Tahir Shamsi

Use of recombinant activated factor VII for massive postpartum hemorrhage

Objective. We hypothesised that patients with massive postpartum hemorrhage (PPH), defined as blood loss >1,500 ml, may benefit from the use of activated recombinant factor VII (rFVIIa). Design. Retrospective cohort study. Setting. Department of Obstetrics & Gynaecology, Dow University of Health Sciences. Population. Thirty‐four women with a diagnosis of massive PPH. Methods. All patients with PPH who were admitted to the Department of Obstetrics & Gynecology and Surgical Intensive Care Unit of Civil Hospital Karachi, Pakistan, were included in the study. From March 2005 to October 2006, 34 patients fulfilled the criteria of massive PPH, of which 18 received rFVIIa to control bleeding, and 16 patients did not. Availability and cost of rFVIIa were the factors in drug allocation. Main outcome measures. Maternal mortality, correction of coagulopathy, the amount of blood products transfused and preservation of fertility. Results. Patients receiving rFVIIa had lower maternal mortality (5/18, 28% versus 8/16, 50%, OR: 0.04 (0.002, 0.83)), and received a lower number of packed red cell transfusions (4.0±4.46 versus 9.61±6.7, p value 0.007), against the comparison group. Patients receiving rFVIIa had lower activated partial thromboplastin (median: 13.0; 25–75th percentile: −25.0, −8.0, signed rank p<0.0001), and lower prothrombin times (median: −8.8; 25–75th percentile: −24.2, −4.8), after administration of drug. There was no significant difference in the rate of hysterectomy between the 2 groups (11/18 (61%) versus 6/16 (38%)). No adverse event attributable to rFVIIa was observed in the study. Conclusion. Activated recombinant factor VII can be a life‐saving drug in patients with massive PPH.

Efficacy of hydroxyurea (HU) in reduction of pack red cell (PRC) transfusion requirement among children having beta-thalassemia major: Karachi HU trial (KHUT).

Background Packed red blood cell (PRC) transfusion with iron chelation is the mainstay of treatment for patients with beta-thalassemia major. Hemoglobin F augmentation is another approach to treat this hemoglobinopathy. This study evaluates the efficacy and safety of hydroxyurea (HU) in minimizing PRC transfusions in patients with beta-thalassemia major. Method Twenty-three patients with beta-thalassemia major received HU at a mean dose of 16u2009mg/kg/d. The results were analyzed at the end of 24 months. Transfusion requirement during the 6 months preceding the study was considered as the control. Result Twenty patients were evaluable after 24 months. The mean volume of PRC transfused was reduced from 2126.45u2009mL to 1489.59u2009mL (P<0.001). The interval between transfusions was increased by 68.7%. Grade I myelosuppression was observed in 4 patients and diarrhea in 2 patients. Conclusions HU was found to be safe in patients with beta-thalassemia major, and resulted in reduction in the transfusion requirements and in increase of the intervals between transfusions.

Women with congenital factor VII deficiency: clinical phenotype and treatment options from two international studies.

A paucity of data exists on the incidence, diagnosis and treatment of bleeding in women with inherited factor VII (FVII) deficiency.

Liver dysfunction in pregnancy: an important cause of maternal and perinatal morbidity and mortality in Pakistan

The objective of this study was to evaluate the maternal and perinatal outcome of women with liver dysfunction during pregnancy. The study involved a prospective observational study design and was carried out at the Dow University of Health Sciences and Civil Hospital Karachi, Pakistan. A total of 800 women, who delivered during the study period from January 2006 to September 2006, constituted the study population. Pregnant women with liver dysfunction underwent evaluation for the aetiology of their liver dysfunction, including screening for hepatitis E. Thirty-five women were identified with liver dysfunction. Fourteen (40%) presented in the second trimester and 21 (60%) presented in the third trimester. Twenty-two of the 35 women (63%) had isolated acute hepatitis E; five (14%) had HELLP (haemolysis, elevated liver enzymes and low platelet count) syndrome; two (6%) had intrahepatic cholestasis of pregnancy (IHCP), two had acute fatty liver of pregnancy (AFLP) and two women had hepatitis A. A specific diagnosis was not reached in two women who died prior to delivery. In women with hepatitis E, the mean values of bilirubin and alanine transaminase were 12 mg/dL and 675 U/L, respectively. Abnormal coagulation parameters were present in 20 (57%) of the women and in 18 of 22 (82%) with hepatitis E. Fulminant hepatic failure (FHF) was seen in four patients. Seven women (20%) underwent caesarean section, 26 (74%) delivered vaginally and two women died undelivered. There were six maternal deaths in the study population; two were due to hepatitis E, one each from HELLP and AFLP, and two remained undiagnosed. The overall perinatal mortality within the group was 43%. Hepatitis E was the most common cause of FHF and maternal death in pregnant women with liver dysfunction.

Successful Management of Acute Catastrophic Juvenile Vaginal Bleeding in Glanzmann's Thromboasthenia by Uterine Tamponade: A Case Report and Review of The Literature.

Glanzmanns thromboasthenia (GT) is a rare platelet disorder, due to membrane defects involving glycoprotein GP IIb/IIIa complex. Symptoms appear in infancy with episodes of bruising, gingival bleeding, epistaxis, or at the time of menarche acute episode of uterine bleeding. Hormonal therapy and antifibrinolytic agents are first-line treatment. Platelet transfusion is given to control hemorrhage when medical treatment fails. However, repeated transfusions may result in development of platelet refractioness, due to development of antibodies against membrane glycoprotein. Activated recombinant FVII is licensed for use in acute control of bleeding in GT. Here we report a case of acute juvenile uterine bleeding at menarche, which responded successfully to uterine tamponade. To our knowledge, this is the first case report on use of balloon tamponade for control of acute catastrophic juvenile bleeding at menarche.