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Biomedical subjects

Ferenc Nagy

Publications and source records attributed to Ferenc Nagy.

At least 19 recordsLinked to original sources
PubMed2007

A case of interstitial pneumonitis in a patient with ulcerative colitis treated with azathioprine.

The early hypersensitivity reaction and late bone marrow depression are well-known side-effects of azathioprine, whereas interstitial pneumonia is a rare complication. A 40-year old male patient had been treated with azathioprine in consequence of extensive ulcerative colitis for 10 years. He then complained of 7 d of fever, cough and catarrhal signs, without symptoms of active colitis. Opportunistic infections were ruled out. The chest X-ray, CT and lung biopsy demonstrated the presence of interstitial inflammation. Azathioprine therapy was discontinued as a potential source of the pulmonary infiltrate. In response to steroid therapy, and intensive care, the pulmonary infiltrate gradually decreased within 4 wk. Three months later, his ulcerative colitis relapsed, and ileo-anal pouch surgery was performed. In cases of atypical pneumonia, without a proven infection, azathioprine-associated interstitial pneumonitis may be present, which heals after withdrawal of the drug.

Adult↗
PubMed2006

[Amyotrophic lateral sclerosis in Baranya county, Hungary].

Amyotrophic lateral sclerosis is a progressive neurodegenerative disease resulting in loss of upper and lower motoneurons. Here we analyzed the clinical and epidemiological data of 202 ALS patients admitted to our department between 1950 and 2004. Risk and protective factors influencing onset and progression of the disease were examined. We also analyzed the relationship between first clinical symptoms/signs and prognosis of the disease. Our data suggest the importance of toxic factors in the initiation of the disease and the effect of clinical phenotype on the progression of ALS.

Adult↗
PubMed2006

[The role of tremorometry in predicting the outcome of ablative surgeries].

Ablative neurosurgical interventions are widely used for the treatment of advanced Parkinson's disease. However, in some cases, the achieved result is temporary and repeat operation is necessary to obtain a permanent effect. By analyzing 30 ablative surgeries using comparative accelerometry, we looked for a biological marker predicting the efficiency. In 27 cases where clinical symptoms were permanently improved, a significant increase in rest tremor frequency was observed in addition to reduction in tremor intensity. In contrast, in those three cases where the clinical effect of the surgery was only temporary, the frequency of tremor remained unchanged despite of the transitory decrease in intensity. We thus hypothesize that postoperative change in frequency of tremor but not the intensity may predict the outcome of ablative treatments.

Follow-Up Studies↗
PubMed2006

Experimental validation of a predicted feedback loop in the multi-oscillator clock of Arabidopsis thaliana.

Our computational model of the circadian clock comprised the feedback loop between LATE ELONGATED HYPOCOTYL (LHY), CIRCADIAN CLOCK ASSOCIATED 1 (CCA1) and TIMING OF CAB EXPRESSION 1 (TOC1), and a predicted, interlocking feedback loop involving TOC1 and a hypothetical component Y. Experiments based on model predictions suggested GIGANTEA (GI) as a candidate for Y. We now extend the model to include a recently demonstrated feedback loop between the TOC1 homologues PSEUDO-RESPONSE REGULATOR 7 (PRR7), PRR9 and LHY and CCA1. This three-loop network explains the rhythmic phenotype of toc1 mutant alleles. Model predictions fit closely to new data on the gi;lhy;cca1 mutant, which confirm that GI is a major contributor to Y function. Analysis of the three-loop network suggests that the plant clock consists of morning and evening oscillators, coupled intracellularly, which may be analogous to coupled, morning and evening clock cells in Drosophila and the mouse.

Arabidopsis↗
PubMed2006

[Myopathy associated with respiratory insufficiency: diagnostic difficulties in adult-onset Pompe disease].

INTRODUCTION: Adult-onset acid maltase deficiency myopathy is a rare lysosomal storage disease with an autosomal recessive pattern of inheritance. The disease can be manifested with respiratory insufficiency and fatigue. METHODS: A case of a 45-year-old male patient is presented, and difficulty in diagnosis is discussed. RESULTS: The patient had been repeatedly examined because of hypersomnia, dyspnea and fatigue for a full year before a neurological consultation was requested. Artificial ventilation resulted in a dramatic improvement of his symptoms. Neurological examination revealed myopathy. Electrophysiological myotonia and glycogen storage in muscle biopsy specimen suggested acid maltase deficiency. The diagnosis was established by genetic testing detecting the previously described homozygous c.-45T > G mutation in the alpha-glucosidase gene. DISCUSSION: Rare hereditary neurological diseases can be also suspected as cause of chronic unexplained respiratory insufficiency resulted in hypersomnia and fatigue due to hypercapnia and myopathy. A proper diagnosis can contribute to early diagnosis and introduction of enzyme replacement therapy may reduce or stop clinical progression. Genetic diagnosis can also provide a possibility for prenatal testing.

