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Biomedical subjects

C Papp

Publications and source records attributed to C Papp.

At least 55 records · Page 3Linked to original sources

[Prenatal diagnosis of hemophilia B].

The authors are reporting on the prenatal diagnosis of the X-linked haemophilia B for the first time in Hungary applying the polymerase chain reaction. DNA sequence containing a HhaI restriction endonuclease site close to the factor IX gene was amplified using polymerase chain reaction. The products from polymerase chain reaction were detected on polyacrylamide gel with ethidium bromide staining after the digestion with HhaI restriction enzyme. In the first step of the diagnosis DNA specimen was prepared from chorion derived from a 11th week gestation of haemophilia B carrier mother. The investigation of fetal DNA proved a male fetus. The detection of HhaI polymorphism of the fetus demonstrated the inheritance of the disease causing allele. The parents asked for the termination of pregnancy based on the result.

Abortion, Induced↗

Ivemark syndrome with asplenia in siblings.

We describe two siblings with Ivemark syndrome. In both cases, absent spleen, symmetric liver, and lungs with three lobes were associated with complex cardiac malformation. The syndrome was diagnosed prenatally in the second case by fetal echocardiography at the twentieth week of pregnancy. The autosomal recessive mode of inheritance of Ivemark syndrome is further supported by these cases.

Abnormalities, Multiple↗

Definite surgical treatment of complicated recurrent pilonidal disease with a modified fasciocutaneous V-Y advancement flap.

BACKGROUND: Different methods for managing pilonidal disease have been described in the literature. Recurrence impairs the success of all forms of therapy, but the lowest rates have been reported for reconstructions involving local flaps. Nevertheless, treatment of pilonidal disease with a fasciocutaneous V-Y flap is not a well-established procedure. We have modified the surgical technique and used it selectively for complicated recurrent cases. Our experience with this method is analyzed, and its validity is evaluated. METHODS: Twenty-four patients with recurrent pilonidal sinus undergoing radical excision and reconstruction with our modified fasciocutaneous V-Y advancement flap between 1986 and 1993 were studied retrospectively. RESULTS: Except for two minor transient wound dehiscences, in all cases primary healing was achieved. Furthermore, an excellent functional result and acceptable scar pattern were obtained in all twenty-four patients, with a mean follow-up of 4.5 years. Neither evidence of recurrence nor impairment of daily life activity was noted. CONCLUSIONS: Treatment of complicated recurrent pilonidal sinus with the described technique offers a safe but demanding method for definite reconstruction, and we support a more common application.

Adolescent↗

Biological mechanisms underlying the clinical effects of RU 486: modulation of cultured endometrial stromal cell stromelysin-1 and prolactin expression.

During in vitro decidualization of human endometrial stromal cells (HESCs), medroxyprogesterone acetate (MPA) inhibits expression of the potent extracellular matrix (ECM)-degrading protease stromelysin-1 (MMP-3), but enhances PRL expression. Consistent with its priming role in vivo, estradiol (E2) augments these effects. In the current study, immunoblot analysis revealed that coincubation with 10(-6) M RU 486 blocked the inhibition in HESC-secreted MMP-3 levels (50,000 mol wt) evoked by 10(-8) M E2 + 10(-7) M MPA. Although MPA can act as a glucocorticoid, the HESCs were refractory to 10(-7) M dexamethasone added alone or with E2. Because E2 elevates progesterone but not glucocorticoid receptor levels, MPA and RU 486 control MMP-3 expression as a progestin and antiprogestin, respectively. To study RU 486 involvement in steroid withdrawal leading to menstruation, HESCs were decidualized during 10 days incubation with E2 + MPA, and parallel cultures were kept in E2 + MPA or withdrawn to either control or RU 486-containing medium. Compared with E2 + MPA-suppressed HESCs, increases in levels of secreted MMP-3 (2.0-fold), and its 2.1-kilobase messenger RNA (10-fold) were observed in HESCs after 4 days of withdrawal to control medium, with much greater increases seen in RU 486-containing medium (10-fold protein, 100-fold messenger RNA). Previously, we showed that RU 486 up-regulated E2 + MPA-inhibited plasminogen activator expression in the cultured HESCs. Extrapolation of these in vitro observations to endometrial events following RU 486 administration suggests that coordinate enhancement of MMP-3 and plasminogen activator expression promotes proteolysis of the stromal/decidual ECM, which leads to endometrial sloughing. Moreover, destabilization of endometrial microvessels resulting from degradation of their surrounding ECM is consistent with the heavy menstrual bleeding stemming from RU 486 administration. However, in contrast to the marked RU 486-initiated reversal of MMP-3 expression, RU 486 did not significantly reverse E2 + MPA-enhanced PRL secretion by the cultured HESCs. Interestingly, decidual PRL, unlike decidual MMP-3, does not appear to play a role in menstruation. Interleukin-1 beta counteracted E2 + MPA-mediated inhibition of secreted MMP-3 levels, implying that leukocyte/trophoblast-derived cytokines can modulate steroid-regulated MMP-3 expression by stromal/decidual cells during menstruation and pregnancy.

