[Children with hemorrhagic diathesis: correct diagnostic and therapeutic approach].
Bleeding defects are of great interest in pediatrics since the prevalence of congenital forms and the early appearance of acquired ones. The pathology itself and the therapy indeed can often interfere with the growing-up patients. Bleeding defects have been identified with an heterogeneous group of clinical disease that differs from one another in etiology, pathogenesis, epidemiology and incidence in population. Bleeding diathesis is the common symptom: bleeding tendency may be mild, moderate or severe, localized or generalized, cutaneous or mucosal, superficial or deep. Bleeding disorders may be classified as a) defects in the primary haemostasis, which include quantitative and qualitative abnormalities of platelets and vascular disorders and b) defects in secondary haemostasis, which include intravascular disorders (blood coagulation). Careful history and clinical examination are essential in diagnosis of bleeding disorders. History of patient should be taken a) to differentiate acquired from congenital disease and to know the way of hereditary transmission (family history); b) to know exactly the disease's start and the mutual relation with former or accompanying disease; c) mutual relation with drugs token. Subsequently a careful physical examination should be done. A specific hemorrhagic diathesis has been seen with a deficiency of primary or secondary haemostasis. A deficient or late haemostatic plug in small vessels can cause superficial, interstitial bleeding that may be intracutaneous or intramucosal and is called purpura. In coagulation factor deficiency the haemostatic plug cannot be consolidated by fibrin: spontaneous hematomas, hemarthrosis and ecchymoses often occurs. The initial laboratory work up for screening patients with bleeding disorders should include first step tests to differentiate bleeding disorders for bone-marrow malignancies; from virus infections carrying screening of major viruses and from hepatic diseases. Second step laboratory examination includes a) platelet count or estimation of platelet number on blood smear; b) bleeding time to test small vessel integrity and platelet function; c) aPTT, PT, AP to measure clotting activity; d) fibrinogen determination. With this battery of screening test it is usually possible to determine the general area of the defect (abnormalities of platelets number or function or congenital defect of one or more clotting factors activity). Acute idiopathic thrombocytopenic purpura is the most common bleeding disorders in childhood. Usually no therapy may be required no matter platelet count. Patients with a significant hemorrhagic tendency are treated either with prednisone (2 mg/kg orally in divided daily doses) for a period of 2 weeks or with a 5 days course of special polyvalent intact immunoglobulin (400 mg/kg/die) for intravenous use.(ABSTRACT TRUNCATED AT 400 WORDS)
Carnelli
V
V
Clinica Pediatrica I, Università degli Studi di Milano, Italia.
Dozzi
M
M
Gibelli
M
M
Giovanniello
A
A
Riva
F
F
Seidita
C
C
Stucchi
C
C
D000293:Adolescent; D002648:Child; D002675:Child, Preschool; D006403:Hematologic Tests; D006467:Hemophilia A / Q000628:therapy; D006474:Hemorrhagic Disorders / Q000097:blood / Q000175:diagnosis / Q000628:therapy; D006487:Hemostasis; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D011696:Purpura, Thrombocytopenic / Q000628:therapy
Il bambino con diatesi emorragica: corretta impostazione diagnostica e terapeutica.