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Biomedical subjects

P Wieacker

Publications and source records attributed to P Wieacker.

At least 55 records · Page 3Linked to original sources
PubMed1992

[A case of pseudo-vaginal, perineoscrotal hypospadia with 5-alpha reductase deficiency].

A case of pseudovaginal perineoscrotal hypospadia (PPSH) is presented. This autosomal recessive disorder, also termed incomplete male pseudohermaphroditism type 2, is mostly caused by a deficiency of 5 alpha-reductase, which controls the conversion of testosterone to 5 alpha-dihydrotestosterone. In genital skin fibroblasts, the activity of the 5 alpha-reductase was strongly reduced, compared with a normal male. The 5 alpha-reductase activity in nongenital skin fibroblasts, however, was in the range of the normal male control. For complete diagnostic evaluation of PPSH 5 alpha-reductase activity it should be determined simultaneously in genital and non genital skin fibroblasts. The conversion of T to DHT in genital skin fibroblasts of a patient with testicular feminisation (Tfm) was found to be of the same order of magnitude as in PPSH. This suggests, that the expression of 5 alpha-reductase is androgen-dependent.

3-Oxo-5-alpha-Steroid 4-Dehydrogenase↗
PubMed1992

Pathophysiology of polyhydramnios in twin transfusion syndrome.

In 3 cases of severe twin transfusion syndrome we demonstrate that the concentration of atrial natriuretic factor (ANF) in the cord blood of recipient twins is significantly elevated compared to that of donor twins. The discrepancy between recipient and donor concentration correlates with the volume of transfusion. The following pathophysiological mechanism for explaining polyhydramnios in recipient twins is proposed: chronic overload in recipient twins causes enhanced release of ANF from the fetal heart. Consequently, increased fetal urine production leads to polyhydramnios, which is additionally enhanced by inhibition of ADH release.

Adult↗
PubMed1991

[Contraception by cyclic treatment with buserelin and progesterone].

Hormonal contraception with a combination of a GnRH-agonist (Buserelin) and progesterone was achieved in 47 high risk patients in 547 cycles. In these patients, oral contraceptives were contraindicated because of severe cardiovascular diseases, thromboembolic complications, benign liver tumours, advanced diabetes, terminal kidney insufficiency and severe migraine. Buserelin was administered intranasally in one daily dose of 300-400 micrograms from the 1st day to the 21st day, one dose of 100 mg of Progesterone was given intravaginally daily from the 12th day to the 21st day. Under these conditions, average E2 concentrations were found in the range of 50-60 pg/ml. The discontinuation of progesterone replacement resulted in withdrawal bleeding. Cycle control was acceptable. In 4 cases, this contraceptive regimen had to be discontinued because of side effects or paradox reactions. One patient conceived. In conclusion, GnRH-analogues in a moderate dose can be used to inhibit ovulation without inhibiting follicular maturation and adequate oestrogen production. This costly regimen of contraception requires strict indication and careful monitoring.

Administration, Intranasal↗
PubMed1991

[Semiquantitative determination of the amniotic fluid volume in normal pregnancies].

The amount of amniotic fluid was determined semiquantitatively in 600 pregnancies between the 12th and 41st week of gestation. Pregnancies at risk were excluded. The largest amniotic fluid area was measured sonographically in two dimensions. The mean plus/minus standard deviations are correlated with gestational age. A diagram has been prepared. Our results show good correlation with values from invasive quantitative examinations of amniotic fluid amount. However, our procedure is easy to perform and without hazard for the pregnancy, since it is non-invasive.

Amniotic Fluid↗
PubMed1991

[Primary ovarian insufficiency in polyendocrinopathy syndrome].

We describe two cases of autoimmune polyendocrinopathy syndrome as a cause of primary ovarian failure. Antibodies against various endocrine organs cause pluriglandular insufficiency, in most cases hypoparathyroidism. Addison's disease, and primary ovarian insufficiency. In these patients, careful examination of all endocrine organs is necessary in order to provide an adequate hormone replacement.

Adult↗
PubMed1991

Fetal urinary tract obstructions: prenatal diagnosis--prenatal and postnatal therapy.

A retrospective study was conducted comprising 78 cases of fetal urinary tract obstructions diagnosed by ultrasound. Thirteen of the obstructions were subvesical and 65 supravesical. In only one fetus with a subvesical obstruction leading to megacystis was a puncture of the fetal bladder performed--in the 17th week of gestation--resulting in restitution of the bladder to its normal size. In all of the remaining fetuses the kidneys, lungs, and bladder changes had already reached an advanced stage by the time the ultrasound diagnosis was made. In the 65 fetuses with supravesical urinary tract obstructions in utero puncture to relieve a rapidly developing hydronephrosis only seemed advisable in two cases. All of the prenatal diagnoses were confirmed postpartum with the exception of two Potter IIa kidneys, which had been interpreted as being hydronephrosis. The time and method of postnatal management are described. The results of the study indicate that in utero intervention is only indicated in the very rare case. Nearly all of the supravesical obstructions remained unchanged, some even for months. In these cases there was no evidence of cystic-dysplastic renal changes after delivery.

