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Biomedical subjects

A Szilágyi

Publications and source records attributed to A Szilágyi.

At least 19 recordsLinked to original sources
PubMed2006

Clinical and immunoserological characteristics of mixed connective tissue disease associated with pulmonary arterial hypertension.

We investigated the clinical characteristics and immunoserological alterations in patients with mixed connective tissue disease (MCTD) associated with pulmonary arterial hypertension (PAH). Anti-U1RNP autoantibodies, anti-endothelial cell antibodies (AECA) and serum thrombomodulin (TM) as well as von Willebrand factor antigen (vWFAg) concentrations were measured in 25 patients with MCTD associated with PAH and in 154 MCTD patients without PAH. The results showed that the probability of survival was lower in MCTD patients with PAH than in the 154 MCTD-non-PAH patients (5-year survival rate in MCTD with PAH: 73%, versus 96% in MCTD-non-PAH; P < 0.01). AECA were more frequently present in the sera of MCTD patients with PAH than in MCTD-non-PAH (P < 0.001). Serum TM and vWFAg levels were higher in MCTD-PAH patients than in MCTD-non-PAH patients (TM: P < 0.001; vWFAg: P < 0.001). Significant correlation was noticed between the quantity of AECA and TM level (r = 0.466) as well as the quantity of AECA and vWFAg level (r = 0.550). In conclusion, our results suggest that in MCTD the presence of AECA and endothelial cell activation may play a role in the development of PAH and in the maintenance of obliterative vascular processes.

Adult↗
PubMed2005

Two successful pregnancies following eight miscarriages in a patient with antithrombin deficiency.

Inherited thrombophilias are associated with an increased risk of maternal thromboembolism and certain adverse pregnancy outcomes, including second- and third-trimester fetal loss, placental abruption, severe intrauterine growth restriction, and early-onset, severe preeclampsia. Pregnant patients with severe thrombophilias, especially antithrombinopathies are at very high risk for both thromboembolism and adverse pregnancy outcomes. A case of a patient with antithrombin deficiency is reported, who had two successful pregnancies after eight miscarriages. Our case shows that a combined treatment with antithrombin substitution and a prophylactic, body-weight-adjusted dose of low-molecular-weight heparin may be successful in preventing pregnancy loss and thromboembolism in antithrombin deficiency during pregnancy, although other complications, such as preeclampsia and intrauterine growth restriction cannot always be prevented.

Abortion, Habitual↗
PubMed2005

Detection of Nepsilon-monomethyllysine using high-performance liquid chromatography and high-performance liquid chromatography-mass spectrometry.

Nepsilon-Monomethyllysine was identified in the serum, urine, brain, and liver samples of rats treated per os with L-deprenyl. The identification procedure included reaction with Fmoc chloride, clean-up, and analysis using HPLC-UV-MS. Oral administration of (-)-N-14C-methyl-N-propynyl(2-phenyl-1-methyl)ethylammonium hydrochloride L-deprenyl) to rats resulted in transfer of the radiolabelled methyl group to the Nepsilon-amino group of the endogenous lysine. The radiolabelled Nepsilon-monomethyllysine was urinary eliminated together with the other radiolabelled deprenyl metabolites, such as deprenyl-N-oxide and methamphetamine. The presence of Nepsilon-monomethyllysine has also been traced, and its concentrations were compared in the serum, liver and brain of rats subjected to L-deprenyl treatment. Methyl group transfer from the L-deprenyl to endogenous compounds; and the urinary elimination of their products may offer a vital way to eliminate or to decrease the degree of drug transmethylation to the lysine constituents of blood vessels' proteins.

Animals↗
PubMed2005

HPLC and HPLC-MS analysis of urinary N(epsilon)-monomethyl-lysine.

Administration of (14)C-labelled L-deprenyl to rats results in the urinary elimination of a 14C-labelled compound. The 9-fluorenylmethoxycarbonyl chloride-reacted urine sample is fractionated by high-performance liquid chromatography (HPLC) on an octadecyl silica stationary phase. N(epsilon)-Monomethyl-lysine is identified in the fraction containing the majority of the radioactivity. Structural elucidation is carried out using HPLC-mass spectrometry in atmospheric pressure chemical ionization mode. Identification of the 14C-labelled fragment in Ne-monomethyl-lysine is an experimental proof that an N-methylated amino acid is generated by transmethylation from a well-known drug. This type of transmethylation may have basic importance in the positive side effects of certain drugs.

Animals↗
PubMed2005

Synchronous spontaneous perforation of the esophagus and a duodenal ulcer.

