[Restrictive dermopathy: a rare, lethal genodermatosis].
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Biomedical subjects
Publications and source records attributed to J G van der Stege.
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Eighty-seven patients undergoing in vitro fertilization were studied to evaluate the prognostic value of different tests in predicting ovarian stimulation response. We studied basal follicle-stimulating hormone (FSH) value on cycle day 3, the clomiphene citrate (CC) challenge test and serum estradiol levels on cycle day 3. Patients with elevated basal FSH levels needed more ampoules of gonadotropins for stimulation and had a higher cancellation rate because of poor response. Patients with abnormal CC challenge test results also needed more ampoules of gonadotropins, but the higher cancellation rate in this group did not reach statistical significance. The basal estradiol level did not contribute to further discrimination between groups. We conclude that basal FSH level on cycle day 3 is a useful prognosticator of ovarian stimulation response.
Perlman syndrome is a rare disease with a poor prognosis. Until now, the specific antenatal diagnostic criteria of this syndrome have not been documented. We report on two pregnancies with Perlman syndrome, in the same woman, in which karyotyping and ultrasound examination were performed. In the first pregnancy, the final diagnosis was made only postnatally, whereas, in the second affected pregnancy, nuchal translucency was seen at 11 weeks. From 23 weeks onwards the classical signs of renal and abdominal enlargement were observed. The impact of non-specific early ultrasound markers on decision-making in pregnancies with family risks, where specific tests are not yet available, must be evaluated.
In this report we describe an uncomplicated pregnancy and puerperium in a 34 year old patient who had a previous puerperal superior sagittal sinus thrombosis. Heparin was given 3 weeks antepartum and oral anticoagulants 3 months postpartum.
Restrictive dermopathy is a lethal autosomal recessive skin disease. Prenatal diagnosis has not yet been reported. We present a case of restrictive demography and describe the associated prenatal sonographic findings. A continuously open mouth was the most striking feature and this sonographic finding may be a marker of a skin disease.
OBJECTIVES: To evaluate our experience with laparoscopic supracervical hysterectomy (LASH) and to assess the short- and medium-term outcome. METHODS: Retrospective analysis of patient and surgery characteristics from chart review and evaluation of patient satisfaction by a questionnaire. RESULTS: Forty-one patients who underwent LASH were studied with a mean length of follow-up of 27 months. Operative complications consisted of one bladder lesion and one bleeding at the trocar site. Postoperative complications were bladder atony (1), paralytic ileus (1), a pulmonary embolism (1) and vaginal hemorrhage from the colpotomy incision (1). Twenty-five percent of the patients continued to menstruate, and 10% had symptoms of discharge. Overall, 98% of the patients were satisfied with their operation. CONCLUSIONS: Although preservation of the cervix with laparoscopic hysterectomy for benign diseases was satisfactory in most of the cases, several women had complications of the remaining cervix. Special attention should be paid to the careful treatment of the cervical stump. Further prospective studies are needed to evaluate the advantages of retaining the cervix at laparoscopic hysterectomy.