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Biomedical subjects

Z Frysák

Publications and source records attributed to Z Frysák.

At least 19 recordsLinked to original sources

[Problems with interpretation of low levels of thyroidal hormones conditioned by changes in transport proteins].

In clinical practice, we often observe conditions accompanied by secondary drop of binding proteins that bind, more or less specifically, thyroidal hormones. This is usually considered as normal situation that is often not properly interpreted from clinical point of view. In other words, we tolerate such conditions because we build on values of free hormones FT3 and FT4. However, it is very rare to observe significant decrease or even absence of thyroxin binding globulin (TBG) due to inborn error of metabolism. In such situations, the overall level of thyroidal hormones becomes a part of evaluated laboratory profile. Unusual laboratory constellation is in sharp contrast to the so-called "healthy patient". Due to increased migration of persons, we had an opportunity to take care of a patient of this kind.

Heroin↗

[Thrombotic thrombocytopenic purpura-hemolytic uremic syndrome (TTP-HUS) leading to the diagnosis of Wilson's disease].

Authors describe the case of a patient suffering from Thrombotic Thrombocytopenic Purpura--Hemolytic-Uremic syndromee. Any cause of the disease was not found, except signs of liver injury. The etiology of indefinite liver disease that had been diagnosed several years before was examined. Wilson's disease was considered as a final eventuality. The finding of 488 micrograms of copper in the dry liver tissue confirmed the diagnosis of Wilson's disease in the end.

Adult↗

[Effect of portosystemic anastomosis on the degree of splenic inhibition].

UNLABELLED: Secondary hypersplenism is a well-known complication of portal hypertension. Reduced platelet count is a more alarming sign for the physician than risk for the patient. Improvement of thrombocytopenia is urgent, when portal hypertension with splenomegaly and thrombocytopenia presents with life-threatening haemorrhage from gastroesophageal varices. In this case, treatment aimed at stopping the bleeding may be more beneficial than any intervention on the spleen. In this study, we evaluated long-term effects of an elective distal splenorenal shunt or small diameter H-shunt on splenomegaly and thrombocytopenia in 26 patients with portal hypertension operated for repeated bleeding from oesophageal varices. 25 patients had splenomegaly and 16 patients had thrombocytopenia before shunting. Surgery corrected splenomegaly in 16 patients (64%), platelet counts increased in 13 of 16 patients with thrombocytopenia (81.2%). CONCLUSION: Selective or partial portal decompression is sufficient to alleviate thrombocytopenia and splenomegaly associated with portal hypertension.

Adult↗

[Hyperparathyroidism in clinical practice. Personal experience].

The period of six years is discussed by the authors during which 39 patients were confirmed having primary or secondary hyperparathyroidism. All of them finally underwent the surgical procedure. The possibility of immunoreactive PTH measurement has been highly appreciated. Despite that key position of the experienced surgeon remains of the utmost importance.

Adult↗

[The hepatic form of Wilson's disease in young patients].

