Hemorrhagic Disorders ; genetics ; Humans

No abstract available.
Diagnosis* ; Hemorrhagic Disorders*

Bruising and bleeding are common events in children. The pediatrician must be able to determine whether a child's symptoms are normal or perhaps indicative of hemorrhagic disorders. A thorough medical history and physical examination should enable the pediatricians to identify those patients warranting further evaluation. This review describes the characteristics of the medical history, physical examination, and clinical laboratory testing that are important in recognizing clinically significant bleeding disorders. This review will help the pediatrician to perform the initial laboratory evaluation, differentiate for patients with bleeding tendency and make the correct diagnosis for a variety of cases.
Child ; Diagnosis ; Diagnosis, Differential* ; Hemorrhage ; Hemorrhagic Disorders* ; Humans ; Physical Examination

In the year 2000, 443 samples of human serum and 131 samples of serum of rodents were assayed by ELISA. Results showed that there were 2.0% of human sera and 2.0% of rodent sera positive with hantaan virus. These preliminary results suggested that Hantaan virus can be the causal factor of hemorrhagic fever in human and in rat
Hantaan virus ; Enzyme-Linked Immunosorbent Assay ; Hemorrhagic Disorders

Cytophagic histiocytic panniculitis is a histiocytic disorder which is characterized by recurrent subcutaneous nodules, fever, pancytopenia, and abnormal hepatocyte function. Most patients had systemic involvement with hepatosplenomegaly and pancytopenia and, after a chronic course, usually developed a hemorrhagic diathesis that led to death. Rarely reported cases were shown to have had a non-fatal course. We report herein a case of cytophagic histiocytic panniculitis in the trunk and both upper arms of 34-year-old woman who had a benign course and also showed histopatholoigcally lipomembranous change in the subcutaneous lesion.
Adult ; Arm ; Female ; Fever ; Hemorrhagic Disorders ; Hepatocytes ; Humans ; Pancytopenia ; Panniculitis*

T Hemoptysis is a common complication of bronchiectasis, but concurrent primary hyperfibrinolysis is very rare and easily gives rise to missed diagnosis. Patients with bronchiectasis complicated by primary hyperfibrinolysis do not respond to regular treatment for hemoptysis, which may potential cause fatal consequences. We report two cases of bronchiectasis combined with primary hyperfibrinolysis. Both of the patients had a history of bronchiectasis and were admitted for intermittent hemoptysis. Conventional treatment such as vasopressin failed to produce a favorable response. The eventual definite diagnosis of primary hyperfibrinolysis was established based on laboratory examinations. After diagnosis, the patients received fibrinogen and cryoglobulin to maintain a fibrinogen level over 1.5 g/L. Both of the patients subsequently showed improved conditions and were discharged.
Aged ; Bronchiectasis ; complications ; Hemorrhagic Disorders ; complications ; Humans ; Male

Cytophagic histiocytic panniculitis(CHP) is a histiocytic disorder that was first described by Winkelmann and Crotty in 1980. We have recently experienced a case of CHP is a 21-year-old female who had developed recurrent fever and erythematous tender subcutaneous nodules, progressing to liver dysfunction and hemorrhagic diathesis. Histopathologically, infiltrates of large cytophagic histiocytes lacking atypia were present in the subcutis. Hemophagocytic histiocytes were observed in the bone marrow. Immunohistochemical studies were performed, and revealed positive immunoreactivity for lysozyme and T cell marker in the subcutaneous inflammatory lesions.
Bone Marrow ; Female ; Fever ; Hemorrhagic Disorders ; Histiocytes ; Humans ; Liver Diseases ; Muramidase ; Panniculitis* ; Young Adult

Numerous advances have been applied for the management of hemophiliac patients and the life expectancy of them was markedly improved. But they have many problems yet such as transmission of infections, adverse reactions to plasma products, development of factor VIII inhibitor and inadequate coagulation in the medical and/or surgical treatment. Anesthetic care should be managed with gentle, atraumatic intubation and careful positioning of patient pointing to the prevention of bleeding from hemorrhagic diathesis. We present a case of hemophilia A who had in operation for olecranon fracture of left ulna. It is important that anesthesiologist should realize that certain pathophysiological changes, especially coagulation disorders, might be occurred during surgical procedures and that perioperative preparation and management for them should be kept well.
Anesthesia, General ; Factor VIII ; Hemophilia A* ; Hemorrhage ; Hemorrhagic Disorders ; Humans ; Intubation ; Life Expectancy ; Olecranon Process ; Plasma ; Ulna

OBJECTIVEThe study was to understand the incidence of traumatic coagulopathy and the clinical blood transfusion in hospitalized trauma patients so as to provide a reference for guiding scientific component transfusion in trauma or surgical patients.

METHODSBy using a software "clinical transfusion database" developed by our department, 1 766 trauma cases who suffered traumatic injury and required hospital admission between 2001 and 2012 were retrieved, and out of them 1 211 patients were given transfusion, and the transfusion-related indicators of the patients such as coagulation, hemoglobin levels before transfusion, trauma situation, massive blood transfusion and total blood transfusion were retrospectively analyzed. According total volume of blood usage during hospitalization,1 211 cases with transfusion were divided into three groups: low volume transfusion group ( ≤ 5 U, n = 471), moderate volume transfusion group (5-10 U, n = 449) and high volume transfusion group (>10 U, n = 291), then the difference of indicators among the 3 groups was compared, and the risk factors of high volume transfusion were analyzed.

RESULTSThere were 33 cases of coagulopathy and 52 cases of massive transfusion in trauma patients with transfusion. The transfusion rate of trauma patients was about 68.6%. There was no association between the total amount of blood transfusion and surgical grade or whether surgery. The most patients were transfused using two components (plasma and red blood cell), the ratio of plasma to RBC transfused in patients with coagulopathy was approximately 1.0. In high volume transfusion group, there were more younger and male patients with more serious injury, their infection and death were significantly higher than that in other two groups (P < 0.01).

CONCLUSIONThere were approximately 69% of hospitalized trauma patients require transfusion, the patients in high volume transfusion group have two populations such as middle-aged and young men who was vulnerable to severe trauma mainly caused by accident injury or fall injury and older women who was vulnerable to osteoporotic hip fractures mainly caused by fall injuries. The coagulation disorders in the patients with trauma coagulopathy should be corrected by transfusion with high ratios of plasma to RBC. Massive transfusion (OR = 95.22), hemorrhagic shock (OR = 17.2), trauma coagulopathy (OR = 4.52) are risk factors of high volume transfusion > 10 U, and massive transfusion also is a risk factor of trauma coagulopathy (OR = 16.257). The routine dynamic monitoring of coagulation should be performed for trauma or surgical patients to guide the clinical transfusion scientifically.

We describe a case of typical cytophagic histiocytic panniculitis occuring in 10-year-old male. He had recurrent subcutaneous nodules, fever, pancytopenia and abnormal liver function tests since 5 months of his age, and recently showed hemorrhagic diathesis. The histologic picture showed lobular histiocytic panniculitis with "bean bag" cytophagic cells.
Child ; Fever ; Hemorrhagic Disorders ; Humans ; Liver Function Tests ; Male ; Pancytopenia ; Panniculitis*