Age of Onset↗
PubMed2006

CONSTITUTIVELY PHOTOMORPHOGENIC1 is required for the UV-B response in Arabidopsis.

CONSTITUTIVELY PHOTOMORPHOGENIC1 (COP1) is a negative regulator of photomorphogenesis in Arabidopsis thaliana. COP1 functions as an E3 ubiquitin ligase, targeting select proteins for proteasomal degradation in plants as well as in mammals. Among its substrates is the basic domain/leucine zipper (bZIP) transcription factor ELONGATED HYPOCOTYL5 (HY5), one of the key regulators of photomorphogenesis under all light qualities, including UV-B responses required for tolerance to this environmental threat. Here, we report that, in contrast with the situation in visible light, COP1 is a critical positive regulator of responses to low levels of UV-B. We show that in the cop1-4 mutant, flavonoid accumulation and genome-wide expression changes in response to UV-B are blocked to a large extent. COP1 is required for HY5 gene activation, and both COP1 and HY5 proteins accumulate in the nucleus under supplementary UV-B. SUPPRESSOR OF PHYTOCHROME A-105 family proteins (SPA1 to SPA4) that are required for COP1 function in dark and visible light are not essential in the response to UV-B. We conclude that COP1 performs a specific and novel role in the plants' photomorphogenic response to UV-B, coordinating HY5-dependent and -independent pathways, which eventually results in UV-B tolerance.

Arabidopsis↗
PubMed2006

[Complex eye movement disturbance in thalamic and mesencephalic infarcts].

A 36 year-old male patient developed sudden double vision and gait imbalance. Neurological examination revealed gaze paresis upward and on the left side downward (vertical "one-and-a-half"-syndrome), horizontal gaze nystagmus on the left bulbus directed to left. The MRI revealed bilateral thalamic and left midbrain ischemic lesions. The brainstem auditory and visual evoked responses were normally configured. Optokinetic nystagmus test found rightward, upward and downward hypometric saccades, convergence-retraction nystagmus--which was not visible at physical neurological examination--saccadic smooth pursuit eye movement and pseudoabducent palsy on both sides. The complex gaze disturbance was attributed to the lesions in the intralaminar nuclei of the thalamus and in the pretectal and rostromedial tegmentum of the mesencephalon. Infarcts may have been due to a variant artery: i.e. the thalamoperforant and the superior paramedian mesencephalic arteries originate with common branch from one of the communicant basilar artery. The authors discuss the mechanism of complex gaze palsy and call attention to the diagnostic value of optokinetic nystagmus examination.

Adult↗
PubMed2006

Diurnal regulation of the brassinosteroid-biosynthetic CPD gene in Arabidopsis.

Plant steroid hormones, brassinosteroids (BRs), are essential for normal photomorphogenesis. However, the mechanism by which light controls physiological functions via BRs is not well understood. Using transgenic plants carrying promoter-luciferase reporter gene fusions, we show that in Arabidopsis (Arabidopsis thaliana) the BR-biosynthetic CPD and CYP85A2 genes are under diurnal regulation. The complex diurnal expression profile of CPD is determined by dual, light-dependent, and circadian control. The severely decreased expression level of CPD in phytochrome-deficient background and the red light-specific induction in wild-type plants suggest that light regulation of CPD is primarily mediated by phytochrome signaling. The diurnal rhythmicity of CPD expression is maintained in brassinosteroid insensitive 1 transgenic seedlings, indicating that its transcriptional control is independent of hormonal feedback regulation. Diurnal changes in the expression of CPD and CYP85A2 are accompanied by changes of the endogenous BR content during the day, leading to brassinolide accumulation at the middle of the light phase. We also show that CPD expression is repressed in extended darkness in a BR feedback-dependent manner. In the dark the level of the bioactive hormone did not increase; therefore, our data strongly suggest that light also influences the sensitivity of plants to BRs.

Arabidopsis↗
PubMed2006

DLG5 variants in inflammatory bowel disease.

