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Biomedical subjects

Z Jakab

Publications and source records attributed to Z Jakab.

At least 19 recordsLinked to original sources
PubMed2006

Leukaemia incidence and survival in children and adolescents in Europe during 1978-1997. Report from the Automated Childhood Cancer Information System project.

Leukaemias constitute approximately one-third of cancers in children (age 0-14 years) and 10% in adolescents (age 15-19 years). Geographical patterns (1988-1997) and time trends (1978-1997) of incidence and survival from leukaemias in children (n=29,239) and adolescents (n=1929) were derived from the ACCIS database, including data from 62 cancer registries in 19 countries across Europe. The overall incidence rate of leukaemia in children was 44 per million person-years during 1988-1997. Lymphoid leukaemia (LL) accounted for 81%, acute non-lymphocytic leukaemia (ANLL) for 15%, chronic myeloid leukaemia (CML) for 1.5% and unspecified leukaemia for 1.3% of cases. Adjusted for sex and age, incidence of childhood LL was significantly lower in the East and higher in the North than in the British Isles. The overall incidence among adolescents was 22.6 per million person-years. The incidence of LL was rising in children (0.6% per year) and adolescents (1.9% per year). During 1988-1997 5-year survival of children with leukaemias was 73% (95% CI 72-74) and approximately 44% for infants and adolescents. Similar differences in survival between children and adolescents were observed for LL, much less so for ANLL. Survival differed between regions; prognosis was better in the North and West than the East. Remarkable improvements in survival occurred in most of the subgroups of patients defined by diagnostic subgroup, age, sex and geographic categories during the period 1978-1997. For children with ANLL most improvements in survival were observed in the 1990s.

Adolescent↗
PubMed2001

[Genetics of renal tumors].

The quintessence of malignant transformation is the genetic alteration of the tumor progenitor cell, i.e. somatic mutation. The genetic change appearing at chromosome and/or gene level results in the disturbance of the balance of cell proliferation and differentiation. In solid tumors, including renal tumors, the basic genetic mechanism proved to be the loss of function of a specific gene pair caused by loss of the particular chromosome or chromosomal region (monosomy, deletion) or by mutation of the gene. In the well studied Wilms' tumor-aniridia-syndrome the distal part of 11p13 region is deleted. The responsible gene is the WT-1 tumor suppressor gene, a Zn finger type transcription factor. In the majority of cases the mutation of this gene leads to the tumor formation without cytogenetically detectable deletion. For manifestation of the tumor the functional damage of both alleles is needed. In other histological types of renal tumors a great variation of chromosome losses and gains, as well as translocations can be identified. In Wilms tumor of embryonic origin, tumor suppressor genes located on the short arms of chromosomes 16 and 17 play a role in the pathogenesis. Besides, the significance of abnormal genomic imprinting of IGF2 and H19 genes located on 11p15 has also been confirmed. If a part of the embryonic cells do not regress, they may develop to papillary carcinoma together with the appearance of trisomies of chromosomes 7 and 17 and loss of Y. In the transformation process from papillary adenoma to carcinoma, duplication of several chromosomal regions is involved (3q+, +8, +12, +16, +20). The origin of renal carcinoma developing from normal nephron cells is associated with a deletion of 3p and 5q+, while during the progression of the disease further variable chromosome losses appear (6q-, 8p-, 14q-, -9). Tumor-specific cytogenetic and molecular genetic changes confirm the morphological classification of epithelial renal tumors pointing at the relation of the various entities or their independence. Based on cytogenetic alterations, a sequential predictive model of renal tumors can be developed. Individual types, together with joining and sequential appearance of aberrations are in line with the multistep mechanism of carcinogenesis. At the same time, the specific cytogenetic and molecular genetic changes confirm the diagnosis, provide further information about the histological type and progression of the disease. In hereditary cases, the members of the family at risk can be identified by recognizing the possibly associating clinical symptoms and/or by detecting the constitutional mutation of the gene using molecular genetic methods. Consequently, the genetic study of renal tumors plays important role not only in diagnosis and choosing adequate therapy but also in prevention of the disease.

Adenoma↗
PubMed2000

Ineffability of qualia: a straightforward naturalistic explanation.

