- How we treat the platelet glycoprotein defects; Glanzmann thrombasthenia and Bernard Soulier syndrome in children and adults. [Review]
- BJBr J Haematol 2018 Aug 17
- The inherited platelet glycoprotein deficiencies, Glanzmann thrombasthenia (GT) and Bernard Soulier syndrome (BSS) are rare but important long-term bleeding disorders. Once diagnosed, affected patien...
The inherited platelet glycoprotein deficiencies, Glanzmann thrombasthenia (GT) and Bernard Soulier syndrome (BSS) are rare but important long-term bleeding disorders. Once diagnosed, affected patients should be referred to a specialist centre for bleeding disorders for general advice and ongoing management. Patients do not require prophylactic treatment and so the management of GT and BSS focuses around prophylactic treatment prior to high risk procedures and treatment in response to non-surgical bleeding events and, in women, the management of menorrhagia and pregnancy. There is no consistent approach to the treatment or prevention of bleeding complications. Management must be tailored for each individual and the approach may not be the same for different events, even for the same patient, depending on the type of accident or invasive procedure, the extent of bleeding and the presence or not of platelet refractoriness.
- Use of Uterine Characteristics to Improve Fertility-Sparing Diagnosis of Adenomyosis. [Journal Article]
- JGJ Gynecol Surg 2018 Aug 01; 34(4):183-189
- Objective: To describe patient demographics, determine accuracy of clinical diagnosis, and evaluate reliability of laparoscopic uterine characteristics in the diagnosis of adenomyosis. Materials an...
Objective: To describe patient demographics, determine accuracy of clinical diagnosis, and evaluate reliability of laparoscopic uterine characteristics in the diagnosis of adenomyosis. Materials and Methods: Enrollment included 117 patients undergoing laparoscopic hysterectomy for benign indications. Intraoperatively, the attending surgeon predicted uterine weight; evaluated the presence of fibroids; and commented on the uterus' shape, color, and consistency while probing it with a blunt instrument. A prediction was also made about whether final pathology would reveal adenomyosis. Standardized video recordings were obtained at the start of the case. Each video was viewed retrospectively twice by three expert surgeons in a blinded fashion. Uterine characteristics were reported again with a prediction of whether or not there would be a pathologic diagnosis of adenomyosis. These data were used to calculate inter-and intrarater reliability of diagnosis. Results: Women with adenomyosis were more likely to complain of midline pain as opposed to lateral or diffuse pain (p = 0.048) with no difference in the timing of the pain (p = 0.404), compared to patients without adenomyosis. Uterine tenderness on examination was not an accurate predictor of adenomyosis (p = 0.566). Preoperative diagnosis of adenomyosis by clinicians was poor, with an accuracy rate of 51.7%. None of the intraoperative uterine characteristics were significant for predicting adenomyosis on final pathology, nor was any combination of the features (p = 0.546). Retrospective video reviews failed to reveal any uterine characteristics that generated consistent inter- or intrarater reliability (Krippendorff's α < 0.7) in making the diagnosis of adenomyosis. Conclusions: Clinical and video diagnosis of adenomyosis have low accuracy with no uterine characteristics consistently or reliably predicting adenomyosis on final pathology. (J GYNECOL SURG 34:183).
- Significant gynecological bleeding in women with low von Willebrand factor levels. [Journal Article]
- BABlood Adv 2018 Jul 24; 2(14):1784-1791
- Gynecological bleeding is frequently reported in women with von Willebrand disease (VWD). Low von Willebrand factor (VWF) may be associated with significant bleeding phenotype despite only mild plasm...
Gynecological bleeding is frequently reported in women with von Willebrand disease (VWD). Low von Willebrand factor (VWF) may be associated with significant bleeding phenotype despite only mild plasma VWF reductions. The contribution of gynecological bleeding to this phenotype has yet to be described. The optimal clinical bleeding assessment tool (BAT) to evaluate bleeding remains unclear. Using a standardized approach to phenotypic assessment, we evaluated gynecological bleeding and directly compared the Condensed Molecular and Clinical Markers for the Diagnosis and Management of type 1 VWD (Condensed MCMDM-1 VWD) and International Society on Thrombosis and Haemostasis (ISTH) BAT scores in 120 women enrolled in the Low von Willebrand in Ireland Cohort study. Heavy menstrual bleeding (HMB) was reported in 89% of female participants; 45.8% developed iron deficiency. Using identical data, Condensed MCMDM-1 VWD menorrhagia domain scores were significantly lower than ISTH BAT scores (2 vs 3; P < .0001), the discrepant results related to 40% of women not seeking medical consultation for HMB, reducing the sensitivity of the Condensed score. For those who reported HMB to physicians, the low VWF diagnosis was not expedited (age at diagnosis 34.2 vs 33.4 years in women failing to present; P = .7). Postpartum hemorrhage (PPH) was self-reported in 63.5% of parous women (n = 74); 21.6% required transfusion, critical care, radiological, or surgical intervention. Our data demonstrate that gynecological bleeding is frequently reported in women with low VWF; despite pregnancy-related increases in plasma VWF levels, these women may experience PPH. Defining the optimal management approach for these patients requires further research. This trial was registered at www.clinicaltrials.gov as #NCT03167320.