Cells, Cultured↗

Reconstruction of the perineum after tumor surgery.

Major defects of the perineum are often complicated by contracture, radiation injuries, fistulas, and infection. In addition, the perineal surface is unique, and any replacement must meet the requirements of mobility, sensitivity, durability, elasticity, and weight bearing. This combination of complex defects and special surface requirements can be met by adequate extirpative procedures and reconstruction with a number of muscle, musculocutaneous, and fasciocutaneous flap options.

Female↗

[A case of detection of pulmonary sequestration during the early stage of pregnancy and subsequent successful therapy].

As result of routine ultrasound screening during the pregnancy, the number of congenital abnormalities causes severe perinatal respiratory or circulatory failure detected antenatally is rising. The severity of the extralobar pulmonary sequestration is variable. The intrauterine diagnosis and the proper prognosis is difficult because of the rarity of this abnormality. The Color-Doppler ultrasound method has a great importance in the diagnosis. With our case report we would like to help in the better understanding of this rare abnormality.

Adult↗

[Plastic surgery treatment of extensive decubitus ulcers of the pelvic area].

Between 1980 and 1995, 207 patients with 219 extensive pelvic pressure sores underwent reconstructive surgery at our department. Eighty patients operated upon between 1992 and 1995 were followed up. The postoperative complication rate was 22% and the relapse rate 27%. Pre- and postoperative management has been standardized for the purposes of quality control. The value of preoperative management, choice of flap, operative procedure and postoperative care are discussed in this paper.

Adolescent↗

Correlation between karyotype and ultrasound findings in patients with failed early pregnancy.

The aim of this retrospetive study was to identify any consistent patterns between ultrasound findings in failed pregnancies and either normal or abnormal karyotypes. The study involved 102 women in whom the diagnosis of early pregnancy failure was made sonographically. The criteria for a failed pregnancy were: a gestational sac with a mean diameter of > 12 mm without a yolk sac; a yolk sac of > 6 mm mean diameter with or without abnormal morphology that ultimately failed to develop an embryonic structure; and an embryo with a crown--rump length (CRL) of > 5 mm without cardiac activity, or the loss of previously identified cardiac activity. All patients underwent elective dilatation and curettage (D & C) and products of conception were sent for karyotyping. Forty-four pregnancies (43%) had abnormal karyotypes. Of these, 33 (75%) were trisomies. The other 11 included four triploidies, one tetraploidy, two with monosomy X, and four others (unbalanced complement, isochromosome, terminal deletion and translocation). Fifty-eight pregnancies (57%) had normal karyotypes, of which 52 were 46,XX and six were 46,XY. The furthest sonographic anatomic landmark achieved did not differ with respect to karyotypic findings. An abnormal yolk sac was found in 10/58 cases (17.2%) with normal karyotypes and 8/44 cases (18.2%) with abnormal karyotypes. There were eight cases of trisomy 16, of which only two manifested an embryonic structure, but neither of which had cardiac activity; the largest was 4 mm. There were four cases of trisomy 22, of which three developed embryos with a CRL of > 10 mm with cardiac activity (11, 11 and 18 mm, respectively). In three cases of mosaicism, embryos developed cardiac activity, and were 9, 19 and 16 mm. Two cases of monosomy X had embryos of 14 and 24 mm. Only one out of five cases with multiple trisomies developed to a point at which any embryonic structure was identifiable on ultrasound examination. The ultrasound appearance of early pregnancy failure in terms of furthest anatomic landmark reached was not significantly different in cases with normal or abnormal karyotype. An abnormally enlarged yolk sac, presumably secondary to hydropic change, is a non-specific finding of failed pregnancy, and did not correlate with karyotypic abnormality (trisomy 22, mosaic, monosomy X) seem to develop further prior to embryonic demise than those with certain others (trisomy 16, multiple trisomies and unusual other variants.

Abortion, Spontaneous↗

[Screening for congenital anomalies in mid-term pregnancy. Prospective epidemiologic study. Fetal trisomy].

The authors have presented their experiences on prenatal screening of fetal trisomies in this second part of a prospective study between 1988 and 1990. They gained their conclusions by processing 63,496 pregnancies during three years. The results show that maternal age plays the most important role in the prenatal screening of fetal trisomies in Hungary. They recommend fetal karyotyping for every pregnant woman aged 35 years or more. They emphasized that using of a combined screening method (i.e. maternal age, serum alpha-fetoprotein, human chorionic gonadotropin, oestriol) is only permissible if the hormonal and cytogenetic laboratory background are provided under standard circumstances. Since these are not available for the vast majority of pregnant women in Hungary they concluded that, at least for the time being, the main criteria for prenatal screening of fetal trisomies is the maternal age. By applying this recommendation 25-30 percentage of Down syndrome fetuses can be detected.

