Genetic bleeding disorders form an important presentation among various genetic disorders occurring in children. A prevalence of 6 per 100,000 population has been reported in India. Bleeding disorders constitute a heterogenous group of disorders with varying clinical presentations. The common bleeding disorders are Hemophilia A and B, von Willebrand disease, and inherited thrombocytopenias. In ...

Haemophilia is a rare and congenital bleeding disorder caused by a genetic defect, resulting in a lack of or insufficient coagulation factors VIII or IX in the body. In affected individuals, this causes an inability to clot blood, leading to bleeds in the joints, muscles and soft tissues. If left untreated, this can lead to disability and sometimes death. It is widely agreed that the optimal st...

This case report presents a patient with advanced diffuse panbronchiolitis accompanied by chronic respiratory failure, marked cachexia, and refractory spontaneous pneumothorax. Because instillation of a pleurodesis agent and thoracoscopy were considered highly risky and invasive, we instead treated the patient with intravenous administration of a coagulation factor XIII concentrate, and the pne...

Inherited Factor VII deficiency is an autosomal recessive coagulation disorder with broad range of bleeding manifestations. The association between and absolute level poor. Usually, the associated FVII levels less than 1% normal value. prolongation prothrombin time only activated partial thromboplastin time. Very few pregnant women have been reported so far in English literature. We, hereby, re...

Hemophilia B is a rare blood coagulation disorder. Complications such as bleeding and hematoma can cause necrosis of flaps, wound disruption, and the disturbance of wound healing. In particular, guidelines for flap operations in hemophilia B patients have still not been defined, and case reports are rare. We reconstructed the heel of a 41-year-old male hemophilia B patient using a reverse sural...

Different mechanisms leading to disseminated intravascular coagulation (DIC) are briefly dis­cussed and the hemorrhagic diathesis which is brought about by this consumption coagulopa­thy is visualized.  Since there is an emergency need for correct management of the disorder. The problem is simplified in order, any physician, to be able to understand the syndrome and to be catious in treating t...

OBJECTIVE To implement an interactive program for teaching coagulation disorders on the World Wide Web. DESIGN AND RESULTS The core materials in this program were derived from a personal computer software program previously designed by the authors. Three modules were developed in this program: (1) a coagulation profile to display typical results of coagulation screening tests for each disorde...

background: haemophilia a (ha) is an x-linked bleeding disorder caused by the absence or reduced activity of coagulation factor viii (fviii). coagulation factors are a group of related proteins that are essential for the formation of blood clots. the aim of this study was to genotype the coagulation factor viii gene mutations using inverse shifting pcr (is-pcr) in an iranian family with severe ...

The severity of sepsis increases along with the degree of coagulation disorder, and a fulminant coagulation abnormality is recognized as disseminated intravascular coagulation (DIC). The mortality in sepsis‑associated DIC remains as high as 40%, which is comparable to that in septic shock. Even though intensive research is ongoing, there is currently no established therapy for this life‑threate...

BACKGROUND Factor VII is essential for coagulation activation by the extrinsic pathway. Hemorrhages of the central nervous system in patients with congenital factor VII deficiency seem to have a higher incidence compared with other congenital coagulopathies. The purpose of this paper is to report two rare cases of subarachnoid hemorrhage and factor VII deficiency. CASE DESCRIPTION Two cases o...