Gastrointestinal Bleeding in Congenital Bleeding Disorders

Author(s):  
Amir Samii ◽  
Mahshaad Norouzi ◽  
Abbas Ahmadi ◽  
Akbar Dorgalaleh

AbstractGastrointestinal bleeding (GIB) is serious, intractable, and potentially life-threatening condition. There is considerable heterogeneity in GIB phenotypes among congenital bleeding disorders (CBDs), making GIB difficult to manage. Although GIB is rarely encountered in CBDs, its severity in some patients makes the need for a comprehensive and precise assessment of underlying factors and management approaches imperative. Initial evaluation of GIB begins with assessment of hematological status; GIB should be ruled out in patients with chronic anemia, and in presentations that include hematemesis, hematochezia, or melena. High-risk patients with recurrent GIB require urgent interventions such as replacement therapy for treatment of coagulation factor deficiency (CFD). However, the best management strategy for CFD-related bleeding remains controversial. While several investigations have identified CBDs as potential risk factors for GIB, research has focused on assessing the risks for individual factor deficiencies and other CBDs. This review highlights recent findings on the prevalence, management strategies, and alternative therapies of GIB related to CFDs, and platelet disorders.

Medicines ◽  
2021 ◽  
Vol 8 (4) ◽  
pp. 16
Author(s):  
Gabriele Savioli ◽  
Iride Francesca Ceresa ◽  
Luca Caneva ◽  
Sebastiano Gerosa ◽  
Giovanni Ricevuti

Coagulopathy induced by major trauma is common, affecting approximately one-third of patients after trauma. It develops independently of iatrogenic, hypothermic, and dilutive causes (such as iatrogenic cause in case of fluid administration), which instead have a pejorative aspect on coagulopathy. Notwithstanding the continuous research conducted over the past decade on Trauma-Induced Coagulopathy (TIC), it remains a life-threatening condition with a significant impact on trauma mortality. We reviewed the current evidence regarding TIC diagnosis and pathophysiological mechanisms and summarized the different iterations of optimal TIC management strategies among which product resuscitation, potential drug administrations, and hemostatis-focused approaches. We have identified areas of ongoing investigation and controversy in TIC management.


2017 ◽  
Vol 11 (2) ◽  
pp. 284-292 ◽  
Author(s):  
Mohammed Al-Dugdugi ◽  
Abdulhameed Alhazmi ◽  
Abdulhadi Khaliel ◽  
Luis Perez

Bochdalek hernia (BH) can be a life-threatening condition in infants. Approximately 85.3% of newborns with a BH are immediately at high risk and have a high mortality rate due to respiratory insufficiency [Kocakusak et al.: Hernia 2005;9: 284–287]. However, BH is almost asymptomatic in adults and discovered only incidentally [Wilkins et al.: Clin Imaging 1994;18: 224–229]. Complicated BH in adults might present with visceral incarceration and lethal complications. Upper gastrointestinal bleeding and acute pancreatitis are rarely reported in the literature as complications of BH in adults. Here we report the case of a 42-year-old male who presented with upper gastrointestinal bleeding and acute pancreatitis. He was found to have abdominal visceral organ herniation to the posterior right thoracic cavity. His diagnosis was achieved early and with a close follow-up, we succeeded in stabilizing the patient’s condition. Then he was subjected to reconstructive thoracotomy for hernial repair and restoring abdominal viscera.


2007 ◽  
Vol 98 (09) ◽  
pp. 530-542 ◽  
Author(s):  
Kenneth Segers ◽  
Björn Dahlbäck ◽  
Gerry Nicolaes

SummaryHuman coagulation factor V (FV) is an essential coagulation protein with functions in both the pro- and anticoagulant pathways. Failure to express and control FV functions can either lead to bleeding, or to thromboembolic disease. Both events may develop into a life-threatening condition. Since the first description of APC resistance, and in particular the description of the so-called factor VLeiden mutation, in which a prominent activated protein C cleavage site in FV has been abolished through a mutation in the FV gene, FV has been in the center of attention of thrombosis research. In this review we describe how the functions of FV are expressed and regulated and provide an extensive description of the role that FV plays in the etiology of thromboembolic disease.


2019 ◽  
Vol 2019 (12) ◽  
Author(s):  
Awadh AlQahtani ◽  
Mohammed N Alali ◽  
Safaa Allehiani

Abstract Acute pancreatitis (AP) is a serious life-threatening condition, especially in high-risk patients. Limited data exist regarding early and late complications of immunosuppressant drugs as a cause of pancreatitis, especially Tacrolimus and Mycophenolate Mofetil (MMF), after transplantation. We report a rare case of late AP secondary to immunosuppressants’ synergistic effect after 5 years of renal transplant in an obese patient on day 4 after laparoscopic sleeve gastrectomy (LSG). We present a 41-year-old Saudi obese male patient, known to have multiple comorbidities and a renal transplantation twice, was on multiple medications, including Prednisolone, MMF and Tacrolimus for 5 years. On day 4 post-LSG, he developed severe epigastric abdominal pain and was diagnosed to have AP as a late complication of immunosuppressants’ synergistic effect. LSG is a common safe procedure, but complications related to medical illnesses or medications can occur. Careful review and sufficient knowledge of early and late complications are required.