Diagnosis, Differential↗
PubMed1990

Oral contraception in disease states.

Oral contraceptives are clearly contraindicated in patients with a history of thromboembolic disease, ischemic heart attack, or cerebral stroke. Patients requiring long-term anticoagulant treatment can be treated with gonadotropin-releasing hormone analogs to prevent ovulation, because ruptured follicles can cause massive intraperitoneal bleeding. Patients with essential hypertension and severe liver diseases should also discontinue treatment 4 weeks before major elective surgery. Migraine and diabetes mellitus are regarded as relative contraindications, depending on the individual situation. Long-term diseases, such as Crohn's disease, epilepsy, and sickle cell anemia, also require individualized consultation.

Cerebrovascular Disorders↗
PubMed1990

[Prenatal diagnosis of Pena-Shokeir syndrome in the 27th week of pregnancy].

We report on the prenatal diagnosis of a Pena-Shokeir syndrome sporadic case of the 27th week of pregnancy. Typical sonographical features are polyhydramnios, hypoplasia of lungs, foetal akinesia, reduced length of proximal and distal extremities and a striking discrepancy between biparietal and abdominal diameter. Differential diagnosis of polyhydramnios caused by foetal neuromuscular disorders is discussed.

Abnormalities, Multiple↗
PubMed1990

[Prognosis of triplet pregnancies].

31 triplet pregnancies (DG), treated during 1975-1989, were analysed retrospectively. 13 DG (D10) were registered up to the end of the 10th week of pregnancy, 16 (DG) (D16) were intact between the 11th and the 16th gestational week. 3 DG resulted from the reduction of high grade multiple pregnancies. 18 DG (62%) of the initial 29 DG had previously been treated for sterility. The spontaneous foetal loss (DF) in group D10, including the perinatal mortality, amounted to 26% (10 of 39 DF). In one case of this group, a previously vital DF died. In another case, a partial bi-phasic abortion occurred followed by a premature birth of the surviving 3rd DF. The mortality rate in group D16, including the perinatal mortality (4 DF), was 25% (12 of 48 DF). 30 DG were still intact after the 16th week of pregnancy. 2 DG (7%) suffered from EPH-gestosis. In 4 DG (13%) a foeto-foetal transfusion syndrome occurred. 5 DF (6%) had some of the serious malformations. 27 DG were completed. 7 DG (26%) ended before the 32nd gestational week. In 13 DG (48%), a Caesarean section was performed. The perinatal mortality amounted to 11% (9 of the 81 DF) including all the births from the 25th gestational week upwards. On comparing these results with the data in the literature, a significant improvement in the prognosis of DG in recent years can be observed. This is due to the progress in antenatal and neonatal care.

Cesarean Section↗
PubMed1990

[GnRH-analogs in the therapy of uterine myomatosis].

Uterine fibroids are the commonest tumors of the female genital tract. Hysterectomy is the typical therapy in patients whose families are complete. In women desirous of children GnRH-analogues can effect a shrinkage of leiomyomata before a myomectomy. Furthermore, GnRH-A treatment can be an alternative to hysterectomy in inoperable patients or in premenopausal women with symptoms of uterine fibroids. In this study eleven patients were treated with 3.2 mg triptorelin monthly and ten patients with 900 micrograms buserelin daily for 6 months. With triptorelin, a 50% reduction of the uterus volume can be observed after 3 months. A further treatment has no benefit. With buserelin, a regression in the same range as with triptorelin can be reached only after 6 months. In contrast, fibroid volumes revealed a regression of 28% with triptorelin and 21% with buserelin in the same time. In this period all fibroid-associated symptoms disappeared. Less bleeding resulted in an increase of hemoglobin. Therefore, treatment with GnRH analogues can be an important factor in the management of uterine fibroids patients in at-risk.

Adult↗
PubMed1989

[Pregnancy in Addison's disease].

A 29-year-old woman with Addison's disease was hospitalized in the eighth week of pregnancy because of an Addisonian crisis. The crisis was successfully treated with physiological saline infusions, as well as hydrocortisone (25 mg/d) and fludrocortisone (0.05 mg/d). The dose of fludrocortisone had to be increased to 0.1 mg/d from the 21st week of pregnancy onwards, because hyponatraemia and hypotension had once again developed. The hydrocortisone dose was raised to 37 mg/d from the 32nd week of pregnancy onwards because of a latent hypoglycaemia and raised further to 50 mg/d from the 35th week onwards because the retardation in fetal growth had become more marked. A mature infant was delivered spontaneously on the 282d day of pregnancy. At the start of labour the patient had been given 100 mg hydrocortisone intravenously and then 50 mg at the moment of delivery. The substitution treatment was gradually reduced to the original (pre-pregnancy) dosage during the first three post-partum days.

Addison Disease↗
PubMed1989

Hirsutism, its pathogenesis.

