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An overview of fibrillary glomerulonephritis (GN) is given as well as the description of clinical course in four patients diagnosed and treated in our department. Fibrillary GN and immunotactoid glomerulopathy are entities, characterized by fibrillar and microtubular deposits in mesangium and the glomerular capillary loops. Decisive for diagnosis of fibrillary GN (resp. immunotactoid GN) remains the electron microscopy (EM) of the renal biopsy (RB) specimen. At the nephrologic division of 1st Internal Department of 1st Medical School of Charles University four cases of patients with fibrillary GN were diagnosed from the mid seventies (when both entities were newly described) by the end of the year 2001. In all patients the diagnosis was proven by EM. RB was indicated mainly for proteinuria, hematuria and decrease of renal function. On conclusion: though fibrillary GN/immunotactoid GN are relatively rare disorders, they represent entities, which should not be omitted in the differential diagnosis of nephrotic syndrome/renal insufficiency and which deserve further study.

Background: Diffuse necrotizing soft-tissue infections are severe due to their rapid progression; there is a vital need for settling proper early diagnosis. The task of the clinician is mainly in distinguishing localized infection from the diffuse process.

Case presentation: In our case report we demonstrate a 32 yr old man with a curious development of severe necrotizing infection of submucosis with gas production as a conclusion of perforation of Schloffer's tumour, which developed after appendectomy carried 20 years ago.

Conclusions: In case of diffuse necrotizing soft-tissue infections only immediate surgical revision can confirm initial stages of severe forms of infection.

von Willebrand disease (vWD) is the most common inherited bleeding disorder, which is highly heterogeneous ranging from asymptomatic laboratory abnormality to a life threatening bleeding. The condition is caused by a quantitative or qualitative deficiency of von Willebrand factor (vWF). Since 1994 it has been classified into six subgroups based on evaluation of the vWF level and function. Correct classification of vWD is required for its optimal management. vWD is inherited as a dominant or recessive trait linked to the vWF gene. However, the inheritance of type 1 vWD is not always linked to the vWF gene and novel modifying genes are expected to play a significant role in the type 1 pathophysiology. Laboratory tests required for correct type classification and for genetic analysis have long been awaited in Czech Republic. The purpose of this report is to inform about discriminatory and genetic tests recently made available at the Institute of Haematology and Blood transfusion.

Unlabelled: We present the results of two experiments aimed at the role of IGF-I and NO in the regulation of bone blood flow. In the first experiment A we determined the blood circulation in tibia and distal part of femur and the blood level of IGF-I after oophorectomy (OOX, 4 weeks before the experiment) and/or administration of NG-nitro-L-arginin methyl ester (L-NAME, Sigma, USA, 0.05% in the food for 10 days before the experiment). In the second experiment B we checked up the possibility of correlation between the bone blood flow and IGF-I level in female rats control and after the administration of estradiol (Agofollin Depot, Biotika, Slovak Republic, 1 mg s.c., two times weekly, for 4 weeks before the experiment). The bone blood flow was ascertained by means of 85-Sr microsphere (NEN, USA) technique, IGF level was estimated with Rat IGF-I RIA kit (DSL, USA).

Results: Experiment A: group I: controls--sham operation, group II: oophorectomy (OOX), group III: L-NAME + sham operation, group IV: OOX + L-NAME. OOX elevated the 85-Sr microsphere uptake and bone blood flow in tibia and distal femur. The administration of L-NAME to non-castrated females lowered significantly the blood flow in the femur only, whereas in OOX females it inhibited completely the usual OOX-induced increase in circulatory indicators in both bones. IGF-I level was higher after OOX, administration of L-NAME did not exert any effect on it. Experiment B: group I: control females, group II: estradiol. After the administration of estradiol, there was marked decrease in the uptake of 85-Sr microspheres and blood flow in both bones, decrease in body weight, cardiac output, heart rate, blood pressure and also in the blood level of IGF-I. Density and ash weight of the tibia were elevated. Important results found in the group I of experiment B seem to be the correlations between the blood level of IGF-I and 85-Sr microsphere uptake in tibia (r = 0.68, p < 0.01), between IGF-I and blood flow in tibia (r = 0.54, p < 0.05) and between IGF-I and 85-Sr microsphere uptake in distal femur (r = 0.55, p < 0.05). However, in the group II of females after estradiol no significant dependence could be demonstrated. The results support the conception of the role of IGF-I and NO in the regulation of local blood flow also in the bones of rats. The possible sequence of the interrelations could be as follows: OOX--increase in the blood level of IGF-I--increase in the production of NO--vasodilatation and increase in the bone blood flow. Significant correlations between the blood level of IGF-I and bone blood flow represent further evidence of the participation of IGF-I in the regulation of bone blood flow in rats.

