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Z Papp

Publications and source records attributed to Z Papp.

At least 217 records · Page 12Linked to original sources
PubMed1990

Discriminant analysis for assessing the value of amniotic fluid microvillar enzymes in the prenatal diagnosis of cystic fibrosis.

We have analysed the sensitivity, specificity, and reliability of biochemical diagnosis based on microvillar membrane enzyme assay and using discriminant analysis in amniotic fluid samples obtained from 54 pregnancies at high risk for cystic fibrosis and 125 normal pregnancies. Our results show that amniotic fluid trehalase, alkaline phosphatase, alkaline phosphatase isoenzymes and gamma-glutamyltransferase enzyme activities measured during 16-20 gestational weeks, in spite of their non-specificity for cystic fibrosis, have a very good predictive value for fetal cystic fibrosis or exclude the possibility of the disease. Overall enzyme activity analysis provided over 90 per cent reliability of the method.

Alkaline Phosphatase↗
PubMed1990

Amniotic fluid microvillar enzyme activity in fetal malformations.

Prenatal diagnosis of cystic fibrosis based on amniotic fluid microvillar enzyme activity assay has become routine practice in the past few years. Normal (median) values of these enzymes were determined in 177 normal healthy pregnancies between 15-20 gestational weeks and were related to enzyme values measured in 50 pregnancies complicated with congenital malformations, 6 monogenic inherited diseases and 4 chromosomal aberrations. It is concluded that increased trehalase activity has diagnostic importance in detecting fetal kidney diseases, and radial-renal syndrome (with elevated GGT activity), while low enzyme activities may indicate chromosomal aberrations (with no signs of intestinal obstruction). With the collection of further data, the analysis of these enzymes might provide an opportunity to set up diagnostic procedures for the detection of other, non-CF-related cases.

Alkaline Phosphatase↗
PubMed1990

First trimester diagnosis of cystic fibrosis with linked DNA probes.

In late 1985 the cystic fibrosis (CF) gene was located to chromosome 7, at 7q 22/31. Several restriction fragment length polymorphism (RFLP) markers are closely linked to the CF gene. These markers permit accurate first-trimester prenatal diagnosis based on analysis of chorionic villus DNA by studies of families with one or more affected children. In our laboratory 13 families at risk of having a child with CF have been counselled by the use of linked DNA probes: xV-2c; pCS.7; Met H; Met D; pJ3.11; KM 19. In all cases one or more of the mentioned probes were sufficiently informative to allow first-trimester prenatal diagnosis. In four of the 13 families tested prenatal diagnosis have been performed.

Cystic Fibrosis↗
PubMed1990

Pathological confirmation of foetal cystic fibrosis following prenatal diagnosis.

Here we report on the results of histopathological analysis of several organs of 5 foetuses and 2 newborn infants with cystic fibrosis. They were examined with HE, PAS, AB, HID and "Stains-all" techniques on paraffin sections. We concluded that there were significant differences in the epithelial mucin composition of several organs of the effected foetuses compared to 6 controls as early as the 17th week of gestation. An increase in the amount of neutral and acidic mucins was observed in the acini of the pancreas, bronchi and the mucosa of the gastrointestinal tract accompanied with a well defined decrease of sialic acid rich components of pharyngeal submucosal glands.

Cystic Fibrosis↗
PubMed1990

First trimester chorionic villus sampling for DNA analysis.

Early prenatal diagnosis of cystic fibrosis (CF) has become possible after the identification of linked DNA markers on chromosome 7. Chorionic villus sampling (CVS) has made possible the first-trimester prenatal diagnosis of CF. We report our experience of 336 pregnant women between 8-12th week. Six different types of sampling devices have been used to get chorionic tissue. Our results proved that the quantity and the quality of the sample gained was the same irrespective of the method employed in obtaining them.

Chorionic Villi Sampling↗
PubMed1990

Genetic counselling and prenatal diagnosis of cystic fibrosis in Debrecen (Hungary)--prenatal diagnosis by microvillar enzyme assay from amniotic fluid.

Amniotic fluid intestinal alkaline phosphatase, gammaglutamyltransferase and trehalase activity were quantitated to assess their reliability for the prenatal diagnosis of cystic fibrosis. To obtain optimal diagnostic discrimination, the three enzyme values obtained for each sample were combined into a single linear discriminant function that proved to be a more accurate indicator of the outcome of the pregnancy. From the cases studied here, it appears that this method can be expected to give a correct prediction in 92.0% of all high risk pregnancies.

Amniotic Fluid↗
PubMed1990

Ultrasound diagnosis and screening of fetal cystic fibrosis.

By ultrasound examination of high risk pregnancies for cystic fibrosis in some cases echogenic areas and dilated bowels could be demonstrated. These signs could be detected in 75% of those cases where biochemical assay of the amniotic fluid proved the fetus to be affected with cystic fibrosis. Having got these results authors started to look for these signs during the screening of normal pregnancies. Out of 22 thousand screened pregnancies 28 amniocenteses have been performed because of the ultrasound finding and in 18 cases the low microvillar enzyme activity also predicted cystic fibrosis.

Amniotic Fluid↗
PubMed1990

Invasive intrauterine procedures in twin pregnancies discordant for fetal malformation.

