scholarly journals Intracranial Hemorrhage in Patients With Hemophilia A

2020 ◽  
Vol 2 (2) ◽  
pp. 59
Author(s):  
Nugroho Setia Budi ◽  
Prananda Surya Airlangga ◽  
Bambang Pujo Semedi
Keyword(s):  
Intracranial Hemorrhage ◽  
Hemophilia A ◽  
Medical Emergency ◽  
Bleeding Disorders ◽  
Target Level ◽  
Successful Management ◽  
Appropriate Treatment ◽  
Critical Bleeding ◽  
Life Threatening ◽  
Day By Day

Introduction: Intracranial hemorrhage in inherited bleeding disorders is a medical emergency. The location of bleeding in most children is subdural and the most common cause is hemophilia. Although intracranial bleeding that occurs in people with hemophilia ranges from less than 5% of events, it is a life-threatening medical emergency so appropriate treatment is needed. Case Report: A boy patient 11 years old, 20kg weights have a seizure at home and followed by a decrease in consciousness. It was founded abnormalities in the form of anemia, prolonged FH (PPT 4x and APTT 4x), and hypocalcemia. The patient then was given main therapy; FVIII 100 IU/dL according to the FVIII target level calculated. The therapy continued with 500IU/12 hours according to the daily target of FVIII 50IU/dL. Discussion: The patient’s condition was getting better day by day. The patient’s consciousness started to improve after 14 days of postoperative. One month after that, the patient received koate treatment as the episodic handler. Diagnosing the exact cause in patients who have intracranial hemorrhage provides appropriate management so that the patients could be helped. Conclusion: Good collaboration between anesthesiologists, neurosurgeons, and pediatrics will increase the probability of successful management of critical bleeding without major sequelae.

scholarly journals Intracranial Hemorrhage in Patients With Hemophilia A

2020 ◽  
Vol 2 (2) ◽  
pp. 22
Author(s):  
Nugroho Setia Budi ◽  
Prananda Surya Airlangga ◽  
Bambang Pujo Semedi
Keyword(s):  
Intracranial Hemorrhage ◽  
Hemophilia A ◽  
Medical Emergency ◽  
Bleeding Disorders ◽  
Target Level ◽  
Successful Management ◽  
Appropriate Treatment ◽  
Critical Bleeding ◽  
Life Threatening ◽  
Day By Day

Introduction: Intracranial hemorrhage in inherited bleeding disorders is a medical emergency. The location of bleeding in most children is subdural and the most common cause is hemophilia. Although intracranial bleeding that occurs in people with hemophilia ranges from less than 5% of events, it is a life-threatening medical emergency so appropriate treatment is needed. Case Report: A boy patient 11 years old, 20kg weights have a seizure at home and followed by a decrease in consciousness. It was founded abnormalities in the form of anemia, prolonged FH (PPT 4x and APTT 4x), and hypocalcemia. The patient then was given main therapy; FVIII 100 IU/dL according to the FVIII target level calculated. The therapy continued with 500IU/12 hours according to the daily target of FVIII 50IU/dL. Discussion: The patient’s condition was getting better day by day. The patient’s consciousness started to improve after 14 days of postoperative. One month after that, the patient received koate treatment as the episodic handler. Diagnosing the exact cause in patients who have intracranial hemorrhage provides appropriate management so that the patients could be helped. Conclusion: Good collaboration between anesthesiologists, neurosurgeons, and pediatrics will increase the probability of successful management of critical bleeding without major sequelae.

Intracranial Hemorrhage: A Devastating Outcome of Congenital Bleeding Disorders—Prevalence, Diagnosis, and Management, with a Special Focus on Congenital Factor XIII Deficiency

2017 ◽  
Vol 44 (03) ◽  
pp. 267-275 ◽  
Author(s):  
Seyed Alavi ◽  
Masumeh Jalalvand ◽  
Vahideh Assadollahi ◽  
Shadi Tabibian ◽  
Akbar Dorgalaleh
Keyword(s):  
Replacement Therapy ◽  
Intracranial Hemorrhage ◽  
Von Willebrand Disease ◽  
Morbidity And Mortality ◽  
High Dose ◽  
Special Focus ◽  
Bleeding Disorders ◽  
Secondary Damage ◽  
Platelet Function Disorders ◽  
Life Threatening