SUMMARY: Although 41% of patients with spontaneous rupture of the esophagus also suffer from gastro duodenal ulcer disease, cases of synchronous spontaneous esophageal and duodenal ulcer perforation have thus far not been reported in the literature. We report on the case of a 61-year-old man who presented with a 72-hour history of esophageal rupture and duodenal ulcer perforation. Following appropriate circulatory resuscitation we performed double resection; involving the esophagus, cardia and the distal part of the stomach, followed by substitution by means of gastro-jejunal transposition as a one-stage procedure. With reference to this case with a favorable outcome, we are presenting an analysis of indications for resectional surgery in advanced spontaneous esophageal perforation.

Digestive System Surgical Procedures↗
PubMed2004

Low-dose ovulation induction with urinary gonadotropins or recombinant follicle stimulating hormone in patients with polycystic ovary syndrome.

Patients with polycystic ovary syndrome (PCOS) are highly sensitive to gonadotropins. In recent years a number of publications have shown that chronic low-dose protocols are effective in reducing complications, in particular ovarian hyperstimulation syndrome (OHSS), especially if recombinant human follicle stimulating hormone (rhFSH) is used. The aim of the present study was to compare the efficacy and safety of rhFSH (Gonal-F, Serono) versus urinary human FSH (uhFSH) (Metrodin, Serono) in a low-dose step-up protocol for ovulation induction in clomiphene-resistent infertile PCOS patients. Twenty PCOS patients were recruited in two centers for an open randomized comparative study. A starting dose of a 75-IU ampule of rhFSH or uhFSH was used for 14 days with an increment of 37.5 IU every 7 days. Human chorionic gonadotropin (hCG) (10,000 IU, Profasi, Serono) was administered if one to three follicles achieved a diameter of > or = 16 mm. Sonographic and hormonal (serum estradiol and progesterone) monitoring of the cycles was performed. All the six pregnancies induced were in the rhFSH group, but two of them ended with miscarriage. There were no differences between the two groups concerning the number of ampules used, the stimulation days, the estradiol levels on the day of hCG administration, and the progesterone levels 7 days after hCG administration. Three patients had grade II, and one patient grade III OHSS. In conclusion, our results support the literature data that rhFSH is superior to uhFSH regarding pregnancy rates, not only in in vitro fertilization cycles, but also with a low-dose protocol in patients with PCOS.

Drug Administration Schedule↗
PubMed2003

Endocrine characteristics of polycystic ovary syndrome (PCOS).

Polycystic ovary syndrome (PCOS) is probably the most prevalent endocrinopathy in women and the most common cause of anovulatory infertility. Patients with PCOS have clinical and biochemical features consistent with the ultrasound diagnosis and they are likely to face the problems of hyperandrogenism, subfertility and recurrent miscarriage. The aim of the present review is to summarize our present knowledge on the hormonal background of this very prevalent syndrome and to give some clinical examples how the present knowledge can be applied to treat PCOS patients according to their current problem, such as menstrual cycle disorder, hirsutism, infertility or to prevent late consequences as diabetes mellitus. The etiology and pathogenesis of PCOS is still a matter of controversies, but it is apparent that inappropriate gonadotropin secretion, obesity, hyperinsulinism and insulin resistance are the major determining factors in the development of ovarian hyperandrogenism an chronic anovulation. Reversal of insulin resistance in PCOS constitutes the fundamental goal in the management of hyperandrogenic anovulatory infertility and in the prevention of long-term consequences. The value of the insulin sensitizer metformin therapy awaits further evaluation and it should be integrated in the spectrum of therapeutical options that include the discussed surgical methods and GnRH analogues as well.

Contraceptives, Oral, Hormonal↗
PubMed2002

Occurrence of osteopenia among adolescent girls with oligo/amenorrhea.

The occurrence of reduced bone mineral density (BMD) among adolescent girls with oligomenorrhea or secondary amenorrhea, due to 'pure' dysfunction of the hypothalamo-pituitary-ovarian (HPO) axis (without anorexia nervosa, excessive sport or ballet, slimming diet, etc.) was examined. The study group consisted of 19 adolescent girls (age 16-18 years) with oligo/amenorrhea. Clinical (height, weight, age at menarche, duration of amenorrhea, body mass index (BMI)), hormonal (follicle stimulating hormone (FSH), luteinizing hormone (LH), estradiol, progesterone, testosterone, prolactin), and ion (calcium, sodium, potassium, phosphate, chloride) parameters and the BMD of the lumbar spine were investigated. Correlations between BMD and other parameters were also examined. Twenty healthy volunteers (same age and regular cycles) served as controls. Three girls had osteoporosis, with a BMD below -2 standard deviations (SD). Ten showed osteopenia, with a BMD value between -1 and -2 SD. Only six of the study group had a normal BMD within +/- 1 SD. A positive correlation was observed between the BMD and the BMI (r = 0.73; p < 0.05). All the controls had normal hormonal, ion and BMD parameters. 'Pure' dysfunction of the HPO axis in adolescents, causing oligomenorrhea or secondary amenorrhea, might result in reduced BMD and, consequently, lower peak bone mass. Treatment of menstrual cycle disorders is necessary for the prevention of osteoporosis.