In this paper I offer an explanation of the ineffability (linguistic inexpressibility) of sensory experiences. My explanation is put in terms of computational functionalism and standard externalist theories of representational content. As I will argue, many or most sensory experiences are representational states without constituent structure. This property determines both the representational function these states can serve and the information that can be extracted from them when they are processed. Sensory experiences can indicate the presence of certain external states of affairs but they cannot convey any more information about them than that. So, format- or code-conversion mechanisms that link different systems of representation (linguistic and perceptual) to each other will fail to extract any relevant information from sensory experiences that could be coded in language. They only way to establish specific roles for sensory experiences in communication and the organization of behavior is to attach to them, by associative links, words, or other behavioral responses. If a sensory experience has no linguistic label associated to it in a particular subject, then no linguistic description can token, or activate, that state in the subject. In other words, no linguistic description can cause a subject to undergo an unlabeled perceptual state. On the contrary, complex, or syntactically structured perceptual states can be built up, on the basis of descriptions, by mechanisms of constructive imagination (conceived here as one sort of format conversion). It is this difference between complex and unstructured representational states that gives us an understanding of the phenomenon we call the ineffability of qualia.

Color Vision Defects↗
PubMed1999

Biclonal chromosomal aberrations in a child with myelodysplastic syndrome.

Hematological malignancies and premalignant diseases are generally of monoclonal origin. The prognostic and therapeutic significance of finding two genetically independent clones remains to be determined. We followed a case of childhood myelodysplastic syndrome showing biclonal chromosomal abnormalities (+8, -7) by conventional cytogenetic examination and double target fluorescence in situ hybridization (FISH). A 7-year-old girl presented with Plaut-Vincent angina and leukopenia. The cytogenetic aberration of +8 was the first sign to suggest MDS. Serial bone marrow controls, prompted by a progressive clinical course detected myelodysplastic changes and a new clonal aberration (-7). The presence of -7 and +8 in two independent clones was verified by double-target FISH. While at diagnosis and during cytokine treatment more cells showed +8, after successful all-trans retinoic acid (ATRA) therapy, the clone with -7 predominated. Following allogeneic bone marrow transplantation the patient displayed donor-derived hematopoesis. Our data stress the significance of cytogenetic and FISH examinations in detecting specific genetic abnormalities and progressive clonal changes as an indicator and guideline for therapy. Different cell clones characterized by different genetic changes might be associated with different biologic features reflected in their response to treatment.

Angina Pectoris↗
PubMed1998

Supraclavicular abscess caused by Salmonella enteritidis in patient without gastrointestinal symptoms.

Soft tissue infections are among the rare manifestations of extra intestinal salmonellosis and occur more frequently in immunocompromised patients. Herein we report a case of a 51-year-old white male with type II. diabetes mellitus, diagnosed with supraclavicular abscess caused by Salmonella enteritidis. The patient denied any gastrointestinal symptoms, and stool cultures were negative. After incision, drainage, and administration of intravenous ampicillin (4 x 1.5 g/day for two weeks) the patient recovered completely. To our knowledge, this is the first reported case of cervical soft tissue abscess caused by Salmonella enteritidis without preceding gastrointestinal symptoms. We feel that the presence of diabetes was a risk factor for developing the infection.

Abscess↗
PubMed1998

[Epidemiology of non-variceal upper gastrointestinal hemorrhage in Vas County in Western Hungary].

Data of 3014 patients with nonvariceal upper gastrointestinal bleeding were retrospectively evaluated in county Vas between 1984 and 1995. The annual incidence of bleeding increased progressively in this period. In this change the role of nonsteroidal antiinflammatory drugs in questionable, because in the investigated period the sale of these drugs decreased, although the purchase of oxicame, one of this drug-group associating with higher risk for bleeding increased. The risk of bleeding enhanced with ageing that can be explained by higher rate of Helicobacter pylori infection, by the greater consumption of nonsteroidal antiinflammatory drugs and by the more frequent serious concomitant diseases in elderly age. The risk of bleeding in males proved to be higher than in females, the frequency of bleeding in men was twice as much as it could be expected according to the sex rate of the peptic lesions. Their results concerning the reduction of mortality underline the importance of the early endoscopic diagnosis and therapy.

Adult↗
PubMed1998

[Efficiency of electro-hydrothermal coagulation in the management of non-variceal upper gastrointestinal bleeding].

UNLABELLED: The data of 596 electro-hydrothermal coagulations in nonvariceal acute gastrointestinal bleeding were retrospectively evaluated and the results were compared to a previous period in which the endoscopic hemostasis was not regularly applied. Initial hemostasis was achieved in 96.1%, the rebleeding rate was 20.5%, perforation occurred in 0.5%. Comparing the two period the need of urgent surgery decreased from 9.2% to 3.4% (p < 0.05) and the mortality from 25.4% to 12% (p < 0.01). CONCLUSION: The electro-hydro-thermal coagulation is a safe and effective method in the treatment of nonvariceal gastrointestinal bleeding.