AbstractIntracranial hemorrhage (ICH) is a medical emergency. In congenital bleeding disorders, ICH is a devastating presentation accompanied with a high rate of morbidity and mortality. The prevalence of ICH is highly variable among congenital bleeding disorders, with the highest incidence observed in factor (F) XIII deficiency (FXIIID) (∼30%). This life-threatening presentation is less common in afibrinogenemia, FVIII, FIX, FVII, and FX deficiencies, and is rare in severe FV and FII deficiencies, type 3 von Willebrand disease and inherited platelet function disorders (IPFDs). In FXIIID, this diathesis most often occurs after trauma in children, whereas spontaneous ICH is more frequent in adults. About 15% of patients with FXIIID and ICH die; the bleeding causes 80% of deaths in this coagulopathy. Although in FXIIID, the bleed most commonly is intraparenchymal (> 90%), epidural, subdural, and subarachnoid hemorrhages also have been reported, albeit rarely. As this life-threatening bleeding causes neurological complications, early diagnosis can prevent further expansion of the hematoma and secondary damage. Neuroimaging plays a crucial role in the diagnosis of ICH, but signs and symptoms in patients with severe FXIIID should trigger replacement therapy even before establishment of the diagnosis. Although a high dose of FXIII concentrate can reduce the rate of morbidity and mortality of ICH in FXIIID, it may occasionally trigger inhibitor development, thus complicating ICH management and future prophylaxis. Nevertheless, replacement therapy is the mainstay of treatment for ICH in FXIIID. Neurosurgery is performed in patients with FXIIID and epidural hematoma and a hemorrhage diameter exceeding 2 cm or a volume of ICH is more than 30 cm3. Contact sports are not recommended in people with FXIIID as they can elicit ICH. However, a considerable number of safe sports and activities have been suggested to have more benefits than dangers for patients with congenital bleeding disorders, and are hence suitable for these patients.

A Review on the Use of Reversal Agents of Direct Oral Anticogulant Drugs in Case of Gastrointestinal Bleeding

2020 ◽  
Vol 15 ◽  
Author(s):  
Veronica Ojetti ◽  
Angela Saviano ◽  
Mattia Brigida ◽  
Luisa Saviano ◽  
Alessio Migneco ◽  
...  
Keyword(s):  
Gastrointestinal Bleeding ◽  
Major Bleeding ◽  
Oral Anticoagulants ◽  
Direct Oral Anticoagulants ◽  
Medical Emergency ◽  
Management Approach ◽  
Emergency Setting ◽  
Reversal Agents ◽  
Life Threatening ◽  
Andexanet Alfa

Background : Major bleeding is a life-threatening condition and a medical emergency with high mortality risk. It is often the complication of anticoagulant’s intake. Anticoagulants are commonly used for the prevention and the treatment of thrombotic events. The standard therapy with vitamin K antagonist (warfarin) has been frequently replaced by direct oral anticoagulants (DOACs). The latter agents (rivaroxaban, apixaban, edoxaban, dabigatran, betrixaban) showed a better efficacy and safety compared to standard warfarin treatment and they are recommended for the reduction of ischemic stroke. Literature data reported a high risk of gastrointestinal bleeding with DOACs, in particular with dabigatran and rivaroxaban. In case of life-threatening gastrointestinal bleeding, these patients could benefit from the use of reversal agents. Methods: We performed an electronic search on PUBMED of the literature concerning reversal agents for DOACs and gastrointestinal bleeding in the Emergency Department from 2004 to 2020. AIM: This review summarizes the current evidences about three reversal agents idarucizumab, andexanet alfa and ciraparantag, and the use of the first two in the emergency setting in patients with an active major bleeding or who need urgent surgery to offer physicians indications for a better management approach in order to increase patient’s safety. Conclusion: Although these agents have been marketed for five years (idarucizumab) and two years (andexanet alfa) respectively, and despite guidelines considering antidotes as first-line agents in treating life-threatening hemorrhage when available, these antidotes seem to gain access very slowly in the clinical practice. Cost, logistical aspects and need for plasma level determination of DOAC for an accurate therapeutic use probably have an impact on this phenomenon.. An expert multidisciplinary bleeding team should be established so as to implement international guidelines based on local resources and organization.

Of Love and Laetrile: Medical Decision Making in a Child's Best Interests

1979 ◽  
Vol 5 (3) ◽  
pp. 269-294
Author(s):  
Eve T. Horwitz
Keyword(s):  
Decision Making ◽  
Medical Treatment ◽  
Burden Of Proof ◽  
State Intervention ◽  
Convincing Evidence ◽  
State Agency ◽  
Best Interests ◽  
Medical Decision ◽  
Appropriate Treatment ◽  
Life Threatening

AbstractTwo recent cases have raised important questions concerning the appropriateness of state intervention in parental choices of unorthodox medical treatment for children with life-threatening conditions. This Note first discusses whether, and if so, when, state intervention in a child's treatment selection by its parents is appropriate, and then analyzes the tests a court should apply in deciding upon an appropriate treatment. The Note recommends a decision-making approach that requires the appropriate state agency to prove, by clear and convincing evidence, that the parents' choice of medical treatment either is directly or is indirectly harming their child. Under this approach, if the state meets its burden of proof the court then must apply the ‘best interests’ test, rather than the ‘substituted judgment’ test, to choose an appropriate medical treatment for the child.