- A Prospective, Multicenter, Pivotal Trial to Evaluate the Safety and Effectiveness of the AEGEA Vapor Endometrial Ablation System. [Journal Article]
- JMJ Minim Invasive Gynecol 2018 Jul 20
- CONCLUSIONS: The AEGEA Vapor System is a safe effective and minimally invasive option for performing in-office endometrial ablation. under minimal anesthesia for the purpose of treating women who suffer from HMB.
- Antiplatelet and anticoagulant agents for primary prevention of thrombosis in individuals with antiphospholipid antibodies. [Review]
- CDCochrane Database Syst Rev 2018 07 13; 7:CD012534
- CONCLUSIONS: There is insufficient evidence to demonstrate benefit or harm of using anticoagulants with or without ASA versus ASA alone in people with aPL antibodies and a history of recurrent pregnancy loss and with no such history; ASA versus placebo in people with aPL antibodies; and ASA with LMWH versus placebo or IVIG, and ASA with high-dose LMWH versus ASA with low-dose LMWH or UFH, in women with aPL antibodies and a history of recurrent pregnancy loss, for the primary prevention of thrombotic events. In a mixed population of people with a history of previous pregnancy loss and without such a history treated with anticoagulant combined with ASA, the incidence of minor bleeding (nasal bleeding, menorrhagia) was increased when compared with ASA alone. Studies that are adequately powered and that focus mainly on thrombotic events are needed to draw any firm conclusions on the primary prevention of thrombotic events in people with antiphospholipid antibodies.
- Combination of microwave endometrial ablation and postoperative dienogest administration is effective for treating symptomatic adenomyosis. [Journal Article]
- JOJ Obstet Gynaecol Res 2018 Jul 12
- CONCLUSIONS: The combination of MEA and postoperative dienogest is useful for treating uterine adenomyosis with menorrhagia and dysmenorrhea.
- Iron Deficiency Anemia with Menorrhagia: Ferric Carboxymaltose a Safer Alternative to Blood Transfusion. [Journal Article]
- JMJ Midlife Health 2018 Apr-Jun; 9(2):92-96
- CONCLUSIONS: Intravenous FCM is an effective and a safe treatment option for IDA with a single administration of high dose without serious adverse effects obviating the need for blood transfusion before surgery.
- Ryzophagia secondary to PCOS -related menorrhagia. [Journal Article]
- MJMed J Malaysia 2018; 73(3):170-171
- We report the peculiar case of a patient who consumed raw rice daily and had iron-deficiency anaemia secondary to menorrhagia with underlying polycystic ovarian syndrome. A 32-year-old lady of Asian ...
We report the peculiar case of a patient who consumed raw rice daily and had iron-deficiency anaemia secondary to menorrhagia with underlying polycystic ovarian syndrome. A 32-year-old lady of Asian descent presented with symptoms of fatigue, lethargy and prolonged, irregular periods for the last two months. There was noticeable increase in body weight, male pattern alopecia and facial acne. In addition, she experienced sudden, unexplained predilection towards consumption of raw rice (up to 300- 400g/day). The patient was treated with oral iron and cyclical progestin. After three weeks, her haemoglobin improved and her ryzophagia subsided. Gynaecologists should be vigilant of pica, which can occur outside of the context of pregnancy and also poses potential health risks including tooth attrition, electrolyte imbalance, intestinal obstruction and poisoning.
- Intravenous immunoglobulin for severe thrombocytopenia in secondary dengue. [Journal Article]
- BCBMJ Case Rep 2018 Jun 27; 2018
- A 30-year-old woman with severe dengue presented on the sixth day of her illness with life-threatening thrombocytopenia, refractory to multiple platelet transfusions. Dengue IgM antibody and the non-...
A 30-year-old woman with severe dengue presented on the sixth day of her illness with life-threatening thrombocytopenia, refractory to multiple platelet transfusions. Dengue IgM antibody and the non-structural-1 antigen tests as of day 3 were negative. The IgG antibody against the same was positive, suggesting a past episode of dengue. Since she had a history of menorrhagia prior to the current illness, a working diagnosis of idiopathic thrombocytopenic purpura was made, for which intravenous immunoglobulin (IVIg) was administered that led to a rapid rise in the platelet count with no adverse events. Subsequently, dengue IgM antibody repeated on day 6 came back positive, confirming dengue. This case report re-emphasises the potential use of IVIg in patients with severe thrombocytopenia in dengue.
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- Non-arteritic anterior ischaemic optic neuropathy secondary to menorrhagia in a young healthy woman. [Journal Article]
- BCBMJ Case Rep 2018 Jun 27; 2018
- Non-arteritic anterior ischaemic optic neuropathy (NAION) may develop due to severe anaemia and hypotension which is seen in acute blood loss. The devastating visual loss is often irreversible. We re...
Non-arteritic anterior ischaemic optic neuropathy (NAION) may develop due to severe anaemia and hypotension which is seen in acute blood loss. The devastating visual loss is often irreversible. We report a case of NAION in a 20-year-old healthy woman, who presented on the third day of a heavy menstrual cycle with hypovolaemic shock. On day 2 of admission, she had sudden right eye blurring of vision at the superior field on awakening from sleep. Funduscopy revealed a pale and swollen right optic disc. There was a dense right superior altitudinal visual field defect. Her haemoglobin level was low (3.6 g/dL), but she refused blood transfusion due to her religious belief (Jehovah's Witness) and opted for conservative management. She later developed right optic atrophy with persistent visual field defect despite an improved haemoglobin level of 10.5 g/dL.