scholarly journals Case Report: Life-Threatening Post-operative Hemorrhage in Klippel–Trenaunay Syndrome Associated With Hypofibrinogenemia

2021 ◽  
Vol 8 ◽  
Author(s):  
Hongna Yang ◽  
Binfeng Du ◽  
Han Liu ◽  
Yong Yao ◽  
Chen Li
Keyword(s):  
Corpus Luteum ◽  
Conservative Therapy ◽  
Postoperative Bleeding ◽  
Rare Condition ◽  
Reproductive Age ◽  
Bleeding Complications ◽  
Rare Congenital Disorder ◽  
First Choice ◽  
Life Threatening ◽  
Klippel Trenaunay Syndrome

Klippel–Trenaunay Syndrome (KTS) is a rare congenital disorder, characterized by venous and lymphatic malformations of the skin, soft tissue, and bone, causing limb hypertrophy. Although, a ruptured hemorrhagic corpus luteum is a rare condition in women of reproductive age, it can result in lethal outcomes. Here, we have described a patient with KTS and hypofibrinogenemia who went through recurrent lethal postoperative bleeding due to a ruptured hemorrhagic corpus luteum. This case suggested that conservative therapy might be the first choice and effective therapy for the patients with KTS, who suffered from bleeding complications of surgical therapy.

scholarly journals Successful Treatment of Uterine Arteriovenous Malformation due to Uterine Trauma

2016 ◽  
Vol 2016 ◽  
pp. 1-3 ◽  
Author(s):  
Burak Karadag ◽  
Onur Erol ◽  
Ozgur Ozdemir ◽  
Aysel Uysal ◽  
Ahmet Sukru Alparslan ◽  
...  
Keyword(s):  
Arteriovenous Malformation ◽  
Uterine Artery ◽  
Massive Bleeding ◽  
Reproductive Age ◽  
Uterine Arteries ◽  
Future Fertility ◽  
First Choice ◽  
Uterine Arteriovenous Malformation ◽  
Life Threatening ◽  
Extensive Lesion

Uterine arteriovenous malformation (AVM) is defined as abnormal and nonfunctional connections between the uterine arteries and veins. Although the patients typically present with vaginal bleeding, some patients may experience life-threatening massive bleeding in some circumstances. The treatment of choice depends on the symptoms, age, desire for future fertility, and localization and size of the lesion; however, embolization of the uterine artery is the first choice in symptomatic AVM in patients at reproductive age with expectations of future fertility. We report a case of acquired AVM (after D/C) with an extensive lesion, which was successfully treated with bilateral uterine artery embolization (UAE).

scholarly journals A rare association of angiokeratoma circumscriptum with Klippel Trenaunay syndrome

2017 ◽  
Vol 3 (2) ◽  
pp. 296
Author(s):  
Aarti S. Salunke ◽  
Ravindranath B. Chavan ◽  
Vasudha A. Belgaumkar ◽  
Pallavi P. Patil
Keyword(s):  
Lower Limb ◽  
Congenital Abnormalities ◽  
Lymphatic Vessels ◽  
Port Wine ◽  
Equal Distribution ◽  
Rare Association ◽  
Rare Congenital Disorder ◽  
Life Threatening ◽  
Clinical Triad ◽  
Klippel Trenaunay Syndrome

<p class="abstract"><span lang="EN-IN">Klippel-Trenaunay syndrome (KTS) is a rare congenital disorder of blood and lymphatic vessels that is characterised by a combined vascular malformation of the capillaries, veins and lymphatics, congenital abnormalities, and associated limb hypertrophy. It may also involve gastrointestinal tract which can lead to life threatening bleeding. This syndrome presents since birth to early infancy or childhood with equal distribution in both genders. An adolescent female presented with right lower limb hypertrophy with port wine stain and overlying hemorrhagic vesiclessince birth. Histopathology of these hemorrhagic vesicles angiokeratoma. On the basis of history and classical clinical triad patient was diagnosed as KTS with angiokeratoma, an association rarely reported in literature.</span></p>

scholarly journals Congenital Combined Bleeding Disorders, a Comprehensive Study of a Large Number of Iranian Patients

2021 ◽  
Vol 27 ◽  
pp. 107602962199681
Author(s):  
Seyed Esmaeil Ahmadi ◽  
Mohammad Jazebi ◽  
Gholamreza Bahoush ◽  
Mohammad Reza Baghaipour ◽  
Fereydoun Ala ◽  
...  
Keyword(s):  
Assessment Tool ◽  
Care Center ◽  
Postoperative Bleeding ◽  
Reproductive Age ◽  
High Rate ◽  
Clotting Factor ◽  
Bleeding Disorders ◽  
Life Threatening ◽  
The Mean ◽  
Iranian Patients