Acute pericardial tamponade: The initial manifestation of systemic lupus erythematosus with Graves’ hyperthyroidism

Lupus ◽  
2021 ◽  
pp. 096120332110047
Author(s):  
Muming Yu ◽  
Yulei Gao ◽  
Heng Jin ◽  
Songtao Shou
Keyword(s):  
Myocardial Infarction ◽  
Systemic Lupus Erythematosus ◽  
Female Patient ◽  
Lupus Erythematosus ◽  
Pericardial Tamponade ◽  
Medical Emergency ◽  
Systemic Lupus ◽  
Initial Manifestation ◽  
Life Threatening ◽  
Obstructive Shock

Acute pericardial tamponade, which can cause obstructive shock, is a serious life-threatening medical emergency that can be readily reversed by timely identification and appropriate intervention. Acute pericardial tamponade can occur for a number of reasons, including idiopathic, malignancy, uremia, iatrogenic, post-myocardial infarction, infection, collagen vascular, hypothyroidism, and others. Systemic lupus erythematosus (SLE) and hyperthyroidism associated with pericardial tamponade are rarely reported. Here, we report the case of a 20-year-old female patient was final diagnosed of SLE with Graves’ hyperthyroidism.

Pyriform sinus rupture caused by blunt trauma

2020 ◽  
pp. 197140092097516
Author(s):  
Emil Jernstedt Barkovich ◽  
M Reza Taheri
Keyword(s):  
Blunt Trauma ◽  
Pyriform Sinus ◽  
Significant Heterogeneity ◽  
Cervical Trauma ◽  
Specific Location ◽  
Appropriate Treatment ◽  
Threatening Condition ◽  
Pharyngeal Perforation ◽  
Life Threatening ◽  
Foreign Body Impaction

Hypopharyngeal perforation (HP) is a potentially life-threatening condition most associated with iatrogenic injury and foreign body impaction. Additionally, a number of cases of posterior HP have been reported following blunt cervical trauma. We present a case of a construction accident causing lateral hypopharyngeal rupture. Visceral perforation was initially diagnosed on computed tomography (CT) imaging and managed conservatively. We speculate this region may be particularly vulnerable to injury due to an anatomic transition in adjacent fascial support. A review of 29 prior cases suggests that this may be the first reported case of blunt trauma causing rupture of the pyriform sinus. However, significant heterogeneity exists in diagnostic approach. Radiography and CT are rapid, sensitive modalities for suggesting pharyngeal perforation, while fluoroscopy and endoscopy can better assess injury size and location and monitor resolution. Early radiologic recognition of hypopharyngeal injury is essential to initiate appropriate treatment. In certain cases, including our own, both the presence and specific location of perforation may be identified on initial CT images.

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 Myelodysplastic Syndrome Clinically Presenting with the “Classic TTP Pentad”

2017 ◽  
Vol 2017 ◽  
pp. 1-6 ◽  
Author(s):  
Santiago Fabián Moscoso Martínez ◽  
Evelyn Carolina Polanco Jácome ◽  
Elizabeth Guevara ◽  
Vijay Mattoo
Keyword(s):  
Myelodysplastic Syndrome ◽  
Clinical Presentation ◽  
Renal Involvement ◽  
Medical Emergency ◽  
Complete Blood Cell Count ◽  
Blood Cell Count ◽  
Thrombocytopenic Purpura ◽  
Life Saving ◽  
Threatening Condition ◽  
Life Threatening

The clinical presentation of myelodysplastic syndrome (MDS) is not specific. Many patients can be asymptomatic and can be detected only due to an abnormal complete blood cell count (CBC) on routine exam or for other reasons while others can be symptomatic as a consequence of underlying cytopenias. Thrombotic thrombocytopenic purpura (TTP) usually is suspected under the evidence of microangiopathic hemolytic anemia (MAHA) and thrombocytopenia and because it is a life-threatening condition (medical emergency) immediate initiation of plasmapheresis could be life-saving. The following case illustrates an unusual presentation of MDS in a patient who came in to the emergency room with the classic TTP “pentad” of fever, renal involvement, MAHA, mental status changes, and thrombocytopenia. We will focus our discussion in the clinical presentation of this case.

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