Waldenstrm macroglobulinemia presenting in the form of cold urticaria and cold purpura]

A Waldenstr?m macroglobulinemia case, manifested in the form of non-specific skin-symptoms (cold urticaria and cold purpura) is described in a 37 years old female patient. As new skin-symptoms progressive telangiectasia and naevi aranei could be observed. Involvement of skin may be explained by macroglobulins with kryoprotein properties, making early diagnosis possible at the same time.     

Prediction of ovulation based on the serum estradiol level and the diameter of the follicle

Experiences with the methods are summarized. Follicular growth was monitored by ultrasound folliculometry (USFM) and estimation of serum estradiol (E2 values in physiologic cycles. USFM was found to predict follicular rupture-ovulation--more precisely than E2 values. Cases of disturbed folliculogenesis (persisting and luteinized unruptured follicle, cysts) are described. The wider use of USFM in Hungary is urged, because appropriate equipment is available while the chances of continuous hormonal monitoring are rather limited.     

Late results after repair of partial atrioventricular septal defect in adolescents and adults

From January 1966 through December 1985, 29 adolescents and adults underwent surgical repair of a partial atrioventricular septal defect at our institution. The patients included 20 females and 9 males, whose ages ranged from 16 to 47 years (mean, 27.6 +/- 10.1 years). Preoperatively, 24 patients were in New York Heart Association functional class I or II, and 5 were in class III. The pulmonary artery systolic pressure ranged from 22 to 62 mmHg (mean, 38.3 +/- 12.7 mmHg). The pulmonary-to-systemic flow ratio ranged from 1.4 to 2.9 (mean, 2.3 +/- 0.5). Upon left ventriculography, regurgitation through the left atrioventricular valve was trivial or nonexistent in 4 patients (13.8%), mild in 14 (48.3%), moderate in 10 (34.5%), and severe in 1 patient (3.4%). All patients underwent patch closure of the ostium primum defect, and all but 2 underwent partial or complete suturing of the septal commissure. One patient died within 30 days, for a hospital mortality of 3.4%. The follow-up period ranged from 7 to 25 years (mean, 15.2 +/- 5.3 years). Postoperatively, all patients were evaluated with 2-dimensional and Doppler echocardiography. One patient underwent early implantation of a permanent pacemaker for persistent complete heart block. Three patients succumbed to late death 10, 15, and 21 years after operation. Among the 25 long-term survivors, 1 patient required late valve replacement because of severe left atrioventricular valve regurgitation. Nine (37.5%) of the other 24 long-term survivors had little or no regurgitation. Of the 11 patients with moderate-to-severe preoperative left atrioventricular valve regurgitation, 4 had moderate postoperative regurgitation. Seventeen patients had a moderate or severe persistent apical systolic murmur. At the latest follow-up in 1991, 5 (20%) of the 25 long-term survivors had significant arrhythmias. At 25 years, the actuarial survival rate was 78.9% +/- 25.6%. All 25 surviving patients were in New York Heart Association class I or II. The rate of freedom from reoperation was 77.7% +/- 25.9%. We conclude that, in adolescents and adults, correction of a partial atrioventricular septal defect entails little risk and is likely to improve the patient's functional status. Repair of the left atrioventricular valve yields good results, even at long-term follow-up.     

Connective tissue metabolism in children with chronic renal failure

To assess the characteristics of connective tissue metabolism in chronic renal failure (CRF), urinary excretion of glycosaminoglycan (GAG) fractions and hydroxyproline (HYP) was determined in ten patients with CRF and in ten age-matched healthy children. CRF was found to be associated with elevated free HYP (19.9±6.1 vs 9.8±3.6 mol/day,P<0.05) and depressed peptide HYP excretion (33.1±13.5 vs 225.2±17.7 mol/day,P<0.01), a low rate of total GAG excretion (7.0±2.4 vs 16.1±1.9 mol uronic acid/day,P<0.05) with low chondroitin 4 — sulphate + chondroitin 6 — sulphate (Ch-Ss) (14.0±9.9 vs 65.0±22.1%) and a high proportion of non-sulphated or under-sulphated fractions, i.e. hyaluronic acid + chondroitin + heparan sulphate (HA+Ch+HS) (75.3±11.4 vs 31.5±5.7%). Urinary 3-methyl-histidine (3-met-HIS) excretion and plasma essential free amino acids did not differ in the two groups. In response to haemodialysis no consistent change occurred in urinary excretion of 3-met-HIS, peptide-bound HYP, total GAG or percentage distribution of individual GAG fractions. After haemodialysis all plasma amino acids decreased significantly, and there was a significant increase in urinary excretion of free HYP (P<0.05). We conclude that the alterations in urinary excretion of total and individual GAGs observed in CRF may reflect disturbed connective tissue metabolism which does not appear to be accounted for by protein malnutrition or enhanced protein breakdown and remains uninfluenced by haemodialysis therapy.     