UNLABELLED: Wilson's disease (WD) is a hereditary disorder of the copper metabolism with very varied clinical and biochemical symptoms. Hepatic and neurological forms are the most frequent manifestations of this rare disease. In schoolchildren and adolescents symptoms of liver damage predominate. In a retrospective study 19 patients were evaluated with biochemical signs of hepatopathy manifested before the age of 18 years. The diagnosis of WD was established at the age of 7 to 27 years. One female patient was admitted with fulminant hepatic failure which was treated by acute transplantation of the liver in the Institute of Clinical and Experimental Medicine in Prague. Only 9 of 18 patients with chronic hepatic affection at the time of diagnosis met the Sternlieb diagnostic criteria. These patients had reduced ceruloplasmin levels (0.08-0.18 g/l) and a high copper content in the hepatic dry matter (783 ug/g +/- 323 [SD]). In the remaining 9 patients the ceruloplasmin level was normal, however, in 8 a high copper content of the hepatic dry matter was found (696 ug/g (+)- 352[SD]. The last patient from this group had Kayser-Fleischer's (K-F) ring. It was possible to confirm the high copper content in the hepatic dry matter only after one year's penicillinamine treatment because at the time of the diagnosis poor coagulation did not permit to perform a liver biopsy. There was a statistically significant difference in the copper content of the hepatic dry matter in patients meeting and not meeting Sternlieb's criteria. Statistically significant differences between both groups were found in the plasma copper levels and in the 24-hour urinary copper excretion. Histological examination of the liver under a light microscope revealed findings from minimal changes associated with the presence of glycogen nuclei in hepatocytes to the picture of active chronic hepatitis. In all 19 patients the gene mutation H1069Q was examined and the results were positive in 39.8%. In 3 asymptomatic patients it was present in the homozygous form. CONCLUSION: Early detection of the atypical form of WD remains very difficult. The gold standard is still in all cases assessment of copper in the dry liver tissue. In the near future an important place will be held also by direct DNA analysis although its use is limited not only by the large number of known mutations but also by the financial costs of the method.

Adolescent↗

[Idiopathic hypoparathyroidism with celiac disease--diagnostic and therapeutic problem].

A middle-aged woman developed gradually diseases where most probably the common denominator is an autoimmune process. The authors describe the clinical condition persisting for seven years, where hypoparathyroidism was associated with coeliac disease which did not respond to dietary measures. Malabsorption was manifested by a selective block for calcium absorption from the gut. The impaired conversion of vitamin D2 to D3 is of interest; it has obviously a multifactorial etiology. The main problem is the patient's tendency to respond by tachyphylaxis to oral vitamin D administration. The only effective therapeutic procedure apart from those listed is so far parenteral administration of vitamin D3, calcitrol.

Adult↗

[Fulminant liver failure as a sequelae of acute dystrophy of the right hepatic lobe].

A 38-year-old female patient developed signs of fulminant hepatic failure, caused by extensive, spontaneous, devastating ischaemia of the right liver lobe. The patient survived the acute attack. During the subsequent eight years hypertrophy of the intact left liver lobe occurred which eventually completely replaced the loss of the dominant right lobe.

Acute Disease↗

[Portosystemic anastomosis at the time of transplantation and TIPS].

Sclerotherapy has become in the treatment of acute haemorrhage of oesophageal varices the method of first choice. The relapse of haemorrhage within a five-year interval is however too frequent after this treatment. TIPS is a new method which can resolve profuse haemorrhage from varices in patients with a markedly reduced hepatic reserve. The high incidence of stenosis or occlusion (50% during the first six months) however foresees the use this method rather for bridging the interval before definite therapy i.e. transplantation of the liver. Patients with a sufficient functional hepatic reserve and those who are candidates for transplantation can be protected with a very good effect against relapsing haemorrhage by a surgical anastomosis.

Esophageal and Gastric Varices↗

[Adrenal incidentalomas. Clinical experience].

In the course of two years the authors treated 27 patients with incidentally detected adrenal tumours. Detailed morphological and laboratory examination revealed four cases of non-classical late type adrenogenital syndrome (AGS) and two pheochromocytomas. From the submitted paper ensues that it is essential to provide interdisciplinary comprehensive treatment in departments which have sufficient experience with this problem.

Adrenal Gland Neoplasms↗

[Colchicine in the treatment of liver cirrhosis. 12 years' experience].

The authors evaluate more than 12 years of their own experience with the administration of colchicine to patients with cirrhosis of the liver. Colchicine did not produce any apparent undesirable effects. The authors emphasize the absence of knowledge of prognostic parameters (except the stage of the disease and its activity) which would make it possible to evaluate in advance the effect of many years' treatment. They deal separately with a patient who entered their investigation in a very advanced stage of the disease and survived after he had recovered full compensation and did not depend on diuretic treatment.

