[Sickle cell anemia and pregnancy: review of 68 cases in Guadeloupe]. / Drépanocytose et grossesse: revue de 68 observations en Guadeloupe.

Pregnancy in women with major sickle cell syndromes is a high risk maternofetal situation. This descriptive study presents the features and the clinical course of 68 pregnancies in sickle cell women who were delivered in Guadeloupe from January 1(st) 1993 to December 31(st) 1997. Specific complications were observed in all hemoglobin types, but with a severer course in SS women. Painful vaso-occlusive crises were the main causes of hospitalisation (88% of SS pregnancies and 27% of SC pregnancies) associated most often with worsening anemia and / or infection. Acute chest syndrome was observed in all genotypes at any time throughout pregnancy and during the post partum period. One death occurred (a 16 years old SBeta(+)thal woman). Fetal mortality and morbidity were also high, intrauterine growth retardation and fetal death being the most frequent fetal complications. The rates of prematurity (21%) and caesarean section (48%) were higher than in the whole population. Three (3) neonatal deaths occurred. A multidisciplinary and specific approach, vigilance of health care providers and patient compliance are required to manage efficiently pregnancy, delivery and post partum in sickle cell women.

[Analysis of hospitalization of adult sickle-cell patients in Guadeloupe]. / Analyse des hospitalisations chez les patients drépanocytaires adultes en Guadeloupe.

PURPOSE: To determine the characteristics of acute hospitalizations in adult patient with sickle-cell disease in Guadeloupe. METHODS: We retrospectively studied clinical features of adult patients followed up by the "Centre Caribeen de la Drépanocytose" (CCD) in 1996. Data were collected from the medical records of the hospitalized patients and the longitudinal records of the CCD. RESULTS: Sixty-three (25%) of the 251 patients who were followed up by the CCD required hospitalization in 87 cases (1.38 hospitalizations/patient). Mean age of the hospitalized patients was 27.5 years (range 17 to 71 years). Most hospitalizations involved men (29 [31%] vs 34 [22%] for women, P < 0.05), and most were for homozygous patients with sickle-cell anemia: 39 (31%) SS, 19 (18.55%) SC and five (21.75%) S beta thal. A painful vaso-occlusive crisis was noted in 67 episodes. There were nine acute chest syndromes (ACS), six of them occurred following a vaso-occlusive crisis. We noted 39 infectious episodes. The increase in C-reactive protein (> 100 mg/L) was associated with ACS or urinary infection. A patient with renal failure died during septicemia. CONCLUSION: This study confirms the need for prevention of painful crises and other severe complications in patients with sickle-cell disease.

Morphological features of the human umbilical vein in normal, sickle cell trait, and sickle cell disease pregnancies.

Pathological changes often occur in the placenta of women with sickle cell disease (SCD). These alterations are caused by sickling of erythrocytes and vasoocclusion in the placental circulation, leading to regional hypoxia. However, the morphological status of the umbilical cord, which is in close physical association with the placenta, is not documented under such conditions. To explore this, the umbilical vein structure in healthy, sickle cell trait (the heterozygous state), and SCD pregnancies was studied using scanning (SEM) and transmission electron microscopy (TEM). Interestingly, the sickle cell trait umbilical vein architecture was morphologically similar to that in control veins, whereas numerous alterations were seen in the SCD umbilical vein wall. In SEM, the SCD umbilical vein endothelial cells showed atypical morphologies. TEM analysis of the tunica media showed (1) smooth muscle cell proliferation and increase in the thickness of the basement membrane underlying the cells; (2) areas of necrosis; (3) reduplication of the inner elastic lamina. Such features were often seen in sickle patients vasculature at autopsy. Our findings could have importance because tissue hypoxemia is an integral part of vasoocclusion. We conclude that the SCD umbilical vein may be an additional tool for studying vasoocclusion in sickle cell disease.