Invasive intrauterine procedures in two twin pregnancies for exencephaly and multiple malformations are reported. In the first case, to ensure the development of the normal fetus, selective feticide of the affected fetus was undertaken by transabdominal intracardial injection of 20% NaCl solution. A healthy newborn infant with normal weight and a fetus papyraceus were delivered at term. In the second case, because of monoamnial placentation, the procedure was regarded too dangerous, therefore, only therapeutic amniocentesis was carried out to decrease the volume of amniotic fluid. The fetuses were delivered in the preterm period. The advantages of the procedure of selective feticide developed by the authors are also discussed.

Abortion, Induced↗
PubMed1989

[Sex determination of the embryo by DNA studies of chorionic villi samples].

The first step in the prenatal diagnosis of X-linked genetic disorders is the determination of the sex of the fetus. A new method for this purpose is based on recombinant DNA technology. The authors give a short account on their experiences with a Y specific DNA probe. Fetal DNA was prepared from chorionic villi taken at the 8th-12th weeks of gestation. The DNA was hybridised with the Y specific probe. This probe was isolated from the 3,4 kilobase human repeat sequence derived from heterochromatin of the Y chromosome and had 1000 times more affinity for male DNA than for female DNA. The method based on hybridisation with the Y specific probe should facilitate first-trimester prenatal sex determination of X-linked genetic disorders.

Abortion, Legal↗
PubMed1989

[Successful delivery of a patient with two artificial valves (mitral and aortic)].

The authors report the case of the successful delivery of a 35-year old woman who underwent a double Sorin tilting disc valve replacement because of severe mitral and aortic valve disease in NYHA III-IV. functional class six years ago. Beside reporting on outcome of the case and complications, they mention the possible other complications too, endangering the life prospects of the mother and child that can come about as a result of the diminished pump function of the myocardium, blood coagulation changed during pregnancy and due to the hemmorrhagic and teratogenic consequences of anticoagulant treatment.

Adult↗
PubMed1989

[Surgical management of uterus bilocularis (the first 40 transabdominal metroplasty procedures)].

Forty patients were diagnosed by hysterosalpingography and/or ultrasound as having septate uterus which were assumed to be responsible for their recurrent abortions (35 cases) or infertility (5 cases). All patients, who have not had children yet, had abdominal metroplasty: 27 became pregnant; of their 37 pregnancies 28 continued to term (all but two with delivery by cesarean section) and 9 aborted including 1 hydatidiform mole and 4 blighted ovum, which were due probably not to uterinal but genetic and/or andrologic causes.

Abdomen↗
PubMed1989

[A life-saving hemostatic procedure by ligation of the hypogastric artery in hemorrhage caused by cervix carcinoma].

Ligation of the hypogastric arteries may be a lifesaving procedure for patients with intractable hemorrhage from pelvic viscera. This is especially true in the field of obstetrics and gynecology in which hemorrhage remains a major cause of mortality. Authors ligated both hypogastric arteries to control intractable hemorrhage in an advanced case of cervical carcinoma. It is emphasized that the technique of hypogastric artery ligation should be practised by all gynecologists.

Adult↗
PubMed1989

[The role of radical abdominal hysterectomy and lymphadenectomy (Wertheim's operation) in contemporary gynecology].

During a 20-year period between January 1964 and December 1983, 317 radical hysterectomies and pelvic lymphadenectomies were performed for stage IB, IIA and IIB carcinoma of the cervix at the Department of Obstetrics and Gynaecology, University Medical School of Debrecen. The 5-year survival rate for Stage IB patients is 88.3 per cent, in Stage IIA 82.7%, in Stage IIB 68.0% and the major prognostic factor clearly being the status of the pelvic lymph nodes. In comparison with the literary data their results seem to suggest that routine pre- and postoperative radiotherapy reduces pelvic metastases and improves survival in patients with positive pelvic lymph nodes. It was noted that closed retroperitoneal suction drainage after radical operation effectively removes fluid from the pelvis and probably prevents some of the postoperative complications. Evidence seems to suggest that properly performed radical pelvic surgery with radiation therapy is successful in treating early cervical carcinoma. There does not appear to be any contraindication to operation in this group of patient as long as the condition is deemed medically operable.

Female↗
PubMed1989

[Ruling out fetal Sanfilippo's syndrome (mucopolysaccharidosis IIIA) in the first trimester of pregnancy].

The defect of the enzyme heparan sulfamidase is the cause of Sanfilippo A syndrome (mucopolysaccharidosis IIIA) which is an autosomal recessive inherited disease. Three children of a marriage couple who attended our genetical counselling died of this disease which at the moment is incurable. At the 10th week of the following pregnancy chorion villi analysis was carried out and normal values of the enzyme heparan sulfamidase were obtained. In view of this it was decided that pregnancy should continue. At the moment the child is one year old and clinical and laboratory findings also show that she is not affected.

Adult↗
PubMed1989

Genetic counseling and termination of pregnancy in Hungary.

The practice of prenatal diagnosis has brought with it the utilization of pregnancy termination as a preventive approach. In this paper the genetic/teratologic, fetal and maternal indications for termination of pregnancy used in Hungary are described, as well as the legal requirements and the proposed mode of termination at the different stages of gestation. The author is the director of the largest prenatal genetic counseling service in Hungary.

Abortion, Induced↗