Adult↗

Effect of physical load on the platelet function and ultrastructure in patients with ischemic heart disease.

The aggregability of the blood platelets and their ultrastructure were examined during the bicycle exercise test in 20 patients with ischemic heart disease and stable angina pectoris. In 8 patients with unequivocally positive exercise test a significant increment of stimulated aggregation from 58.2 (CV7%) to 71.2 (13.6%) resulted in comparison with a group of patients who did not fulfilled criteria of exercise testing. In this group the aggregability dropped from 56.0 (5%) to 38.7 (8.4%) eventually. No changes in a diameter and shape were observed during the exercise test was positive the amount of granules decreased. It is considered to be an indirect proof of the functional changes in thrombocytes during a certain rise in aggregability.

Adult↗

[Life prognosis in patients with prehepatic portal hypertension treated with portosystemic shunts].

The authors present the results of the long-term follow up of nine patients with prehepatic portal hypertension treated by a portosystemic anastomosis. The evaluation was made 5-21 years after operation, according to criteria focused on the therapeutic effect of the anastomosis and its possible pathophysiological sequelae. In the authors' opinion the anastomosis is the operation of choice suited for patients lacking a tendency of normalization of the portal circulation and with dangerous manifestations of portal overpressure. The indication is restricted to early age of the patients (for technical reasons). Conversely the patient should not attain adult age without unresolved hypertension. For correct timing of the operation knowledge of the development of the disease is essential. Primarily infact livers do not undergo pathological changes even after years of hypertension nor several years after an anastomosis. This is consistent with the fact that these patients lack clinical manifestations of portosystemic encephalopathy. Investigations of the course of flow curves during semiquantitative evaluation of hepatobiliary sequential scintigraphy supported the previous view that establishment of the anastomosis and elimination of portal overpressure improves hepatic perfusion. Hepatobiliary sequential scintigraphy not previously used in this indication may be of value in the follow up of the disease and evidence of the anastomosis. This preliminary conclusion is being tested in a prospective study.

Adult↗

Ultrasonography of primary liver carcinoma (PLC) and targetted cytologic examination.

A primary liver cell carcinoma was disclosed in 11 cases out of a total of 61 patients with a focal hepatic lesion. The authors investigate the diagnostic significance of abdominal ultrasonography (US) as a major diagnostic modality in early detection of PLC. It is concluded that abdominal US is an important contribution to diagnosis of hepatic lesions smaller than 1 cm in diameter. The echographic features of PLC are often unspecific thus requiring a complementary targetted cytologic examination. A short comparison of US computed tomography and liver scintigraphy is given.

Aged↗

[New possibilities of treatment of pancreatic pseudocyst].

The authors evaluate the therapeutic results in patients with pancreatic pseudocysts where percutaneous puncture under ultrasonic control was used. Five patients were thus cured. In six patients, despite repeated punctures, treatment was not successful and internal drainage was made by surgery. The authors consider treatment of pseudocysts by puncture in suitably selected patients fully indicated because it is an unpretentious and relatively safe method.

Adult↗

Effect of colchicine on the activity of cathepsin B and D in human liver cirrhosis.

The activity of cathepsin B and D in human liver biopsy specimens from cirrhotic patients before and after one year of colchicine treatment was studied. The hydroxyproline content as a marker of the amount of collagen in tissue specimens was also determined. The hydroxyproline content in the liver samples was two or threefold that of the control group. After colchicine it remained unchanged or in some patients its values were decreased. Cathepsin B activity was higher in cirrhotic liver samples as compared with the controls, whereas the increased activity of cathepsin D was not significant. The ratio of cathepsin B and D activity to hepatic hydroxyproline content was significantly reduced in cirrhotic livers. Colchicine treatment was followed by an increase in the levels of the enzymes investigated as well as by a significant rise in the ratio of cathepsin B and D activity to hepatic hydroxyproline content.

Cathepsin B↗