Adult↗
PubMed2001

Kallmann's syndrome: pregnancy through intracytoplasmic sperm injection and complicated by gestational diabetes.

Kallmann's syndrome is characterized by hypogonadotropic hypogonadism and anosmia. Assisted reproductive techniques such as intracytoplasmic sperm injection (ICSI) may be required to treat the infertile couple with oligozoospermia. Rare complications have been described in patients with Kallmann's syndrome, but gestational diabetes has not previously been reported. A case of Kallmann's syndrome with infertility is reported. Ovulation was successfully induced by human menopausal gonadotropin therapy, but pregnancy could not be achieved by artificial insemination or by conventional in vitro fertilization, although the husband had only moderate oligozoospermia. A high fertilization rate of the retrieved oocytes and successful pregnancy was achieved by ICSI. The pregnancy was complicated by gestational diabetes that was managed by insulin therapy. Successful ovulation induction in Kallmann's syndrome is not rare, but ICSI may be needed in selected cases. Some recent data have suggested that diabetes may occur in patients with Kallmann's syndrome, but further investigation is needed to establish whether gestational diabetes is associated with Kallmann's syndrome or is purely coincidental.

Adult↗
PubMed2000

Structural differences between mesophilic, moderately thermophilic and extremely thermophilic protein subunits: results of a comprehensive survey.

BACKGROUND: Proteins from thermophilic organisms usually show high intrinsic thermal stability but have structures that are very similar to their mesophilic homologues. From prevous studies it is difficult to draw general conclusions about the structural features underlying the increased thermal stability of thermophilic proteins. RESULTS: In order to reveal the general evolutionary strategy for changing the heat stability of proteins, a non-redundant data set was compiled comprising all high-quality structures of thermophilic proteins and their mesophilic homologues from the Protein Data Bank. The selection (quality) criteria were met by 64 mesophilic and 29 thermophilic protein subunits, representing 25 protein families. From the atomic coordinates, 13 structural parameters were calculated, compared and evaluated using statistical methods. This study is distinguished from earlier ones by the strict quality control of the structures used and the size of the data set. CONCLUSIONS: Different protein families adapt to higher temperatures by different sets of structural devices. Regarding the structural parameters, the only generally observed rule is an increase in the number of ion pairs with increasing growth temperature. Other parameters show just a trend, whereas the number of hydrogen bonds and the polarity of buried surfaces exhibit no clear-cut tendency to change with growth temperature. Proteins from extreme thermophiles are stabilized in different ways to moderately thermophilic ones. The preferences of these two groups are different with regards to the number of ion pairs, the number of cavities, the polarity of exposed surface and the secondary structural composition.

Amino Acids↗
PubMed2000

Mirror image mutations reveal the significance of an intersubunit ion cluster in the stability of 3-isopropylmalate dehydrogenase.

The comparison of the three-dimensional structures of thermophilic (Thermus thermophilus) and mesophilic (Escherichia coli) 3-isopropylmalate dehydrogenases (IPMDH, EC 1.1.1.85) suggested that the existence of extra ion pairs in the thermophilic enzyme found in the intersubunit region may be an important factor for thermostability. As a test of our assumption, glutamine 200 in the E. coli enzyme was turned into glutamate (Q200E mutant) to mimic the thermophilic enzyme at this site by creating an intersubunit ion pair which can join existing ion clusters. At the same site in the thermophilic enzyme we changed glutamate 190 into glutamine (E190Q), hereby removing the corresponding ion pair. These single amino acid replacements resulted in increased thermostability of the mesophilic and decreased thermostability of the thermophilic enzyme, as measured by spectropolarimetry and differential scanning microcalorimetry.

3-Isopropylmalate Dehydrogenase↗
PubMed2000

Oral manifestations of patients with Turner syndrome.

OBJECTIVE: The purpose of this study was to conduct a complete analysis of orofacial abnormalities of patients with Turner syndrome. STUDY DESIGN: Twenty nine patients with Turner syndrome and 30 without Turner syndrome (control group) were studied. The subjects were screened for cariological and periodontal alterations, orthodontic anomalies, tooth crown morphologic condition, alveolar arch dimensions, and temporomandibular joint function. RESULTS: Significantly lower mean values for decayed, missing, and filled teeth were found for patients with Turner syndrome as compared with the control group. Plaque and gingival indices were significantly higher in the study group. Orthodontic anomalies were more frequent and severe in patients with Turner syndrome. Our investigation showed reduced tooth crown size in the study group. The alveolar arch of the maxilla was narrower and of normal length, but the mandibular arch was shorter and broader. There was no significant alteration in temporomandibular joint function in the 2 examined groups. CONCLUSIONS: Our examination showed the importance of early diagnosis of oral anomalies and timely treatment of dental problems for patients with Turner syndrome.