Adolescent↗
PubMed1997

[2q21-qter trisomy in hepatoblastoma].

Cytogenetic findings and fluorescens in situ hybridization results of a hepatoblastoma of a boy of two and half years of age are presented. Cytogenetic analysis and fluorescens in situ hybridization technique were performed using tumor tissue obtained by biopsy. The direct culture was harvested after 16 hour colcemid treatment. The results of G-banding were as follows: 47,XY,add(4) (q26),-9,+20. There were considerable variation in the degree of condensation and hence in the number of visible G-bands both between metaphases and between homologous chromosomes in the same metaphases. Fluorescens in situ hybridisation were carried out by whole chromosome painting probes: 2,3,4, and 20. The karyotype of the malignant cells was adjusted accordingly: 47,XY,der(4)(q35),dir ins(9;2)(p22;q?21q?25),+20. The results confirm the most common primary chromosome abnormalities in hepatoblastoma are the following: trisomy 2, trisomy 20 and 4q structural rearrangement. Fluorescens in situ hybridization confirms the importance of trisomy 2q21-qter in hepatoblastoma. Authors recommend the use of fluorescens in situ hybridization to correct any tumor karyotype with difficult or ambiguous chromosome morphology.

Biopsy↗
PubMed1997

Diagnostic and prognostic significance of chromosome abnormalities in childhood acute lymphoblastic leukemia.

Current intensive chemotherapies cure about 70% of the children with ALL. On the other hand a significant number of the children are not cured despite intensive treatment. At the same time some highly curable patients are treated too intensively and suffer from unnecessary side effects of the chemo- and radiotherapy applied. In order to further improve the therapeutic results in this disease, we have to distinguish between the cases with a better and a worse prognosis. The initial karyotype (both numerical and structural chromosome abnormalities) proved to be one of the most reliable prognostic parameters, leading to the suggestion of developing genotype-specific therapies. Although the prognosis in patients with pseudodiploid karyotype is usually unfavorable, a significantly better prognosis can be observed in those with more than 50 chromosomes. Because the latter patients can achieve remission on a metabolite-based therapy, the toxic effects of more aggressive chemotherapy with anthracyclines and genotoxic agents can be avoided; thus, the reliable and accurate identification of patients with > 50 chromosomes is of particular importance. For this purpose three methods: chromosome analysis, DNA flow cytometry, and fluorescence in situ hybridization can be used. In 1993 it was decided to develop a comprehensive nationwide project in order to perform the initial genetic analysis of all ALL children diagnosed in the hematological/oncological centers of Hungary. Here the data obtained on 187 ALL patients diagnosed in the period from 1993 to 1995 are presented. In about 75% of patients (in 140 of 187) chromosome analysis was performed, in 78 cases (55.7%) successfully. The proportion of patients with abnormal karyotype was 36 of 78 (46.1%), and hyperdiploidy with more than 50 chromosomes was detected in 13 of 78 (16.6%) children. The lower ratio of hyperdiploid cases in our patients as compared to the data in the literature may be due to technical difficulties and the small number of patients studied, but it may reflect real geographic characteristics. Using flow cytometry, seven of 31 patients investigated (22.5%) proved to be hyperdiploid with a DNA index above 1.16. A higher ratio of hyperdiploid patients in this study calls attention to the significance of simultaneous application of the two methods. Taken together, 16 of 80 (20.0%) successfully studied patients proved to be hyperdiploid (> 50 chromosomes and/or DNA index above 1.16). The pattern of chromosome involvement in our study determined by chromosome analysis and/or FISH technique proved also to be different from the data of large international series. In addition to trisomies of chromosomes 4, 6, 10, 14, 17, 18, 21, and X, which are known to be the most frequently involved chromosomes, trisomies of chromosomes 3, 8, 11, and 13 were also observed with a high frequency. Comparison of survival curves of various cytogenetic subgroups showed a significant difference between diploid-pseudodiploid and diploid-hyperdiploid A (with 47-50 chromosomes) subgroups. No favorable prognosis of hyperdiploid patients (> 50 chromosomes) could be proved. Because of the small number of patients studied, prognostic differences of cytogenetic subgroups need further confirmation. The clinical and genetic differences observed, however, call attention to the necessity for further genetic studies of ALL patients in Hungary, because these differences may reflect real geographic characteristics and may be related to different environmental mutagen/carcinogen effects of the given geographic area. It is essential to determine whether or not these differences really exist and if they do to reveal the causes leading to these differences. In our view this is one of the routes by which the therapeutic results in childhood ALL can be further improved simultaneously with the avoidance of early and late toxicity of chemotherapy.