Progressive muscular dystrophy causes both skeletal and significant cardiological changes. Electrocardiographic and echocardiographic examinations were provided in 30 patients with muscular dystrophy (17 of them with progressive muscular dystrophy Duchenne type, 13 with skeletal muscular dystrophy). In 50% cases were found left ventricle filling disorder, in two cases echocardiographic signs of pulmonary hypertension. ECG showed in one third of cases incomplete right bundle branch block, supraventricular tachycardia was also frequently found.

Nephrotic syndrome (NS) remains a serious clinical setting characterized by marked proteinuria, hypoproteinemia and hypercholesterolemia, usually accompanied by the presence of oedemas. It could be presumed, that the newly discovered hormone leptin plays an important role in the complex metabolic processes occurring in patients with NS, in which apart from the changes in the hydratation, and the protein and lipid spectre profile changes, the alteration of the metabolism of glycides elicited by the treatment with corticosteroids (CS) is often observed. The aim of the study was to investigate the plasma levels of leptin and its plasma soluble receptor (sLe-R) before and after the treatment with CS and to evaluate their relationship with albuminemia and/or proteinuria. The study group consisted of 15 men and 15 women (mean age 49 +/- 13.7 years) with newly diagnosed NS, verified by renal biopsy, in which subsequently CS treatment was started. Before the treatment (period 1) and further one month (period 2) and six months (period 3) after the start of the treatment the following parameters were measured: body mass index (BMI), serum levels of creatinine, albumin, cholesterol, triglyceride, cholinesterase, proteinuria/24 hour and plasma levels of leptin and sLe-R. In comparison to the relatively high values of BMI in the period 1 a decrease of BMI towards the physiologic range was observed during the treatment periods. Statistically significant changes were also observed in proteinuria (decrease) and in serum cholesterol and albumin levels of whereas in other biochemical parameters, including plasma leptin and sLe-R levels, statistically significant changes were not found. A trend to negative correlation with borderline statistical significance could be observed between leptin and sLe-R. The results of our relatively unique study on leptin--dealing with long-term follow-up of the patients with NS suggest that regardless prominent metabolic alterations present in NS the plasma levels of leptin and sLe-R remain relatively stable, and that of regulation of leptin in this setting is probably complex and multifactorial.

Authors deal with question, if there is possibility to infer bone histological structure (described by histomorphometric parameters of trabecular bone volume and trabecular thickness) from bone density, ash weight or even from weight of animal (rat). Both tibias of each of 30 intact male rats, 90 days old, were processed. Left tibia was utilized to the determination of histomorphometric parameters of undecalcified bone tissue patterns by automatic image analysis. Right tibia was used to the determination of values of bone density, using Archimedes' principle. Values of bone density, ash weight, ash weight related to bone volume and animal weight were correlated with histomorphometric parameters (trabecular bone volume, trabecular thickness) by Pearson's correlation test. One could presume the existence of relation between data, describing bone mass at the histological level (trabecular bone of tibia) and other data, describing mass of whole bone or even animal mass (weight). But no statistically significant correlation was found. The reason of the present results could be in the deviations of trabecular density in marrow of tibia. Because of higher trabecular bone density in metaphyseal and epiphyseal regions, the histomorphometric analysis of trabecular bone is preferentially done in these areas. It is possible, that this irregularity of trabecular tibial density could be the source of the deviations, which could influence the results of correlations determined. The values of bone density, ash weight and animal weight do not influence trabecular bone volume and vice versa: static histomorphometric parameters of trabecular bone do not reflect bone density, ash weight and weight of animal.