OBJECTIVES: Genetic variants within DLG5 were recently reported to be associated with inflammatory bowel disease (IBD). The aim of our study was to test for allelic and haplotype associations of six DLG5 variants in 668 IBD patients from two European populations. Furthermore, we evaluated whether DLG5 variants alter gastrointestinal permeability in Crohn's disease (CD). METHODS: Six DLG5 variants (p.R30Q, p.P1371Q, p.G1066G, rs2289308, DLG_e26, p.D1507D) were genotyped in two study populations: (1) German IBD patients (CD n = 250; ulcerative colitis (UC) n = 150) and German healthy controls (n = 422); (2) Hungarian IBD patients (CD n = 144; UC n = 124) and Hungarian healthy controls (n = 205). Subtyping analysis was performed in respect of CARD15 mutations and clinical characteristics. We also tested for differences within DLG5 genotypes in German CD patients with respect to gastroduodenal and intestinal permeability measured by triple-sugar-test. RESULTS: Allele as well as genotype frequencies of DLG5 variants did not differ between IBD patients and controls in either study population. Indeed, the p.R30Q polymorphism was found more frequently in controls than in patients. The distribution of DLG5 genotypes in German and Hungarian CD patients with CARD15 mutations was not different from patients without mutated CARD15. We did also not observe any association between DLG5 variants and clinical parameters. Importantly, DLG5 variants were not associated with gastroduodenal or intestinal permeability. CONCLUSIONS: We could not replicate that DLG5 is a relevant disease susceptibility gene for IBD in German or Hungarian subjects. In addition, we have no evidence that DLG5 variants are involved in altered gastrointestinal permeability in CD.

Adult↗
PubMed2006

[Azathioprine-associated interstitial pneumonitis].

Azathioprine-associated interstitial pneumonitis. The early hypersensitivity reaction and the late bone marrow depression are well known side effects of the azathioprine; the interstitial pneumonia is a rare complication. A 40-year old male patient was treated with azathioprine due to extensive ulcerative colitis for ten years. He complained seven days of fever, cough and catarrhal signs, without the symptoms of active colitis. The opportunistic infections were ruled out. Chest X-ray, CT and lung biopsy proved the presence of interstitial inflammation. The azathioprine therapy was discontinued as the potential source of the pulmonary infiltrate. As a result of steroid therapy, as well as emergency unit care, the pulmonary infiltrates decreased gradually. Three months later his ulcerative colitis relapsed, for this an ileo-anal pouch surgery was done. In case of atypical pneumonia, without proven opportunistic infection, azathioprine-associated interstitial pneumonitis may be present, which heal after cessation of the drug.

Adult↗
PubMed2006

Forward genetic analysis of the circadian clock separates the multiple functions of ZEITLUPE.

The circadian system of Arabidopsis (Arabidopsis thaliana) includes feedback loops of gene regulation that generate 24-h oscillations. Components of these loops remain to be identified; none of the known components is completely understood, including ZEITLUPE (ZTL), a gene implicated in regulated protein degradation. ztl mutations affect both circadian and developmental responses to red light, possibly through ZTL interaction with PHYTOCHROME B (PHYB). We conducted a large-scale genetic screen that identified additional clock-affecting loci. Other mutants recovered include 11 new ztl alleles encompassing mutations in each of the ZTL protein domains. Each mutation lengthened the circadian period, even in dark-grown seedlings entrained to temperature cycles. A mutation of the LIGHT, OXYGEN, VOLTAGE (LOV)/Period-ARNT-Sim (PAS) domain was unique in retaining wild-type responses to red light both for the circadian period and for control of hypocotyl elongation. This uncoupling of ztl phenotypes indicates that interactions of ZTL protein with multiple factors must be disrupted to generate the full ztl mutant phenotype. Protein interaction assays showed that the ztl mutant phenotypes were not fully explained by impaired interactions with previously described partner proteins Arabidopsis S-phase kinase-related protein 1, TIMING OF CAB EXPRESSION 1, and PHYB. Interaction with PHYB was unaffected by mutation of any ZTL domain. Mutation of the kelch repeat domain affected protein binding at both the LOV/PAS and the F-box domains, indicating that interaction among ZTL domains leads to the strong phenotypes of kelch mutations. Forward genetics continues to provide insight regarding both known and newly discovered components of the circadian system, although current approaches have saturated mutations at some loci.

Alleles↗
PubMed2006

Implanted deep brain stimulator and 1.0-Tesla magnetic resonance imaging.

There is a great need for MRI examinations of patients who have previously undergone deep brain stimulator (DBS) implantation. The current guidelines pertain only to a 1.5-Tesla horizontal-bore scanner complying with strict safety regulations. Moreover, almost all published in vitro and in vivo studies concerning patient safety are carried out on 1.5 Tesla MR scanners. The aim of our work is to share our clinical experience of 1.0-Tesla brain MR imaging. During the past four years, 34 patients with different types of implanted DBS systems underwent 1.0-Tesla MR examinations to answer diagnostic or clinical questions. Apart from the scanner type applied, all other safety instructions were strictly followed. The MRI itself made no significant difference to the measured impedances or the stimulation parameters required to achieve the optimal therapeutic results. From theoretical considerations, it may be assumed that 1.0-Tesla MRI can be performed safely on DBS-implanted patients, provided that all other recommendations are adhered to.