Congenital combined bleeding disorders (CBDs) are extremely rare disorders which mainly occur in regions with a high rate of consanguineous marriage. These disorders can present with a variety of symptoms ranging from mucocutaneous bleeding to life-threatening episodes. This study aims to evaluate the prevalence and clinical course of Iranian patients with congenital CBDs. This study is conducted on 450 patients with CBDs who were referred to the Iranian Comprehensive Hemophilia Care Center (ICHCC) between 2010 and 2020. All these patients were diagnosed through evaluation of past medical history and coagulation laboratory investigation. Out of 450 patients, 33 were entered in this study. Having excluded cases with factor (F) V and FVIII deficiency, as well as those with hereditary combined Vitamin K dependent clotting factor deficiency (VKCFD), We found the most common CBDs to be FV-FVII deficiency (n: 6, 18.1%), together with FVII and FX deficiency (n: 6, 18.1%). The most common reason for referral of these patients to ICHCC was postoperative bleeding (14.3%). The mean of The International Society on Thrombosis and Hemostasis-Bleeding Assessment Tool (ISTH-BAT) and condensed MCMDM-1VWD bleeding assessment tool were 9.6 ± 4.79 and 9.1 ± 4.87, respectively (P < 0.005). In 10 females of reproductive age, the mean of Pictorial Bleeding Assessment Chart (PBAC) score was 649.3 ± 554. Among all patients, 23 (69.7%) received on-demand replacement therapy, whereas 5 patients (15.1%) received prophylaxis. In Iran, the coinheritance of bleeding disorders is surprisingly higher than expected. Moreover, patients with congenital CBDs may experience serious bleeding manifestations.

Ion Channel Remodelling in Atrial Fibrillation

2011 ◽  
Vol 7 (2) ◽  
pp. 97 ◽  
Author(s):  
Niels Voigt ◽  
Dobromir Dobrev ◽  
◽  
Keyword(s):  
Atrial Fibrillation ◽  
Ion Channel ◽  
Antiarrhythmic Drugs ◽  
Current Knowledge ◽  
Bleeding Complications ◽  
Large Size ◽  
Cardiovascular Morbidity And Mortality ◽  
Number Of Patients ◽  
Life Threatening ◽  
Underlying Mechanisms

Atrial fibrillation (AF) is the most common arrhythmia and is associated with substantial cardiovascular morbidity and mortality, with stroke being the most critical complication. Present drugs used for the therapy of AF (antiarrhythmics and anticoagulants) have major limitations, including incomplete efficacy, risks of life-threatening proarrhythmic events and bleeding complications. Non-pharmacological ablation procedures are efficient and apparently safe, but the very large size of the patient population allows ablation treatment of only a small number of patients. These limitations largely result from limited knowledge about the underlying mechanisms of AF and there is a hope that a better understanding of the molecular basis of AF may lead to the discovery of safer and more effective therapeutic targets. This article reviews the current knowledge about AF-related ion-channel remodelling and discusses how these alterations might affect the efficacy of antiarrhythmic drugs.

scholarly journals The Global Registry for Hereditary Angioedema due to C1-Inhibitor Deficiency

2021 ◽  
Author(s):  
Andrea Zanichelli ◽  
Henriette Farkas ◽  
Laurance Bouillet ◽  
Noemi Bara ◽  
Anastasios E. Germenis ◽  
...  
Keyword(s):  
Hereditary Angioedema ◽  
Rare Condition ◽  
C1 Inhibitor ◽  
C1 Inhibitor Deficiency ◽  
Therapeutic Decisions ◽  
Life Threatening ◽  
Enhance Patient Care ◽  
Electronic Support ◽  
The Impact ◽  
Global Registry

AbstractHereditary angioedema (HAE) is a rare condition, mostly due to genetic deficiency of complement C1 inhibitor (C1-INH). The rarity of HAE impedes extensive data collection and assessment of the impact of certain factors known to affect the course of this disabling and life-threatening disease. Establishing a global registry could assist to overcome such issues and provides valuable patient data from different countries. The HAE Global Registry is a disease-specific registry, with web-based electronic support, where data are provided by physicians and patients through a dedicated application. We collected data between January 1, 2018, and August 31, 2020. Data on 1297 patients from 29 centers in 5 European countries were collected. At least one attack was recorded for 497 patients during the study period. Overall, 1182 patients were diagnosed with HAE type 1 and 115 with type 2. At the time of database lock, 389 patients were taking long-term prophylactic medication, 217 of which were on danazol. Most recorded attacks affected the abdomen, were generally moderate in severity, and occurred in patients who were not on prophylactic treatment (70.6%, 6244/8848). The median duration of attacks was 780 min (IQR 290–1740) in patients on prophylactic medication and 780 min (IQR 300–1920) in patients not on continuous prophylactic medication. In conclusion, the establishment of a registry for C1-INH-HAE allowed collection of a large amount of data that may help to better understand the clinical characteristics of this disease. This information may enhance patient care and guide future therapeutic decisions.