Adult↗

Impact of prenatal mid-trimester screening on the prevalence of fetal structural anomalies: a prospective epidemiological study.

The objective of this study was to evaluate the effectiveness of the measurement of maternal serum alpha-fetoprotein (MSAFP) at 16 weeks and a subsequent routine ultrasound screening at 18-20 weeks' gestation and the impact on the birth prevalence of congenital structural anomalies in an unselected pregnant population of Hungary in a prospective epidemiological study. A total of 63,794 pregnant women (representing one-sixth of the population of Hungary) were offered this screening program over 3 years (1988-90). Of the pregnant population, 75.7% (48,312) received MSAFP screening and in 81.0% (51,675), at least one ultrasound scan was performed. In the screened pregnancies, 496 craniospinal, thoraco-abdominal, urogenital and other severe major anomalies occurred; 317 were detected at 18-20 weeks (sensitivity 63.1%; specificity 100.0%; positive predictive value 100.0%). The sensitivity of ultrasound scanning was significantly higher (p < 0.05) than that of the MSAFP screening. (At the time of ultrasound scanning the MSAFP value was known.) In this study, the less serious anomalies such as hydrocele, hypospadias and undescended testicle were not systematically searched for, but the birth prevalences were calculated. The overall mid-trimester prevalence of severe plus less severe major anomalies was 2.26%. The birth prevalences of severe major anomalies were 0.57 (craniospinal), 4.36 (thoracoabdominal and urogenital) and 1.21 (other severe) per 1000. These values were lower than the mid-trimester prevalences which were 2.94, 5.20 and 2.06 per 1000, respectively. The prevalence values at the age of 1 year were also calculated (0.36, 2.21, 0.54 per 1000, respectively). We conclude that our screening program with availability of termination of pregnancy could significantly (p < 0.05) reduce the prevalence of severe major abnormalities at birth. Training programs in cardiac scanning are required.

Abnormalities, Multiple↗

Biological mechanisms underlying the clinical effects of RU 486: modulation of cultured endometrial stromal cell plasminogen activator and plasminogen activator inhibitor expression.

The abortifacient and menstrual effects of the potent antiprogestin, RU 486, are associated with both endometrial hemorrhage and extracellular matrix (ECM) degradation. Such processes reflect reduced perivascular decidual cell hemostatic and increased ECM-degrading protease activity. Therefore, we assessed the effects of RU 486 administration on the expression of immunoreactive (ir) endometrial stromal cell urokinase-type (uPA) and tissue-type (tPA) plasminogen activator and their activities as well as levels of ir type 1 plasminogen activator inhibitor (PAI-1) using a well characterized in vitro model of decidualization. Thus, confluent stromal cell cultures were exposed to vehicle control, 10(-8) mol/L estradiol (E2), 10(-7)-10(-8) mol/L medroxyprogesterone acetate (MPA), E2 plus MPA, or 10(-6)-10(-7) mol/L RU 486 alone or in combination with MPA or E2 plus MPA for 3-4 days. Compared to the vehicle control, E2 and RU 486 used alone had no effect on levels of ir PAI-1, uPA, or tPA or on PA activity in the conditioned medium. In contrast, MPA and E2 plus MPA decreased ir uPA and tPA levels and their corresponding activities, whereas MPA increased, and E2 plus MPA further increased ir PAI-1 release. These effects of progestin were blocked by a log higher concentration of RU 486. Similar results were obtained for steady state PAI-1 messenger ribonucleic acid levels. To determine if RU 486 reversed progestin-inhibited stromal cell uPA and tPA release and progestin-enhanced PAI-1 expression, confluent cultures were exposed to 10(-8) mol/L E2 plus 10(-7) mol/L MPA for 10 days, washed, and reexposed to E2 plus MPA, steroid-free medium, or RU 486 for 3-5 or 9-11 days. Compared with cultures maintained in E2 plus MPA for 3-5 days, withdrawal to a steroid-free medium failed to affect stromal cell ir PAI-1, uPA, or tPA levels. In contrast, exposure to RU 486 for 3-5 days increased ir uPA and tPA levels 5- to 8-fold (P < 0.02) while reducing PAI-1 levels by 85% (P < 0.04). By 9-11 days of treatment, steroid withdrawal and RU 486 exerted similar effects on ir PAI-1, tPA, and uPA levels. Comparable results were obtained for PAI-1, uPA, and tPA steady state messenger ribonucleic acid levels. These findings indicate that RU 486 blocks and reverses progestin-inhibited PA expression, suggesting a mechanism for RU 486-induced endometrial hemorrhage and ECM dissolution.

Cells, Cultured↗