prevalence of hepatitis in patients with congenital coagulation disorders

The present study aimed to determine the prevalence of hepatitis B and C infection, vaccination rates and the development of inhibitors among patients with congenital coagulation disorders. A cross-sectional study "was conducted at the Centre of Congenital Coagulation Disorders in Al-Mansour Pediatric Teaching Hospital in Baghdad in the period between 1st of March to 31st of August 2008. The study sample was comprised of 243 patients with different congenital coagulation disorders who attended the centre during the study period. Data were collected through precoded and structured questionnaire introduced only by the researcher by interviewing the patient or his/her relative or care taker, in addition to recording laboratory investigations from the patient file. The result showed that Hemophilia and Von Willebrand disease constituted the majority [90.1%] of the studied sample, while rare bleeding disorders were 9.9% of the studied sample. Hepatitis C virus [HCV] infection was found in 40.3% of all patients with congenital coagulation disorders. Most patients [82.7%] had received hepatitis B vaccination. Inhibitors were found only in patients with hemophilia A and occurred in 2.5% of them. The prevalence of HCV infection among patients with congenital coagulation disorders was similar to other studies in mediterranean region, there was a high rate of vaccination against hepatitis B virus among the studied sample

Congenital hypofibrinogenemia.

Congenital afibrinogenemia/hypofibrinogenemia is an extremely rare coagulation disorder. We describe a case of congenital hypofibrinogenemia in a 6-year female child, who presented with recurrent ecchymotic spots with no frank bleeding.

Efecto de la restricción motriz temprana sobre el desarrollo dendrítico de las neuronas de Purkinje / Effect of early motor restriction over dendritic development of Purkinje cells

El objetivo principal del presente estudio fue evaluar si la experiencia somatomotriz temprana modifica el desarrollo dendrítico de las células de Purkinje en ratas expuestas a entornos restrictivos durante el período de postlactancia. Ratas albinas de la cepa Sprague-Dawley (machos y hembras) fueron expuestas a restricción de movimiento mediante su ubicación permanente en cajas metálicas de pequeño tamaño (10x10x10 cm) entre los días postnatales 18 y 31. El día 31 se evaluó la conducta exploratoria, y al día siguiente fueron anestesiadas, sacrificadas por decapitación y sus cerebelos procesados con el método de Golgi-Cox-Sholl. Un total de 1.106 células de Purkinje localizadas en la región vermiana fueron dibujadas bajo cámara lúcida (400x), evaluando su desarrollo dendrítico. Los resultados conductuales y morfométricos revelaron que la restricción de movimiento produjo un significativo retraso de la ramificación dendrítica de las células de Purkinje, asociado a una pobre conducta exploratoria

Normas para atendimento aos hemofílicos e portadores de coagulopatias congênitas / Rules for attending patients with hemophilia and congenital blood coagulation disorders

O objetivo primordial do trabalho foi dar as linhas gerais de diagnóstico e conduta frente às diversas intercorrências médicas, odontológicas, psico-sociais e fisioterápicas...

Deficiencia congénita en factor X de la coagulación sanguínea: resultado exitoso del empleo de concentrados de complejo protrombínico en el control de la tendencia hemorrágica en operación cesárea en 2 embarazos / Congenital deficiency of blood coagulation factor X: successful result of prothrombin complex concentrates use in hemorrhagic diathesis in cesarean section in 2 pregnancies

There is little experience in the prevention of the severe hemorrhagic diathesis of factor X coagulation factor deficiency, before surgical procedures. A female with congenital deficiency of factor X that received prothrombin complex concentrates that contained 1000 IU of factor X (16.7 U/I Kg BW) inmediately prior to a cesarean section in two occasions, is reported. Factor X concentration rose from 1 to 25 percent in the first ocassion and from 10 to 63 percent in the second. In both episodes, factor X decreased in the first 24 h of the postoperative period and required new infusions of prothrombin complex concentrates. No episodes of abnormal bleeding were observed. It is concluded that the infusion of prothrombin complex concentrates prevents the hemorrhagic diathesis of factor X deficiency, despite its modest increase in plasma. A initial infusion of 1.000 UI of factor X (16-20 IU/Kg BW), followed by 500 IU (8-10 IU/Kg) every 24 hours is suggested for an adequate management of this condition

Surgery in patients with congenital coagulation disorders.

BACKGROUND. Surgery is occasionally necessary in patients with congenital coagulation disorders. Major surgery for patients with haemophilia was not being done in India until recently. This paper reports the experience of a single referral centre. METHODS. The data of 52 patients who were operated upon were collected from the hospital records retrospectively between 1984 and 1986 and prospectively thereafter. They included the surgical procedure performed, replacement therapy used and complications encountered. RESULTS. Fifty-nine procedures were performed of which 26 were major, 30 minor and 3 were diagnostic angiograms. Blood components produced in the hospital blood bank were commonly used for replacement and primary haemostasis was achieved in all patients. Delayed bleeding due to inadequate factor levels occurred in 12 procedures and was controlled by increasing the factor replacement. One patient died of suspected acute myocardial ischaemia. CONCLUSION. In India surgical procedures can be safely performed in patients with congenital coagulation disorders.