2020 ◽  
Vol 2 (1) ◽  
pp. 64-68
Author(s):  
Melissa Hearrell ◽  
Aikaterini Anagnostou

Anaphylaxis is a serious allergic reaction that is rapid in onset and may be life threatening. An informal review of the literature was performed in a nonsystematic way for this article. Key published work was identified and included. The incidence and prevalence of anaphylaxis have increased over time. Anaphylaxis is unpredictable and can be the result of various allergic triggers, including food, insect venom, and medication. In children, the most frequent trigger is food. The diagnosis is based on clinical criteria. After confirmation of the relevant allergen trigger, prevention occurs through strict avoidance of the allergen and optimal management of existing comorbidities. Patients with anaphylaxis require immediate assessment and treatment. The management of a patient with anaphylaxis should start with the removal of exposure to the known or suspected trigger, followed by the assessment of the patient’s circulation, airway patency, breathing, and mental status. The administration of epinephrine at a dose of 0.01 mg/kg (1:1000) intramuscularly is the first-line treatment for anaphylaxis, and there are no absolute contraindications to this treatment. The maximum single dose of epinephrine is 0.5 mg and may be repeated after 5‐10 minutes if needed. After administration of epinephrine, patients with anaphylaxis should be placed supine with their lower limbs elevated. They should not be placed in the upright position. Studies of fatal and near-fatal allergic reactions identified potential risk factors for fatalities such as asthma, peanuts and/or tree nuts, and delayed epinephrine use, and provided important information that may help minimize the future risk. Patients and their families need to be well educated on how to manage potential anaphylactic reactions with training in the use of epinephrine autoinjectors and personalized emergency management plans. Health care professionals must be familiar with this clinical emergency and able to respond to anaphylaxis in a timely and appropriate manner.


2017 ◽  
Vol 08 (04) ◽  
pp. 202-204
Author(s):  
Vipul D. Yagnik

ABSTRACTDieulafoy’s lesion is a rare but potentially a life-threatening condition. It accounts for 1%–2% of acute gastrointestinal (GI) bleeding. The lesion is most frequently located in the stomach and may be located anywhere in the alimentary tract. It can be present as severe GI bleeding or chronic GI blood loss. The cause of lesion remains uncertain. The range of clinical presentation varies from acute ill hospitalized patients as well as in the newborn.


2014 ◽  
Vol 96 (5) ◽  
pp. e31-e33 ◽  
Author(s):  
R Bini ◽  
A Addeo ◽  
L Maganuco ◽  
D Fontana ◽  
T Viora ◽  
...  

Mucormycosis is a life threatening condition caused by invasion of fungi of the order Mucorales. Gastrointestinal invasion is very rare and often lethal, particularly in disseminated mucormycosis. We present the case of a 26-year-old woman from North Africa with type 2 diabetes who, after a cholecystectomy, developed unexplained septic shock and haematemesis due to gastric necrosis. Computed tomography (CT) revealed a disseminated fungal invasion of the lungs, kidney and paranasal sinuses. A gastrectomy and subsequent amphotericin B treatment resolved her condition. The number of patients with mucormycosis is increasing. Early diagnosis of high risk patients with CT and biopsies from which fungi are directly isolated must be followed by surgery and systemic amphotericin B infusion.


Blood ◽  
2011 ◽  
Vol 117 (23) ◽  
pp. 6091-6099 ◽  
Author(s):  
Lawrence Tim Goodnough ◽  
Aryeh Shander

Abstract Intracerebral hemorrhage in patients with warfarin-associated coagulopathy is an increasingly common life-threatening condition that requires emergent management. The evolution of therapeutic options in this setting, as well as recently published guidelines, has resulted in some heterogeneity in recommendations by professional societies. This heterogeneity can be attributed to lack of evidence-based support for plasma therapy; the variability in availability of prothrombin complex concentrates; the variability in the coagulation factor levels and contents of prothrombin complex concentrates; ambiguity about the optimal dose and route of administration of vitamin K; and the lack of standardized clinical care pathways, particularly in community hospitals, for the management of these critical care patients. In this review, we summarize the relevant literature about these controversies and present recommendations for management of patients with warfarin-associated coagulopathy and intracerebral hemorrhage.


2018 ◽  
Author(s):  
Franco van Wyk ◽  
Anahita Khojandi ◽  
Robert L. Davis ◽  
Rishikesan Kamaleswaran

AbstractRationale: Sepsis is a life-threatening condition with high mortality rates and expensive treatment costs. To improve short- and long-term outcomes, it is critical to detect at-risk sepsis patients at an early stage.Objective: Our primary goal was to develop machine learning models capable of predicting sepsis using streaming physiological data in real-time.Methods: A dataset consisting of high-frequency physiological data from 1,161 critically ill patients admitted to the intensive care unit (ICU) was analyzed in this IRB-approved retrospective observational cohort study. Of that total, 634 patients were identified to have developed sepsis. In this paper, we define sepsis as meeting the Systemic Inflammatory Response Syndrome (SIRS) criteria in the presence of the suspicion of infection. In addition to the physiological data, we include white blood cell count (WBC) to develop a model that can signal the future occurrence of sepsis. A random forest classifier was trained to discriminate between sepsis and non-sepsis patients using a total of 108 features extracted from 2-hour moving time-windows. The models were trained on 80% of the patients and were tested on the remaining 20% of the patients, for two observational periods of lengths 3 and 6 hours.Results: The models, respectively, resulted in F1 scores of 75% and 69% half-hour before sepsis onset and 79% and 76% ten minutes before sepsis onset. On average, the models were able to predict sepsis 210 minutes (3.5 hours) before the onset.Conclusions: The use of robust machine learning algorithms, continuous streams of physiological data, and WBC, allows for early identification of at-risk patients in real-time with high accuracy.


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