Factor VIII-hydrolyzing IgG in acquired and congenital hemophilia

Anti-factor VIII (FVIII) inhibitory IgG may arise as alloantibodies to therapeutic FVIII in patients with congenital hemophilia A, or as autoantibodies to endogenous FVIII in individuals with acquired hemophilia. We have described FVIII-hydrolyzing IgG both in hemophilia A patients with anti-FVIII IgG and in acquired hemophilia patients. Here, we compared the properties of proteolytic auto- and allo-antibodies. Rates of FVIII hydrolysis differed significantly between the two groups of antibodies. Proline-phenylalanine-arginine-methylcoumarinamide was a surrogate substrate for FVIII-hydrolyzing autoantibodies. Our data suggest that populations of proteolytic anti-FVIII IgG in acquired hemophilia patients are different from that of inhibitor-positive hemophilia A patients.     

Interventional radiology in congenital and acquired cardiovascular diseases

The paper outlines the present-day achievements in interventional radiology in cardiovascular diseases: balloon valvuloplasty in cardiac diseases (isolated pulmonary arterial stenosis, aortic and mitral stenosis), balloon vasodilatation (peripheral pulmonary arterial stenosis, aortic coarctation), embolization of the vessels and pathological communications, atrioseptostomy, transcatheter closure of atrial septal defects.     

Substructure of solitary cilia in mouse kidney

Summary Recent scanning electron microscopic studies confirm the presence of solitary cilia on most epithelial cells along the mammalian nephron and collecting ducts.By transmission electron microscopy we have found that the axonemata of such cilia consist of a maximal number of 9 doublet and no singlet filaments. 10% of the cross-sectioned cilia contain 9 doublets arranged in a peripheral ring (9+0 pattern). 30 % of the cross-sections contain 8 or 7 doublets in peripheral ring and 1 or 2 doublets in the central region (8+1 and 7+2 patterns). Serial sections and goniometer tilt reveal the central doublets to originate as dislodged peripheral doublets. 60% of the sectioned cilia contain filament numbers between 8 and 4. In patterns of 5 and 4 filaments single microtubules predominate.The functional significance of these atypical cilia is discussed.We are indebted to Prof. B. Afzelius and Prof. Th. Brun for valuable information and discussions during this work. The technical assistance of Miss K. Weltzin, Mr. E. Erichsen, Mr. R. Jensen and Mr. J. Røli is greatly appreciated     

De novo malignant solitary fibrous tumor of the kidney

Background

To study the expression of MK-1 and RegIV and to detect their pathological significances in benign and malignant lesions of gallbladder.

Methods

The expression of MK-1 and RegIV was detected by immunohistochemical method in paraffin-embedded sections of surgical resected specimens from gallbladder adenocarcinoma (n = 108), peritumoral tissues (n = 46), adenomatous polyp (n = 15), and chronic cholecystitis (n = 35).

Results

The positive rate of MK-1 or RegIV expression was significantly higher in gallbladder adenocarcinoma than that in peritumoral tissues (X² _MK-1 = 18.76, P < 0.01; X² _RegIV = 9.92, P < 0.01), denomatous polyp (X² _MK-1 = 9.49, P < 0.01; X² _RegIV = 8.59, P < 0.01) and chronic cholecystitis (X² _MK-1 = 24.11, P < 0.01; X² _RegIV = 19.24, P < 0.01). The positive cases of MK-1 and/or RegIV in the benign lesions showed moderately- or severe-atypical hyperplasia of gallbladder epitheli. The positive rates of MK-1 were significantly higher in the cases of well-differentiated adenocarcinoma, no-metastasis of lymph node, and no-invasiveness of regional tissues than those in the ones of differentiated adenocarcinoma, metastasis of lymph node, and invasiveness of regional tissues in gallbladder adenocarcinoma (P < 0.05 or P < 0.01). On the contrary, the positive rates of RegIV were significantly lower in the cases of well-differentiated adenocarcinoma, no-metastasis of lymph node, and no-invasiveness of regional tissues than those in the ones of differentiated adenocarcinoma, metastasis of lymph node, and invasiveness of regional tissues in gallbladder adenocarcinoma (P < 0.05 or P < 0.01). Univariate Kaplan-Meier analysis showed that decreased expression of MK-1 (P = 0.09) or increased expression of RegIV (P = 0.003) was associated with decreased overall survival. Multivariate Cox regression analysis showed that decreased expression of MK-1 (P = 0.033) and increased expression of RegIV (P = 0.008) was an independent prognostic predictor in gallbladder adenocarcinoma.

