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INTRODUCTION: Sickle cell disease (SCD) is a major risk factor as far as pregnancy and obstetric complications are concerned. It possesses major perinatal and postnatal mortality. The management of pregnancy along with SCD requires a multispecialty team consisting of hematologists, obstetricians, anesthesiologists, neonatologists and intensivists. OBJECTIVES: The objective of this study was to investigate the effect of sickle cell hemoglobinopathy on pregnancy, labor, puerperium, and fetal outcome in the rural and urban localities of Maharashtra, India. MATERIAL AND METHODS: The present study is a comparative retrospective analysis of 225 pregnant women with SCD (genotype AS and SS) and 100 age- and gravida-matched pregnant women with normal hemoglobin (genotype AA) as a control who were treated between June 2013 to June 2015, in Indira Gandhi Government Medical College (IGGMC), Nagpur, India. We analyzed various data regarding obstetrical outcomes and complications in sickle cell disease mothers. RESULTS: Out of 225 pregnant women, 38 (16.89%) were diagnosed with homozygous sickle cell disease (SS group) while 187 (83.11%) were diagnosed with sickle cell trait (AS group). The most common antenatal complications were sickle cell crisis (17; 44.74%) and jaundice (15; 39.47%) in the SS group whereas pregnancy-induced hypertension (PIH) was noted in 33 (17.65%) in the AS group. Intrauterine growth restriction (IUGR) was recorded in 57.89% of the SS group and 21.39% of the AS group. A higher chance of emergency lower segment cesarean section (LSCS; 66.67% in the SS group and 79.09% in the AS group) was recorded as compared with the control group at 32%. CONCLUSION: In order to minimize risks to the mother and fetus and for better outcomes it is prudent to manage pregnancy with SCD vigilantly in the antenatal period. In the antenatal period mothers with this disease should be screened for hydrops or bleeding manifestations such as intracerebral hemorrhage in the fetus. Better feto-maternal outcomes can be achieved by effective multispecialty intervention.
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Patients with paroxysmal nocturnal hemoglobinuria (PNH) may present with thrombosis at unusual sites, of which cerebral sinovenous thrombosis (CSVT) is one and screening for PNH is recommended in this condition. Though many patients diagnosed with PNH develop CSVT, it is unclear how many patients with PNH would present for the first time with thrombosis. We analysed the results of screening for PNH by flowcytometry in our patients with CSVT. The laboratory data of patients referred for thrombophilia and PNH testing in CSVT was examined to assess the frequency of PNH at presentation in these patients. FLAER and CD24 on granulocytes and FLAER and CD14 on monocytes respectively were used to screen the leucocytes for PNH by flowcytometry. The data for Protein C, S and Antithrombin deficiency, antiphospholipid antibodies and the Factor V Leiden mutation was examined and circumstantial risk factors were also assessed. Of the 180 cases of CSVT screened by flowcytometry for PNH, not a single case tested positive. Positivity for anti-phospholipid antibodies was the most common thrombophilic risk factor (5%). Pregnancy was the most common circumstantial risk factor. Our data on FLAER based flowcytometry in the North Indian population with CSVT suggests that PNH is not a common risk factor in our patients with thrombosis at this unusual site.
