1/170. fibrinogen St. Gallen I (gamma 292 Gly--> Val): evidence for structural alterations causing defective polymerization and fibrinogenolysis. fibrinogen St. Gallen I was detected in an asymptomatic Swiss woman. Routine coagulation tests revealed a prolonged thrombin and reptilase time. Functionally measured fibrinogen levels were considerably lower than those determined immunologically. polymerization of fibrin monomers derived from purified fibrinogen was delayed in the presence of either calcium or EDTA. Normal fibrinopeptide a and B release by thrombin was established. An abnormal degradation of fibrinogen St. Gallen I by plasmin was observed. Fragment D1 of normal fibrinogen was fully protected against further proteolysis in the presence of 10 mM calcium, whereas fibrinogen St. Gallen I was partially further degraded to fragments D2 and D3. In the presence of 10 mM EDTA, the conversion of variant fragment D1 to D2 was accelerated whereas the degradation of fragment D2 to D3 was delayed in comparison to degradation of fragments D1 and D2 of normal fibrinogen. Three high-affinity calcium binding sites were found in both normal and variant fibrinogen. mutation screening with SSCP analysis suggested a mutation in exon VIII of the gamma-chain gene. Cycle sequencing of this gene portion revealed a single base substitution from G to T of the base 7527, leading to replacement of gamma 292 glycine by valine. The same mutation has already been described for the fibrinogen variant baltimore I. Molecular modeling was performed of a part of the gamma-chain containing the mutation site, based on recently published X-ray crystal structures of human fibrinogen fragment D and of a 30 kD C-terminal part of the gamma-chain. Significant structural alterations due to the substitution of glycine by valine at gamma 292 were observed, e.g. spreading of the protein backbone, probably leading to a modified accessibility of the plasmic cleavage sites in the gamma-chain at 356 Lys and 302 Lys. A shift of gamma 297 Asp that is involved in interactions of fragment D with the Gly-Pro-Arg-Pro-peptide was noted by molecular modeling. The latter observation is compatible with delayed polymerization of fibrin monomers. ( info) |
2/170. OB/GYN virtual consult--menorrhagia for 6 months in a 31-year-old. What treatment options would you advise for a young woman with vaginal bleeding of 6 months' duration? Malcolm Griffiths, MD, in the Department of obstetrics and Gynaecology, Luton & Dunstable Hospital, Luton, UK, moderates this case. ( info) |
3/170. Bleeding from self-administration of phenindione: a detailed case study. A young woman presented with a 2 year history of a severe bleeding disorder and marked deficiencies in all four vitamin-K-dependent factors. Metabolic studies with tracer doses of tritium-labelled vitamin K1 suggested that the patient might be taking an oral anticoagulant; and subsequently her plasma was found to contain a substance identical to phenindione in its spectrophotometric and chromatographic properties. The half-disappearance times of factors II, IX, X were measured after the administration of a concentrate of these factors and were found to conform with published figures. The concentrate controlled the patient's excessive bruising and prolonged skin and gingival bleeding. It would therefore seem that factor vii may not be essential in reversal of the bleeding disorder induced by anticoagulant overdose. ( info) |
4/170. Embolic occlusion of the blood supply to uterine myomas: report of 2 cases. Uterine myomas are the most common tumours of the female genital tract and with menorrhagia provide the most common indications for hysterectomy. Whilst myomectomy is a surgical alternative, it is associated with greater morbidity and a higher blood transfusion rate. Arterial embolization of myomas is a nonsurgical treatment option that can be performed as an outpatient procedure, is cheaper than myomectomy, permits preservation of reproductive potential, and may not only be associated with less morbidity than myomectomy but also may not cause adhesions which could compromise fertility. This paper details 2 cases treated by this technique. ( info) |
5/170. Vaginal expulsion of submucosal fibroids after uterine artery embolization. A report of three cases. BACKGROUND: Since October 1996, at our fibroid center, we have been using the uterine artery embolization (UAE) procedure as a nonsurgical means to treat patients with fibroids and menorrhagia. We have performed this procedure on over 180 patients, 3 of whom experienced vaginal expulsion of submucosal fibroids from two to seven months later. CASES: A 37-year-old woman underwent UAE in November 1997 and expelled five submucosal fibroids two to three months later. A 43-year-old woman underwent UAE in August 1997 and expelled a submucosal fibroid four months later. A 46-year-old woman underwent UAE in April 1997 and expelled a submucosal fibroid seven months later. CONCLUSION: The use of UAE to treat patients with fibroids and menorrhagia is relatively new. Our experience has revealed that a significant percentage of patients who have had the embolization procedure will have reduction in menorrhagia and also in the volume of their fibroids. Complications and side effects have been few. Vaginal expulsion of submucosal fibroids can be viewed as a side effect of the procedure, and, to the best of our knowledge, these are the first reported cases of this postembolization occurrence. ( info) |