Adolescent↗
PubMed2000

Hormonal and clinical effects of chronic gonadotropin-releasing hormone agonist treatment in polycystic ovary syndrome.

The aim of the study was to evaluate the hormonal (focusing on the urinary steroid profile) and clinical effects of chronic gonadotropin-releasing hormone (GnRH) agonist treatment in patients with polycystic ovary syndrome (PCOS) suffering from hirsutism. A long-acting GnRH agonist was administered for 6 months in eight PCOS patients. Hormonal effects were measured by determining serum luteinizing hormone (LH), follicle stimulating hormone (FSH), prolactin, testosterone and estradiol concentrations, and by profiling urinary steroids using capillary gas chromatography of 24-hour urine samples. To evaluate 5 alpha-reductase enzyme activity, the ratios of androsterone to etiocholanolone and 5 alpha-tetrahydrocortisol to tetrahydrocortisol were calculated in urine samples. The ratio of androgen to cortisol metabolites was also determined before, and 3 and 6 months after therapy. LH and estradiol levels were suppressed significantly after the first injection and testosterone after the second injection of the GnRH agonist. Thus, serum testosterone was normalized. Ratios of urinary steroids reflecting 5 alpha-reductase enzyme activity (androsterone to etiocholanolone and 5 alpha-tetrahydrocortisol to tetrahydrocortisol) and the ratio of androgen to cortisol metabolites decreased significantly after 3 months of treatment. Degree of hirsutism, assessed by Ferriman-Gallwey score, diminished after 6 months, but not significantly. In conclusion, our data show that long-acting GnRH agonist treatment of PCOS patients is effective in reducing serum and urinary androgen levels, but it is not accompanied by an effective reduction in hirsutism during a 6-month treatment period. A longer or a combined treatment would be needed to achieve significant improvement in hirsutism. Gas chromatographic profiling of urinary steroids and the use of specific ratios of the excreted metabolites seems to be a sensitive tool both in the diagnosis of PCOS and in monitoring ovarian suppression.

Adult↗
PubMed2000

[Morphologic alterations of tooth crown in patients with Turner syndrome and its association with orthodontic anomalies].

In females with numerical and/or structural aberration of the X chromosome as in Turner's syndrome, somatic development deviates from the normal pattern. The aim of the present study was to investigate the tooth crown dimensions, and report the size of the alveolar arch of the maxilla and the mandible of patients with Turner's syndrome. The study population consisted of 29 girls with Turner's syndrome and 30 healthy female controls. All mesiodistal dimensions were significantly smaller in Turner's syndrome compared with those of normal controls. The labiolingual dimensions were smaller in the patient group with the exception of the canines and the lower central incisors. The alveolar arch of the maxilla was narrower and of normal length, but the mandibular arch was shorter and broader. Orthodontic anomalies were found more frequent and serious of patients with Turner's syndrome. Our examination showed the importance of early diagnosis of oral anomalies as well as dental treatment of patients with Turner's syndrome.

Adolescent↗
PubMed2000

[Correlations between the prevalence of caries and the microbiologic parameters of saliva in patients with Turner syndrome].

The aim of our study was to investigate the associations between caries prevalence and microbiological composition and flow rate of saliva in patients with Turner's syndrome. The examined population consisted of 29 patients with Turner's syndrome and 30 healthy control girls. DMF-T means, stimulated salivary flow, Streptococcus mutans and Lactobacillus counts in saliva were determined. Stimulated salivary flow was significantly higher in the patients group. Caries prevalence was found significantly lower in patients with Turner's syndrome, which finding may have been caused by the significantly lower counts of salivary Streptococcus mutans and Lactobacillus. Statistically significant correlation was found between DMF-T mean values and salivary microbiological counts.

Adolescent↗
PubMed2000

[Cephalometric analysis in patients with Turner syndrome].

The aim of the study is to assess the cephalometric values of patients with Turner syndrome and to compare them to those of healthy patients in order to determine the severity of orthodontic anomalies and the possibilities of treatment. For our work the modified Ricketts analysis was used. Significant differences were found between the two groups regarding the B1, B2, P, AB ii angles and in relations of C:R. Early stomatologic treatment seems to be very important in this patient's group to prevent dental, periodontal and temporomandibular disorders.

Adult↗