Individualized dose of anti-thymocyte globulin based on weight and pre-transplantation lymphocyte counts in pediatric patients: a single center experience.

Anti-thymocyte globulin (ATG) has become a standard in preventing GVHD in related and unrelated donor transplantation, but there is no consensus on the best administration schedule. The PARACHUTE trial reported excellent CD4 immune reconstitution (CD4 IR) using a dosing schedule based on the patient's weight and pre-conditioning absolute lymphocyte count (ALC). In 2015 we introduced the PARACHUTE dosing schedule for pediatric patients at our center.

[Monitoring of anticoagulant treatment with Unfractionated Heparin in pediatrics].

Unlabelled: Unfractionated heparin (UFH) is the most widely used anticoagulant in hospitalized patients. The therapeutic range (TR) was defined in adults according to the prolongation of the activated Partial Thromboplastin Time (aPTT). However, the recommendation is to maintain a therapeutic range with anti-factor Xa assay (antiFXa).

American Society of Hematology, ABHH, ACHO, Grupo CAHT, Grupo CLAHT, SAH, SBHH, SHU, SOCHIHEM, SOMETH, Sociedad Panamena de Hematología, Sociedad Peruana de Hematología, and SVH 2023 guidelines for diagnosis of venous thromboembolism and for its management in special populations in Latin America.

Implementation of international guidelines in Latin American settings requires additional considerations (ie, values and preferences, resources, accessibility, feasibility, and impact on health equity). The purpose of this guideline is to provide evidence-based recommendations about the diagnosis of venous thromboembolism (VTE) and its management in children and during pregnancy. We used the GRADE ADOLOPMENT method to adapt recommendations from 3 American Society of Hematology (ASH) VTE guidelines (diagnosis of VTE, VTE in pregnancy, and VTE in the pediatric population).

[Association between von Willebrand disease and angiodysplasia].

Von Willebrand factor (vWf) is a fundamental multimeric plasma glycoprotein in the coagulation process. Its function is to mediate platelet adhesion and to stabilize circulating factor VIII. A functional or quantitative alteration of vWf gives rise to von Willebrand disease (vWD).

Maternal and perinatal outcomes of a venous thromboembolism high-risk cohort using a multidisciplinary treatment approach.

Objective: To evaluate the maternal and perinatal outcomes in a cohort of pregnant women at high risk of venous thromboembolism (VTE).

Methods: Women at high risk of VTE were evaluated in a multidisciplinary program using a complete diagnostic workup, and specific prophylactic or therapeutic treatment.

Results: Women were considered at high risk of VTE in 57% (85/148) because of prior (75) or current (10) thromboembolism, and in 27% (40/148) of the cases due to adverse obstetric history.

[Liver transplantation in hemophilia A. Report of one case].

Due to blood derivative requirements, many patients with hemophilia were exposed to Hepatitis C virus infection (HCV) before the availability of HCV testing. We report a 46-year-old male with Hemophilia A with a hepatitis virus C infection since 2004 causing a cirrhosis. Due to a hepatopulmonary syndrome, he received a liver allograph using a factor VIII replacement protocol, after eradicating the virus C.

[Heavy menstrual bleeding affects quality of life in adolescents].

Introduction: Heavy menstrual bleeding (HMB) occurs in 37% of adolescents and compromise their quality of life.

Objective: To measure the magnitude of the impact of the SME on the quality of life in adolescents.

Patients And Method: We interviewed adolescents diagnosed with HMB between 10 and 18 years old and one of their guardians.

Bleeding risk of surgery and its prevention in patients with inherited platelet disorders.

Excessive bleeding at surgery is a feared complication in patients with inherited platelet disorders. However, very few studies have evaluated the frequency of surgical bleeding in these hemorrhagic disorders. We performed a worldwide, multicentric, retrospective study to assess the bleeding complications of surgery, the preventive and therapeutic approaches adopted, and their efficacy in patients with inherited platelet disorders: the Surgery in Platelet disorders And Therapeutic Approach (SPATA) study.

Factors associated with thrombotic complications in pediatric patients with vascular malformations.