A new psychoactive 5H-2,3-benzodiazepine with a unique spectrum of activity

The neuropharmacological effects of 1-(4-amino-phenyl)-4-methyl-7,8-dimethoxy-5H-2,3-benzodiazepine (GYKI 52 322) were investigated and compared with those of chlordiazepoxide and chlorpromazine. This novel 2,3-benzodiazepine displays neuroleptic activity in the apomorphine-climbing (ED50 = 1.15 mg/kg i.p.) and swim-induced grooming (ED50 = 6.9 mg/kg i.p.) tests in mice and it inhibits the conditioned avoidance response in rats (ED50 = 8.2 mg/kg i.p. and 9.8 mg/kg p.o.). However, it does not antagonize apomorphine-evoked vomiting in dogs; or stereotypy, hypermotility and turning in rats even at as high a dose as 50 mg/kg i.p. On the other hand it is active in the hole board test in mice (MED (minimal effective dose) = 0.5 mg/kg i.p.) and in the lick conflict assay in rats (MED = 5 mg/kg i.p.), indicating anxiolytic property. It shows antiaggressive effect in the fighting mice test (ED50 = 8.1 mg/kg p.o.) and the carbachol-rage procedure in cats (active at 10 mg/kg i.p.) According to the biochemical findings, this compound does not bind to the central dopamine receptors (IC50 greater than 10(-4) mol/l), but it shows affinity to the 5-HT1 receptors (IC50 = 7.1 x 10(-6) mol/l) and inhibits brain cAMP-phosphodiesterase (IC50 = 2.4 x 10(-5) mol/l). The substance causes no elevation of dopamine turnover and serum prolactin level suggesting fewer side effects. So the term "atypical neuroleptic agent" is proposed to characterize this molecule.     

The cerebrocortical microcirculatory effect of nitric oxide synthase blockade is dependent upon baseline red blood cell flow in the rat

The effects of nitric oxide synthase (NOS) blockade on the cerebrocortical microcirculation were investigated under physiological conditions in anesthetized ventilated rats using laser-Doppler (LD) flowmetry. LD flow values of the parietal cortex were determined before and after systemic administration of the NOS inhibitor N(G)-nitro-L-arginine-methyl-esther. NOS blockade reduced the LD flow significantly and the magnitude of the reduction was in close correlation with the baseline value. Synchronized sinus-wave-like LD flow oscillations were observed frequently after NOS inhibition and their appearance was also dependent on the high baseline flow values. These results indicate marked, baseline-dependent differences in the cerebrocortical blood flow response to the inhibition of the nitric oxide pathway, and may suggest that areas with high resting red blood cell flow express high NOS activity.     

Inflammatory responses in blood samples of human immunodeficiency virus-infected patients with pulmonary infections

We analyzed the characteristics of the inflammatory response occurring in blood during pulmonary infections in human immunodeficiency virus (HIV)-infected patients. A prospective study of consecutive hospital admissions of HIV-infected patients with new-onset radiologic pulmonary infiltrates was carried out in a tertiary university hospital from April 1998 to May 2001. Plasma cyclic AMP receptor protein (CRP), interleukin 1beta (IL-1beta), IL-6, IL-8, IL-10, and tumor necrosis factor alpha (TNF-alpha) levels were determined at the time of admission and 4, 5, and 6 days later. Patients were included in a protocol addressed to study etiology and outcome of disease. A total of 249 episodes of infection were included, with the main diagnoses being bacterial pneumonia (BP) (118 episodes), Pneumocystis carinii pneumonia (PCP) (41 episodes), and mycobacteriosis (36 episodes). For these three patient groups, at the time of admission the median CRP and cytokine levels were as follows: CRP, 10.2, 3.8 and 5 mg/dl, respectively (P = 0.0001); IL-8, 19, 3, and 2.9 pg/ml (P = 0.045); and TNF-alpha, 46.4, 44, and 75 pg/ml, respectively (P = 0.029). There were no significant differences in levels of IL-1beta, IL-6, or IL-10 among the patient groups. A total of 23 patients died. At the time of admission, HIV-infected patients with BP had higher plasma CRP and IL-8 levels than did PCP and mycobacteriosis patients. TNF-alpha levels were higher in patients with mycobacteriosis. An elevated IL-8 level (>61 pg/ml) at the time of admission was an independent factor associated with higher mortality (odds ratio, 12; 95% confidence interval, 1.2 to 235.5).