Artifacts↗
PubMed2006

Uniform qualitative electrophysiological changes in postoperative rest tremor.

Ablation and deep brain stimulation (DBS) can treat pharmacologically uncontrollable tremor. Here, we compared the postoperative electrophysiological changes in resting hand tremor after 32 ablations and 12 DBS implantations in patients with severe tremor-dominant idiopathic Parkinson's disease (PD) and essential tremor (ET). Short- and long-term accelerometric data were acquired after surgery and were compared to the preoperative tremor. After effective surgical treatments, significant rest tremor reduction and increase in both frequency and approximate entropy (ApEn) were detected in all PD cases, irrespective of the type and target of intervention. However, the long-term effect of DBS implantation on tremor reduction was significantly better compared to that after ablative treatments. In cases of thalamotomy, the postoperative increase in frequency and ApEn was significantly larger in essential tremor compared to PD, suggesting that the etiology of tremor may influence the size of the similar changes. However, cases where clinical tremor re-emerged 6 to 12 months after the surgery, no change in frequency and ApEn was detected on the second postoperative day, despite an initial tremor reduction and clinical improvement similar to the effective operations. Our results suggest that uniform postoperative changes in rest tremor and the increase in frequency and ApEn could be due to attenuation of pathological oscillators and might be immediate indicators of the effectiveness of neurosurgical treatments relieving tremor.

Cautery↗
PubMed2006

Neurosurgical treatment of tremor in mitochondrial encephalopathy.

A 53-year-old woman underwent several ischemic stroke-like episodes and later developed incomplete, bilateral ophthalmoplegia, left vision deterioration, and bilateral tremor. The clinical course, laboratory data, and muscle histology led to a diagnosis of mitochondrial encephalomyopathy. No other etiology could be identified in the background of her disabling bilateral postural-kinetic tremor. As this tremor did not respond to pharmacological therapy, left thalamotomy and subsequently right thalamic deep brain stimulator (DBS) implantation were performed, which resulted in an excellent clinical outcome. The Fahn-Tolosa-Marin Tremor Rating Scale improved from 110 to 11 points. This case suggests that the rare tremor caused by mitochondrial encephalopathy may be treated long-term with either thalamotomy or thalamic DBS implantation.

Electric Stimulation Therapy↗
PubMed2006

Multiple phytohormones influence distinct parameters of the plant circadian clock.

Circadian systems coordinate endogenous events with external signals. In mammals, hormone-clock feedbacks are a well-known integration system. Here, we investigated phytohormone effects on plant-circadian rhythms via the promoter:luciferase system. We report that many hormones control specific features of the plant-circadian system, and do so in distinct ways. In particular, cytokinins delay circadian phase, auxins regulate circadian amplitude and clock precision, and brassinosteroid and abscisic acid modulate circadian periodicity. We confirmed the pharmacology in hormone synthesis and perception mutants, as rhythmic expression is predictably altered in an array of hormone-related mutants. We genetically dissected one mechanism that integrates hormone signals into the clock, and showed that the hormone-activated ARABIDOPSIS RESPONSE REGULATOR 4 and the photoreceptor phytochrome B are elements in the input of the cytokinin signal to circadian phase. Furthermore, molecular-expression targets of this signal were found. Collectively, we found that plants have multiple input/output feedbacks, implying that many hormones can function on the circadian system to adjust the clock to external signals to properly maintain the clock system.

Arabidopsis↗
PubMed2006

Endogenous testosterone concentration, mental rotation, and size of the corpus callosum in a sample of young Hungarian women.

In the present study brain laterality, hemispheric communication, and mental rotation performance were examined. A sample of 33 women were tested for a possible linear relationship of testosterone level and mental rotation with structural background of the brain. Subjects with a smaller splenial area of corpus callosum tended to have lower levels of testosterone (r =.37, p<.05). However, there were no significant differences in mean scores of mental rotation of object and hand between groups with high and low levels of testosterone. There was a significant difference in relative size of the 6th area (slice) of the corpus callosum between groups with good and poor scores on mental rotation of an object and also in relative size of the 4th and 5th slices of the corpus callosum between groups on mental rotation of the hand. The good and poor scorers' show different relations with the measures of the corpus. The mental rotation of hand was associated with the parietal areas of the corpus callosum, while the mental rotation of object was associated only with the occipital area. These observations suggest that higher testosterone levels may be associated with a larger splenial area, which represents an important connection between the parieto-occipitocortical areas involved in activation of mental images. Further srudy is encouraged.

Adult↗