[Children with hemorrhagic diathesis: correct diagnostic and therapeutic approach]. / Il bambino con diatesi emorragica: corretta impostazione diagnostica e terapeutica.

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)

[Treatment of hemophilia in children]. / Il trattamento dell'emofilia in età pediatrica.

Thanks to recent developments and evolution in prenatal diagnosis and early onset within the first year of life, hemophilia may now be considered a pathology of primarily pediatric interest. The treatment of hemophilia in children has furthermore undergone a number of changes that include 2 main events in therapy that have served to modify the quality of life of the hemophiliac. The first of these events regards blood products and the prevention of viral infections, hepatitis and HIV transmission. Prevention is based on various factors which include: donor selection, immunization, product testing and heat treatment of blood products. The second extremely important aspect of treatment in hemophilia is the concept of global assistance, which includes: the treatment of the bleeding episode itself, and an ongoing psycho-social support system. In this paper we suggest some practical treatment schedules for the therapy of bleeding episodes in addition to examining the severe side effects of HIV and Hepatitis viruses. The message which our paper attempts to transmit is that the hemophilic child must be ideally assisted in an exclusively pediatric environment.

Factors determining the patency of femoropopliteal bypass grafts: an analysis of 350 procedures.

In an attempt to define preoperatively assessed factors that might provide prognostic indications of early graft failure, a series of 350 consecutive femoropopliteal bypass operations have been analyzed with regard to various parameters. Data regarding sex, presence of diabetes and coronary heart disease, severity of symptoms, angiographic assessment of outflow vessels, hemodynamic investigations (ankle systolic pressure index and pulse volume recording), graft material and the site of the distal anastomosis were entered into a computer to study the influence of these factors alone and in combination on early patency rates. Among these factors only the ankle systolic pressure index (ASPI) and pulse volume recording (PVR) significantly affected patency and turned out to be of predictive value in graft prognosis. In particular, when these parameters were severely depressed (ASPI less than 0.40 and PVR less than 2) they became a valuable indicator of early graft thrombosis. By combining different variables we were not able to identify a specific pattern of characteristics for the patient whose graft would probably be doomed to occlusion. Considering the scarcity of accurate prognostic indicators in screening subjects from unsuccessful femoropopliteal reconstruction, we believe that a patient should not be a priori excluded from being considered for surgery if his hemodynamic features (ASPI and PVR) are not greatly reduced.

Measurement of vessel width on fundus photographs.

Many factors play a role in determining the width of retinal vessels, based on fundus photographs. We have tried to estimate their influence by comparing experimentally six different measuring methods, taking into account film and developer, intra- and interindividual reproducibility of results under short- and long-term conditions, and training in measuring technique. Our method of choice was tenfold projection of the negatives on a fine-grained wax layer screen and use of a very narrow marker line to be aligned with the vessel borders. Width determinations on pictures of good definition, yet low contrast, repeated by a trained observer on consecutive days, lay with a 95% probability within an interval of about 9 micron on the retina. The width so determined is, of course, relative and only a rough approximation to the absolute width, because of photographic bias, interobserver difference, and uncertainly of the refractive power of the eye. The method appears to be especially suitable for the study of intraindividual variations; however, because of photographic bias, conclusions should be drawn only when based on several pictures.