No abstract available.
Purpura ; Purpura, Thrombocytopenic, Idiopathic

Objective: To report the clinical manifestations and laboratory features of five patients with congenital thrombotic thrombocytopenic purpura (cTTP) and explore its standardized clinical diagnosis and treatment along with a review of literature. Methods: Clinical data of patients, such as age of onset, disease manifestation, personal history, family history, and misdiagnosed disease, were collected. Treatment outcomes, therapeutic effects of plasma infusion, and organ function evaluation were observed. The relationship among the clinical manifestations, treatment outcomes, and ADAMTS13 gene mutation of patients with cTTP was analyzed. Additionally, detection of ADAMTS13 activity and analysis of ADAMTS13 gene mutation were explored. Results: The age of onset of cTTP was either in childhood or adulthood except in one case, which was at the age of 1. The primary manifestations were obvious thrombocytopenia, anemia, and different degrees of nervous system involvement. Most of the patients were initially suspected of having immune thrombocytopenia. Acute cTTP was induced by pregnancy and infection in two and one case, respectively. ADAMTS13 gene mutation was detected in all cases, and there was an inherent relationship between the mutation site, clinical manifestations, and degree of organ injury. Therapeutic or prophylactic plasma transfusion was effective for treating cTTP. Conclusions: The clinical manifestations of cTTP vary among individuals, resulting in frequent misdiagnosis that delays treatment. ADAMTS13 activity detection in plasma and ADAMTS13 gene mutation analysis are important bases to diagnose cTTP. Prophylactic plasma transfusion is vital to prevent the onset of the disease.
Female ; Pregnancy ; Humans ; Adult ; Blood Component Transfusion ; Plasma ; Purpura, Thrombotic Thrombocytopenic/therapy* ; Mutation ; Purpura, Thrombocytopenic, Idiopathic ; ADAMTS13 Protein/therapeutic use*

No abstract available.
Pregnancy* ; Purpura, Thrombotic Thrombocytopenic*

No abstract available.
Purpura, Thrombotic Thrombocytopenic*

No abstract available.
Plasmapheresis* ; Prednisolone* ; Purpura, Thrombotic Thrombocytopenic*

No abstract available.
Purpura, Thrombocytopenic, Idiopathic*

No abstract available.
Purpura, Thrombocytopenic, Idiopathic*

No abstract available.
Methylprednisolone* ; Purpura, Thrombocytopenic, Idiopathic*

No abstract available.
Hemorrhage* ; Purpura, Thrombocytopenic, Idiopathic*

Background: Idiopathic or Immune Thrombocytopenic Pupura (ITP) is a common disease in Vietnamese children. This is a hemostatic disorder disease diagnosed by clinical symptoms combining with tests of platelet quantity in peripheral blood and other tests. Objectives: to study epidemiology, clinical, para-clinical characteristics and their relations in ITP disease. Subjects and methods: This was a prospective and retrospective study. The study included 579 pediatric patients from 3 months to 16 ages who were diagnosed and treated ITP disease in National Pediatric Hospital from 1/1/2003 to 12/31/2004. Results: Data were analyzed, including 191 infants from >3-<12 months (33.0%), 293 children from 1 - 10 ages (50.6%), 95 children >10-<16 ages (16.4%). The male/female ratio was highest in infants and decreased with age (P < 0.001). The disease occurred more in September, October and November with advantage factor being acute respiratory infection before 2 to 3 weeks. Subcutaneous hemorrhage ratio was highest with 557 cases (96.2%), intracranial hemorrhage happened in 7 cases (1.7%). There were differences among age groups about hemorrhage situation. Anemia level did not corresponding with the decrease of the platelet count. Conclusion: Characteristics of ITP depend on the number of platelet and age. \r\n', u'\r\n', u'\r\n', u'
Purpura ; Thrombocytopenic ; Idiopathic/ epidemiology