Hirsutism can be regarded as a virilizing symptom and may be defined as a male type of body hair distribution in the female. The pathogenesis of hirsutism may be due to an increased androgen production or to an enhanced sensitivity of the hair follicles in sexual areas. The androgen production in the female depends upon direct secretion by the ovaries and the adrenals and upon peripheral conversion of androgen precursors and finally on the metabolic clearance rate which may be regarded as a function of androgen production. More than 98% of the androgens circulating in the blood are bound to specific plasma proteins such as steroid hormone binding globulin (SHBG), cortisol binding globulin (CBG) and albumin. The synthesis of SHBG is controlled by the ratio of oestradiol to testosterone (T). Elevation of oestrogens results in an increase of SHBG. In hirsutism the plasma concentrations are decreased, resulting in elevated levels of free androgens. The intracellular reduction of T to 5 alpha-DHT (dihydrotestosterone) has to be considered as a basic requirement for the androgen-mediated growth of the hair follicle in sexual skin areas. The sensitivity of these areas for androgen depends upon the activity of the local 5 alpha-reductase. In patients suffering from hirsutism, the conversion rate of T to 5 alpha-DHT is significantly increased, almost reaching male levels. DHT is further metabolized by the target cells to 3 alpha- and 3 beta-androstanediol and the corresponding glucuronides. The elucidation of its complex pathogenesis is still incomplete; however,the information available so far provides a reasonable basis for further diagnostic and therapeutic approaches.

Female↗
PubMed1989

Prenatal diagnosis of Wiedemann-Beckwith syndrome.

We report the prenatal diagnosis of Wiedemann-Beckwith syndrome in the 28th week of pregnancy in a primigravida. The cause of hospitalization was severe polyhydramnios with premature labour. Sonography revealed macrosomia, nephromegaly, cysts of adrenal glands and a striking macroglossia with opened mouth in all controls. The combination of all these symptoms suggested the prenatal diagnosis of Wiedemann-Beckwith syndrome, which could be confirmed post partum after a normal delivery in the 33rd week of pregnancy. The newborn (2700 g, 48 cm) exhibited typical features of this syndrome as moderate hypoglycemia and polycythemia in addition to the sonographic finding. Pathogenetic aspects and differential diagnosis of macrosomia are discussed.

Adult↗
PubMed1988

[Effects of sex steroids on the lipoprotein profile].

Plasma testosterone levels were measured pre- and postoperatively as well as during estrogen substitution in a 47-year-old woman with excessive hyperandrogenaemia due to a Leydig-cell tumour of the left ovary. In addition, the lipoprotein profile was determined before and 14 days after removal of the tumour and four weeks later during estrogen substitution. The preoperative plasma testosterone level was between 6.25 and 8.50 ng/ml; at the same time there was a hypercholesterolaemia with increased beta-lipoprotein and decreased alpha-lipoprotein fractions: the ratio of beta- to alpha-lipoprotein cholesterol was 3.0, a type IIa hyperlipoproteinaemia. After the testosterone concentration had returned to normal both the beta- and pre-beta cholesterol fell significantly, while the alpha-lipoprotein fraction remained unaltered. The alpha-cholesterol fraction increased significantly after estrogen substitution, the beta- to alpha-lipoprotein ratio being 1.20. It is concluded from these results that hyperandrogenaemia goes together with raised peripheral beta and pre-beta-cholesterol concentration, while estrogens cause a rise in the alpha-fraction.

Cholesterol↗
PubMed1988

[Use of gonadotropin releasing hormone analogs in Marcumar-treated patients for contraception and prevention of life-threatening ovulation hemorrhages].

Five severe cases of intra-abdominal gynaecological haemorrhage (four haemorrhages from corpora lutea haemorrhagica and one haemorrhage of the endometritis genitalis externa) are described in relation to prolonged marcumar therapy. The problem of contraception in patients under marcumar therapy is highlighted and the possibility is discussed of prevention of ovulation and cardio-vascularly neutral treatment, to achieve contraception as well as avoidance of haemorrhage at ovulation with the Gn-RH analogue Buserelin.

4-Hydroxycoumarins↗
PubMed1988

[Effect of sex steroids on the lipoprotein profile].

Hyperandrogenemia is associated with relatively high LDL- and low HDL-cholesterol levels, increasing the risk of the development of arteriosclerotic disease. In three women suffering from androgen producing ovarian tumors, this observation could be confirmed. After surgical removal of the ovaries, lipoprotein profiles returned to normal. In contrast, androgens did not affect lipoproteins in two testicular feminized individuals. Despite elevated testosterone levels, the lipoprotein profiles were absolutely normal. This observation indicates that the affects on the lipoprotein profile is receptor mediated.

Adolescent↗
PubMed1988

[Gonadotropin resistance in Rothmund-Thomson syndrome].

A case of Rothmund-Thomson syndrome of a 24-year old woman with primary amenorrhoea is presented. This autosomal recessive disorder is characterised by atrophy, hyperpigmentation and teleangiectasiae of the skin, furthermore by juvenile cataracts and congenital bone defects as saddle nose. Endocrinologic and morphologic parameters suggest a resistant ovary syndrome as cause of this hypergonadotropic hypogonadism.

Adult↗