Conclusions

The expression of MK-1 and/or RegIV might be closely related to the carcinogenesis, clinical biological behaviors, and prognosis of gallbladder adenocarcinoma.     

Evaluation of significant gene expression changes in congenital and acquired cholesteatoma

Molecular Biology Reports - Etiopathogenesis of acquired and congenital cholesteatoma is still unclear. The clinical behavior of adult acquired, pediatric acquired and congenital cholesteatomas...     

Intracardiac atrial signal amplitude in congenital and acquired complete heart block

Background

Good and reliable atrial sensing is a fundamental part of atrioventricular (AV) synchrony in dual chamber pacemakers. Due to the floating nature of atrial sensing electrode in single pass dual chamber pacemakers (VDD) compared with two-lead dual chamber pacemakers (DDD), they are more prone to atrial under-sensing and the resulting loss of AV synchrony. We hypothesized that there is a relation between the chronicity of AV block and the amplitude of intracardiac atrial signal amplitudes (IASA).

Methods

Detailed demographic, electrocardiographic and echocardiographic data were recorded in 34 consecutive patients with congenital and acquired complete heart block (CHB). The intracardiac atrial signal amplitudes (IASA) were recorded at implantation time, 48 hours and 2 months post-implantation and compared between the two groups of patients.

Results

The mean age of the study group was 38.73±12.53 years (congenital: 30.08±11.07, acquired: 47.38±6.5). There were no important differences in left atrial or ventricular sizes and in P-wave amplitude in lead II, but the IASA was significantly higher in the congenital group at implantation time (5.21±1.86 vs. 3.38±0.84 mV, P<0.001) and during the follow-up.

Conclusion

The intracardiac atrial signal amplitudes were higher in congenital CHB compared with the acquired CHB. Chronicity (and may be the congenital type) of CHB may be an affecting factor in case selection for VDD pacemaker implantation.     

Lipodystrophic syndromes: congenital or acquired diseases of adipose tissue

Lipodystrophic syndromes regroup a heterogeneous group of genetic or acquired diseases. Lipodystrophy, an altered development and/or repartition of body fat, is associated with alterations of lipid and glucose metabolism with insulin resistance. Genetic forms, rare, can be generalized and recessive resulting from mutations in the seipin or AGPAT2 gene. Partial lipodystrophies are dominant and observed in patients mutated in the gene encoding PPAR-gamma or lamin A/C, a gene seen also mutated in patients with syndromes of premature aging. Acquired forms are common and regroup the highly prevalent Metabolic Syndrome, hypercorticism together with lipodystrophy related to antiretroviral treatment of HIV-infected patients.     

The characteristics of a change in the congenital and acquired forms of behavior in rats with genetic catalepsy

In two groups of rats--outbred control rats and GC line ones the behaviour in the open field was studied, as well as alimentary situational instrumental conditioned reflex was elaborated. Rats of GC line were less mobile in the open field as compared with the control ones; their investigating reflex was low; several parameters showed a higher level of agitation and fear. Handling of rats of GC line improves these behavioural parameters and brings them closer to the norm. Elaboration of alimentary situational conditioned reflex in rats of GC line is delayed as compared with the control ones. Passive-defensive reflex expressed in the form of cataleptic stupor, is of dominant character and significantly prevails over cognitive and alimentary reflexes.     

Functional heterogeneity of the atrial myocardium in patients with congenital and acquired heart defects]

In experiments on atrium trabeculae the heterogeneity of myocardium contractile activity in patients operated on for inborn or acquired heart defects was studied. Contractile activity was assessed in isometric regime of muscle drugs work. The degree of functional heterogeneity was reported to differ in myocardium biopsies from patients with inborn or acquired heart defects. The difference was expressed in susceptibility to stimulating action or electrical impulses and in the degree of the change of myocardium contractile activity. The study of human myocardium functional heterogeneity is likely to present a new approach to increase efficacy of the work of pathologically changed heart muscle.