Background And Objectives: Thrombosis is an uncommon disorder in children. Patients with slowflow vascular malformations have higher risk of developing localized intravascular coagulation, which is closely related to the presence of thrombotic events. These episodes cause pain, can be recurrent and determine a clear deterioration in the quality of life.

Prediction of Esophageal Variceal Bleeding in Liver Cirrhosis: Is There a Role for Hemostatic Factors?

Variceal bleeding is a frequent and ominous complication of liver cirrhosis. Fortunately, primary prophylaxis with β blockers or esophageal band ligation effectively reduces the risk of variceal bleeding and its associated mortality. Periodic endoscopic surveillance of esophageal varices (EV) is currently recommended in every patient with cirrhosis for early detection of EV and initiation of primary variceal bleeding prophylaxis is indicated for high-risk patients.

[Correlation between aPTT and antifactor Xa to determine therapeutic ranges for unfractionated heparin].

Background: The therapeutic range (TR) of activated partial thromboplastin time (aPTT) for unfractionated heparin (UFH) dosing was established in the 1970 decade. Since then aPTT determination has changed. Current TR may be sub or supra-therapeutic depending on the reagents of the test, and therefore, responsible for complications of therapy.

[New oral anticoagulant drugs].

Thromboembolic disease (TED) is the leading cause of morbidity and mortality worldwide. The hallmark of oral long-term anticoagulant therapy has been the use of vitamin K antagonists, whose anticoagulant effect is exerted inhibiting vitamin K epoxide reductase. Warfarin and acenocoumarol are the most commonly used.

Management of children with inherited mild bleeding disorders undergoing adenotonsillar procedures.

Objective: To evaluate the incidence of perioperative bleeding among children with mild bleeding disorders who underwent adenotonsillar surgery in our hospital and were treated with our perioperative bleeding prophylaxis protocol.

Methods: Retrospective chart review was aimed at determining the perioperative bleeding rate in children with mild bleeding disorders subjected to our prophylactic protocol while undergoing adenotonsillar procedures. Low von Willebrand factor (LVWF), unspecific platelet function disorders (UPFD) and mild factor VII deficiency were considered as mild bleeding disorders.

[Use of desmopressin in children with inherited platelet dysfunctions undergoing adenotonsillar procedures].

Introduction And Goals: Adenotonsillar surgery represents a major haemostatic challenge in paediatric patients with mild inherited platelet dysfunction. While there are recommendations for perioperative haemostatic management, there are no reports of the outcomes with the different recommendations in these children when undergoing adenotonsillectomy. Our objective was to evaluate the management of perioperative bleeding with desmopressin in children with mild platelet dysfunctions who underwent adenotonsillar surgery in our hospital.

[Introns 1 and 22 inversions and F8 gene sequencing for molecular diagnosis of hemophilia A in Chile].

Background: Hemophilia A is an inherited disorder caused by alterations in factor VIII gene (F8) located on the X-chromosome, the intron 22 inversion being the most common mutation. The rest are predominantly point mutations distributed along this large gene of 26 exons.

Aim: To implement a molecular diagnostic test to detect mutations in the F8 gene in Chilean patients with Hemophilia A.

Diagnosis of mild platelet function disorders. Reliability and usefulness of light transmission platelet aggregation and serotonin secretion assays.

Light transmission platelet aggregation (PA), adapted to measure platelet secretion (PS), is the reference test for diagnosing platelet functional disorders (PFD). Problems with these assays include lack of standardisation, unknown reproducibility and lack of universally accepted diagnostic criteria. We addressed these issues in patients with inherited mucocutaneous bleeding (MCB).

High prevalence of bleeders of unknown cause among patients with inherited mucocutaneous bleeding. A prospective study of 280 patients and 299 controls.

Background And Objectives: Mucocutaneous bleeding (MCB) is the main expression of inherited disorders of primary hemostasis. However, the relative prevalence of these disorders, their clinical differential diagnosis, and the proportion of patients with MCB of unknown cause (BUC) after an initial comprehensive laboratory testing are unknown.

Design And Methods: We studied prospectively 280 consecutive patients with MCB and 299 matched controls, using strict inclusion and exclusion criteria.