The results of kidney transplantation from very young paediatric cadaveric donors up to five years, which were transplanted to adult recipients, are evaluated in the first retrospective study of all Czech transplantcentres. In general, 42 of these transplantations were carried out during 1994-2001. In 28 cases single kidney was transplanted, in 14 cases en bloc graft of both children kidneys was transplanted. The reasons of kidney failure by recipients are usual (in 42.9% glomerulonephritis). An average age of the donors was 34.7 months (median 39 months). An average age of the recipients during transplantation was 42.6 years (median 43.5 years). All the kidneys were placed into retroperitoneum. As long as only single kidney was transplanted, the rules, already propagated by Salvatiera in 1970, were respected. At the transplantation of en bloc graft, Kinne's method with possible vessels elongation of the graft and with uretero-uretero anastomoses (in so called Ostrava modification) was used. The higher occurrence of the primary graft non-function (as 50% losses of all grafts) was confirmed in the study. Its reason was an acute thrombosis of vessels or rotation of graft stem. One-, three- and five-years patients and grafts survival were 97.6-90.5-90.5% and 76.2-73.8-73.8% actually. Patients survival are very good while grafts survival are average, momentarily a bit worse than national data published by the Czech Transplant Society, which determines one-, three- and five-years grafts survival with 90.3-82.9-75.5% and 79.1-71.1-65% actually. The kidney transplantation from paediatric cadaveric donors to adult recipients is acceptable under present allocated criteria, in case that some principles are kept. The study confirms the international experiences about rather higher incidence of surgical complications and primary graft non-function. In conclusion, some general references for reduction of complications, as well as for improvement of these transplantation results are introduced.

Unlabelled: Parathyroid hormone (PTH)--besides its main osteotrophic action--exerts also vascular effect demonstrated formerly also in bones. The aim of the presented work was to verify this effect of PTH using the radioactive microsphere method and to ascertain simultaneously whether NO does participate or not in this effect of PTH. We performed two experiments which were arranged in the same way, as follows: group I--controls, group II--PTH, group III--L-NAME, group IV--L-NAME + PTH. Parathyroid hormone 1.34 fragment (Sigma, USA) was administered to each animal 6-12 minutes before the injection of radioactive microspheres in the dose of 3 micrograms in the experiment A, 10 micrograms in the experiment B. NG-nitro-L-arginin methyl ester (Sigma, USA) was given in the food for ten days before the experiment in the concentration of 0.025% in experiment A, 0.05% in experiment B.

Results: We present the results of experiment A in the part "Results" of the paper briefly in percentages--they are similar to the results of experiment B, but without statistical significance. Administration of PTH increased statistically significantly the microsphere uptake in tibia and distal femur and also the blood flow in both bones, increased the cardiac output and lowered blood pressure. Administration of L-NAME alone induced decrease of the heart rate only. After the injection of PTH to the rats fed L-NAME there was--compared to the injection of PTH only--the blood flow through both bones significantly lower. The observed increase in the bone blood flow as well as changes in the general circulation show that i.v. injection of PTH under the experimental conditions used does induce vasodilatation in female rats. Influence of these changes by the administration of L-NAME indicates possible participation of the nitric oxide (NO) in the observed effect of PTH on the vessles.

The authors described anomalous development of maxillary lateral incisor non-yet published in stomatologic literature. Diagnostics and clinical findings of the anomaly and the prognosis of the affected tooth they based on the description of six longitudinal observed patients. The principal symptom of the anomaly is the disturbance of time-plan of the tooth development. Terms of the tooth mineralization, the time of eruption and root completion cannot be included either in deciduous or in permanent dentition. The tooth is the only product of the dental lamina; it has no predecessor or successor. Frequent disturbances of the eruption pathway result often in anomalous position of the tooth. Taking into consideration the fact that the anomaly appeared always in connection with hypodontia syndrome, it is possible to consider it to be the symptom of hypodontia syndrome. This fact is more significant as it enables to establish the diagnosis of hypodontia syndrome already in early deciduous dentition.