6/170. Balloon endometrial ablation as a safe alternative to hysterectomy. Each year, 600,000 hysterectomies are performed in the united states, costing an estimated $4 billion. Approximately 50% of these hysterectomies are performed for menorrhagia or abnormal uterine bleeding. menorrhagia can have a negative impact on a woman's lifestyle and self-perception, often leading her to seek definitive treatment. Pharmacologic treatment for menorrhagia is not always successful, and dilatation and curettage provides relief for only the first few menstrual cycles. Surgical options include hysterectomy and two forms of endometrial ablation. Current research demonstrates that thermal uterine balloon therapy is the safest of these options. Uterine balloon therapy, an outpatient procedure, has resulted in successful reduction in menstrual flow in 70% to 90% of patients, and it soon may be performed as an office-based procedure. ( info) |
| factor v deficiency is a rare hereditary disorder. We report a patient with factor v deficiency who presented with menorrhagia and pelvic haematoma. The Haematology Department at the Royal Brisbane Hospital performed the definitive factor assays leading to the diagnosis. The challenges of her management were obtaining adequate supplies of factor V and her socioeconomic circumstances. The main future challenge will be the supervision of her pregnancies. ( info) |
8/170. Ill patient with unknown or hidden pregnancy. Many pregnancies are inadvertently interrupted because diagnostic and therapeutic procedures that are potentially teratogenic are unwittingly instituted. To prevent this ever-present possibility, it is essential that the family physician as well as the hospital's admitting physician know for certain whether a patient in her childbearing years is pregnant. The simple direct question "Are you pregnant?" unfortunately does not always elicit a reliable answer. The patient may not know that she is pregnant, a common occurrence in early pregnancy, or for any of a number of reasons she may choose to conceal her pregnancy. A pregnancy test thus becomes an important diagnostic tool in the armamentarium of every conscientious physician. It should be given routinely to all women of childbearing age before instituting extensive diagnostic x-ray studies, radiation therapy, or chemotherapy that may be contraindicated in pregnancy. Because most contraindicated procedures are performed in a hospital, it is suggested that a pregnancy test on urine be made mandatory on a woman's admission to a hospital. ( info) |
9/170. Effect of the levonorgestrel-releasing intrauterine system on uterine myomas in a renal transplant patient. The levonorgestrel-releasing intrauterine system (LNG-IUS) has been used in the treatment of both idiopathic menorrhagia and adenomyosis. An electronic search of the on-line medical literature revealed no reports of its use for menorrhagia secondary to uterine myomas. Presented here is the successful treatment of uterine myomas with menorrhagia in a woman with a renal transplant. There was a significant reduction in menorrhagia, dysmenorrhea, and uterine and myoma size with the use of the LNG-IUS. We believe that this system provides an alternative to conventional hysterectomy and gonadotrophin-releasing hormonal analog medical treatment for uterine myomas, with a possibly inhibitory effect on myoma growth. ( info) |
10/170. Adverse events during use of intranasal desmopressin acetate for haemophilia A and von Willebrand disease: a case report and review of 40 patients. We report our experience with the incidence of adverse events during the use of Stimate brand intranasal desmopressin acetate (IN DDAVP) for patients with haemophilia A (HA) or von Willebrand disease (vWD) after noting two severe adverse events in one adult patient. All patients with documented vWD (type 1 or 2 A) or haemophilia A (mild, moderate or symptomatic carrier) from the Emory Comprehensive Hemophilia Center who had IN DDAVP challenge testing or were using Stimate for treatment of bleeding were evaluated for adverse events by patient report or nursing observation of clinical signs and symptoms. Forty patients were studied. Sixty-eight per cent (27/40) experienced clinical signs and/or symptoms. The majority of these symptoms were mild, however several patients reported moderate to severe side-effects and one adult patient required medical intervention for symptomatic hyponatraemia. In our experience, two-thirds of patients tested experienced adverse signs and/or symptoms with the use of Stimate; considerably higher than that reported from preliminary results in the literature. Young age did not correlate positively with adverse reactions. Severe adverse events requiring medical intervention were rare, however symptoms such as moderate to severe headache, nausea, vomiting and weakness may necessitate evaluation for hyponatraemia. This is the first report of symptomatic hyponatraemia in an adult patient with recommended dosing of Stimate. Side-effects may be minimized if patients adhere to instructions regarding fluid intake and composition while using IN DDAVP. ( info) |