Child↗
PubMed1995

[Early stage gastric cancer in Vas County].

UNLABELLED: Between 1983 and 1992, 44 patients with early gastric cancer underwent operative treatment. This group comprised 13.3 percent of all patients with gastric cancer operated on during this period. Every patient underwent oesophago-gastro-duodenoscopy before operation. The indication for surgery was histologically confirmed carcinoma in 37 patients, gastric haemorrhage in 2 patients and gastric ulcer unresponsive to medical treatment in 5 patients. The gastric carcinoma was limited to the mucosa in 28 cases and involved the submucosa in 16. Five patients, one with mucosal and four with submucosal early gastric cancer had regional metastatic lymph node involvement. Life-table calculated patient survival rate at 5 years, excluding the perioperative mortality, was 79.4 percent. CONCLUSIONS: 1. The prevalence of early gastric cancer proved to be similar to previously published in West-Europe and United States. 2. If early gastric cancer is limited only to the mucosa, regional metastatic lymph node is relatively rare. 3. Gastrointestinal bleeding can be the first clinical sign of early gastric cancer. 4. Therapy resistant gastric ulcer requires surgery irrespective of the histological examination of the biopsy specimen.

Adult↗
PubMed1993

[Treatment of carcinoid tumor with a long-acting somatostatin analog].

A case of carcinoid tumour of the small intestine has been reported, which caused hormone producing multiple hepatic metastases. The 45 year old man had flush syndrome several times a day, that was caused by a carcinoid tumour originated from the jejunum. After the operation of the primary tumour he was treated by Sandostatin (Sandoz, Basel), which significantly reduced the symptoms and slowed down the progression. The neural elements containing substance P, neuropeptide Y, vasoactive intestinal polypeptide, serotonin, dopamine-beta-hydroxylase and somatostatin, which are thought to cause the symptoms, were investigated in the small intestine and the liver metastasis by an immunocytochemical method. It seems to be an interesting observation, that a large number of neuropeptide Y immunoreactive nerve fibers and some substance P and vasoactive intestinal polypeptide immunoreactive nerve processes and cell bodies were observed however, no trace of somatostatin and serotonin immunoreactive nerve elements could be detected in the hepatic metastasis. That can be a novel addition to the possible aetiology of the carcinoid syndrome and to the way how the somatostatin treatment acts.

Carcinoid Tumor↗
PubMed1991

[Initial experience with ultrasonic examination of the rectum].

The authors report their initial results obtained by the use of transrectal sonography in examination of known or suspected rectal and perirectal masses. 42 patients were examined with commercially available endosonographic probes. 22 patients had known rectal cancer. 13 patients underwent surgical exploration. Malignant infiltration of perirectal fat were detected as accurately with US as with histology in 9 cases. Lymph node involvement was accurately identified in 11 cases. They recommend this new technique for the assessment of invasion of rectal tumours and lymph node involvement, for postoperative follow-up and for examination of benign diseases of the rectum.

Adult↗
PubMed1991

[Ultrasonic diagnosis with secretin stimulation in patients with pancreas divisum].

The diagnostic value of secretin provoked abdominal ultrasound was studied on 34 patients with pancreas divisum and on 20 control subjects. The patients received a 1.0 unit/kg body weight dose of secretin. The degree of ductal expansion and the time required to return to the initial state were registered and these values were compared to the clinical diagnosis. The control subject's ductal diameters prior to secretin administration were 1 mm in all cases (maximum expansion 2 mm, return to the initial value within 10 minutes). The pancreas divisum patients could be placed in two groups based on their initial ductal diameter. Fourteen patients had initial ductal diameters of 2 mm or greater (A group mean +/- SD: 2.4 +/- 0.3) while 21 patients had an initial value of less than 2 m (B group; 1.7 +/- 0.3). Following secretin administration the ductal diameter of the A group's patients increased on average +/- SD to 1.3 +/- 0.5 times the initial value and in the B group 3.2 +/- 1.1 times the initial value (p less than 0.01). In the A group the ductal diameter returned to its initial value within 10 minutes, while it took 35 minutes for the same to occur in the B group. A relationship can be observed between the clinical diagnosis, the initial ductal diameter, the degree of expansion following secretin administration and the time required to return to the initial state.

Adult↗