Intraorbital Abscess: A Rare Cause of Orbit Compression

FACE ◽  
2021 ◽  
pp. 273250162110050
Author(s):  
Samuel Ruiz ◽  
Rizal Lim
Keyword(s):  
Pediatric Population ◽  
Rare Complication ◽  
Disease Process ◽  
Rare Condition ◽  
External Drainage ◽  
Direct Extension ◽  
Life Threatening ◽  
History Of ◽  
High Index Of Suspicion ◽  
Prompt Diagnosis

Introduction: Intraorbital abscess is a rare complication of rhinosinusitis that affects most commonly the pediatric population. It is thought to be caused by direct extension or venous spread of infections from contiguous sites and can lead to life-threatening complications, like permanent visual loss and cerebral abscesses. Objectives: Intraorbital abscess is a rare condition that requires prompt diagnosis and treatment to avoid serious complications. Our objectives are to provide an overview of this rare disease process and its management including our successful treatment experience. Case Description: We present a 2 case report of a 13-year-old pediatric male and a 66-year-old male with history of chronic sinusitis who presented with a right intraorbital abscess successfully treated with external drainage with decompression of the orbit. Conclusion: When intraorbital abscess is encountered, a high index of suspicion is needed to allow prompt and accurate diagnosis for this infrequent condition. Timely surgical drainage of the abscess is needed to prevent the development of fatal complications.

scholarly journals Successful Management of Acquired Hemophilia A Associated with Bullous Pemphigoid: A Case Report and Review of the Literature

2017 ◽  
Vol 2017 ◽  
pp. 1-7 ◽  
Author(s):  
Quentin Binet ◽  
Catherine Lambert ◽  
Laurine Sacré ◽  
Stéphane Eeckhoudt ◽  
Cedric Hermans
Keyword(s):  
Bullous Pemphigoid ◽  
Neutralizing Antibodies ◽  
Hemophilia A ◽  
Rare Condition ◽  
Term Treatment ◽  
Acquired Hemophilia A ◽  
Acquired Hemophilia ◽  
Short Term Treatment ◽  
Life Threatening ◽  
Dose Levels

Background. Acquired hemophilia A (AHA) is a rare condition, due to the spontaneous formation of neutralizing antibodies against endogenous factor VIII. About half the cases are associated with pregnancy, postpartum, autoimmune diseases, malignancies, or adverse drug reactions. Symptoms include severe and unexpected bleeding that may prove life-threatening.Case Study. We report a case of AHA associated with bullous pemphigoid (BP), a chronic, autoimmune, subepidermal, blistering skin disease. To our knowledge, this is the 25th documented case of such an association. Following treatment for less than 3 months consisting of methylprednisolone at decreasing dose levels along with four courses of rituximab (monoclonal antibody directed against the CD20 protein), AHA was completely cured and BP well-controlled.Conclusions. This report illustrates a rare association of AHA and BP, supporting the possibility of eradicating the inhibitor with a well-conducted short-term treatment.

scholarly journals Platelet Phenotyping and Function Testing in Thrombocytopenia

2021 ◽  
Vol 10 (5) ◽  
pp. 1114
Author(s):  
Kerstin Jurk ◽  
Yavar Shiravand
Keyword(s):  
Function Analysis ◽  
Point Of Care ◽  
Bleeding Complications ◽  
Diagnostic Significance ◽  
Blood Samples ◽  
Skilled Personnel ◽  
Life Threatening ◽  
And Function ◽  
Function Testing

Patients who suffer from inherited or acquired thrombocytopenia can be also affected by platelet function defects, which potentially increase the risk of severe and life-threatening bleeding complications. A plethora of tests and assays for platelet phenotyping and function analysis are available, which are, in part, feasible in clinical practice due to adequate point-of-care qualities. However, most of them are time-consuming, require experienced and skilled personnel for platelet handling and processing, and are therefore well-established only in specialized laboratories. This review summarizes major indications, methods/assays for platelet phenotyping, and in vitro function testing in blood samples with reduced platelet count in relation to their clinical practicability. In addition, the diagnostic significance, difficulties, and challenges of selected tests to evaluate the hemostatic capacity and specific defects of platelets with reduced number are addressed.

Painless spontaneous haemoperitoneum secondary to a uterine leiomyoma/fibroid: unusual presentation of a life-threatening differential

2021 ◽  
Vol 14 (10) ◽  
pp. e243465
Author(s):  
Chiamaka Maduanusi ◽  
Sathiyaa Balachandran ◽  
Sahathevan Sathiyathasan ◽  
Kazal Omar
Keyword(s):  
Uterine Leiomyoma ◽  
Rare Complication ◽  
Abdominal Mass ◽  
Uterine Fibroids ◽  
Reproductive Age ◽  
Haemodynamic Instability ◽  
Hypovolaemic Shock ◽  
Important Differential Diagnosis ◽  
Life Threatening ◽  
Definitive Management

This is a case of a 47-year-old woman with a spontaneous haemoperitoneum secondary to uterine leiomyomas (fibroids), an important differential diagnosis in patients with uterine fibroids and hypovolaemic shock. Uterine fibroids are very common in women of reproductive age, yet little is taught about their potential to cause hypovolaemic shock. Although it is a rare complication, given the prevalence of fibroids, it is important to bear this life-threatening differential in mind to optimise the care for these women. Presentation typically involves abdominal pain, syncope, haemodynamic instability and an intra-abdominal mass. CT of the abdomen and pelvis can be helpful in identifying the source of the haemoperitoneum, but should not delay surgery, which is the definitive management.

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