requires clarification.
The effects of creatinine (5.6-22.6 mg/100 ml), guanidinosuccinic acid (8.7-35.2 mg/100 ml) and of urea (60-600 mg/100 ml) on the mechanical function and oxygen consumption in isolated guinea pig hearts have been assessed. None of the parameters measured (dp/dt max, dp/dt min and Q O2) was significantly affected by creatinine or guanidinosuccinic acid. However, urea significantly reduced mechanical activity and caused a marked increase of oxygen consumption, indicating impairment of heart function expressed as a diminution of the ratio formula (see text). Pretreatment with creatinine and guanidinosuccinic acid did not alter the effect of norepinephrine on mechanical activity and oxygen consumption when compared with the effects of norepinephrine (1 X 10(-8) g/ml and 1 X 10(-7) g/ml) given alone. In contrast, urea pretreatment lowered the norepinephrine induced increase of left ventricular pressure rise/fall and of oxygen consumption. In addition, reduction of the increase in the ratio formula (see text): after urea perfusion indicates diminution of the "economic" effect of norepinephrine.
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A 34-year-old woman developed uremia secondary to severe renal thrombotic microangiopathy after 3 years intake of oral contraceptives. In this particular case manifestation of end stage renal failure was preceded by an unusually long lasting period of nine months with benign hypertension. Even during the final stage (3-4 weeks) prior to complete development of uremia only once hemolysis but no malignant hypertension was observed. Only close long term follow up including renal biopsy and subtile functional tests may provide information whether and/or when hypertension due to oral contraceptives turns to become--at least in part--renal hypertension and also becomes persistent. This observation does not give evidence that benign hypertension causes renovascular damage and thus renal failure. Plasma renin activity was found to be basically elevated and furthermore stimulated e.g. by dialyses. However, this single observation does not permit any conclusion about a pathogenetic role of renin in creating hypertension by e.g. renal vasoconstriction or--despite hypertension--collapse of the capillary network.